Materiały źródłowe

• #1 Churg-Strauss syndrome: Symptoms, treatment, rash pictures

  https://www.medicalnewstoday.com/articles/churg-strauss-syndrome

  Churg-Strauss syndrome, or eosinophilic granulomatosis with polyangiitis (EGPA), is a rare condition that causes vasculitis, meaning it restricts blood flow. This restriction of blood flow affects multiple organs, especially the lungs. […] Churg-Strauss syndrome has three main characteristics: Hypereosinophilia: This is when a certain type of white blood cells, called eosinophils, cluster within the blood vessels and tissues. Vasculitis: This involves inflammation of the blood vessels, which restricts blood flow. Granulomatosis: This process happens as a result of vasculitis, which causes inflammatory nodular lesions called granulomas to form. […] The lack of blood flow can damage multiple organs, but it particularly affects the lungs. Without treatment, this can have severe effects on the body.

• #1 Churg Strauss Syndrome – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/churg-strauss-syndrome

  People who suffer with EGPA usually have severe asthma that may have developed as an adult. They often have sinus and nose symptoms and may have a number of other symptoms including: rashes, nerve damage including pins and needles or numbness, bowel trouble and blood loss, anaemia, heart problems, muscle and joint pain and tiredness. Symptoms often develop in phases: […] First prodromal phase People with EGPA often suffer allergies, asthma and sinus symptoms starting in their teens and twenties. […] Second eosinophilic phase High levels of eosinophils are found in blood tests, and often in affected organs. […] Final vasculitic phase Inflammation of small and medium vessels (vasculitis) begins, often in the twenties and thirties. Symptoms of tiredness, weight loss and fever often precede this.

• #1 Churg-Strauss Disease Clinical Presentation: History, Physical Examination

  https://emedicine.medscape.com/article/1178795-clinical

  Clinical features of Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), follow two-pronged themes of eosinophilic tissue infiltration or small- and medium-vessel vasculitis. Lanham divided the clinical evolution of EGPA into 3 phases. […] The initial prodromal phase commonly includes nonspecific symptoms of malaise, fever, migrating polyarthralgia, and weight loss along with a severe adult-onset form of asthma that is refractory to conventional treatment. The bronchial asthma is the initial disease manifestation in as many as 80%90% of cases of EGPA. […] The frequency of CSD-related reactive airway disease increases and the severity of the disease worsens, as the vasculitic stage of EGPA is reached. In some cases, an unexpected remission of asthma occurs with the onset of vasculitic manifestations of the second phase of EGPA.

• #1 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537099/

  The second phase is characterized by eosinophilic infiltrates in end organs along with peripheral eosinophilia. Common manifestations include patchy peripheral nodular pulmonary infiltrates, eosinophilic gastroenteritis, and serosal effusions. […] The third phase is characterized by the onset of vasculitis, which can develop 3 to 9 years after the initial onset of asthma. Neurological symptoms are a hallmark of this phase. […] Asthma is nearly ubiquitous in the prodromal phase, reported in 96% to 100% of patients. The mean age of onset is between 35 and 50, typically occurring 3 to 9 years before the onset of vasculitis, despite variability in clinical manifestations. […] Patients with EGPA often present with adult-onset asthma featuring an eosinophilic phenotype, and it is characterized by rhinitis, sinusitis, and nasal polyposis. Chronic rhinitis, the most common extrathoracic manifestation, occurs in about 75% of cases.

• #1 Churg-Strauss Syndrome Symptoms, Causes, and Treatment

  https://www.verywellhealth.com/churg-strauss-sydrome-4174962

  Symptoms emerge during the three stages of Churg-Strauss syndrome and depend on which organs are affected, though most people have asthma or asthma-like symptoms. […] The symptoms of the disorder will correspond to the organ systems where the eosinophils are most concentrated. For example, high numbers of eosinophils in the lungs will produce respiratory symptoms like wheezing, while a high number of the cells in the intestines will cause gastrointestinal symptoms such as abdominal pain. […] Symptoms of Churg-Strauss are grouped into three distinct phases, but people may not experience the phases in order and don’t always experience all three phases. […] The first symptoms (which occur during the prodromal phase) are usually respiratory. These initial symptoms can include: itchy, runny nose, sinus pressure and pain, nasal polyps, coughing or wheezing.

• #1 Churg-Strauss Syndrome

  https://www.aaaai.org/conditions-treatments/related-conditions/churg-strauss-syndrome

  Churg-Strauss syndrome (CSS) or eosinophilic granulomatosis with polyarteritis (EGPA) is a type of vasculitis. These are disorders characterized by inflammation of blood vessels. This inflammation often affects the lungs, skin, nerves and stomach. Asthma is the most common sign of CSS/EGPA. Symptoms may also include other lung problems, allergic rhinitis (hay fever), skin rashes, stomach pain and numbness in the hands and feet. […] CSS/EGPA can result in a variety of symptoms, from mild to very severe and life-threatening. Symptoms often occur in stages, though not all people will develop these stages in the same order. If caught early, later stages can be prevented through treatment. […] The earliest stage is the allergic stage. Common symptoms in this stage include: Coughing, wheezing, shortness of breath Itchy, runny and stuffy nose Sinus pressure and pain that can be associated with nasal polyps

• #1 How You Get a Diagnosis of Churg-Strauss Syndrome

  https://www.webmd.com/lung/churg-strauss-diagnosis

  It can take a few steps to find out whether you have Churg-Strauss syndrome. This rare disease is hard to diagnose based on its typical symptoms like fever, asthma, appetite loss, and tiredness. The trouble is that many conditions can cause these same symptoms. […] Most people with Churg-Strauss syndrome have this breathing problem. But just because you have asthma doesn’t mean you definitely have this condition. Only a small number of people with asthma have it. […] Inflammation from Churg-Strauss syndrome can damage nerves in your hands and feet. This leads to symptoms like numbness, pain, and burning. […] People with Churg-Strauss syndrome often have swelling in their sinuses, called sinusitis. […] High eosinophil levels can mean that you have Churg-Strauss syndrome or asthma. […] In Churg-Strauss syndrome, your immune system — your body’s defense against germs — makes proteins called autoantibodies that attack your own tissues. […] Churg-Strauss syndrome can sometimes damage the heart.

• #1 Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/942

  Eosinophilic granulomatosis with polyangiitis (also known as Churg-Strauss syndrome) is characterised by the pathological triad of tissue eosinophilia, granulomatous inflammation, and vasculitis. […] Typically patients are initially diagnosed with asthma, and then months to years later develop manifestations due to tissue eosinophilia, followed by vasculitis. […] Cardiac involvement causes significant morbidity and mortality. […] Key diagnostic factors include history of asthma, allergic rhinitis, or sinusitis, focal numbness or weakness, nasal discharge or stuffiness, or facial pain, palpable purpura and petechiae, wheeze, haemoptysis, and skin nodules. […] Other diagnostic factors include fatigue, arthralgias, myalgias, shortness of breath or cough, abdominal pain, sensory or motor deficits, tachypnoea, rales, peripheral oedema, orthopnoea, and hepatojugular reflux.

• #1 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537099/

  Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, belongs to a group of diseases characterized by necrotizing vasculitis affecting small- and medium-sized systemic blood vessels, also known as antineutrophil cytoplasmic autoantibody (ANCA)associated vasculitis (AAV). EGPA is distinguished from other conditions in this group by its association with asthma, rhinosinusitis, and peripheral eosinophilia. The manifestations of EGPA are typically present in 2 main phenotypesone characterized by eosinophilic damage and the other by ANCA-associated damage. […] The clinical features of EGPA follow 2 main patternseosinophilic tissue infiltration and small- to medium-vessel vasculitis. The clinical course is characterized by 3 phases, as mentioned below, although not every patient experiences all phases, and they may overlap in an individual’s disease course. In the initial prodromal phase, nonspecific symptoms such as malaise, fever, migrating polyarthralgia, and weight loss are common, often accompanied by a severe adult-onset form of asthma that is refractory to conventional treatment.

• #1 Churg-Strauss Disease Clinical Presentation: History, Physical Examination

  https://emedicine.medscape.com/article/1178795-clinical

  During the second phase of illness, eosinophilic infiltrates are seen in end organs along with peripheral eosinophilia. […] Fever always is present during periods of exacerbation. […] This is a late phase that can present 3 to 9 years after the onset of the initial asthma. It is characterized by systemic vasculitis and neurologic sequela. […] Symptoms related to congestive heart failure ranging from congestive heart failure to cardiogenic shock. […] Neurologic findings are most commonly peripheral polyneuropathy, usually mononeuritis multiplex pattern with motor and sensory deficits unexplainable by a single central lesion. […] Eosinophilic gastroenteritis may result in abdominal pain, weight loss, or bloody diarrhea. […] Acute renal failure, developing rapidly over several weeks, is a characteristic feature of the rapidly progressive glomerulonephritis of EGPA. […] The duration of the first phase, prior to progression of illness, averages about 28 months (range, 472 mo). However, a few patients remain in the prodromal stage of illness for 30 years or more before the second or third phase of EGPA manifests.

• #1 Churg-Strauss Syndrome Symptoms, Causes, and Treatment

  https://www.verywellhealth.com/churg-strauss-sydrome-4174962

  In the second phase, symptoms can be more generalized and include: fatigue, night sweats, abdominal pain, fever. […] During the third phase, the inflammatory symptoms of vasculitis appear and can include: joint pain, weight loss, rashes, numbness or tingling, muscle pain. […] More serious symptoms can develop if particular organs, such as the heart and kidneys, are involved. Many people with Churg-Strauss syndrome will experience neurological symptoms, including polyneuropathy. […] Churg-Strauss syndrome can be fatal if left untreated. Complications from chronic vasculitis can increase a person’s risk of developing serious conditions such as aneurysms, heart disease, and stroke. Diagnosing the condition early and beginning treatment can help reduce inflammation and prevent the progression to more serious symptoms.

• #1 Churg-Strauss Syndrome

  https://www.aaaai.org/conditions-treatments/related-conditions/churg-strauss-syndrome

  The next stage is hypereosinophilia, meaning that there are abnormally high levels of a certain type of white blood cell, the eosinophil, in the bloodstream. Hypereosinophilia can result in: Fever Night sweats Fatigue Weight loss Coughing Abdominal pain Sometimes gastrointestinal bleeding […] The third stage is the systemic vasculitis stage, marked by severe blood vessel inflammation in multiple parts of the body, including: Skin Heart Nerves Muscles Bones Gastrointestinal tract […] Many possible symptoms include: Weight loss Fatigue Joint pains Rashes Numbness Tingling in hands or feet Abdominal pain Cough Chest pain Shortness of breath.

• #1 Churg-Strauss syndrome: Symptoms, treatment, rash pictures

  https://www.medicalnewstoday.com/articles/churg-strauss-syndrome

  Because Churg-Strauss syndrome can target different organs within the body, symptoms can vary widely from person to person. The condition often has three distinct phases, though this does not happen in every case. […] Many symptoms vary, but asthma commonly occurs, affecting 96% of people with Churg-Strauss syndrome. […] In phase one, nonspecific symptoms can include: malaise, fever, weight loss, migrating polyarthralgia, which causes joint pain. Additionally, people can experience a form of asthma that does not respond to conventional treatment methods. This typically develops before other symptoms. […] Phase two is when the eosinophils cluster, often causing symptoms of eosinophilic gastroenteritis, a digestive disease, and serosal effusion, which affects the ears. […] Phase three involves the onset of vasculitis, which generally causes neurological symptoms. Vasculitis may not develop until 39 years after asthma symptoms begin. Common symptoms include pain, tingling, and numbness in the extremities, such as the hands and feet. This eventually leads to weakness and muscle wasting in those areas. […] Churg-Strauss syndrome responds well to treatment, so the outlook for people with the condition is generally positive. However, asthma symptoms may persist and can affect a persons quality of life. […] The condition can recur, with relapse occurring in 2030% of cases.

• #1 Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA) : Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/types-vasculitis/churgstrauss-syndrome-css/

  The third phase of the illness is a vasculitis, which involves the skin, lungs, nerves, kidneys, and other organs. Particular mention should be made of the frequent devastating involvement of the nerves (called mononeuritis multiplex), which produces severe tingling, numbess, shooting pains, and severe muscle wasting/power loss in the hands or feet. […] The following features are consistent with a diagnosis of GPA: asthma, eosinophilia [10% on differential WBC count], mononeuropathy, transient pulmonary infiltrates on chest X-rays, paranasal sinus abnormalities, biopsy containing a blood vessel with extravascular eosinophils. […] EGPA usually responds to prednisone. Initially, high doses of oral prednisone are used in an attempt to get the disease into remission as quickly as possibly (e.g., using oral prednisone 40-60 mg/day). […] With present therapy, constitutional symptoms begin to resolve quite quickly, with gradual improvement in cardiac and renal function, as well as improvement in the pain that results from peripheral nerve involvement.

• #1 Churg-Strauss syndrome

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20193985

  Adult-onset asthma is the most common sign of Churg-Strauss syndrome. The disorder can also cause other problems, such as nasal allergies, sinus problems, rash, gastrointestinal bleeding, and pain and numbness in your hands and feet. […] Churg-Strauss syndrome varies greatly from person to person. Some people have only mild symptoms. Others have severe or life-threatening complications. […] Almost everyone with the condition has asthma, chronic sinusitis and elevated counts of white blood cells called eosinophils. […] Other signs and symptoms might include: Loss of appetite and weight loss, Joint and muscle pain, Abdominal pain and gastrointestinal bleeding, Weakness, fatigue or a general feeling of being unwell, Rash or skin sores, Pain, numbness, and tingling in your hands and feet.

• #1 What Is Eosinophilic Granulomatosis with Polyangiitis: Symptoms, Causes, Diagnosis, Treatments

  https://www.webmd.com/lung/egpa

  EGPA is an extremely rare disease that causes inflammation of your blood vessels. It can damage small and medium-sized blood vessels. Doctors also call it Churg-Strauss vasculitis, eosinophilic granulomatosis with polyangiitis (EGPA), and allergic angiitis. […] The symptoms of EGPA depend on the stage of your disease. There are three. They dont always happen in sequence, and you may not get all three. […] In this phase, you have too many. They can build up in various body parts, including your lungs, digestive tract, and skin. Symptoms include: Weight loss, No appetite, Fever, Night sweats, Joint pain, Asthma, Fatigue, Cough, Belly pain. […] Inflammation narrows your blood vessels. That means less blood reaches your body. Watch for signs like: Weakness, fatigue, or a general bad feeling, Weight loss without trying, Swollen lymph nodes, Skin sores or rashes, Achy, swollen joints, Pain, numbness, or tingling in your feet and hands, Belly pain, Diarrhea, nausea, and vomiting, Blood in your pee or poop. […] Most of the time, symptoms will go away with treatment. Your doctor will call this remission. The best results tend to happen when they know a lot about the condition and start treatment ASAP.

• #1 Churg-Strauss syndrome from an orthopaedic perspective | HKMJ

  https://www.hkmj.org/abstracts/v21n6/565.htm

  Churg-Strauss syndrome, which has been frequently described by physicians in the literature, is a small and medium-sized vessel systemic vasculitis typically associated with asthma, lung infiltrates, and hypereosinophilia. […] The usual presentation is sub-optimised control of asthma together with involvement of other organs such as the heart, skin, and nervous system. […] The symptoms rendered the patient unable to walk. […] Neurological examination revealed decreased sensation over both lower limbs in a glove and stocking distribution. […] The initial symptoms of neuropathy usually include an acute onset of tingling or painful paresthesia in the extremities. […] As the disease progresses, it eventually evolves into a polyneuropathic pattern. […] Muscle weakness, to a variable extent and degree of asymmetry, may also be evident.

• #1 EGPA (Formerly Churg-Strauss Syndrome): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/churg-strauss-syndrome-eosinophilic-granulomatosis-with-polyangiitis-egpa

  Vasculitis (polyangiitis) is usually the last stage (phase 3) of the disease. It can cause: Symptoms of internal bleeding, like blood in your stool or coughing up blood or discolored spots under your skin. Symptoms of nerve inflammation or damage, like numbness and tingling, weakness or nerve pain. Symptoms of heart disease, like irregular heartbeats, faintness, shortness of breath and swelling (edema). […] Polyangiitis will often affect your kidneys as well, but usually without symptoms.

• #1 Peripheral Neuropathy as Initial Manifestation of Churg-Strauss Syndrome

  https://clinmedjournals.org/articles/iaim/international-archives-of-internal-medicine-iaim-3-021.php?jid=iaim

  Laboratory examination revealed white blood count 28.5 × 10^9 (eosinophils 61%); C-reactive protein level was 62 mg/dL (normally 0.0-5.0 mg/dL); creatine kinase and transaminase levels was increased, respectively 62 mg/dL and 93 mg/dL. […] Nerve conduction studies showed a neurogenic suffering of the investigated muscles, compatible with axonal motor nerve distress with blocks of motor conduction and sensory axonal suffering with asymmetric distribution; these findings were suggestive of multiplex mononeuritis. […] Neurological symptoms can be seen in 60-70% of patients with EGPA, secondary only to pulmonary manifestations in the overall frequency of organ involvement. […] An early recognition of this entity is the key in leading to timely treatment and positive outcome. […] The vasculitic phase is heralded by constitutional symptoms (e.g., fever, weight loss, fatigue) and often by an apparently paradoxical improvement of asthma. Peripheral neuropathy is a cardinal feature of this phase, affecting 70% of the patients.

• #1 Eosinophilic Granulomatosis with Polyangiitis – Apfed

  https://apfed.org/about-ead/eosinophilic-granulomatosis-with-polyangiitis/

  The lungs are the most frequently involved organ system involved by EGPA. In addition to asthma, most patients develop pneumonia and have symptoms of shortness of breath and cough. Neurologic symptoms occur in three-quarters of patients, with damage to nerves outside of the brain and spinal cord (mononeuritis multiplex) being the most frequent manifestation. Weakness and tingling or pricking pins and needles sensation (paresthesias) can be disabling and often fail to respond to treatment. […] Half of patients present with rashes of all types that are often biopsied to help establish a diagnosis. The involvement of the heart, usually with cardiomyopathy or myocarditis, often portends a worse prognosis. Gastrointestinal symptoms can occur and usually include abdominal pain and diarrhea; ischemic bowel disease and inflammation of the pancreas or gallbladder are often the culprits.

• #1 Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-023-00958-w

  The vast majority (90%) of patients with EGPA are affected by asthma, which usually arises in adulthood, rarely shows seasonal exacerbations and tends to worsen over time. […] Lung infiltrates are common (40-50%); they are often multiple and migratory and respond to treatment with systemic glucocorticoids. Peripheral neuropathy occurs in 50-70% of patients, has a mononeuritis multiplex pattern, is usually sensory but might also cause motor deficits, and has an axonal damage pattern on nerve conduction studies. Skin lesions are also frequent, but quite heterogeneous, with palpable purpura being the most vasculitis-specific lesion. […] Other organ manifestations that contribute to the clinical phenotype of EGPA include myocarditis and pericarditis, gastroenteritis, renal disease (revealed by proteinuria, haematuria and/or varying degrees of kidney failure) and systemic manifestations such as fatigue, weight loss, myalgia and arthralgia.

• #1 Churg-Strauss Syndrome | Understand the Symptoms & Treatment

  https://www.buoyhealth.com/learn/churg-strauss-syndrome

  Main symptoms: Asthma: In more than 90% of people with EGPA. […] Upper airway disease: This includes repeat sinus infections, nasal polyps, hay fever, or ear infections. These symptoms occur in about 75% of people with EGPA. […] Skin lesions: Tender bumps on elbows, backs of hands, or front of legs. Occurs in over half of people with EGPA. […] GI symptoms (abdominal pain, diarrhea, gastrointestinal bleeding) in the vasculitic phase. […] Difficulty breathing. […] Coughing up blood. […] Heart failure, abnormal electrical activity in the heart, inflammation around the heart. […] Blood clots. […] Nerve issues (numbness, lack of sensation, difficulty moving part of body). […] Muscle aches and joint inflammation during the vasculitic phase. […] Enlarged lymph nodes. […] Treatment is necessary to slow the progression of the disease and may be able to reverse damage done to organs.

• #1 Peripheral Neuropathy as Initial Manifestation of Churg-Strauss Syndrome

  https://clinmedjournals.org/articles/iaim/international-archives-of-internal-medicine-iaim-3-021.php?jid=iaim

  It is characterized by axonal damage on electrophysiological studies and frequently affects the peroneal, tibial, ulnar, and median nerves; the most common pattern is multiplex mononeuritis; sensory deficits and neuropathic pain are also frequent. […] The diagnosis of polyneuropathy is based on clinical and electrophysiologic studies, but precise histology, immunohistochemistry and morphometric studies of the peripheral nerve biopsy may be decisive to make diagnosis. […] Nerve recovery is very slow, and only partial, thus early treatment is essential in preventing extensive damage and reduced quality of life. […] Churg-Strauss syndrome frequently presents polyneuropathy as a complication; since its remission depends on immunosuppression therapy, it is important to recognize it at an early stage.

• #1 Eosinophilic Granulomatosis with Polyangiitis formally Churg Strauss Syndrome — Vasculitis Ireland Awareness (VIA)

  https://www.vasculitis-ia.org/vasculitis-types/eosinophilic-granulomatosis-with-polyangiitis-formally-churg-strauss-syndrome

  Eosinophilic Granulomatosis with Polyangiitis (EGPA), previously known as Churg-Strauss Syndrome, is an inflammatory disease of small and medium sized blood vessels. The symptoms of Eosinophilic Granulomatosis with Polyangiitis (EGPA) can vary widely among individuals and depend on which organs are affected. Common symptoms may include: […] Asthma: Many individuals with EGPA have a history of asthma or may develop asthma-like symptoms such as wheezing, coughing, and shortness of breath. […] Peripheral neuropathy: Nerve damage leading to symptoms such as numbness, tingling, weakness, or pain, typically in the hands and feet. […] It’s important to note that not all individuals with EGPA will experience all of these symptoms, and the severity of symptoms can vary widely among patients. Additionally, EGPA is a progressive condition, meaning symptoms may worsen over time if left untreated. Prompt diagnosis and appropriate management are essential to help alleviate symptoms and prevent complications.

• #1 Peripheral Neuropathy as Initial Manifestation of Churg-Strauss Syndrome

  https://clinmedjournals.org/articles/iaim/international-archives-of-internal-medicine-iaim-3-021.php?jid=iaim

  Peripheral neuropathies are well-known complications of primary systemic vasculitides. In some cases, peripheral neuropathy is one of the first symptoms of systemic vasculitis. Churg-Strauss syndrome is a systemic disorder characterized by asthma, hypereosinophilia, and systemic vasculitis and frequently involves peripheral nerves and skin. […] We describe a case of Churg-Strauss syndrome in a young man (without asthma) having signs of rapidly evolving peripheral neuropathy. […] His medical history revealed no history of asthma, but in the last year some episodes of obstructive-congestive rhinitis were described. […] His neurological examination showed: Left eyelid ptosis, less power in left deltoid and triceps, difficulty in tightening the fist bilaterally, dysesthesia with hypoesthesia from the forearm to the hand, bilaterally.

• #1 Churg-Strauss syndrome | STROKE MANUAL

  https://www.stroke-manual.com/churg-strauss-syndrome/

  Churg-Strauss Syndrome (CSS, now more commonly referred to as Eosinophilic Granulomatosis with Polyangiitis or EGPA) is a rare systemic vasculitis that primarily affects small-sized vessels. CSS typically affects middle-aged individuals. Peripheral nervous system involvement is common, but cerebral infarction or hemorrhage may also occur. Involvement of the CNS is associated with an increased risk for mortality and requires aggressive treatment with steroids and immunosuppressants. The diagnostic evaluation involves a combination of clinical, laboratory, imaging, and histopathological criteria; the condition should be considered in any patient who has asthma and develops eosinophilia and peripheral and/or CNS signs. […] Clinical presentation is characterized by a wide range of symptoms due to its systemic involvement. Systemic symptoms include fever, malaise, and weight loss. Asthma, typically severe and adult-onset, is common. Other symptoms include allergic rhinitis and nasal polyps, chronic sinusitis with nasal obstruction, pulmonary infiltrates (nonfixed), palpable skin purpura and subcutaneous nodules, mononeuritis multiplex causing asymmetric pain, weakness, and sensory loss, cardiovascular symptoms such as myocarditis (heart failure, arrhythmias) and pericarditis (chest pain), gastrointestinal symptoms like abdominal pain, diarrhea, and gastrointestinal bleeding, and glomerulonephritis.

• #1 Eosinophilic granulomatosis with polyangiitis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/eosinophilic-granulomatosis-with-polyangiitis?lang=us

  Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome is characterized by asthma, eosinophilia and small vessel necrotizing vasculitis and involves the lungs and other organs. […] Clinically, patients with EGPA pass through three phases. Although these do provide a general overview of progression, the phases do overlap: […] Almost all patients have symptomatic asthma and chronic sinusitis. Extrapulmonary signs and symptoms will vary depending on end-organ involvement and the phase of the disease and include diarrhea, skin purpura, arthralgias, renal impairment and neurological symptoms. […] The condition generally has a low mortality rate compared with other systemic vasculitides. Cardiac involvement may, however, be a significant contributor to disease-related death and may occur in up to 60% of cases.

• #1 Churg-Strauss syndrome

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20193985

  Churg-Strauss syndrome is rare, and it’s more likely that these symptoms have some other cause. But it’s important that your doctor evaluate them. Early diagnosis and treatment improve the chances of a good outcome. […] Churg-Strauss syndrome can affect many organs, including the lungs, sinuses, skin, gastrointestinal system, kidneys, muscles, joints and heart. Without treatment, the disease can be fatal. […] Complications, which depend on the organs involved, can include: Peripheral nerve damage. Churg-Strauss syndrome can damage the nerves in your hands and feet, leading to numbness, burning and loss of function. […] Heart-related complications of Churg-Strauss syndrome include inflammation of the membrane surrounding your heart, inflammation of the muscular layer of your heart wall, heart attack and heart failure.

• #1 Eosinophilic Granulomatosis with Polyangiitis (EGPA) – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/eosinophilic-granulomatosis-with-polyangiitis-egpa

  Eosinophilic granulomatosis with polyangiitis (EGPA) occurs in up to 20 people/million, depending on the population being studied. Peak age of incidence is between 40 and 50 years. […] The syndrome has 3 phases, which may also overlap: Prodromal: This phase may persist for years. Patients have allergic rhinitis, nasal polyposis, asthma, or a combination. Eosinophilic phase: Peripheral blood and tissue eosinophilia is typical. Clinical presentation, which may resemble Lffler syndrome, includes chronic eosinophilic pneumonia and eosinophilic gastroenteritis. Vasculitic phase: Potentially life-threatening vasculitis develops. Organ dysfunction and systemic symptoms (eg, fever, malaise, weight loss, fatigue) are common in this phase. […] Various organs and systems may be affected: Respiratory: Asthma, often with onset during adulthood, occurs in most patients and tends to be severe and corticosteroid-dependent. Sinusitis is common, but not destructive, without severe necrotizing inflammation. Patients may be short of breath. Transient patchy pulmonary infiltrates are common. Neurologic: Neurologic manifestations are very common. Multiple mononeuropathy (mononeuritis multiplex) occurs in up to three-fourths of patients. Central nervous system involvement is rare but can include hemiparesis, confusion, seizures, and coma, with or without cranial nerve palsies or evidence of cerebral infarction. Cutaneous: The skin is affected in approximately one-half of patients. Nodules and papules appear on extensor surfaces of extremities. They are caused by extravascular palisading granulomatous lesions with central necrosis. Purpura or erythematous papules, due to leukocytoclastic vasculitis with or without prominent eosinophilic infiltration, may develop. Musculoskeletal: Arthralgias, myalgias, or even arthritis can occur. Cardiac: Cardiac involvement, a major cause of mortality, includes heart failure due to myocarditis and endomyocardial fibrosis, coronary artery vasculitis (possibly with myocardial infarction), valvular disorders, and pericarditis. The predominant histopathologic finding is eosinophilic myocarditis. Gastrointestinal: Up to one-third of patients present with gastrointestinal symptoms (eg, abdominal pain, diarrhea, bleeding, acalculous cholecystitis) due to eosinophilic gastroenteritis or mesenteric ischemia due to vasculitis. Renal: The kidneys are affected less often than in other vasculitic disorders associated with antineutrophil cytoplasmic autoantibodies. Typically, pauci-immune (few if any immune complexes), focal segmental necrotizing glomerulonephritis with crescent formation is present; eosinophilic or granulomatous inflammation of the kidneys is rare. […] Renal, cardiac, or neurologic involvement indicates a worse prognosis.

• #1 SciELO Brazil – Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis* Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis*

  https://www.scielo.br/j/abd/a/3sQNtqTK6mLdD9SSDBhsV7F/?lang=en

  About 50% of patients will present with skin changes. These include: 1) hemorrhagic lesions, such as palpable purpuras, petechiae, ecchymosis and hemorrhagic bullae, and 2) dermal or subcutaneous papules and nodules, frequently located on the scalp or distributed bilaterally over the extensor surfaces of the extremities. Other changes that can also be observed less frequently are urticaria, erythematous macules and livedo reticularis. […] CSS has three evolutionary phases. In the first phase, that can last years or decades, only respiratory manifestations such as asthma, rhinitis and nasal polyps occur. In the second phase, there is peripheral and tissue eosinophilia, affecting primarily the lungs, intestines and myocardium. In the third phase, there is systemic vasculitis affecting nerves, kidneys and skin.

• #1 A case of Churg-Strauss syndrome misdiagnosed as dermatitis – Wang – Journal of Xiangya Medicine

  https://jxym.amegroups.org/article/view/4047/html

  Skin lesions are important features of CSS, occur in about 55% of the cases and often occur in the third stage which is the most severe clinical stage. The main types of skin lesions found in CSS include (I) erythematous maculopapules resembling erythema multiforme; (II) hemorrhagic lesions ranging from petechiae to extensive ecchymosis, and these lesions are often associated with wheals, necrosis and ulceration; (III) cutaneous and subcutaneous nodules that are usually deep-seated and tender with a predilection for the scalp. […] The diagnosis of CSS should based on the following 6 classification criteria, and 4 out of 6 criteria should present in the diagnosis of CSS: asthma, eosinophilia greater than 10%, paranasal sinusitis, unfixed pulmonary infiltration and histological proof of vasculitis and mono- or poly-neuropathy. Our patient conformed 5 criteria except for mono- or poly-neuropathy.

• #1 Churg-Strauss syndrome and hemorrhagic vasculitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4211497/

  Churg-Strauss syndrome (CSS) is a rare syndrome characterized by sinusitis, asthma and peripheral eosinophilia. This vasculitic syndrome affects medium and small-sized vessels, the lung being the most commonly affected organ, followed by the skin. […] The constitutional symptoms are weight loss, fatigue, fever, arthralgias and myalgias. […] Asthma is present in 95% cases of CSS, and precedes the vasculitic fase in about 8-10 years. […] Cutaneous manifestations are common (50-66%) in the vasculitic phase, usually like subcutaneous nodules on the extensor surfaces of the arms, hands and legs, but may also emerge as palpable purpura, erythematous maculopapular rash, necrotic bullae, digital ischaemia and hemorrhagic lesions. […] The analytical changes are often normocytic normochromic anemia, eosinophilia, elevated erythrocyte sedimentation rate (ESR), elevated C reactive protein (CRP), hipergammaglobulinemia, elevated IgE, positive rheumatoid factor (low titers), p-ANCA positive, increased urea and creatinine values, gross hematuria and protenuria and erythrocyte cylinders in urine sediment.

• #1 Churg-Strauss syndrome (now called EGPA – Eosinophilic Granulomatosis with Polyangiitis)

  https://patient.info/doctor/churg-strauss-syndrome-pro

  EGPA symptoms will depend on which systems are involved. In any patient with asthma and/or nasal polyposis, any new or worsening general or constitutional symptoms – including fever, joint pain, diffuse muscle pain, major involuntary weight loss, chest pain, palpitations or abdominal pain – may be the first signs of a vasculitis, including EGPA. […] The most prominent symptoms and signs include: […] Cardiac involvement is common. This includes heart failure, myocarditis and myocardial infarction. […] Complications of vasculitis depend on the specific organ system involvement. Cardiac and neurological complications are particularly serious and are more likely in patients with a delayed diagnosis. […] Without treatment, the five-year survival rate is about 25%. However, patient outcomes have dramatically improved in recent years. With appropriate and timely treatment, the survival rate at five years is now 90%. Relapses are not uncommon. […] Five features have been recognised as being associated with an increased risk of mortality: Proteinuria (greater than 1 gm per day), Renal insufficiency (Cr greater than 1.58 mg/dl), Cardiomyopathy, GI tract involvement, CNS involvement.

• #1 Churg-Strauss Syndrome – DoveMed

  https://www.dovemed.com/diseases-conditions/churg-strauss-syndrome

  During this stage the symptoms may include: Chest pain, Abdominal pain due to decreased blood flow to various organs, Perforation of intestines, Appendicitis, Pain in abdomen due gallbladder inflammation, Severe damage to heart muscles due to myocarditis. […] In many cases, damage to the kidney may not be readily apparent. Symptoms due to kidney damage occur after the kidneys have been severely damaged.

• #1 Churg-Strauss syndrome

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20193985

  If Churg-Strauss syndrome affects your kidneys, you can develop glomerulonephritis. This disease hampers your kidneys’ filtering ability, leading to a buildup of waste products in your bloodstream. […] There’s no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms. […] Prednisone, which reduces inflammation, is the most commonly prescribed drug for Churg-Strauss syndrome. Your doctor might prescribe a high dose of corticosteroids or a boost in your current dose of corticosteroids to get your symptoms under control quickly. […] For people with mild symptoms, a corticosteroid alone may be enough. Other people may need to add another drug to help suppress their immune systems. […] Mepolizumab (Nucala) is currently the only drug approved by the U.S. Food and Drug Administration for treatment of Churg-Strauss syndrome.

• #1 Eosinophilic Granulomatosis with Polyangiitis – Bone, Joint, and Muscle Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/bone-joint-and-muscle-disorders/vasculitic-disorders/eosinophilic-granulomatosis-with-polyangiitis

  Any combination of these symptoms may occur. Symptoms may occur in episodes. In subsequent episodes, people may have the same symptoms as the first episode or different ones. […] Inflammation of the kidneys may not cause symptoms until the kidneys malfunction and kidney failure develops. Other complications include heart failure, heart attack, pericarditis, and heart valve disorders.

• #1 Eosinophilic Granulomatosis with Polyangiitis (EGPA) – Rare Awareness Rare Education

  https://rareportal.org.au/rare-disease/eosinophilic-granulomatosis-with-polyangiitis-egpa/

  Symptoms of eosinophilic granulomatosis with polyangiitis (EGPA) vary widely between individuals and change as the disease progresses. […] Asthma is typically the first symptom of EGPA, sometimes occurring years before any other signs. It is usually severe and worsens over time (preexisting and progressive asthma). This may coincide with other allergy-like symptoms such as hay-fever (allergic rhinitis), painful sinuses or face (sinusitis) and runny or blocked nose. Small ball-like bumps can often be found inside the nose or sinus (nasal polyps) early in the disease. Some people also experience flu-like symptoms such as feeling generally unwell with aches, tiredness, fever, and swollen lymph nodes. […] Symptoms typically progress to include signs of organ damage during the eosinophilic and vasculitic phases of EGPA. The symptoms and their severity depend upon which blood vessels and organs are affected. The symptoms of EGPA can be extremely broad and are not just limited to these characteristic symptoms listed below: abdominal pain, nausea, stomach cramps and/or vomiting (gastritis, enteritis, or colitis), skin rashes which may cause scarring, nerve damage (neuropathy) including pain or burning, numbness or tingling, weakness or loss of feeling anywhere in the body, kidney disease which may result in high blood pressure (renal hypertension), pus, blood, small clumps of cells (polyps) or other unusual substances in the lungs (pulmonary infiltrates). The most common sign of this is pneumonia, chest pain and/or heart palpitations. […] There may also be complications associated with EGPA, including life-threatening respiratory issues, kidney failure, blood clots (thrombosis), aneurysms, permanent loss of feeling in hands or feet, stroke, and heart failure.

• #1 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): A Clinical and Therapeutic Approach | Thoracic Key

  https://thoracickey.com/eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome-a-clinical-and-therapeutic-approach/

  Digestive tract involvement of CSS carries a poor prognosis. […] Cardiac involvement is also responsible for a dismal prognosis, as it is the major cause of morbidity in CSS patients and the first cause of their mortality (48 % of deaths). […] Kidney involvement is usually rare, compared to other ANCA-associated vasculitides. […] These symptoms include arthralgias, usually without arthritis, and myalgias: 53 % of our patients had polyarthralgias, predominantly affecting the large joints, whose evolution often paralleled that of the vasculitis, but no joint deformation was observed. […] Eye involvement is rare in CSS.

• #1 Churg-Strauss Syndrome | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/c/churg-strauss-syndrome.html

  Signs of Churg-Strauss Syndrome tend to be general. […] Depending on what organs of the body are affected symptoms can include: […] Flu-like symptoms including a fever and general feeling of being weak and tired […] Loss of appetite […] Weight loss […] Muscle pain […] Asthma or lung abnormalities sometimes occur a year or more before the more generalized, system-wide symptoms of Churg-Strauss appear.

• #1 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) – Vasculitis Syndromes – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.9.4.

  Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) (EGPA) is a multisystem necrotizing granulomatous inflammation with eosinophilic infiltration of various tissues and organs. The lung is the most commonly involved organ, followed by the skin. EGPA can affect any other organ in the cardiovascular, gastrointestinal (GI), renal, and central nervous systems. […] […] 1) General symptoms: Fever, weakness, anorexia, weight loss. […] […] 2) Respiratory manifestations: Asthma (in 95% of patients; usually severe), allergic rhinitis, often nasal polyps, acute or chronic sinusitis, pleural effusion, rarely hemoptysis due to alveolar bleeding. […] […] 3) Nervous system manifestations: Mononeuritis multiplex (in ~70% of patients), symmetric polyneuropathy (~60%), central nervous system symptoms (rare). […]

• #1 What Are the Signs and Symptoms of Churg-Strauss Syndrome?

  https://www.emedicinehealth.com/what_are_the_symptoms_of_churg-strauss_syndrome/article_em.htm

  Allergic rhinitis is a symptom of Churg-Strauss syndrome. […] Because Churg-Strauss syndrome can affect any of the body’s tissues, the signs and symptoms the disease vary widely. […] Signs and symptoms of Churg-Strauss syndrome include the following: Asthma (97% of patients have this symptom, may be severe), allergic rhinitis (runny nose), sinus infection (sinusitis), feeling unwell (malaise), fatigue, flu-like symptoms, weight loss, fever, body aches/muscle pains, cough, including coughing up blood, skin rashes, including blisters and hives, abdominal pain, diarrhea, joint pain, numbness and tingling in extremities (peripheral neuropathy), vision problems, heart problems, including heart attack and heart muscle inflammation. […] Churg-Strauss syndrome has three phases: The first phase consists of asthma with associated with allergic rhinitis (runny nose) and sinus infection (sinusitis). The second phase involves lung and gastrointestinal dysfunction due to high levels of eosinophils (a type of white blood cell) in the tissues and organs. The third phase is the vasculitis phase, which usually develops within 3 years of disease onset and can affect any of the body’s tissues or organ systems, causing most of the complications.

• #1 Churg-Strauss Syndrome | Understand the Symptoms & Treatment

  https://www.buoyhealth.com/learn/churg-strauss-syndrome

  Churg-Strauss syndrome is a rare disease that causes inflammation in the small and medium arteries in the body. […] Most commonly, it causes asthma, sinus inflammation, or a rash. But it can lead to a range of other symptoms. […] Patients may also have tender red bumps on elbows, hands, knees and/or issues with nerves (weakness, numbness, pain). […] When someone has clear phases, they often happen in this order: […] People have asthma, seasonal allergies or hay fever, and other issues related to allergies. […] These cells may invade organs, especially the lungs and digestive tract. This can cause symptoms such as shortness of breath, cough, stomach pain, and diarrhea. […] People in this phase may have fever, weight loss, and fatigue. There are other symptoms depending on which organs are affected. They include red/purple raised skin bumps or pain, numbness, and or weakness in a specific area of the body.

• #1 Churg-Strauss Syndrome: Causes, Symptoms, Treatments, and More

  https://resources.healthgrades.com/right-care/symptoms-and-conditions/churg-strauss-syndrome

  A later CSS stage is the systemic vasculitis stage. Symptoms are related to severe inflammation of the blood vessels in various areas of the body, including: cough, shortness of breath, chest pain, numbness, weight loss, fatigue, abdominal pain, rashes, joint pain, tingling in the extremities. Symptoms vary depending on which organs are involved. CSS can affect the lungs, heart, gastrointestinal tract, kidneys, central nervous system, and skin. […] The outlook for people with CSS has improved in recent years with increased recognition of the condition and advances in treatment. With early diagnosis and prompt treatment, the 5-year survival rate for people with CSS is 90%. It’s important to remember that everyone is different; talk with your doctor about your specific condition. It is possible for relapses to occur. Regular monitoring and closely following your doctor’s treatment plan may help to prevent relapses and improve your quality of life. […] CSS treatment regimens typically involve corticosteroids, immunosuppressants, and biologic medications. Some people may also require additional therapies, such as plasmapheresis or intravenous antibody treatment.

• #1 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537099/

  Even when other systemic manifestations of the disease are controlled with therapy, asthma often remains uncontrolled, significantly impacting the quality of life. […] The clinical course is characterized by 3 phases, as mentioned below, although not every patient experiences all phases, and they may overlap in an individual’s disease course. […] The presence of ANCA alone is not an absolute indicator of vasculitis. In one study, about 47% of patients had vasculitis without seropositivity for ANCA, while 29% tested positive for myeloperoxidase (MPO) ANCA without evidence of vasculitis. Approximately 41% of patients had eosinophilic tissue infiltrates and involvement without vasculitis. […] The estimated prevalence of the disease is approximately 1 to 3 per million adults worldwide. […] The relapse rate is estimated at approximately 20% to 30%, often presenting with fever, joint pain, and constitutional symptoms.

• #1 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765

  To diagnose Churg-Strauss syndrome, doctors usually request several types of tests, including: […] A blood test can detect certain antibodies in your blood that can suggest, but not confirm, a diagnosis of Churg-Strauss syndrome. It can also measure the level of eosinophils, although other diseases, including asthma, can increase the number of these cells. […] X-rays and CT scans can reveal abnormalities in your lungs and sinuses. If you develop signs of heart failure, your doctor may also suggest regular echocardiograms. […] If other tests suggest Churg-Strauss syndrome, you might have a small sample of tissue removed for examination under a microscope. The tissue can come from your lungs or another organ, such as skin or muscle, to confirm or rule out the presence of vasculitis. […] There’s no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms.

• #1

  https://step2.medbullets.com/rheumatology/322113/eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome

  A 35-year-old man presents to his primary care physician for numbness and weakness of the left lower extremity and „cold-like” symptoms. He has also noticed skin changes in the affected leg. His medical history is significant for asthma treated with albuterol. Physical exam is significant for 3/5 strength throughout the lower extremity and decreased touch sensation. There are cutaneous and subcutaneous nodules. Laboratory findings are significant for positive MPO-ANCA/p-ANCA antibodies and a serum eosinophil level of 8000/L. […] Symptoms/physical exam […] disease phases […] eosinophilic […] peripheral blood eosinophilia […] eosinophils infiltrate organs (e.g., lungs) […] vasculitic […] vascular and extravascular granulomatosis […] e.g., fever, weight loss, and skin lesions (tender subcutaneous nodules) […] Other findings […] peripheral neuropathy […] gastrointestinal involvement. […] positive MPO-ANCA/p-ANCA antibodies […] peripheral blood eosinophilia (characteristic finding). […] Improved with immunosuppressive treatment.

• #1 Peripheral polyneuropathy and Churg–Strauss syndrome | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-peripheral-polyneuropathy-churgstrauss-syndrome-S2173580814000418

  Treatment must be aggressive and immediate, using high-dose corticosteroids and/or cytostatic drugs. Initial treatment uses oral corticosteroids (1mg/kg/day) to induce remission as soon as possible. The dose is then progressively decreased to continue with maintenance treatment. In cases of complications or poor response to these drugs, intravenous cyclophosphamide pulses, followed by azathioprine, are to be used.

• #1 Mayo Clinic Health Library – Churg-Strauss syndrome | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20193985

  There’s no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms. […] Prednisone, which reduces inflammation, is the most commonly prescribed drug for Churg-Strauss syndrome. […] For people with mild symptoms, a corticosteroid alone may be enough. Other people may need to add another drug to help suppress their immune systems. […] Mepolizumab (Nucala) is currently the only drug approved by the U.S. Food and Drug Administration for treatment of Churg-Strauss syndrome. […] Early diagnosis and treatment significantly improve the outlook of this condition.

• #1 Churg Strauss Syndrome Houston | Eosinophilic Granulomatosis Cypress TX

  https://www.desaivascular.com/churg-strauss-syndrome-vascular-surgeon-cypress-tx/

  Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis is an extremely rare condition characterized by blood vessel inflammation. It has the potential to permanently harm small and medium-sized blood vessels and disrupt the blood supply to organs and tissues. […] Signs and symptoms of the Churg-Strauss syndrome include: Loss of appetite, Weight loss, Joint and muscle pain, Abdominal pain, Gastrointestinal bleeding, Weakness, Fatigue or feeling generally unwell, Rash or skin sores, Numbness and tingling of the hands and feet. […] Treatments consist of symptomatic management of Churg Strauss syndrome symptoms with medications which include: Corticosteroids: The most given medicine for Churg-Strauss syndrome is prednisone, which lowers inflammation. To get your symptoms under control fast, your doctor may recommend a high dose of corticosteroids and then gradually lower the dosage to minimize the risk of side effects. Immunosuppressive Drugs: The doctor may prescribe azathioprine, cyclophosphamide, or methotrexate in addition to corticosteroids for severe symptoms. Intravenous Immune Globulin (IVIG): This is given as an intravenous (IV) infusion once a month. This option is considered if other treatments are not effective.

• #1 A case of Churg-Strauss syndrome misdiagnosed as dermatitis – Wang – Journal of Xiangya Medicine

  https://jxym.amegroups.org/article/view/4047/html

  Most of CSS patients are sensitive to systemic corticosteroids which are a first-line therapy for CSS. Meanwhile, there are some alternative immunosuppressive drugs such as cyclophosphamide, azathioprine and mycophenolate that have been used to treat the cases resistant to corticosteroids. Combination of corticosteroids and cyclophosphamide is the most efficient therapy of CSS. […] A high dose of oral corticosteroid (prednisolone 1 mg/kg) for 10 days, human immune globulin (0.3 g/kg) for 7 days and some other symptomatic treatments such as anti-infectious, liver protection, nutrition support were used immediately at the time of diagnosis of CSS. However, the patient still aggravated after the therapy, and plasmapheresis was performed one time after the 7 days of diagnosis of CSS.

• #1 Churg-Strauss syndrome from an orthopaedic perspective | HKMJ

  https://www.hkmj.org/abstracts/v21n6/565.htm

  With treatment, mononeuritis multiplex regresses progressively and patients may recover without sequelae. […] Prompt diagnosis and treatment are of paramount importance in managing CSS with neuropathy. […] If medical treatment is delayed, atrophy and weakness of the limbs may be irreversible and will require additional rehabilitation therapy including strengthening exercises, balance training, and ambulation training. […] The clinical presentation is variable. Some people have only mild symptoms, while others experience severe or life-threatening complications. […] The symptoms usually regress quickly with treatment. […] Peripheral neuropathy, as in our case, is common in patients with CSS (65-75%). […] There is speculation that the initial clinical course and the degree of systemic inflammatory involvement may influence long-term functional prognosis.

• #1 Eosinophilic Granulomatosis with Polyangiitis – Vasculitis Foundation

  https://vasculitisfoundation.org/education/vasculitis-types/eosinophilic-granulomatosis-with-polyangiitis/

  Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is a form of vasculitis […] The symptoms of EGPA can range from mild to life threatening, depending on which organs are involved and the extent of disease. Symptoms may vary among individuals. Almost all patients have asthma and/or sinus or nasal polyps, and a higher-than-normal level of white blood cells, which are called eosinophils. Symptoms include: Fever, Fatigue/malaise, Rapid and sudden weight loss, Muscle and joint pain, Skin rashes, Numbness or tingling of the hands or feet, Sudden loss of strength in the hands or feet, Chest pain or palpitations, Increasing shortness of breath or coughing, Abdominal pain, Presence of blood in the stools. […] Because relapses are common with EGPA, follow-up medical care is essential.

• #1 Churg-Strauss syndrome from an orthopaedic perspective | HKMJ

  https://www.hkmj.org/abstracts/v21n6/565.htm

  In this article, we have reviewed the clinical features, diagnostic criteria, and treatment options of CSS. […] Because signs and symptoms are both numerous and at times unassuming, it is notoriously difficult to diagnose CSS at the very initial phase. […] Nonetheless, significant neuropathic involvement may be prevented if a patient receives adequate therapy to induce remission of disease and prevent relapse. […] With treatment, most of the symptoms in any of the three phases can be relieved.

• #1 Learn About Eosinophilic Granulomatosis with Polyangiitis (EGPA) | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/egpa/about-epga

  Eosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare form of vasculitis, characterized by inflammation within small blood vessels. This inflammation results in blood flow restriction, which can cause organ damage throughout the body if left untreated. EGPA is a serious, but treatable disease. Relapses can occur so it is important to maintain ongoing communication with your healthcare provider about management of this chronic disease. […] In most patients, EGPA affects your lungs, but it can also affect other organs in the body. EGPA affects people differently, depending on which organs are affected by the disease as well as the severity. It is referred to by the following phases: […] Phase One. Typically presents as adult-onset asthma. These asthmatic symptoms usually begin years before any other symptoms of EGPA appear. Other early warning symptoms include nasal polyps and allergic rhinitis. […] Phase three. Involves vasculitis (inflammation of a blood vessel or blood vessels) and may include damage to the lungs, skin, nerves, kidneys and other organs.

• #2 Churg-Strauss Syndrome And Social Security Disability Insurance

  https://myphiladelphiadisabilitylawyer.com/disabling-conditions/churg-strauss-syndrome/

  Churg-Strauss syndrome is a rare disorder characterized by an abnormal clustering of white blood cells and the inflammation of blood vessels. The disorder also causes the development of inflammatory lesions known as granulomas. The inflammation can limit blood flow to critical organs and tissue. Most affected individuals have a medical history of allergies, asthma and other pulmonary ailments. […] The disorder can produce a variety of signs and symptoms, which typically appear between the ages of 30 and 50. While some patients only have mild symptoms, others can experience severe, life-threatening complications. […] During the allergic stage, affected individuals may experience asthma and hay fever as well as sinus pain and inflammation. The eosinophilic stage is characterized by an abnormally high percentage of white blood cells, which can cause serious damage to blood vessels and tissue. Signs during the eosinophilic stage depend upon which organs are affected. They may include fever, joint pain and night sweats as well as fatigue, abdominal pain and bleeding. The vasculitic stage is characterized by symptoms like rashes, unintentional weight loss and swollen lymph nodes as well as a generalized feeling of fatigue, chest pains and shortness of breath. You may also experience severe pain and numbness in your hands and feet. Advanced symptoms include severe abdominal pain, diarrhea, nausea and vomiting. The disorder may also cause neurological problems. […] The symptoms and complications of the syndrome must also negatively affect your social functioning, the ability to perform tasks associated with normal daily living or the ability to complete work tasks in a timely manner.

• #2 Eosinophilic granulomatosis with polyangiitis – Wikipedia

  https://en.wikipedia.org/wiki/Eosinophilic_granulomatosis_with_polyangiitis

  Eosinophilic granulomatosis with polyangiitis consists of three stages, but not all patients develop all three stages or progress from one stage to the next in the same order; whereas some patients may develop severe or life-threatening complications such as gastrointestinal involvement and heart disease, some patients are only mildly affected, e.g. with skin lesions and nasal polyps. […] The second stage is characterized by an abnormally high level of eosinophils (a type of white blood cell) in the blood and tissues as a result of abnormal eosinophil proliferation, impaired eosinophil apoptosis, and increased toxicity due to eosinophil metabolic products. […] The symptoms of hypereosinophilia depend on which part of the body is affected, but most often it affects the lungs and digestive tract.

• #2 Churg-Strauss Disease Clinical Presentation: History, Physical Examination

  https://emedicine.medscape.com/article/1178795-clinical

  During the second phase of illness, eosinophilic infiltrates are seen in end organs along with peripheral eosinophilia. […] Fever always is present during periods of exacerbation. […] This is a late phase that can present 3 to 9 years after the onset of the initial asthma. It is characterized by systemic vasculitis and neurologic sequela. […] Symptoms related to congestive heart failure ranging from congestive heart failure to cardiogenic shock. […] Neurologic findings are most commonly peripheral polyneuropathy, usually mononeuritis multiplex pattern with motor and sensory deficits unexplainable by a single central lesion. […] Eosinophilic gastroenteritis may result in abdominal pain, weight loss, or bloody diarrhea. […] Acute renal failure, developing rapidly over several weeks, is a characteristic feature of the rapidly progressive glomerulonephritis of EGPA. […] The duration of the first phase, prior to progression of illness, averages about 28 months (range, 472 mo). However, a few patients remain in the prodromal stage of illness for 30 years or more before the second or third phase of EGPA manifests.

• #2 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): A Clinical and Therapeutic Approach | Thoracic Key

  https://thoracickey.com/eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome-a-clinical-and-therapeutic-approach/

  CSS is most commonly revealed by the onset of vasculitis manifestations mononeuritis multiplex, purpura and general symptoms and eosinophilia, in a previously asthmatic patient. […] General symptoms, primarily fatigue, malaise, fever (58 %) and weight loss, are usually the first and foremost manifestations of this vasculitis. […] Lung involvement, as noted above, is almost universal in CSS patients. Asthma (96100 % of patients) most often precedes systemic vasculitis (mean interval: 8.910.9 years), but sometimes occurs simultaneously or later. […] Peripheral neuropathies are the second most common extrapulmonary manifestations of CSS, mainly mononeuritis multiplex, due to peripheral nerve vasculitis of the vasa nervorum. […] Cutaneous involvement is diverse and frequent (4070 %) in CSS patients.

• #2 Churg-Strauss Syndrome

  https://www.aaaai.org/conditions-treatments/related-conditions/churg-strauss-syndrome

  The next stage is hypereosinophilia, meaning that there are abnormally high levels of a certain type of white blood cell, the eosinophil, in the bloodstream. Hypereosinophilia can result in: Fever Night sweats Fatigue Weight loss Coughing Abdominal pain Sometimes gastrointestinal bleeding […] The third stage is the systemic vasculitis stage, marked by severe blood vessel inflammation in multiple parts of the body, including: Skin Heart Nerves Muscles Bones Gastrointestinal tract […] Many possible symptoms include: Weight loss Fatigue Joint pains Rashes Numbness Tingling in hands or feet Abdominal pain Cough Chest pain Shortness of breath.

• #2 Churg-Strauss syndrome

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20193985

  Adult-onset asthma is the most common sign of Churg-Strauss syndrome. The disorder can also cause other problems, such as nasal allergies, sinus problems, rash, gastrointestinal bleeding, and pain and numbness in your hands and feet. […] Churg-Strauss syndrome varies greatly from person to person. Some people have only mild symptoms. Others have severe or life-threatening complications. […] Almost everyone with the condition has asthma, chronic sinusitis and elevated counts of white blood cells called eosinophils. […] Other signs and symptoms might include: Loss of appetite and weight loss, Joint and muscle pain, Abdominal pain and gastrointestinal bleeding, Weakness, fatigue or a general feeling of being unwell, Rash or skin sores, Pain, numbness, and tingling in your hands and feet.

• #2 What Is Eosinophilic Granulomatosis with Polyangiitis: Symptoms, Causes, Diagnosis, Treatments

  https://www.webmd.com/lung/egpa

  EGPA is an extremely rare disease that causes inflammation of your blood vessels. It can damage small and medium-sized blood vessels. Doctors also call it Churg-Strauss vasculitis, eosinophilic granulomatosis with polyangiitis (EGPA), and allergic angiitis. […] The symptoms of EGPA depend on the stage of your disease. There are three. They dont always happen in sequence, and you may not get all three. […] In this phase, you have too many. They can build up in various body parts, including your lungs, digestive tract, and skin. Symptoms include: Weight loss, No appetite, Fever, Night sweats, Joint pain, Asthma, Fatigue, Cough, Belly pain. […] Inflammation narrows your blood vessels. That means less blood reaches your body. Watch for signs like: Weakness, fatigue, or a general bad feeling, Weight loss without trying, Swollen lymph nodes, Skin sores or rashes, Achy, swollen joints, Pain, numbness, or tingling in your feet and hands, Belly pain, Diarrhea, nausea, and vomiting, Blood in your pee or poop. […] Most of the time, symptoms will go away with treatment. Your doctor will call this remission. The best results tend to happen when they know a lot about the condition and start treatment ASAP.

• #2 Peripheral Neuropathy as Initial Manifestation of Churg-Strauss Syndrome

  https://clinmedjournals.org/articles/iaim/international-archives-of-internal-medicine-iaim-3-021.php?jid=iaim

  It is characterized by axonal damage on electrophysiological studies and frequently affects the peroneal, tibial, ulnar, and median nerves; the most common pattern is multiplex mononeuritis; sensory deficits and neuropathic pain are also frequent. […] The diagnosis of polyneuropathy is based on clinical and electrophysiologic studies, but precise histology, immunohistochemistry and morphometric studies of the peripheral nerve biopsy may be decisive to make diagnosis. […] Nerve recovery is very slow, and only partial, thus early treatment is essential in preventing extensive damage and reduced quality of life. […] Churg-Strauss syndrome frequently presents polyneuropathy as a complication; since its remission depends on immunosuppression therapy, it is important to recognize it at an early stage.

• #2 Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-023-00958-w

  Eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) is a rare small-vessel vasculitis that occurs in patients with asthma and eosinophilia and is histologically characterized by tissue eosinophilia, necrotizing vasculitis and eosinophil-rich granulomatous inflammation. […] EGPA usually evolves through three different phases: a prodromic allergic phase, which can last for several years and is marked by asthma and chronic rhinosinusitis; an eosinophilic phase, during which eosinophilia and end-organ involvement appear; and a vasculitic phase, characterized by clinical manifestations due to small-vessel vasculitis (for example, mononeuritis multiplex and glomerulonephritis). However, these phases often overlap, do not necessarily develop in the aforementioned sequence and some patients do not manifest vasculitic complications.

• #2 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537099/

  The second phase is characterized by eosinophilic infiltrates in end organs along with peripheral eosinophilia. Common manifestations include patchy peripheral nodular pulmonary infiltrates, eosinophilic gastroenteritis, and serosal effusions. […] The third phase is characterized by the onset of vasculitis, which can develop 3 to 9 years after the initial onset of asthma. Neurological symptoms are a hallmark of this phase. […] Asthma is nearly ubiquitous in the prodromal phase, reported in 96% to 100% of patients. The mean age of onset is between 35 and 50, typically occurring 3 to 9 years before the onset of vasculitis, despite variability in clinical manifestations. […] Patients with EGPA often present with adult-onset asthma featuring an eosinophilic phenotype, and it is characterized by rhinitis, sinusitis, and nasal polyposis. Chronic rhinitis, the most common extrathoracic manifestation, occurs in about 75% of cases.

• #2 Churg-Strauss Syndrome | Understand the Symptoms & Treatment

  https://www.buoyhealth.com/learn/churg-strauss-syndrome

  Main symptoms: Asthma: In more than 90% of people with EGPA. […] Upper airway disease: This includes repeat sinus infections, nasal polyps, hay fever, or ear infections. These symptoms occur in about 75% of people with EGPA. […] Skin lesions: Tender bumps on elbows, backs of hands, or front of legs. Occurs in over half of people with EGPA. […] GI symptoms (abdominal pain, diarrhea, gastrointestinal bleeding) in the vasculitic phase. […] Difficulty breathing. […] Coughing up blood. […] Heart failure, abnormal electrical activity in the heart, inflammation around the heart. […] Blood clots. […] Nerve issues (numbness, lack of sensation, difficulty moving part of body). […] Muscle aches and joint inflammation during the vasculitic phase. […] Enlarged lymph nodes. […] Treatment is necessary to slow the progression of the disease and may be able to reverse damage done to organs.

• #2 Churg-Strauss syndrome from an orthopaedic perspective | HKMJ

  https://www.hkmj.org/abstracts/v21n6/565.htm

  Churg-Strauss syndrome, which has been frequently described by physicians in the literature, is a small and medium-sized vessel systemic vasculitis typically associated with asthma, lung infiltrates, and hypereosinophilia. […] The usual presentation is sub-optimised control of asthma together with involvement of other organs such as the heart, skin, and nervous system. […] The symptoms rendered the patient unable to walk. […] Neurological examination revealed decreased sensation over both lower limbs in a glove and stocking distribution. […] The initial symptoms of neuropathy usually include an acute onset of tingling or painful paresthesia in the extremities. […] As the disease progresses, it eventually evolves into a polyneuropathic pattern. […] Muscle weakness, to a variable extent and degree of asymmetry, may also be evident.

• #2 Eosinophilic Granulomatosis with Polyangiitis (EGPA) – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/eosinophilic-granulomatosis-with-polyangiitis-egpa

  Eosinophilic granulomatosis with polyangiitis (EGPA) occurs in up to 20 people/million, depending on the population being studied. Peak age of incidence is between 40 and 50 years. […] The syndrome has 3 phases, which may also overlap: Prodromal: This phase may persist for years. Patients have allergic rhinitis, nasal polyposis, asthma, or a combination. Eosinophilic phase: Peripheral blood and tissue eosinophilia is typical. Clinical presentation, which may resemble Lffler syndrome, includes chronic eosinophilic pneumonia and eosinophilic gastroenteritis. Vasculitic phase: Potentially life-threatening vasculitis develops. Organ dysfunction and systemic symptoms (eg, fever, malaise, weight loss, fatigue) are common in this phase. […] Various organs and systems may be affected: Respiratory: Asthma, often with onset during adulthood, occurs in most patients and tends to be severe and corticosteroid-dependent. Sinusitis is common, but not destructive, without severe necrotizing inflammation. Patients may be short of breath. Transient patchy pulmonary infiltrates are common. Neurologic: Neurologic manifestations are very common. Multiple mononeuropathy (mononeuritis multiplex) occurs in up to three-fourths of patients. Central nervous system involvement is rare but can include hemiparesis, confusion, seizures, and coma, with or without cranial nerve palsies or evidence of cerebral infarction. Cutaneous: The skin is affected in approximately one-half of patients. Nodules and papules appear on extensor surfaces of extremities. They are caused by extravascular palisading granulomatous lesions with central necrosis. Purpura or erythematous papules, due to leukocytoclastic vasculitis with or without prominent eosinophilic infiltration, may develop. Musculoskeletal: Arthralgias, myalgias, or even arthritis can occur. Cardiac: Cardiac involvement, a major cause of mortality, includes heart failure due to myocarditis and endomyocardial fibrosis, coronary artery vasculitis (possibly with myocardial infarction), valvular disorders, and pericarditis. The predominant histopathologic finding is eosinophilic myocarditis. Gastrointestinal: Up to one-third of patients present with gastrointestinal symptoms (eg, abdominal pain, diarrhea, bleeding, acalculous cholecystitis) due to eosinophilic gastroenteritis or mesenteric ischemia due to vasculitis. Renal: The kidneys are affected less often than in other vasculitic disorders associated with antineutrophil cytoplasmic autoantibodies. Typically, pauci-immune (few if any immune complexes), focal segmental necrotizing glomerulonephritis with crescent formation is present; eosinophilic or granulomatous inflammation of the kidneys is rare. […] Renal, cardiac, or neurologic involvement indicates a worse prognosis.

• #2 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): A Clinical and Therapeutic Approach | Thoracic Key

  https://thoracickey.com/eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome-a-clinical-and-therapeutic-approach/

  Digestive tract involvement of CSS carries a poor prognosis. […] Cardiac involvement is also responsible for a dismal prognosis, as it is the major cause of morbidity in CSS patients and the first cause of their mortality (48 % of deaths). […] Kidney involvement is usually rare, compared to other ANCA-associated vasculitides. […] These symptoms include arthralgias, usually without arthritis, and myalgias: 53 % of our patients had polyarthralgias, predominantly affecting the large joints, whose evolution often paralleled that of the vasculitis, but no joint deformation was observed. […] Eye involvement is rare in CSS.

• #2 SciELO Brazil – Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis* Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis*

  https://www.scielo.br/j/abd/a/3sQNtqTK6mLdD9SSDBhsV7F/?lang=en

  About 50% of patients will present with skin changes. These include: 1) hemorrhagic lesions, such as palpable purpuras, petechiae, ecchymosis and hemorrhagic bullae, and 2) dermal or subcutaneous papules and nodules, frequently located on the scalp or distributed bilaterally over the extensor surfaces of the extremities. Other changes that can also be observed less frequently are urticaria, erythematous macules and livedo reticularis. […] CSS has three evolutionary phases. In the first phase, that can last years or decades, only respiratory manifestations such as asthma, rhinitis and nasal polyps occur. In the second phase, there is peripheral and tissue eosinophilia, affecting primarily the lungs, intestines and myocardium. In the third phase, there is systemic vasculitis affecting nerves, kidneys and skin.

• #2 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) – Vasculitis Syndromes – Rheumatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.16.9.4.

  Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) (EGPA) is a multisystem necrotizing granulomatous inflammation with eosinophilic infiltration of various tissues and organs. The lung is the most commonly involved organ, followed by the skin. EGPA can affect any other organ in the cardiovascular, gastrointestinal (GI), renal, and central nervous systems. […] […] 1) General symptoms: Fever, weakness, anorexia, weight loss. […] […] 2) Respiratory manifestations: Asthma (in 95% of patients; usually severe), allergic rhinitis, often nasal polyps, acute or chronic sinusitis, pleural effusion, rarely hemoptysis due to alveolar bleeding. […] […] 3) Nervous system manifestations: Mononeuritis multiplex (in ~70% of patients), symmetric polyneuropathy (~60%), central nervous system symptoms (rare). […]

• #2 SciELO Brazil – Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis* Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis*

  https://www.scielo.br/j/abd/a/3sQNtqTK6mLdD9SSDBhsV7F/?lang=en

  Despite the mild cutaneous manifestations of the patient, there were four undisputable diagnostic criteria present: history of asthma, eosinophilia, neuropathy and vascular eosinophilic infiltrate on the histology. […] CSS prognosis is variable and depends on the initial extension of the disease and the organs affected. Five factors were described that are associated to a higher mortality among patients; which are: creatinine higher than 1.58mg/dL, proteinuria higher than 1 g/day, gastrointestinal involvement, central nervous system involvement and cardiomyopathy. […] Cutaneous manifestations of CSS are one type of clinical presentation for this condition. Recognition of these lesions by the dermatologist was essential for the clinical suspicion and confirmation of diagnosis, that allowed adequate treatment, reducing morbidity and contributing for the prevention of irreversible lesions in vital organs.

• #2 Churg-Strauss syndrome (now called EGPA – Eosinophilic Granulomatosis with Polyangiitis)

  https://patient.info/doctor/churg-strauss-syndrome-pro

  EGPA symptoms will depend on which systems are involved. In any patient with asthma and/or nasal polyposis, any new or worsening general or constitutional symptoms – including fever, joint pain, diffuse muscle pain, major involuntary weight loss, chest pain, palpitations or abdominal pain – may be the first signs of a vasculitis, including EGPA. […] The most prominent symptoms and signs include: […] Cardiac involvement is common. This includes heart failure, myocarditis and myocardial infarction. […] Complications of vasculitis depend on the specific organ system involvement. Cardiac and neurological complications are particularly serious and are more likely in patients with a delayed diagnosis. […] Without treatment, the five-year survival rate is about 25%. However, patient outcomes have dramatically improved in recent years. With appropriate and timely treatment, the survival rate at five years is now 90%. Relapses are not uncommon. […] Five features have been recognised as being associated with an increased risk of mortality: Proteinuria (greater than 1 gm per day), Renal insufficiency (Cr greater than 1.58 mg/dl), Cardiomyopathy, GI tract involvement, CNS involvement.

• #2 Churg-Strauss Syndrome: Causes, Symptoms, Treatments, and More

  https://resources.healthgrades.com/right-care/symptoms-and-conditions/churg-strauss-syndrome

  A later CSS stage is the systemic vasculitis stage. Symptoms are related to severe inflammation of the blood vessels in various areas of the body, including: cough, shortness of breath, chest pain, numbness, weight loss, fatigue, abdominal pain, rashes, joint pain, tingling in the extremities. Symptoms vary depending on which organs are involved. CSS can affect the lungs, heart, gastrointestinal tract, kidneys, central nervous system, and skin. […] The outlook for people with CSS has improved in recent years with increased recognition of the condition and advances in treatment. With early diagnosis and prompt treatment, the 5-year survival rate for people with CSS is 90%. It’s important to remember that everyone is different; talk with your doctor about your specific condition. It is possible for relapses to occur. Regular monitoring and closely following your doctor’s treatment plan may help to prevent relapses and improve your quality of life. […] CSS treatment regimens typically involve corticosteroids, immunosuppressants, and biologic medications. Some people may also require additional therapies, such as plasmapheresis or intravenous antibody treatment.

• #2 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537099/

  Even when other systemic manifestations of the disease are controlled with therapy, asthma often remains uncontrolled, significantly impacting the quality of life. […] The clinical course is characterized by 3 phases, as mentioned below, although not every patient experiences all phases, and they may overlap in an individual’s disease course. […] The presence of ANCA alone is not an absolute indicator of vasculitis. In one study, about 47% of patients had vasculitis without seropositivity for ANCA, while 29% tested positive for myeloperoxidase (MPO) ANCA without evidence of vasculitis. Approximately 41% of patients had eosinophilic tissue infiltrates and involvement without vasculitis. […] The estimated prevalence of the disease is approximately 1 to 3 per million adults worldwide. […] The relapse rate is estimated at approximately 20% to 30%, often presenting with fever, joint pain, and constitutional symptoms.

• #2 Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-023-00958-w

  The vast majority (90%) of patients with EGPA are affected by asthma, which usually arises in adulthood, rarely shows seasonal exacerbations and tends to worsen over time. […] Lung infiltrates are common (40-50%); they are often multiple and migratory and respond to treatment with systemic glucocorticoids. Peripheral neuropathy occurs in 50-70% of patients, has a mononeuritis multiplex pattern, is usually sensory but might also cause motor deficits, and has an axonal damage pattern on nerve conduction studies. Skin lesions are also frequent, but quite heterogeneous, with palpable purpura being the most vasculitis-specific lesion. […] Other organ manifestations that contribute to the clinical phenotype of EGPA include myocarditis and pericarditis, gastroenteritis, renal disease (revealed by proteinuria, haematuria and/or varying degrees of kidney failure) and systemic manifestations such as fatigue, weight loss, myalgia and arthralgia.

• #2 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765

  To diagnose Churg-Strauss syndrome, doctors usually request several types of tests, including: […] A blood test can detect certain antibodies in your blood that can suggest, but not confirm, a diagnosis of Churg-Strauss syndrome. It can also measure the level of eosinophils, although other diseases, including asthma, can increase the number of these cells. […] X-rays and CT scans can reveal abnormalities in your lungs and sinuses. If you develop signs of heart failure, your doctor may also suggest regular echocardiograms. […] If other tests suggest Churg-Strauss syndrome, you might have a small sample of tissue removed for examination under a microscope. The tissue can come from your lungs or another organ, such as skin or muscle, to confirm or rule out the presence of vasculitis. […] There’s no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms.

• #2 Churg-Strauss syndrome

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20193985

  If Churg-Strauss syndrome affects your kidneys, you can develop glomerulonephritis. This disease hampers your kidneys’ filtering ability, leading to a buildup of waste products in your bloodstream. […] There’s no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms. […] Prednisone, which reduces inflammation, is the most commonly prescribed drug for Churg-Strauss syndrome. Your doctor might prescribe a high dose of corticosteroids or a boost in your current dose of corticosteroids to get your symptoms under control quickly. […] For people with mild symptoms, a corticosteroid alone may be enough. Other people may need to add another drug to help suppress their immune systems. […] Mepolizumab (Nucala) is currently the only drug approved by the U.S. Food and Drug Administration for treatment of Churg-Strauss syndrome.

• #3 Churg-Strauss Syndrome

  https://www.aaaai.org/conditions-treatments/related-conditions/churg-strauss-syndrome

  The next stage is hypereosinophilia, meaning that there are abnormally high levels of a certain type of white blood cell, the eosinophil, in the bloodstream. Hypereosinophilia can result in: Fever Night sweats Fatigue Weight loss Coughing Abdominal pain Sometimes gastrointestinal bleeding […] The third stage is the systemic vasculitis stage, marked by severe blood vessel inflammation in multiple parts of the body, including: Skin Heart Nerves Muscles Bones Gastrointestinal tract […] Many possible symptoms include: Weight loss Fatigue Joint pains Rashes Numbness Tingling in hands or feet Abdominal pain Cough Chest pain Shortness of breath.

• #3 Churg-Strauss syndrome

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20193985

  Adult-onset asthma is the most common sign of Churg-Strauss syndrome. The disorder can also cause other problems, such as nasal allergies, sinus problems, rash, gastrointestinal bleeding, and pain and numbness in your hands and feet. […] Churg-Strauss syndrome varies greatly from person to person. Some people have only mild symptoms. Others have severe or life-threatening complications. […] Almost everyone with the condition has asthma, chronic sinusitis and elevated counts of white blood cells called eosinophils. […] Other signs and symptoms might include: Loss of appetite and weight loss, Joint and muscle pain, Abdominal pain and gastrointestinal bleeding, Weakness, fatigue or a general feeling of being unwell, Rash or skin sores, Pain, numbness, and tingling in your hands and feet.

• #3 Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537099/

  The second phase is characterized by eosinophilic infiltrates in end organs along with peripheral eosinophilia. Common manifestations include patchy peripheral nodular pulmonary infiltrates, eosinophilic gastroenteritis, and serosal effusions. […] The third phase is characterized by the onset of vasculitis, which can develop 3 to 9 years after the initial onset of asthma. Neurological symptoms are a hallmark of this phase. […] Asthma is nearly ubiquitous in the prodromal phase, reported in 96% to 100% of patients. The mean age of onset is between 35 and 50, typically occurring 3 to 9 years before the onset of vasculitis, despite variability in clinical manifestations. […] Patients with EGPA often present with adult-onset asthma featuring an eosinophilic phenotype, and it is characterized by rhinitis, sinusitis, and nasal polyposis. Chronic rhinitis, the most common extrathoracic manifestation, occurs in about 75% of cases.

• #3 SciELO Brazil – Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis* Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis*

  https://www.scielo.br/j/abd/a/3sQNtqTK6mLdD9SSDBhsV7F/?lang=en

  About 50% of patients will present with skin changes. These include: 1) hemorrhagic lesions, such as palpable purpuras, petechiae, ecchymosis and hemorrhagic bullae, and 2) dermal or subcutaneous papules and nodules, frequently located on the scalp or distributed bilaterally over the extensor surfaces of the extremities. Other changes that can also be observed less frequently are urticaria, erythematous macules and livedo reticularis. […] CSS has three evolutionary phases. In the first phase, that can last years or decades, only respiratory manifestations such as asthma, rhinitis and nasal polyps occur. In the second phase, there is peripheral and tissue eosinophilia, affecting primarily the lungs, intestines and myocardium. In the third phase, there is systemic vasculitis affecting nerves, kidneys and skin.

• #3 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765

  To diagnose Churg-Strauss syndrome, doctors usually request several types of tests, including: […] A blood test can detect certain antibodies in your blood that can suggest, but not confirm, a diagnosis of Churg-Strauss syndrome. It can also measure the level of eosinophils, although other diseases, including asthma, can increase the number of these cells. […] X-rays and CT scans can reveal abnormalities in your lungs and sinuses. If you develop signs of heart failure, your doctor may also suggest regular echocardiograms. […] If other tests suggest Churg-Strauss syndrome, you might have a small sample of tissue removed for examination under a microscope. The tissue can come from your lungs or another organ, such as skin or muscle, to confirm or rule out the presence of vasculitis. […] There’s no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms.

• #3 Churg-Strauss syndrome

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20193985

  If Churg-Strauss syndrome affects your kidneys, you can develop glomerulonephritis. This disease hampers your kidneys’ filtering ability, leading to a buildup of waste products in your bloodstream. […] There’s no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms. […] Prednisone, which reduces inflammation, is the most commonly prescribed drug for Churg-Strauss syndrome. Your doctor might prescribe a high dose of corticosteroids or a boost in your current dose of corticosteroids to get your symptoms under control quickly. […] For people with mild symptoms, a corticosteroid alone may be enough. Other people may need to add another drug to help suppress their immune systems. […] Mepolizumab (Nucala) is currently the only drug approved by the U.S. Food and Drug Administration for treatment of Churg-Strauss syndrome.

• #4 Churg-Strauss Syndrome

  https://www.aaaai.org/conditions-treatments/related-conditions/churg-strauss-syndrome

  The next stage is hypereosinophilia, meaning that there are abnormally high levels of a certain type of white blood cell, the eosinophil, in the bloodstream. Hypereosinophilia can result in: Fever Night sweats Fatigue Weight loss Coughing Abdominal pain Sometimes gastrointestinal bleeding […] The third stage is the systemic vasculitis stage, marked by severe blood vessel inflammation in multiple parts of the body, including: Skin Heart Nerves Muscles Bones Gastrointestinal tract […] Many possible symptoms include: Weight loss Fatigue Joint pains Rashes Numbness Tingling in hands or feet Abdominal pain Cough Chest pain Shortness of breath.

• #4 SciELO Brazil – Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis* Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis*

  https://www.scielo.br/j/abd/a/3sQNtqTK6mLdD9SSDBhsV7F/?lang=en

  About 50% of patients will present with skin changes. These include: 1) hemorrhagic lesions, such as palpable purpuras, petechiae, ecchymosis and hemorrhagic bullae, and 2) dermal or subcutaneous papules and nodules, frequently located on the scalp or distributed bilaterally over the extensor surfaces of the extremities. Other changes that can also be observed less frequently are urticaria, erythematous macules and livedo reticularis. […] CSS has three evolutionary phases. In the first phase, that can last years or decades, only respiratory manifestations such as asthma, rhinitis and nasal polyps occur. In the second phase, there is peripheral and tissue eosinophilia, affecting primarily the lungs, intestines and myocardium. In the third phase, there is systemic vasculitis affecting nerves, kidneys and skin.

• #5 Churg-Strauss Syndrome

  https://www.aaaai.org/conditions-treatments/related-conditions/churg-strauss-syndrome

  The next stage is hypereosinophilia, meaning that there are abnormally high levels of a certain type of white blood cell, the eosinophil, in the bloodstream. Hypereosinophilia can result in: Fever Night sweats Fatigue Weight loss Coughing Abdominal pain Sometimes gastrointestinal bleeding […] The third stage is the systemic vasculitis stage, marked by severe blood vessel inflammation in multiple parts of the body, including: Skin Heart Nerves Muscles Bones Gastrointestinal tract […] Many possible symptoms include: Weight loss Fatigue Joint pains Rashes Numbness Tingling in hands or feet Abdominal pain Cough Chest pain Shortness of breath.

• #5 SciELO Brazil – Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis* Cutaneous manifestations of Churg-Strauss syndrome: key to diagnosis*

  https://www.scielo.br/j/abd/a/3sQNtqTK6mLdD9SSDBhsV7F/?lang=en

  About 50% of patients will present with skin changes. These include: 1) hemorrhagic lesions, such as palpable purpuras, petechiae, ecchymosis and hemorrhagic bullae, and 2) dermal or subcutaneous papules and nodules, frequently located on the scalp or distributed bilaterally over the extensor surfaces of the extremities. Other changes that can also be observed less frequently are urticaria, erythematous macules and livedo reticularis. […] CSS has three evolutionary phases. In the first phase, that can last years or decades, only respiratory manifestations such as asthma, rhinitis and nasal polyps occur. In the second phase, there is peripheral and tissue eosinophilia, affecting primarily the lungs, intestines and myocardium. In the third phase, there is systemic vasculitis affecting nerves, kidneys and skin.

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