Materiały źródłowe

• #1 Granulomatosis with polyangiitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/granulomatosis-with-polyangiitis/symptoms-causes/syc-20351088

  Symptoms of granulomatosis with polyangiitis (GPA) can vary widely from person to person. Symptoms can start quickly, or they can develop over months. […] Early on, GPA can cause general symptoms such as: Fever. Tiredness and not feeling well, also called malaise. Weight loss without a clear reason for it. Muscle aches and pain. Joint stiffness. […] Specific symptoms depend on what parts of the body GPA affects. […] If GPA affects the ears, nose or throat, symptoms can include: Pus-like drainage with crusts from the nose. Sores in the nose or mouth. Stuffiness. Sinus infections. Nosebleeds. Earaches or fluid that drains from the ear. Inflammation of the cartilage of the ears or bridge of the nose. […] If GPA affects the lungs or windpipe, symptoms can include: Coughing, sometimes with bloody phlegm. Shortness of breath or wheezing. A high-pitched breathing sound called stridor. Hoarse voice.

• #1 Granulomatosis with polyangiitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/granulomatosis-with-polyangiitis/symptoms-causes/syc-20351088

  If GPA affects the kidneys, you might not have symptoms. Or you might have symptoms such as: High blood pressure. Leg swelling. Blood in the urine. […] If GPA affects the eyes, symptoms can include: Eye redness. Eye burning or pain. Feeling of something being stuck in the eye. Blurry vision or double vision. Bulging of the eyes. Swelling of the eyelids. […] Other symptoms of GPA can include: Loss of feeling, tingling, or deep aching in the limbs, fingers or toes due to nerve damage. Skin rashes such as purple patches or spots that may be harder to see on Black or brown skin. Digestive issues including stomach pain, diarrhea and bloody stool. […] GPA symptoms can seem like those of some other health conditions. Early symptoms may feel like a cold or flu and can last for weeks or longer. See your healthcare professional if you have a runny nose that doesn’t get better with cold medicines. Be sure to get a healthcare checkup if you have a runny nose along with: Nosebleeds and pus-like fluid. Bloody cough. Any other symptoms of granulomatosis with polyangiitis. […] The disease can become worse quickly. Without treatment, it can lead to other serious health conditions called complications. That’s why it’s important for a healthcare professional to find it and start treatment early.

• #2 Granulomatosis with polyangiitis (GPA)

  https://www.nhs.uk/conditions/granulomatosis-with-polyangiitis/

  Granulomatosis with polyangiitis (GPA) is a rare condition where blood vessels become inflamed, mainly in the ears, nose, sinuses, kidneys and lungs. It can be serious if not treated but medicines can help manage the symptoms. […] Symptoms vary between people, and can be mild or severe. In some people, they come on slowly over many months, and in others they appear suddenly. […] General symptoms may be mild and start before symptoms in specific parts of the body. They can include: high temperature, night sweats, feeling weak and tired, joint pain, loss of appetite, weight loss. […] Other symptoms depend on which parts of your body are affected, so you may not have all the symptoms. […] Symptoms that may affect specific parts of the body include: Ear infection, hearing loss, blocked or runny nose, crusting of blood and mucus in the nose, nosebleeds, sinus pain; Conjunctivitis, swollen eyelids, double vision, blurred vision; A cough that does not go away, coughing up blood, shortness of breath, chest pain, wheezing; High blood pressure, swelling in the legs, kidney failure; Rashes, ulcers, small spots; Tummy pain, diarrhoea, blood in poo. […] Having the symptoms does not definitely mean you have GPA, but it’s important to get checked by a GP. […] If you have been diagnosed with GPA, and have new or returning symptoms, see the specialist treating you.

• #3 Granulomatosis with Polyangiitis – Vasculitis Foundation

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

  Granulomatosis with polyangiitis (GPA) is a form of vasculitis, a family of rare disorders characterized by inflammation of the blood vessels, which can restrict blood flow and damage vital organs and tissues. GPA typically affects the sinuses, lungs, and kidneys but can involve any organs. GPA can worsen rapidly, so early diagnosis and treatment are essential to prevent organ damage or failure. […] In early stages, GPA symptoms resemble a common cold with persistent runny nose and nasal congestion, but it may also include nosebleeds, shortness of breath, coughing up blood, sinus pain, hoarseness or ear symptoms. Besides potential damage to the kidneys and respiratory tract, other serious complications may include vision or hearing loss. […] The symptoms of GPA can vary greatly from person to person. For some, the disease is mild, while for others it may be severe or even life-threatening. GPA symptoms may come on slowly over a period of months or develop rapidly in a matter of days. Early symptoms can include runny nose/nasal congestion, nosebleeds, sinus pain, ear pain, hearing loss, shortness of breath, coughing up blood, hoarseness, and joint pain. Other symptoms include fever, fatigue, muscle pain, loss of appetite/weight loss, eye problems, skin rashes or other lesions, night sweats, numbness in the fingers, toes, or limbs, weakness, and kidney problems.

• #3 Granulomatosis with Polyangiitis – Vasculitis Foundation

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

  Serious complications may occur including bleeding and scarring of the lungs, kidney damage or failure, heart disease, hearing loss, skin ulcers, deep vein thrombosis (blood clot), permanent nerve damage from neuropathy, or damage to the bridge of the nose from weakened cartilage. […] There is no cure for GPA at this time, but early diagnosis and effective treatment can bring the disease into remission, and many patients can lead full, productive lives. Left untreated, GPA can lead to potentially life-threatening organ damage or failure. Even with treatment, many patients will experience periods of relapse, so ongoing medical care is important.

• #5 Granulomatosis with Polyangiitis (GPA) Signs & Symptoms | Rush

  https://www.rush.edu/conditions/granulomatosis-polyangiitis-gpa

  A rare form of vasculitis, granulomatosis with polyangiitis (GPA) causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys. GPA is an autoimmune disease, which means your immune system mistakenly attacks your blood vessels. […] Serious complications, including kidney failure, usually result from a lack of treatment. […] Early symptoms of GPA include the following: A runny, stuffed-up nose, Frequent sinusitis, A lingering fever, Night sweats, Fatigue, Nose bleeds, Chronic ear infections, Joint pain, Loss of appetite, weight loss, Blood in the urine, Eye redness, pain or vision problems, Chest pain, Coughing and bloody phlegm, Shortness of breath and wheezing, Weakness, Skin sores and rashes. […] With treatment, most patients recover; however, GPA returns in about half of all patients. In these cases, the disease usually comes back within two years of stopping treatment.

• #6 Granulomatosis with Polyangiitis (GPA) – Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/types-vasculitis/granulomatosis-with-polyangiitis/

  Granulomatosis with Polyangiitis can affect virtually any site in the body, but it has a predisposition for certain organs. The classic organs involved in Granulomatosis with Polyangiitis are the upper respiratory tract (sinuses, nose, ears, and trachea [the windpipe]), the lungs, and the kidneys. […] Granulomatosis with Polyangiitis involvement of nearly all organs has been described, including the meninges (the layers of protective tissue around the brain and spinal cord), the prostate gland, and the genitourinary tract. In addition to involving specific organs, Granulomatosis with Polyangiitis also commonly results in generalized symptoms of fatigue, low-grade fever, and weight loss. […] Granulomatosis with Polyangiitis is a disease involving granulomatous inflammation, necrosis and vasculitis that most frequently targets the upper respiratory tract, lower respiratory tract, and kidneys.

• #6 Toothache and hearing loss: early symptoms of granulomatosis with polyangiitis (GPA)

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4964197/

  Granulomatosis with polyangiitis is an uncommon condition characterised by vasculitis and associated granuloma formation with a highly specific autoantibody, namely proteinase 3-anti-neutrophil cytoplasmic antibody (ANCA). […] Symptoms can be non-specific, and isolated hearing loss can predate other symptoms by months, leading to lengthy delays in diagnosis and treatment. […] In addition, ear involvement is a common, but non-specific finding that occurs along a spectrum of severity, from mild inflammation to profound and sometimes to irreversible hearing loss. […] After 4 months from her initial symptoms, she became increasingly symptomatic with right-sided facial pain, sweats, loss of taste and smell, and bilateral hearing loss. […] Although not specific, hearing loss can be a presenting feature in an important minority of cases, and can often predate other more characteristic symptoms by several months.

• #7 Granulomatosis with polyangiitis: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000135.htm

  Granulomatosis with polyangiitis (GPA) is a rare disorder in which blood vessels become inflamed. This leads to damage in major organs of the body. […] GPA mainly causes inflammation of blood vessels in the lungs, kidneys, nose, sinuses, and ears. This is called vasculitis or angiitis. Other areas may also be affected in some cases. The disease can be fatal and prompt treatment is important. […] Frequent sinusitis and bloody noses are the most common symptoms. Other early symptoms include a fever that has no clear cause, night sweats, fatigue, and a general ill feeling (malaise). […] Other common symptoms may include: Chronic ear infections, Pain, and sores around the opening of the nose, Cough with or without blood in the sputum, Chest pain and shortness of breath as the disease progresses, Loss of appetite and weight loss, Skin changes such as bruises and ulcers of the skin, Kidney problems, Bloody urine, Eye problems ranging from mild conjunctivitis to severe swelling of the eye.

• #7 Granulomatosis with polyangiitis: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000135.htm

  Without treatment, people with severe forms of this disease can die within a few months. […] With treatment, the outlook for most patients is good. Most people who receive corticosteroids and other medicines that slow the immune response get much better. […] Complications most often occur when the disease is not treated. People with GPA develop tissue damage in the lungs, airways, and the kidneys. Kidney involvement may result in blood in the urine and kidney failure. […] If untreated, kidney failure and possibly death occur in most cases.

• #11 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis): Practice Essentials, Background, Etiology

  https://emedicine.medscape.com/article/332622-overview

  Chronic sinusitis is the most common initial complaint in GPA, but other ENT manifestations may also occur, such as the following: Rhinitis (22%), Epistaxis (11%), Collapse of nasal support, resulting in saddle nose deformity, Nasal crusting, Conductive/sensorineural hearing loss, Stridor, possibly progressing to respiratory compromise, from tracheal or subglottic granulomatous masses. […] Pulmonary involvement in GPA can be asymptomatic, insidious in onset, or severe and fulminant due to involvement of the airways or pulmonary parenchyma. Pulmonary disease may cause any of the following: Pulmonary infiltrates (71%), including nodules, Pulmonary fibrosis, Pulmonary artery hypertension, Cough (34%), Hemoptysis (18%), Chest discomfort (8%), Dyspnea (7%), Stridor/Wheezing, Diffuse alveolar hemorrhage due to alveolar capillaritis (5%-45%), Atelectasis, with dullness on percussion, decreased breath sounds, and crackles on auscultation, Tracheal/subglottic stenosis.

• #11 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis): Practice Essentials, Background, Etiology

  https://emedicine.medscape.com/article/332622-overview

  Kidney disease is present in 18% of patients at initial diagnosis and is usually asymptomatic, but patients may report hematuria, with glomerulonephritis developing in a majority of patients within the first two years of disease onset; presentation is similar to that in glomerulonephritis from other causes and includes asymptomatic hematuria, rise in serum creatinine, and variable proteinuria. Kidney failure is present in 11% at presentation. […] Peripheral nervous system (PNS) involvement may occur in as many as 67% of patients, typically later in the disease course, and includes the following: Mononeuritis multiplex, Sensorimotor polyneuropathy, Cranial nerve palsies, Sensorineural hearing loss. CNS manifestations reflect vasculitis of small to medium-sized vessels of the brain or spinal cord and granulomatous masses that involve the orbit, optic nerve, meninges, or brain.

• #11 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis): Practice Essentials, Background, Etiology

  https://emedicine.medscape.com/article/332622-overview

  Cutaneous findings are variable and nonspecific, and usually affect the lower extremities. Palpable purpura or skin ulcers (45%) in the extremities; ulcerations may resemble pyoderma gangrenosum, Petechiae, vesicles, pustules, hemorrhagic bullae, livedo reticularis, digital necrosis, subungual splinter hemorrhages, and genital ulcers. […] Ophthalmic manifestations can occur from inflammation of these blood vessels or secondary from the formation of granulomas: Conjunctivitis, Episcleritis/scleritis, Uveitis, Optic nerve vasculitis, Nasolacrimal duct obstruction. […] Myalgias, Arthralgias, usually polyarticular and symmetrical, affecting small and medium joints, Arthritis, typically affecting large joints, but rarely deforming. […] The remission rate in GPA ranges from 30-93%, depending on the definition of remission and the remission induction therapy used. With aggressive therapy for active disease, more than 50% of patients with GPA recover renal function and are able to become dialysis independent. Unfortunately, relapse is common in GPA. Typically, up to half of patients with GPA experience relapse within 5 years. The rate (18-40% at 24 mo) and time to first relapse (15-29 mo) varies. Factors associated with relapse include the following: Old age at the time of diagnosis, Seropositivity for PR3-ANCA, Prior history of relapsing disease, Lung involvement prior to remission, Upper respiratory tract involvement prior to remission, Persistence of elevated ANCA titers, particularly PR3-ANCA, Infection with and chronic nasal carriage of Staphylococcus aureus.

• #11 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis): Practice Essentials, Background, Etiology

  https://emedicine.medscape.com/article/332622-overview

  Untreated GPA has a 90% mortality rate within 2 years. Historically, patients with untreated GPA had a mean survival of 5 months from diagnosis; the mortality rate was 82% at 1 year. The introduction of corticosteroids prolonged the median survival by only 7.5 months. With the introduction of rituximab and cyclophosphamide, however, long-term survival has improved dramatically. According to a meta-analysis, with current treatments, the 5-year survival rate ranges from 74-79%. The 1-year mortality rate is still high, around 11% (range, 2.2-25%), depending on disease severity and the intensity of treatment. The most common causes of death in GPA are as follows: Infection, Respiratory and kidney failure, Malignancy, Cardiovascular events.

• #11 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis): Practice Essentials, Background, Etiology

  https://emedicine.medscape.com/article/332622-overview

  Rising PR3-ANCA (C-ANCA) titers may correlate with disease activity in approximately two-thirds of patients. However, the relationship is unreliable; thus, negative PR3-ANCA results do not necessarily exclude the possibility of relapse. Poorer survival is associated with older age, target organ involvement, and target organ damage. Kidney involvement has been consistently shown to confer a poorer prognosis. An absence of kidney involvement is associated with a 100% 5-year survival rate, compared with approximately 70% in individuals with kidney disease. An increased risk of cardiovascular events is also noted. Overall, the 10-year survival rate ranges from 75-88%. Currently, most morbidity in GPA is treatment related. […] In a longitudinal cohort consisting of 158 patients with GPA, from the National Institutes of Health (NIH), 86% of patients experienced permanent damage from their disease. Permanent damage includes the following: End-stage kidney disease, Chronic pulmonary dysfunction, Hearing loss, Destructive sinus disease, Saddle nose deformities, Perforation of the nasal septum, Proptosis, Blindness.

• #11 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis): Practice Essentials, Background, Etiology

  https://emedicine.medscape.com/article/332622-overview

  Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology. GPA is one of the antineutrophil cytoplasmic antibody (ANCA) associated vasculitic disorders, which can affect the ears, nose, throat, respiratory tract, skin, kidneys, and peripheral nervous system. Its hallmark features include necrotizing granulomatous inflammation and pauci-immune vasculitis in small- and medium-sized blood vessels. […] GPA has a spectrum of clinical presentations that include nonspecific symptoms and organ specific involvement. Initial nonspecific constitutional complaints include: Fevers, night sweats, Fatigue, lethargy, Loss of appetite, Weight loss/anorexia, Arthralgias, Rhinosinusitis/nasal crusting, Cough, Dyspnea. These signs and symptoms can be present for weeks to months without evidence of organ involvement. The diagnosis of GPA should be suspected when a patient has constitutional symptoms with evidence of renal involvement, respiratory tract involvement (upper or lower), mononeuropathy, or new skin rashes.

• #12 Granulomatosis with Polyangiitis (GPA) – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

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

  Onset of granulomatosis with polyangiitis may be insidious or acute; the full spectrum of the disease may take years to evolve. Some patients present initially with upper and lower respiratory tract symptoms; at some point later, the kidneys are affected. In other patients, onset of systemic manifestations is relatively acute; several organs and systems, such as the upper respiratory tract, peripheral nervous system (causing multiple mononeuropathy [mononeuritis multiplex]), kidneys (causing glomerulonephritis), and lower respiratory tract (causing hemorrhage, lung nodules, cavities, or a combination), are simultaneously affected. […] Upper respiratory tract: Sinus pain, serosanguineous or purulent discharge, and epistaxis may occur. The mucosa appears granular (like cobblestones) and is friable; ulcers, thick dark crusts, and septal perforation are common. Nasal chondritis can occur with swelling, pain, and collapse of the nasal bridge (saddle nose). Patients may report recurrent sinusitis that has responded inadequately to multiple antibiotic regimens and has required one or more sinus operations before diagnosis.

• #12 Granulomatosis with Polyangiitis (GPA) – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

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

  Lower respiratory tract: Respiratory manifestations are common. Inflammation of the major bronchi and branches can cause localized wheezing, postobstructive pneumonia, and atelectasis. Single or multiple pulmonary nodules, with or without cavitation, and parenchymal infiltrates, sometimes cause symptoms, such as chest pain, shortness of breath, and productive cough. Dyspnea with bilateral infiltrates, with or without hemoptysis, may indicate alveolar hemorrhage and must be evaluated immediately. […] Kidneys: Symptoms and signs of glomerulonephritis develop. Urinary sediment is frequently abnormal, and serum creatinine may increase rapidly. Edema and hypertension may result. Rapidly progressive glomerulonephritis, which is life threatening, can develop. […] Prognosis depends on the severity and extent of disease and at least as much on how rapidly treatment occurs. Use of immunosuppressants for severe disease has dramatically improved prognosis. With treatment, complete remission is possible for approximately 80% of patients, but approximately half of them eventually relapse; relapse may occur during remission maintenance therapy or after treatment is stopped.

• #51 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a necrotizing vasculitis affecting small to medium-sized vessels. GPA is characterized by a pulmonary-renal syndrome associated with otorhinolaryngological manifestations. GPA typically presents with a triad of symptoms as follows: upper respiratory tract, including sinusitis, crusting rhinitis, saddle nose deformity, otitis media, mastoiditis, and hearing loss, and lower respiratory tract, such as lung nodules and alveolar hemorrhage. GPA shows a significant overlap with MPA. Alveolar hemorrhage and crescentic necrotizing glomerulonephritis are also observed in MPA. GPA typically presents with nonspecific symptoms of generalized systemic disease, including fever, malaise, weight loss, polyarthralgia, and myalgia. Studies show that approximately 90% of patients experience upper respiratory tract issues. Early symptoms often include nasal and sinus pain, sinus stuffiness, purulent nasal discharge, nasal ulcerations, epistaxis, and otitis media. Clinical signs such as sinusitis, crusting rhinitis, otitis media, mastoiditis, and hearing loss should alert for GPA. Symptoms include cough, hemoptysis, dyspnea, sometimes pleuritic chest pain, and tracheal obstruction can be noted. Upon presentation, renal involvement is noted in only 10% to 20% of cases, but glomerulonephritis eventually develops in 80% of patients within 2 years of disease onset. The most common manifestation is rapidly progressive crescentic glomerulonephritis, leading to chronic kidney disease or end-stage renal disease. Eye involvement occurs in more than half of the people with the disease. Scleritis and conjunctivitis are the most common manifestations. Dermatologic involvement is reported in 50% to 60% of patients with GPA, with purpura commonly involving the lower extremities. Nervous system involvement is observed in about 30% to 40% of the patients, with peripheral neuropathies being the most common. Arthralgia and myalgia are observed in 70% of patients. GPA is associated with significant morbidity and mortality either due to irreversible organ dysfunction or due to the consequences of intensive/prolonged use of glucocorticoids and immunosuppressive agents. The average life expectancy for a patient with GPA without any treatment is 5 months, with a 1-year survival rate of less than 20%. More than 80% of treated patients have recently survived at least 8 to 9 years. This improvement in survival rates is particularly notable as the disease primarily affects individuals older than 65.

• #59

  https://arthritis.ca/about-arthritis/arthritis-types-(a-z)/types/granulomatosis-with-polyangiitis-(gpa)

  Although the disease can involve any organ system, it most commonly affects the upper respiratory tract (sinuses, nose, ears), lungs, kidneys, skin and nerves. […] The first signs of GPA can be vague and may include fever, loss of appetite, weight loss and fatigue. Almost always there are symptoms related to the upper respiratory tract such as sinus pain, runny nose, bloody fluid from the nose, crusting in the nose, and reduced hearing. A common sign of the disease is the lack of improvement and progressive worsening of these symptoms after multiple courses of antibiotics. […] The lungs are also commonly affected. Symptoms can include a cough with or without bloody phlegm, shortness of breath, and chest discomfort. […] Kidney involvement can present with red, brown or foamy urine, high blood pressure or swelling in the feet and ankles.

• #59

  https://arthritis.ca/about-arthritis/arthritis-types-(a-z)/types/granulomatosis-with-polyangiitis-(gpa)

  Muscle and joint pain or, occasionally, joint swelling are also common. […] Involvement of the skin is quite common and at times will be the first symptom identified. Skin lesions often appear as small red or purple raised areas or blister-like ulcers or nodules that may or may not be painful. […] Some patients experience eye problems. These can include pain, redness, altered vision and protrusion of the eyeball. […] This disease can get worse quickly, so getting diagnosed quickly and onto a treatment plan is very important. […] With appropriate treatment, the outlook is good for people with GPA, and it is not unusual for people to see their symptoms disappear completely. However, about half of all people with the disease may require long-term use of medications to keep the disease in remission.

• #60 Granulomatosis with Polyangiitis (GPA) – Vasculitis UK

  https://www.vasculitis.org.uk/about-vasculitis/gpa-granulomatosis-with-polyangiitis

  Skin rashes, ulcers, and necrosis (death of tissue) […] Eyes red (blood shot) eyes, painful, dry or gritty eyes, visual loss or other changes in vision […] Nerves loss of sensation, weakness, unusual painful symptoms in the hands and feet […] Bowels Diarrhoea, bleeding and abdominal pain. […] The overall prognosis in GPA depends on the amount of damage that has been done to organs, especially the kidneys, when the disease was active. Relapses are common and can occur in up to 40% of patients at 2 years after remission. Your doctor will have to change your immunosuppressive treatment in the event of a relapse. In very few cases, it may be reasonable to simply increase or commence a small dose of prednisolone (steroids). […] The disease may be present for months or years before a diagnosis is made. […] The disease commonly relapses after the initial treatment.

• #61 Pathology Outlines – Granulomatosis with polyangiitis (GPA)

  https://www.pathologyoutlines.com/topic/lungnontumorgranulomatosiswithpolyangiitis.html

  Granulomatosis with polyangiitis (GPA) is a systemic autoimmune vasculitis syndrome commonly involving the lower respiratory tract, the upper respiratory tract and the kidney. Characterized by a necrotizing vasculitis and a systemic granulomatous inflammatory process which replaces the involved tissues. […] Typically manifest in upper / lower respiratory tract and kidney; pulmonary symptoms / signs in the absence of upper respiratory symptoms / signs are unusual. Most common presenting symptoms / signs (local): rhinorrhea, purulent / bloody nasal discharge, oral or nasal ulcers, sinus pain, cough, hemoptysis and chest pain. Most common presenting symptoms / signs (systemic): polyarthralgias, myalgias, fever, malaise, weight loss. […] Can be fatal without treatment. Excellent prognosis with treatment; 5 year survival is > 80%. Increased incidence of infection with higher disease burden, long term exposure to glucocorticoids and kidney involvement. […] Multiple bilateral pulmonary nodules with frequent cavitation; rarely manifests as solitary lung lesion.

• #62 Granulomatosis with polyangiitis – Wikipedia

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

  With corticosteroids and cyclophosphamide, five-year survival is over 80%. Long-term complications are common, mainly chronic kidney failure, hearing loss, and deafness. The risk of relapse is increased in people with GPA who test positive for anti-PR3 ANCA antibodies and is higher than the relapse risk for microscopic polyangiitis. Today, medication toxicity is managed more carefully and long-term remissions are possible. Some affected individuals can lead relatively normal lives and remain in remission for 20+ years after treatment.

• #65 Granulomatosis with Polyangiitis (GPA) – EyeWiki

  https://eyewiki.org/Granulomatosis_with_Polyangiitis_(GPA)

  Renal involvement occurs in 75% of patients with the disease and can present with symptoms of glomerulonephritis. Roughly 60% of patients have musculoskeletal symptoms such as arthralgias, fatigue, and malaise. […] Ocular inflammation can lead to severe ocular morbidity resulting in vision loss in up to 8% of patients, but has been reported as high as 37% of patients with inadequately treated disease. In roughly 15% of patients, ocular symptoms can be the first signs of the disease. […] Scleritis is one of the most common and early appearing presentations of GPA and can occur in roughly 50% of patients. […] The orbit is commonly involved in GPA often due to contiguous disease in the nasal and paranasal sinuses. Orbital presentations include diplopia, swelling, epiphora, and proptosis due to orbital granuloma.

• #65 Granulomatosis with Polyangiitis (GPA) – EyeWiki

  https://eyewiki.org/Granulomatosis_with_Polyangiitis_(GPA)

  Granulomatosis with polyangiitis (GPA), previously known as Wegener Granulomatosis, is an autoimmune disorder characterized by granulomatous necrotizing vasculitis that typically affects small and/or medium sized blood vessels in the orbit, respiratory tract (sinuses, nose, throat, lungs), and kidneys. […] Patients can present with any number of these symptoms across a spectrum of severity. […] Ocular symptoms can include eye pain, diplopia, decreased vision, decreased peripheral vision/ visual field, and redness. Systemic symptoms can include rhinitis, epistaxis, collapsed nasal septum, (aka „saddle-nose” deformity) hearing loss, hemoptysis, shortness of breath, joint pain, neuropathy. […] GPA has the capability to affect nearly any organ system, although the most commonly affected are the sinuses, respiratory tracts and kidneys. The clinical presentation, however, is often nonspecific with varying symptoms from arthralgias to sinusitis. The upper respiratory tract (sinuses, nose, ears, and trachea) is the most often involved system, with up to 85% of patients having disease, and 81% of patients having ENT findings as their initial symptoms. Chronic sinusitis unresponsive to treatment is the most common presentation, with most patients eventually developing lung disease as well.

• #65 Granulomatosis with Polyangiitis (GPA) – EyeWiki

  https://eyewiki.org/Granulomatosis_with_Polyangiitis_(GPA)

  Proptosis is the most common presentation of orbital GPA, and because it is often present concomitantly with respiratory and renal disease, these manifestations together are highly suggestive of the disease. […] GPA can present in a variety of clinical manifestations, and can be broadly categorized into a limited form and a systemic form. The limited form is defined by the absence of renal involvement and is largely isolated to the upper and lower respiratory tracts. The systemic form may involve the kidneys as well as other organs outside of the respiratory tract. […] GPA should be suspected in any person who is between 64-75 years of age presenting with general symptoms of upper respiratory tract and pulmonary involvement as these are the most common symptoms. Pulmonary symptoms and systemic fever malaise weight loss, and myalgia are often clinical signs of the disease.

• #66 (Wegener’s) Granulomatosis with Polyangiitis – Symptoms & Causes

  https://www.uchealth.com/en/conditions/granulomatosis-with-polyangiitis

  GPA may come back even after successful treatment. Continue to follow up with your healthcare provider, even when you are in remission. […] Call your healthcare provider if you have the symptoms listed above. This is especially true if you have tried over-the-counter treatments that seem appropriate, such as allergy medicines, with no success. Always call your healthcare provider if you have changes in vision. Call if you have a cough that produces bloody mucus. These may be symptoms of granulomatosis with polyangiitis (GPA) or another serious health problem.

• #67 GPA Vasculitis | Diagnosis & Disease Info – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/ddi/gpa-vasculitis/

  Symptoms of GPA vasculitis may range from mild to life-threatening and may develop quickly (over a few days) or slowly (over a few months). Symptoms seen early in the disease can include: upper respiratory symptoms resembling a cold, sinus pain, nasal congestion, and a runny nose, nosebleeds, hearing loss or ear pain, hoarseness, feeling short of breath, chest pain, persistent cough that may produce bloody sputum. […] Other symptoms can include: tear in the nasal septum, eye problems, including conjunctivitis, ulceration of the cornea, inflammation of the sclera and membrane covering the sclera, sores on the skin, lack of appetite and weight loss, fever, fatigue and joint pain, night sweats, numbness in the limbs, toes, or fingers, inflammation and narrowing of the trachea. […] Among people diagnosed with GPA vasculitis, 75% may develop kidney problems. They may be asymptomatic or may present with edema in the legs. […] The symptoms of GPA Vasculitis may vary in their severity and onset. In the early stages, patients may experience non-specific symptoms like leg edema. About 75% of people diagnosed with GPA Vasculitis have kidney problems.

• #68 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis) Clinical Presentation: History, Physical Examination

  https://emedicine.medscape.com/article/332622-clinical

  Other common otorhinolaryngologic signs and symptoms include the following: Rhinitis (22%); Epistaxis (1%); Saddle nose deformity (caused by collapse of nasal support); Serous otitis media; Hearing loss; Tracheal or subglottic granulomatous masses can cause stridor; Hoarseness. […] Pulmonary involvement can be asymptomatic, insidious in onset, or severe and fulminant. Pulmonary disease may cause any of the following: Cough (34%); Pulmonary infiltrates (71% of patients); Pulmonary nodules or cavitation; Hemoptysis (18%); Chest discomfort (8%); Dyspnea (7%); Pleural effusion; Diffuse alveolar hemorrhage (DAH) due to alveolar capillaritis (5%-45%). […] Kidney disease most commonly, crescentic necrotizing glomerulonephritis is present in 17% of patients at initial diagnosis and is usually asymptomatic. Painless hematuria in association with normal renal function may occur. Kidney failure is present in 11% at presentation.

• #68 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis) Clinical Presentation: History, Physical Examination

  https://emedicine.medscape.com/article/332622-clinical

  The nervous system is affected in numerous patients. Peripheral nervous system (PNS) involvement may occur in as many as 15% of patients with GPA, typically later in the disease course. PNS manifestations include mononeuritis multiplex, sensorimotor polyneuropathy, and cranial nerve palsies. […] Musculoskeletal symptoms are common. Arthralgias are usually polyarticular and symmetric, affecting small and large joints. Arthritis can also occur, and typically affects large joints, but is rarely deforming. […] The skin is involved in 45% of patients with GPA due to vasculitis. Cutaneous involvement can include the following: Purpura on the lower extremities; Skin ulcers; Urticaria; Livedo reticularis; Nodules; Erythema nodosum, pyoderma gangrenosum; Sweet syndrome. […] Cardiac involvement most often consists of the following: Pericarditis; Coronary vasculitis; Myocarditis; Endocarditis; Conduction system granulomata; Valvular involvement; Myocardial infarction or sudden death (due to necrotizing vasculitis of the coronary vessels); High risk of deep venous thrombosis.

• #69 Granulomatosis with polyangiitis. Wegener granulomatosis

  https://dermnetnz.org/topics/granulomatosis-with-polyangiitis

  Granulomatosis with polyangiitis may complain of various non-specific symptoms especially: […] Symptoms affecting the ear, nose and throat are common, affecting 70% of patients at onset and in 92% of patients during granulomatosis with polyangiitis. […] Lung involvement occurs in 45% of patients with granulomatosis with polyangiitis at presentation and 87% during the disease, most often resulting in: […] Renal involvement occurs in 20% of patients at presentation and in 85% during granulomatosis with polyangiitis. […] Eye symptoms occur in 28-58% of patients with granulomatosis with polyangiitis and are part of the initial presentation in 8 to 16% of patients. […] The skin may be affected in 40-50% of patients with granulomatosis with polyangiitis. […] Musculoskeletal problems occur in 30-50% of patients at presentation and in 65-75% of patients during the disease.

• #69 Granulomatosis with polyangiitis. Wegener granulomatosis

  https://dermnetnz.org/topics/granulomatosis-with-polyangiitis

  Neurological symptoms are rarely a presenting feature but may occur in 22-50% of patients during granulomatosis with polyangiitis. […] Cardiac problems occur in 12-30% of patients with granulomatosis with polyangiitis. […] Granulomatosis with polyangiitis may be slowly or rapidly progressive. There is often no way to predict which patients will continue to have limited, milder forms of the disease and which will go on to more severe progressive, generalised forms of the disease.

• #72 Granulomatosis with polyangiitis – Autoimmune Association

  https://autoimmune.org/disease-information/granulomatosis-with-polyangiitis/

  Granulomatosis with Polyangiitis (GPA) (formerly called Wegener’s Granulomatosis) is a rare autoimmune disease in which blood vessels and other tissues become inflamed. This inflammation limits blood flow to important organs in the body, potentially leading to long-term damage. Disease onset and severity varies among patients, and earlier diagnosis and treatment can prevent life-threatening organ failure. Although the disease can involve any organ system, GPA mainly affects the respiratory tract (sinuses, nose, trachea [windpipe], and lungs) and kidneys. The most common sign of GPA is upper respiratory tract distress such as sinus pain, discolored or bloody fluid from the nose, and nasal ulcers. A common sign of the disease is a constant runny nose or other cold symptoms that do not respond to usual treatment or become increasingly worse.

• #77 Granulomatosis with Polyangiitis (GPA) Characteristics – Vasculitides for Medicine

  https://www.picmonic.com/pathways/medicine/courses/standard/pathology-196/vasculitides-39285/granulomatosis-with-polyangiitis-gpa-characteristics_416

  Granulomatosis with polyangiitis, formerly Wegeners granulomatosis, is a small and medium vessel necrotizing vasculitis that typically affects the nose, lungs, and kidneys. […] The lungs and upper airways are classically involved and can cause chronic sinusitis, otitis media, perforation of the nasal septum, and saddle nose deformity. Individuals also commonly complain of cough and hemoptysis. […] The kidneys are also classically involved and can cause glomerulonephritis with hematuria. […] The lungs and upper airways are classically involved with granulomatosis with polyangiitis and can present with chronic sinusitis, ulcerative lesions of the nose and palate, and nodules and cavities in the lungs. […] The kidneys are classically involved in granulomatosis with polyangiitis and can exhibit a spectrum of diseases, including focal necrosis of glomeruli to diffuse necrosis and parietal cell proliferation to form crescents seen in crescentic glomerulonephritis.

• #78 Audiological Manifestations in Patients with Granulomatosis with Polyangiitis

  https://www.mdpi.com/1648-9144/60/2/267

  Granulomatosis with polyangiitis (GPA), also known as Wegener’s granulomatosis, is a rare, autoimmune, multisystemic disease characterized by vasculitis and necrotizing granuloma, mostly affecting the upper and lower respiratory tract and kidneys. Patients with GPA usually report fatigue, night sweats, weight loss, and respiratory symptoms such as cough, recurrent sinusitis, epistaxis, and hemoptysis. Otorhinolaryngological symptoms may be the first clinical manifestation of the disease, as the upper respiratory tract is often affected. Otologic manifestations occur in 8–65% of patients with GPA. Otologic involvement in GPA can range from serous otitis media to sensorineural hearing loss and can occur during the course of the disease, in addition to being the first and only sign of GPA. Hearing loss in GPA can vary in severity and can affect one or both ears. Delayed diagnosis and therapy can negatively affect the prognosis of hearing. Early detection and appropriate medical management are important to control inflammation and minimize the impact on hearing.

• #78 Audiological Manifestations in Patients with Granulomatosis with Polyangiitis

  https://www.mdpi.com/1648-9144/60/2/267

  92.85% of patients from the GPA group reported hearing-related symptoms: hearing loss, tinnitus, and fullness in the ears. The arithmetic means of all hearing thresholds at frequencies from 125 Hz to 8000 Hz were significantly higher in the GPA group. The results revealed statistically significant differences between the two groups in the Speech Detection Threshold, Speech Recognition Threshold, Speech Discomfort level, and Word Recognition Scores. The frequency of hearing loss, the average hearing thresholds, and speech thresholds were higher in GPA patients than in healthy individuals. The most common type of hearing loss was sensorineural. Audiological assessments should be considered during the routine evaluation of patients with GPA disease to prevent hearing-related disabilities. […] In our study, 92.9% of patients with GPA reported hearing-related symptoms, such as hearing loss, tinnitus, fullness in the ears, and otalgia. Our study revealed that otological symptoms as initial manifestations of GPA have been observed in five patients (35.7% cases): two patients presented with GPA as acute serous otitis media, two others were diagnosed with GPA after persistent purulent inflammation of the middle ear, and in one case, GPA was diagnosed at the onset of sensorineural hearing loss with facial nerve palsy. A study by Fauci et al. revealed that serous otitis media was the initial symptom of GPA in 25% of GPA patients, and hearing loss was the initial symptom in 6% of cases.

• #79 ENT manifestations of Granulomatosis with polyangiitis

  https://www.oaepublish.com/articles/2574-1209.2021.52

  Ear, nose, and throat (ENT) symptoms represent the most frequent manifestations at the onset of granulomatosis with polyangiitis (GPA). […] The burden of diagnosing a limited disease with localized manifestations in the upper aerodigestive tract rests mainly with the otolaryngologist. […] Approximately 63% of GPA patients present with rhinogenic, otologic, or laryngopharyngeal manifestations. Individually, rhinogenic, otologic, and laryngopharyngeal manifestations represent 41%, 16%, and 6% of GPA manifestations, respectively. […] Symptoms of chronic sinusitis are the most common initial complaint in GPA. Sino-nasal involvement in GPA is often misdiagnosed as chronic sinusitis or rhinitis that fails to respond to the initial conventional therapy. […] Stridor, possibly leading to respiratory compromise, from tracheal or subglottic granulomatous masses.

• #80 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-is-Granulomatosis-with-Polyangiitis.aspx

  The symptoms of GPA vary, depending on which part of the body is affected. General symptoms include fatigue, a high temperature, weakness, a loss of appetite, weight loss, and pain in the joints. […] Symptoms affecting specific parts of the body are listed below. […] Blocked or runny nose […] Nosebleeds […] Crusts around the nostrils […] Sinusitis (facial pain) […] Earache […] Hearing loss. […] A persistent cough […] Shortness of breath […] Wheezing […] Chest pain. […] Blood in the urine […] High blood pressure […] Glomerulonephritis (inflammation of the kidneys). […] Rashes […] Lumps […] Necrosis (death of tissue) […] Ulcers […] Small purple spots. […] Conjunctivitis […] Bloodshot eyes […] Swollen eyelids […] Dry eyes […] Loss of vision […] Double vision.

• #80 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-is-Granulomatosis-with-Polyangiitis.aspx

  Stomach pain […] Diarrhea […] Blood in stool. […] Loss of sensation […] Weakness […] Hotness in the hands and feet […] Pins and needles in the hands and feet […] Electric shocks in the hands and feet. […] About half the patients may experience relapses of the condition after the end of the first course of treatment, making a careful follow up mandatory.

• #81 New Granulomatosis with polyangiitis (Werner’s) treatments 2025 | Everyone.org

  https://everyone.org/explore/treatment/?id=45

  Kidney involvement is common and can significantly impact prognosis. Symptoms of renal involvement may initially be subtle but can rapidly progress to more severe manifestations. Patients may notice changes in urine color or appearance, such as dark, tea-colored, or blood-tinged urine (hematuria). Proteinuria (protein in the urine) may occur, although it is typically identified through laboratory testing rather than patient-reported symptoms. […] Joint and muscle involvement is frequently observed in Granulomatosis with polyangiitis. Patients commonly experience joint pain (arthralgia), stiffness, and swelling, typically affecting multiple joints symmetrically. The joints most often involved include the knees, ankles, wrists, and elbows. […] General systemic symptoms are prevalent in Granulomatosis with polyangiitis. Individuals often experience fatigue and malaise, characterized by persistent tiredness and a general sense of feeling unwell. Fever, typically low-grade and persistent, is frequently reported. Unintended weight loss, loss of appetite (anorexia), and night sweats may also occur, reflecting the systemic inflammatory nature of the disease.

• #82 Granulomatosis with Polyangiitis

  https://healthlibrary.gradyhealth.org/Library/TestsProcedures/Neurological/134,118

  GPA may come back even after successful treatment. Continue to follow up with your healthcare provider, even when you are in remission. Ongoing GPA can cause: Collapse of cartilage in the nose, Infections of sinuses and ears, Hearing loss, Kidney damage, Kidney failure, Death. […] Call your healthcare provider if you have the symptoms listed above. This is especially true if you have tried over-the-counter treatments that seem appropriate, such as allergy medicines, with no success. Always call your healthcare provider if you have changes in vision. Call if you have a cough that makes bloody mucus. These may be symptoms of granulomatosis with polyangiitis (GPA) or another serious health problem. […] Most people with GPA first report vague symptoms. Biopsy of affected areas is the only way to know for sure if its GPA. Most people with GPA will find relief of symptoms by taking strong medicines. GPA may come back, even after successful treatment. Ongoing GPA can have serious complications.

• #83 Granulomatosis with Polyangiitis | Physical Therapy Tarzana | Rheumatology Therapeutics Medical Center

  https://arthritisconsult.com/disease-treated/granulomatosis-with-polyangiitis/

  The lungs are affected in most people with GPA, even without the presence of symptoms. When present, the typical symptoms include cough, hemoptysis (coughing up blood), shortness of breath, and chest discomfort. […] Kidney involvement, occurring in more than 75% of patients usually does not cause overt symptoms. […] Pain in the muscles and joints affects two-thirds of people with GPA. […] GPA can affect the eyes in several ways. Symptoms include eye redness, burning, or pain. Double vision or a decrease in vision are serious symptoms requiring immediate medical attention. […] Nearly half of people with GPA develop skin involvement. These often appear as small red or purple raised areas or blister-like lesions, ulcers, or nodules typically of the lower extremities that may or may not be painful. […] Some may experience narrowing of the trachea, these symptoms would voice change, hoarseness, shortness of breath, or cough. The nervous system and heart occasionally may be affected.

• #85 Granulomatosis with Polyangiitis | Saint Luke’s Health System

  https://www.saintlukeskc.org/health-library/granulomatosis-polyangiitis-0

  Granulomatosis with polyangiitis (GPA) is an autoimmune disorder. An autoimmune disorder occurs when the body’s immune system attacks and destroys healthy tissue. This condition causes inflammation, swelling, and irritation in blood vessels and other tissues. This inflammation reduces or stops the flow of blood to organs in the body. The condition mostly affects the respiratory system. This includes the sinuses, nose, windpipe, and lungs. It may also affect the kidneys, or it can damage any organ in the body. […] Most people with GPA first have vague symptoms that may include: Tiredness or exhaustion, Joint pain, Upper respiratory symptoms that dont respond to treatments for allergies or colds: Runny nose, Bloody discharge, Sinus pain, Congestion, Blockage of the eustachian tubes in the ears, Cough, Cough that produces blood, Shortness of breath, Eye infections, redness, burning, or pain, Weakness, Skin sores or ulcers, Hoarseness, Fever, Night sweats.

• #87 Granulomatosis with polyangiitis (Wegener’s granulomatosis)

  https://patient.info/doctor/granulomatosis-with-polyangiitis-wegeners-granulomatosis-pro

  Granulomatosis with polyangiitis (GPA) – formerly known as Wegener’s granulomatosis – is a rare form of vasculitis. It is thought to be an autoimmune inflammatory process affecting endothelial cells. It is a multisystem disease which can affect many parts of the body, categorised by the ELK classification: it most commonly presents with lesions in the upper respiratory tract (E indicating ears/nose/throat, almost 100%), lungs (L most patients) and kidneys (K 75%). Many other areas of the body may also be affected, with joint inflammation occurring in 25-50% of all cases. The sinuses, eyes and skin may also be affected. […] As a multisystem disease, GPA often presents with nonspecific symptoms and can be difficult to recognise in primary care. The delay from onset to diagnosis ranges from 2-20 months.

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