Materiały źródłowe

• #1 Diagnosis and Management of Sarcoidosis | AAFP

  https://www.aafp.org/pubs/afp/issues/2016/0515/p840.html

  Sarcoidosis is a systemic disease of unknown etiology characterized by the presence of noncaseating granulomas in any organ, most commonly the lungs and intrathoracic lymph nodes. A diagnosis of sarcoidosis should be suspected in any young or middle-aged adult presenting with unexplained cough, shortness of breath, or constitutional symptoms, especially among blacks or Scandinavians. Diagnosis relies on three criteria: (1) a compatible clinical and radiologic presentation, (2) pathologic evidence of noncaseating granulomas, and (3) exclusion of other diseases with similar findings, such as infections or malignancy. […] According to an international consensus statement, there are three criteria for diagnosing sarcoidosis: (1) a compatible clinical and radiologic presentation, (2) pathologic evidence of noncaseating granulomas, and (3) exclusion of other diseases with similar findings.

• #1 How Sarcoidosis is Diagnosed – Foundation for Sarcoidosis Research

  https://www.stopsarcoidosis.org/what-is-sarcoidosis/diagnosis/

  Sarcoidosis is a diagnosis of exclusion, meaning that doctors will oftentimes have to rule out other possible diseases before confirming that your symptoms are caused by sarcoidosis. There is no objective test which can easily diagnose sarcoidosis. Numerous exams and tests are required to confirm your diagnosis and help your doctor decide on the best treatment options. […] Your health care provider will take your medical history and perform a physical examination. Medical tests provide additional information. Because many of the symptoms that occur with sarcoidosis also occur with other diseases, your health care provider may work to rule out other possible explanations. […] The average age of diagnosis in the US is 55, though people of all ages can be affected and many are diagnosed anywhere between ages 20 and 70.

• #1 Clinical manifestations and diagnosis of sarcoidosis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-sarcoidosis

  Patients with pulmonary symptoms — Lung or thoracic lymph node involvement occurs in approximately 90 to 95 percent of patients with sarcoidosis. For those with symptoms due to thoracic disease, common presenting complaints include cough, dyspnea, fatigue, and chest pain. The chest pain is usually constant and can be localized to substernal, subcostal, or infrascapular areas. The location and severity of chest pain are not generally related to the location or extent of granulomatous lesions or lymph node enlargement. […] Pulmonary symptoms are frequently accompanied by systemic manifestations such as fatigue, malaise, fever, and weight loss, particularly in the elderly. Systemic inflammation may also contribute to muscle weakness and exercise intolerance. […] Chest imaging — Thoracic imaging is essential in the diagnosis of sarcoidosis because intrathoracic lymph node and/or lung involvement occurs in over 90 percent of patients. In patients with suspected sarcoidosis who have not already received chest imaging, we prioritize imaging before conducting additional noninvasive work-up. Diagnostic imaging begins with a chest radiograph, followed by high-resolution computed tomography (HRCT).

• #1 Clinical manifestations and diagnosis of sarcoidosis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-pulmonary-sarcoidosis

  Patients with pulmonary symptoms — Lung or thoracic lymph node involvement occurs in approximately 90 to 95 percent of patients with sarcoidosis. For those with symptoms due to thoracic disease, common presenting complaints include cough, dyspnea, fatigue, and chest pain. The chest pain is usually constant and can be localized to substernal, subcostal, or infrascapular areas. The location and severity of chest pain are not generally related to the location or extent of granulomatous lesions or lymph node enlargement. […] Pulmonary symptoms are frequently accompanied by systemic manifestations such as fatigue, malaise, fever, and weight loss, particularly in the elderly. Systemic inflammation may also contribute to muscle weakness and exercise intolerance. […] Common extrapulmonary findings and pathognomonic syndromes — Although sarcoidosis frequently involves the lung, approximately 50 percent of patients on careful review also have extrathoracic manifestations at presentation. It should be noted that more sensitive testing, such as 18-fluorodeoxyglucose-positron emission tomography (18F-FDG-PET) scan will reveal an even higher proportion with clinically silent extrapulmonary disease, but 18F-FDG-PET scanning is not recommended for routine evaluation of patients with sarcoidosis.

• #1 Clinical manifestations and diagnosis of sarcoidosis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-sarcoidosis

  Chest radiograph — All patients undergoing evaluation for possible sarcoidosis should receive a chest radiograph. Most patients present with bilateral hilar adenopathy, with or without additional pulmonary involvement. […] The diagnosis of sarcoidosis requires compatible clinical and radiographic manifestations, exclusion of other diseases that may present similarly, and usually histopathologic detection of non-necrotizing granulomas. […] Patients with certain pathognomonic syndromes highly suggestive of sarcoidosis can often defer biopsy after completion of the noninvasive work-up. […] Patients with sarcoidosis who present with asymptomatic bilateral hilar lymph node involvement generally have self-limited disease that can be monitored to ensure stability or resolution. […] The presence of Löggren syndrome has a high diagnostic specificity for sarcoidosis.

• #1 Sarcoidosis – Pulmonary Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pulmonary-disorders/sarcoidosis/sarcoidosis

  Sarcoidosis is most often suspected when hilar adenopathy, with or without lung infiltrates, is incidentally detected on chest radiography. Bilateral hilar adenopathy is the most common abnormality. […] If sarcoidosis is suspected, a chest radiograph should be the first test if it has not already been done. The chest radiograph appearance tends to roughly predict the likelihood of spontaneous remission of pulmonary sarcoidosis. […] A normal chest radiograph (stage 0) does not exclude the diagnosis of sarcoidosis, particularly when cardiac or neurologic involvement is suspected. A high-resolution CT is more sensitive for detecting hilar and mediastinal lymphadenopathy and parenchymal abnormalities. […] When imaging suggests sarcoidosis, the diagnosis is confirmed by demonstration of noncaseating granulomas on biopsy and exclusion of alternative causes of granulomatous disease.

• #1 Diagnosing Sarcoidosis | NYU Langone Health

  https://nyulangone.org/conditions/sarcoidosis/diagnosis

  To diagnose sarcoidosis, CT imaging is often used to examine small structures of the lungs to determine if granulomas may be present. […] An ultrasound of the heart, called an echocardiogram, can look at the functioning of the heart to see if there are any signs of weakening of the walls of the heart. […] This specialized MRI represents another way to look at the function of the heart, which can be difficult on standard MRI and CT scans. […] PET/CT scans combine the standard CT scan with a radiolabeled, or marked, sugar infusion. […] An advanced imaging study combining PET and MRI technologies can be used to help differentiate active inflammation from areas of old or inactive disease. […] Pulmonary function testing assesses lung function and possible damage by measuring how much air your lungs can hold, how quickly you can inhale and exhale, and how much oxygen your lungs deliver to the blood.

• #1 Pulmonary Sarcoidosis: Diagnosis and Differential Diagnosis

  https://www.mdpi.com/2075-4418/11/9/1558

  Sarcoidosis can mimic a large number of conditions, including, among others, infection, vasculitis, drug reaction and malignancy. […] This review aims to summarise recent advances in the diagnosis of sarcoidosis, with emphasis on the role of imaging and the importance of a careful exclusion of alternative diagnoses. […] Pulmonary imaging has a key role in the diagnosis of sarcoidosis, and all patients evaluated for suspected sarcoidosis should have a chest X-ray. […] Chest high resolution CT (HRCT) is more sensitive than chest X-ray and provides a more precise assessment of hilar, mediastinal and parenchymal abnormalities. […] Fluorine 18 fluorodeoxyglucose (FDG) PET/CT is a useful tool for assessing sarcoidosis activity, with a sensitivity of 89 to 100%. […] Because of the high prevalence of pulmonary involvement, bronchoscopy, with its ancillary sampling techniques, has the highest diagnostic yield in sarcoidosis, unless more easily accessible biopsy sites, such as skin or superficial lymph nodes, are available.

• #1 Sarcoidosis Diagnosis and Detection: Summary of Clinical Practice Guidelines – Pulmonology Advisor

  https://www.pulmonologyadvisor.com/news/sarcoidosis-diagnosis-and-detection-summary-of-clinical-practice-guidelines/

  For patients with extracardiac sarcoidosis and suspected cardiac involvement, the committee suggested cardiac magnetic resonance imaging (MRI) rather than a cardiac positron emission tomography or TTE to obtain diagnostic and prognostic data. […] Overall, the committee found that the quality of evidence was poor in most of the literature reviewed and stressed the need for future high-quality research. […] They concluded, Clinicians should apply caution when interpreting the recommendations and should always consider unique individual clinical circumstances including the individual values and preferences when managing patients with sarcoidosis.

• #1 Clinical manifestations and diagnosis of sarcoidosis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-sarcoidosis

  A definitive diagnostic test for sarcoidosis does not exist. Instead, the diagnosis of sarcoidosis generally requires three elements: compatible clinical and radiographic manifestations, exclusion of other diseases that may present similarly, and in many cases, histopathologic detection of non-necrotizing granulomas. […] Although confirmation of sarcoidosis usually requires diagnostic tissue sampling, invasive testing may be deferred in patients with clinical presentations that overwhelmingly favor the diagnosis. […] Biopsies should be performed on the most accessible lesion, which may include cutaneous lesions, subcutaneous nodules, palpable lymph nodes, glandular tissues, or certain other ocular lesions. […] Endobronchial diagnostic approaches include endoscopic ultrasound-guided fine-needle aspiration or cryobiopsy of mediastinal lymph nodes and endobronchial biopsy or transbronchial biopsy of the lung.

• #1 Clinical manifestations and diagnosis of sarcoidosis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-pulmonary-sarcoidosis

  Although confirmation of sarcoidosis usually requires diagnostic tissue sampling, invasive testing may be deferred in patients with clinical presentations that overwhelmingly favor the diagnosis. […] Patients with certain pathognomonic syndromes highly suggestive of sarcoidosis can often defer biopsy after completion of the noninvasive work-up. […] Patients with sarcoidosis who present with asymptomatic bilateral hilar lymph node involvement (radiographic stage I) generally have self-limited disease that can be monitored to ensure stability or resolution. […] Patients with suggestive clinical signs, symptoms, and imaging across multiple organ systems, a high clinical sarcoidosis diagnosis score may be considered sufficiently diagnostic to preclude biopsy. […] Biopsies should be performed on the most accessible lesion, which may include cutaneous lesions, subcutaneous nodules, palpable lymph nodes, enlarged parotid glands, conjunctival lesions, enlarged lacrimal glands, or certain other ocular lesions.

• #1 Diagnosis and Management of Sarcoidosis | AAFP

  https://www.aafp.org/pubs/afp/issues/2016/0515/p840.html

  An asymptomatic patient with stage I sarcoidosis (i.e., bilateral hilar lymphadenopathy on chest radiography) without suspected infection or malignancy does not require invasive tissue biopsy because the results would not affect the recommended management approach (i.e., monitoring). If there would be an indication for treatment with a confirmed diagnosis, pathologic evidence of noncaseating granulomas should be obtained from the most accessible and safest biopsy site. […] Because the lungs and intrathoracic lymph nodes are commonly affected, flexible bronchoscopy with biopsies have high diagnostic yields and low risk of complications.

• #1 Clinical manifestations and diagnosis of sarcoidosis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-sarcoidosis

  The main purpose of bronchoalveolar lavage in this setting is to exclude alternative diagnoses, such as chronic beryllium disease, eosinophilic lung disease, infections, and malignancy. […] BAL lymphocytosis greater than 15 percent, especially when accompanied by an elevated CD4:CD8 ratio >3.5, confers 93 to 96 percent specificity for the diagnosis of sarcoidosis. […] The characteristic morphologic feature of sarcoidosis is the non-necrotizing granuloma. […] Although it is the most common noninfectious cause of granulomatous inflammation, sarcoidosis remains a diagnosis of exclusion among granulomatous disorders. […] The differential diagnosis of granulomatous lung diseases includes many conditions; it is particularly important to exclude infections and malignancy. […] Once the diagnosis of sarcoidosis is confirmed, the functional severity of pulmonary disease should be determined and additional screening for occult extrathoracic disease is warranted.

• #1 Sarcoidosis Workup: Laboratory Studies, Imaging Studies, Other Tests

  https://emedicine.medscape.com/article/301914-workup

  Serum markers such as serum amyloid A (SAA), soluble interleukin-2 receptor (sIL-2R), lysozyme, angiotensin-converting enzyme (ACE), and the glycoprotein KL-6 have been reported to be markers of sarcoidosis. […] Elevated 1, 25-dihydroxyvitamin D levels are associated with protracted treatment in sarcoidosis. In one study, serum 1, 25-dihydroxyvitamin D levels were associated with patients requiring repeated regimens of systemic immunosuppressive therapy or longer than 1 year of therapy. The majority (71%) of the patients with levels higher than 51 pg/mL required long-term immunosuppressive therapy. […] ACE levels may be elevated. NCGs secrete ACE, which may function as a cytokine. Serum ACE levels are elevated in 60% of patients at the time of diagnosis. Serum ACE levels may correlate with total body granuloma load.

• #1 Clinical manifestations and diagnosis of sarcoidosis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-sarcoidosis

  Pulmonary function tests (PFTs), including spirometry and diffusing capacity for carbon monoxide (DLCO) are obtained in patients with pulmonary sarcoidosis to assess the severity of respiratory impairment and to monitor the course of disease. […] The specific testing and optimal frequency of disease monitoring in sarcoidosis have not been established. Monitoring should include broad questions about respiratory complaints and other new or worsening symptoms that could indicate extrapulmonary disease.

• #1 Diagnosing Sarcoidosis | Sarcoidosis NewsEnvelope icon

  https://sarcoidosisnews.com/sarcoidosis-diagnosis/

  In about one-third of patients, sarcoidosis has some form of skin involvement, typically seen as red nodules, bumps, and plaques. In these cases, a punch biopsy — in which a small circle of skin tissue is removed — is often done. The sample is then analyzed in a laboratory for the presence of granulomas and inflammatory molecules. […] In rare cases, when other tests are inconclusive, mediastinoscopy can be used for further airway and lymph node inspection. They may be needed to confirm a sarcoidosis diagnosis, as well as to rule out other disorders. […] Granulomas in the lungs can reduce lung volume, resulting in poorer lung function. As such, a lung function test is commonly given to determine how well the lungs are expanding and supplying tissues systemwide with oxygen. […] Sarcoidosis can also affect the heart. An electrocardiogram (EKG), which measures the heart’s electrical activity, or an echocardiogram, which uses sound waves to get a picture of the heart’s structures, can be used to assess heart function in sarcoidosis patients. […] A thorough eye examination can reveal the presence of inflammation, small granulomas inside the eyelids or on the white of the eye, or damage to the optic nerve.

• #1 Key Tests for Sarcoidosis Diagnosis and Management | myadlm.org

  https://myadlm.org/cln/cln-stat/2020/may/21/key-tests-for-sarcoidosis-diagnosis-and-management

  To identify the manifestations of sarcoidosis, the American Thoracic Society (ATS) is recommending four key lab tests to screen for renal sarcoidosis, abnormal calcium metabolism, hepatic sarcoidosis, and hematological abnormalities. […] Its a diagnosis by exclusion, requiring lab tests such as histopathology specimens, tissue cultures, and serology tests. Diagnosis is based on three major criteria: a compatible clinical presentation, finding non-necrotizing granulomatous inflammation in one or more tissue samples, and the exclusion of alternative causes of granulomatous disease. […] The ATS clinical practice guideline calls for testing approaches such as imaging and lab tests (calcium, creatinine, alkaline phosphatase (ALP), and complete blood count) to detect organ involvement, Crouser said.

• #1 Key Tests for Sarcoidosis Diagnosis and Management | myadlm.org

  https://myadlm.org/cln/cln-stat/2020/may/21/key-tests-for-sarcoidosis-diagnosis-and-management

  Guideline authors suggested that clinicians use a baseline serum creatinine test to screen for renal sarcoidosis in patients without symptoms or established renal sarcoidosis. […] For patients with sarcoidosis with neither hepatic symptoms nor established hepatic sarcoidosis, the guideline recommends baseline serum ALP testing to screen for hepatic sarcoidosis. […] Thus, screening for calcium metabolism was highly recommended, Crouser said. The panel suggested baseline serum calcium testing to screen for abnormal calcium metabolism in patients with no signs of hypercalcemia. […] The panelists also suggested that clinicians use baseline complete blood cell count (CBC) testing to screen for hematological abnormalities. Anemia is common in sarcoidosis, often reflecting granulomatous bone marrow involvement. […] Thus, CBC testing detects the two most common disease-relevant hematological findings, anemia and leukopenia, and is useful for clinical purposes including the assessment of dyspnea, disease activity, and to screen for bone marrow involvement, he said.

• #1 Cardiac Sarcoidosis Diagnosis | How is it diagnosed?

  https://www.nationaljewish.org/conditions/cardiac-sarcoidosis/diagnosis

  Diagnosing cardiac sarcoidosis can be very challenging, because the symptoms of cardiac sarcoidosis are similar to those of many other diseases. […] There are no widely accepted guidelines for either screening or diagnosing sarcoidosis of the heart. […] Moreover, the currently available diagnostic tests are variable in their ability to detect cardiac sarcoidosis. […] About one in four people with another form of sarcoidosis will have cardiac sarcoidosis as well; some say that number is even higher. […] Initial cardiac evaluation of cardiac sarcoidosis may include: Electrocardiogram (EKG). This test checks for an abnormal heart rhythm or abnormalities in the electrical system of the heart. […] Signal-averaged EKG. This is a special EKG that averages several hundred heartbeats to detect subtle abnormalities in the movement of electrical signals through the heart.

• #1 Cardiac Sarcoidosis Diagnosis | How is it diagnosed?

  https://www.nationaljewish.org/conditions/cardiac-sarcoidosis/diagnosis

  Echocardiogram (ultrasound of the heart). The ultrasound shows provides assessment of the pumping and relaxing function of the heart, valve function and estimate of pressures in the heart. […] Holter monitor (extended EKG). A Holter monitor is a monitor that continually records the electrical signals of your heart for 24 hours or more. […] Additional imaging tests for cardiac sarcoidosis may include: Single photon emission computed tomography (SPECT). This test can show how blood flows to tissues and organs in the body. […] Positron emission tomography (PET)/CT Scan. A PET scan can identify active inflammation in the heart muscle suggesting active cardiac sarcoidosis. […] Cardiac MRI. A cardiac MRI is the best test to see evidence of any scar tissue in the heart which may lead to the cardiac complications from cardiac sarcoidosis. […] A positive heart biopsy confirms cardiac sarcoidosis. However, it may more often be negative or normal even when there is sarcoidosis in the heart, especially if heart function is normal. For this reason, taking a biopsy is a less common test for detecting cardiac sarcoidosis.

• #1 Diagnosing Sarcoidosis | NYU Langone Health

  https://nyulangone.org/conditions/sarcoidosis/diagnosis

  If sarcoidosis appears to affect your lungs, your doctor may perform a bronchoscopy, a test that enables the close examination of these organs. […] Our dermatologists, part of NYU Grossman School of Medicines Ronald O. Perelman Department of Dermatology, have extensive experience in diagnosing sarcoidosis through clinical evaluation and skin biopsy. […] In patients with suspected neurosarcoidosis, a type of sarcoidosis that affects the nervous system, a lumbar puncture is sometimes necessary to assess for evidence of inflammation in the central nervous system.

• #1 Sarcoidosis – Diagnosis | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/sarcoidosis/diagnosis

  Eye exams look for eye damage, which can occur without symptoms in a person with sarcoidosis. […] Lung function tests check whether you have breathing problems. […] Heart tests monitor how well your heart is working. Sarcoidosis only rarely affects the heart, but cardiac sarcoidosis may be life threatening.

• #1 Establishing a Diagnosis of Pulmonary Sarcoidosis

  https://www.mdpi.com/2077-0383/12/21/6898

  The value of multidisciplinary team (MDT) discussion has already been scientifically illustrated and evaluated in ILD and subsequently implemented particularly in the diagnostic guidelines of idiopathic pulmonary fibrosis. […] A systematic and multidisciplinary approach, preferably implemented in a local care pathway for sarcoidosis and including MDT discussion, currently provides the best guarantee for establishing the right diagnosis.

• #1 Sarcoidosis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/sarcoidosis/diagnosis-treatment/drc-20350363

  Sarcoidosis can be hard to diagnose because there may not be many symptoms in the early stages. When symptoms do occur, they may be much like those of other conditions. […] No tests can specifically diagnose sarcoidosis. Tests can help rule out other conditions and show what body systems may be affected by sarcoidosis. […] Your healthcare professional may take a small sample of tissue called a biopsy. The sample is taken from any part of your body that may be affected by sarcoidosis. The sample is used to look for the granulomas commonly seen with the condition. […] Ongoing monitoring may include tests based on your condition. For example, you may have regular chest X-rays, blood and urine tests, EKGs, and exams of your lungs, eyes, skin and other organs. Follow-up care is likely lifelong.

• #1 Sarcoidosis – EMCrit Project

  https://emcrit.org/ibcc/sarcoidosis/

  Sarcoidosis is an immune-mediated granulomatous disease which may manifest with inflammation in various organs, including the lungs (90%), eye (~20%), skin (~15%), and nervous system (~10%). (34607912) […] The average age at diagnosis is ~35-50 years old. (31485575) […] The following staging system applies only to disease visible on chest radiograph. For example, if a patient had bulky hilar lymphadenopathy (visible on chest radiograph) and subtle interstitial infiltrates visible only on CT scan, they would be classified as Stage-1 disease. […] Most patients do not require treatment for pulmonary involvement. […] Disease severity alone isn’t necessarily an indication for therapy (if sarcoidosis is quiescent). However, limited pulmonary reserve function should lower the threshold for treatment. Moderate to severe disease may be considered as a DLCO 65% predicted, or spirometric volumes 70% predicted. (Shah 2019)

• #1 Sarcoidosis – Wikipedia

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

  Serum markers of sarcoidosis, include: serum amyloid A, soluble interleukin-2 receptor, lysozyme, angiotensin converting enzyme, and the glycoprotein KL-6. […] Angiotensin-converting enzyme blood levels are used in the monitoring of sarcoidosis. […] A bronchoalveolar lavage can show an elevated (of at least 3.5) CD4/CD8 T cell ratio, which is indicative (but not proof) of pulmonary sarcoidosis. […] Differential diagnosis includes metastatic disease, lymphoma, septic emboli, rheumatoid nodules, granulomatosis with polyangiitis, varicella infection, tuberculosis, and atypical infections, such as Mycobacterium avium complex, cytomegalovirus, and cryptococcus. […] Sarcoidosis is confused most commonly with neoplastic diseases, such as lymphoma, or with disorders characterized also by a mononuclear cell granulomatous inflammatory process, such as the mycobacterial and fungal disorders.

• #1 Establishing a Diagnosis of Pulmonary Sarcoidosis

  https://www.mdpi.com/2077-0383/12/21/6898

  Three major criteria must be met to make a diagnosis of sarcoidosis: (1) a compatible clinical presentation; (2) findings of non-caseating granulomatous inflammation in one or more tissue samples; and (3) the exclusion of alternative causes of granulomatous disease or diseases capable of producing a similar clinical picture. […] The diagnosis requires clinic-radiographic findings compatible with sarcoidosis, histologic or cytological confirmation of granulomatous inflammation, exclusion of known causes of granulomatous disease, and presence of disease in at least two organs or tissues. […] In some patients, a definite diagnosis may require the continuous gathering of information during follow-up. After diagnosis, a healthy degree of skepticism remains indispensable, especially in the case of an unexpected course of disease during follow-up.

• #2 A Comprehensive Review of Sarcoidosis Diagnosis and Monitoring for the Pulmonologist

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8589876/

  Sarcoidosis is a systemic granulomatous disease with heterogenous clinical manifestations. Here we review the diagnosis of sarcoidosis and propose a clinically feasible diagnostic work-up and monitoring protocol. As sarcoidosis is a systemic disease, a multidisciplinary approach is recommended for best outcomes. However, since the lungs are frequently involved, the pulmonologist is often the referral physician for diagnosis and management. When sarcoidosis is suspected, diagnosis needs to be confirmed and organ involvement/impairment assessed. This process is also required to establish whether the patient is likely to benefit from treatment, as many cases of sarcoidosis are self-limited and remit spontaneously. Whether or not treatment is started, effective regular follow-up is necessary to monitor changes in the disease, including extension, progression, remissions, flare-ups, and complications.

• #2 Establishing a Diagnosis of Pulmonary Sarcoidosis

  https://www.mdpi.com/2077-0383/12/21/6898

  Three major criteria must be met to make a diagnosis of sarcoidosis: (1) a compatible clinical presentation; (2) findings of non-caseating granulomatous inflammation in one or more tissue samples; and (3) the exclusion of alternative causes of granulomatous disease or diseases capable of producing a similar clinical picture. […] The diagnosis requires clinic-radiographic findings compatible with sarcoidosis, histologic or cytological confirmation of granulomatous inflammation, exclusion of known causes of granulomatous disease, and presence of disease in at least two organs or tissues. […] In some patients, a definite diagnosis may require the continuous gathering of information during follow-up. After diagnosis, a healthy degree of skepticism remains indispensable, especially in the case of an unexpected course of disease during follow-up.

• #2 Clinical manifestations and diagnosis of sarcoidosis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-pulmonary-sarcoidosis

  Patients with pulmonary symptoms — Lung or thoracic lymph node involvement occurs in approximately 90 to 95 percent of patients with sarcoidosis. For those with symptoms due to thoracic disease, common presenting complaints include cough, dyspnea, fatigue, and chest pain. The chest pain is usually constant and can be localized to substernal, subcostal, or infrascapular areas. The location and severity of chest pain are not generally related to the location or extent of granulomatous lesions or lymph node enlargement. […] Pulmonary symptoms are frequently accompanied by systemic manifestations such as fatigue, malaise, fever, and weight loss, particularly in the elderly. Systemic inflammation may also contribute to muscle weakness and exercise intolerance. […] Common extrapulmonary findings and pathognomonic syndromes — Although sarcoidosis frequently involves the lung, approximately 50 percent of patients on careful review also have extrathoracic manifestations at presentation. It should be noted that more sensitive testing, such as 18-fluorodeoxyglucose-positron emission tomography (18F-FDG-PET) scan will reveal an even higher proportion with clinically silent extrapulmonary disease, but 18F-FDG-PET scanning is not recommended for routine evaluation of patients with sarcoidosis.

• #2 Sarcoidosis – Diagnosis | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/sarcoidosis/diagnosis

  Sarcoidosis is diagnosed based on your symptoms, a physical exam, and imaging tests or a biopsy. Before diagnosing you with sarcoidosis, your healthcare provider will rule out other possible conditions. […] Your healthcare provider may have you undergo certain tests and procedures to diagnose sarcoidosis. […] Chest X-rays look for granulomas or scarring in the lungs and heart. This will also help figure out the stage of the disease. […] A biopsy of the skin, lymph nodes, lungs, or other affected organs may help confirm your sarcoidosis diagnosis. […] Blood tests check your blood counts, hormone levels, and how well your kidneys are working. […] Other imaging tests look for granulomas or inflammation in the heart, eyes, lymph nodes, or other areas. […] Neurological tests, such as electromyography, evoked potentials, spinal taps, or nerve conduction tests, look for problems with the nervous system caused by sarcoidosis.

• #2 Diagnosing Sarcoidosis | NYU Langone Health

  https://nyulangone.org/conditions/sarcoidosis/diagnosis

  To diagnose sarcoidosis, CT imaging is often used to examine small structures of the lungs to determine if granulomas may be present. […] An ultrasound of the heart, called an echocardiogram, can look at the functioning of the heart to see if there are any signs of weakening of the walls of the heart. […] This specialized MRI represents another way to look at the function of the heart, which can be difficult on standard MRI and CT scans. […] PET/CT scans combine the standard CT scan with a radiolabeled, or marked, sugar infusion. […] An advanced imaging study combining PET and MRI technologies can be used to help differentiate active inflammation from areas of old or inactive disease. […] Pulmonary function testing assesses lung function and possible damage by measuring how much air your lungs can hold, how quickly you can inhale and exhale, and how much oxygen your lungs deliver to the blood.

• #2 Diagnosis and Management of Cardiac Sarcoidosis: Key Points

  https://www.acc.org/Latest-in-Cardiology/ten-points-to-remember/2024/04/22/13/10/diagnosis-and-management-of-cs

  The following are key points to remember from an American Heart Association (AHA) scientific statement on the diagnosis and management of cardiac sarcoidosis (CS): […] Diagnosis […] Cardiac magnetic resonance (CMR) has emerged as a key diagnostic test for CS. In a meta-analysis, CMR had a high sensitivity (95%) and specificity (85%) for the diagnosis of CS. Myocardial late gadolinium enhancement (LGE) is the strongest predictor for all-cause mortality and sustained VA among individuals with known or suspected CS. […] Fluorodeoxyglucosepositron emission tomography (FDG-PET) can identify metabolically active, inflammatory lesions in CS. […] Because of the focal and patchy nature of the disease, unguided endomyocardial biopsy has a low sensitivity of approximately 20%. […] The most recent Japanese guideline is unique in that it does not require biopsy evidence of noncaseating granulomas, and it is the only guideline to include an imaging diagnostic algorithm for isolated CS. […] A multidisciplinary team approach for accurate diagnosis, treatment, and comprehensive care is recommended. […] There are significant unmet needs in the optimal diagnostic and management strategies in sarcoidosis.

• #2 Clinical manifestations and diagnosis of sarcoidosis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-sarcoidosis

  A definitive diagnostic test for sarcoidosis does not exist. Instead, the diagnosis of sarcoidosis generally requires three elements: compatible clinical and radiographic manifestations, exclusion of other diseases that may present similarly, and in many cases, histopathologic detection of non-necrotizing granulomas. […] Although confirmation of sarcoidosis usually requires diagnostic tissue sampling, invasive testing may be deferred in patients with clinical presentations that overwhelmingly favor the diagnosis. […] Biopsies should be performed on the most accessible lesion, which may include cutaneous lesions, subcutaneous nodules, palpable lymph nodes, glandular tissues, or certain other ocular lesions. […] Endobronchial diagnostic approaches include endoscopic ultrasound-guided fine-needle aspiration or cryobiopsy of mediastinal lymph nodes and endobronchial biopsy or transbronchial biopsy of the lung.

• #2 Clinical manifestations and diagnosis of sarcoidosis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-pulmonary-sarcoidosis

  Endobronchial diagnostic approaches include endoscopic ultrasound-guided fine-needle aspiration or cryobiopsy of mediastinal lymph nodes and endobronchial biopsy or transbronchial biopsy of the lung. […] The main purpose of bronchoalveolar lavage in this setting is to exclude alternative diagnoses, such as CBD, eosinophilic lung disease, infections, and malignancy. […] The characteristic morphologic feature of sarcoidosis is the non-necrotizing granuloma. […] Although it is the most common noninfectious cause of granulomatous inflammation, sarcoidosis remains a diagnosis of exclusion among granulomatous disorders. […] Once the diagnosis of sarcoidosis is confirmed, the functional severity of pulmonary disease should be determined and additional screening for occult extrathoracic disease is warranted.

• #2 Clinical manifestations and diagnosis of sarcoidosis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-pulmonary-sarcoidosis

  Pulmonary function tests (PFTs), including spirometry and diffusing capacity for carbon monoxide (DLCO) are obtained in patients with pulmonary sarcoidosis to assess the severity of respiratory impairment and to monitor the course of disease. […] The specific testing and optimal frequency of disease monitoring in sarcoidosis have not been established. Monitoring should include broad questions about respiratory complaints and other new or worsening symptoms that could indicate extrapulmonary disease.

• #2 Key Tests for Sarcoidosis Diagnosis and Management | myadlm.org

  https://myadlm.org/cln/cln-stat/2020/may/21/key-tests-for-sarcoidosis-diagnosis-and-management

  Guideline authors suggested that clinicians use a baseline serum creatinine test to screen for renal sarcoidosis in patients without symptoms or established renal sarcoidosis. […] For patients with sarcoidosis with neither hepatic symptoms nor established hepatic sarcoidosis, the guideline recommends baseline serum ALP testing to screen for hepatic sarcoidosis. […] Thus, screening for calcium metabolism was highly recommended, Crouser said. The panel suggested baseline serum calcium testing to screen for abnormal calcium metabolism in patients with no signs of hypercalcemia. […] The panelists also suggested that clinicians use baseline complete blood cell count (CBC) testing to screen for hematological abnormalities. Anemia is common in sarcoidosis, often reflecting granulomatous bone marrow involvement. […] Thus, CBC testing detects the two most common disease-relevant hematological findings, anemia and leukopenia, and is useful for clinical purposes including the assessment of dyspnea, disease activity, and to screen for bone marrow involvement, he said.

• #2 Cardiac Sarcoidosis Diagnosis | Temple Health

  https://www.templehealth.org/services/conditions/cardiac-sarcoidosis/diagnosis

  When diagnosing cardiac sarcoidosis, your doctor will perform a physical exam and a blood pressure test (usually high, with a difference between right and left arm), review your medical history, and order tests such as: […] Echocardiogram is a non-invasive test that uses ultra sound (sound waves) and a device called a transducer which is placed on the surface of the chest to create a moving picture of the heart. This helps to determine how well the heart is able to pump blood. […] Electrocardiogram (EKG or ECG) is a simple non-invasive test that can be done in the doctors office using small adhesive pads called electrodes that are placed on the arms, legs, and chest. These electrodes are connected to a machine that detects and prints out the heart’s electrical impulses, giving a 10-second snapshot of what the heart is doing right at that moment.

• #2 Cardiac Sarcoidosis Diagnosis and Treatment: Key Points

  https://www.acc.org/Latest-in-Cardiology/ten-points-to-remember/2023/03/30/14/48/cardiac-sarcoidosis-phenotypes

  Cardiac Sarcoidosis Diagnosis and Treatment: Key Points […] The following are key points to remember from a state-of-the-art review on cardiac sarcoidosis (CS) phenotypes, diagnosis, treatment, and prognosis: […] In pre-existing sarcoidosis, elevated troponins, B-type natriuretic peptide (BNP), anti-heart, and anti-intercalated disk antibodies support CS diagnosis. Echo strain imaging and electrocardiography (ECG) may be normal in CS. Computed tomography of the chest can suggest intrathoracic sarcoidosis, but diagnosis relies on cardiac MRI and/or 18F-fluorodeoxyglucose positron emission tomography (18F-FDG-PET). […] Guidelines recommend CS screening in patients with known extracardiac sarcoidosis with symptom assessment, ECG, and echo. Cardiac MRI and FDG-PET are recommended if these studies are abnormal. […] For histological proof, guidelines recommend extracardiac over endomyocardial biopsy (EMBx). Targeted EMBx using imaging or intracardiac voltage mapping increases its sensitivity. Histo-pathological findings include non-necrotic granulomas with isolated giant cells when other causes are excluded. […] Initial CS treatment is comprised of steroids (usually prednisone 0.5 mg/kg/day). Prednisone is tapered down every 4 weeks in decrements of 5-10 mg until a dose of 10 mg/day is achieved. […] Efficacy of treatment is checked with symptom assessment, ECG, arrhythmia burden, cardiac biomarkers, and left ventricular ejection fraction (LVEF). […] The authors recommend a second immunosuppressant (methotrexate, cellcept, azathioprine, leflunomide, and cyclophosphamide) when either steroids fail or there is a need to reduce steroid toxicity risk or with rapidly progressive heart failure, life-threatening arrhythmias, or extensive inflammation on imaging. […] When all therapies fail, biological antitumor necrosis factor agents like infliximab may help. […] For symptomatic ventricular arrhythmias, the effect of immunosuppression is not well established. If inflammation is detected, steroids are recommended with antiarrhythmics. […] Clinically manifest CS has a 10% risk for sudden cardiac death over 5-year follow-up. North American guidelines recommend implantable cardioverter-defibrillator for LVEF 35% with indication for pacemaker.

• #2 Clinical manifestations and diagnosis of sarcoidosis – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-pulmonary-sarcoidosis

  Although confirmation of sarcoidosis usually requires diagnostic tissue sampling, invasive testing may be deferred in patients with clinical presentations that overwhelmingly favor the diagnosis. […] Patients with certain pathognomonic syndromes highly suggestive of sarcoidosis can often defer biopsy after completion of the noninvasive work-up. […] Patients with sarcoidosis who present with asymptomatic bilateral hilar lymph node involvement (radiographic stage I) generally have self-limited disease that can be monitored to ensure stability or resolution. […] Patients with suggestive clinical signs, symptoms, and imaging across multiple organ systems, a high clinical sarcoidosis diagnosis score may be considered sufficiently diagnostic to preclude biopsy. […] Biopsies should be performed on the most accessible lesion, which may include cutaneous lesions, subcutaneous nodules, palpable lymph nodes, enlarged parotid glands, conjunctival lesions, enlarged lacrimal glands, or certain other ocular lesions.

• #2 Pulmonary Sarcoidosis: Diagnosis and Differential Diagnosis

  https://www.mdpi.com/2075-4418/11/9/1558

  Sarcoidosis can mimic a large number of conditions, including, among others, infection, vasculitis, drug reaction and malignancy. […] This review aims to summarise recent advances in the diagnosis of sarcoidosis, with emphasis on the role of imaging and the importance of a careful exclusion of alternative diagnoses. […] Pulmonary imaging has a key role in the diagnosis of sarcoidosis, and all patients evaluated for suspected sarcoidosis should have a chest X-ray. […] Chest high resolution CT (HRCT) is more sensitive than chest X-ray and provides a more precise assessment of hilar, mediastinal and parenchymal abnormalities. […] Fluorine 18 fluorodeoxyglucose (FDG) PET/CT is a useful tool for assessing sarcoidosis activity, with a sensitivity of 89 to 100%. […] Because of the high prevalence of pulmonary involvement, bronchoscopy, with its ancillary sampling techniques, has the highest diagnostic yield in sarcoidosis, unless more easily accessible biopsy sites, such as skin or superficial lymph nodes, are available.

• #2 Diagnosis and Management of Sarcoidosis | AAFP

  https://www.aafp.org/pubs/afp/issues/2016/0515/p840.html

  Sarcoidosis is a systemic disease of unknown etiology characterized by the presence of noncaseating granulomas in any organ, most commonly the lungs and intrathoracic lymph nodes. A diagnosis of sarcoidosis should be suspected in any young or middle-aged adult presenting with unexplained cough, shortness of breath, or constitutional symptoms, especially among blacks or Scandinavians. Diagnosis relies on three criteria: (1) a compatible clinical and radiologic presentation, (2) pathologic evidence of noncaseating granulomas, and (3) exclusion of other diseases with similar findings, such as infections or malignancy. […] According to an international consensus statement, there are three criteria for diagnosing sarcoidosis: (1) a compatible clinical and radiologic presentation, (2) pathologic evidence of noncaseating granulomas, and (3) exclusion of other diseases with similar findings.

• #2 Establishing a Diagnosis of Pulmonary Sarcoidosis

  https://www.mdpi.com/2077-0383/12/21/6898

  Establishing a Diagnosis of Pulmonary Sarcoidosis […] Due to the lack of a simple and reliable test, making the diagnosis is often challenging. There are three criteria that must always be considered: (1) compatible clinical presentation; (2) evidence of granuloma formation (usually non-caseating); and (3) exclusion of alternative causes of granulomatous disease. […] In general, it is advisable to always maintain a healthy dose of skepticism when making the diagnosis of sarcoidosis, especially when the clinical course of disease gives rise to this. […] Although HRCT is the cornerstone for diagnosis, its imaging findings are currently not considered diagnostically sufficient. Sarcoidosis has many lookalikes and currently remains a diagnosis by exclusion. […] The aim of the article is to give an overview of the diagnostic criteria, differential diagnosis, clinical presentations, and approach to the diagnosis of sarcoidosis.

• #3 Sarcoidosis – Diagnosis | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/sarcoidosis/diagnosis

  Sarcoidosis is diagnosed based on your symptoms, a physical exam, and imaging tests or a biopsy. Before diagnosing you with sarcoidosis, your healthcare provider will rule out other possible conditions. […] Your healthcare provider may have you undergo certain tests and procedures to diagnose sarcoidosis. […] Chest X-rays look for granulomas or scarring in the lungs and heart. This will also help figure out the stage of the disease. […] A biopsy of the skin, lymph nodes, lungs, or other affected organs may help confirm your sarcoidosis diagnosis. […] Blood tests check your blood counts, hormone levels, and how well your kidneys are working. […] Other imaging tests look for granulomas or inflammation in the heart, eyes, lymph nodes, or other areas. […] Neurological tests, such as electromyography, evoked potentials, spinal taps, or nerve conduction tests, look for problems with the nervous system caused by sarcoidosis.

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