Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 9-22: Sarcoidosis + Key Features Download Section PDF Listen +++ +++ Essentials of Diagnosis ++ Symptoms related to Lung Skin Eyes Peripheral nerves Liver Kidney Heart Other tissues Demonstration of noncaseating granulomas in biopsy specimen Exclusion of other granulomatous disorders +++ General Considerations ++ A systemic disease of unknown etiology Granulomatous inflammation of the lungs is present in 90% of cases +++ Demographics ++ Highest incidence in North American blacks and northern European whites Among blacks, women are more frequently affected than men Disease onset is usually in the third or fourth decade + Clinical Findings Download Section PDF Listen +++ +++ Symptoms and Signs ++ Malaise, fever, and insidious dyspnea Symptoms from erythema nodosum, iritis, peripheral neuropathy, arthritis or cardiomyopathy may also prompt initial evaluation Some patients are asymptomatic, and diagnosis is made after abnormal findings are noted on chest radiograph Crackles are uncommon on chest examination Parotid gland enlargement, hepatosplenomegaly, and lymphadenopathy may be noted Myocardial sarcoidosis is found in 5% of patients and can lead to Restrictive cardiomyopathy Cardiac dysrhythmias Conduction disturbances +++ Differential Diagnosis ++ Other granulomatous diseases must be excluded Tuberculosis Histoplasmosis Coccidioidomycosis Lymphoma (including lymphocytic interstitial pneumonitis) Idiopathic pulmonary fibrosis Pneumoconiosis (especially berylliosis) Syphilis + Diagnosis Download Section PDF Listen +++ +++ Laboratory Tests ++ Leukopenia Elevation of erythrocyte sedimentation rate Hypercalcemia in 5%, hypercalciuria in 20% Angiotensin-converting enzyme (ACE) levels Commonly elevated in active disease Neither sensitive nor specific enough to be of diagnostic value Physiologic testing may reveal evidence of airflow obstruction, but restrictive changes with decreased lung volumes and diffusing capacity are more common Skin test anergy is present in 70% +++ Imaging Studies ++ Radiographic findings are variable Stage I: hilar adenopathy alone Stage II: hilar adenopathy with parenchymal involvement Stage III: parenchymal involvement alone Stage IV: advanced fibrotic changes principally in the upper lobes Parenchymal involvement usually manifests as diffuse reticular infiltrates, but focal infiltrates, acinar shadows, nodules, and rare cavitation are seen Pleural effusion occurs in < 10% of patients +++ Diagnostic Procedures ++ ECG may show conduction disturbances and dysrhythmias Biopsy demonstrating noncaseating granulomas is required for diagnosis Easily accessible biopsy sites include lymph nodes, skin lesions, and salivary glands Transbronchial biopsy has a yield of 75–90% Some experts believe biopsy is unnecessary in stage I disease with a presentation highly suggestive of sarcoidosis Bronchoalveolar lavage is usually characterized by an increase in lymphocytes with a high CD4/CD8 ratio; this may be used to follow disease activity, but not for diagnosis + Treatment Download Section PDF Listen +++ +++ Medications ++ Corticosteroids (eg, oral prednisone, 0.5–1.0 mg/kg/day) are indicated for Constitutional symptoms Hypercalcemia Iritis Uveitis Arthritis CNS involvement Cardiac involvement Granulomatous hepatitis Cutaneous lesions other than erythema nodosum Progressive pulmonary lesions Long-term therapy is usually required over months to years Immunosuppressive medications, most commonly methotrexate, azathioprine, or cyclosporine, or anti-TNF therapy with infliximab Used in steroid-intolerant patients or steroid-refractory disease However, sound clinical research to support specific agents is lacking + Outcome Download Section PDF Listen +++ +++ Follow-Up ++ At a minimum, yearly physical examination, pulmonary function studies, chemistry panel, ophthalmologic evaluation, chest radiograph, and ECG +++ Complications ++ Hemoptysis Pneumothorax Mycetoma formation in lung cavities Respiratory failure in advanced disease +++ Prognosis ++ 20% of patients with lung involvement suffer irreversible lung impairment, with progressive fibrosis, bronchiectasis, and cavitation Outlook is best for patients with stage I disease, worse with radiographic parenchymal involvement Erythema nodosum is associated with a good outcome Death from respiratory insufficiency occurs in about 5% of patients + References Download Section PDF Listen +++ + +Adler BL. Anti-tumor necrosis factor agents in sarcoidosis: a systematic review of efficacy and safety. Semin Arthritis Rheum. 2019 Jun;48(6):1093–104. [PubMed: 30446173] + +Birnie DH et al. Cardiac manifestations of sarcoidosis: diagnosis and management. Eur Heart J. 2017 Sep 14;38(35):2663–70. [PubMed: 27469375] + +Carmona EM et al. Pulmonary sarcoidosis: diagnosis and treatment. Mayo Clin Proc. 2016 Jul;91(7):946–54. [PubMed: 27378039] + +Grunewald J et al. Sarcoidosis. Nat Rev Dis Primers. 2019 Jul 4;5(1):45. [PubMed: 31273209] + +Judson MA. Corticosteroids in sarcoidosis. Rheum Dis Clin North Am. 2016 Feb;42(1):119–35. [PubMed: 26611555] + +Llabres M et al. Synthetic pharmacotherapy for pulmonary sarcoidosis. Expert Opin Pharmacother. 2019 Aug;20(11):1397–404. [PubMed: 31090462] + +Llanos O et al. Sarcoidosis. Med Clin North Am. 2019 May;103(3):527–34. [PubMed: 30955519] + +Patterson KC et al. The pathogenesis of pulmonary sarcoidosis and implications for treatment. 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