Snapshot A previously healthy 30-year-old African-American woman reports having fatigue, arthralgia, and a nodular rash over the trunk and upper extremities for three weeks. On exam, her physician identifies 12 0.3-0.8 cm, pale, indurated plaques over the chest, back, and upper extremities. Her liver is palpable 2 cm below the right costal margin with a percusion span of 14 cm, and her spleen tip is palpable 3 cm below the left costal margin. A CXR shows bilateral lymphadenopathy. Introduction Multisystem, chronic, inflammatory/fibrosing disease of unknown etiology Characterized by noncaseating granulomas lungs involved in 90% of the cases can affect all organ systems Most common in African-American females and northern European Caucasians Often presents in 3rd and 4th decade of life Presentation Symptoms fever malaise cough weight loss dyspnea arthritis in the ankle and legs 50% of cases are identified incidentally on CXR Physical exam signs of multisystem involvment including can be remembered by GRUELING Granulomas Rheumatoid arthritis Uveitis Erythema nodosum Lymphadenopahy Interstitial fibrosis Negative TB Gammaglobulinemia may also identify cranial nerve defects and cardiac arryhthmias Evaluation Diagnosis is primarily made by clinical observation CXR findings used to stage the disease stage I - bilateral hilar lyphadenopathy stage II - hilar adenopathy and pulmonary infiltrates stage III - isolated pulmonary infiltrates Labs serum ACE elevated (neither sensitive nor specific) hypercalcemia hypercalciuria increased alkaline phosphatase indicative of liver involvement lymphopenia Serum protein electrophoresis (SPE) shows polyclonal gammopathy PFTs restrictive pattern FEV/FVC increases to 100% decreased FEV decreased FVC decreased TLC decrease diffusion capacity Lymph node or lung biopsy noncaseating granulomas very suggestive of sarcoidosis Memory aid - LOFGREN (Lofgren's syndrome is an acute type of sarcoidosis) Lymphadenopathy (bilateral, hilar) Osteoarthropathy Fever Granulomatis uveitis Red eye Erythema Nodosum Differential Pleural disease, alvelolar disease (pulmonary edema), interstitial lung disease, neuromuscular disease, idiopathic pulmonary fibrosis, TB, lyphoma, histoplasmosis, cocci, pneumoconioses, HIV, berylliosis Treatment Non-operative systemic corticosteroids indicated for treatment of constitutional systems, hypercalcemia, or systemic involvement usually indicated when disease does not remit after months or if eye/heart involves Operative transplantation of affected organs may be indicated depending on severity of disease and organs affected Prognosis, Prevention, and Complications Prognosis 50% of cases remit spontaneously Prevention etiology is unknown, therefore no preventive measures can be taken at this time Complications multi-organ system involvement can result in severe and deadly sequelae