Snapshot A previously healthy 30-year-old African American woman has fatigue, arthralgia, and a nodular rash over the trunk and upper extremities for three weeks. There are twelve 3-8 mm, pale, indurated plaques over the chest, back, and upper extremities. The liver is palpable 2 cm below the right costal margin with a percussion span of 14 cm, and the spleen tip is palpable 3 cm below the left costal margin. There is no pain or limitation of any of the joints. A chest radiograph shows bilateral lymphadenopathy. Introduction Idiopathic condition characterized by granulmomatous inflammation of multiple organs lungs most commonly involved organ commonly causes restrictive lung disease may also cause obstructive or mixed pattern mediated by CD4 TH cells ↑ IFN-gamma granulomas can affect all organ systems liver/spleen bone heart Epidemiology most common in black females smoking does not ↑ risk presents most commonly in 3rd or 4th decade Associated conditions diabetes insipidus (granulomatous infiltration of posterior pituitary) Presentation Symptoms may be asymptomatic 50% as incidental chest radiograph findings cough fever, malaise arthritis symptoms mainly in the ankle and legs can be GRUELING Granulomas Rheumatoid arthritis Uveitis Erythema nodosum Lymphadenopahy Interstitial fibrosis Negative TB Gammaglobulinemia Physical exam skin lesions nodular granulomatous lesions facial rash erthyma nodosum painful nodules also seen in rheumatic fever eye lesions uveitis ↓ vision and glaucoma enlarged salivary and lacrimal glands Evaluation Diagnosis is clinical and often one of exclusion Chest radiograph bilateral hilar lymphadenopathy lungs involved in 90% of the cases Labs ↑ serum ACE (not uniformly) hypercalcemia ↑ 1-α-hydroxylase → hypervitaminosis D can cause hypercalemia and renal failure chronically commonly tested, less commonly seen (only about 11%) serum protein electrophoresis (SPE) shows polyclonal gammopathy lack of response to CD4 TH skin tests (like PPD) due to ↑ lung activity and ↓ systemic activity Pulmonary function tests restrictive pattern is common (normal FEV1/FVC with normal TLC) however, obstructive or mixed pattern may also be seen Bronchoalveolar lavage high CD4:CD8 T-cell ratio due to CD4 infiltrate into the interstitium as well as intralveolar space contrasted to the low ratio in hypersensitivity pneumonitis and HIV Biopsy noncaseating granulomas with multinucleated giant cells very suggestive of sarcoidosis laminated calcium concretions present (cause of density on CXR) Treatment Conservative observationfine in most cases majority experience remission within two years without treatment Pharmacologic topical preferred when possible (skin, eyes, nasal, airway, etc) corticosteroids indications extra-pulmonary manifestations: cardiac, neurologic, or uveitis symptomatic hepatosplenomegaly significant hypercalcemia only if refractory to observations Prognosis Relapsing/remitting course with 50% resolving spontaneously Progression Stage I - bilateral hilar lymphadenopathy Stage II - bilateral hilar lymphadenopathy + upper lobe infiltrates Stage III - lung infiltrates only Stage IV - lung fibrosis