- multisystem disease
- characterized by non-caseating granulomas
- etiology is unknown
Presentation
- Lungs: dry, non-productive cough, dyspnea, chest pain
- Eyes: blurry vision, conjunctivitis, eye pain, uveitis (common), iritis
- Skin: erythema nodosum, papules, macules, nodules, lupus pernio
- Heart: arrhythmias, cardiomyopathy
Diagnosis
- diagnosis of exclusion
- initial labs: CBC, serum chemistry (ALT, AST, ALP, BUN, Cr, Ca, other electrolytes), urinalysis, 24 h urine excretion of calcium, CRP, ESR, tuberculin skin test
- imaging: CXR, ECG
- pulmonary function tests: spirometry, diffusion capacity of carbon monoxide (DLCO)- single breath
- biopsy (skin, lymph nodes or conjunctiva) of affected area, and if no suitable area is present then bronchoscopy with transbronchial biopsy.
Management
- Corticosteroids are the mainstay therapy when required. Given if severe lung disease, involvement of eyes/ CNS/ heart, or hypercalcemia.
- Prednisone 40 mg daily for 8 to 12 weeks then taper to 10 mg every other day over 8 to 12 months. <<source: Ferri’s clinical advisor 2016>>
