Sarcoidosis

(AJRCCM 2020;201:e26; JAMA 2022;327:856)

• Prevalence: African Americans, northern Europeans, and females; onset in 3rd-5th decade

• Pathophysiology: depression of cellular immune system peripherally, activation centrally

Clinical Manifestations of Sarcoidosis

• Löfgren syndrome:

• Diagnostic studies: LN bx → noncaseating granulomas + multinucleated giant cells Endobronchial ultrasonography superior to conventional bronch (JAMA 2013;309:2457) 18FDG PET can be used to identify extent and potentially targets for dx bx ↑ ACE (Se 60%, 90% w/ active dis., Sp 80%, false ⊕ in granulomatous diseases)

• To assess extent: CXR, PFTs, full ophtho exam, ECG, CBC (lymphopenia, ↑ eos), Ca, LFTs; ± Holter, echo, cardiac MRI, brain MRI, etc., based on s/s

• Rx: steroids if sx or extrathoracic organ dysfxn (eg, prednisone 20-40 mg/d), improves sx, but doesn’t Δ long-term course; hydroxychloroquine for extensive skin disease; MTX, AZA, mycophenolate, or anti-TNF for chronic/refractory disease

• Prognosis: ~²∕³ spontaneously remit w/in 10 y (60-80% of stage I, 50-60% stage II, 30% stage III), w/ relapses uncommon; ~1∕³ have progressive disease