SHM's Clinical Quick Talks-Sarcoidosis

Pdf Summary
Sarcoidosis is a multisystem disorder characterized by the formation of noncaseating granulomas, with an unknown etiology but suspected triggers including environmental factors (e.g., beryllium), infections, and genetic predispositions. It commonly affects the lungs, lymph nodes, skin, eyes, and other organs.<br /><br />Clinical presentations vary by organ system: neurological involvement typically shows facial nerve palsy; ocular findings most commonly include uveitis; cardiac manifestations range from myocarditis to arrhythmias and sudden death; pulmonary involvement presents as interstitial fibrosis and hilar adenopathy in 90% of patients. Other affected systems include the gastrointestinal tract (splenomegaly, hepatic granulomas), kidneys (nephritis, nephrolithiasis), musculoskeletal system (arthritis), lymph nodes (bilateral/hilar/paratracheal adenopathy), and skin (erythema nodosum, lupus pernio—the latter pathognomonic).<br /><br />Classic pathognomonic presentations include lupus pernio (violaceous facial plaques), uveoparotid fever (fever, anterior uveitis, parotid gland enlargement, facial nerve palsy), and Löfgren syndrome (fever, hilar adenopathy, erythema nodosum/bilateral ankle inflammation).<br /><br />Pulmonary sarcoidosis is staged via chest X-ray from Stage I (bilateral hilar adenopathy) to Stage IV (fibrosis and structural lung changes). Histopathology reveals non-necrotizing granulomas but may be unnecessary if clinical signs are definitive.<br /><br />Diagnostic workup should exclude infections like mycobacterial, fungal diseases, and HIV, especially since some fungal infections can mimic sarcoidosis. Screening for cardiac, ophthalmologic, and pulmonary involvement is essential.<br /><br />Treatment is reserved for symptomatic patients or those at risk of organ damage or quality-of-life impairment. Options range from local treatments (inhaled steroids, skin ointments, eye drops) to systemic therapies: first-line oral glucocorticoids; second-line immunosuppressants like methotrexate; and third-line TNF-α inhibitors. Personalized management is crucial, with organ transplantation considered for end-stage damage, particularly of lungs, heart, or liver.<br /><br />In summary, sarcoidosis requires a comprehensive evaluation due to its multi-organ effects, with treatment tailored to symptom severity and disease progression.
Asset Subtitle
Rida Aziz, Nicole Terrigno
Keywords
Sarcoidosis
noncaseating granulomas
multisystem disorder
pulmonary sarcoidosis
lupus pernio
uveitis
Löfgren syndrome
glucocorticoids
immunosuppressants
TNF-alpha inhibitors