SHM's Clinical Quick Talks-Sarcoidosis

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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.

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Rida Aziz, Nicole Terrigno

Meta Tag

Concept Sarcoidosis

Concept Noncaseating Granuloma

Concept Pulmonary Manifestations

Concept Lupus Pernio

Concept Uveoparotid Fever

Keywords

Sarcoidosis

noncaseating granulomas

multisystem disorder

pulmonary sarcoidosis

lupus pernio

uveitis

Löfgren syndrome

glucocorticoids

immunosuppressants

TNF-alpha inhibitors

Sarcoidosis

Noncaseating Granuloma

Pulmonary Manifestations

Lupus Pernio

Uveoparotid Fever