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Jared Freitas


  • Multisystem disordered defined by forming noncaseating granulomas in different tissues

  • Cardiac and neuro manifestations can be isolated


  • Constitutional symptoms: fatigue, night sweats, weight loss, fevers, arthralgias, myalgias

  • Pulmonary symptoms (most common): dyspnea, cough, and wheezing

  • Extrapulmonary manifestations:

    • Cutaneous: Highly variable, but present in 25% of patients

      • Papules, macules, or plaques commonly involving neck, upper back, extremities

      • Lupus pernio: indurated, violaceous bumps on nose, lips, cheeks, ears

      • Erythema nodosum

    • Neuro

      • Affects 5-10% pts; involving any part of CNS or PNS

      • CN palsies, hypothalamic/pituitary dysfunction, seizures, myelopathy or radiculopathy, hydrocephalus, aseptic meningitis

    • Cardiac

      • Granulomas can affect pericardium, myocardium and endocardium resulting in valvular disorders, conduction system and cardiomyopathy
    • Liver/Spleen

      • Granulomas in liver and spleen can lead to elevated LFTs, cirrhosis, anemia, leukopenia and thrombocytopenia (splenic sequestration)
    • Ocular

      • Uveitis, secondary glaucoma, retinal vasculitis, keratoconjunctivitis

Work Up

  • Combination of clinical features, radiographic manifestations, exclusion of other similarly presenting diseases, and noncaseating granulomas on pathology

    • CXR: hilar and mediastinal lymphadenopathy ± pulmonary infiltrates

    • High-Resolution Chest CT

      • Lymphadenopathy (bilateral and symmetric), with micro or macronodules, fibrotic changes (reticular opacities, traction bronchiectasis, volume loss)
    • PFTs

      • May show restrictive disease (decreased TLC & VC) and diffusion impairment (reduced DLCO). Occasionally obstructive with endobronchial disease.
    • Labs: CBC w/ diff, CMP, UA, quant-gold for TB. ACE level is of limited clinical use

    • Biopsy

      • Important to rule out mimics. The differential for “noncaseating granulomas” is extensive, including lymphoma and fungal infections.

      • Not required for pts w/ asymptomatic bilateral hilar adenopathy or Lofgren syndrome (fever, erythema nodosum, arthralgias, and bilateral hilar LAD)


  • Most pts do not require therapy: monitor symptoms, CXR, PFTs at 3-6 month intervals

  • Indications for treatment: progressive disease or severe disease at presentation

  • Mainstay of treatment is oral steroids:

    • Dosing usually 0.3-0.6 mg/kg daily for 4-6 weeks

    • If only symptoms is cough, could consider inhaled glucocorticoids

  • If unresponsive or unable to tolerate steroids may require alternative agents (MTX, AZA)

Last update: 2022-06-27 03:18:16