Sarcoidosis¶
Jared Freitas
Background¶
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Multisystem disordered defined by forming noncaseating granulomas in different tissues
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Cardiac and neuro manifestations can be isolated
Presentation¶
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Constitutional symptoms: fatigue, night sweats, weight loss, fevers, arthralgias, myalgias
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Pulmonary symptoms (most common): dyspnea, cough, and wheezing
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Extrapulmonary manifestations:
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Cutaneous: Highly variable, but present in 25% of patients
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Papules, macules, or plaques commonly involving neck, upper back, extremities
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Lupus pernio: indurated, violaceous bumps on nose, lips, cheeks, ears
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Erythema nodosum
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Neuro
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Affects 5-10% pts; involving any part of CNS or PNS
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CN palsies, hypothalamic/pituitary dysfunction, seizures, myelopathy or radiculopathy, hydrocephalus, aseptic meningitis
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Cardiac
- Granulomas can affect pericardium, myocardium and endocardium resulting in valvular disorders, conduction system and cardiomyopathy
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Liver/Spleen
- Granulomas in liver and spleen can lead to elevated LFTs, cirrhosis, anemia, leukopenia and thrombocytopenia (splenic sequestration)
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Ocular
- Uveitis, secondary glaucoma, retinal vasculitis, keratoconjunctivitis
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Work Up¶
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Combination of clinical features, radiographic manifestations, exclusion of other similarly presenting diseases, and noncaseating granulomas on pathology
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CXR: hilar and mediastinal lymphadenopathy ± pulmonary infiltrates
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High-Resolution Chest CT
- Lymphadenopathy (bilateral and symmetric), with micro or macronodules, fibrotic changes (reticular opacities, traction bronchiectasis, volume loss)
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PFTs
- May show restrictive disease (decreased TLC & VC) and diffusion impairment (reduced DLCO). Occasionally obstructive with endobronchial disease.
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Labs: CBC w/ diff, CMP, UA, quant-gold for TB. ACE level is of limited clinical use
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Biopsy
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Important to rule out mimics. The differential for “noncaseating granulomas” is extensive, including lymphoma and fungal infections.
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Not required for pts w/ asymptomatic bilateral hilar adenopathy or Lofgren syndrome (fever, erythema nodosum, arthralgias, and bilateral hilar LAD)
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Management¶
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Most pts do not require therapy: monitor symptoms, CXR, PFTs at 3-6 month intervals
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Indications for treatment: progressive disease or severe disease at presentation
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Mainstay of treatment is oral steroids:
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Dosing usually 0.3-0.6 mg/kg daily for 4-6 weeks
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If only symptoms is cough, could consider inhaled glucocorticoids
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If unresponsive or unable to tolerate steroids may require alternative agents (MTX, AZA)