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Sarcoidosis

Lale Ertuglu

Background

  • Multisystem disordered defined by forming noncaseating granulomas in different tissues

Presentation

  • Constitutional symptoms: fatigue, night sweats, weight loss, fevers, arthralgias, myalgias
  • Pulmonary symptoms (most common): dyspnea, cough, and chest pain
  • 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% of patients; 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
      • Arrhythmias are most common manifestation
    • 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
    • Lofgren Syndrome
      • Acute presentation with fever, bilateral hilar adenopathy, erythema nodosum or ankle arthralgia
  • Incidental finding in chest imaging: >90% of patients have pulmonary or thoracic lymphadenopathy on presentation and ~50% of patients present with only incidental radiological findings

Evaluation

  • Combination of clinical features, radiographic manifestations, exclusion of other similarly presenting diseases, and noncaseating granulomas on pathology
  • CXR: hilar and mediastinal lymphadenopathy ± pulmonary infiltrates. CXR stages are defined as below (stages do not represent disease activity)
    • Stage 1: bilateral hilar adenopathy only
    • Stage 2: bilateral hilar adenopathy + pulmonary infiltrates
    • Stage 3: pulmonary infiltrates without hilar adenopathy
    • Stage 4: pulmonary fibrosis that mainly involves upper lung zones
  • High-Resolution chest CT: Lymphadenopathy (bilateral and symmetric), perilymphatic micro or macronodules, fibrotic changes (reticular opacities, traction bronchiectasis, volume loss, cysts)
  • 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 or tuberculin skin test, HIV. Depending on endemic fungi, serologic testing for histoplasmosis or coccidiomycosis can also be included.
  • ECG: should be obtained since AV block is the most common finding of cardiac sarcoidosis.
  • Biopsy
    • Important to rule out mimics. The differential for “noncaseating granulomas” is extensive, including lymphoma and fungal infections
    • Not required for patients with asymptomatic bilateral hilar adenopathy or pathognomonic presentations including Lofgren syndrome and some cases of lupus pernio

Management

  • Most 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 symptom is cough, could consider inhaled glucocorticoids
    • If unresponsive or unable to tolerate steroids may require alternative agents (MTX, AZA, TNFi)