From IDWiki


  • Thought to be related to abnormal T-cell activation

Differential Diagnosis

  • Hilar lymphadenopathy
    • Sarcoidosis
    • Infection: TB, fungal, HIV, mycoplasma
    • Malignancy: lymphoma
    • Others

Scadding Classification

  1. Stage I: Bilateral hilar lymphadenopathy (70% resolve)
  2. Stage II: Above, with interstitial lung disease (50% resolve)
  3. Stage III: Interstitial lung disease alone (15% resolve)
  4. Stage IV: Fibrotic, "burnt out" lungs (0% resolve)

Risk Factors

  • Women more than men (2:1)
  • More common in African-Americans

Clinical Manifestations

Extrapulmonary disease

  • Skin: Erythema nodosum and lupus pernio, and others
  • Cardiac: 40% of patients, though only 5-10% are symptomatic
  • CNS: 5-10%, multiple presentations
  • Eyes: 10% of all uveitis cases, usually bilateral
  • Hypercalcemia
  • Nephrocalcinosis


  • Evidence of granulomatous inflammation (often on BAL or EBUS) without infection
  • Either lung involvement or multiorgan involvement


  • Labs
    • CBC, lytes, creatinine, calcium, liver panel
  • Imaging
    • High-res CT scan
  • Other
    • PFTs: most commonly restrictive with decreased DLCO, but can show combined restriction-obstruction, or rarely any other pattern. Often normal.
    • EKG for cardiac involvement
    • Eye exam for uveitis


  • Only treat if symptomatic, as many will resolve spontaneously
    • Spontaneous remission depends on Scadding stage (I 70%, II 50%, III 15%, IV 0%)
  • Prednisone 20-40mg daily for 8-12 weeks, then taper
    • Add vitamin D and calcium if serum calcium is low
    • Can still use bisphosphates for bone protection
  • Second-line steroid-sparing agents include
    • Methotrexate
    • Azathioprine, leflonamide, MMF, hydroxychloroquine, thalidomide
    • TNG-alpha inhibitors are last line

Management by organ system

Organ Clinical Features Treatment
Lungs Dyspnea w FEV1 or FVC <70% Prednisone 24-40 mg/day
Lungs Cough, wheeze Inhaled corticosteroid
Eyes Anterior uveitis Topical corticosteroid
Eyes Posterior uveitis Prednisone 20-40 mg/day
Eyes Optic neuritis Prednisone 20-40 mg/day
Skin Lupus pernio Prednisone 20-40 mg/day
Hydroxychloroquine 400 mg/day
Thalidomide 100-150 mg/day
Methotrexate 10-15 mg/week
Skin Plaques or nodules Prednisone 20-40 mg/day
Hydroxychloroquine 400 mg/day
Skin Erythema nodosum NSAID
CNS Cranial nerve palsies Prednisone 20-40 mg/day
CNS Intracerebral involvement Prednisone 40 mg/day
Azathioprine 150 mg/day
Hydroxychloroquine 400 mg/day
Heart Complete heart block Pacemaker
Heart Ventricular fibrillation or tachycardia AICD
Heart Decreased LVEF <35% AICD and prednisone 30-40 mg/day
Liver Cholestatic hepatitis with constitutional symptoms Prednisone 20-40 mg/day
Ursodiol 15 mg/kg per day
MSK Arthralgias NSAID
MSK Granulomatous arthritis Prednisone 20-40 mg/day
MSK Myositis or myopathy Prednisone 20-40 mg/day
Calcium Kidney stones, fatigue Prednisone 20-40 mg/day
Hydroxychloroquine 400 mg/day

from the [NEJM sarcoidosis review article][1]

Further Reading