Sarcoidosis differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Roshan Dinparasti Saleh M.D.

Overview

Sarcoidosis has many differentials, which can be classified depending on the organ involved, pathologic findings and laboratory findings.

Differential Diagnosis

Sarcoidosis has been defined as a multisystem granulomatous disorder of unknown cause, but granulomatous inflammation alone is not sufficient for the diagnosis of sarcoidosis because alternative etiologies of granulomatous inflammation need to be excluded.

Causes of granulomatous reaction beside sarcoidosis

• Mycobacterium tuberculosis
• Mycoplasma
• Pneumocystis jiroveci
• Brucellosis
• Cat-scratch fever
• Atypical mycobacteria
• Toxoplasmosis
• Berylliosis
• Hard metal
• Zirconium
• Tattoo
• Hypersensitivity pneumonitis
• Medications (e.g., methotrexate)
• ANCA-associated vasculitis
• Necrotizing sarcoid granuloma
• Lymphoma
• Cancer
• Granulomatous lesions of unknown significance
• Crohn’s disease
• Lymphocytic interstitial pneumonia
• Behçet’s disease
• Rheumatoid nodules

Data supporting the likelihood of sarcoidosis

1. Demographics
   1. U.S. African American
   2. Northern European
2. Medical history
   1. Non-smoker
   2. No symptoms (in patient with bilateral hilar adenopathy on CXR)
   3. Family history of sarcoidosis
   4. Symptoms involving more than two organs commonly involved by sarcoidosis
3. Laboratory data
   1. Elevated ACE level (especially if > 2× ULN)
   2. Elevated calcium level
   3. Elevated alkaline phosphatase level
   4. Elevated soluble IL-2 receptor
   5. Leukopenia
4. Radiographic findings
   1. CXR:
      1. bilateral hilar adenopathy especially if without symptoms
      2. Upper lobe disease
   2. HRCT:
      1. Disease along bronchovascular bundle
      2. Subpleural reticulonodular infiltrates
      3. Mediastinal adenopathy
      4. Peribronchial thickening
      5. Traction bronchiectasis of upper lobe
5. Skin lesions
   1. Lupus pernio
   2. Erythema nodosum
   3. Maculopapular lesions
6. Ocular disease
   1. Uveitis
   2. Optic neuritis
7. Neurological disease
   1. Seventh cranial nerve palsy
8. Renal disease
   1. Nephrocalcinosis
   2. Interstitial nephritis

Data weakening the likelihood of sarcoidosis

1. Demographics
   1. Age< 18 years

1. Medical history
   1. Exposure to tuberculosis
   2. Exposure to organic bioaerosol
   3. Exposure to beryllium
   4. Intravenous drug abuse

1. Laboratory data
   1. Positive anti-neutrophil cytoplasm antibody (ANCA)

1. Radiographic findings
   1. CXR:
      1. Pleural effusion
   2. HRCT:
      1. Subpleural honeycombing

1. Ocular disease
   1. Episcleritis

1. Renal disease
   1. Glomerulonephritis