Sarcoidosis

Key Facts
  • Common systemic disease
  • Etiology unknown
  • Widespread noncaseating granulomas, that resolve or cause fibrosis
  • May have associated erythema nodosum, uveitis, hypercalcemia, arthritis
  • 95% of patients have abnormal chest radiograph with lymphadenopathy and or lung opacities
  • HRCT centriacinar nodules along bronchovascular bundles, septa,and periphery of lobule
  • Definitive diagnosis with transbronchial, lymph node, or liver biopsy
  • Most patients have a good prognosis with resolution of findings in < 2 years
  • Major complications include respiratory failure from fibrosis, mycetomas,hemorrhage, and cor pulmonale
Imaging Findings

Chest radiography

  • Abnormal chest radiograph – 95%
  • 5 to 15% have normal chest radiograph at onset
    • Lymphadenopathy, (80%) most common finding – bilateral hilar/paratracheal
      Usually not visible at 2 years; may persist for many years
      Nodes may calcify, sometimes eggshell calcification
    • Lung disease, (<50%), often manifest with nodal regression
    • Pattern and Distribution:
      • Reticulonodular opacities (90%)
      • Large airspace nodules with air bronchograms
      • Fibrosis mid and upper lung zones
      • Upper lobe cyst formation with aspergilloma

      Stage 0 – clear chest radiograph (5 – 15%, at presentation)

      Stage 1 – lymphadenopathy (45 – 65%); 60% resolve completely

      Stage 2 – lymphadenopathy and lung opacities (30 – 40%)

      Stage 3 – Lung opacities (10 – 15%)

      Stage 4 – fibrosis with or without lymphadenopathy (5 – 25%)

    • Atypical appearance
      • Atypical lymphadenopathy - unilateral hilar, posterior mediastinal
      • Airway compression
      • Unilateral lung disease
      • Cavitary lung lesions
      • Pleural effusion

CT/HRCT

  • CT may show lymphadenopathy at left paratracheal, AP window, anterior mediastinum, retroperitoneal
  • HRCT, may be abnormal with normal CXR
  • Predominantly involves the mid and upper lung zones
  • Pattern: 1 – 5 mm centrilobular nodules along brochovascular structures, septa, and periphery of lobule
  • Often extends in a swath from the hilum to lung periphery
  • Ground glass opacities
  • Progressive massive fibrosis, distortion, honeycombing, cysts, bullae, traction bronchiectasis
  • Mycetomas in cavities and cysts
  • Large and small airway stenoses
Differential Diagnosis
  • Granulomatous diseases : TB, fungal infection, berylliosis, extrinsic allergic alveolitis
  • Diseases with granuloma-like reactions: lymphoma, carcinoma, metastases
  • Chronic eosinophilic pneumonia, BOOP
Pathological Features
  • Common systemic disease
  • Widespread noncaseating granulomas, that resolve or cause fibrosis
  • Etiology unknown
  • Well-formed granulomas ; central epitheliod histiocytes and multinucleate giant cells surrounded by lymphocytes, monocytes and fibroblasts
  • Lymphatic distribution
Clinical Presentation
  • 10 times more common in blacks, female predominance
  • Onset – usually age 20 to 40
  • Asymptomatic, or fatigue, malaise, weight loss, fever, respiratory symptoms, erythema nodosum, uveitis, skin lesions, arthropathy, bone lesions
  • In < 2% TB precedes sarcoidosis or develops later
  • 80% of cases resolve completely; in 20% fibrosis develops that may destroy and distort the lung
  • Anemia, leukopenia, elevated sedimentation rate, hypercalcemia, nephrolitiasis
  • Cutaneous anergy
  • Raised ACE level, not specific
  • Diagnosis from lung, lymph nodes, and liver
  • Bronchoscopy and transbronchial biopsy – positive in 90% of cases, even if CXR is normal
  • BAL – increased CD4/CD8 ratio, nonspecific finding
  • Usually not treated; steroids in severe cases
  • Recurrence in transplanted lung has been reported
  • Prognosis worse in blacks
  • Mortality – 2 – 7%; death from respiratory failure, cor pulmonale, hemorrhage
References

Miller BH, Rosado-de-Christenson ML, McAdams HP, et al. Thoracic sarcoidosis: radiologic-pathologic correlation Radiographics 15:421-437, 1995
Rockoff SD , Rohatgi PK. Unusual manifestations of thoracic sarcoidosis AJR 144:513-528, 1985
Traill ZC, Maskell GF , Gleeson FV. High-resolution CT findings of pulmonary sarcoidosis AJR 168:1557-1560, 1997