Fact-sheet: Thoracic sarcoidosis


Updated on 04/18/2018 at 4:34 PM

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Definition

Systemic granulomatosis of unknown etiology affecting young adults.
Thoracic, peripheral lymph node, ophthalmologic, dermatologic, ENT, and hepatic involvement.
The mediastinopulmonary location is the most common.

Clinical features

Cough.
Dyspnea in advanced forms.

Laboratory findings

Hypercalciuria due to elevated calcitriol.
Lymphopenia predominating in CD4 T cells.
Polyclonal hypergammaglobulinemia on serum protein electrophoresis.
Negative tuberculin skin test in 80% of cases (tuberculin anergy).
Elevated angiotensin-converting enzyme (ACE).

Biopsy:

Sampling of a peripheral lesion or bronchial biopsy: epithelioid and giant cell granulomas without caseating necrosis.

Radiography

Stage 0: normal radiograph.
Stage I: isolated bilateral hilar and mediastinal lymphadenopathy.
Stage II: hilar and mediastinal lymphadenopathy associated with nonfibrotic pulmonary parenchymal involvement.
Stage III: isolated nonfibrotic pulmonary parenchymal involvement.
Stage IV: diffuse pulmonary fibrosis, often apical predominant.

CT

Reversible lesions:

  • Lymphatic micronodules (bronchovascular bundles, interlobular septa, subpleural regions).
  • Confluent nodules.
  • Peribronchovascular thickening.
  • Airspace opacities.
  • Multifocal, irregularly distributed ground-glass opacities.
  • Posterior and upper lobe predominance of abnormalities.
  • Lymphadenopathy.

Irreversible lesions: (stage IV)

  • Bronchovascular distortion: retraction, angulation, and irregularity of bronchial walls, traction bronchiectasis
  • Fibrosis predominating in the upper and middle lobes with honeycombing.
  • Hilo-peripheral or septal linear opacities.