Sarcoidosis, Fibrotic

Sarcoidosis, Fibrotic

Melissa L. Rosado-de-Christenson, MD, FACR

Axial HRCT shows multifocal areas of architectural distortion with intrinsic traction bronchiectasis image in a patient with sarcoidosis. A few perilymphatic nodules image are still visible.

Axial HRCT of a patient with end-stage sarcoidosis shows advanced central fibrosis and architectural distortion. Mass-like fibrosis image encases the hila and exhibits traction bronchiectasis.



  • American Thoracic Society definition

    • Multisystem granulomatous disorder of unknown etiology; affects young and middle-aged adults

    • Frequent hilar lymphadenopathy, pulmonary involvement, eye and skin lesions

    • Typical clinical and imaging findings supported by histologic evidence of noncaseating epithelioid granulomas

    • Exclusion of other granulomatous diseases and local sarcoid reactions

  • Löfgren syndrome: Bilateral hilar lymphadenopathy, erythema nodosum, fever, arthralgias/arthritis

  • Heerfordt syndrome: Uveitis, parotitis, fever

  • Lupus pernio: Chronic subcutaneous induration and purplish discoloration of central face and hands

    • Produced by granulomatous infiltration; associated with sarcoidosis


General Features

  • Best diagnostic clue: Predominant fibrosis and architectural distortion of upper lobe

  • Patient position/location: Central upper lung fibrosis

  • Morphology

    • Architectural distortion

    • Cystic changes

    • Subpleural honeycomb lung

CT Findings

  • Lung

    • Smooth or nodular peribronchovascular thickening, follows lymphatic pathways in lung

    • Decreased number of small perilymphatic nodules as they aggregate into mass-like lesions

    • Cystic changes

      • May exhibit intracystic soft tissue related to saprophytic fungus (mycetoma)

      • True cavities uncommon

    • Peripheral subpleural honeycomb lung

    • Linear opacities

  • Airways

    • Traction bronchiectasis (central)

    • Bronchial wall thickening and airway stenosis

  • Distribution

    • Predominantly upper lung zone

    • Posterior displacement of main and upper bronchi

      • Secondary to volume loss in posterior segments of upper lobes

      • Hilar retraction from volume loss

    • Central perihilar conglomerate masses cut swath through lung

  • Lymphadenopathy

    • All nodal stations on CT

    • Bilateral hilar, right paratracheal, AP window, subcarinal

    • Rarely: Dominant anterior mediastinal, unilateral hilar, posterior mediastinal

    • Calcification may be present in varying degrees

      • “Eggshell”

      • Central smudgy or putty-like

    • As lung findings in sarcoid advance, adenopathy typically resolves or decreases in size

  • Complications

    • Pulmonary artery hypertension: Enlarged central pulmonary arteries, dilatation of right atrium and ventricle

  • Pleura

    • Pleural effusion/thickening or pneumothorax uncommon

    • Ipsilateral pleural thickening; typically precedes mycetoma formation

Radiographic Findings

  • Radiography

    • Findings not as specific as CT

    • Radiographic staging of sarcoidosis

      • Stage 0: Normal chest radiograph

      • Stage I: Lymphadenopathy, no pulmonary disease

      • Stage II: Lymphadenopathy and pulmonary disease

      • Stage III: Pulmonary disease, no lymphadenopathy

      • Stage IV: Pulmonary fibrosis

Imaging Recommendations

  • Best imaging tool: CT to characterize lung disease



  • Progressive massive fibrosis

  • Irregular conglomerate masses

    • Associated cavitation and traction bronchiectasis

  • Cicatricial emphysema

  • Small perilymphatic nodules

  • Lymphadenopathy; may exhibit calcification

Post-Primary Tuberculosis

  • Mycobacterium tuberculosis infection

  • Upper lobe predominant; apical and posterior segments

  • Frequent cavitation; associated bronchiolitis

  • Upper lobe architectural distortion, hilar retraction, traction bronchiectasis

Chronic Hypersensitivity Pneumonitis

  • Exposure history

  • Architectural distortion, septal thickening, honeycomb lung, traction bronchiectasis

  • Ground-glass opacities, centrilobular nodules


  • History of IV drug abuse

  • Background nodules usually pinpoint in size

  • Progressive massive fibrosis (PMF) may be of high density


General Features

Sep 20, 2016 | Posted by in RESPIRATORY IMAGING | Comments Off on Sarcoidosis, Fibrotic
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