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Sarcoidosis - Stage I

Pathophysiology:  A non-caseating granulomatous disease of unknown etiology.  It is largely believed this is an immunologic reaction to antigenic stimuli, probably inhaled antigens.

 

The disease involves multiple organ systems including: lungs, heart, skin, central and peripheral nervous systems, and bone.  The lung is the most commonly involved organ. Adenitis, vasculitis, alveolitis, and DAD occur with resulting small nodular changes and interstitial fibrosis.  End stage lung changes and marked lung distortion can occur.

 

Clinical Clues:

 

  1. Sarcoid is one of the DISCORDANT lung diseases. This can be vital in diagnosis. The often marked lung findings are discordant with the clinical findings; the patients are often asymptomatic **

  2. Sarcoidosis is the “syphilis of the chest and can look at times like many diverse lung diseases and processes.

  3. Check for angiotensin converting enzyme (ACE) levels and serum calcium.

 

Imaging Clues:

 

  1. pleural effusions are rare, and rarely is the major finding

  2. asymmetric or focal adenopathy is rare at presentation

 

Staging of Sarcoidosis: (thoracic disease)

 

Stage 0:  Disease elsewhere, no thoracic involvement

Stage I:  Hilar, paratracheal, and aortopulmonary adenopathy only (Garlin’s triad)

Stage II:  Adenopathy and interstitial lung disease changes

Stage III: Interstitial lung disease only

Stage IV: End stage lung changes with lung distortion

 

CXR/CT Findings (Stage I):  “potato” hila: lobular and bulky hilar, paratracheal, and aortopulmonary adenopathy.

 

“Aunt Sophies”: a differential gamut of hilar adenopathy and enlarged hila

 

  1. Enlarged central pulmonary arteries: PAH, CHF, chronic lung sepsis  (eg. CF, chronic bronchiectasis)

  2. Hilar and mediastinal adenopathy:

        Infections: granulomatous (eg. TB), viral (eg. infectious mononucleosis)

        Tumor: nodal metastases, lymphoma, local infiltration of small cell cancer, Castleman's disease

        Inflammatory: silicosis, berylliosis

        Infiltrative diseases: eg. pulmonary amyloidosis

 


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