Sarcoidosis


Immunopathogenesis: Please see under https://ci-and-pulmonary-disease

Clinical Features: Lancet 2014

Most common clinical presentations are : 
  • persistent cough, 
  • localization of disease in the skin, 
  • eye, and 
  • peripheral lymph nodes, 
  • erythema nodosum, 
  • fatigue, and 
  • incidental abnormal chest radiograph 

DDx
  • infections, particularly tuberculosis; 
  • occupationally induced, environmentally induced, and drug-induced granulomatosis; chronic pulmonary berylliosis is dependent on a focused questionnaire and on beryllium hypersensitivity
  • common variable immune deficiency. Diagnosis of common variable immune deficiency relies on hypogammaglobulinaemia,
  • Blau’s syndrome; 
  • sarcoid-like reactions in cancers and lymphomas, and 
  • other idiopathic granulomatosis.  

Diagnosis: Lancet 2014

Three most important advancement in diagnosis has been:
  • PET scan
  • Rapid on-site assessment by the well trained cytologists 
  • EUS guided Needle aspirations
Diagnosis is based on the following. The weight of each of them depends on the clinical scenarios. 
  • Clinical Picture and Radiological Picture
  • Caseating granulomas, and 
  • evidence of no alternative findings 
Most common diagnostic signs are 
  • bilateral intrathoracic hilar lymphadenopathy or diffuse micronodular pulmonary infiltration at chest radiograph, 
  • associated with a typical lymphatic distribution or a galaxy sign on CT  and 
  • the presence of some extrapulmonary localisations of disease—eg, in the eye and skin 

Sarcoidosis Lancet 2014


Sarcoidosis Lancet 2014


Additional Clinical Features: Sarcoidosis NEJM 2007
  • Constitutional symptoms such as fatigue may predominate.
  • Cardiac sarcoidosis is much more common than reported previously and may cause loss of ventricular function and sudden death.
  • Cardiac and neurologic sarcoidosis may occur without apparent disease activity in other organs. 
  • Chest radiographic patterns (stages 1, 2, and 3) do not reflect the chronology of the disease. 
  • A response to corticosteroid therapy does not establish the diagnosis of sarcoidosis.
  • Measurement of the serum angiotensin-converting–enzyme level is an insensitive and nonspecific diagnostic test and a poor therapeutic guide.
  • For patients without apparent lung involvement, 18FDG PET is useful in identifying sites for diagnostic biopsy.
  • 18FDG PET and MRI with gadolinium detect cardiac and neurologic involvement. 
  • CT imaging is unnecessary for most patients with sarcoidosis. CT is indicated when the chest radiograph is atypical for sarcoidosis or when hemoptysis occurs. 
Clinical Course
  • Acute  : 2 years
  • Chronic : 3–5 years
  • Refractory : progressing despite treatmen
References:
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