sarcoidosis%20-%20pulmonary
SARCOIDOSIS - PULMONARY
Sarcoidosis is a multisystem disease which may present with non-specific symptoms or symptoms related to organ-specific involvement.
Non-specific symptoms include fever, malaise, fatigue and weight loss.
Pulmonary involvement is seen in >90% of sarcoidosis patients.

Diagnosis

  • Diagnosis is often delayed because of the usual non-specific presentation

Diagnosis is based on the following:

  • Compatible clinical or radiologic picture or both
  • Histologic proof of non-caseating granuloma
  • Exclusion of similar diseases

Staging

Staging of Pulmonary Sarcoidosis based on Chest X-ray (CXR)

Stage 0

  • Normal CXR

Stage 1

  • Lymphadenopathy
    • Hilar & paratracheal nodes are usually involved
    • Parenchymal granulomas may be found on biopsy even in the absence of infiltrates on CXR

Stage 2

  • Lymphadenopathy w/ parenchymal infiltrates

Stage 3

  • Parenchymal infiltration w/o lymphadenopathy
  • Progressive loss of lung function may occur

Stage 4

  • Advanced fibrosis
  • Other findings include emphysema, bullae, cysts, hilar retraction & honey-combing
  • Progressive loss of lung function may occur

History

  • A thorough clinical history & physical exam should be obtained
  • Though the cause of sarcoidosis is unknown, the patient should be questioned carefully about exposure to both inorganic & organic antigens
  • Investigate possible environmental & occupational exposures that may reveal an alternative diagnosis
    • Exposure to metal dusts, fumes & organic antigens may cause granulomatous diseases that are difficult to differentiate from sarcoidosis

Extrapulmonary Disease

  • Physical exam should include a search for evidence of extrapulmonary disease
    • Evidence of multi-organ involvement is supportive of a diagnosis of sarcoidosis

Laboratory Tests

Biopsy & Histology

  • Transbronchial lung biopsy to demonstrate granulomas is recommended in most cases
    • Has a higher yield for patients w/ parenchymal lung disease seen on CXR or CT scan
  • Accessible skin lesions &/or lymph nodes may also be biopsied
  • Surgical biopsy is indicated when transbronchial or bronchial biopsies are not diagnostic & no other accessible biopsy sites are identified

Other Diagnostic Procedures/Laboratory Exams

Bronchoalveolar Lavage (BAL)

  • Increased lymphocytes are seen or an increased CD4:CD8 ratio
  • Findings are supportive, but not definitive of a diagnosis

Angiotensin Converting Enzyme (ACE) Levels

  • Elevations are not diagnostic since other diseases & granulomatous conditions may present w/ high levels of ACE
  • Additional evaluation is recommended in all patients diagnosed w/ sarcoidosis

Laboratory Exams

  • Pulmonary function tests
    • Spirometry
    • Diffusing capacity for carbon monoxide (DLco)
  • Peripheral blood counts
  • Serum chemistries
    • Blood urea nitrogen (BUN), creatinine, calcium
    • Liver enzymes: Aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP) 
  • Urinalysis
    • Patients may present w/ hypercalciuria
  • Electrocardiogram (ECG)
    • Patients may manifest w/ cardiac arrhythmias
  • Routine ophthalmologic evaluation
    • To determine if eye involvement is present
  • Tuberculin test

Imaging

Chest X-ray (CXR)

  • Presence of bilateral hilar enlargement may speed up the diagnostic process
  • Interstitial disease alone may be seen

Computed Tomography (CT) scan

  • May be used to identify patients w/ parenchymal disease not seen on CXR
  • Demonstrates hilar & mediastinal lymphadenopathy
  • May also be done when patients present w/ atypical clinical or radiologic findings
  • Allows detection of complications of the disease (eg bronchiectasis, aspergilloma, pulmonary fibrosis)
  • Consider CT when there is clinical suspicion of the disease but the CXR is normal

Total Body Gallium Scan

  • Appearance of a Panda pattern combined w/ a Lambda pattern may support diagnosis
  • Findings are present only in a small number of patients
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