DIAGNOSIS

Symptoms

  • Gradual onset of dyspnea and dry cough over weeks or months3
  • Cyanosis is a common presenting symptom1
  • About 1/2 of patients with DIP will develop clubbing3

DIAGNOSTIC TESTS

  • Pulmonary function tests
    • DLCO is moderately decreased3,7
    • Normal lung volumes or a mild restrictive abnormality3
  • HRCT
    • Features: ground-glass opacities, irregular linear or reticular opacities, sometimes cysts8
    • Distribution: apicobasal gradient, peripheral predominance8

RB-ILD, hypersensitivity pneumonitis, sarcoidosis, and infections such as Pneumocystis carinii pneumonia may not be distinguishable from DIP on HRCT.3

  • Surgical lung biopsy should be used in certain cases9
    • Histopathology results featuring diffuse accumulation of macrophages in distal airspaces, mild interstitial fibrosis, mild chronic inflammation indicate a diagnosis of DIP8
  • Bronchoalveolar lavage findings are not usually very specific1
    • Features:
      • Increased number of alveolar macrophages
      • Large number of which have granules of smoker's-pigment consisting of intracellular yellow, gold, brown, or black smoke particulates3
  • Chest X-ray is not sensitive enough for detection of DIP and appears normal in 3%-22% of biopsy-proven cases3
    • Features:
      • Widespread patchy ground glass opacification
      • Lower zone predilection
      • Sometimes a peripheral predominance

See also

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Interstitial lung disease in systemic sclerosis with a focus on chest CT

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