Idiopathic pulmonary fibrosis differential diagnosis
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Differentiating Idiopathic pulmonary fibrosis from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Differentiating Idiopathic Pulmoary Fibrosis from other Disease
The key issue facing clinicians is whether the presenting history, symptoms/signs, radiology, and pulmonary function testing are collectively in keeping with the diagnosis of IPF (which carries the relatively poor prognosis described above) or whether the findings are due to another process. It has long been recognized that patients with interstitial lung disease related to asbestos exposure, drugs (particularly chemotherapeutic agents), a connective tissue disease, or other diseases may have features that are difficult to distinguish from IPF. Important differential diagnostic considerations include asbestosis; interstitial lung disease related to scleroderma, mixed connective tissue disease, or rheumatoid arthritis; advanced sarcoidosis, hypersensitivity pneumonitis, or Langerhans cell histiocytosis; chronic pulmonary aspiration; radiation-induced fibrosis; as well as previous therapy with cyclophosphamide, nitrofurantoin, methotrexate, and other drugs.
When diagnostic uncertainty remains, a surgical lung biopsy may be required to establish the diagnosis. Generally, lung biopsy is only undertaken when it is deemed that its risks are outweighed by the potential benefits of identifying a disease process that may be amenable to a treatment that the patient would likely be able to tolerate.
The 2002 American Thoracic Society/European Respiratory Society Consensus Guidelines on the Idiopathic Interstitial Pneumonias have formalized criteria for situations in which it is possible to establish the diagnosis of IPF without a lung biopsy.