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For further information, see CMDT Part 9-21: Diffuse Interstitial Pneumonias

Key Features

Essentials of Diagnosis

  • Important to identify specific fibrosing disorders

  • Idiopathic disease may require biopsy for diagnosis

  • Accurate diagnosis identifies patients most likely to benefit from therapy

General Considerations

  • Historically, diagnosis was based on clinical and radiographic criteria, with lung biopsy uncommon

  • Several histologic patterns once grouped together as idiopathic pulmonary fibrosis (IPF) are now understood to represent different natural histories and responses to therapy

  • Evaluation must first identify patients whose disease is truly idiopathic (Table 9–17)

  • Most identifiable causes of interstitial lung diseases

    • Infectious

    • Drug-related

    • Exposures

    • Associated with other medical conditions

  • A specific diagnosis allows providers to give accurate information on natural history and to distinguish patients most likely to benefit from treatment

  • Assessment of pulmonary hypertension is recommended in advanced disease

Table 9–17.Idiopathic interstitial pneumonias.

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