SynopsisCodesDifferential Diagnosis & PitfallsBest TestsDrug Reaction DataReferences
Nonspecific interstitial pneumonia
Other Resources UpToDate PubMed

Nonspecific interstitial pneumonia

Contributors: Christine Osborne MD, Mary Anne Morgan MD
Other Resources UpToDate PubMed


Part of the family of idiopathic interstitial pneumonias, but lacks the histopathologic features to specifically classify the disease. Patients typically present with the subacute development of dyspnea and cough. Other presenting symptoms such as Raynaud phenomenon, dry mouth or eyes, or rash or skin changes may point to an underlying collagen-vascular disease. Physical examination typically reveals bibasilar crackles.

While there is no definitive cause, there are associations with collagen-vascular diseases including polymyositis-dermatomyositis, rheumatoid arthritis, Sjögren syndrome, and systemic sclerosis, infections such as human immunodeficiency virus (HIV), and medications such as flecainide, amiodarone, methotrexate, carmustine, nitrofurantoin, statins, and chlorambucil.


J84.113 – Idiopathic non-specific interstitial pneumonitis

129452008 – Nonspecific interstitial pneumonia

Differential Diagnosis & Pitfalls

  • Usual interstitial pneumonia (idiopathic pulmonary fibrosis)
  • Desquamative interstitial pneumonia
  • Extrinsic allergic alveolitis
  • Respiratory bronchiolitis with interstitial lung disease
  • Acute interstitial pneumonia
  • Cryptogenic organizing pneumonia
  • Infection

Best Tests

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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Last Updated:09/18/2016
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Nonspecific interstitial pneumonia
A medical illustration showing key findings of Nonspecific interstitial pneumonia : Exertional dyspnea, Dry cough, Bibasilar crackles
Copyright © 2024 VisualDx®. All rights reserved.