interstitial pneumonia
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Introduction
Includes:
- acute interstitial pneumonia
- desquamative interstitial pneumonia
- lymphocytic interstitial pneumonia
- nonspecific interstitial pneumonia*
* distinguishing features of lymphocytic interstitial pneumonia vs nonspecific interstitial pneumonia unknown
Etiology
- connective tissue disease
- > 50% of patients develop connective tissue disease within 22 months[1]
Epidemiology
- tend to affect a younger population[1]
Clinical manifestations
- cough
- dyspnea on exertion
- bilateral basilar crackles
- no clubbing
Laboratory
- followup testing for connective tissue disease
- pulse oximetry may be normal
Diagnostic procedures
- pulmonary function testing
- restrictive pattern with low DLCO
- bronchoscopy with lung biopsy*
* when CT findings classic for usual interstitial pneumonia, biopsy not needed for diagnosis[1]
Radiology
- high-resolution computed tomography (CT)
- increased interstitial markings
- bronchiectasis
- diffuse ground glass opacities
- bilateral lower lobe reticular changes[1]
More general terms
More specific terms
- acute interstitial pneumonia; Hamman-Rich syndrome; idiopathic alveolar fibrosis; rapidly progressive pulmonary fibrosis (IAF)
- respiratory bronchiolitis associated interstitial lung disease; desquamative interstitial pneumonia (RBILD)
References
- ↑ Jump up to: 1.0 1.1 1.2 1.3 1.4 Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18, 19. American College of Physicians, Philadelphia 2012, 2015, 2018, 2022.
- ↑ Romagnoli M, Nannini C, Piciucchi S et al Idiopathic nonspecific interstitial pneumonia: an interstitial lung disease associated with autoimmune disorders? Eur Respir J. 2011 Aug;38(2):384-91 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21273390
- ↑ Travis WD, Costabel U, Hansell DM et al An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013 Sep 15;188(6):733-4 PMID: https://www.ncbi.nlm.nih.gov/pubmed/24032382