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interstitial pneumonia
Includes:
- acute interstitial pneumonia
- desquamative interstitial pneumonia
- lymphocytic interstitial pneumonia
- Sjogren's syndrome
- rheumatoid arthritis
- systemic lupus erythematosus
- HIV1 infection
- nonspecific interstitial pneumonia*
- scleroderma
- polymyositis/dermatomyositis
- undifferentiated connective tissue disease
* 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
- erythrocyte sedimentation rate
- rheumatoid factor
- antinuclear antibody
- also see rheumatoid arthritis & lupus erythematosus
- pulse oximetry may be normal
Special laboratory:
- 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]
Specific
acute interstitial pneumonia; Hamman-Rich syndrome; idiopathic alveolar fibrosis; rapidly progressive pulmonary fibrosis (IAF)
respiratory bronchiolitis associated interstitial lung disease; desquamative interstitial pneumonia (RBILD)
General
pneumonia (PNA)
interstitial lung disease; diffuse parenchymal lung disease (DPLS, ILD)
References
- 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: 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: 24032382