Idiopathic pulmonary fibrosis: Difference between revisions
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==Clinical Features== | ==Clinical Features== | ||
*Diagnosis of exclusion | |||
*Presentations with rapid deterioration without obvious cause commen | |||
*May co-exist with pulmonary HTN and heart failure | |||
*Diagnostic criteria for acute exacerbation of IPF: | |||
**Dx of IPF | |||
**Unexplained worsening of dyspnea within 30 days | |||
**Hypoxemia deviated from baseline ABG | |||
**No evidence of pulmonary infection | |||
**Exclusion of alternative causes (i.e. VTE) | |||
**CT with b/l ground-glass abnormalities/consolidation on a background reticular/honeycomb pattern c/w interstitial pneumonia<ref>Akira M, Kozuka T, Yamamoto S, Sakatani M. Computed tomography findings in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2008;178:372-8.</ref> | |||
***100% have b/l ground-glass opacities | |||
***~70% have consolidation | |||
==Diagnostics== | |||
*CBC, leukocytosis | |||
*CRP elevated | |||
*LDH elevated | |||
*ABG with hypoxemia, hypercapnea | |||
*EKG | |||
*CXR with likely need for CT | |||
*Echo to assess for pulm HTN, r/o CHF | |||
*BAL in ICU to r/o infection | |||
==DDx== | ==DDx== | ||
Revision as of 21:22, 28 April 2016
Background
- Median survival time 3 yrs after diagnosis
- Prevalence about 10-20 cases per 100,000 people
- Acute exacerbations carry mortality up to 80%
- 60% die from idiopathic pulmonary fibrosis, others die from:
- VTE
- Cardiovascular (ACS, CHF)
- Infection
Clinical Features
- Diagnosis of exclusion
- Presentations with rapid deterioration without obvious cause commen
- May co-exist with pulmonary HTN and heart failure
- Diagnostic criteria for acute exacerbation of IPF:
- Dx of IPF
- Unexplained worsening of dyspnea within 30 days
- Hypoxemia deviated from baseline ABG
- No evidence of pulmonary infection
- Exclusion of alternative causes (i.e. VTE)
- CT with b/l ground-glass abnormalities/consolidation on a background reticular/honeycomb pattern c/w interstitial pneumonia[1]
- 100% have b/l ground-glass opacities
- ~70% have consolidation
Diagnostics
- CBC, leukocytosis
- CRP elevated
- LDH elevated
- ABG with hypoxemia, hypercapnea
- EKG
- CXR with likely need for CT
- Echo to assess for pulm HTN, r/o CHF
- BAL in ICU to r/o infection
DDx
Pulmonary Fibrosis
- Interstitial pneumonias (acute, lymphocytic)
- Lung malignancy
- Aspiration pneumonia or pneumonitis
- Bacterial, viral, or fungal pneumonia
- Cryptogenic organizing pneumonia
- Interstitial lung disease associated with collagen vascular disease
- Drug-induced pulmonary toxicity (amiodarone, bleomycin, amphotericin B, carbamazepine, etc.)
- Eosinophilic granuloma (Histiocytosis X)
- Radiation pneumonitis
- Sarcoidosis
- Pneumoconiosis (Workplace exposure)
- Asbestosis
- Berylliosis
- Chemical worker's lung
- Coal worker's pneumoconiosis
- Silicosis
Management
See Also
Sources
- Bhatti H et al. Approach to acute exacerbation of idiopathic pulmonary fibrosis. Ann Thorac Med. 2013. Vol 8, Issue 2. Pg 71-77.
- Collard HR et al. Acute Exacerbations of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2007 Oct 1; 176(7): 636–643.
- Ryerson CJ et al. Acute exacerbation of idiopathic pulmonary fibrosis: shifting the paradigm. Eur Respir J. 2015 Aug;46(2):512-20.
- Juarez MM et al. Acute exacerbation of idiopathic pulmonary fibrosis—a review of current and novel pharmacotherapies. J Thorac Dis. 2015 Mar; 7(3): 499–519.
- ↑ Akira M, Kozuka T, Yamamoto S, Sakatani M. Computed tomography findings in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2008;178:372-8.