Idiopathic pulmonary fibrosis: Difference between revisions
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==Management== | ==Management== | ||
*All treatments controversial and of questionable efficacy | |||
*Methylprednisolon 500-1000 mg qd for 3 days<ref>Rice AJ, Wells AU, Bouros D, Du Bois RM, Hansell DM, Polychronopoulos V, et al. Terminal diffuse alveolar damage in relation to interstitial pneumonias. An autopsy study. Am J Clin Pathol 2003;119:709-14.</ref><ref>Saydain G, Islam A, Afessa B, Ryu JH, Scott JP, Peters SG. Outcome of patients with idiopathic pulmonary fibrosis admitted to the intensive care unit. Am J Respir Crit Care Med 2002;166:839-42.</ref> | |||
*Heparin drip | |||
**Alveolar injury predisposes to prothrombotic state | |||
**Prevents further vascular injury | |||
*Cyclosporine A 1-2 mg/kg/day with steroids<ref>Meduri GU, Golden E, Freire AX, Taylor E, Zaman M, Carson SJ, et al. Methylprednisolone infusion in early severe ARDS: Results of a randomized controlled trial. Chest 2007;131:954-63.</ref> | |||
==Mechanical Ventilation Strategies== | |||
*Current strategies resemble ARDS treatments | |||
*Low TVs at 6 cc/kg | |||
==See Also== | ==See Also== | ||
Revision as of 21:30, 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
- All treatments controversial and of questionable efficacy
- Methylprednisolon 500-1000 mg qd for 3 days[2][3]
- Heparin drip
- Alveolar injury predisposes to prothrombotic state
- Prevents further vascular injury
- Cyclosporine A 1-2 mg/kg/day with steroids[4]
Mechanical Ventilation Strategies
- Current strategies resemble ARDS treatments
- Low TVs at 6 cc/kg
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.
- ↑ Rice AJ, Wells AU, Bouros D, Du Bois RM, Hansell DM, Polychronopoulos V, et al. Terminal diffuse alveolar damage in relation to interstitial pneumonias. An autopsy study. Am J Clin Pathol 2003;119:709-14.
- ↑ Saydain G, Islam A, Afessa B, Ryu JH, Scott JP, Peters SG. Outcome of patients with idiopathic pulmonary fibrosis admitted to the intensive care unit. Am J Respir Crit Care Med 2002;166:839-42.
- ↑ Meduri GU, Golden E, Freire AX, Taylor E, Zaman M, Carson SJ, et al. Methylprednisolone infusion in early severe ARDS: Results of a randomized controlled trial. Chest 2007;131:954-63.