Ver el código de «Idiopathic pulmonary fibrosis»

==Background==
*Normal lung parenchyma is interspersed with areas of decrease compliance
**Unlike [[ARDS]], in which lung injury more uniform
**Mechanical ventilation strategies learned from ARDS not completely transferrable
*Median survival time 3 yrs after diagnosis
*Prevalence about 10-20 cases per 100,000 people
*AE-IPF = Acute Exacerbation of IPF
*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 common
*May co-exist with [[pulmonary hypertension]] and [[heart failure]]
*Diagnostic criteria for acute exacerbation of IPF:
**Diagnosis 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 bilateral ground-glass abnormalities/consolidation on a background reticular/honeycomb pattern consistent with 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 bilateral ground-glass opacities
***~70% have consolidation

==Differential Diagnosis==
{{Pulmonary fibrosis differential}}

==Diagnosis==
*CBC, leukocytosis
*CRP elevated
*LDH elevated
*ABG with hypoxemia, hypercapnea
*ECG
*CXR with likely need for CT
*Echo to assess for pulm HTN, r/o CHF
*BAL in ICU to r/o infection

==Management==
*All treatments controversial and of questionable efficacy
*[[Methylprednisolone]] 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 1-2mg/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 controversial, with some contending mechanical ventilation adds insult to AE-IPF
*Non-invasive may be use as a temporizing measure
*Low TVs at 6 cc/kg
*Permissive hypercapnea may be necessary
*Maintaining minute ventilations
**High respiratory rates may be necessary
**Heavy sedation with possible paralysis with cisatricurium
*Deviations from ARDS treatment strategies
**Must restrict PEEP given to AE-IPF
**No place for [[recruitment maneuver]] or prone postioning
**Questionable benefit of [[APRV]] (BiVent) and high-frequency oscillation ventilation

==Disposition==
*ICU
*ECMO has been used as a bridge to lung transplant
*Transfer to transplant center if candidate
**Lung transplant is the only proven therapy to increase long term survival

==See Also==
*[[Pulmonary hypertension]]

==References==
<references/>

[[Category:Pulmonary]]