Acute Interstitial Pneumonia and Acute Exacerbations of Idiopathic Pulmonary Fibrosis

Swigris, Jeffrey J.; Brown, Kevin K.

doi:10.1055/s-2006-957337

Cited by 29 publications

(15 citation statements)

References 27 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…ALI/ARDS develops by different insults to the lung, and the mainstay of its treatment is provision of excellent supportive care and etiologic management of the underlying cause [46]. AIP is precisely an ARDS of 'unknown cause', and no specific clinical clues to differentiate between 'known and unknown cause' ARDS exist [49]. Criteria for the diagnosis of AIP are the same as in IPF exacerbations with the exception of the 'incubation' time (2 months instead of 4 weeks) and the prerequisite of normal chest roentgenogram.…”

Section: Etiologic and Pathogenetic Considerationsmentioning

confidence: 99%

“…Echocardiography may also be useful. When early undiagnosed IPF presents with fulminant respiratory insufficiency and ARDS [3,29], honeycombing with bibasilar and subpleural distribution on HRCT [1] can establish the diagnosis of IPF exacerbation and differentiate definitely from AIP [49]. In IPF exacerbations, HRCT reveals new bilateral ground-glass abnormalities or consolidations (or both) upon UIP pattern [6].…”

Section: Clinical and Laboratory Assessmentmentioning

confidence: 99%

See 1 more Smart Citation

Clinical review: Idiopathic pulmonary fibrosis acute exacerbations - unravelling Ariadne's thread

et al. 2010

View full text Add to dashboard Cite

Idiopathic pulmonary fibrosis (IPF) is a dreadful, chronic, and irreversibly progressive fibrosing disease leading to death in all patients affected, and IPF acute exacerbations constitute the most devastating complication during its clinical course. IPF exacerbations are subacute/acute, clinically significant deteriorations of unidentifiable cause that usually transform the slow and more or less steady disease decline to the unexpected appearance of acute lung injury/acute respiratory distress syndrome (ALI/ARDS) ending in death. The histological picture is that of diffuse alveolar damage (DAD), which is the tissue counterpart of ARDS, upon usual interstitial pneumonia, which is the tissue equivalent of IPF. ALI/ARDS and acute interstitial pneumonia share with IPF exacerbations the tissue damage pattern of DAD. 'Treatment' with high-dose corticosteroids with or without an immunosuppressant proved ineffective and represents the coup de grace for these patients. Provision of excellent supportive care and the search for and treatment of the 'underlying cause' remain the only options. IPF exacerbations require rapid decisions about when and whether to initiate mechanical support. Admission to an intensive care unit (ICU) is a particular clinical and ethical challenge because of the extremely poor outcome. Transplantation in the ICU setting often presents insurmountable difficulties.

show abstract

Section: Etiologic and Pathogenetic Considerationsmentioning

confidence: 99%

Section: Clinical and Laboratory Assessmentmentioning

confidence: 99%

Clinical review: Idiopathic pulmonary fibrosis acute exacerbations - unravelling Ariadne's thread

et al. 2010

View full text Add to dashboard Cite

show abstract

“…In the present study, we define rapidly progressive IP as interstitial lung disease with acute to subacute respiratory failure, with bilateral infiltrative shadows on chest high-resolution computed tomography, and no known alternative causes of ALI (such as pneumonia, sepsis, trauma, toxic inhalation, blood infusion, acute pancreatitis, thromboembolism, or heart failure). Rapidly progressive IP includes idiopathic acute IP and IP with clinically amyopathic dermatomyositis [7,8,9]. Acute IP is a rapidly progressive condition of unknown cause that occurs in a previously healthy individual and produces the histologic findings of DAD [10].…”

Section: Introductionmentioning

confidence: 99%

“…In this study, rapidly progressive IP also includes patients with AE-IP. Such patients are often resistant to intensive therapy, resulting in a fulminant and devastating course, with mortality rates ranging from 50 to 90% [7,14,17]. Therefore, novel effective therapies for these rapidly progressive and fatal lung diseases are needed.…”

Section: Introductionmentioning

confidence: 99%

Direct Hemoperfusion Using Immobilized Polymyxin B in Patients with Rapidly Progressive Interstitial Pneumonias: A Retrospective Study

et al. 2010

View full text Add to dashboard Cite

Background: Rapidly progressive interstitial pneumonia (IP), including acute exacerbation of IP, has a high mortality rate. Direct hemoperfusion with a polymyxin B-immobilized fiber column (PMX-DHP) was recently identified as an effective treatment for sepsis-associated acute respiratory distress syndrome. However, little is known about the effectiveness of PMX-DHP for rapidly progressive IP. Objectives: The present study investigates whether PMX-DHP is safe and effective against rapidly progressive IP. Methods: We retrospectively examined the effects of PMX-DHP in 33 consecutive patients with rapidly progressive IP who were resistant to steroid pulse therapy. Patients were hospitalized at Nagasaki University Hospital between 2006 and 2009. Results: Seventy-two hours after PMX-DHP, the arterial oxygen tension/inspiratory oxygen fraction ratio (median 127–153 mm Hg) had significantly improved. One week after PMX-DHP, the arterial oxygen tension/inspiratory oxygen fraction ratio (median 127–227 mm Hg), the alveolar-arterial difference of oxygen (median 371–177 mm Hg) and the number of positive criteria for systemic inflammatory response syndrome had significantly improved, despite the ineffectiveness of corticosteroid pulse therapy. The serum level of monocyte chemotactic protein 1 was significantly decreased immediately after PMX-DHP. Conclusions: PMX-DHP was safe and effective in improving oxygenation and systemic inflammatory response syndromein patients with rapidly progressive IP. The beneficialeffects of PMX-DHP may be at least partially due to the inhibition of monocyte activation.

show abstract

“…The fact that smokers with IPF have a worse outcome than nonsmokers [12] is difficult to reconcile with the idea that AE-IPF are more frequent in nonsmokers. In addition, there is a high prevalence of smoking in patients with rheumatoid arthritis, who may present with a combination of pulmonary fibrosis and emphysema [13] and have a worse outcome with AE of ILD than is seen in other connective tissue diseases [14,15,16]. …”

Section: Incidence - Etiology - Risk Factorsmentioning

confidence: 99%

Acute Exacerbations of Idiopathic Pulmonary Fibrosis

Αντωνίου

Wells²

2013

Respiration

View full text Add to dashboard Cite

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and ultimately fatal disease, with a highly variable course in individual patients. Episodes of rapid deterioration are not uncommon, often following a period of stability. In cases of uncertain etiology, with typical clinical and high-resolution computed tomography (HRCT) features, the term ‘acute exacerbation of IPF' (AE-IPF) has been coined to describe a combination of diffuse alveolar damage and preexisting usual interstitial pneumonia. In 2007, a consensus definition and diagnostic criteria were proposed. Although the presence of overt infection is currently an exclusion criterion, it appears likely that occult infection, reflux and thoracic surgical procedures are all trigger factors for AE-IPF. The development of new, usually bilateral infiltrates (ground-glass attenuation with variable admixed consolidation) is a defining HRCT feature. The outcome is poor with a short-term mortality in excess of 50% despite therapy. A number of pathophysiologic pathways are activated, with immunologic dysregulation, epithelial damage and circulating fibrocytes all believed to play a pathogenetic role. Acute exacerbations are less prevalent in other fibrotic lung diseases than in IPF and may have a better outcome, with the exception of acute exacerbations of rheumatoid lung. In AE-IPF, the exclusion of alternative causes of rapid deterioration, including heart failure and infection, is the main goal of investigation. Empirical high-dose corticosteroid steroid therapy is generally used in AE-IPF, without proven benefit.

show abstract

Acute Interstitial Pneumonia and Acute Exacerbations of Idiopathic Pulmonary Fibrosis

Cited by 29 publications

References 27 publications

Clinical review: Idiopathic pulmonary fibrosis acute exacerbations - unravelling Ariadne's thread

Clinical review: Idiopathic pulmonary fibrosis acute exacerbations - unravelling Ariadne's thread

Direct Hemoperfusion Using Immobilized Polymyxin B in Patients with Rapidly Progressive Interstitial Pneumonias: A Retrospective Study

Acute Exacerbations of Idiopathic Pulmonary Fibrosis

Contact Info

Product

Resources

About