Baseline BAL Neutrophilia Predicts Early Mortality in Idiopathic Pulmonary Fibrosis

Kinder, Brent W.; Brown, Kevin K.; Schwarz, Marvin I.; Ix, Joachim H.; Kervitsky, Alma; King, Talmadge E.

doi:10.1378/chest.07-1948

Cited by 247 publications

(205 citation statements)

References 25 publications

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“…Neutrophilic alveolitis is a feature of patients with IPF and the degree of neutrophilia in BALF has been related to mortality in one large series [5], so it is interesting to speculate that the neutrophilia drives matrix turnover via MMP-8, at least in some patients. Alternatively, MMP-8 has been implicated in BALF MMP pg .…”

Section: Mmp-8mentioning

confidence: 99%

“…In addition to myofibroblast focus formation and epithelial cell injury, there is variable evidence of inflammation, as evidenced by increased macrophage and neutrophil counts [5], intra-alveolar coagulopathy [6] and the formation of new blood vessels in the IPF lung [7]. Abnormal angiogenesis has furthermore been linked to the development of fibrotic disorders of the lung [7].…”

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confidence: 99%

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Metalloproteinases in idiopathic pulmonary fibrosis

2011

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In this article, we outline the current state of knowledge about the balance between collagen production and degradation in idiopathic pulmonary fibrosis (IPF). The dysregulated action of metalloproteinases implicated in IPF may play a central role in IPF pathogenesis. Inhibiting metalloproteinases in IPF may, therefore, have therapeutic potential, but our knowledge of their pathophysiological role is held back by limited animal models and the lack of specific inhibitors.

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Section: Mmp-8mentioning

confidence: 99%

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confidence: 99%

Metalloproteinases in idiopathic pulmonary fibrosis

2011

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“…In human IPF, the search for prognostic factors has been extensive as the course of the disease can vary greatly among patients, from rapidly to slowly progressive to a step‐like process 6. Several factors have been associated with poor survival in humans, including decreased walking distance in 6‐minute walk test (6MWT),8 increasing grade of interstitial fibrosis on thoracic radiographs,9 fibrosis score and traction bronchiectasis in thin‐section computed tomography,10 increased bronchoalveolar lavage (BAL) neutrophilia,11 and presence of pulmonary hypertension 12. In human IPF with a step‐like progression, periods of relative stability are interrupted by acute exacerbations (AEs), which are associated with high mortality and a histopathologic pattern of diffuse alveolar damage (DAD) 6.…”

mentioning

confidence: 99%

Long‐Term Outcome and Use of 6‐Minute Walk Test in West Highland White Terriers with Idiopathic Pulmonary Fibrosis

Lilja‐Maula

Laurila

Syrjä

et al. 2014

Veterinary Internal Medicne

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BackgroundIdiopathic pulmonary fibrosis (IPF) is an incurable interstitial lung disease occurring mainly in West Highland White Terriers (WHWTs). The effects of IPF on survival and on exercise tolerance in WHWTs are unknown.ObjectivesTo evaluate survival, prognostic factors, and exercise tolerance in WHWTs with IPF.AnimalsPrivately owned WHWTs; 15 with IPF and 11 healthy controls.MethodsProspective case‐control study conducted in 2007–2012. For survival, descriptive statistics and Kaplan–Meier (KM) survival curves with Cox proportional hazard ratios were performed. For the prognostic factor study, KM curves, Cox regression analysis, and logistic regression models were used. The 6‐minute walk test (6MWT) was used for measurement of exercise tolerance.ResultsThe median IPF‐specific survival of deceased WHWTs (7/15) with IPF was 32 (range 2–51) months from onset of clinical signs. The risk of death from birth in WHWTs with IPF in age‐adjusted Cox model was significantly higher (hazard ratio 4.6; 95% confidence interval 1.05–19.74, P = .04) than in control WHWTs. No significant prognostic factors were identified. In 6MWT, WHWTs with IPF walked a shorter distance, median 398 m (range 273–519 m), than healthy controls, median 492 m (420–568 m), P = .05, and the partial pressure of oxygen in arterial blood in diseased dogs had a moderate positive correlation with walking distance (Kendall′s tau‐b = 0.69, P = .06).Conclusion and Clinical Importance IPF had a negative impact on life expectancy, but individual survival varied considerably. 6MWT proved to be a well‐tolerated, noninvasive test to evaluate exercise tolerance.

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“…Data regarding the prognostic value of BAL neutrophilia are conflicting. It has been shown in IPF that there is an association, although weak, with increased early mortality after adjustment for disease severity, in contrast to what was observed in previous studies [111,112]. In pulmonary fibrosis associated with SSc, BAL neutrophilia is a marker of disease severity as it is linked to disease extension in HRCT and greater lung function impairment [113,114] but it showed no prognostic significance after adjustment for disease severity [113].…”

Section: The Role Of Biomarkersmentioning

confidence: 82%

Recent advances in the pathogenesis and clinical evaluation of pulmonary fibrosis

Margaritopoulos¹,

Romagnoli²,

Poletti³

et al. 2012

European Respiratory Review

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Interstitial lung diseases (ILDs) are a group of heterogeneous disorders, either idiopathic or associated with injurious or inflammatory causes, in which the major site of damage is the lung interstitium. For a long time, knowledge regarding pathogenesis was trivial and there were difficulties in diagnosing and subsequently treating these diseases.During the past decade, however, there has been an impressive development in the field of ILDs. Idiopathic pulmonary fibrosis, the most common and fatal form of ILD, was initially believed to be due to an inflammatory response to unknown lung injury, whereas nowadays it is believed to be the result of multiple injuries at different sites of the lung followed by aberrant repair.The integration of clinical, radiological and histological data, namely a multidisciplinary team (MDT) approach, has provided grounds for a more accurate diagnosis of ILDs, and helped the identification of different entities and development of different therapeutic approaches. However, because of the complexity of ILDs, even this approach may fail to establish a confident diagnosis.How should the clinician behave in this case and what are the pitfalls of the MDT approach? In addition, since diagnosis is the major predictor of prognosis, are there any other tools available to predict prognosis?

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Baseline BAL Neutrophilia Predicts Early Mortality in Idiopathic Pulmonary Fibrosis

Cited by 247 publications

References 25 publications

Metalloproteinases in idiopathic pulmonary fibrosis

Metalloproteinases in idiopathic pulmonary fibrosis

Long‐Term Outcome and Use of 6‐Minute Walk Test in West Highland White Terriers with Idiopathic Pulmonary Fibrosis

Recent advances in the pathogenesis and clinical evaluation of pulmonary fibrosis

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