Sex differences in physiological progression of idiopathic pulmonary fibrosis

Han, MeiLan K.; Murray, Susan; Fell, Charlene D.; Flaherty, Kevin R.; Toews, Galen B.; Myers, Jeffrey L.; Colby, Thomas V.; Travis, William D.; Kazerooni, Ella A.; Gross, Barry H.; Martínez, Fernando J.

doi:10.1183/09031936.00165207

Cited by 90 publications

(50 citation statements)

References 29 publications

(30 reference statements)

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“…A study of 215 patients that examined gender differences in IPF found that female sex conferred a significant survival advantage over male sex (HR 0.63; CI 0.41e0.97) after adjusting for age, smoking history, DL CO , and maximum desaturation area [46].…”

Section: Male Sexmentioning

confidence: 99%

Natural history of idiopathic pulmonary fibrosis

Kim

Perlman

Tomic

2015

Respiratory Medicine

146

109

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Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. IPF has a poor prognosis, with a median survival time of 2-3 years from diagnosis, but varying from a few months to a decade. The natural history of IPF is highly variable and the course of disease in an individual patient is difficult to predict. Some patients with IPF experience rapid decline, others progress much more slowly, and some patients show periods of relative stability interspersed with acute deteriorations in respiratory function. Many clinical, radiographic, serologic, and histopathologic variables have been shown to predict mortality in IPF. However, the accuracy of these predictors varies due to the retrospective nature of some of the studies and variations in study design. The ability to identify clinical characteristics that predict disease progression and survival would be useful for counseling patients, treatment decision-making, and prompt consideration for lung transplantation. A number of indices for predicting mortality in patients with IPF are available, but they require further validation. As high-resolution computed tomography scans become more widely available and patients with IPF are diagnosed earlier, survival times following diagnosis will improve. Early referral to interstitial lung disease specialty centers is important for accurate diagnosis and may be associated with improved outcomes. The goal of this review is to examine the natural history of IPF, discuss predictors of mortality, and highlight the importance of prompt diagnosis and referral for patients with IPF.

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Section: Male Sexmentioning

confidence: 99%

Natural history of idiopathic pulmonary fibrosis

Kim

Perlman

Tomic

2015

Respiratory Medicine

146

109

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“…Two studies also assessed the association of 6MWD with hospitalisation and both of them (100%) found statistically significant associations [109,120]. In patients with ILD, four (50%) out of eight studies found statistically significant associations of 6MWD with mortality (table S24) [69,[123][124][125][126][127][128][129]. Six (66%) out of nine studies in patients with pulmonary hypertension found statistically significant associations of 6MWD with mortality (table S25) [43,[130][131][132][133][134][135][136][137].…”

Section: Relationship Of 6mwd To Clinical Outcomesmentioning

confidence: 99%

“…14 studies included patients with COPD [109][110][111][112][113][114][115][116][117][118][119][120][121][122], eight studies included patients with ILD [69,[123][124][125][126][127][128][129], nine studies included patients with PAH [43,[130][131][132][133][134][135][136][137] and four included studies patients with other chronic respiratory disease or on the list for transplantation [138][139][140][141]. Average 6MWD at baseline was between 300 and 450 m ( fig.…”

Section: Relationship Of 6mwd To Clinical Outcomesmentioning

confidence: 99%

An official systematic review of the European Respiratory Society/American Thoracic Society: measurement properties of field walking tests in chronic respiratory disease

Singh¹,

Puhan²,

et al. 2014

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This systematic review examined the measurement properties of the 6-min walk test (6MWT), incremental shuttle walk test (ISWT) and endurance shuttle walk test (ESWT) in adults with chronic respiratory disease.Studies that report the evaluation or use of the 6MWT, ISWT or ESWT were included. We searched electronic databases for studies published between January 2000 and September 2013.The 6-min walking distance (6MWD) is a reliable measure (intra-class correlation coefficients ranged from 0.82 to 0.99 in seven studies). There is a learning effect, with greater distance walked on the second test (pooled mean improvement of 26 m in 13 studies). Reliability was similar for ISWT and ESWT, with a learning effect also evident for ISWT (pooled mean improvement of 20 m in six studies). The 6MWD correlates more strongly with peak work capacity (r50.59-0.93) and physical activity (r50.40-0.85) than with respiratory function (r50.10-0.59). Methodological factors affecting 6MWD include track length, encouragement, supplemental oxygen and walking aids. Supplemental oxygen also affects ISWT and ESWT performance. Responsiveness was moderate to high for all tests, with greater responsiveness to interventions that included exercise training.The findings of this review demonstrate that the 6MWT, ISWT and ESWT are robust tests of functional exercise capacity in adults with chronic respiratory disease. OverviewThe aim of this systematic review was to examine the measurement properties for the 6-min walk test (6MWT), incremental shuttle walk test (ISWT) and endurance shuttle walk test (ESWT) in adults with chronic respiratory disease. A companion paper describes the standard operating procedures for the tests [1]. The key findings of this systematic review are as follows.1) The 6-min walking distance (6MWD) is a valid and reliable measure of exercise capacity for people with chronic lung disease. The 6MWD correlates more strongly with measures of peak work capacity and physical activity than with respiratory function or quality of life, which supports its conceptualisation as a test of functional exercise performance.2) The ISWT offers a different protocol to the 6MWT as it is incremental and externally paced. The ISWT is a valid and reliable measure of cardiopulmonary exercise capacity in chronic obstructive pulmonary disease (COPD), where there is a strong relationship between ISWT distance and peak oxygen uptake (V9O 2 peak) or work rate on a cardiopulmonary exercise test (CPET).3) A learning effect is observed for the 6MWT and the ISWT. The second test usually is the better compared with the first, but this is inconsistent.4) The ESWT is a test of endurance capacity. It is externally paced and is performed along the same course as the ISWT. Two tests do not appear to be necessary if the second test is conducted on the same day.5) Reference equations have been proposed for the 6MWD and the ISWT. Age, height and weight are included in most equations. The influence of race and ethnicity is unclear.6) The safety profile of ...

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“…In their study, walk distance was a better predictor of prognosis than FVC. Lama et al , in a smaller study containing 83 patients with IPF, found that desaturation below 88% at any time during a 6-min walk test was predictive of a poorer outcome [52] , an effect that Han et al have shown to be more pronounced in males than females [53] .…”

Section: Physiology Datamentioning

confidence: 97%

The diagnosis of idiopathic pulmonary fibrosis and its complications

Maher

2008

Expert Opinion on Medical Diagnostics

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Computed tomography combined with clinical data is sufficient for diagnosing IPF in approximately two-thirds of patients with the condition. For the remaining patients, histological assessment is important in achieving a precise diagnosis. Serial measurements of carbon monoxide diffusing capacity and forced vital capacity provide the best prognostic indicator in IPF. Potential biomarkers for diagnosing IPF include KL-6, MMP1 and MMP7. Brain naturetic peptide shows promise as a non-invasive screening tool for the diagnosis of IPF-associated pulmonary hypertension.

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Sex differences in physiological progression of idiopathic pulmonary fibrosis

Cited by 90 publications

References 29 publications

Natural history of idiopathic pulmonary fibrosis

Natural history of idiopathic pulmonary fibrosis

An official systematic review of the European Respiratory Society/American Thoracic Society: measurement properties of field walking tests in chronic respiratory disease

The diagnosis of idiopathic pulmonary fibrosis and its complications

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