Does the presence of connective tissue disease modify survival in patients with pulmonary fibrosis?

Navaratnam, Vidya; Ali, Noman; Smith, Chris; McKeever, Tricia M.; Fogarty, A.; Hubbard, Richard

doi:10.1016/j.rmed.2011.08.015

Cited by 57 publications

(42 citation statements)

References 22 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This is based on studies conducted in the community and collectively involving different CTDs [3,4], as well as other studies emanating from highly specialised centres and usually focused on one of the CTDs [19]. This observation holds true when considering individual CTDs, and is only partially attributable to the higher frequency of the NSIP pattern in this patient population [3].…”

Section: Does the Diagnosis Of Established Ctd Affect Prognosis Or Mamentioning

confidence: 99%

“…Although it is clear that patients with CTD-ILD have improved survival compared to those with IPF [3,4,7], studies have generally not demonstrated a survival advantage in patients with UCTD-ILD as compared to those with no CTD features [6]. In the study by VIJ et al [41], the 5-year overall survival of patients diagnosed with UCTD-ILD (52%) did not differ from that of patients with IPF (48%), and was significantly lower than that of subjects with CTD-ILD (95%, p,0.01).…”

Section: Ctd Features May Be Missed In the Diagnostic Approach Of Ildmentioning

confidence: 99%

“…The long-term prognosis of patients with CTD-ILD has recently been shown to be less severe than that of idiopathic pulmonary fibrosis (IPF) [3,4]. However, a subset of patients present with symptoms that belong to the spectrum of clinical manifestations of CTDs and/or biological autoimmune features, but do not fulfil diagnostic criteria for a given autoimmune disease.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Significance of connective tissue diseases features in pulmonary fibrosis

Cottin

2013

Eur Respir Rev

View full text Add to dashboard Cite

Interstitial lung disease (ILD) can occur in any of the connective tissue diseases (CTD) with varying frequency and severity, and an overall long-term prognosis that is less severe than that of idiopathic pulmonary fibrosis (IPF). Because ILD may be the presenting manifestation of CTD and/or the dominant manifestation of CTD, clinical extra-thoracic manifestations should be systematically considered in the diagnostic approach of ILD. When present, autoantibodies strongly contribute to the recognition and classification of the CTD. Patients with clinical extrathoracic manifestations of CTD and/or autoantibodies (especially with a high titer and/or the antibody is considered “highly specific” of an autoimmune condition), but who do not fit with established international CTD criteria may be called undifferentiated CTD or “lung-dominant CTD”. Although it remains to be determined which combination of symptoms and serologic tests best identify the subset of patients with clinically relevant CTD features, available evidence suggests that such patients may have distinct clinical and imaging presentation and may portend a distinct clinical course. However, autoantibodies alone when present in IPF patients do not seem to impact prognosis or management. Referral to a rheumatologist and multidisciplinary discussion may contribute to management of patients with undifferentiated CTD

show abstract

Section: Does the Diagnosis Of Established Ctd Affect Prognosis Or Mamentioning

confidence: 99%

Section: Ctd Features May Be Missed In the Diagnostic Approach Of Ildmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Significance of connective tissue diseases features in pulmonary fibrosis

Cottin

2013

Eur Respir Rev

View full text Add to dashboard Cite

show abstract

“…Mortality rates were compared between the groups using Cox regression, adjusting for age, sex and year of diagnosis. 324 individuals with CTD-ILD had better survival (median survival 6.5 yrs) than 2,209 individuals with pulmonary fibrosis and absence of CTD (median survival 3.1 yrs) (HR 0.76, 95% CI 0.62-0.92), although with significant mortality in both groups (124 versus 230 deaths per 1,000 personyrs, respectively) [51]. The median survival was 8.8 yrs in patients with systemic sclerosis-ILD, 6.6 yrs in rheumatoid arthritis-ILD and 5.6 yrs in other CTDs (p50.0217) [51].…”

Section: Ild Associated With Ctdmentioning

confidence: 93%

“…This concept has gained further support from a large study of data derived from The Health Improvement Network, a large primary care database in the UK, involving 446 general physicians from 2000 to 2009 [51]. Mortality rates were compared between the groups using Cox regression, adjusting for age, sex and year of diagnosis.…”

Section: Ild Associated With Ctdmentioning

confidence: 99%

Interstitial lung disease

Cottin

2013

Eur Respir Rev

View full text Add to dashboard Cite

This article reviews the most important articles published in interstitial lung disease, as reviewed during the Clinical Year in Review session at the 2012 annual European Respiratory Society Congress in Vienna, Austria.Since the recent international guidelines for the management of idiopathic pulmonary fibrosis (IPF), important new evidence is available. The anti-fibrotic drug pirfenidone has been recently approved in Europe. Other pharmacological agents, especially nintedanib, are still being tested. The so-called triple combination therapy, anticoagulation therapy and endothelin receptor antagonists, especially ambrisentan, are either harmful or ineffective in IPF and are not recommended as treatment. Although the clinical course of IPF is highly variable, novel tools have been developed for individual prediction of prognosis. Acute exacerbations of IPF are associated with increased mortality and may occur with higher frequency in IPF patients with associated pulmonary hypertension.Interstitial lung disease associated with connective tissue disease has been definitely established to have a better long-term survival than IPF. A subset of patients present with symptoms and/or biological autoimmune features, but do not fulfil diagnostic criteria for a given autoimmune disease; this condition is associated with a higher prevalence of nonspecific interstitial pneumonia pattern, female sex and younger age, although survival relevance is unclear.

show abstract

Patient Selection and Indications for Lung Transplantation

Garrity

2014

Textbook of Organ Transplantation

View full text Add to dashboard Cite

Does the presence of connective tissue disease modify survival in patients with pulmonary fibrosis?

Abstract: The prognosis of individuals with CTD-PF appears to be significantly better than those with IPF-CS, but remains an important cause of death in patients with connective tissue disease, and requires more effective treatment options.

Cited by 57 publications

References 22 publications

Significance of connective tissue diseases features in pulmonary fibrosis

Significance of connective tissue diseases features in pulmonary fibrosis

Interstitial lung disease

Patient Selection and Indications for Lung Transplantation

Contact Info

Product

Resources

About