Original ArticlesInterstitial lung disease clinics for the management of idiopathic pulmonary fibrosis: a potential advantage to patients☆
Section snippets
Materials and methods
A general respiratory clinic is defined here as a clinic with a wide case mix of respiratory diseases (e.g., emphysema, asthma) in which patients with IPF are seen by a consultant, resident, or fellow. A clinical nurse specialist is not aligned to the clinic. There is direct access to cardiothoracic surgical opinion, and radiology and pathology opinions are given by specialists with a declared interest in respiratory diseases. The clinic is not aligned to a transplant program.
An ILD clinic is
Results
The study followed 148 patients (86 men, 62 women, mean age 61 years) with a confirmed diagnosis of IPF. Of these, 10 underwent transplantation and were excluded from final analysis, leaving 138 patients (79 men, 59 women, mean age 61 years). Twenty-four were diagnosed on the basis of typical clinical features (prior to HRCT), 54 were diagnosed on the basis of HRCT scanning; and 60 had a surgical lung biopsy, each of which had variegate lung fibrosis consistent with UIP. Eighty-four patients
Discussion
Current medical management has largely failed to demonstrate a significant advantage for patients with IPF. The result of this study indicates that a specific clinic for patients with IPF may offer a significantly improved outcome, particularly in patients younger than 60 years of age. Such a clinic may facilitate the collection of patients for the development of suitably powered randomized studies.
Age is a potential confounding factor when evaluating the results. The patients attending the
Acknowledgements
The Greater Manchester Lung Fibrosis Consortium: Dr. G. Archer, Stepping Hill Hospital, Stockport; Dr. T. Baker, Maelor Hospital, Wrexham; Dr. B. Bradley, Bolton General Hospital, Manchester; Dr. A. Burton, Royal Preston Hospital, Preston; Dr. K. B. Carroll, Wythenshawe Hospital, Manchester; Dr. M. Chopra, Tameside Hospital, Manchester; Dr. J. Earis, Fazakerley Hospital, Liverpool; Dr. J. J. Egan, Wythenshawe Hospital, Manchester; Dr. M. Finnegan, Bury General Hospital, Bury; Dr. Jim Finnerty,
References (26)
- et al.
Survival in patients with cryptogenic fibrosing alveolitisa population-based cohort study
Chest
(1998) - et al.
Advanced cryptogenic fibrosing alveolitispreliminary report on treatment with cyclosporin A
Respir Med
(1989) - et al.
Idiopathic pulmonary fibrosis and cyclosporinea lesson from single lung transplantation
Chest
(1998) - et al.
Clinical deterioration in patients with idiopathic pulmonary fibrosiscause and assessment
Am J Med
(1990) - et al.
The prognosis of cryptogenic fibrosing alveolitis
Thorax
(1972) - et al.
Randomised controlled trial comparing prednisolone alone with cyclophosphamide and low dose prednisolone in combination in cryptogenic fibrosing alveolitis
Thorax
(1989) - et al.
Azathioprine combined with prednisolone in the treatment of idiopathic pulmonary fibrosisa prospective double blind, randomised, placebo controlled clinical trial
Am Rev Respir Dis
(1991) - et al.
Cyclosporin as a treatment for interstitial lung disease of unknown aetiology
Thorax
(1991) - et al.
Improvement of the native lung following single lung transplant in patients with desquamative interstitial pneumonitis
Am J Respir Crit Care Med
(1997) - et al.
Hospital care for adults with cystic fibrosisan overview and comparison between special cystic fibrosis clinics and general clinics using a patient questionnaire
Thorax
(1994)
Optimizing the care of lung cancer patientsa multidisciplinary approach
Hematol Oncol Clin North Am
State of the artidiopathic pulmonary fibrosis: clinical relevance of pathologic classification
Am J Respir Crit Care Med
The predictive value of appearances on thin-section computed tomography in fibrosing alveolitis
Am Rev Respir Dis
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Implementing an interstitial lung disease clinic improves survival without increasing health care resource utilization
2019, Pulmonary Pharmacology and TherapeuticsCitation Excerpt :The lack of HRU increase observed in the SCC is complex to assess, but could partially attributed to: joint management and integrated care of connective tissue disease-related ILD with rheumatologists; reduction of clinic visits by focusing on home care; reduction of adverse events secondary to immunosuppressive treatment by increasing surveillance; and prevention of hospital admissions by optimizing outpatient medical care and initiating pulmonary rehabilitation [38]. Our outcome Results are consistent with those of Lok [39] and Lamas [4], who found that delayed access to a tertiary specialized referral centre is associated with worse survival. We expand these findings to subjects with non-IPF-ILD, who actually represents the majority of ILD patients.
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Supported by South Manchester Endowment Fund. Dr. S. Lok is supported by New Heart New Start. We would like to thank Dr. Brian Farragher, from the Department of Medical Statistics, for his advice.