Management of interstitial lung disease associated with connective tissue disease
TLDR
Despite similarities in clinical and pathologic presentation, the prognosis and treatment of CTD associated ILD (CTD-ILD) can differ greatly from that of other forms of ILD, such as idiopathic pulmonary fibrosis.Abstract:
The lung is a common site of complications of systemic connective tissue disease (CTD), and lung involvement can present in several ways. Interstitial lung disease (ILD) and pulmonary hypertension are the most common lung manifestations in CTD. Although it is generally thought that interstitial lung disease develops later on in CTD it is often the initial presentation (“lung dominant” CTD). ILD can be present in most types of CTD, including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, polymyositis or dermatomyositis, Sjogren’s syndrome, and mixed connective tissue disease. Despite similarities in clinical and pathologic presentation, the prognosis and treatment of CTD associated ILD (CTD-ILD) can differ greatly from that of other forms of ILD, such as idiopathic pulmonary fibrosis. Pulmonary hypertension (PH) can present as a primary vasculopathy in pulmonary arterial hypertension or in association with ILD (PH-ILD). Therefore, detailed history, physical examination, targeted serologic testing, and, occasionally, lung biopsy are needed to diagnose CTD-ILD, whereas both non-invasive and invasive assessments of pulmonary hemodynamics are needed to diagnose pulmonary hypertension. Immunosuppression is the mainstay of treatment for ILD, although data from randomized controlled trials (RCTs) to support specific treatments are lacking. Furthermore, treatment strategies vary according to the clinical situation—for example, the treatment of a patient newly diagnosed as having CTD-ILD differs from that of someone with an acute exacerbation of the disease. Immunosuppression is indicated only in select cases of pulmonary arterial hypertension related to CTD; more commonly, selective pulmonary vasodilators are used. For both diseases, comorbidities such as sleep disordered breathing, symptoms of dyspnea, and cough should be evaluated and treated. Lung transplantation should be considered in patients with advanced disease but is not always feasible because of other manifestations of CTD and comorbidities. Clinical trials of novel therapies including immunosuppressive therapies are needed to inform best treatment strategies.read more
Citations
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Journal ArticleDOI
Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases
Vincent Cottin,Nikhil Hirani,David L. Hotchkin,Anoop M. Nambiar,Takashi Ogura,Maria Otaola,Dirk Skowasch,Jong Sun Park,Hataya K. Poonyagariyagorn,Wim A. Wuyts,Athol U. Wells +10 more
TL;DR: Although these conditions are rare, a proportion of patients with interstitial lung diseases (ILDs) may develop a progressive-fibrosing phenotype, and identification of these patients is crucial, and requires a multidisciplinary approach, to ensure optimal diagnosis and management.
Journal ArticleDOI
Spectrum of Fibrotic Lung Diseases.
TL;DR: Pulmonary Fibrotic Diseases This review covers fibrotic pulmonary diseases, and although idiopathic pulmonary fibrosis is very common, the review focuses mainly on fibrotics diseases other than IPF.
Journal ArticleDOI
A population-based cohort study of rheumatoid arthritis-associated interstitial lung disease: comorbidity and mortality
Charlotte Hyldgaard,Ole Hilberg,Alma B Pedersen,Sinna Pilgaard Ulrichsen,Anders Løkke,Elisabeth Bendstrup,Torkell Ellingsen +6 more
TL;DR: ILD is a serious complication in RA, with a significantly increased mortality compared with a large matched cohort of RA comparisons without ILD, and the HRR for death was highest in the first months after the diagnosis of RA-ILD was made.
Journal ArticleDOI
The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype
TL;DR: ILDs with a progressive-fibrosing phenotype appear to be more common in older adults and are associated with a complex network of environmental and genetic factors, and where further data are needed in comparison to the well-studied IPF indication is noted.
Journal ArticleDOI
Riociguat for idiopathic interstitial pneumonia-associated pulmonary hypertension (RISE-IIP): a randomised, placebo-controlled phase 2b study.
Steven D. Nathan,Jürgen Behr,Harold R. Collard,Vincent Cottin,Marius M. Hoeper,Fernando J. Martinez,Tamera J. Corte,Tamera J. Corte,Anne Keogh,Hanno Leuchte,Nesrin Mogulkoc,Silvia Ulrich,Wim A. Wuyts,Zhen Yao,Francis Boateng,Athol U. Wells +15 more
TL;DR: Riociguat was associated with increased serious adverse events and mortality, and an unfavourable risk-benefit profile, and should not be used in patients with PH-IIP.
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