Progression and mortality of interstitial lung disease in mixed connective tissue disease: a long-term observational nationwide cohort study

Reiseter, Silje; Gunnarsson, Ragnar; Aaløkken, Trond Mogens; Lund, May Brit; Mynarek, Georg; Corander, Jukka; Haydon, Joanna; Molberg, Øyvind

doi:10.1093/rheumatology/kex077

Cited by 85 publications

(71 citation statements)

References 29 publications

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“…In an analysis of 137 patients with RA-ILD at a single US center, a baseline FVC below the mean of the cohort (68.7% predicted) and a decline from baseline in FVC ≥ 10% predicted at any time over the follow-up period (median 4.8 years) were predictors of mortality (HRs 1.46 and 2.57, respectively) after controlling for age, sex, smoking, and HRCT pattern [41]. A greater extent of fibrosis on HRCT has also been associated with worse survival in patients with MCTD [3].…”

Section: Progression Of Fibrosing Ildsmentioning

confidence: 99%

“…Interstitial lung disease (ILD) is a common manifestation of systemic autoimmune diseases (also referred to as connective tissue diseases) including systemic sclerosis (SSc) [1], rheumatoid arthritis (RA) [2], mixed connective tissue disease (MCTD) [3], polymyositis/dermatomyositis [4], and Sjögren's syndrome [5]. The reported prevalence of ILD in patients with systemic autoimmune disease varies widely depending on the methodology used and the population studied.…”

Section: Introductionmentioning

confidence: 99%

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Progressive fibrosing interstitial lung disease associated with systemic autoimmune diseases

Fischer

Distler

2019

Clin Rheumatol

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Interstitial lung disease (ILD) is a common manifestation of systemic autoimmune diseases and a leading cause of death in these patients. A proportion of patients with autoimmune ILDs develop a progressive fibrosing form of ILD, characterized by increasing fibrosis on high-resolution computed tomography, worsening of lung function, and early mortality. Autoimmune disease-related ILDs have a variable clinical course and not all patients will require treatment, but all patients should be monitored for signs of progression. Apart from systemic sclerosis-associated ILD, the limited evidence to support the efficacy of immunosuppression as a treatment for ILDs is based mainly on small retrospective series and expert opinion. Non-clinical data suggest that there are commonalities in the mechanisms that drive progressive fibrosis in ILDs with an immunological trigger as in other forms of progressive fibrosing ILD. This suggests that nintedanib and pirfenidone, drugs known to slow disease progression in patients with idiopathic pulmonary fibrosis, may also slow the progression of ILD associated with systemic autoimmune diseases. In the SENSCIS® trial, nintedanib reduced the rate of ILD progression in patients with systemic sclerosis-associated ILD. The results of other large clinical trials will provide further insights into the role of anti-fibrotic therapies in the treatment of autoimmune disease-related ILDs.

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Section: Progression Of Fibrosing Ildsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Progressive fibrosing interstitial lung disease associated with systemic autoimmune diseases

Fischer

Distler

2019

Clin Rheumatol

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“…The involvement of the interstitium in the CTD‐ILD may affect the progression and the clinical outcome of the underlying rheumatological disorder . Recent publications had addressed the burden of CTD‐ILD on the average annual healthcare costs in systemic sclerosis and rheumatological arthritis .…”

Section: Connective Tissue Disease Associated With Ildmentioning

confidence: 99%

Contemporary Concise Review 2018: Interstitial lung disease

2019

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“…Most CTDs, irrespective of the cause, tend to affect the pulmonary system and cause interstitial lung disease. [20,21] In addition, many of them also suffer from co-morbid conditions such as cardiovascular disease (17% and 67% among survivors and deceased respectively) [22] and cardiac anomalies such as LV dysfunction (25.6%) [23], valvular regurgitation (aortic or mitral) (60%) along with changes to the LV structure. [24,25] These cardiovascular conditions in the long run could result in heart failure and further reduce exercise tolerance.…”

Section: Central Factors (Cardiovascular and Pulmonary)mentioning

confidence: 99%

Exercise-based evaluations and interventions for pulmonary hypertension with connective tissue disorders

Babu

Morris

Arena

et al. 2018

Expert Review of Respiratory Medicine

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Exercise intolerance is a common and often significant limitation in pulmonary arterial hypertension (PAH). This intolerance greatly affects the quality of life and function of the individual with PAH, irrespective of its etiology. In PAH associated with connective tissue disorders (PAH-CTD), exercise intolerance is further amplified by the presence of coexisting musculoskeletal manifestations of CTD. The evaluation of exercise capacity and prescription for exercise training therefore becomes a challenge to the clinician. Areas covered: This review highlights factors contributing to exercise intolerance in PAH-CTD, evaluation methods of exercise capacity and an overview on exercise training and a roadmap for future research. Expert commentary: Exercise intolerance is a complex interplay of cardiovascular, pulmonary, and musculoskeletal systems. Data from cardiopulmonary exercise tests have shown predictive abilities for both diagnosis and prognosis. In its absence, the 6-min walk test can be used to provide similar information thereby making the role of exercise testing an invaluable evaluation method in PAH-CTD. Exercise training data in PAH-CTD are still sparse, though, data from PAH studies suggest potential benefit. However, more research is required in this area of testing and training for greater understanding on exercise hemodynamic, phenotypes, and training benefits.

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Progression and mortality of interstitial lung disease in mixed connective tissue disease: a long-term observational nationwide cohort study

Cited by 85 publications

References 29 publications

Progressive fibrosing interstitial lung disease associated with systemic autoimmune diseases

Progressive fibrosing interstitial lung disease associated with systemic autoimmune diseases

Contemporary Concise Review 2018: Interstitial lung disease

Exercise-based evaluations and interventions for pulmonary hypertension with connective tissue disorders

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