New treatment paradigms for connective tissue disease-associated interstitial lung disease

Kouranos, Vasileios; Miranda, Gina; Corte, Tamera J.; Renzoni, Elisabetta A.

doi:10.1097/mcp.0000000000000508

Cited by 12 publications

(9 citation statements)

References 63 publications

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“…These therapies have been shown to improve parameters of pulmonary function that are related to prognosis, such as DLCO, DLCO/ FVC and TLC [45,50,51], but to date no study has actually demonstrated improved survival in patients treated with immunosuppressive agents. Hence, there is a need for new parameters that better predict long time survival under immunosuppression [52].…”

Section: Discussionmentioning

confidence: 99%

Prognostic value of cardiopulmonary exercise testing in patients with systemic sclerosis

et al. 2019

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Background: Systemic sclerosis (SSc) is a severe rheumatic disease of the interstitial tissue, in which heart and lung involvement can lead to disease-specific mortality. Our study tests the hypothesis that in addition to established prognostic factors, cardiopulmonary exercise testing (CPET) parameters, particularly peak oxygen uptake (peakVO 2) and ventilation/carbon dioxide (VE/VCO 2)-slope, can predict survival in patients with SSc. Subjects and methods: We retrospectively assessed 210 patients (80.9% female) in 6 centres over 10 years with pulmonary testing and CPET. Survival was analysed with Cox regression analysis (adjusted for age and gender) by age, comorbidity (Charlson-Index), body weight, body-mass index, extensive interstitial lung disease, pulmonary artery pressure (measured by echocardiography and invasively), and haemodynamic, pulmonary and CPET parameters. Results: Five-and ten-year survival of SSc patients was 93.8 and 86.9%, respectively. There was no difference in survival between patients with diffuse (dcSSc) and limited cutaneous manifestation (lcSSc; p = 0.3). Pulmonary and CPET parameters were significantly impaired. Prognosis was worst for patients with pulmonary hypertension (p = 0.007), 6-min walking distance < 413 m (p = 0.003), peakVO 2 < 15.6 mL•kg − 1 •min − 1 , and VE/VCO 2-slope > 35. Age (hazard ratio HR = 1.23; 95% confidence interval CI: 1.14;1.41), VE/VCO 2-slope (HR = 0.9; CI 0.82;0.98), diffusion capacity (Krogh factor, HR = 0.92; CI 0.86;0.98), forced vital capacity (FVC, HR = 0.91; CI 0.86;0.96), and peakVO 2 (HR = 0.87; CI 0.81;0.94) were significantly linked to survival in multivariate analyses (Harrell's C = 0.95). Summary: This is the first large study with SSc patients that demonstrates the prognostic value of peakVO 2 < 15.6 mL•kg − 1 •min − 1 (< 64.5% of predicted peakVO 2) and VE/VCO 2-slope > 35.

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Section: Discussionmentioning

confidence: 99%

Prognostic value of cardiopulmonary exercise testing in patients with systemic sclerosis

et al. 2019

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“…Once the diagnosis of SSc-ILD is established, it is important for the treating clinician to identify the constellation of factors that designate one patient to receive routine monitoring (as their risk for progressive ILD may be low), and for another patient to initiate or escalate immunomodulatory treatment (as risk for progressive ILD is high; Table 1) 18,19 .…”

Section: Screening and Monitoringmentioning

confidence: 99%

Management of systemic sclerosis-associated interstitial lung disease

Roofeh

Jaafar

Vummidi

et al. 2019

Current Opinion in Rheumatology

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Purpose of review-To review the recently published data and provide a practical overview for management of systemic sclerosis-interstitial lung disease (SSc-ILD). Recent Findings-Published evidence shows considerable practitioner variability in screening patients for ILD. Recent published data supports use of cyclophosphamide or mycophenolate mofetil as first line treatment of SSc-ILD. For patients not responding to first line therapies, consideration is given to rituximab as rescue therapy. Recent trials of hematopoietic autologous stem cell transplantation have demonstrated benefit in patients with progressive SSc-ILD. Antifibrotic agents are approved in idiopathic pulmonary fibrosis; studies with anti-fibrotics are underway for SSc-ILD. Summary-The specter of rapidly progressive lung disease requires clinicians to risk stratify patients according to known predictors for progression and rigorously monitor for symptoms and advancing disease. The above-mentioned therapies promise improved efficacy and favorable side effect profiles compared to cyclophosphamide.

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“…Interstitial lung disease (ILD) includes a heterogeneous group of parenchymal lung pathologies with different clinical, histological, radiological, and serological features (1). To correctly classify ILD is crucial, since follow-up, treatment, and prognosis are strongly dependent on ILD subtype (2,3). Considering that ILD may complicate the course of any connective tissue disease (CTD) and that signs of CTD are frequently not easy to identify (4-7), an underlying CTD should be ruled out in every ILD, even when the suspect is low or even absent.…”

Section: Introductionmentioning

confidence: 99%

Multidisciplinary Approach in the Early Detection of Undiagnosed Connective Tissue Diseases in Patients With Interstitial Lung Disease: A Retrospective Cohort Study

et al. 2020

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New treatment paradigms for connective tissue disease-associated interstitial lung disease

Abstract: Ultimately, further research into the mechanisms linking autoimmunity to fibrosis and randomized controlled clinical trials are needed, with the aim of preventing irreversible damage of lung tissue, while minimizing burden of treatment.

Cited by 12 publications

References 63 publications

Prognostic value of cardiopulmonary exercise testing in patients with systemic sclerosis

Prognostic value of cardiopulmonary exercise testing in patients with systemic sclerosis

Management of systemic sclerosis-associated interstitial lung disease

Multidisciplinary Approach in the Early Detection of Undiagnosed Connective Tissue Diseases in Patients With Interstitial Lung Disease: A Retrospective Cohort Study

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