Objectives: Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are the leading causes of death in systemic sclerosis (SSc). Although the six-minute walk test (6MWT) is used for evaluating ILD and PAH, no data are available on the evolution of the six-minute walk distance (6MWD) in SSc patients without ILD and PAH and whether the baseline 6MWD could serve as individual reference value for the management of those who will develop PAH or ILD.
Methods: Prospectively collected data of the first 6MWT (at baseline or 6-month follow-up) and the 6MWTs at 18-, 30-, 42-, 54-, and 66-month visit of 165 consecutive SSc patients without ILD and PAH, included in the Ghent University SSc Cohort between May 2006 and December 2016 were analysed.
Results: 96-100% of the included patients performed a 6MWT during the follow-up visits. The mean 6MWD during the baseline 6MWT of 165 SSc patients without ILD and PAH (35% limited, 56% limited cutaneous, 9% diffuse cutaneous SSc) was 484.20+/-92.65m with no significant difference in the 6MWD at different follow-up visits as compared to baseline. In 46 SSc patients without ILD and PAH who performed a 6MWT at baseline and at 66-month visit, the 6MWD walked at 66-month visit correlated with the baseline 6MWD (r=0.564, p<0.001).
Conclusions: In SSc without ILD and PAH, the 6MWT is feasible and the 6MWD is clinically stable over a 66 months period. Hence, the individual 6MWD might be used as individual reference value in management of those who will develop PAH or ILD.
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J Clin Med
November 2024
Laboratory of Research in Respiratory Physiotherapy (LFIP), Department of Physiotherapy, Londrina State University (UEL), Londrina 86038-3500, Brazil.
Eur J Case Rep Intern Med
October 2024
Department of Dermatology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, New York, USA.
Unlabelled: A 43-year-old male with a history of intravenous drug use and alcohol consumption presented to the emergency department with three-month history of failure to thrive. The patient exhibited a constellation of constitutional symptoms including cough, weight loss, fatigue, decreased appetite, nausea and vomiting. The skin examination revealed multiple subcutaneous hyperpigmented, indurated plaques and nodules on the trunk and arms.
View Article and Find Full Text PDFIn Vivo
October 2024
Rheumatology/Immunology and Allergy, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, R.O.C.
Background/aim: Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) is a severe complication characterized by elevated pulmonary artery pressure, which can lead to right heart failure and death, if untreated. Standard treatments often fail to adequately manage symptoms, highlighting the need for novel therapeutic approaches. This study investigated the efficacy of molecular hydrogen (H) therapy in a patient with CTD-PAH.
View Article and Find Full Text PDFFront Cardiovasc Med
October 2024
Scleroderma Unit, Rheumatology Unit, Azienda Ospedaliero-Universitaria Policlinico di Modena, University of Modena and Reggio Emilia, Modena, Italy.
Introduction: Reduced TAPSE/sPAP ratio has recently emerged as a predictive parameter risk factor for PH, however its role in SSc has been poorly investigated. The aim of the study was to investigate the prognostic value of the TAPSE/sPAP ratio for the prediction of mortality and cardiovascular events in patients with SSc complicated by PH. A comparison between SSc patients with PAH (SSc-PAH) and those with PH and significant ILD (SSc-PH) was also carried out.
View Article and Find Full Text PDFRom J Intern Med
October 2024
2Department of Rheumatology, University of Medicine and Pharmacy of Craiova, 2-4 Petru Rareș Street, 200349 Craiova, Romania.
Background: Systemic sclerosis (SSc) is a complex connective tissue disease characterized by microangiopathy, immune dysregulation, and fibrosis. Early detection of microvascular abnormalities using nailfold videocapillaroscopy (NVC) is crucial in assessing disease progression and associated disease's involvement such as interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH).
Objective: This study aims to explore the relationships correlation between NVC patterns, clinical manifestations, and systemic complications in SSc.
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