Grégory Pugnet1,2, Zora Marjanovic3,4, Christophe Deligny3,4, Karine Boussardon3,4, Ilham Benzidia3,4, Mathieu Puyade3,4, Pauline Lansiaux3,4, Els Vandecasteele3,4, Vanessa Smith3,4, Dominique Farge1,2. 1. From the Service de Médecine Interne, CHU Toulouse Purpan; Faculté de Médecine de Toulouse, Toulouse; Hématologie clinique et thérapie cellulaire, and Unité de Médecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, UF 04, Centre de référence des maladies auto-immunes systémiques rares d'Ile-de-France, Filière (FAI2R), AP-HP, hôpital Saint-Antoine; Université Paris Diderot, Sorbonne Paris Cité, Paris, France; Service de médecine interne, Centre national de référence "Lupus, syndrome des antiphospholipides et autres maladies auto-immunes systémiques rares," Hôpital Pierre Zobda Quitman, CHU de Martinique, Fort de France, Martinique; Service de Médecine Interne et Maladies Infectieuses, CHU de Poitiers, Poitiers, France; Department of Cardiology, Department of Internal Medicine, and Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. dominique.farge-bancel@aphp.fr pugnet.g@chu-toulouse.fr. 2. G. Pugnet, MD, PhD, Service de Médecine Interne, CHU Toulouse Purpan, and Faculté de Médecine de Toulouse; Z. Marjanovic, MD, Hématologie clinique et thérapie cellulaire, AP-HP, hôpital Saint-Antoine; C. Deligny, MD, PhD, Service de médecine interne, Centre national de référence "Lupus, syndrome des antiphospholipides et autres maladies auto-immunes systémiques rares," Hôpital Pierre Zobda Quitman, CHU de Martinique; K. Boussardon, MSc, Physiotherapist, Unité de Médecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, UF 04, Centre de référence des maladies auto-immunes systémiques rares d'Ile-de-France, Filière (FAI2R), AP-HP, Hôpital Saint-Louis; I. Benzidia, MD, Unité de Médecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, UF 04, Centre de référence des maladies auto-immunes systémiques rares d'Ile-de-France, Filière (FAI2R), AP-HP, Hôpital Saint-Louis; M. Puyade, MD, Service de Médecine Interne et Maladies Infectieuses, CHU de Poitiers; P. Lansiaux, PhD, Unité de Médecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, UF 04, Centre de référence des maladies auto-immunes systémiques rares d'Ile-de-France, Filière (FAI2R), AP-HP, Hôpital Saint-Louis; E. Vandecasteele, MD, Department of Cardiology, Ghent University Hospital; V. Smith, MD, PhD, Department of Internal Medicine, Ghent University, and Department of Rheumatology, Ghent University Hospital; D. Farge, MD, PhD, Unité de Médecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, UF 04, Centre de référence des maladies auto-immunes systémiques rares d'Ile-de-France, Filière (FAI2R), AP-HP, Hôpital Saint-Louis, Université Paris Diderot, Sorbonne Paris Cité. dominique.farge-bancel@aphp.fr pugnet.g@chu-toulouse.fr. 3. From the Service de Médecine Interne, CHU Toulouse Purpan; Faculté de Médecine de Toulouse, Toulouse; Hématologie clinique et thérapie cellulaire, and Unité de Médecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, UF 04, Centre de référence des maladies auto-immunes systémiques rares d'Ile-de-France, Filière (FAI2R), AP-HP, hôpital Saint-Antoine; Université Paris Diderot, Sorbonne Paris Cité, Paris, France; Service de médecine interne, Centre national de référence "Lupus, syndrome des antiphospholipides et autres maladies auto-immunes systémiques rares," Hôpital Pierre Zobda Quitman, CHU de Martinique, Fort de France, Martinique; Service de Médecine Interne et Maladies Infectieuses, CHU de Poitiers, Poitiers, France; Department of Cardiology, Department of Internal Medicine, and Department of Rheumatology, Ghent University Hospital, Ghent, Belgium. 4. G. Pugnet, MD, PhD, Service de Médecine Interne, CHU Toulouse Purpan, and Faculté de Médecine de Toulouse; Z. Marjanovic, MD, Hématologie clinique et thérapie cellulaire, AP-HP, hôpital Saint-Antoine; C. Deligny, MD, PhD, Service de médecine interne, Centre national de référence "Lupus, syndrome des antiphospholipides et autres maladies auto-immunes systémiques rares," Hôpital Pierre Zobda Quitman, CHU de Martinique; K. Boussardon, MSc, Physiotherapist, Unité de Médecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, UF 04, Centre de référence des maladies auto-immunes systémiques rares d'Ile-de-France, Filière (FAI2R), AP-HP, Hôpital Saint-Louis; I. Benzidia, MD, Unité de Médecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, UF 04, Centre de référence des maladies auto-immunes systémiques rares d'Ile-de-France, Filière (FAI2R), AP-HP, Hôpital Saint-Louis; M. Puyade, MD, Service de Médecine Interne et Maladies Infectieuses, CHU de Poitiers; P. Lansiaux, PhD, Unité de Médecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, UF 04, Centre de référence des maladies auto-immunes systémiques rares d'Ile-de-France, Filière (FAI2R), AP-HP, Hôpital Saint-Louis; E. Vandecasteele, MD, Department of Cardiology, Ghent University Hospital; V. Smith, MD, PhD, Department of Internal Medicine, Ghent University, and Department of Rheumatology, Ghent University Hospital; D. Farge, MD, PhD, Unité de Médecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, UF 04, Centre de référence des maladies auto-immunes systémiques rares d'Ile-de-France, Filière (FAI2R), AP-HP, Hôpital Saint-Louis, Université Paris Diderot, Sorbonne Paris Cité.
Abstract
OBJECTIVE: To assess the reproducibility and the utility of the 6-minute walk test (6MWT) in systemic sclerosis (SSc). METHODS: All patients with SSc who underwent at least two 6MWT within a minimum 3-month interval plus simultaneous routine clinical, biological, and functional evaluations were consecutively enrolled in this observational study over 6 years. Following American Thoracic Society guidelines, each 6MWT was repeated twice to assess the 6-minute walk distance (6MWD) reproducibility, with the highest value being reported for subsequent analysis. RESULTS: Among 56 (38 female) included patients aged 46 ± SD 12.7 years, with 17 ± 10 modified Rodnan skin score (mRSS) and 1 ± 0.8 Scleroderma Health Assessment Questionnaire (SHAQ) at first referral, 277 6MWT evaluations (5 ± 3.9 6MWT per patient) were performed over 23 ± 22.5 months followup. Meanwhile, 8 deaths (87.5% SSc-related) occurred. The mean 6MWD absolute value was 457 ± 117 m with a 4 ± 2.2 mean Borg dyspnea score. The 6MWD intraclass correlation coefficient was 0.996 (95% CI 0.995-0.999, p < 0.0001). In multivariate linear regression analysis, these factors were independently associated with a lower 6MWD: sex (R2 = 0.47, p < 0.0001), mRSS (R2 = 0.47, p = 0.008), tendon friction rub (R2 = 0.47, p = 0.003), SHAQ (R2 = 0.47, p = 0.02), muscle disability score (R2 = 0.47, p = 0.03), DLCO% (R2 = 0.47, p = 0.0008), and left ventricular ejection fraction (R2 = 0.47, p = 0.006). The 6MWD at first referral was an independent predictor for the overall mortality (HR 0.99, 95% CI 0.988-0.999) and the SSc-related mortality (HR 0.99, 95% CI 0.988-0.999). CONCLUSION: We show strong reproducibility for the 6MWD and confirm the 6MWT utility to assess the overall prognosis of patients with SSc.
OBJECTIVE: To assess the reproducibility and the utility of the 6-minute walk test (6MWT) in systemic sclerosis (SSc). METHODS: All patients with SSc who underwent at least two 6MWT within a minimum 3-month interval plus simultaneous routine clinical, biological, and functional evaluations were consecutively enrolled in this observational study over 6 years. Following American Thoracic Society guidelines, each 6MWT was repeated twice to assess the 6-minute walk distance (6MWD) reproducibility, with the highest value being reported for subsequent analysis. RESULTS: Among 56 (38 female) included patients aged 46 ± SD 12.7 years, with 17 ± 10 modified Rodnan skin score (mRSS) and 1 ± 0.8 Scleroderma Health Assessment Questionnaire (SHAQ) at first referral, 277 6MWT evaluations (5 ± 3.9 6MWT per patient) were performed over 23 ± 22.5 months followup. Meanwhile, 8 deaths (87.5% SSc-related) occurred. The mean 6MWD absolute value was 457 ± 117 m with a 4 ± 2.2 mean Borg dyspnea score. The 6MWD intraclass correlation coefficient was 0.996 (95% CI 0.995-0.999, p < 0.0001). In multivariate linear regression analysis, these factors were independently associated with a lower 6MWD: sex (R2 = 0.47, p < 0.0001), mRSS (R2 = 0.47, p = 0.008), tendon friction rub (R2 = 0.47, p = 0.003), SHAQ (R2 = 0.47, p = 0.02), muscle disability score (R2 = 0.47, p = 0.03), DLCO% (R2 = 0.47, p = 0.0008), and left ventricular ejection fraction (R2 = 0.47, p = 0.006). The 6MWD at first referral was an independent predictor for the overall mortality (HR 0.99, 95% CI 0.988-0.999) and the SSc-related mortality (HR 0.99, 95% CI 0.988-0.999). CONCLUSION: We show strong reproducibility for the 6MWD and confirm the 6MWT utility to assess the overall prognosis of patients with SSc.
Entities:
Keywords:
REPRODUCIBILITY OF RESULTS; SCLERODERMA; SURVIVAL ANALYSIS; SYSTEMIC SCLEROSIS; WALK TEST
Authors: George Hung; Valentina Mercurio; Steven Hsu; Stephen C Mathai; Ami A Shah; Monica Mukherjee Journal: Curr Rheumatol Rep Date: 2019-12-07 Impact factor: 4.592
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