Association of Short-Term Longitudinal Changes in Clinical and Physiologic Variables with Overall Survival in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD)

Vincent Sobanski¹, Jeska de Vries-Bouwstra², Anna-Maria Hoffmann-Vold³, Dörte Huscher⁴, Margarida Alves⁵, Marco Matucci-Cerinic⁶, Gabriela Riemekasten⁷, Mengtao Li⁸, Gabriele Valentini⁹, László Czirják¹⁰, Otylia Kowal-Bielecka¹¹, Yannick Allanore¹², Nils Schoof⁵ and Oliver Distler¹³, ¹Department of Internal Medicine and Clinical Immunology, University of Lille, Lille, France, ²Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands, ³Oslo University Hospital, Oslo, Norway, ⁴Charité – Universitaetsmedizin Berlin and Berlin Institute of Health, Berlin, Germany, ⁵Boehringer Ingelheim International GmbH, Ingelheim, Germany, ⁶Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, ⁷Rheumatology, University Hospital Schleswig-Holstein - Campus Lübeck, Lübeck, Germany, ⁸Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China, ⁹Department of Rheumatology, Second University of Naples, Napoli, Italy, ¹⁰Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary, ¹¹Department of Rheumatology and Internal Medicine, Medical University of Białystok, Białystok, Poland, ¹²Cochin Hospital, Paris Descartes University, Paris, France, Paris, France, ¹³Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: interstitial lung disease, longitudinal studies and systemic sclerosis

Session Information

Date: Monday, October 22, 2018

Title: Systemic Sclerosis and Related Disorders – Clinical Poster II

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Interstitial lung disease (ILD) is one of the leading causes of morbidity and mortality in patients with SSc. However, the impact of changes in pulmonary function on clinical outcomes in SSc-ILD patients is not well understood. We assessed the association of changes in clinical and physiologic variables with survival in SSc-ILD patients.

Methods: Adult patients from the EUSTAR (European Scleroderma Trial and Research) database enrolled since January 2009, fulfilling 1980 ACR or 2013 ACR/EULAR criteria for SSc, with signs of lung fibrosis on X-ray and/or HRCT and/or an available date of ILD diagnosis, with ≥1 follow-up visit within 12 months after the first visit with ILD diagnosis (index visit) were eligible for the study. 12-month absolute changes in lung function including predicted forced vital capacity (FVC%pred) and predicted diffusing capacity of carbon monoxide (DLco%pred), changes in the modified Rodnan skin score (mRSS) and occurrence of digital ulcer (DU) were assessed for associations with overall survival. 2 disease progression definitions were analysed for association with survival: (A) lung and skin progression defined as ‘absolute decline in >10%FVCpred or mRSS >25% & >5 points’ (FVC/mRSS) and (B) ‘absolute decline in ≥10%FVCpred or ≥5%FVCpred. & DLco ≥15%pred’ (FVC/DLco). Survival time was defined as the duration between the visit where change was determined and the last visit with survival status, censored after 5 years of observation. Kaplan–Meier (KM) survival was analysed by Breslow test, and Cox’s proportional hazards models (Cox-PH) adjusted for age, gender, pulmonary hypertension, smoking and treatment status.

Results: Of 7752 patients enrolled in the EUSTAR database between 2009 and April, 2018, 857 fulfilled the inclusion criteria. In the KM analysis, a deterioration of ≥10%FVCpred within 12 months since index date as well as both FVC/mRSS and FVC/DLco progression definitions were associated with significantly lower survival (Fig.1). No associations with survival were found for changes in DLco%pred, mRSS, or occurrence of DU. Cox-PH multivariate models confirmed lower survival in patients with FVC decline ≥10%pred (OR=3.1 [1.6;6.0]), with FVC/DLco decline (OR=2.7 [1.5;4.9]), or with FVC/mRSS deterioration (OR=1.9 [1.1;3.4]).

Conclusion: Our study showed that a decline in FVC of ≥10%pred within 12 months follow up is associated with lower survival in SSc-ILD patients. Also both disease progression definitions showed an association with reduced survival. Short term changes in clinical and physiological variables over 12 months should be considered for prognostic stratification of SSc-ILD patients in addition to baseline values.

Figure 1: KM survival analysis:(L-R) Change in FVC%pred; FVC/DLco; FVC/mRSS

Disclosure: V. Sobanski, None; J. de Vries-Bouwstra, None; A. M. Hoffmann-Vold, None; D. Huscher, Actelion, Böhringer-Ingelheim, 9; M. Alves, Boehringer Ingelheim, 3; M. Matucci-Cerinic, None; G. Riemekasten, None; M. Li, None; G. Valentini, None; L. Czirják, None; O. Kowal-Bielecka, Bayer, Roche, 5; Y. Allanore, Actelion, Boehringer, Roche, Sanofi, Inventiva, Medac, Bayer, BMS, Pfizer, 2, 5; N. Schoof, Boehringer Ingelheim, 3; O. Distler, Actelion, Bayer, Boehringer Ingelheim, Mitsubishi Tanabe Pharma, Roche, 2,Actelion, AnaMar, Bayer, Boehringer Ingelheim, ChemolmAb, espeRare foundation, Genentech/Roche, GSK, Inventiva, Italfarmaco, Lilly, medac, Medlmmune, Mitsubishi Tanabe Pharma, Novartis, Pfizer, Sanofi, Sinoxa, UCB, 5,Patent mir-29 for the treatment of systemic sclerosis licensed, 9.

To cite this abstract in AMA style:

Sobanski V, de Vries-Bouwstra J, Hoffmann-Vold AM, Huscher D, Alves M, Matucci-Cerinic M, Riemekasten G, Li M, Valentini G, Czirják L, Kowal-Bielecka O, Allanore Y, Schoof N, Distler O. Association of Short-Term Longitudinal Changes in Clinical and Physiologic Variables with Overall Survival in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD) [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/association-of-short-term-longitudinal-changes-in-clinical-and-physiologic-variables-with-overall-survival-in-patients-with-systemic-sclerosis-associated-interstitial-lung-disease-ssc-ild/. Accessed .

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