Incidences and Predictors of Organ Manifestations in the Early Course of Systemic Sclerosis: A Longitudinal Study

Veronika K. Jaeger¹, Elina G. Wirz^2,3, Yannick Allanore⁴, Gabriela Riemekasten⁵, Eric Hachulla⁶, Oliver Distler⁷, Paolo Airo⁸, Patricia E. Carreira⁹, Alexandra Balbir-Gurman¹⁰, Mohammed Tikly¹¹, Serena Vettori¹², Nemanja Damjanov¹³, Ulf Müller-Ladner¹⁴, Jorg HW. Distler¹⁵, Mangtao Li¹⁶, Ulrich A. Walker¹⁷ and EUSTAR collaborators, ¹Department of Rheumatology, University Hospital Basel, Basel, Switzerland, ²Department of Rheumatology, University Hospital Basel, Basel, Switzerland, Basel, Switzerland, ³Department of Dermatology, University Hospital Basel, Basel, Switzerland, ⁴Department of Rheumatology A, Paris Descartes University, Cochin Hospital, Paris, France, ⁵Clinic of Rheumatology, University of Luebeck, Lübeck, Germany, ⁶Service de Médecine Interne, Hôpital Huriez, Université de Lille, Lille, France, ⁷Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, ⁸UO Reumatologia ed Immunologia Clinica, Spedali Civili, Brescia, Italy, ⁹Servicio de Reumatologia, Hospital Universitario 12 de Octubre, Madrid, Spain, ¹⁰B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion - Institute of Technology, Haifa, Israel, ¹¹Division of Rheumatology, Chris Hani Baragwanath Academic Hospital, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa, ¹²Department of Internal and Experimental Medicine, Rheumatology Unit, Second University of Naples, Naples, Italy, ¹³Institute of Rheumatology, University of Belgrade Medical School, Belgrade, Serbia, ¹⁴Internal Medicine and Rheumatology, Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Bad Nauheim, Germany, ¹⁵Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany, ¹⁶Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China, ¹⁷Rheumatology, University Hospital, Basel, Basel, Switzerland

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Systemic sclerosis

Session Information

Date: Tuesday, November 10, 2015

Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud's - Clinical Aspects and Therapeutics II

Session Type: ACR Concurrent Abstract Session

Session Time: 2:30PM-4:00PM

Background/Purpose:

Systemic sclerosis (SSc) is a rare and clinically heterogeneous
autoimmune disorder characterised by fibrosis and microvascular obliteration of
the skin and internal organs, particularly the heart, lungs, kidneys and the
digestive tract. Organ involvement mostly manifests after a variable period of
the onset of Raynaud’s phenomenon (RP). Using data from the large, multinational
EUSTAR cohort, we aimed to map the incidence and predictors of pulmonary,
cardiac, gastrointestinal (GI) and renal involvement in the early course of
SSc.

Methods:

Patients from the EUSTAR cohort with early SSc, defined as those who
had a visit within the first year of RP onset were studied. Outcome measures
were analysed as a function of time after RP onset using Kaplan-Meier methods,
and Cox regression analysis was used to evaluate predictors of incident organ
manifestations.

Results:

Of the 695 SSc patients in the EUSTAR database who had a baseline
visit within one year after RP onset, the incident non-RP manifestations (in
order of frequency) were: skin sclerosis (75%), GI symptoms (71%), impaired diffusing
capacity for carbon monoxide of <80% predicted (65%), digital ulcers (34%),
cardiac involvement (32%), impaired forced vital capacity (FVC) of <80%
predicted (31%), increased resting systolic pulmonary artery pressure estimated
by echocardiography (PAPsys)>40mmHg (14%), and renal crisis (3%; Figure 1).

Diffuse skin involvement was a significant predictor of incident FVC
impairment (Figure 2; Table 1), older age and male sex were predictors of
incident PAPsys>40mmHg (Figure 2; Table 1), and anti-topoisomerase
positivity and older age were predictors of incident cardiac manifestations
(Table 1). Incidence rates were highest for diastolic dysfunction, followed by
conduction blocks and pericardial effusion. Male sex, anti-RNA-polymerase-III
positivity and older age were predictors of renal crisis (Table 1).

Conclusion:

In SSc patients
presenting early after RP onset, approximately half of all incident organ
manifestations occur within 2 years. These findings may have implications for the design of new
diagnostic and therapeutic strategies aimed to ‘widen’ the still very narrow
‘window of opportunity’. They may also enable
physicians to counsel and manage patients presenting early in the course of SSc
more accurately.

Disclosure: V. K. Jaeger, None; E. G. Wirz, None; Y. Allanore, None; G. Riemekasten, None; E. Hachulla, None; O. Distler, Consultancy relationships and/or has received research funding from Actelion, Pfizer, Ergonex, BMS, Sanofi-Aventis, United BioSource Corporation, Medac, Biovitrium, Boehringer Ingelheim, Bayer Pharma AG, Novartis, 4D Science and Active Biotech in the area, 2; P. Airo, None; P. E. Carreira, None; A. Balbir-Gurman, None; M. Tikly, None; S. Vettori, None; N. Damjanov, None; U. Müller-Ladner, None; J. H. Distler, None; M. Li, None; U. A. Walker, None.

To cite this abstract in AMA style:

Jaeger VK, Wirz EG, Allanore Y, Riemekasten G, Hachulla E, Distler O, Airo P, Carreira PE, Balbir-Gurman A, Tikly M, Vettori S, Damjanov N, Müller-Ladner U, Distler JH, Li M, Walker UA. Incidences and Predictors of Organ Manifestations in the Early Course of Systemic Sclerosis: A Longitudinal Study [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/incidences-and-predictors-of-organ-manifestations-in-the-early-course-of-systemic-sclerosis-a-longitudinal-study/. Accessed .

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