Immunosuppressive "Routine"� Treatment of SSc Patients with Limited Cutaneous Involvement and Interstitial Lung Disease

Dörte Huscher¹, Sabine Adler², Elise Siegert³, Giuseppina Abignano⁴, Yannick Allanore⁵, Jerome Avouac⁶, Kerstin Becker⁷, Laszlo Czirjak^8,9, Francesco Del Galdo¹⁰, Christopher P. Denton¹¹, Oliver Distler¹², Ivan Foeldvari¹³, Beata Garay-Toth¹⁴, Serena Guiducci¹⁵, Veronika K. Jaeger¹⁶, Veronika Lóránd⁹, Marco Matucci-Cerinic¹⁷, Britta Maurer¹⁸, Ulf Müller-Ladner¹⁹, Svetlana I. Nihtyanova²⁰, Ingo H. Tarner²¹, Gabriele Valentini²², Serena Vettori²³, Ulrich A. Walker²⁴ and Gabriela Riemekasten²⁵, ¹Charité-University Hospital and German Rheumatism Research Centre, Berlin, Germany, ²Rheumatology, Immunology, Allergology, University Hospital Bern, Bern, Switzerland, ³Rheumatology and Clinical Immunology, Charité – University Hospital, Berlin, Berlin, Germany, ⁴Clinical and Experimental Medicine, Second University of Naples, Napoli, Italy, ⁵Department of Rheumatology A, Paris Descartes University, Cochin Hospital, Paris, France, ⁶Paris Descartes University, Rheumatology A department, Cochin Hospital, Paris, France, ⁷Rheumatology and Clinical Immunology, Charité University Hospital, Berlin, Germany, ⁸Rheumatology, Pecs, Hungary, ⁹Rheumatology and Immunology Medical School, University of Pécs, Pécs, Hungary, ¹⁰Scleroderma Group, Leeds Institute of Molecular Medicine, Leeds, United Kingdom, ¹¹Rheumatology and Connective Tissue Diseases, University College London, London, United Kingdom, ¹²Department of Rheumatology, Research of Systemic Autoimmune Diseases, University Hospital Zurich, Zurich, Switzerland, ¹³Hamburger Zentrum für Kinder-und Jugendrheumatologie, Hamburg, Germany, ¹⁴FESCA, Budapest, Hungary, ¹⁵Rheumatology, University of Florence, Florence, Italy, ¹⁶Department of Rheumatology, University Hospital Basel, Basel, Switzerland, ¹⁷Dept of Medicine/Div of Rheum, University of Florence, Firenze, Italy, ¹⁸r, University Hospital Zurich, Zurich, Switzerland, ¹⁹Internal Medicine and Rheumatology, Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Bad Nauheim, Germany, ²⁰Rheumatology, UCL Division of Medicine, London, United Kingdom, ²¹Rheumatology and Clinical Immunology, Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Bad Nauheim, Germany, ²²Internal and Experimental Medicine, Rheumatology Unit, Second University of Naples, Naples, Italy, ²³Department of Internal and Experimental Medicine, Rheumatology Unit, Second University of Naples, Naples, Italy, ²⁴Rheumatology, Systemic Sclerosis, Basel, Switzerland, ²⁵Clinic of Rheumatology, University of Luebeck, Lübeck, Germany

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: observation, systemic sclerosis and treatment

Session Information

Date: Monday, November 9, 2015

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

Session Type: ACR Poster Session B

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

Background/Purpose:

Interstitial
lung disease (ILD) represents one of the most frequent causes of death in systemic
sclerosis (SSc) patients. Yet, there are no approved drugs for treatment of SSc-ILD.
At present, data on immunosuppressive therapy in patients with ILD and limited
cutaneous (lc) involvement are missing.

Methods:

The European
Scleroderma Trials and Research (EUSTAR) database was established in 2004 to annually
collect data on specialized care of patients with SSc. Data from patients with lc
SSc and ILD, where immunosuppressive therapy can be assumed to be mainly
intended to treat ILD, were analyzed to describe current use of immunosuppressants
and to recognize patterns of treatment choices.

Results:

Out of a
cohort of 12,536 SSc patients, we identified 1,814 adult patients fulfilling
the 1980 ACR or the 2013 ACR-EULAR criteria, with lc involvement and signs of ILD
(either on chest X-ray or high resolution computed tomography) with at least
one valid report on immunosuppressive treatment.

Mean age
was 58.7±13.1
years, with 88.9%
females and a mean SSc disease duration of 10.2±8.6 years. Mean mRSS was 6.1±4.7.

Compared to
the 1,119 (62%) patients who had at least one episode of immunosuppressive
therapy the 695 (38%) patients without use of immunosuppressants ever were on
average 5 years older, had a 2.4 years longer median disease duration, higher
DLCO (single breath) and FVC values and consequently less often a pulmonary
restrictive defect.

The
immunosuppressants most frequently used were prednisolone (pred; 52.6%),
azathioprine (aza; 13.9%), cyclophosphamide (cyc; 13.8%), methotrexate (mtx; 11.7%),
and mycophenolate mofetil (mmf; 11.0%); all others were prescribed in less than
2%. With regard to highest treatment intensity ever received, 31.6% of the patients
got monotherapies and 26.3% combinations of two drugs, while triple therapy was
comparably rare with 3.6%. Overall, immunosuppressive treatment was less
frequent than in diffuse cutaneous type (82%), this was true for mmf or cyc
monotherapy and any combination therapies.

When comparing
patient characteristics at the treatment start of the most frequent regimens, differences
compared to the “never IS” group and between treatment arms become apparent
with regard to age, NYHA class, Scl70 antibodies, mRSS, ground glass
opacification, lung function parameters and the proportion with pulmonary
restrictive defect (table 1).

Conclusion:

“Everyday”
use of immunosuppressants is frequent in SSc-ILD patients with lc involvement,
showing a wide variety of single and combined immunosuppressive therapies with
distinct patient patterns between treatment regimens. However, prospective
studies are still necessary to define indications and outcomes. The EU-funded
international FP7 DeSScipher research project was initiated to achieve this
goal, comprising 5 prospective observational trials addressing the most
frequent medical problems in SSc patients.

Disclosure: D. Huscher, None; S. Adler, None; E. Siegert, None; G. Abignano, None; Y. Allanore, None; J. Avouac, None; K. Becker, None; L. Czirjak, None; F. Del Galdo, None; C. P. Denton, GlaxoSmithKline, 2,Actelion Pharmaceuticals US, 5,GlaxoSmithKline, 5,Serono, 5,Inventiva, 5,CSL Behring, 2,Bayer, 5; 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; I. Foeldvari, None; B. Garay-Toth, None; S. Guiducci, None; V. K. Jaeger, None; V. Lóránd, None; M. Matucci-Cerinic, None; B. Maurer, None; U. Müller-Ladner, None; S. I. Nihtyanova, None; I. H. Tarner, None; G. Valentini, None; S. Vettori, None; U. A. Walker, None; G. Riemekasten, None.

To cite this abstract in AMA style:

Huscher D, Adler S, Siegert E, Abignano G, Allanore Y, Avouac J, Becker K, Czirjak L, Del Galdo F, Denton CP, Distler O, Foeldvari I, Garay-Toth B, Guiducci S, Jaeger VK, Lóránd V, Matucci-Cerinic M, Maurer B, Müller-Ladner U, Nihtyanova SI, Tarner IH, Valentini G, Vettori S, Walker UA, Riemekasten G. Immunosuppressive “Routine”� Treatment of SSc Patients with Limited Cutaneous Involvement and Interstitial Lung Disease [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/immunosuppressive-routine%ef%bf%bd-treatment-of-ssc-patients-with-limited-cutaneous-involvement-and-interstitial-lung-disease/. Accessed .

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