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Ann Rheum Dis. 2015 Apr;74(4):730-7. doi: 10.1136/annrheumdis-2013-204487. Epub 2014 Jan 3.

Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis.

Author information

1
Department of Dermatology, Cologne University Hospital, Cologne, Germany.
2
Department of Dermatology, Medical University of Graz, Graz, Germany.
3
Department of Rheumatology, Asklepios Clinic Altona, Hamburg, Germany.
4
Department of Internal Medicine, Division of Rheumatology, University of Heidelberg, Heidelberg, Germany.
5
Department of Rheumatology, University of Erlangen, Erlangen, Germany.
6
Department of Dermatology, University of Tuebingen, Aachen, Germany.
7
Department of Rheumatology, Clinic of Rheumatology of Aachen, Aachen, Germany.
8
Department of Dermatology, University-Hospital Carl Gustav Carus, Dresden, Germany.
9
Department of Dermatology, Munich University of Technology, Munich, Germany.
10
Department of Rheumatology, University of Tuebingen, Tuebingen, Germany.
11
Institute of Medical Statistics, Informatics and Epidemiology, University of Cologne, Cologne, Germany.
12
Department of Dermatology and Venereology, University of Muenster, Muenster, Germany.
13
Department of Internal Medicine and Nephrology (Centre for interdisciplinary Rheumatology), Robert-Bosch-Hospital, Stuttgart, Germany.
14
Department of Dermatology, Helios Clinic Oberhausen, Oberhausen, Germany.
15
Department of Haemato-Oncology and Rheumatology, University of Heidelberg, Heidelberg, Germany.
16
Department of Rheumatology and Clinical Immunology, Kerckhoff Clinic, Bad Nauheim, Germany.
17
Clinical Research Unit for Rheumatology, University Medical Center Freiburg, Freiburg, Germany.
18
Clinic for Dermatology, Hamburg Alstertal, Hamburg, Germany.
19
Department of Dermatology, Ulm University Hospital, Ulm, Germany.
20
Department of Rheumatology and Clinical Immunology, University of Berlin, Charité, Germany.
21
Department of Dermatology and Allergology, Ludwig Maximilian University, Munich, Germany.
22
Department of Dermatology and Venereology, Ruhr University Bochum, Bochum, Germany.
23
Department of Rheumatology and Clinical Immunology, University Medical Center Freiburg, Freiburg, Germany.
24
Department of Dermatology and Venerology, University of Berlin, Charité, Berlin, Germany.
25
Department of Rheumatology, Johanniter-Hospital, Treuenbrietzen, Germany.

Abstract

BACKGROUND:

Systemic sclerosis (SSc)-overlap syndromes are a very heterogeneous and remarkable subgroup of SSc-patients, who present at least two connective tissue diseases (CTD) at the same time, usually with a specific autoantibody status.

OBJECTIVES:

To determine whether patients, classified as overlap syndromes, show a disease course different from patients with limited SSc (lcSSc) or diffuse cutaneous SSc (dcSSc).

METHODS:

The data of 3240 prospectively included patients, registered in the database of the German Network for Systemic Scleroderma and followed between 2003 and 2013, were analysed.

RESULTS:

Among 3240 registered patients, 10% were diagnosed as SSc-overlap syndrome. Of these, 82.5% were female. SSc-overlap patients had a mean age of 48±1.2 years and carried significantly more often 'other antibodies' (68.0%; p<0.0001), including anti-U1RNP, -PmScl, -Ro, -La, as well as anti-Jo-1 and -Ku antibodies. These patients developed musculoskeletal involvement earlier and more frequently (62.5%) than patients diagnosed as lcSSc (32.2%) or dcSSc (43.3%) (p<0.0001). The onset of lung fibrosis and heart involvement in SSc-overlap patients was significantly earlier than in patients with lcSSc and occurred later than in patients with dcSSc. Oesophagus, kidney and PH progression was similar to lcSSc patients, whereas dcSSc patients had a significantly earlier onset.

CONCLUSIONS:

These data support the concept that SSc-overlap syndromes should be regarded as a separate SSc subset, distinct from lcSSc and dcSSc, due to a different progression of the disease, different proportional distribution of specific autoantibodies, and of different organ involvement.

KEYWORDS:

Autoimmune Diseases; Epidemiology; Systemic Sclerosis

PMID:
24389298
PMCID:
PMC4392314
DOI:
10.1136/annrheumdis-2013-204487
[Indexed for MEDLINE]
Free PMC Article

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