Moinzadeh, Pia, Aberer, Elisabeth, Ahmadi-Simab, Keihan, Blank, Norbert, Distler, Joerg H. W., Fierlbeck, Gerhard, Genth, Ekkehard, Guenther, Claudia, Hein, Ruediger, Henes, Joerg, Herich, Lena, Herrgott, Ilka, Koetter, Ina ORCID: 0000-0002-9262-005X, Kreuter, Alexander, Krieg, Thomas, Kuhr, Kathrin, Lorenz, Hanns-Martin, Meier, Florian, Melchers, Inga, Mensing, Hartwig, Mueller-Ladner, Ulf, Pfeiffer, Christiane, Riemekasten, Gabriela, Sardy, Miklos ORCID: 0000-0003-4306-5093, Schmalzing, Marc, Sunderkoetter, Cord, Susok, Laura, Tarner, Ingo H., Vaith, Peter, Worm, Margitta ORCID: 0000-0002-3449-1245, Wozel, Gottfried, Zeidler, Gabriele and Hunzelmann, Nicolas (2015). Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis. Ann. Rheum. Dis., 74 (4). S. 730 - 738. LONDON: BMJ PUBLISHING GROUP. ISSN 1468-2060

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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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Moinzadeh, PiaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Aberer, ElisabethUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ahmadi-Simab, KeihanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Blank, NorbertUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Distler, Joerg H. W.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fierlbeck, GerhardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Genth, EkkehardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Guenther, ClaudiaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hein, RuedigerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Henes, JoergUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Herich, LenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Herrgott, IlkaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Koetter, InaUNSPECIFIEDorcid.org/0000-0002-9262-005XUNSPECIFIED
Kreuter, AlexanderUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Krieg, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kuhr, KathrinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lorenz, Hanns-MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Meier, FlorianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Melchers, IngaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mensing, HartwigUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mueller-Ladner, UlfUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pfeiffer, ChristianeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Riemekasten, GabrielaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sardy, MiklosUNSPECIFIEDorcid.org/0000-0003-4306-5093UNSPECIFIED
Schmalzing, MarcUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sunderkoetter, CordUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Susok, LauraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tarner, Ingo H.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vaith, PeterUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Worm, MargittaUNSPECIFIEDorcid.org/0000-0002-3449-1245UNSPECIFIED
Wozel, GottfriedUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zeidler, GabrieleUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hunzelmann, NicolasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-408832
DOI: 10.1136/annrheumdis-2013-204487
Journal or Publication Title: Ann. Rheum. Dis.
Volume: 74
Number: 4
Page Range: S. 730 - 738
Date: 2015
Publisher: BMJ PUBLISHING GROUP
Place of Publication: LONDON
ISSN: 1468-2060
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
CONNECTIVE-TISSUE DISEASE; RHEUMATOID-ARTHRITIS; CLINICAL-FEATURES; GERMAN NETWORK; PM-SCL; SCLERODERMA; AUTOANTIBODIES; CLASSIFICATION; FREQUENCY; ENTITYMultiple languages
RheumatologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/40883

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