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

Moinzadeh P, Aberer E, Ahmadi-Simab K, Blank N, Distler J, Fierlbeck G, Genth E, Günther C, Hein R, Henes J, Herich L, Herrgott I, Koetter I, Kreuter A, Krieg T, Kuhr K, Lorenz HM, Meier F, Melchers I, Mensing H, Mueller-Ladner U, Pfeiffer C, Riemekasten G, Sardy M, Schmalzing M, Sunderkoetter C, Susok L, Tarner IH, Vaith P, Worm M, Wozel G, Zeidler G, Hunzelmann N (2014)

Publication Type: Journal article

Publication year: 2014

Journal

Book Volume: 74

Pages Range: 730-7

Journal Issue: 4

DOI: 10.1136/annrheumdis-2013-204487

Abstract

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

Authors with CRIS profile

Involved external institutions

Universitätsklinikum Köln Germany (DE) Asklepios Kliniken Germany (DE) Ruprecht-Karls-Universität Heidelberg Germany (DE) Eberhard Karls Universität Tübingen Germany (DE) Universitätsklinikum Carl Gustav Carus Dresden Germany (DE) Technische Universität München (TUM) Germany (DE) Universität zu Köln Germany (DE) Westfälische Wilhelms-Universität (WWU) Münster Germany (DE) Robert-Bosch-Krankenhaus Germany (DE) HELIOS Kliniken Germany (DE) Kerckhoff-Klinik Germany (DE) Universitätsklinikum Freiburg Germany (DE) Dermatologisches Ambulatorium Hamburg-Alstertal Germany (DE) Universitätsklinikum Ulm Germany (DE) Humboldt-Universität zu Berlin Germany (DE) Ludwig-Maximilians-Universität (LMU) Germany (DE) Ruhr-Universität Bochum (RUB) Germany (DE) Johanniter-Krankenhaus im Fläming Treuenbrietzen GmbH Germany (DE)

How to cite

APA:

Moinzadeh, P., Aberer, E., Ahmadi-Simab, K., Blank, N., Distler, J., Fierlbeck, G.,... Hunzelmann, N. (2014). Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis. Annals of the Rheumatic Diseases, 74(4), 730-7. https://dx.doi.org/10.1136/annrheumdis-2013-204487

MLA:

Moinzadeh, Pia, et al. "Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis." Annals of the Rheumatic Diseases 74.4 (2014): 730-7.

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