Universität zu Köln

Moinzadeh, Pia, Kuhr, Kathrin, Siegert, Elise, Mueller-Ladner, Ulf, Riemekasten, Gabriela, Guenther, Claudia, Koetter, Ina, Henes, Joerg, Blank, Norbert, Zeidler, Gabriele, Pfeiffer, Christiane, Juche, Aaron, Jandova, Ilona, Ehrchen, Jan, Schmalzing, Marc, Susok, Laura, Schmeiser, Tim, Sunderkoetter, Cord, Distler, Joerg H. W., Worm, Margitta, Kreuter, Alexander, Krieg, Thomas and Hunzelman, Nicolas (2020). Older age onset of systemic sclerosis - accelerated disease progression in all disease subsets. RHEUMATOLOGY, 59 (11). S. 3380 - 3390. OXFORD: OXFORD UNIV PRESS. ISSN 1462-0332

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Abstract

Objectives. Systemic sclerosis is a heterogeneous, multisystem disease. It can occur at any age, but most patients develop the disease between the age of 40 to 50 years. There is controversial evidence on whether/how the age at disease onset affects their clinical phenotype. We here investigate the relationship between age at disease onset and symptoms in a large cohort of SSc patients (IcSSc, dcSSc and SSc-overlap syndromes). Methods. Clinical data of the registry of the German Network for Systemic Scleroderma including 3281 patients were evaluated and subdivided into three age groups at disease onset (<40years, 40-60 years, >60 years). Results. Among all SSc patients, 24.5% developed their first non-Raynaud phenomenon symptoms at the age <40years, and 22.5% were older than 60 years of age. In particular, older patients at onset developed the IcSSc subset significantly more often. Furthermore, they had pulmonary hypertension more often, but digital ulcerations less often. Remarkably, the course of the disease was more rapidly progressing in the older cohort (>60 years), except for gastrointestinal and musculoskeletal involvement. No significant difference was found for the use of corticosteroids. However, significantly, fewer patients older than 60 years received immunosuppressive treatment. Conclusion. In this large registry, similar to 25% of patients developed SSc at an age above 60years with an increased frequency of IcSSc. In this age group, an onset of internal organ involvement was significantly accelerated across all three subsets. These findings suggest that, in the elderly cohort, more frequent follow-up examinations are required for an earlier detection of organ complications.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Moinzadeh, Pia UNSPECIFIED UNSPECIFIED UNSPECIFIED Kuhr, Kathrin UNSPECIFIED UNSPECIFIED UNSPECIFIED Siegert, Elise UNSPECIFIED UNSPECIFIED UNSPECIFIED Mueller-Ladner, Ulf UNSPECIFIED UNSPECIFIED UNSPECIFIED Riemekasten, Gabriela UNSPECIFIED UNSPECIFIED UNSPECIFIED Guenther, Claudia UNSPECIFIED UNSPECIFIED UNSPECIFIED Koetter, Ina UNSPECIFIED UNSPECIFIED UNSPECIFIED Henes, Joerg UNSPECIFIED UNSPECIFIED UNSPECIFIED Blank, Norbert UNSPECIFIED UNSPECIFIED UNSPECIFIED Zeidler, Gabriele UNSPECIFIED UNSPECIFIED UNSPECIFIED Pfeiffer, Christiane UNSPECIFIED UNSPECIFIED UNSPECIFIED Juche, Aaron UNSPECIFIED UNSPECIFIED UNSPECIFIED Jandova, Ilona UNSPECIFIED UNSPECIFIED UNSPECIFIED Ehrchen, Jan UNSPECIFIED UNSPECIFIED UNSPECIFIED Schmalzing, Marc UNSPECIFIED UNSPECIFIED UNSPECIFIED Susok, Laura UNSPECIFIED UNSPECIFIED UNSPECIFIED Schmeiser, Tim UNSPECIFIED UNSPECIFIED UNSPECIFIED Sunderkoetter, Cord UNSPECIFIED UNSPECIFIED UNSPECIFIED Distler, Joerg H. W. UNSPECIFIED UNSPECIFIED UNSPECIFIED Worm, Margitta UNSPECIFIED UNSPECIFIED UNSPECIFIED Kreuter, Alexander UNSPECIFIED UNSPECIFIED UNSPECIFIED Krieg, Thomas UNSPECIFIED UNSPECIFIED UNSPECIFIED Hunzelman, Nicolas UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-312607
DOI:	10.1093/rheumatology/keaa127
Journal or Publication Title:	RHEUMATOLOGY
Volume:	59
Number:	11
Page Range:	S. 3380 - 3390
Date:	2020
Publisher:	OXFORD UNIV PRESS
Place of Publication:	OXFORD
ISSN:	1462-0332
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language SCLERODERMA; CLASSIFICATION; MORTALITY; CRITERIA Multiple languages Rheumatology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/31260