Older age onset of systemic sclerosis - accelerated disease progression in all disease subsets
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Older age onset of systemic sclerosis - accelerated disease progression in all disease subsets. / Moinzadeh, Pia; Kuhr, Kathrin; Siegert, Elise; Mueller-Ladner, Ulf; Riemekasten, Gabriela; Günther, Claudia; Kötter, Ina; Henes, Jörg; Blank, Norbert; Zeidler, Gabriele; Pfeiffer, Christiane; Juche, Aaron; Jandova, Ilona; Ehrchen, Jan; Schmalzing, Marc; Susok, Laura; Schmeiser, Tim; Sunderkoetter, Cord; Distler, Jörg H W; Worm, Margitta; Kreuter, Alexander; Krieg, Thomas; Hunzelmann, Nicolas; Registry of the German Network for Systemic Scleroderma.
In: RHEUMATOLOGY, Vol. 59, No. 11, 01.11.2020, p. 3380-3389.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Older age onset of systemic sclerosis - accelerated disease progression in all disease subsets
AU - Moinzadeh, Pia
AU - Kuhr, Kathrin
AU - Siegert, Elise
AU - Mueller-Ladner, Ulf
AU - Riemekasten, Gabriela
AU - Günther, Claudia
AU - Kötter, Ina
AU - Henes, Jörg
AU - Blank, Norbert
AU - Zeidler, Gabriele
AU - Pfeiffer, Christiane
AU - Juche, Aaron
AU - Jandova, Ilona
AU - Ehrchen, Jan
AU - Schmalzing, Marc
AU - Susok, Laura
AU - Schmeiser, Tim
AU - Sunderkoetter, Cord
AU - Distler, Jörg H W
AU - Worm, Margitta
AU - Kreuter, Alexander
AU - Krieg, Thomas
AU - Hunzelmann, Nicolas
AU - Registry of the German Network for Systemic Scleroderma
N1 - © The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology.
PY - 2020/11/1
Y1 - 2020/11/1
N2 - 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 (lcSSc, 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 (<40 years, 40-60 years, >60 years).RESULTS: Among all SSc patients, 24.5% developed their first non-Raynaud phenomenon symptoms at the age <40 years, and 22.5% were older than 60 years of age. In particular, older patients at onset developed the lcSSc 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, ∼25% of patients developed SSc at an age above 60 years with an increased frequency of lcSSc. 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.
AB - 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 (lcSSc, 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 (<40 years, 40-60 years, >60 years).RESULTS: Among all SSc patients, 24.5% developed their first non-Raynaud phenomenon symptoms at the age <40 years, and 22.5% were older than 60 years of age. In particular, older patients at onset developed the lcSSc 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, ∼25% of patients developed SSc at an age above 60 years with an increased frequency of lcSSc. 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.
KW - Adrenal Cortex Hormones/therapeutic use
KW - Adult
KW - Age of Onset
KW - Disease Progression
KW - Female
KW - Fingers
KW - Germany/epidemiology
KW - Humans
KW - Hypertension, Pulmonary/etiology
KW - Immunosuppressive Agents/therapeutic use
KW - Kaplan-Meier Estimate
KW - Male
KW - Middle Aged
KW - Phenotype
KW - Scleroderma, Systemic/drug therapy
KW - Skin Ulcer/etiology
KW - Symptom Assessment
U2 - 10.1093/rheumatology/keaa127
DO - 10.1093/rheumatology/keaa127
M3 - SCORING: Journal article
C2 - 32333004
VL - 59
SP - 3380
EP - 3389
JO - RHEUMATOLOGY
JF - RHEUMATOLOGY
SN - 1462-0324
IS - 11
ER -