Older age onset of systemic sclerosis – accelerated disease progression in all disease subsets

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 (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.

Systemic sclerosis mortality trends in Spain from 1980 to 2019: age–period–cohort and joinpoint analysis

“…PYLL have been found to be higher in SSc than in other autoimmune disorders such as rheumatoid arthritis 23 . In our study, rates for women were higher than for men, in contrast to previous studies 17,24 …”

“…23 In our study, rates for women were higher than for men, in contrast to previous studies. 17,24 We hypothesize that the falling trend recorded in younger cohorts from 1990 might indicate a stabilization of the Spanish mortality rates of SSc. This is supported by previous studies, which have shown that, despite SSc mortality overtaking mortality of the general population, trends have been falling in the past decades.…”

“…In addition, SSc cases with late onset (at > 60 years) are associated with a shorter survival. 5,17 The cohort analysis showed progressive fall in the relative risk of mortality until 1920, followed by an increase until 1980, and it appears that this increase was mainly due to an increase in mortality in males but not females. This concurs with Ranque and Mouthon, 18 who found that that SSc incidence increased between the 1950s and 1980s, related to a greater awareness by physicians and more accurate diagnosis of the disease.…”

“…In line with previous SSc mortality studies, the age analysis showed higher mortality rates with increasing age. In addition, SSc cases with late onset (at > 60 years) are associated with a shorter survival 5,17 …”

Nationwide and international registries on scleroderma. Past, present, and future

“…It has also served to immunologically characterize German patients on a national level 21 . It has also deepened the knowledge and prognostic evaluation of patients with overlap syndrome 22 and late-onset SSc 23 and the excessive use of corticosteroids and immunosuppressants in patients with SSc 24 .…”