Artikel
A late onset of systemic sclerosis correlates with a more rapidly progressing clinical phenotype in lcSSc patients – Data of the German Network for Systemic Sclerosis
Suche in Medline nach
Autoren
Veröffentlicht: | 8. Oktober 2019 |
---|
Gliederung
Text
Background: Systemic sclerosis (SSc) is a heterogeneous multisystem connective tissue disease. The majority of affected patients develop initial clinical symptoms between the age of 30-50 years. It is not yet known whether an aging population affects the clinical phenotype of SSc.
Investigating the relationship between the age at disease onset and clinical characteristics in SSc patients using the registry of the German Network for Systemic Scleroderma.
Methods: Clinical data of the patient registry including 2928 patients were evaluated. Three age groups at disease onset (<40 years, 40-60 years, and >60 years) were correlated with clinical characteristics.
Results: Among all SSc patients, 27% of patients developed non-raynaud symptoms initially at the age <40 years, 44% developed SSc between 40-60 years, and 29% were older than 60 years of age. In particular, SSc patients with disease onset >60 years developed significantly (p<0.001) more often the lcSSc subtype (71%) as well as anti-centromere antibodies (46%), had a significantly lower modified Rodnan Skin Score (mRSS) (mean mRSS, 7.8±8.1), less often digital ulcerations (20%), had more often pulmonary hypertension (PH) (17%) and a significantly lower mean diffusing capacity of the lung for carbon monoxide/single breath (DLCO/SB) level (70±22%). For lung fibrosis, heart or kidney involvement no significant difference between the three main age groups could be observed. The more progressive subsets (i.e., dcSSc and SSc overlap syndromes) occurred significantly more often at younger ages (p<0.001).
Conclusion: In this registry, approximately one-third of patients developed SSc at an age above 60 years. These are mostly of the limited cutaneous subtype with significantly more frequent occurrence of PH. Patients with the lcSSc subtype at a higher age (>60yrs) had a more rapid disease progression to PH. These findings may have an important impact on recommendations on diagnosis, frequency of follow-ups and therapy of SSc in different age groups.