The Challenge of Very Early Systemic Sclerosis: A Combination of Mild and Early Disease?

Blaja, Elisabeth; Jordan, Suzana; Mihai, Carmen-Marina; Dobrota, Rucsandra; Becker, Mike Oliver; Maurer, Britta; Matucci-Cerinic, Marco; Distler, Oliver (2021). The Challenge of Very Early Systemic Sclerosis: A Combination of Mild and Early Disease? Journal of rheumatology, 48(1), pp. 82-86. Journal of Rheumatology Pub. Co. 10.3899/jrheum.190976

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OBJECTIVE

To address the hypothesis that very early patients with systemic sclerosis (SSc) are a heterogeneous group with mild or early disease, we analyzed the extent of heterogeneity in clinical, epidemiological, and immunological characteristics of these patients.

METHODS

We performed an analysis of very early SSc patients from the Zurich cohort, who fulfilled neither the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism nor the 1980 ACR classification criteria, but had a clinical expert diagnosis of SSc with Raynaud phenomenon (RP) and additional features of SSc (puffy fingers, SSc-specific antibodies, SSc pattern on nailfold capillaroscopy, or any organ involvement characteristic for SSc). Disease duration was defined from first RP symptom.

RESULTS

One hundred and two patients fulfilled the inclusion criteria and were analyzed. Their clinical presentation was heterogeneous with the large majority presenting with RP, antinuclear antibodies, and nailfold capillaroscopy changes, but with varying presentations of other features such as SSc-specific antibodies and early signs of organ involvement. While 54.1% (52/96) of patients had a disease duration of < 5 years, as many as 29.1% (28/96) of patients had a disease duration of > 10 years, indicating long-standing mild disease. Patients with very early, potentially progressive disease did not differ from patients with long-standing mild disease in terms of their clinical features at first presentation.

CONCLUSION

This study showed that patients with very early SSc are a mixture with mild or early disease. This needs to be considered in clinical practice for risk stratification and for the study design of patients considered as early SSc.

Item Type:	Journal Article (Original Article)
Division/Institute:	04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Rheumatology and Immunology
UniBE Contributor:	Maurer, Britta
Subjects:	600 Technology > 610 Medicine & health
ISSN:	0315-162X
Publisher:	Journal of Rheumatology Pub. Co.
Language:	English
Submitter:	Brigitte Isenschmid
Date Deposited:	20 Dec 2021 12:12
Last Modified:	05 Dec 2022 15:55
Publisher DOI:	10.3899/jrheum.190976
PubMed ID:	32173655
Uncontrolled Keywords:	autoimmune diseases disease duration systemic sclerosis
URI:	https://boris.unibe.ch/id/eprint/161814