Vasculitis in systemic sclerosis: association with Sjögren's syndrome and the CREST syndrome variant
Oddis, C.V.; Eisenbeis, C.H.; Reidbord, H.E.; Steen, V.D.; Medsger, T.A.
Journal of Rheumatology 14(5): 942-948
1987
ISSN/ISBN: 0315-162X PMID: 3430523 Document Number: 289636
We describe 7 patients with established systemic sclerosis who developed clinical evidence of vasculitis 1 to 33 (mean 12.7) years after the first symptoms of scleroderma. Six had the CREST variant of systemic sclerosis and also had features of Sjogren's syndrome (SS). Five of 6 patients tested had serum anti-SSA (Ro) antibodies. Vasculitis presented primarily as cutaneous lesions with ulceration and/or mononeuritis multiplex, and 6 patients had severe systemic manifestations. Vasculitis was histopathologically documented in 6 cases in biopsies of skin (4 of 4), muscle (2 of 3) and sural nerve (3 of 3). Patients with systemic sclerosis with CREST syndrome and SS appear to be at increased risk to develop vasculitis.