The British Journal of Rheumatology, Vol 37, 1287-1291, Copyright © 1998 by British Society for Rheumatology
J Font, M Ramos-Casals, R Cervera, X Bosch, E Mirapeix, M Garcia-Carrasco, RM Morla and M Ingelmo
OBJECTIVE: To evaluate the prevalence of cytoplasmic (c) and perinuclear
(p) antineutrophil cytoplasmic antibodies (ANCA) in patients with primary
Sjogren's syndrome (SS), and to correlate the presence of ANCA with
extraglandular and immunological manifestations related to SS. METHODS: In
a cross-sectional study, we included 82 consecutive patients (75 female and
seven male; mean age 61 yr; range 33-87 yr) attending our unit. All
patients fulfilled four or more of the diagnostic criteria for SS proposed
by the European Community Study Group in 1993. Extraglandular
manifestations such as arthralgia and/or arthritis, Raynaud's phenomenon,
autoimmune thyroiditis, peripheral neuropathy, renal involvement and
cutaneous vasculitis were also recorded. Serum samples were examined by
indirect immunofluorescence (IIF) and by ELISA using as substrates
myeloperoxidase (MPO) and proteinase 3 (PR3). RESULTS: ANCA were detected
in nine (11%) patients: seven had pANCA and two an atypical pattern. These
two atypical ANCA became cANCA when paraformaldehyde fixation was applied.
ELISA findings showed that two patients had antibodies against MPO, and no
patient had antibodies to PR3. The most common extraglandular
manifestations in the ANCA-positive patients were articular involvement in
six (66%) patients, peripheral neuropathy in five (55%), Raynaud's
phenomenon in four (44%) and cutaneous vasculitis in four (44%). Of the
four patients with cutaneous vasculitis and ANCA, two had a mononuclear
inflammatory vascular disease (MIVD) in the biopsy specimen. When compared
with patients without ANCA, those with these antibodies had a higher
prevalence of cutaneous vasculitis (44% vs 8%, P = 0.01), Raynaud's
phenomenon (44% vs 8%, P = 0.01) and peripheral neuropathy (55% vs 7%, P
< 0.001). CONCLUSION: ANCA positivity can be found in patients with
primary SS and its detection is associated with the presence of clinical
manifestations attributable to vascular involvement (cutaneous vasculitis,
peripheral neuropathy and Raynaud's phenomenon).
ORIGINAL PAPERS
Antineutrophil cytoplasmic antibodies in primary Sjogren's syndrome: prevalence and clinical significance
Department of Medicine, Institut d'Investigacions Biomediques August Pi i Sunyer, Hospital Clinic, School of Medicine, University of Barcelona, Catalonia, Spain.
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