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).
Leg ulceration in rheumatoid arthritis (RA) without systemic vasculitis is a difficult clinical problem and a common cause of morbidity. We have assessed venous function, arterial pressures and range of ankle movement in 23 RA patients with a leg ulcer and compared the results with those in the non-ulcerated contralateral limb and in 25 RA patients matched for age and duration of arthritis. We found evidence of venous insufficiency in RA ulcer patients compared to disease controls. Ankle movement was more restricted in the ulcerated limb compared to the non-ulcerated contralateral leg. There was no difference in large-vessel arterial function between groups. These findings have implications for therapy and rates of healing.
Background: Vasculitides are diseases of unknown origin in the majority of cases, but sometimes are the consequence of viral infections; for instance, hepatitis B virus (HBV)-related polyarteritis nodosa (PAN) or hepatitis C virus (HCV)-associated cryoglobulinaemia.
Objective: To investigate the role of hepatitis G or GB virus C (GBV-C) in various forms of medium- and small-vessel vasculitides.
Design: Retrospective analyses of sera.
Setting: Tertiary care hospital in Bobigny, France.
Patients: Fifty-six vasculitides: 19 HBV-PAN, 10 PAN without HBV infection, 11 microscopic polyangiitis (MPA), seven Churg-Strauss syndrome (CSS) and nine Wegener's granulomatosis (WG). Every sample was collected before treatment.
Measurements: GBV-C RNA was detected using a reverse transcription-polymerase chain reaction assay with primers derived from the conserved GBV-C helicase and NS5a regions.
Results: GBV-C was detected in five of the 56 samples (8.9%): four patients with HBV-related PAN and one with MPA; three of these patients (two with HBV-PAN, one with MPA) had been transfused and two HBV-PAN were i.v. drug addicts. GBV-C was not found in CSS or in WG.
Conclusion: GBV-C infection was observed only in patients who had been transfused or who were addicts. This virus is unlikely to have a primary role in vasculitides.