1/3. Orbital inflammatory pseudotumor due to hypersensitivity vasculitis and mononeuritis multiplex in a patient with atypical, cANCA-positive Wegener's granulomatosis.OBJECTIVE: We report on a 60-year-old woman with a retro-orbital pseudotumor and polyneuropathy. The retro-orbital inflammation was histologically diagnosed as hypersensitivity vasculitis (HV). As cytoplasmatic antineutrophilic cytoplasmatic antibody (cANCA) and anti-proteinase-3 antibody were detected, the differential diagnosis also included atypical Wegener's granulomatosis. hypersensitivity vasculitis is defined as small-vessel vasculitis mediated by the deposition of immune complexes (arthus reaction) after exposure to various agents such as drugs, toxins, and infections. Since an inflammatory retro-orbital pseudotumor due to HV has not previously been reported, the following case is presented. methods AND MAIN OUTCOME MEASURES: magnetic resonance imaging (MRI) revealed retro-orbital infiltrate without granuloma. histology from an orbital biopsy confirmed HV. electromyography was used for the diagnosis of polyneuropathy. serum investigation indicated erythrocyte sedimentation rate (ESR) >100 mm/h, c-reactive protein (CRP) 223 mg/l, antinuclear antibodies 1:80, and cANCA 100 U/ml. RESULTS: The bilateral orbital pseudotumor, polyneuropathy, and serum levels of inflammation reactants (ESR and CRP) improved from therapy with corticosteroids (1 g of methylprednisolone initially) and azathioprine (150 mg/day). CONCLUSIONS: Because of cANCA and anti-proteinase-3 antibody positivity, this case can be viewed more as an atypical Wegener's granulomatosis than a systemic HV. The causal variety of inflammatory orbital pseudotumor, including HV and different therapeutic consequences, requires histological differentiation from usual orbital pseudotumors.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/3. Mononeuritis multiplex in diabetes mellitus: evidence for underlying immune pathogenesis.Four patients with type 2 diabetes mellitus developed mononeuritis multiplex subacutely. sural nerve biopsies showed multifocal axonal loss in all patients, with epineurial perivascular inflammation affecting small calibre vessels in three. Three patients improved with immunotherapy. These observations suggest that mononeuritis multiplex in diabetes may be caused by an immune mediated vasculopathy and that it is pathogenetically akin to the more common and better recognised diabetic amyotrophy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/3. Severe proximal myopathy and mononeuritis multiplex in rheumatoid arthritis: manifestations of rheumatoid vasculitis.Vascular injury is considered to be a key finding in the pathogenesis of rheumatoid arthritis (RA). Manifestations are varied depending on the vessel size and the organ system involved. Vasculitis leading to symptomatic inflammatory myositis is a rare complication of RA. We describe a 62-year-old man with seropositive erosive RA of 1-year duration, who presented with severe proximal weakness and mononeuritis multiplex. His joint disease was clinically mild at the time of presentation. creatine kinase was normal and the electromyogram did not suggest myopathy. However, muscle biopsy revealed extensive small vessel vasculitis and severe inflammatory myositis. This report emphasizes the importance of fully evaluating patients with RA who present with proximal myopathy. The myopathy in our patient was not related to active joint disease, disuse atrophy, or complication of therapy. rheumatoid vasculitis leading to myositis is a rare and not well-recognized complication of RA for which aggressive immunosuppressive therapy is warranted.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |