MPO-ANCA vasculitis was a predominant form of AAV in Japan. Classification based on ANCA subtype would be clinically relevant in the prediction of organ involvement and relapse.
A 70-year-old man with systemic lupus erythematosus (SLE) presented with simultaneous right oculomotor nerve palsy and right facial nerve palsy. Brain magnetic resonance imaging and cerebrospinal fluid analysis revealed no abnormality. Coexistent Sjögren’s syndrome was diagnosed on the basis of anti-SS-A antibody positivity, salivary gland scintigraphy, and histological findings on minor salivary gland biopsy. As there was no obvious cause of multiple cranial neuropathies, we supposed that the palsies were induced by either of the underlying diseases. The patient was treated with a high-dose of prednisolone and intravenous cyclophosphamide, and both palsies recovered almost completely within two weeks.
A 40-year-old woman with a 13-year history of systemic lupus erythematosus (SLE) was admitted to our hospital because of intermittent abdominal pain, low-grade fever, and watery diarrhea. Physical examination revealed diminished bowel sounds and tenderness of the lower abdomen without rebound tenderness. Laboratory tests revealed a leukocyte count of 9,900/μL and C-reactive protein of 6.72 mg/dL. Complement components C3 and C4 were 59 and 6 mg/dL, respectively (normal: 86-160 and 17-45 mg/dL, respectively), and anti-double stranded DNA antibody was 80.1 IU/mL (normal <12). Contrast-enhanced computed tomography (CT) of the abdomen revealed marked thickening of the entire colonic mucosa, the so-called "doughnut sign" (Picture 1, 2). There was no hydronephrosis or thickened bladder wall. A diagnosis of lupus enteritis was made from these typical CT findings. The dose of prednisolone was increased from 10 mg/day to 40 mg/day (1 mg/kg), and her fever and abdominal symptoms were resolved within a few days.
A 59-year-old woman with a 10-year history of rheumatoid arthritis (RA) presented with chronic ulcers on both feet while undergoing treatment with etanercept. Rheumatoid vasculitis (RV) was diagnosed, and the patient was treated with immunosuppressant drugs and skin grafting. Although anti-tumor necrosis factor (TNF) agents are known to induce vasculitis, vasculitis can also be caused by active RA. Accordingly, the cause of vasculitis in RA patients receiving anti-TNF therapy must be evaluated carefully.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.