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Background: Granulomatosis with polyangiitis (GPA) is a rare systemic autoimmune vasculitis disease that is highly correlated with antineutrophil cytoplasmic antibodies (ANCAs).It used to be called"Wegener's granulomatosis". The clinical manifestations are diverse, mainly involving the upper respiratory tract, lungs and kidneys, and this disease can involve the brain parenchyma as an isolated solid mass. Only one case has been reported thus far. To provide further information on this rare case, we report a case of granulomatous polyangiitis involving the fourth ventricle and review the relevant literature.Case characteristics: A 32-year-old Chinese female developed fever, cough, and shortness of breath for 20 days. An 8*8 cm skin ulcer was seen on the right lower limb. CT showed multiple large patches of increased density in both lungs. The patient’s serological anti-neutrophil cytoplasmic antibody cANCA was positive. Later, the patient developed dizziness and a headache. Magnetic resonance imaging of the head showed a mass of approximately 21x24 mm2 in the fourth ventricle. The patient had a craniotomy for mass resection, and macroscopically, the mass was gray–red and measured 2.5x2x2 cm and was soft, had local hemorrhage and necrosis, and had no capsule. The main microscopic features included necrotizing granulomatous vasculitis, the patient’s immunohistochemistry was positive for CD68 and negative for GFAP, and the acid-fast staining and hexaamine silver staining were negative. Combined with the clinical history, serology, and imaging, the pathological diagnosis was GPA in the fourth ventricle. The patient was switched to rituximab combined with steroid therapy because she did not tolerate cyclophosphamide. After 5 months of follow-up, the patient’s lung lesions and skin ulcers had completely improved, but the brain lesions had further progressed.Conclusion: When a patient has multiple system diseases, abnormal clinical manifestations, and positive serological ANCAs, a diagnosis of GPA should be carefully considered, and biopsies of easy-to-access sites should be performed. If the patient’s histopathological manifestations include necrotizing granulomatous vasculitis, a diagnosis of GPA is more likely. If a patient subsequently develops an intraventricular mass, the clinicians should consider a diagnosis of GPA, which can rarely involve the cerebral ventricle to avoid an unnecessary biopsy or surgical treatment of intracranial lesions. When a patient is intolerant to the traditional treatment drug cyclophosphamide and needs to be switched to rituximab, the treatment effect of intracerebral lesions is not ideal; therefore, the treatment of lesions involving GPA in the ventricle is worthy of further exploration. |
