Granulomatosis with polyangiitis with intestinal involvement successfully treated with rituximab and surgery.

Intestinal perforation is a rare and life-threatening complication in granulomatosis with polyangiitis (GPA). A 55-year-old Japanese man who had been undergoing maintenance haemodialysis was diagnosed with GPA via a pathologically proven nasal granuloma and proteinase 3-antineutrophil cytoplasmic antibody-positive status. He was started on 60 mg prednisolone. Haematochezia was observed due to the colon ulcer after treatment initiation. Two doses of intravenous cyclophosphamide were administered every 2 weeks. Despite the treatment, there were two episodes of severe abdominal pain with peritonitis within a week. Perforation of descending colon and severe ischaemia of the ascending colon was observed during each emergency laparotomy, and ileostomy was performed. Vasculitis in the small vessels was confirmed. In conclusion, patient with severe intestinal involvement and who was unresponsive to conventional therapy was treated with surgery followed by rituximab administration, and remission was achieved.

Subarachnoid small vein occlusion due to inflammatory fibrosis-a possible mechanism for cerebellar infarction in cryptococcal meningoencephalitis: a case report.

BACKGROUND: Cryptococcal meningoencephalitis (CM) causes cerebral infarction, typically, lacunar infarction in the basal ganglia. However, massive cerebral infarction leading to death is rare and its pathophysiology is unclear. We report a case of CM causing massive cerebellar infarction, which led to cerebral herniation and death. CASE PRESENTATION: A 56-year-old man who suffered from dizziness and gait disturbance for one month was admitted to our hospital and subsequently diagnosed with a cerebellar infarction. He had a past medical history of hepatitis type B virus infection and hepatic failure. Although the findings on magnetic resonance imaging (MRI) imitated an arterial infarction of the posterior inferior cerebellar artery, an accompanying irregular peripheral edema was observed. The ischemic lesion progressed, subsequently exerting a mass effect and leading to impaired consciousness. External and internal decompression surgeries were performed. Cryptococcus neoformans was confirmed in the surgical specimen, and the patient was diagnosed with CM. In addition, venule congestion in the parenchyma was observed with extensive fibrosis and compressed veins in the subarachnoid space. The patient died 26 days after admission. Autopsy revealed that pathological changes were localized in the cerebellum. CONCLUSION: C. neoformans can induce extensive fibrosis of the subarachnoid space, which may compress small veins mechanically inducing venule congestion and massive cerebral infarction. In such cases, the clinical course can be severe and even rapidly fatal. An atypical pattern of infarction on MRI should alert clinicians to the possibility of C. neoformans infection.

Non-traumatic recurrent dissection and its spontaneous repair in the circle of Willis: report of two autopsy cases.

Post-mortem examinations of the circle of Willis in two cases of subarachnoid hemorrhage disclosed a wide spectrum of vasculopathy ranging from a minimal tear between the intima and media, and between the media and adventitia, to complete transmural disruption leading to the formation of pseudoaneurysms. The presence of coexistence of the focal lesions with complete replacement of the entire arterial wall with thick fibrous connective tissues and the vasculopathy was suggestive of the spontaneous repair of recurrent non-traumatic dissection of intracranial arteries. The patients were 58-year-old and 43-year-old females. There was no history of injury to the head or neck in either case. They were hypertensive, but the degree of atherosclerotic changes in the circle of Willis was compatible with age. There was no histological evidence of vasculitis. The role of hypertension and medial mucoid degeneration in the genesis of non-traumatic dissection of intracranial arteries was discussed.