PR3ANCA Related Cerebral Vasculitis in Ulcerative Colitis Presenting with Orbital Involvement: A Case Report with Review of Literature

Unnikrishnan, Athira; Azodi, Shila; Ansari, Nadeem A.; Brown, Megan; Kamnetz, Joshua; Uchiyama, Robert C.

doi:10.1155/2014/582094

Cited by 7 publications

(13 citation statements)

References 17 publications

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“…One of the cases that did not undergo brain biopsy did have T2 hyperintensity of the centrum semiovale, which our patient had. This finding has been reported as typical of microangiopathy associated with CNS vasculitis [ 6 , 7 ]. Almost all 15 cases had recovery of their neurological deficits with prednisone use, while two patients showed recovery with prednisone combined with cyclophosphamide, and one case recovered with a combination of prednisone, azathioprine, and cyclosporine A. Steroid treatment does seem to afford the best outcome for CNS vasculitis, whether associated with UC or not.…”

Section: Discussionsupporting

confidence: 77%

“…Though the exact incidence specifically of CNS vasculitis secondary to UC is not known, it is estimated that cerebrovascular manifestations are seen in 0.12–4% of inflammatory disease patients [ 5 ]. These include arterial and venous thrombosis, seizures, leukoencephalitis, and vasculitis [ 6 ]. Unnikrishnan et al report that there have only been fifteen case reports of possible cerebral vasculitis in UC.…”

Section: Discussionmentioning

confidence: 99%

“…Unnikrishnan et al report that there have only been fifteen case reports of possible cerebral vasculitis in UC. Among these reported cases, eleven patients had confirmed vasculitis based on histopathology, angiogram, or serology [ 6 ]. Four patients out of the 15 had brain biopsies.…”

Section: Discussionmentioning

confidence: 99%

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A Case of Cerebral Vasculitis Associated with Ulcerative Colitis

Raj

Arkebauer

Waters

et al. 2015

Case Reports in Rheumatology

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Ulcerative colitis (UC) is a chronic, debilitating condition characterized by inflammation of the colonic mucosa. It is regarded as a systemic inflammatory disorder that can affect a number of organ systems. Central nervous system disease associated with UC is a rare sequela of inflammatory bowel disease, occurring in less than 5% of cases. These manifestations include arterial and venous thrombosis, leukoencephalitis, seizures, and vasculitis. We present a case of a 61-year-old female with a two-year history of well-controlled ulcerative colitis, who developed altered mental status and weakness. On brain imaging, she was found to have cerebral lesions which were biopsied. Histopathology subsequently revealed coagulative necrosis and inflammation characteristic of vasculitis. Rheumatology serologies were negative, and the patient was started on steroids that dramatically improved her neurological function, with no residual deficits, and led to resolution of the brain lesions.

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Section: Discussionsupporting

confidence: 77%

Section: Discussionmentioning

confidence: 99%

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A Case of Cerebral Vasculitis Associated with Ulcerative Colitis

Raj

Arkebauer

Waters

et al. 2015

Case Reports in Rheumatology

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“…The current understanding of this rare association stems predominantly from case reports and small case series with not more than 10 patients. Various vasculitides have been associated with IBD, especially TAK [6–8] but also GPA [9], isolated cutaneous vasculitis [10], and central nervous system vasculitis [11]. …”

mentioning

confidence: 99%

Vasculitis in patients with inflammatory bowel diseases: A study of 32 patients and systematic review of the literature

Khalidi

Dehghan

et al. 2016

Seminars in Arthritis and Rheumatism

120

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Background Published small case series suggest that inflammatory bowel disease [IBD; Crohn’s disease (CD) or ulcerative colitis (UC)] and vasculitis co-occur more frequently than would be expected by chance. Objectives To describe this association by an analysis of a large cohort of carefully studied patients and through a systematic literature review. Methods Patients with both IBD and vasculitis enrolled in the Vasculitis Clinical Research Consortium (VCRC) Longitudinal Studies, followed in Canadian Vasculitis research network (CanVasc) centers and/or in the University of Toronto’s IBD clinic were included in this case series. A systematic literature review of patients with IBD and vasculitis involved a PubMed search through February 2014. The main characteristics of patients with Takayasu arteritis (TAK) and IBD were compared to those in patients with TAK without IBD followed in the VCRC. Results The study identified 32 patients with IBD and vasculitis: 13 with large-vessel vasculitis [LVV; 12 with TAK, 1 with giant cell arteritis (GCA); 8 with CD, 5 with UC]; 8 with ANCA-associated vasculitis [AAV; 6 granulomatosis with polyangiitis (GPA), 2 with eosinophilic granulomatosis with polyangiitis (EGPA)]; 5 with isolated cutaneous vasculitis; and 6 with other vasculitides. Patients with LVV and AAV were mostly female (18/21). The diagnosis of IBD preceded that of vasculitis in 12/13 patients with LVV and 8/8 patients with AAV. The review of the literature identified 306 patients with IBD and vasculitis: 144 with LVV (133 TAK; 87 with IBD preceding LVV), 19 with AAV [14 GPA, 1 EGPA, 4 microscopic polyangiitis (MPA)], 66 with isolated cutaneous vasculitis, and 77 with other vasculitides. Patients with IBD and TAK were younger and had more frequent headaches, constitutional symptoms, or gastrointestinal symptoms compared to those patients in the VCRC who had TAK without IBD. Conclusions These findings highlight the risk of vasculitis, especially TAK, in patients with IBD (both CD and UC).

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“…We performed skin biopsies of two nodules on her lower extremities to evaluate the evidence for systemic vasculitis, but no specific findings were observed. Unnikrishnan et al 14) reviewed 15 case reports of possible cerebral vasculitis in UC. Among them, 11 patients had definite vasculitis based on histopathology, angiography or serology.…”

Section: Discussionmentioning

confidence: 99%

Recurrent Cerebral Hemorrhage Associated with Ulcerative Colitis

Yanagisawa

Mizukami

Akiyama

et al. 2019

J St. Marianna Univ

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Ulcerative colitis (UC) is a rare cause of stroke. Most such cases result from cerebral venous thrombosis, and cerebral vasculitis is one cause of venous thrombus formation. Here, we report a patient with UC who experienced recurrent cerebral hemorrhage. A 53-year-old Japanese woman with UC presented with sudden onset of disorientation and sensory aphasia. Brain computed tomography revealed cerebral hemorrhage in the left temporal lobe. She had a history of UC for two decades but had discontinued her medication. On the fifth hospital day, another cerebral hemorrhage occurred in the right occipital lobe. After steroid therapy, these abnormal findings on MRI improved within a short time, and she was discharged from hospital with no sequelae. Physicians should be alert to cerebral hemorrhage in patients with UC, consider cerebral vasculitis as an etiology, and treat with steroid therapy for a good outcome.

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PR3ANCA Related Cerebral Vasculitis in Ulcerative Colitis Presenting with Orbital Involvement: A Case Report with Review of Literature

Cited by 7 publications

References 17 publications

A Case of Cerebral Vasculitis Associated with Ulcerative Colitis

A Case of Cerebral Vasculitis Associated with Ulcerative Colitis

Vasculitis in patients with inflammatory bowel diseases: A study of 32 patients and systematic review of the literature

Recurrent Cerebral Hemorrhage Associated with Ulcerative Colitis

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