Bolis, Samer, DO

Kim, Susan, MD

Malik, Anam, MD

English

Lehigh Valley Health Network: LVHN Scholarly Works

Lehigh Valley Health NetworkLVHN Scholarly WorksDepartment of MedicineA Case of Refractory Primary Angiitis of theCentral Nervous System.Anam Malik MDLehigh Valley Health Network, Anam.Malik@lvhn.orgSamer Bolis DOLehigh Valley Health Network, Samer.Bolis@lvhn.orgSusan Kim MDLehigh Valley Health Network, Susan.Kim@lvhn.orgFollow this and additional works at: https://scholarlyworks.lvhn.org/medicinePart of the Internal Medicine Commons, Medical Sciences Commons, and the RheumatologyCommonsThis Poster is brought to you for free and open access by LVHN Scholarly Works. It has been accepted for inclusion in LVHN Scholarly Works by anauthorized administrator. For more information, please contact LibraryServices@lvhn.org.Published In/Presented AtMalik, A. Bolis, S. Kim, S. (2017, October 28). A Case of Refractory Primary Angiitis of the Central Nervous System. Poster Presented at:American College of Physicians, Harrisburg, PA.Anam Malik MD1, Samer Bolis DO1, Susan Kim MD2A Case of Refractory Primary Angiitis of the Central Nervous System© 2017 Lehigh Valley Health NetworkINTRODUCTION1Department of Internal Medicine, 2Department of Rheumatology, Lehigh Valley Health Network, Allentown, Pennsylvania•  Primary angiitis of the CNS (PACNS) is a rare vasculitis that is particularly challenging to diagnose. •  It should be considered in the setting of recurrent strokes, continued cognitive decline, and recurrent or progressive focal neurological deficits. •  It is essentially a diagnosis of exclusion after ruling out systemic involvement with confirmatory cerebrovascular imaging or histopathological evidence of vasculitis.1 •  Current treatment recommendations include high dose corticosteroids and cyclophosphamide. Limited data supports the use of rituximab in refractory cases of PACNS.2,3 References:1.  Birnbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol. 2009;66(6):704-9.2.  Salvarani C, Brown RD, Huston J, Morris JM, Hunder GG. Treatment of primary CNS vasculitis with rituximab: case report. Neurology. 2014;82(14):1287-8.3.  De boysson H, Arquizan C, Guillevin L, Pagnoux C. Rituximab for primary angiitis of the central nervous system: report of 2 patients from the French COVAC cohort and review of the literature. J Rheumatol. 2013;40(12):2102-3.CASE•  Our patient is a 56-year-old male with recurrent multifocal cerebral infarcts, encephalopathy and seizures secondary to PACNS diagnosed a year prior with cerebral arteriogram. •  He presented with worsening right sided weakness, expressive aphasia, and urinary incontinence.•  MRI upon admission revealed new areas of acute/subacute infarctions in the basal ganglia and corona radiata. •  Lumbar puncture was unremarkable. Other labs confirmed no underlying systemic vasculitis with negative ANA and ANCA profiles. Renal biopsy showed diabetic nephropathy without signs of connective tissue disease. •  Cerebral arteriogram was repeated, showing multiple small peripheral arterial narrowing and beading consistent with a diagnosis of PACNS.•  Despite increasing IV corticosteroids and treatment with Cytoxan for over 6 months, our patient continued to demonstrate decline. •  During the hospital course, Rituxan infusions were initiated and patient began to show clinical improvement over the following 6 weeks. He unfortunately regressed after a few months of treatment.DISCUSSION•  Most patients with PACNS demonstrate improvement in symptoms and on imaging when trialed on steroids with or without Cytoxan.•  No studies suggest an optimal treatment regimen or duration. •  Three case reports in the current literature describe clinical and radiographic improvement in adults with PACNS after treatment with rituximab.2,3•  In one case where the diagnosis was made by angiographic findings and an abnormal CSF, rituximab was given as first-line therapy.2•  In the other two cases, the diagnosis of PACNS was histopathological with evidence of a lymphocytic vasculitic process involving the brain and spinal cord. Rituximab was used as first-line in one and after failing Cytoxan in the other.2•  Our patient was deemed to have failed Cytoxan and unfortunately Rituxan. •  This case shows the difficulty of diagnosing PACNS. The goal standard for the diagnosis of PACNS is a brain biopsy. •  Only a positive biopsy can ascertain the diagnosis which we did not have. Staining, immunohistochemistry studies, and immunophenotyping are needed to rule out other mimickers.  •  Refractory cases are rare and require reconsidering the diagnosis. 

A Case of Refractory Primary Angiitis of the Central Nervous System.

https://scholarlyworks.lvhn.org/cgi/viewcontent.cgi?article=2477&context=medicine

A Case of Refractory Primary Angiitis of the Central Nervous System.

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