Neurol India Home 

Year : 2021  |  Volume : 69  |  Issue : 4  |  Page : 1097--1098

Isolated Cerebral Vasculitis in a Patient with Rheumatoid Arthritis

Bhupen Barman1, Kalyan Sarma2, Pranjal Phukan3,  
1 Department of Internal Medicine, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, Meghalaya
2 Department of Neuroimaging and Interventional Neuroradiology, All India Institute of Medical Sciences, New Delhi, India
3 Department of Radiology and Imaging, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, Meghalaya

Correspondence Address:
Pranjal Phukan
Department of Radiology and Imaging, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong - 793 018, Meghalaya

How to cite this article:
Barman B, Sarma K, Phukan P. Isolated Cerebral Vasculitis in a Patient with Rheumatoid Arthritis.Neurol India 2021;69:1097-1098

How to cite this URL:
Barman B, Sarma K, Phukan P. Isolated Cerebral Vasculitis in a Patient with Rheumatoid Arthritis. Neurol India [serial online] 2021 [cited 2021 Dec 4 ];69:1097-1098
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Vasculitis constitutes a heterogeneous group of chronic inflammatory diseases characterized by inflammation and necrosis of blood vessels often due to an immune reaction. They can occur independently or as a secondary feature of established disease. Rheumatoid arthritis (RA) is a chronic, systemic inflammatory disease characterized by symmetric, peripheral polyarthritis. Central nervous system (CNS) lesions are usually spared in RA and CNS involvement is quite unusual in patients with RA, although cerebral vasculitis, rheumatoid nodules and meningitis have all been reported.[1]

A 37-year-old woman was admitted with a 1-month history of a headache to hospital. She had been diagnosed with seropositive RA twelve years before, with occasional urticarial rash and redness of the left eye. She received methotrexate 7.5 mg per week, hydroxychloroquine (HCQS) 200 mg twice daily, low doses of prednisone 7.5 mg per day and nonsteroidal anti-inflammatory drugs (NSAIDs). She presented with recurrent history of left-sided frontal headache radiating towards back and neck. Along with her also complaining of persistent sleep disturbance, fine tremor and also had one episode of single partial seizure. There was no fever or vomiting. Her body temperature was normal and her blood pressure was 110/70 mmHg. Physical examination revealed typical joint deformities of RA, but without any subcutaneous nodules or skin lesions. Evidence of synovitis was found at both wrists as well as at the second and third metacarpophalangeal joints of both hands. She had a history of redness over left eye four years back and was diagnosed with a case of iridocyclitis which improved with an oral steroid. Also, she had pulmonary tuberculosis three years back which was treated with a full course of anti-tubercular therapy under DOTS (directly observed treatment, short-course) regimen and got cured. Her neurological examination was normal including her deep tendon and plantar reflexes. There was no evidence of signs of meningeal irritation or focal neurological deficit. Laboratory tests revealed the following results: Erythrocyte sedimentation rate of 77 mm/h, C-reactive protein of 16 mg/L (normal: <6) and hemoglobin of 14.9 gm/dL. The white blood cell count was normal (8180/mm3) as were her platelets (380,000/mm3). Liver and kidney function tests were normal, as well as blood glucose levels. No obvious infection, disseminated intravascular coagulation, atlantoaxial dislocation or other collagen diseases were recognized by physical and blood examinations. She was positive for rheumatoid factor and anti-CCP. Her antinuclear antibody (ANA) was positive but ds-DNA was negative and complements level and antineutrophil cytoplasm antibodies (ANCA) were normal. Serology for human immunodeficiency virus, hepatitis B, and C viruses were non-reactive. The fundus examination was normal. Magnetic Resonance Imaging (MRI) [Figure 1]a and [Figure 1]b of her brain demonstrated hyperintensity foci on Fluid attenuated inversion recovery (FLAIR) images within the deep and subcortical white matter of the right occipital lobe with irregular beading like narrowing at the M1 segment of left middle cerebral artery (MCA) resulting in reduction in flow signal intensity of the distal MCA branches on time-of-flight magnetic resonance angiography (TOF MRA). She was treated with pulse dose methylprednisolone (1 gm/day for 3 days) followed by oral steroids in tapering doses. Her methotrexate therapy was intensified to 15 mg per week and HCQS was continued. Repeat MRI was done after two months of follow-up revealed good flow signal intensity through right MCA on TOF MRA [Figure 2]. Her headache disappeared in two weeks and there were no symptoms of meningitis or focal neurological signs after 2 months of follow-up.{Figure 1}{Figure 2}

CNS complications are well documented in autoimmune diseases such as Sjögren's syndrome, systemic lupus erythematosus (SLE), and Behçet's disease. Although rare, wide spectrum of neurological involvement can be seen in patients with RA viz spinal cord compression due to atlantoaxial subluxation, polymyositis, mononeuritis multiplex, peripheral neuropathy, rheumatoid nodules in the central or peripheral nervous system, and inflammatory CNS lesion like vasculitis.[2] The neurological involvement in rheumatoid cerebral vasculitis is manifested by focal deficit like hemiplegia, simple or complex seizure, the involvement of cranial nerve leading to visual field defect, altered sensorium, acute confusional state, or mild cognitive impairment/dementia. The present case report is similar to the majority of previously reported cases in that our patient displayed CNS vasculitis and with few features of systemic vasculitis.[3],[4],[5] Our patient had a long-standing history of RA, which required steroid therapy and the diagnosis of rheumatoid cerebral vasculitis was made by cerebral MRI. Other causes of cerebral vasculitis were ruled out as far as possible based on history, clinical features, laboratory investigations, neuroimaging, and response to the therapy. In our case, T2 FLAIR MRI demonstrates hyperintensity foci within the deep and subcortical white matter of the right occipital lobe. Headache is a common symptom and may suggest temporal arteritis, particularly as this condition can occur in patients with RA. The young age and normal doppler study of extracranial arteries of our patient weighed against temporal arteritis, and the positive tests for RA factor supported the diagnosis of rheumatoid vasculitis. Glucocorticoids at different dosages and administrations have been reported in the treatment of CNS rheumatoid vasculitis. Several other treatment options such as cyclophosphamide, azathioprine, intravenous immunoglobulin, and rituximab are available for patients with corticosteroid resistant or refractory vasculitis.[5] In our case, pulse methylprednisolone and intensification of the methotrexate therapy have given a good outcome.

Neurological involvement in RA is rare and, in some cases, can be life-threatening. A high index of clinical suspicion is necessary for its diagnosis and subsequent management. History of the patient and meticulous clinical examination can provide valuable pointers towards its diagnosis, especially when corroborated with biochemical and neuroimaging tests. The role of imaging modalities like MRI brain as components of the work-up of such patients is very essential.

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