Primary Central Nervous System Vasculitis - Long Term Clinical Course and Treatment Response

Abstract

Background: Primary CNS Vasculitis (PCNSV) is an uncommon inflammatory condition selectively affecting blood vessels of the central nervous system and has diagnostic difficulties. Long term treatment outcomes are unclear.

Objective: To elucidate the clinical progression and long-term outcomes of patients with biopsy proven PCNSV.

Methods and material: Clinical data of eight consecutive patients with PCNSV established by meningocortical biopsy were reviewed. Patients had undergone extensive evaluation for secondary causes.

Results: The average age at presentation was 33.5 years (25 to 47 years). Three were females. The average follow-up period was 65 months (24 to 108 months). Clinical presentation included acute or subacute focal neurological deficits, seizures, headache and cognitive decline. MRI findings consisted of multifocal gray and white matter signal changes with microhaemorrhages. On contrast multifocal areas of enhancement affecting gray matter, white matter, vessel wall, dura and leptomeningeal areas were observed. Diagnostic yield of DSA was poor. Histopathology showed granulomatous angiitis in six and lymphocytic angiitis in two. Treatment protocols followed were similar for all patients: initial induction with intravenous methylprednisolone and cyclophosphamide, followed by maintenance immunosuppressants (with or without oral steroids). Disease control was reflected by significant improvement on MRI. Multiple clinical relapses occurred over the years (13 relapses, 0 to 4 per patient). Serial MRI studies helped detect subclinical disease activity. Treatment escalation aimed at achieving "no evidence of disease activity" helped in better disease control and remission in patients with active disease. Rituximab was useful in select cases to control disease activity and as rescue therapy (n = 3). Mean MRS score at last follow up was 0 or 1 (unchanged from baseline). Treatment complications included infections and avascular necrosis of hip (n = 1). One patient died due to severe COVID-19 pneumonitis.

Conclusion: PCNSV has varied clinical presentation and diagnostic challenges. Immunosuppressive therapy is necessary and is effective but with associated risks. Histopathological confirmation helps to prognosticate and initiate early aggressive treatment for better results. Close clinico-radiological follow up and long-term immunotherapy to ensure disease remission can help manage these patients successfully.

Keywords: Primary central nervous system vasculitis; diagnosis; outcome; treatment.