Primary Angiitis of the Central Nervous System: Differences in the Profile Between Subtypes and Outcomes From an Indian Cohort
Year: 2024
Type: article
Study design: Observational Study
Abstract: Objective We sought to distinguish the subtypes of Primary angiitis of Central Nervous system (PACNS) and describe their outcomes in an Indian Cohort. Background PACNS is a rare disease with significant morbidity and mortality that has distinct subtypes with diagnostic complexity. Design/Methods This retrospective single center study included adult patients diagnosed with PACNS as per Calabrese and Malleck's criteria, between 2000-2019. Small and medium vessel vasculitis were defined, and their clinical and radiological profile, treatment and outcomes were compared. Functional outcomes were noted at 6 months, 1 year and at last follow up, while relapses were noted at last follow up. A poor outcome was defined as modified Rankin scale >2. Results Of the 72 patients, 50(69.4%) were males. The small vessel vasculitis subtype had a younger age at onset (30.5 vs 40.5 years, P=.014), presented less often as a stroke (22% vs 62%, P=.001) and had greater delay in diagnosis and treatment initiation (median of 620 days vs 118 days, P=.001) compared with medium vessel vasculitis subtype. Although no difference was noted at 6 months, small vessel vasculitis group had poor outcomes at 1 year and last follow up (57% vs 20%, P=.011 and 72% vs 34%, P= .005 respectively) and had more relapses at last follow up (89% vs 30%, P <.001) when compared to medium vessel vasculitis group. On analyzing the entire cohort, 50/72(69%) and 37/53 (69.8%) patients had a good outcome at 6 months and 1 year respectively. Relapse was noted in 35/72 (49%) at final follow up. The choice of the treatment regimen (steroids or steroids with cyclophosphamide) did not predict outcomes or relapses. Conclusions Small vessel vasculitis subtype of PACNS is a distinct entity that has diagnostic and treatment delays, poor long-term outcomes and more relapses. Recognizing the different subtypes of PACNS may help to expedite diagnosis and treatment.
Source: Neurology
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W4403206747