Introduction: Primary and secondary CNS vasculitis encompass a range of disorders that are notoriously challenging to diagnose. While rare, their protean clinical presentations make them frequent considerations in the differential diagnosis of various clinical scenarios. This study investigates the scope of primary and secondary CNS vasculitis in the US inpatient population and identifies risk factors for mortality and non-routine discharge.
Methods: A subset of adult patients (age ≥18 years) with a discharge diagnosis of “cerebral arteritis” (ICD-9-CM 437.4) were identified in the HCUP National Inpatient Sample database for years 2000-2011. We report patient characteristics and outcomes as well as complications and comorbidities. Logistic regression analysis was performed to identify factors associated with in-hospital death and non-routine discharge.
Results: A total of 6,269 discharges were identified, including 1,561 with cerebral arteritis as the primary discharge diagnosis. Mean age was 53.6 years (SD 16.7); sex was 67% female (n=6264). Mean Charlson Comorbidity Index was 1.2 (SD 1.5) indicating low comorbidity burden. The most common complications or comorbidities included disorders of cognition (15%, n=6269), ischemic stroke (12.2%, n=6269), any late effect of stroke (12%, n=6269), any intracranial hemorrhage (8.6%, n=6269), TIA (7.6%, n=6269), seizures/epilepsy (6.3%, n=6269), headache (4%, n=6269) and hydrocephalus (1.8%, n=6269). Combined, a range of inflammatory disorders were common (22.4%, n=6269) but individual diagnoses were not highly represented except SLE (9.2%) and RA (3.5%). In-hospital mortality was 5.1% (n=6269); discharges were routine for 48.9% (n=6269). Major complications associated with in-hospital mortality were hydrocephalus (OR 2.4, CI 1.2-4.8) and intracranial hemorrhage (OR 1.8, CI 1.3-2.7). Conditions associated with non-routine discharge were weakness (OR 2.2, CI 1.8-2.7), cognitive diagnoses (OR 1.9, CI 1.6-2.4) and ischemic stroke (OR 1.3, CI 1.1-1.6).
Conclusions: Cognitive disorders, in addition to various stroke subtypes, are the most prominent complications of CNS vasculitis in the US. In-hospital mortality in CNS vasculitis is linked with hydrocephalus and intracranial hemorrhage.