Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis

Abstract

The objectives of this study are to compare the initial clinical, laboratory, and imaging features in primary central nervous system vasculitis (PCNSV) vs secondary central nervous system vasculitis (SCNSV) and follow up after treatment with intravenous cyclophosphamide (IV-CYC) plus glucocorticosteroids (GCS): methylprednisolone (MP). Neurological, laboratory, and neuroimaging findings were analyzed in PCNSV and SCNSV patients. Cerebral biopsy (CB) was performed in nine patients. Both groups received at onset MP plus IV-CYC for 6 months, followed by bimonthly IV-CYC plus prednisone (PND) for 12 months. All patients were followed during 36 months. Thirty patients were included (12 PCNSV and 18 SCNSV). Focal and non-focal neurological manifestations were similar in both groups, headache being the most frequent manifestation in both groups. Fatigue, myalgias, arthralgias, neuropathy, low leukocytes and platelets, elevated erythrocyte sedimentation rate, positive antinuclear antibodies (ANA), anti-double-stranded DNA (dsDNA), antineutrophil cytoplasmic antibodies (ANCA), low complement, and rheumatoid factor were more frequent in SCNSV (p < 0.05). In cerebrospinal fluid, pleocytosis and proteins were higher in PCNSV (p < 0.05). Periventricular and subcortical hyperintense lesions were observed in cranial magnetic resonance imaging in both vasculitides. Cerebral angiography and angioresonance showed narrowing of vasculature in all patients in both groups. CB showed gliosis and lymphocytic infiltration within and around the walls in four patients and granulomatous infiltration in the other patients. After treatment, the Kaplan-Meier survival curve showed a higher relapse-free survival in PCNSV (p < 0.05). Neurological manifestations and neuroimaging findings were similar in both groups of vasculitides, but general symptoms, joint, musculoskeletal, and peripheral neuropathy were preponderant in SCNSV. After treatment with IV-CYC and GCS, patients with PCNSV had a higher relapse-free survival than those with SCNSV.

CSF Neurofilament light chain level predicts axonal damage in cerebral vasculitis

“…However, except for the increased CSF cell count, there was no initial evidence for PACNS or systemic vasculitis and thus immunosuppressive treatment was not started. Previous reports also described relapsing courses of PACNS, but showed clinically silent MRI pathologies and abnormal angiographies which in turn led to early immunosuppressive treatment …”

“…It is tempting to speculate whether the high CSF cell count in combination with two past cerebrovascular events in the absence of clinical and radiological pathologies, including conventional angiography, should already have led to the initiation of an immunosuppressive treatment . Physicians need to keep in mind that the highly increased CSF cell count seems to be a rare case in PACNS . Thus, a multistep diagnostic approach is essential to prevent initially incorrect or delayed treatment …”

A Closer Look at Angiitis of the central nervous system

“…Nevertheless, MRI is much more sensitive than CT in detecting changes in cerebral vasculitis, except for cerebral hemorrhage. The sensitivity is estimated at more than 75%, even close to 100%, 4 , 21 while its specificity is poor.…”

“…In SACNS, the brain involvement is usually a late and rare manifestation of the disease and is always accompanied with other symptoms and signs of the primary disease. Recently, Vera-Lastra et al 21 conducted a study and compared various aspects, including clinical features, in PACNS vs SACNS. According to their observations, headache was the most frequent neurological manifestation in both groups with similar frequencies; however, the symptom was more severe in PACNS than SACNS patients.…”

The diagnosis of primary central nervous system vasculitis

“…But not least among these difficulties of diagnosis is that the criteria on which a clinical diagnosis of primary CNS vasculitis can be made have not been firmly established or uniformly accepted 9 11. There is no real consensus on defining the disease; criteria for allowing a diagnosis based on angiography (either formal contrast or magnetic resonance (MR)/CT angiography) without histology are very commonly used in published studies and reports 9 12–20. Other authors do recommend relying on histology 10 11 20–23.…”