A retrospective study has investigated the central nervous system’s (CNS) involvement in granulomatosis with polyangiitis (GPA) in a group of Greek patients. The study found that CNS involvement does not affect long-term patient outcomes.
ANCA-associated vasculitis (AAV) refers to a group of rare diseases characterized by inflammatory cell infiltration and the death of blood vessel walls. One of these diseases is GPA, formerly known as Wegener’s granulomatosis. Some 20-50% of GPA patients suffer from neurological complications, including those linked to the CNS.
The study, “Central nervous system involvement in patients with granulomatosis with polyangiitis: a single-center retrospective study,” published in the journal Clinical Rheumatology, set out to estimate the frequency of CNS involvement in patients with biopsy-proven ANCA-positive GPA, and factors associated with its occurrence. Patients were diagnosed from 1995 to 2015 at Laiko Hospital in Athens, Greece.
Of the 139 patients with biopsy-proven AVV, 77 were diagnosed with GPA. Of these, nine (seven men and two women) were diagnosed as having CNS involvement. The median age was 49, with ages ranging from 26-67.
CNS symptoms included sensor or sensorimotor symptoms in three patients; headaches and hearing loss in three; delirium and seizures in two; double vision in one; and cerebellar symptoms with wide gait and intention tremor in one patient.
Manifestations of the disease in the ear, nose, and throat system were more common among GPA patients with CNS involvement than in those without (77.8% vs. 25.4%). Patients with CNS involvement, on the other hand, had a lower frequency of vasculitis in the lungs (44.4% vs. 79.4%).
Six patients had available samples of cerebrospinal fluid (CSF), the fluid that surrounds the brain and the spinal cord. The samples did not show any specific abnormality, although they did show a slightly increased level of white blood cells.
Looking at treatment strategies for the patients, the team found that 66.7% of patients responded to immunosuppressive treatment. Relapse rates were similar in GPA patients with or without CNS involvement, while patient survival was found to be unaffected by CNS involvement in patients receiving treatment at an early stage.
Nonetheless, “the development of certain criteria for the diagnosis of CNS involvement in the context of GPA and subsequent trials to define the optimal type of therapy for these patients are needed,” the authors concluded.