Clinical analysis of 19 cases with granulomatosis with polyangiitis complicated with hypertrophic cranial pachymeningitis / 中华风湿病学杂志

ABSTRACT

Objective:To discuss the clinical features and treatment of 19 patients with granulomatosis with polyangiitis (GPA) complicated with hypertrophic cranial pachymeningitis (HCP).Methods:The clinical features of 19 patients diagnosed with GPA complicated with HCP in Peking Union Medical College Hospital were retrospectively analyzed.Results:Among the 315 patients with GPA, 19 (12 males, 7 females, with) were diagnosed with HCP at Peking Union Medical College Hospital. The median age was 57 (19-64) years. In the neurological manifestations per se, all patients had headache, 16 patients had cerebral involvement, which included 8 cases at the frontal area, 8 cases at the temporal area, 8 cases at the skull base area (4 cases with parasellar involvements including 3 cases with cavernous sinus involvement and 2 cases with orbital involvement), 6 cases of tentorium involvement, 2 cases of cerebral palsy, 1 case of calvarium, 1 case of occipital, and 1 case with combined spinal pachymeningitis, respectively. In systemic manifestations, 10 patients had fever, 8 patients had weight lose, 4 patients had lung involvement, 3 patients had kidney involvement, 16 patients had nasosinusitis, 10 patients had tympanitis, and 16 patients had localized GPA. The laboratory tests showed that 15 patients had positive anti-neutrophil cytoplasmic antibodies (ANCA), including 8 cases with positive proteinase 3 (PR3)-ANCA and 6 cases with positive myeloperoxidase (MPO)-ANCA. Sixteen patients had lumbar puncture examination, 9 cases had elevated cerebrospinal pressure, 10 cases had elevated level of protein in cerebrospinal fluid. Nineteen patients were treated with glucocorticoids (12 patients accepted pulse therapy) and immunosuppressive agents. Twelve patients were treated with intrathecal injections of dexamethasone combined with or without Methotrexate (MTX). All 19 patients were improved.Conclusion:HCP, as a rare but serious manifestation of GPA, is not rare in active cases and should be intensively treated.