Hypertrophic Pachymeningitis as a Delayed Complication of Granulomatosis with Polyangiitis

Abstract

A 69-year-old man presented with upper airway symptoms, multiple lung nodules and masses, proteinuria and hematuria, and an increased level of proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA). Granulomatosis with polyangiitis (GPA) was diagnosed by a transbronchial lung biopsy. All of these symptoms were ameliorated and the level of PR3-ANCA declined following treatment with prednisolone and cyclophosphamide. The patient developed a headache 16 months after the onset of symptoms, and contrastenhanced magnetic resonance imaging showed the thickening of the dura mater, which suggested that hypertrophic pachymeningitis (HP) had developed as a complication of GPA. HP can be a unique complication of GPA at recurrence, and can occur without the relapse of other lesions or an increase in PR3-ANCA level.

ANCA-Associated Vasculitis: Advancement in Pathogenesis, Clinical Features and Management

“…Despite neurological manifestation is not uncommon with GPA, CNS clinical features in GPA could cause fatal events. Hypertrophic pachy meningitis with unchanged PR3-ANCA level was reported as a unique consequence of GPA [31,32]. Because of central and peripheral nervous system involvement, inpatients with neurologic disorders are more susceptible to GPA [33].…”

Hypertrophic pachymeningoencephalitis associated with temporal giant cell arteritis

“…Contrast-enhanced MRI can be indicative of HP, but leptomeningeal biopsy remains the gold standard for definite diagnosis 1. An autoimmune disorder known to be associated with HP is granulomatosis with polyangiitis (Wegener’s granulomatosis) 2. A few cases of temporal arteritis (giant cell arteritis (GCA)) accompanied by HP have been reported 3–7.…”