Pachymeningeal Thickening as a Neurological Manifestation of Granulomatosis With Polyangiitis

Abstract

BACKGROUNDGranulomatosis with polyangiitis (GPA) is a form of vasculitis associated with antineutrophil cytoplasmic antibodies, characterized by granulomatous inflammation and varied degrees of focal necrotizing vasculitis, affecting medium and small blood vessels. This disease has a tropism to the respiratory tract and kidneys. Among the neurological manifestations, peripheral neuropathy is the most common type of neurological involvement and central nervous system involvement has been reported in 7-11% of patients. We report a case of GPA in a patient with neurological symptoms and pachymeningeal thickening. CASE REPORTA 46-year-old female patient sought the Emergency Department due to two episodes of seizures, being hospitalized for investigation. Patient had been using hydroxychloroquine 400 mg daily for 1 year due to a history of bilateral parotitis, arthralgias, purpuric dermatitis, reagent ANA and reagent anti-SSA(Ro). Magnetic resonance imaging demonstrated an area of gadoliniumenhancing nodular thickening of the pachymeningeal lining in the left temporo-occipital transition, with a thickness of 0.6 cm, and vasogenic edema in the white matter of the adjacent parenchyma. She had elevated acute phase reagents, c-ANCA reagent up to 1/20, nonreactive antiproteinase-3 and antimyeloperoxidase. The biopsy of the meningeal lesion showed chronic inflammation with palisade granuloma and central necrosis, favoring the diagnosis of granulomatosis with polyangiitis involving the central nervous system. The patient was discharged from the hospital asymptomatic and on high-dose prednisone, with an outpatient plan to start immunosuppressants. CONCLUSIONWe report a case of granulomatosis with polyangiitis presenting central nervous system evidenced by imaging exam, an uncommon form of manifestation of this condition.