Hypertrophic pachymeningitis as an important neurological complication of granulomatosis with polyangiitis

Abstract

Headache is a common symptom in patients with granulomatosis with polyangiitis (GPA) mainly due to chronic sinusitis or orbital disease. Meningeal involvement may thus remain unrecognized for a long time. This can lead to a significant delay in accurate diagnosis, serious local damage of the central nervous system and high relapse rates. New diagnostic techniques such as contrast MRI allow one to identify inflammation of the dura mater in the course of GPA more frequently. The objective of this article is to characterize hypertrophic pachymeningitis (HP) in patients with GPA and report diagnostic difficulties associated with this complication.

Keywords: diagnosis and therapy; granulomatosis with polyangiitis; headache; hypertrophic pachymeningitis.

Fig. 1

Extensive dural thickening at the left tentorium cerebelli on T1 coronal gadolinium-enhanced T1-weighted MRI in patient with granulomatosis with polyangiitis from Department of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdańsk, Poland.

Fig. 2

Extensive dural thickening at the left tentorium cerebelli and falx cerebri on T1 axial gadoliniumenhanced T1-weighted MRI resembling Eiffel Tower at night [19].