Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2018;56(6):399-405.
doi: 10.5114/reum.2018.80719. Epub 2018 Dec 23.

Hypertrophic pachymeningitis as an important neurological complication of granulomatosis with polyangiitis

Żaneta Smoleńska  1 , Anna Masiak  1 , Zbigniew Zdrojewski  1
Affiliations
Review

Hypertrophic pachymeningitis as an important neurological complication of granulomatosis with polyangiitis

Żaneta Smoleńska et al. Reumatologia. 2018.

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.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Fig. 1
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
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].
See this image and copyright information in PMC

References

    1. Stone JH. Wegener`s Granulomatosis Etanercept Trial Research G. Limited versus severe Wegener’s granulomatosis: baseline data on patients in the Wegener’s granulomatosis etanercept trial. Arthritis Rheum. 2003;48:2299–2309. - PubMed
    1. de Groot K, Schmidt DK, Arlt AC, et al. Standardized neurologic evaluations of 128 patients with Wegener granulomatosis. Arch Neurol. 2001;58:1215–1221. - PubMed
    1. Anderson JM, Jamieson DG, Jefferson JM. Non-healing granuloma and the nervous system. Q J Med. 1975;44:309–323. - PubMed
    1. Nishino H, Rubino FA, DeRemee RA, et al. Neurological involvement in Wegener’s granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol. 1993;33:4–9. - PubMed
    1. Fragoulis GE, Lionaki S, Venetsanopoulu A, et al. Central nervous system involvement in patients with granulomatosis with polyangiitis: a single-center retrospective study. Clin Rheumatol. 2018;37:737–747. - PubMed

LinkOut - more resources