Central nervous involvement, mainly with symptoms of cranial neuropathies, occurs in 2–8% of patients with granulomatosis with polyangiitis (GPA). Meningeal involvement, with persistent and severe headache as main manifestation and abnormal thickening and enhancement of the dural mater on postcontrast magnetic resonance imaging, is extremely rare. We present a case of pachymeningitis due to limited GPA, providing simultaneously a literature review. 1. Introduction Granulomatosis with polyangiitis (GPA, formerly known as Wegener) is a rare, systemic disease of unknown etiology, characterized by necrotizing granulomatous inflammation and vasculitis, which in its classic form chiefly affects the upper and lower respiratory tracts and kidney [1]. Involvement of the nervous system is seen in about 23–54% of cases. The most common neurological manifestations are mononeuritis multiplex or, less frequently, distal symmetric sensorimotor polyneuropathies [2, 3]. Involvement of the central nervous system occurs in 2–8% of the patients with GPA and is commonly associated with dysfunction of one or several cranial nerves, most commonly the second, third, sixth, and seventh nerves. Meningeal inflammation, also known as hypertrophic pachymeningitis due to the typical presentation of thickening and contrast enhancement seen on magnetic resonance imaging (MRI), is extremely rare. We describe a patient with limited GPA with nasal lesions, otitis media, mastoiditis, sensorineural hearing loss, pachymeningitis, and positive cytoplasmic and proteinase-3 (PR-3) anti neutrophil cytoplasmic antibodies (ANCA). A literature review of pachymeningitis due to GPA is also provided. 2. Case Presentation In January 2012, a 50-year-old man presented with nasal obstruction, a “clogged” sensation in his left ear, tinnitus, vertigo, and severe headache on the left parietofrontal area of the face. The patient reported that the nasal congestion with fluid-thickened secretion started one year ago. After 3 months, he presented with a “clogged” sensation in his left ear with headache, which led to a diagnosis of serous otitis media. Myringotomy was performed, without resolution of symptoms. In August 2011, arthralgias, especially of the large joints (knees, elbows), was initiated, which resolved with low-dose prednisone for one month. In October 2011, the “clogged” sensation in his left ear and headache were deteriorated, and brain computed tomography (CT) was performed, which showed left mastoiditis. The patient was treated with antibiotics and nonsteroidal anti-inflammatory drugs, and after
References
[1]
J. C. Jennette, R. J. Falk, P. A. Bacon et al., “2012 revised International Chapel Hill consensus conference nomenclature of vasculitides,” Arthritis & Rheumatism, vol. 65, pp. 1–11, 2013.
[2]
G. S. Hoffman, G. S. Kerr, R. Y. Leavitt et al., “Wegener granulomatosis: an analysis of 158 patients,” Annals of Internal Medicine, vol. 116, no. 6, pp. 488–498, 1992.
[3]
H. Nishino, F. A. Rubino, R. A. DeRemee, J. W. Swanson, and J. E. Parisi, “Neurological involvement in Wegener's granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic,” Annals of Neurology, vol. 33, no. 1, pp. 4–9, 1993.
[4]
G. Di Comite, E. P. Bozzolo, L. Praderio, M. Tresoldi, and M. G. Sabbadini, “Meningeal involvement in Wegener's granulomatosis is associated with localized disease,” Clinical and Experimental Rheumatology, vol. 24, supplement 41, pp. S60–S64, 2006.
[5]
A. G. Fam, E. Lavine, L. Lee, B. Perez-Ordonez, and M. Goyal, “Cranial pachymeningitis: an unusual manifestation of Wegener's granulomatosis,” Journal of Rheumatology, vol. 30, no. 9, pp. 2070–2074, 2003.
[6]
Case Records of the Massachusetts General Hospital Weekly Clinicopathological Exercises, “Case 12-1988. A 72-year-old man with headache and multiple cranial-nerve palsies,” The New England Journal of Medicine, vol. 318, pp. 760–768, 1988.
[7]
N. J. Newman, T. L. Slamovits, S. Friedland, and W. B. Wilson, “Neuro-ophthalmic manifestations of meningocerebral inflammation from the limited form of Wegener's granulomatosis,” American Journal of Ophthalmology, vol. 120, no. 5, pp. 613–621, 1995.
[8]
U. Specks, K. G. Moder, and T. J. McDonald, “Memingeal involvement in Wegener granulomatosis,” Mayo Clinic Proceedings, vol. 75, no. 8, pp. 856–859, 2000.
[9]
J. M. Murphy, B. Gomez-Anson, J. H. Gillard et al., “Wegener granulomatosis: MR imaging findings in brain and meninges,” Radiology, vol. 213, no. 3, pp. 794–799, 1999.
[10]
R. Seror, A. Mahr, J. Ramanoelina, C. Pagnoux, P. Cohen, and L. Guillevin, “Central nervous system involvement in wegener granulomatosis,” Medicine, vol. 85, no. 1, pp. 54–65, 2006.
[11]
M. Spranger, S. Schwab, H.-M. Meinck et al., “Meningeal involvement in Wegener's granulomatosis confirmed and monitored by positive circulating antineutrophil cytoplasm in cerebrospinal fluid,” Neurology, vol. 48, no. 1, pp. 263–265, 1997.
[12]
S. Bawa, C. Mukhtyar, S. Edmonds, and M. Webley, “Refractory Wegener's meningitis treated with rituximab,” Journal of Rheumatology, vol. 34, no. 4, pp. 900–902, 2007.
[13]
A. Sharma, S. Kumar, A. Wanchu et al., “Successful treatment of hypertrophic pachymeningitis in refractory Wegener's granulomatosis with rituximab,” Clinical Rheumatology, vol. 29, no. 1, pp. 107–110, 2010.
[14]
N. Tamura, R. Matsudaira, M. Hirashima et al., “Two cases of refractory Wegener's granulomatosis successfully treated with rituximab,” Internal Medicine, vol. 46, no. 7, pp. 409–414, 2007.
[15]
J. Hermann, P. Reittner, M. Scarpatetti, and W. Graninger, “Successful treatment of meningeal involvement in Wegener's granulomatosis with infliximab,” Annals of the Rheumatic Diseases, vol. 65, no. 5, pp. 691–692, 2006.
