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BMC Neurology
Published in: BMC Neurology 1/2018

Open Access 01-12-2018 | Case report

Severe localised granulomatosis with polyangiitis (Wegener’s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review

Authors: James E. Peters, Vivek Gupta, Ibtisam T. Saeed, Curtis Offiah, Ali S. M. Jawad

Published in: BMC Neurology | Issue 1/2018

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Abstract

Background

Granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis) is a multisystem vasculitis of small- to medium-sized blood vessels. Cranial involvement can result in cranial nerve palsies and, rarely, pituitary infiltration.

Case presentation

We describe the case of a 32 year-old woman with limited but severe GPA manifesting as progressive cranial nerve palsies and pituitary dysfunction. Our patient initially presented with localised ENT involvement, but despite treatment with methotrexate, she deteriorated. Granulomatous inflammatory tissue around the skull base resulted in cavernous sinus syndrome, facial nerve palsy, palsies of cranial nerves IX-XII (Collet-Sicard syndrome), and the rare complication of cranial diabetes insipidus due to pituitary infiltration. The glossopharyngeal, vagus and accessory nerve palsies resulted in severe dysphagia and she required nasogastric tube feeding. Her neurological deficits substantially improved with treatment including high dose corticosteroid, cyclophosphamide and rituximab.

Conclusions

This case emphasises that serious morbidity can arise from localised cranial Wegener’s granulomatosis in the absence of systemic disease. In such cases intensive induction immunosuppression is required. Analysis of previously reported cases of pituitary involvement in GPA reveals that this rare complication predominantly affects female patients.  
Literature
1.
Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised international Chapel Hill consensus conference nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11.CrossRefPubMed
2.
Godman GC, Churg J. Wegener's granulomatosis: pathology and review of the literature. AMA Arch Pathol. 1954;58(6):533–53.PubMed
3.
Fauci AS, Wolff SM. Wegener's granulomatosis: studies in eighteen patients and a review of the literature. Medicine (Baltimore). 1973;52(6):535–61.CrossRef
4.
5.
Ntatsaki E, Carruthers D, Chakravarty K, D'Cruz D, Harper L, Jayne D, et al. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014;53(12):2306–9.CrossRef
6.
Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016;75(9):1583–94.CrossRefPubMed
7.
Carrington CB, Liebow A. Limited forms of angiitis and granulomatosis of Wegener's type. Am J Med. 1966;41(4):497–527.CrossRefPubMed
8.
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(8):2299–309.CrossRefPubMed
9.
Peters JE, Salama AD, Ind PW. Wegener's granulomatosis presenting as acute systemic vasculitis following 20 years of limited tracheobronchial disease. J Laryngol Otol. 2009;123(12):1375–7.CrossRefPubMed
10.
Stegeman CA, Tervaert JW, de Jong PE, Kallenberg CG. Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener's granulomatosis. Dutch Co-Trimoxazole Wegener study group. N Engl J Med. 1996;335(1):16–20.
11.
Peters JE, Burke CJ, Morris VH. Three cases of rheumatoid arthritis with laryngeal stridor. Clin Rheumatol. 2011;30(5):723–7.CrossRefPubMed
12.
de Groot K, Schmidt DK, Arlt AC, Gross WL, Reinhold-Keller E. Standardized neurologic evaluations of 128 patients with Wegener granulomatosis. Arch Neurol. 2001;58(8):1215–21.CrossRefPubMed
13.
Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116(6):488–98.CrossRefPubMed
14.
Nishino H, Rubino FA, DeRemee RA, Swanson JW, Parisi JE. Neurological involvement in Wegener's granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol. 1993;33(1):4–9.CrossRefPubMed
15.
Drachman DA. Neurological complications of Wegener's granulomatosis. Arch Neurol. 1963;8(2):145–55.CrossRef
16.
Seror R, Mahr A, Ramanoelina J, Pagnoux C, Cohen P, Guillevin L. Central nervous system involvement in Wegener granulomatosis. Medicine (Baltimore). 2006;85(1):54–65.CrossRef
17.
Yokoseki A, Saji E, Arakawa M, Kosaka T, Hokari M, Toyoshima Y, et al. Hypertrophic pachymeningitis: significance of myeloperoxidase anti-neutrophil cytoplasmic antibody. Brain. 2014;137(Pt 2):520–36.CrossRefPubMed
18.
Shinkawa Y, Hatachi S, Yagita M. Intravascular large B-cell lymphoma with a high titer of proteinase-3-anti-neutrophil cytoplasmic antibody mimicking granulomatosis with polyangiitis. Mod Rheumatol. 2016:1–3.
19.
De Parisot A, Puechal X, Langrand C, Raverot G, Gil H, Perard L, et al. Pituitary involvement in granulomatosis with polyangiitis: report of 9 patients and review of the literature. Medicine (Baltimore). 2015;94(16):e748.CrossRef
20.
Kapoor E, Cartin-Ceba R, Specks U, Leavitt J, Erickson B, Erickson D. Pituitary dysfunction in granulomatosis with polyangiitis: the Mayo Clinic experience. J Clin Endocrinol Metab. 2014;99(11):3988–94.CrossRefPubMed
21.
Katzman GL, Langford CA, Sneller MC, Koby M, Patronas NJ. Pituitary involvement by Wegener's granulomatosis: a report of two cases. AJNR Am J Neuroradiol. 1999;20(3):519–23.PubMed
22.
Eli IM, Raheja A, Corn HJ, Simmons DL, Palmer CA, Couldwell WT. Sellar Wegener granulomatosis masquerading as Cabergoline-resistant Prolactinoma. World Neurosurg. 2016;95:622. e621-622 e625CrossRefPubMed
23.
De Groot K, Rasmussen N, Bacon PA, Tervaert JW, Feighery C, Gregorini G, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52(8):2461–9.CrossRefPubMed
24.
Faurschou M, Westman K, Rasmussen N, de Groot K, Flossmann O, Hoglund P, et al. Brief report: long-term outcome of a randomized clinical trial comparing methotrexate to cyclophosphamide for remission induction in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2012;64(10):3472–7.CrossRefPubMed
25.
Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363(3):221–32.CrossRefPubMedPubMedCentral
26.
Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010;363(3):211–20.CrossRefPubMed
27.
Martinez Del Pero M, Chaudhry A, Jones RB, Sivasothy P, Jani P, Jayne D. B-cell depletion with rituximab for refractory head and neck Wegener's granulomatosis: a cohort study. Clin Otolaryngol. 2009;34(4):328–35.CrossRefPubMed
28.
Guerry MJ, Brogan P, Bruce IN, D'Cruz DP, Harper L, Luqmani R, et al. Recommendations for the use of rituximab in anti-neutrophil cytoplasm antibody-associated vasculitis. Rheumatology (Oxford). 2012;51(4):634–43.CrossRef
29.
Haynes BF, Fauci AS. Diabetes insipidus associated with Wegener's granulomatosis successfully treated with cyclophosphamide. N Engl J Med. 1978;299(14):764.CrossRefPubMed
30.
31.
Lohr KM, Ryan LM, Toohill RJ, Anderson T. Anterior pituitary involvement in Wegener's granulomatosis. J Rheumatol. 1988;15(5):855–7.PubMed
32.
Rosete A, Cabral AR, Kraus A, Alarcon-Segovia D. Diabetes insipidus secondary to Wegener's granulomatosis: report and review of the literature. J Rheumatol. 1991;18(5):761–5.PubMed
33.
Czarnecki EJ, Spickler EM. MR demonstration of Wegener granulomatosis of the infundibulum, a cause of diabetes insipidus. AJNR Am J Neuroradiol. 1995;16(4 Suppl):968–70.PubMed
34.
Roberts GA, Eren E, Sinclair H, Pelling M, Burns A, Bradford R, et al. Two cases of Wegener's granulomatosis involving the pituitary. Clin Endocrinol. 1995;42(3):323–8.CrossRef
35.
Bertken RD, Cooper VR. Wegener granulomatosis causing sellar mass, hydrocephalus, and global pituitary failure. West J Med. 1997;167(1):44–7.PubMedPubMedCentral
36.
Hajj-Ali RA, Uthman IW, Salti IA, Zaatari GS, Haddad MC, Nasr FW. Wegener's granulomatosis and diabetes insipidus. Rheumatology (Oxford). 1999;38(7):684–5.CrossRef
37.
Miesen WM, Janssens EN, van Bommel EF. Diabetes insipidus as the presenting symptom of Wegener's granulomatosis. Nephrol Dial Transplant. 1999;14(2):426–9.CrossRefPubMed
38.
Goyal M, Kucharczyk W, Keystone E. Granulomatous hypophysitis due to Wegener's granulomatosis. AJNR Am J Neuroradiol. 2000;21(8):1466–9.PubMed
39.
Tappouni R, Burns A. Pituitary involvement in Wegener's granulomatosis. Nephrol Dial Transplant. 2000;15(12):2057–8.CrossRefPubMed
40.
Woywodt A, Knoblauch H, Kettritz R, Schneider W, Gobel U. Sudden death and Wegener's granulomatosis of the pituitary. Scand J Rheumatol. 2000;29(4):264–6.CrossRefPubMed
41.
Garovic VD, Clarke BL, Chilson TS, Specks U. Diabetes insipidus and anterior pituitary insufficiency as presenting features of Wegener's granulomatosis. Am J Kidney Dis. 2001;37(1):E5.CrossRefPubMed
42.
Tao J, Dong Y. Pituitary involvement in Wegener's granulomatosis: a case report and review of the literature. Chin Med J. 2003;116(11):1785–8.PubMed
43.
Muir BM, Hulett RL, Zorn JG. Wegener's granulomatosis complicated by central diabetes insipidus in a pediatric patient. AJR Am J Roentgenol. 2004;182(6):1560–2.CrossRefPubMed
44.
Vittaz L, Ramanoelina J, Mahr A, Cohen R, Cohen P, Reach G, et al. Pituitary involvement in Wegener's granulomatosis. Two cases. Presse Med. 2004;33(22):1585–90.CrossRefPubMed
45.
Duzgun N, Morris Y, Gullu S, Gursoy A, Ensari A, Kumbasar OO, et al. Diabetes insipidus presentation before renal and pulmonary features in a patient with Wegener's granulomatosis. Rheumatol Int. 2005;26(1):80–2.CrossRefPubMed
46.
Spisek R, Kolouchova E, Jensovsky J, Rusina R, Fendrych P, Plas J, et al. Combined CNS and pituitary involvement as a primary manifestation of Wegener granulomatosis. Clin Rheumatol. 2006;25(5):739–42.CrossRefPubMed
47.
48.
Thiryayi W, Donaldson MH, Border D, Tyagi A. An enhancing pituitary lesion in a young woman: a diagnostic dilemma. J Clin Neurosci. 2007;14(3):286–8.CrossRefPubMed
49.
Yong TY, Li JY, Amato L, Mahadevan K, Phillips PJ, Coates PS, et al. Pituitary involvement in Wegener's granulomatosis. Pituitary. 2008;11(1):77–84.CrossRefPubMed
50.
Cunnington JR, Jois R, Zammit I, Scott D, Isaacs J. Diabetes insipidus as a complication of Wegener's granulomatosis and its treatment with biologic agents. Int J Rheumatol. 2009;2009:346136.CrossRefPubMedPubMedCentral
51.
Xue J, Wang H, Wu H, Jin Q. Wegener's granulomatosis complicated by central diabetes insipidus and peripheral neutrophy with normal pituitary in a patient. Rheumatol Int. 2009;29(10):1213–7.CrossRefPubMed
52.
Barlas NB, Hassan HH, Al Badr FB, Bilal A. Structural and functional involvement of pituitary gland in Wegener's granulomatosis. Clin Neuroradiol. 2011;21(1):31–3.CrossRefPubMed
53.
Santoro SG, Guida AH, Furioso AE, Glikman P, Rogozinski AS. Panhypopituitarism due to Wegener's granulomatosis. Arq Bras Endocrinol Metabol. 2011;55(7):481–5.CrossRefPubMed
54.
Tenorio Jimenez C, Montalvo Valdivieso A, Lopez Gallardo G, McGowan B. Pituitary involvement in Wegener's granulomatosis: unusual biochemical findings and severe malnutrition. BMJ Case Rep. 2011;2011
55.
Hughes J, Barkhoudarian G, Ciarlini P, Laws ER, Mody E, Inzucchi SE, et al. Refractory pituitary granulomatosis with polyangiitis (Wegener's) treated with rituximab. Endocr Pract. 2013;19(1):e1–7.CrossRefPubMed
56.
Pereira EA, Plaha P, Hofer M, Karavitaki N, Cudlip SA. Hypophyseal Wegener's granulomatosis presenting by visual field constriction without hypopituitarism. Clin Neurol Neurosurg. 2013;115(6):762–4.CrossRefPubMed
57.
Esposito D, Trimpou P, Giugliano D, Dehlin M, Ragnarsson O. Pituitary dysfunction in granulomatosis with polyangiitis. Pituitary. 2017;20(5):594–601.CrossRefPubMedPubMedCentral
Metadata
Title
Severe localised granulomatosis with polyangiitis (Wegener’s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review
Authors
James E. Peters
Vivek Gupta
Ibtisam T. Saeed
Curtis Offiah
Ali S. M. Jawad
Publication date
01-12-2018
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2018
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/s12883-018-1058-8

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