Skip to main content
Download PDF

Granulomatosis with polyangiitis in a patient with polydipsia, facial nerve paralysis, and severe otologic complaints: a case report and review of the literature

Journal of Medical Case Reports volume 16, Article number: 291 (2022) Cite this article

Abstract

Background

Granulomatosis with polyangiitis, formerly known as Wegener granulomatosis, is a necrotizing vasculitis with granulomatous inflammation that belongs to the class of antineutrophil cytoplasmic antibodies-positive diseases. It occurs in a localized and a systemic form and may present with a variety of symptoms. Involvement of the upper respiratory tract is very common, while neurologic, endocrinological, and nephrological dysfunction may occur.

Case presentation

We describe the case of a 29-year-old Central European male patient presenting with severe bilateral sensorineural hearing loss, otorrhea, and one-sided facial nerve paralysis. The patient was unsuccessfully treated with i.v. antibiotics at another hospital in Berlin, and tympanic tubes were inserted. After presentation to our emergency room, he was hospitalized and further diagnostics started. Increased fluid intake and 12 kg weight gain over the last months were reported. The patient was diagnosed with granulomatosis with polyangiitis and diabetes insipidus. The patient’s condition improved after treatment with rituximab.

Discussion

A comprehensive PubMed search of all articles with granulomatosis with polyangiitis and diabetes insipidus was conducted to assess which combination of symptoms occurs simultaneously and whether other parts of the pituitary are commonly involved. The 39 selected articles, describing 61 patients, showed that ear–nose–throat involvement occurred most commonly, in 71% of cases. Of patients, 59% had involvement of the anterior pituitary gland, while true panhypopituitarism occurred in 13% of cases. Only one case report featured the same set of symptoms as described herein.

Conclusion

Granulomatosis with polyangiitis is a highly variable disease, commonly involving the upper airways, but that may present with symptoms solely related to the pituitary gland. Clinicians should have a low threshold to investigate for granulomatosis with polyangiitis in patients with therapy-resistant otorrhea. Patients may present with a complex set of symptoms, and integrating different specialists when additional symptoms occur may lead to faster diagnosis.

Peer Review reports

Background

Granulomatosis with polyangiitis (GPA) is a disorder formerly known as Wegener granulomatosis. Its hallmark is necrotizing vasculitis with granulomatous inflammation. It involves primarily small and medium-sized vessels with a predilection for the upper and lower airways. Necrotizing glomerulonephritis is common [1]. The current definition was formed in 2012 by the Chapel Hill Conference for Consensus Criteria [2].

GPA belongs to a group of anti-neutrophil cytoplasmic antibody (ANCA)-positive diseases called ANCA-associated vasculitis, although ANCA testing may be negative in 10–20% of cases [3].

Research led to the identification of different subsets of ANCA-positive diseases. Anti-proteinase 3 activity (PR3-ANCA) is most common in GPA, while patients with microscopic polyangiitis and Churg–Strauss syndrome are most often anti-myeloperoxidase ANCA (MPO-ANCA) positive [4].

GPA occurs in a systemic and a localized form. ENT involvement occurs in up to 90% of individuals. The sinonasal area is most commonly affected [5,6,7]. Otologic involvement is the second most common presentation [8, 9]. Localized forms of GPA with involvement of the ear and/or the upper respiratory tract have been described, especially in younger patients [10]. Dysfunction of the central nervous system is common at some point of the disease; the result can be dysfunction of cranial nerves II, VI, and VII, but also endocrine involvement of the pituitary gland [11,12,13,14]. Severe headache is the most common nonendocrine symptom of hypophysitis. Chiasmal syndrome and ocular paresis may occur due to the increased sellar mass. Isolated DI centralis may occur as an endocrine symptom and is characterized by deficient secretion of arginine vasopressin (AVP), leading to clinical symptoms of polyuria and polydipsia. More frequently, combined hormonal insufficiency of the anterior and posterior pituitary occurs. Hypogonadotropic hypogonadism, secondary hypothyroidism, and potentially life-threatening secondary adrenal insufficiency are the most frequent endocrine disorders of the anterior pituitary, at least in patients with primary hypophysitis, in contrast to the finding of vulnerable gonadal and growth hormone axis and more robust adrenal and thyroid axes in patients with pituitary adenoma [15]. However, data on hypophyseal function of patients with secondary hypophysitis due to GPA are scarce.

We present the case of a patient with localized, severe granulomatosis with polyangiitis with otitis media, facial nerve palsy, and pituitary dysfunction resulting in DI.

This case is presented according to the CARE guidelines [16].

Additionally, a comprehensive systematic review of existing literature was performed to examine how often and to what extent hypophyseal dysfunction and ENT involvement occur in patients with GPA.

Case presentation

The patient is a white German male of 29 years of age who presented with otalgia and serous otorrhea (Figs. 1, 2) to the otolaryngology resident on call in the ER of the Charité University Hospital in Berlin in November 2018. The patient reported having had these symptoms for 1 month. He had visited an otolaryngology clinic in October 2018 with facial nerve palsy with House–Brackmann score (HB) < III. At the clinic, treatment was started with i.v. antibiotics and prednisolone as well as tympanocentesis with insertion of T-tubes. Audiometric analysis revealed combined sensorineural and conductive hearing loss on the right side and conductive hearing loss on the left side (Fig. 3). A computed tomography (CT) scan report on his temporal bone described fluid retention on the mastoid on both sides, possibly with osteolysis. Routine laboratory analysis including human immunodeficiency virus and hepatitis showed slightly elevated C-reactive protein (34.9 mg/l) but no further anomalies. On microbiologic testing, the middle ear fluid revealed biological flora without detection of pathogens.

Fig. 1
figure 1

Left ear of the patient 1 day after presentation to our clinic with visible serous otorrhea after insertion of Titan tubes

Full size image
Fig. 2
figure 2

Right ear of the patient 1 day after presentation to our clinic with visible serous otorrhea after insertion of Titan tubes

Full size image
Fig. 3
figure 3

Audiometric analysis on 26/11/2018, showing mixed hearing loss on the right side with Fletcher index of 80 dB and conductive hearing loss on the left side with Fletcher index of 40 dB

Full size image

Facial nerve function normalized and otorrhea decreased after receiving treatment. The patient was discharged after 7 days with middle ear tubes in situ.

Upon presentation to the ER, facial nerve function was still abnormal with HB score of II, and extensive serous otorrhea persisted. The middle ear ventilation tubes were in situ. The patient also reported increased fluid intake of 9 L per day and weight gain of 12 kg in 7 weeks.

Laboratory analysis showed positive c-ANCA at 87.0 U/ml with increased anti-PR3 activity in combination testing. Anti-MPO activity was negative.

On the basis of this testing, a diagnosis of granulomatosis with polyangiitis was established.

A magnetic resonance imaging (MRI) scan of the brain showed slight widening of the pituitaries infundibulum and unclear inhomogeneity between the anterior and posterior pituitary (Figs. 4, 5).

Fig. 4
figure 4

Coronal imaging of the hypophysis on a 3-T T1-weighted MRI image

Full size image
Fig. 5
figure 5

Saggital imaging of the hypophysis on a 3-T T1-weighted MRI image

Full size image

Clinical and laboratory evaluation of the anterior pituitary function did not indicate insufficiency of the somatotropic, gonadotropic, thyrotropic, or corticotropic axis. In addition, serum prolactin was within the reference range.

Plasma sodium concentration was 144 mmol/l with plasma osmolality of 300 mosm/kg. In a water deprivation test, urine osmolality increased insufficiently from 131 to 418 mosm/kg. Measurement of hypertonic saline-stimulated plasma copeptin (2.63 pmol/l) confirmed the diagnosis of DI.

Treatment for GPA was successfully started with 1 g rituximab i.v., and ANCA values decreased to 22.2 U/ml at 4 months after discharge and finally to 13.4 U/ml in October 2019 (Additional file 1).

Discussion

GPA is a variable disease that may present with a complex set of symptoms [11,12,13,14]. The patient in our case presented with otalgia, otorhea, facial nerve dysfunction, hearing loss, and polydipsia. Upon presentation to our department, the posterior pituitary gland function was already affected. While the resulting increase in fluid intake was not seen as a burden by the patient, medical history showed that symptoms of DI had existed before otologic involvement. Referral to specialists in endocrinology and rheumatology was sought after the diagnosis of GPA.

A comprehensive systematic PubMed search was performed using the search term ‘Wegener granulomatosis AND pituitary dysfunction OR granulomatosis with polyangiitis AND pituitary dysfunction OR Wegener granulomatosis AND diabetes insipidus OR granulomatosis with polyangiitis AND diabetes insipidus’. The article references were scanned for additional literature, resulting in a total of 65 articles. All case reports with confirmed diagnosis of GPA in addition to diabetes insipidus were included. Articles published in foreign language (other than English or German) and for which no full text was available were excluded. In total, 39 articles were selected for analysis after applying inclusion and exclusion criteria.

The 39 selected articles described 61 different cases for analysis (Table 1). ENT involvement in patients with GPA was very common, with 43 patients reported to have any ENT area affected (72%). Sinonasal disease was very common, with 21 patients affected (35%). Otologic complaints were less common, being reported in nine patients (15%). Two of the selected case series, representing a total of 17 patients, did not specify the involved subarea. This may result in underestimation of the total percentage of patients affected per subarea.

Table 1 Published articles with patients matching our inclusion criteria, with publication date and site of ENT involvement
Full size table

Of the 61 cases, 36 (59%) experienced at least one symptom of anterior pituitary dysfunction. Hyperprolactemia was the most common hormonal dysbalance (n = 20, 33%), while hypothyroidism (n = 15, 25%) and hypogonadism (n = 14, 23%) ranked second and third. Panhypopituitarism was relatively uncommon and occurred in eight (13%) patients.

Most of the patients examined in this study had an established diagnosis of GPA before developing symptoms suggestive of DI (n = 33, 54%). The majority of patients (n = 35, 57%) developed ENT symptoms suggestive of GPA earlier than symptoms suggestive of DI. In 11 cases of this subgroup (31%), the ENT symptoms occurred days to months before DI symptoms. In 18 cases (51%), ENT symptoms developed years prior to diagnosis. Six cases (17%) did not include a timeframe for the occurrence of the symptoms.

DI was diagnosed before the diagnosis of GPA in 17 cases (28%). In this subgroup, the symptoms of DI developed days to month before diagnosis of GPA in three (18%) cases and years before diagnosis of GPA in another three (18%) cases. The other cases did not offer a timeframe (Table 2).

Table 2 Evaluation of pituitary function tests and latency of occurrence of symptoms per case
Full size table

Among the reviewed articles, only one report matched our patient’s complaints of therapy-resistant otitis media, facial nerve paralysis, sensorineural hearing loss, and DI [42]. Although GPA can occur as a very limited localized disease, e.g., as antibiotic-resistant otitis media with or without mastoiditis [51,52,53], we want to highlight that GPA can also occur with a combination of involved localized areas, as described herein. The current case, as well as the cases reviewed, highlight the complexity of this disease.

This review supports previous research and suggests that more than 70% of patients with GPA and DI have symptoms in the field of otolaryngology [6]. We found that ENT-related symptoms might occur more often before symptoms of DI. These results might explain why early involvement of ENT specialists was found to result in substantially increased survival [54].

In line with our findings, in many of the cases reviewed, patients experienced ENT symptoms at least 1 year before symptoms of DI occurred. Sinonasal disease is the most common manifestation in the head and neck area, but other symptoms occur frequently. The otolaryngologist has to manage acute and chronic symptoms, so knowledge about the different forms of the disease is fundamental.

The specific enlargement of the pituitary infundibulum on MRI is the result of involvement of the stalk and the hypothalamus in patients with DI [55, 56]. These images allow for distinction from other pathologies affecting the gland. While panhypopituitarism occurred in only a few patients, our study suggests that anterior pituitary dysfunction is slightly more common than anticipated before [27, 29]. Even with these numbers, it may be possible that gland dysfunction is underdiagnosed due to the blunted response when treated with corticosteroids, which most patients with GPA receive during the course of their disease. Hyperprolactemia, hypothyroidism, and hypogonadism are the most common abnormalities in our study. Care by the attending physician in regard to possible dysfunction of pituitary gland function is important, especially because several patients developed DI years after diagnosis of GPA. If polydipsia and polyuria are present, tests for anterior pituitary gland function must be performed.

Conclusion

This case demonstrates a relatively rare occurrence of DI in a patient with GPA and demonstrates the difficulty of diagnosing the disease. All healthcare professionals involved in the diagnostic process of the disease must have knowledge about its possible variable course. This is especially important since delayed diagnosis can lead to significant morbidity and possibly mortality, while appropriate treatment options exist [57].

Availability of data and materials

All data generated or analyzed during this study are included in this published article and its additional information files.

Abbreviations

GPA:

Granulomatosis with polyangiitis

ANCA:

Antineutrophil cytoplasmic antibodies

ENT:

Ear–nose–throat

PR3-ANCA:

Anti-proteinase 3 antineutrophil cytoplasmic antibodies

MPO-ANCA:

Anti-myeloperoxidase antineutrophil cytoplasmic antibodies

AVP:

Arginine vasopressin

ER:

Emergency room

HB:

House–Brackmann score

CT:

Computed tomography

IGF:

Insulin-like growth factor

LH:

Luteinizing hormone

FSH:

Follicle-stimulating hormone

T3:

Triiodothyronine

T4:

Thyroxine

ACTH:

Adrenocorticotropin

DI:

Diabetes insipidus

References

  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.

    Article  CAS  PubMed  Google Scholar 

  2. Jennette JC. Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Clin Exp Nephrol. 2013;17(5):603–6.

    Article  PubMed  PubMed Central  Google Scholar 

  3. McCarthy E, Mustafa M, Watts M. ANCA-negative granulomatosis with polyangiitis: a difficult diagnosis. EJCRIM 2017;4. https://doi.org/10.12890/2017_000625.

  4. Jennette JC, Falk RJ. Pathogenesis of antineutrophil cytoplasmic autoantibody-mediated disease. Nat Rev Rheumatol. 2014;10:463.

    Article  CAS  PubMed  Google Scholar 

  5. Gubbels SP, Barkhuizen A, Hwang PH. Head and neck manifestations of Wegener’s granulomatosis. Otolaryngol Clin N Am. 2003;36(4):685–705.

    Article  Google Scholar 

  6. Cannady SB, Batra PS, Koening C, Lorenz RR, Citardi MJ, Langford C, Hoffman GS. Sinonasal Wegener granulomatosis: a single-institution experience with 120 cases. Laryngoscope. 2009;119(4):757–61.

    Article  PubMed  Google Scholar 

  7. McDonald TJ, DeRemee RA. Head and neck involvement in Wegener’s Granulomatosis (WG). In: Gross WL, editor. ANCA-associated vasculitides. Boston, MA: Springer US; 1993. p. 309–13. https://doi.org/10.1007/978-1-4757-9182-2_49.

    Chapter  Google Scholar 

  8. Ohtani I, Baba Y, Suzuki C, Sakuma H, Kano M. Temporal bone pathology in Wegener’s granulomatosis. Fukushima J Med Sci. 2000;46(1–2):31–9.

    Article  CAS  PubMed  Google Scholar 

  9. Santos F, Salviz M, Domond H, Nadol JB. Otopathology of vasculitis in granulomatosis with polyangitis. Otol Neurotol. 2015;36(10):1657–62.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Stone JH. Limited versus severe Wegener’s granulomatosis: baseline data on patients in the Wegener’s granulomatosis etanercept trial. Arthritis Rheum. 2003;48(8):2299–309.

    Article  PubMed  Google Scholar 

  11. De Parisot A, Puéchal 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. 2015;94(16):e748–e748.

    Article  PubMed  PubMed Central  Google Scholar 

  12. 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.

    Article  CAS  PubMed  Google Scholar 

  13. Esposito D, Trimpou P, Giugliano D, Dehlin M, Ragnarsson O. Pituitary dysfunction in granulomatosis with polyangiitis. Pituitary. 2017;20(5):594–601.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Byrne TN, Stone JH, Pillai SS, Rapalino O, Deshpande V. A 53-year-old man with diplopia, polydipsia, and polyuria. N Engl J Med. 2016;375(15):1469–80.

    Article  PubMed  Google Scholar 

  15. Honegger J, Schlaffer S, Menzel C, Droste M, Werner S, Elbelt U, et al. Endocrinology on behalf of the PWG of the GS of diagnosis of primary hypophysitis in Germany. J Clin Endocrinol Metab. 2015;100(10):3841–9.

    Article  CAS  PubMed  Google Scholar 

  16. Gagnier JJ, Kienle G, Altman DG, Moher D, Sox H, Riley D, et al. The CARE Guidelines: consensus-based clinical case reporting guideline development. Glob Adv Health Med. 2013;2(5):38–43.

    Article  PubMed  PubMed Central  Google Scholar 

  17. 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):e5.1-e5.3.

    Article  Google Scholar 

  18. 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.

    Article  CAS  Google Scholar 

  19. Katzman GL, Langford CA, Sneller MC, Koby M, Patronas NJ. Pituitary involvement by Wegener s granulomatosis: a report of two cases. Am J Neuroradiol. 1999;20(3):519–23.

    CAS  PubMed  PubMed Central  Google Scholar 

  20. Hajj-Ali RA, Uthman IW, Salti IA, Zaatari GS, Haddad MC, Nasr FW. Wegener’s granulomatosis and diabetes insipidus. Rheumatology. 1999;38(7):684–5.

    Article  CAS  PubMed  Google Scholar 

  21. Muir BM, Hulett RL, Zorn JG. wegener’s granulomatosis complicated by central diabetes insipidus in a pediatric patient. Am J Roentgenol. 2004;182(6):1560–2.

    Article  Google Scholar 

  22. Düzgün N, Morris Y, Güllü S, Gürsoy A, Ensari A, Kumbasar ÖÖ, et al. Diabetes insipidus presentation before renal and pulmonary features in a patient with Wegener’s granulomatosis. Rheumatol Int. 2005;26(1):80–2.

    Article  PubMed  Google Scholar 

  23. Hurst NP, Dunn NA, Chalmers TM. Wegeners granulomatosis complicated by diabetes insipidus. Ann Rheum Dis. 1983;42(5):600–1.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  24. Haynes BF, Fauci AS. Diabetes insipidus associated with Wegener’s granulomatosis successfully treated with cyclophosphamide. N Engl J Med. 1978;299(14):764.

    Article  CAS  PubMed  Google Scholar 

  25. Czarnecki EJ, Spickler EM. MR demonstration of Wegener granulomatosis of the infundibulum, a cause of diabetes insipidus. Am J Neuroradiol. 1995;16(4):968–70.

    CAS  PubMed  PubMed Central  Google Scholar 

  26. Bertken R, Cooper V. Wegener Granulomatosis causing sellar mass hydrocephalus, and global pituitary failure. West J Med. 1997;167:43–6.

    Google Scholar 

  27. 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.

    Article  CAS  PubMed  Google Scholar 

  28. Goyal M, Kucharczyk W, Keystone E. Granulomatous hypophysitis due to Wegeners granulomatosis. Am J Neuroradiol. 2000;21(8):1466–9.

    CAS  PubMed  PubMed Central  Google Scholar 

  29. Seror R, Mahr A, Ramanoelina J, Pagnoux C, Cohen P, Guillevin L. central nervous system involvement in Wegener granulomatosis. Medicine. 2006;85(1):53–65. https://doi.org/10.1097/01.md.0000200166.90373.41.

    Article  PubMed  Google Scholar 

  30. Špíšek R, Kolouchová E, Jenšovský 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.

    Article  PubMed  Google Scholar 

  31. McIntyre EA, Perros P. Fatal inflammatory hypophysitis. Pituitary. 2007;10(1):107–11.

    Article  PubMed  Google Scholar 

  32. Yong TY, Li JYZ, Amato L, Mahadevan K, Phillips PJ, Coates PS, et al. Pituitary involvement in Wegener’s granulomatosis. Pituitary. 2008;11(1):77–84.

    Article  PubMed  Google Scholar 

  33. 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;2009:1–4. https://doi.org/10.1155/2009/346136.

    Article  Google Scholar 

  34. 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.

    Article  PubMed  Google Scholar 

  35. 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.

    Article  CAS  PubMed  Google Scholar 

  36. Santoro SG, Guida AH, Furioso AE, Glikman P, Rogozinski AS. Panhypopituitarism due to Wegener’s granulomatosis. Arq Bras Endocrinol Metab. 2011;55:481–5.

    Article  Google Scholar 

  37. Tenorio Jimenez C, Valdivieso AM, Gallardo GL, Mcgowan B. Pituitary involvement in Wegener’s granulomatosis: unusual biochemical findings and severe malnutrition. BMJ Case Rep. 2011;2011:bcr0220113850.

    Article  PubMed  Google Scholar 

  38. Hughes J, Barkhoudarian G, Ciarlini P, Laws E, Mody E, Inzucchi S, et al. Refractory pituitary granulomatosis with polyangiitis (Wegener’s) treated with rituximab. Endocr Pract. 2012;19(1):e1-7.

    Article  Google Scholar 

  39. Pereira EAC, 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.

    Article  PubMed  Google Scholar 

  40. 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.

    Article  CAS  PubMed  Google Scholar 

  41. Peters JE, Gupta V, Saeed IT, Offiah C, Jawad ASM. Severe localised granulomatosis with polyangiitis (Wegener’s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review. BMC Neurol. 2018;18(1):59.

    Article  PubMed  PubMed Central  Google Scholar 

  42. Ohashi K, Morishita M, Watanabe H, Sada K-E, Katsuyama T, Miyawaki Y, et al. Central diabetes insipidus in refractory antineutrophil cytoplasmic antibody-associated vasculitis. Intern Med. 2017;56(21):2943–8.

    Article  PubMed  PubMed Central  Google Scholar 

  43. Xie Y, Shao H, Dai C, Ma Z. Diabetes insipidus induced by suspected eosinophilic granulomatous with polyangiitis. Postgrad Med J. 2017;93(1099):298–9.

    Article  PubMed  Google Scholar 

  44. 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.e1-622.e5.

    Article  Google Scholar 

  45. Vandergheynst F, Kazakou P, Couturier B, Dumarey N, Ghanooni R, Pozdzik A, et al. From polyuria to renal mass: an unexpected link. Am J Med. 2015;128(10):e15–8.

    Article  PubMed  Google Scholar 

  46. Bando H, Iguchi G, Fukuoka H, Taniguchi M, Kawano S, Saitoh M, et al. A diagnostic pitfall in IgG4-related hypophysitis: infiltration of IgG4-positive cells in the pituitary of granulomatosis with polyangiitis. Pituitary. 2015;18(5):722–30.

    Article  CAS  PubMed  Google Scholar 

  47. Sampei S, Watanabe R, Ishii T, Harigae H. Granulomatosis with polyangiitis preceded by central diabetes insipidus. Intern Med. 2014;53(15):1725–6.

    Article  PubMed  Google Scholar 

  48. Slabu H, Arnason T. Pituitary granulomatosis with polyangiitis. BMJ Case Rep. 2013;2013:bcr2013008656.

    Article  PubMed  PubMed Central  Google Scholar 

  49. van Durme CM, Kisters JM, van Paassen P, van Etten RW, Tervaert JWC. Multiple endocrine abnormalities. Lancet. 2011;378(9790):540.

    Article  PubMed  Google Scholar 

  50. Dutta P, HayatBhat M, Bhansali A, Bambery P, Kakar M. Wegener’s granulomatosis presenting as diabetes insipidus. Exp Clin Enocrinol Diabetes. 2006;114:533–6.

    Article  CAS  Google Scholar 

  51. Kukushev G, Kalinova D, Sheytanov I, Rashkov R. Two clinical cases of granulomatosis with polyangiitis with isolated otitis media and mastoiditis. Reumatologia. 2017;55(5):256–60.

    Article  PubMed  PubMed Central  Google Scholar 

  52. Lee JH, Kim KW, Myong NH, Jung JY. Wegener’s granulomatosis presenting as bilateral otalgia with facial palsy: a case report. Korean J Audiol. 2013;17(1):35–7.

    Article  PubMed  PubMed Central  Google Scholar 

  53. Wierzbicka M, Szyfter W, Puszczewicz M, Borucki L, Bartochowska A. Otologic symptoms as initial manifestation of Wegener granulomatosis: diagnostic dilemma. Otol Neurotol. 2011. https://doi.org/10.1097/MAO.0b013e31822558fd.

    Article  PubMed  Google Scholar 

  54. Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol. 2008;26:S94-104.

    CAS  PubMed  Google Scholar 

  55. Tien R, Kucharczyk J, Kucharczyk W. MR imaging of the brain in patients with diabetes insipidus. Am J Neuroradiol. 1990;12(3):533–42.

    Google Scholar 

  56. Higuchi M, Arita N, Mori S, Satoh B, Mori H, Hayakawa T. Pituitary granuloma and chronic inflammation of hypophysis : clinical and immunohistochemical studies. Acta Neurochir. 1993;121:152–8.

    Article  CAS  PubMed  Google Scholar 

  57. Gómez-Puerta JA, Hernández-Rodríguez J, López-Soto A, Bosch X. Antineutrophil cytoplasmic antibody-associated vasculitides and respiratory disease. Chest. 2009;136(4):1101–11.

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

Not applicable.

Funding

Open Access funding enabled and organized by Projekt DEAL. No funding received by the authors

Author information

Authors and Affiliations

  1. Department of Otolaryngology, Head and Neck Surgery, Charité-Universitätsmedizin Berlin, Mittelallee 2, 13353, Berlin, Germany

    Lukas Koenen, Heidi Olze, Sören Zappe & Steffen Dommerich

  2. Department of Medicine B-Hepatology, Gastroenterology, Endocrinology, Diabetes, Hematology, Oncology, Palliative Care, Brandenburg Medical School, Neuruppin, Germany

    Ulf Elbelt

Authors
  1. Lukas Koenen
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ulf Elbelt
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Heidi Olze
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Sören Zappe
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Steffen Dommerich
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

Conceptualization: LK, SD; Data curation: LK; Formal analysis: UE, LK; Investigation: SD, LK; Methodology: LK; Project administration: LK; Supervision: SD, HO, SZ; Validation: UE, LK; Visualization: LK; Writing of original draft: LK, UE; Writing, review and editing: SD, HO. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Lukas Koenen.

Ethics declarations

Ethics approval and consent to participate

Written consent was obtained from the subject mentioned in this case and is available upon request; ethics approval is not applicable to this type of study in Germany

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Competing interests

The authors declare that they have no competing interests

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary Information

Rights and permissions

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Koenen, L., Elbelt, U., Olze, H. et al. Granulomatosis with polyangiitis in a patient with polydipsia, facial nerve paralysis, and severe otologic complaints: a case report and review of the literature. J Med Case Reports 16, 291 (2022). https://doi.org/10.1186/s13256-022-03492-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1186/s13256-022-03492-7

Keywords

Journal of Medical Case Reports

ISSN: 1752-1947