Granulomatosis with polyangiitis manifested as diabetes insipidus

Authors: Ondřej Pátek 1;  Miroslava Horáčková 1;  Lenka Vítová 1;  Rudolf Horváth 2;  Jaromír Háček 3;  Otto Schück 1
Authors‘ workplace: Interní klinika 2. LF UK a FN v Motole, Praha 1;  Oddělení revmatologie dětí a dospělých FN v Motole, Praha 2;  Ústav patologie a molekulární medicíny 2. LF UK a FN v Motole, Praha 3
Published in: Vnitř Lék 2016; 62(7-8): 679-683
Category: Case Reports


The case report shows a surprising presentation of pulmonary granulomatosis with polyangiitis (GPA) through symptoms of diabetes insipidus (DI) with granulomatous infiltration of the pituitary gland. The pituitary hormonal dysfunction as a result of granulomatosis of the pituitary gland is rare. Several studies have demonstrated that the incidence of the pituitary dysfunction reaches approx. 1 % of the patients with GPA. However it is mostly presented in patients with the disease already diagnosed. The patient described by us had no clinical expressions of GPA in the respiratory tract. He presented with polyuria and polydipsia. It was not until a more detailed examination of these symptoms was performed that a focal lung disease was detected and diagnosed as GPA.

Key words:
diabetes insipidus – granulomatosis with polyangiitis – granulomatous infiltration of the pituitary gland – pituitary hormonal dysfunction


1. Sampei S, Watanabe R, Ishii T et al. Granulomatosis with polyangiitis preceded by central diabetes insipidus. Intern Med 2014; 53(15): 1725–1726.

2. Slabu H, Arnason T. Pituitary granulomatosis with polyangiitis. BMJ Case Rep 2013; 2013. pii: bcr2013008656. Dostupné z DOI: <–008656>.

3. Ball GV, Bridges SL. Vasculitis. Oxford University Press: Oxford 2002. ISBN 978–0192630537.

4. De Parisot A, Puéchal X, Langrand C et al. Pituitary involvement in granulomatosis with polyangiitis: report of 9 patients and review of the literature. Medicine (Baltimore) 2015; 94(16): e748. Dostupné z DOI: <>.

5. Miesen WM, Janssens EN, van Bommel EF. Diabetes insipidus as the presenting symptom of Wegener’s granulomatosis. Nephrol Dial Transplant. 1999; 14(2): 426–429.

6. Garovic VD, Clarke BL, Chilson TS et al. Diabetes insipidus and anterior pituitary insufficiency as presenting features of Wegener’s granulomatosis. Am J Kidney Dis 2001; 37(1): E5.

7. Al-Fakhouri A, Manadan A, Gan J et al. Central diabetes insipidus as the presenting symptom of granulomatosis with polyangiitis. J Clin Rheumatol. 2014; 20(3): 151–154.

8. Yong TY, Li JY, Amato L et al. Pituitary involvement in Wegener’s granulomatosis. Pituitary 2008; 11(1): 77–84.

9. Kapoor E, Cartin-Ceba R, Specks U et al. Pituitary dysfunction in granulomatosis with polyangiitis: the Mayo Clinic experience. J Clin Endocrinol Metab 2014; 99(11): 3988–3994.

10. Düzgün N, Morris Y, Güllü S et al. Diabetes insipidus presentation before renal and pulmonary features in a patient with Wegener’s granulomatosis. Rheumatol Int 2005; 26(1): 80–82.

11. 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): 4S968–4S970.

12. Tenorio Jimenez C, Montalvo Valdivieso A, López Gallardo G et al. Pituitary involvement in Wegener’s granulomatosis: unusual biochemical findings and severe malnutrition. BMJ Case Rep 2011; 2011. pii: bcr0220113850.Dostupné z DOI: <>.

13. Spísek R, Kolouchová E, Jensovský J et al. Combined CNS and pituitary involvement as a primary manifestation of Wegener granulomatosis. Clin Rheumatol 2006; 25(5): 739–742.

14. Tappouni R, Burns A. Pituitary involvement in Wegener’s granulomatosis. Nephrol Dial Transplant 2000; 15(12): 2057–2058.

15. Hoffman GS, Kerr GS, Leavitt RY et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992; 116(6): 488–498.

16. Fujisawa I. Magnetic resonance imaging of the hypothalamic-neurohypophyseal system. J Neuroendocrinol 2004; 16(4): 297–302.

Diabetology Endocrinology Internal medicine

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