Advertisement

Pituitary

, Volume 10, Issue 1, pp 107–111 | Cite as

Fatal inflammatory hypophysitis

  • Elizabeth A. McIntyre
  • Petros PerrosEmail author
Case Report

Abstract

A young female patient presented as an acute medical emergency with hypoglycaemia. Investigations revealed panhypopituitarism and an inflammatory pituitary mass. An antibody screen was negative for anti-neutrophil cytoplasmic antibodies with cytoplasmic distribution (cANCA). Pituitary histology showed lymphocytic infiltration and a few Langerhan’s cells. The pituitary mass rapidly expanded to involve the optic nerves and led to bilateral blindness. Later, the patient developed diarrhoea, a vasculitis rash, scleritis, and proteinuria. In subsequent investigations cANCA became positive. The patient responded to steroids and cyclophosphamide treatment and remained in partial remission for six months before dying of severe sepsis. This is the first description of Wegener’s granulomatosis presenting with acute anterior pituitary failure in the absence of other organ involvement and negative serology.

Keywords

Pituitary mass Wegener’s granulomatosis Hypophysitis 

References

  1. 1.
    Sam S, Molitch ME (2005) The pituitary mass: diagnosis and management. Rev Endocr Metab Disord 6:55–62PubMedCrossRefGoogle Scholar
  2. 2.
    Cheung CC, Ezzat S, Smyth HS, Asa SL (2001) The spectrum and significance of primary hypophysitis. J Clin Endocrinol Metab 86:1048–1053PubMedCrossRefGoogle Scholar
  3. 3.
    Rodriguez-Pereira C, Borras-Moreno JM, Pesudo-Martinez JV, Vera-Roman JM (2005) Cerebral solitary Langerhans cell histiocytosis: report of two cases and review of the literature. Br J Neurosurg 19:192–197PubMedGoogle Scholar
  4. 4.
    Caturegli P, Newschaffer C, Olivi A, Pomper MG, Burger PC, Rose NR (2005) Autoimmune hypophysitis. Endocr Rev 26:599–614PubMedCrossRefGoogle Scholar
  5. 5.
    Laws ER, Vance ML, Jane JA Jr (2006) Hypophysitis. Pituitary 9:331–333PubMedCrossRefGoogle Scholar
  6. 6.
    Sautner D, Saeger W, Lüdecke DK, Jansen V, Puchner MJA (1995) Hypophysitis in surgical and autoptical specimens. Acta Neuropathol (Berl) 90:637–644Google Scholar
  7. 7.
    Lury KM (2005) Inflammatory and infectious processes involving the pituitary gland. Top Magn Reson Imaging 16:301–306PubMedCrossRefGoogle Scholar
  8. 8.
    Burt MG, Morey AL, Turner JJ, Pell M, Sheehy JP, Ho KK (2003) Xanthomatous pituitary lesions: a report of two cases and review of the literature. Pituitary 6:161–168PubMedCrossRefGoogle Scholar
  9. 9.
    Roncaroli F, Bacci A, Frank G, Calbucci F (1998) Granulomatous hypophysitis caused by a ruptured intrasellar Rathke’s cleft cyst: report of a case and review of the literature. Neurosurgery 43:146–149PubMedCrossRefGoogle Scholar
  10. 10.
    Toth M, Szabo P, Racz K, Szende B, Balogh I, Czirjak S, Slowik F, Glaz E (1996) Granulomatous hypophysitis associated with Takayasu’s disease. Clin Endocrinol 45:499–503CrossRefGoogle Scholar
  11. 11.
    de Bruin WI, van’t Verlaat JW, Graamans K, de Bruin TW (1991) Sellar granulomatous mass in a pregnant woman with active Crohn’s disease. Neth J Med 39:136–141PubMedGoogle Scholar
  12. 12.
    Slot MC, Tervaert JW (2004) Wegener’s granulomatosis. Autoimmunity 37:313–315PubMedCrossRefGoogle Scholar
  13. 13.
    Seror R, Mahr A, Ramanoelina J, Pagnoux C, Cohen P, Guillevin L (2006) Central nervous system involvement in Wegener granulomatosis. Medicine (Baltimore) 85:54–65Google Scholar
  14. 14.
    Miesen WM, Janssens EN, van Bommel EF (1999) Diabetes insipidus as the presenting symptom of Wegener’s granulomatosis. Nephrol Dialys Transplant 14:426–429CrossRefGoogle Scholar
  15. 15.
    Tappouni R, Burns A (2000) Pituitary involvement in Wegener’s granulomatosis. Nephrol Dialys Transplant 15:2057–2058CrossRefGoogle Scholar
  16. 16.
    Goyal M, Kucharczyk W, Keystone E (2000) Granulomatous hypophysitis due to Wegener’s granulomatosis. Am J Neuroradiol 21:1466–1469PubMedGoogle Scholar
  17. 17.
    Garovic VD, Clarke BL, Chilson TS, Specks U (2001) Diabetes insipidus and anterior pituitary insufficiency as presenting features of Wegener’s granulomatosis. Am J Kidn Dis 37:E5Google Scholar
  18. 18.
    Duzgun N, Morris Y, Gullu S, Gursoy A, Ensari A, Kumbasar OO, Duman M (2005) Diabetes insipidus presentation before renal and pulmonary features in a patient with Wegener’s granulomatosis. Rheumatol Internat 26:80–82CrossRefGoogle Scholar
  19. 19.
    Batsakis JG, Luna MA (1987) Midfacial necrotizing lesions. Semin Diagn Pathol 4:90–116PubMedGoogle Scholar
  20. 20.
    Stone JH (2003) Limited versus severe Wegener’s granulomatosis: baseline data on patients in the Wegener’s granulomatosis etanercept trial. Arthr Rheumat 48:2299–2309CrossRefGoogle Scholar
  21. 21.
    Bosch X, Guilabert A, Font J (2006) Antineutrophil cytoplasmic antibodies. Lancet 368:404–418PubMedCrossRefGoogle Scholar
  22. 22.
    Haynes BF, Fauci AS (1978) Diabetes insipidus associated with Wegener’s granulomatosis successfully treated with cyclophosphamide. N Engl J Med 299:764PubMedCrossRefGoogle Scholar
  23. 23.
    Murphy JM, Gomez-Anson B, Gillard JH, Antoun NM, Cross J, Elliot JD, Lockwood M (1999) Wegener granulomatosis: MR imaging findings in brain and meninges. Radiology 213:794–799PubMedGoogle Scholar
  24. 24.
    Kallenberg CGM, Mulder AHL, Tervaert JWC (1992) Antineutrophil cytoplasmic antibodies: a still-growing class of autoantibodies in inflammatory disorders. Am J Med 93:675–681PubMedCrossRefGoogle Scholar
  25. 25.
    George J, Levy Y, Kallenberg CGM, Schoenfeld Y (1997) Infections and Wegener’s granulomatosis-a cause and effect relationship? Q J Med 90:367–373Google Scholar
  26. 26.
    Katzman GL, Langford CA, Sneller MC, Koby M, Patronas NJ (1999) Pituitary involvement by Wegener’s granulomatosis: a report of two cases. AJNR Am J Neuroradiol 20:519–23PubMedGoogle Scholar
  27. 27.
    Goyal M, Kucharczyk W, Keystone E (2000) Granulomatous hypophysitis due to Wegener’s granulomatosis. AJNR Am J Neuroradiol 21:1466–1499PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2007

Authors and Affiliations

  1. 1.Endocrine UnitNewcastle Hospitals NHS Trust, Freeman HospitalNewcastle upon TyneUK

Personalised recommendations