Clinical Characteristics of Primary Hypophysitis – A Single-Centre Series of 60 Cases

Abstract

Objective Clinical data on primary hypophysitis are still scarce. Especially non-surgical cases are underreported. We sought to analyse clinical characteristics of primary hypophysitis, particularly in clinically diagnosed patients.

Design Retrospective single centre study in 60 patients with primary hypophysitis.

Methods Symptoms, MRI, histopathological findings, treatment and outcomes were analysed in 12 histopathologically and 48 clinically diagnosed patients. Diagnostic criteria for clinical diagnosis were: a) MRI findings compatible with primary hypophysitis; b) course of disease excluding other differential diagnoses. Mean duration of follow-up was 69 months.

Results Female sex was predominant (73%). Fatigue (52%), headache (38%) and diabetes insipidus (38%) were the most frequent symptoms. 42% had a concomitant autoimmune disease. The corticotropic, thyrotropic, gonadotropic, somatotropic axis was impaired in 67%, 57%, 52%, 20%, respectively. Men had a higher number of impaired hormone axes (p=0.022) with the gonadotropic axis being affected more frequently in men (p=0.001). Infundibular thickening (56%) and space occupying lesions (46%) were typical MRI findings. Pituitary size was frequently enlarged at presentation (37%) but diminished during observation (p=0.029). Histopathologically and clinically diagnosed cases did not differ.

Conclusions The cohort of clinically diagnosed patients did not differ from our histopathologically diagnosed patients or from published cohorts with predominantly surgical patients. Thus, diagnosis of primary hypophysitis using clinical criteria seems feasible.

Key words

autoimmune hypophysitis - hypopituitarism - insufficiencies - diabetes insipidus - autoimmunity

Publikationsverlauf

Eingereicht: 24. Februar 2020
Eingereicht: 02. April 2020

Angenommen: 22. April 2020

Artikel online veröffentlicht:
07. August 2020

• References

• 1 Honegger J, Schlaffer S, Menzel C. et al. Diagnosis of primary hypophysitis in Germany. J Clin Endocrinol Metab 2015; 100: 3841-3849
• 2 Thoda P, Pappa D, Sakali A-K. et al. IgG4 related hypophysitis. Endocrine Abstracts. 19th European Congress of Endocrinology, Lisbon, Portugal. BioScientifica. 2017
• 3 Leporati P, Landek-Salgado MA, Lupi I. et al. IgG4-related hypophysitis: A new addition to the hypophysitis spectrum. J Clin Endocrinol Metab 2011; 96: 1971-1980
• 4 Wong S, Lam WY, Wong WK. et al. Hypophysitis presented as inflammatory pseudotumor in immunoglobulin G4-related systemic disease. Hum Pathol 2007; 38: 1720-1723
• 5 Shimatsu A, Oki Y, Fujisawa I. et al. Pituitary and Stalk Lesions (Infundibulo-hypophysitis) Associated with Immunoglobulin G4-related Systemic Disease: An Emerging Clinical Entity. Endocr J 2009; 56: 1033-1041
• 6 Gutenberg A, Buslei R, Fahlbusch R. et al. Immunopathology of primary hypophysitis. Am J Surg Pathol 2005; 29: 329-338
• 7 Honegger J, Fahlbusch R, Bornemann A. et al. Lymphocytic and granulomatous hypophysitis: Experience with nine cases. Neurosurgery 1997; 40: 713-723
• 8 Yabe S, Murakami M, Maruyama K. et al. Western blot analysis of rat pituitary antigens recognized by human antipituitary antibodies. Endocr J 1995; 42: 115-119
• 9 Kikuchi T, Yabe S, Kanda T. et al. Antipituitary antibodies as pathogenetic factors in patients with pituitary disorders. Endocr J 2000; 47: 407-416
• 10 Takao T, Nanamiya W, Matsumoto R. et al. Antipituitary antibodies in patients with lymphocytic hypophysitis. Horm Res 2001; 55: 288-292
• 11 Crock PA. Cytosolic autoantigens in lymphocytic hypophysitis. J Clin Endocrinol Metab 1998; 83: 609-618
• 12 O’Dwyer DT, Smith AI, Matthew ML. et al. Identification of the 49-kDa autoantigen associated with lymphocytic hypophysitis as alpha-enolase. J Clin Endocrinol Metab 2002; 87: 752-757
• 13 Tanaka S, Tatsumi K-I, Takano T. et al. Anti-alpha-enolase antibodies in pituitary disease. Endocr J 2003; 50: 697-702
• 14 Leung GKK, Lopes M-BS, Thorner MO. et al. Primary hypophysitis: A single-center experience in 16 cases. J Neurosurg 2004; 101: 262-271
• 15 Chiloiro S, Tartaglione T, Angelini F. et al. An overview of diagnosis of primary autoimmune hypophysitis in a prospective single-center experience. Neuroendocrinology 2017; 104: 280-290
• 16 Angelousi A, Cohen C, Sosa S. et al. Clinical, endocrine and imaging characteristics of patients with primary hypophysitis. Horm Metab Res 2018; 50: 296-302
• 17 Khare S, Jagtap VS, Budyal SR. et al. Primary (autoimmune) hypophysitis: a single centre experience. Pituitary 2013; 18: 1-7
• 18 Gutenberg A, Hans V, Puchner MJ. et al. Primary hypophysitis: Clinical-pathological correlations. Eur J Endocrinol 2006; 155: 101-107
• 19 Wang S, Wang L, Yao Y. et al. Primary lymphocytic hypophysitis: Clinical characteristics and treatment of 50 cases in a single centre in China over 18 years. Clin Endocrinol (Oxf) 2017; 87: 177-184
• 20 Gutenberg A, Larsen J, Lupi I. et al. A radiologic score to distinguish autoimmune hypophysitis from nonsecreting pituitary adenoma preoperatively. Am J Neuroradiol 2009; 30: 1766-1772
• 21 Bidlingmaier M, Friedrich N, Emeny RT. et al. Reference intervals for insulin-like growth factor-1 (IGF-I) from birth to senescence: Results from a multicenter study using a new automated chemiluminescence IGF-I immunoassay conforming to recent international recommendations. J Clin Endocrinol Metab 2014; 99: 1712-1721
• 22 Ho KKY. Consensus guidelines for the diagnosis and treatment of adults with GH deficiency II: A statement of the GH Research Society in association with the European Society for Pediatric Endocrinology, Lawson Wilkins Society, European Society of Endocrinology, J. Eur J Endocrinol 2007; 157: 695-700
• 23 Statistisches Bundesamt Körpermaße der Bevölkerung - Mikrozensus Statistisches Bundesamt. Wiesbaden. 2009
• 24 Imber BS, Lee HS, Kunwar S. et al. Hypophysitis: A single-center case series. Pituitary 2015; 18: 630-641
• 25 Ezzat S, Josse RG. Autoimmune hypophysitis. Trends Endocrinol Metab 1997; 8: 74-80
• 26 Caturegli P, Newschaffer C, Olivi A. et al. Autoimmune hypophysitis. Endocr Rev 2005; 26: 599-614
• 27 Buxton N, Robertson I. Lymphocytic and granulocytic hypophysitis: A single centre experience. Br J Neurosurg 2001; 15: 242-246
• 28 Howlett T, Levy MJ, Robertson IJ. How reliably can autoimmune hypophysitis be diagnosed without pituitary biopsy. Clin Endocrinol (Oxf) 2010; 73: 18-21
• 29 Freda PU, Post KD. Differential diagnosis of sellar masses. Endocrinol Metab Clin North Am 1999; 28: 81-117
• 30 Honegger J, Buchfelder M, Schlaffer S. et al. Treatment of primary hypophysitis in Germany. J Clin Endocrinol Metab 2015; 100: 3460-3469