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CC BY-NC-ND 4.0 · Journal of Diabetes and Endocrine Practice 2022; 05(01): 021-028
DOI: 10.1055/s-0042-1755931
Original Article

Diagnosis and Management of Cushing's Disease: A Survey of Endocrinologists from the Middle East and North Africa

Salem A. Beshyah 
1   Department of Endocrinology, Yas Clinic Khalifa City, Abu Dhabi, United Arab Emirates
2   Department of Medicine, Dubai Medical College, Dubai, United Arab Emirates
,
Mussa H. Almalki
3   Obesity, Endocrine and Metabolism Centre, King Fahad Medical City, Riyadh, Saudi Arabia
4   Department of Medicine, College of Medicine, King Fahad Medical City, King Saud bin Abdul Aziz University for Health Sciences, Riyadh, Saudi Arabia
,
Said Azzoug
5   Department of Endocrinology, Public Hospital Establishment, Ibn Ziri, Bologhine, Algiers, Algeria
,
Maya Barake 
6   Department of Endocrinology, Clemenceau Medical Center, Beirut, Lebanon
,
Khaled M. A. Al Dahmani
7   Department of Medicine, United Arab Emirates University, United Arab Emirates
8   Division of Endocrinology, Tawam Hospital, Al Ain, United Arab Emirates
,
Melika Chihaoui
9   Department of Endocrinology, La Rabta University Hospital, Faculty of Medicine of Tunis, University of Tunis-El Manar, Tunisia
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Abstract

Background Cushing's disease is the most prevalent cause of endogenous Cushing's syndrome. This study aimed to scope the current clinical practice pattern in managing Cushing's disease by endocrinologists in the Middle Eastern and North African (MENA) region.

Methods A questionnaire dealing with diagnosis, treatment, and follow-up of patients with Cushing's disease was adopted and sent electronically to a convenience sample of endocrinologists from the MENA region.

Results Out of 125 responses received, 88 were eligible for inclusion in the analysis. Most respondents selected the overnight dexamethasone suppression test (ONDST) and 24-hour urinary-free cortisol (UFC) as the best screening tests, 58 (66.7%) and 50 (57.5%) respectively, followed by midnight serum cortisol and midnight salivary cortisol. Measurement of serum adrenocorticotropic hormone (ACTH; 86.2%) and classic high-dose dexamethasone suppression test (40.2%) were selected for localization of the primary lesion. The primary choice of treatment was transsphenoidal pituitary surgery (98.8%). For the recurrence of Cushing's disease, medical therapy was the preferred modality followed by repeated pituitary surgery or bilateral adrenalectomy. In case of treatment failure following the first pituitary surgery and ketoconazole treatment, 36.9% selected pasireotide, while 32.1% chose bilateral adrenalectomy.

Conclusion ONDST and UFC are the two most common tests used to screen an index case with features of hypercortisolism. Pituitary surgery is the primary choice of treatment in Cushing's disease. However, medical treatment by ketoconazole is preferred for recurrent cases. Bilateral adrenalectomy is followed by pasireotide may be chosen in case of treatment failure after pituitary surgery and ketoconazole.

Keywords

clinical practice - Cushing's disease - diagnosis - management - MENA region

Authors' Contributions

All authors contributed to the conceptualization, design of the study, and the process of data collection; S.A.B., M.A.E., and K.M.A.D., were actively involved in interpretation of data and drafted the manuscript for all authors to critique and make relevant corrections. All the authors have read and agreed to final version of this manuscript.


Compliance with Ethical Principles

The study was conducted following the principles of the Declaration of Helsinki. The Institutional Review Board approved the study at Sheikh Khalifa Medical City, Abu Dhabi, United Arab Emirates. All participants provided informed consent before they could proceed to the survey questions.


Financial Support and Sponsorship

None.




Publication History

Article published online:
05 August 2022

© 2022. Gulf Association of Endocrinology and Diabetes (GAED). All rights reserved. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Pivonello R, De Martino MC, De Leo M, Lombardi G, Colao A. Cushing's syndrome. Endocrinol Metab Clin North Am 2008; 37 (01) 135-149 , ix ix.  
    CrossrefPubMedGoogle Scholar
  • 2 Nieman LK, Biller BM, Findling JW. et al. The diagnosis of Cushing's syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 2008; 93 (05) 1526-1540
    Google Scholar
  • 3 Biller BM, Grossman AB, Stewart PM. et al. Treatment of adrenocorticotropin-dependent Cushing's syndrome: a consensus statement. J Clin Endocrinol Metab 2008; 93 (07) 2454-2462
    CrossrefPubMedGoogle Scholar
  • 4 Juszczak A, Morris D, Grossman A. Cushing's syndrome. In: Feingold KR, Anawalt B, Boyce A. et al, eds. Endotext [Internet]. South Dartmouth, MA: MDText.com, Inc.; 2000
    Google Scholar
  • 5 Malek M, Esfehanian F, Amouzegar A. et al. A survey of clinical practice patterns in diagnosis and management of Cushing's disease in Iran. Med J Islam Repub Iran 2016; 30: 334
    PubMedGoogle Scholar
  • 6 Beshyah SA, Sherif IH, Chentli F. et al. Management of prolactinomas: a survey of physicians from the Middle East and North Africa. Pituitary 2017; 20 (02) 231-240
    CrossrefPubMedGoogle Scholar
  • 7 Beshyah SA, Khalil AB, Sherif IH. et al. Clinical practice patterns in management of Graves' disease in the Middle East and North Africa. Endocr Pract 2017; 23 (03) 299-308
    CrossrefPubMedGoogle Scholar
  • 8 Ahmad MM, Buhairy B, Al Mousawi F. et al. Physicians' perceptions and practices in management of acromegaly in the MENA region. Hormones (Athens) 2018; 17 (03) 373-381
    CrossrefPubMedGoogle Scholar
  • 9 Beshyah SA, Al-Saleh Y, El-Hajj Fuleihan G. Management of osteoporosis in the Middle East and North Africa: a survey of physicians' perceptions and practices. Arch Osteoporos 2019; 14 (01) 60
    CrossrefPubMedGoogle Scholar
  • 10 Beshyah SA, Sherif IH, Mustafa HE, Saadi HF. Patterns of clinical management of hypothyroidism in adults: An electronic survey of physicians from the Middle East and Africa. J Diab Endo Practice 2021; 4: 75-82
    Article in Thieme ConnectGoogle Scholar
  • 11 Dorn LD, Lucke JF, Loucks TL, Berga SL. Salivary cortisol reflects serum cortisol: analysis of circadian profiles. Ann Clin Biochem 2007; 44 (pt. 3): 281-284
    CrossrefPubMedGoogle Scholar
  • 12 Poll EM, Kreitschmann-Andermahr I, Langejuergen Y. et al. Saliva collection method affects predictability of serum cortisol. Clin Chim Acta 2007; 382 (1-2): 15-19
    CrossrefPubMedGoogle Scholar
  • 13 Smith RE, Maguire JA, Stein-Oakley AN. et al. Localization of 11 beta-hydroxysteroid dehydrogenase type II in human epithelial tissues. J Clin Endocrinol Metab 1996; 81 (09) 3244-3248
    PubMedGoogle Scholar
  • 14 Badrick E, Kirschbaum C, Kumari M. The relationship between smoking status and cortisol secretion. J Clin Endocrinol Metab 2007; 92 (03) 819-824
    CrossrefPubMedGoogle Scholar
  • 15 Atkinson AB, Kennedy A, Wiggam MI, McCance DR, Sheridan B. Long-term remission rates after pituitary surgery for Cushing's disease: the need for long-term surveillance. Clin Endocrinol (Oxf) 2005; 63 (05) 549-559
    CrossrefPubMedGoogle Scholar
  • 16 Hammer GD, Tyrrell JB, Lamborn KR. et al. Transsphenoidal microsurgery for Cushing's disease: initial outcome and long-term results. J Clin Endocrinol Metab 2004; 89 (12) 6348-6357
    CrossrefPubMedGoogle Scholar
  • 17 Mancini T, Porcelli T, Giustina A. Treatment of Cushing disease: overview and recent findings. Ther Clin Risk Manag 2010; 6: 505-516
    CrossrefPubMedGoogle Scholar
  • 18 Chow JT, Thompson GB, Grant CS, Farley DR, Richards ML, Young Jr WF. Bilateral laparoscopic adrenalectomy for corticotrophin-dependent Cushing's syndrome: a review of the Mayo Clinic experience. Clin Endocrinol (Oxf) 2008; 68 (04) 513-519
    CrossrefPubMedGoogle Scholar
  • 19 Engelhardt D, Weber MM. Therapy of Cushing's syndrome with steroid biosynthesis inhibitors. J Steroid Biochem Mol Biol 1994; 49 (4-6): 261-267
    CrossrefPubMedGoogle Scholar
  • 20 Stricker BH, Blok AP, Bronkhorst FB, Van Parys GE, Desmet VJ. Ketoconazole-associated hepatic injury. A clinicopathological study of 55 cases. J Hepatol 1986; 3 (03) 399-406
    CrossrefPubMedGoogle Scholar
  • 21 de Bruin C, Pereira AM, Feelders RA. et al. Coexpression of dopamine and somatostatin receptor subtypes in corticotroph adenomas. J Clin Endocrinol Metab 2009; 94 (04) 1118-1124
    CrossrefPubMedGoogle Scholar
  • 22 Tateno T, Kato M, Tani Y, Oyama K, Yamada S, Hirata Y. Differential expression of somatostatin and dopamine receptor subtype genes in adrenocorticotropin (ACTH)-secreting pituitary tumors and silent corticotroph adenomas. Endocr J 2009; 56 (04) 579-584
    CrossrefPubMedGoogle Scholar
  • 23 Batista DL, Zhang X, Gejman R. et al. The effects of SOM230 on cell proliferation and adrenocorticotropin secretion in human corticotroph pituitary adenomas. J Clin Endocrinol Metab 2006; 91 (11) 4482-4488
    CrossrefPubMedGoogle Scholar
  • 24 Saveanu A, Jaquet P. Somatostatin-dopamine ligands in the treatment of pituitary adenomas. Rev Endocr Metab Disord 2009; 10 (02) 83-90
    CrossrefPubMedGoogle Scholar
  • 25 Hofland LJ, van der Hoek J, Feelders R. et al. The multi-ligand somatostatin analogue SOM230 inhibits ACTH secretion by cultured human corticotroph adenomas via somatostatin receptor type 5. Eur J Endocrinol 2005; 152 (04) 645-654
    CrossrefPubMedGoogle Scholar
  • 26 Durán-Prado M, Gahete MD, Martínez-Fuentes AJ. et al. Identification and characterization of two novel truncated but functional isoforms of the somatostatin receptor subtype 5 differentially present in pituitary tumors. J Clin Endocrinol Metab 2009; 94 (07) 2634-2643
    CrossrefPubMedGoogle Scholar
  • 27 Stalla GK, Brockmeier SJ, Renner U. et al. Octreotide exerts different effects in vivo and in vitro in Cushing's disease. Eur J Endocrinol 1994; 130 (02) 125-131
    CrossrefPubMedGoogle Scholar
  • 28 Lamberts SW, Uitterlinden P, Klijn JM. The effect of the long-acting somatostatin analogue SMS 201-995 on ACTH secretion in Nelson's syndrome and Cushing's disease. Acta Endocrinol (Copenh) 1989; 120 (06) 760-766
    CrossrefPubMedGoogle Scholar
  • 29 Mazziotti G, Floriani I, Bonadonna S, Torri V, Chanson P, Giustina A. Effects of somatostatin analogs on glucose homeostasis: a meta-analysis of acromegaly studies. J Clin Endocrinol Metab 2009; 94 (05) 1500-1508
    CrossrefPubMedGoogle Scholar