Skip to main content
Log in

Is there a real adrenal axis dysfunction in patients with amyloidosis associated with familial Mediterranean fever?

  • Original Article
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

Systemic amyloidosis with AA-type amyloid deposition is the major complication of FMF, leading to end stage renal disease. There is no clear data on the prevalence of adrenal involvement in patients with FMF amyloidosis. The aim of this study is to determine the adrenal axis function in patients FMF with amyloidosis. Twenty patients with FMF with amyloidosis (F/M: 10/10, mean age; 38 ± 11 SD years), twenty without amyloidosis (F/M: 14/6, mean age 32 ± 10 years), and healthy controls (F/M: 12/8, mean age: 30 ± 7.6 SD years) were recruited. A dose of 250 mg tetracosactide (Synacthen) was then administered intravenously and further blood samples collected 30 and 60 min later. Blood samples were separated and collected at 4°C, and serum cortisol levels were measured. A normal cortisol response to Synacthen was defined as a post-stimulation peak cortisol value of >18 mg/d either at 30 or 60 min. sample. The mean disease duration was 8.8 ± 6 SD years, (range, 2–21) in FMF patients without amyloidosis compared to 16 ± 9.5 years (range, 0–30) in FMF with amyloidosis (P = 0.001). The cortisol concentrations increased significantly at 30 and 60 min compared to baseline after injection of synacthen in all groups. There were no statistically significant differences found among three groups, for basal, 30 and 60 min for cortisol levels (P = 0.154). FMF patients with amyloidosis do not exhibit overt adrenal insufficiency even though their basal cortisol levels were mildly lower.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Livneh A, Langevitz P, Zemer D, Zaks N, Kees S, Lidar T, Migdal A, Padeh S, Pras M (1997) Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum 40:1879–1885

    Article  PubMed  CAS  Google Scholar 

  2. Onen F (2006) Familial Mediterranean fever. Rheumatol Int 26:489–496

    Article  PubMed  Google Scholar 

  3. Takahashi N, Suzuki E, Geyjo F (2002) Reactive amyloidosis and familial Mediterranean fever (FMF). Intern Med 5:329–330

    Article  Google Scholar 

  4. Dorin RI, Qualls CR, Crapo LM (2003) Diagnosis adrenal insufficiency. Ann Intern Med 139:194–204

    PubMed  Google Scholar 

  5. Suliman AM, Smith TP, Labib M, Fiad TM, McKenna TJ (2002) The low-dose ACTH test does not provide a useful assessment of the hypothalamic-pituitary-adrenal axis in secondary adrenal insufficiency. Clin Endocrinol 56:539–553

    Article  Google Scholar 

  6. Lidar M, Livneh A (2007) Familial Mediterranean fever: clinical, molecular and management advancements. Neth J Med 65:318–324

    PubMed  CAS  Google Scholar 

  7. Sav T, Ozbakır O, Kelestimur F, Gursoy S, Baskol M, Kula M, Dundar M (2006) Adrenal axis function in patients with familial Mediterranean fever. Clin Rheumatol 25:458–461

    Article  PubMed  Google Scholar 

  8. Straub RH, Lehel K, Herfarth H, Weber M, Falk W, Preuner J, Schölmerich J (2002) Dehydroepiandrosterone in relation to other adrenal hormones during an acute inflammatory stressful disease state compared with chronic inflammatory disease: role of interleukin-6 and tumor necrosis factor. Eur J Endocrinol 146:365–374

    Article  PubMed  CAS  Google Scholar 

  9. Korkmaz C, Özdoğan H, Kasapçapur Ö, Yazici H (2002) Acute phase response in familial Mediterranean fever. Ann Rheum Dis 61:79–81

    Article  PubMed  CAS  Google Scholar 

  10. Willis AC, Vince FP (1997) The prevalence of Addisson’s disease in Coventry, UK. Postgrad Med J 73:286–288

    Article  PubMed  CAS  Google Scholar 

  11. Arık N, Tasdemir I, Karaaslan Y, Yasavul U, Turgan Ç, Çağlar Ş (1990) Subclinical adrenocortical insufficiency in renal amyloidosis. Nephron 56:246–248

    Article  PubMed  Google Scholar 

  12. Danby P, Harris KP, Williams B, Feehally J, Walls J (1990) Adrenal dysfunction in patients with renal amyloid. Q J Med 76:915–922

    Google Scholar 

  13. Cronstein B (2007) Interleukin-6—a key mediator of systemic and local symptoms in rheumatoid arthritis. Bull NYU Hosp Jt Dis 65:11–15

    Google Scholar 

  14. Baykal Y, Saglam K, Yilmaz MI, Taslipinar A, Akinci SB, Inal A (2003) Serum sIL-2r, IL-6, IL-10 and TNF-α level in familial Mediterranean fever patients. Clin Rheumatol 22:99–101

    Article  PubMed  CAS  Google Scholar 

  15. Gonzalez–Gonzalez JG, De La Garza-Hernandez NE, Mancillas-Adame LG, Montes-Villareal J, Villareal-Perez JZ (1998) A high-sensitivity test in the assessment of adrenocortical insufficiency: 10 microgr vs 250 microg cosyntropin dose assessment of adrenocortical insufficiency. J Endocrinol 159:275–280

    Article  PubMed  Google Scholar 

  16. Oelkers W, Diederich S, Bahr V (1992) Diagnosis and therapy surveillance in Addison’s disease: rapid adrenocorticitropin (ACTH) test and measurement of plasma ACTH, renin activity, and aldestrone. J Clin Endocrinol Metab 75:259–264

    Article  PubMed  CAS  Google Scholar 

  17. Vestergaard P, Hoeck HC, Jakobsen PE, Laurberg P (1997) Reproducibility of growth hormone and cortisol responses to the insulin tolerance test and the short ACTH test in normal adults. Horm Metab Res 29:106–110

    Article  PubMed  CAS  Google Scholar 

  18. Çalışkan M, Gulu H, Yılmaz S, Erdoğan D, Unler GK, Ciftci O, Topcu S, Kayhan Z, Yucel E, Muderrisoglu H (2007) Impaired coronary microvascular function in familial Mediterranean fever. Atherosclerosis 195:161–167

    Article  Google Scholar 

Download references

Conflict of interest

All authors have nothing to disclose.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Seval Masatlioglu.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Yılmaz, G., Masatlioglu, S., Yetkin, D.O. et al. Is there a real adrenal axis dysfunction in patients with amyloidosis associated with familial Mediterranean fever?. Rheumatol Int 32, 3421–3424 (2012). https://doi.org/10.1007/s00296-011-2181-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-011-2181-7

Keywords

Navigation