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

Guven Yılmaz1, Seval Masatlioglu, Demet Ozgil Yetkin

  • 1Haydarpaşa Numune Training and Research Hospital, Istanbul, Turkey.

Rheumatology International
|November 8, 2011
PubMed

Insights

Patients with Familial Mediterranean Fever (FMF) and amyloidosis show normal adrenal function despite mildly lower baseline cortisol. This study assessed adrenal axis function in FMF patients with and without amyloidosis.

Area of Science:

  • Endocrinology
  • Rheumatology
  • Nephrology

Background:

  • Systemic amyloidosis, particularly AA-type, is a significant complication of Familial Mediterranean Fever (FMF).
  • AA-type amyloidosis in FMF can lead to end-stage renal disease, highlighting the systemic impact of the condition.
  • The prevalence and impact of adrenal involvement in FMF patients with amyloidosis remain unclear.

Purpose of the Study:

  • To investigate and determine the adrenal axis function in patients diagnosed with Familial Mediterranean Fever (FMF) and concurrent amyloidosis.
  • To compare adrenal function between FMF patients with amyloidosis, FMF patients without amyloidosis, and healthy controls.
  • To assess for overt adrenal insufficiency in FMF patients experiencing amyloid deposition.

Main Methods:

  • Recruitment of three groups: 20 FMF patients with amyloidosis, 20 FMF patients without amyloidosis, and 20 healthy controls.
  • Intravenous administration of 250 mg tetracosactide (Synacthen) to stimulate the adrenal axis.
  • Measurement of serum cortisol levels at baseline, 30 minutes, and 60 minutes post-Synacthen injection.

Main Results:

  • A normal cortisol response to Synacthen was defined as a peak cortisol level >18 mg/dL at 30 or 60 minutes post-injection.
  • Cortisol levels significantly increased from baseline in all groups after Synacthen administration.
  • No statistically significant differences in basal, 30-min, or 60-min cortisol levels were observed among the three groups (P = 0.154).

Conclusions:

  • FMF patients with amyloidosis do not exhibit overt adrenal insufficiency.
  • While basal cortisol levels may be mildly lower in FMF patients with amyloidosis, their adrenal axis remains responsive.
  • These findings suggest that amyloid deposition in FMF does not typically lead to clinically significant adrenal dysfunction.

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