Familial Mediterranean Fever Complicated by a Triad of Adrenal Crisis: Amyloid Goiter and Cardiac Amyloidosis

Abstract

Background. FMF is a common disease in the Mediterranean populations and may be complicated by AA amyloidosis. The coexistence of adrenal and thyroid amyloidosis in AA amyloidosis secondary to familial Mediterranean fever (FMF) is an extremely rare reported condition. We presented a previously unreported triad of adrenal, thyroid, and cardiac amyloidosis secondary to FMF. Presentation of Case. We reported a 23-year-old Palestinian male patient presented with hypotension, vomiting, diarrhea, and abdominal pain. The patient was subsequently diagnosed to have an adrenal crisis with both amyloid goiter and cardiac amyloidosis. Conclusion. It is crucial to recognize the adrenal crisis in patients with AA amyloidosis secondary to FMF who present similarly to acute FMF inflammatory episodes. The adrenal crisis has high morbidity and mortality, especially if not recognized early in the course of the disease.

Deep Dive Into Familial Mediterranean Fever in a Child Without Fever

“…Incidentally, it was discovered soon after the initial workup that our patient also had added heart sounds, who was later diagnosed to have pericardial effusion. Abukhalaf and Ceylan et al [13,14] also reported cardiac complications among their patients, namely cardiac amyloidosis and right bundle branch block, respectively. Despite the differences in age groups, clinical presentations, family history, or laboratory results, colchicine remains the first-line treatment modality for a majority of the tabulated patients, with documented recovery.…”

“…Incidentally, it was discovered soon after the initial workup that our patient also had added heart sounds, who was later diagnosed to have pericardial effusion. Abukhalaf and Ceylan et al [ 13 , 14 ] also reported cardiac complications among their patients, namely cardiac amyloidosis and right bundle branch block, respectively.…”

Genetic and Epigenetic Regulation of MEFV Gene and Their Impact on Clinical Outcome in Auto-Inflammatory Familial Mediterranean Fever Patients

“…Familial Mediterranean fever (FMF) is the most common monogenic auto-inflammatory disease, affecting mainly the Mediterranean races [19] with a high prevalence in the Egyptian population [20]. It is characterized by recurrent fever attacks, erysipelas-like erythema, peritonitis, arthritis, and pleurisy, with amyloidosis as the major long-term complication [21,22]. Colchicine is the mainstay of FMF treatment, which is safe and effective in preventing FMF clinical attacks and secondary amyloidosis [23].…”

Tertiary adrenal insufficiency in a patient with familial Mediterranean fever: Association or coincidence?

“…Considering the previous reports describing the case of hypoadrenal syndrome with FMF, hypoadrenocorticism was suspected due to nonepisodic symptoms. [ 1 , 2 ] While loss of appetite, nausea, arthralgia, weight loss, hyperpigmentation, loss of axillary or pubic hair, or hypotension are often observed with hypoadrenocorticism, the patient showed none of these symptoms. The patient also had no history of prior glucocorticoid use.…”

“…Familial Mediterranean fever and adrenal insufficiency may present similar symptoms, such as fever, malaise, or arthralgia; therefore, the differentiation of adrenal insufficiency from FMF is challenging due to the heterogeneity of the clinical manifestations of FMF. [ 1 , 2 ] Moreover, in the case of a patient with isolated adrenocorticotropic hormone deficiency, blurring and exacerbating the symptoms of FMF were reported. [ 4 ] Therefore, coexistence of adrenal insufficiency should be considered in a patient with FMF, and it is reported that the combination of unexplained shock, nausea, and noninflammatory diarrhea may be suggestive of adrenal insufficiency.…”