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A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
Hypoadrenal syndrome in a patient with amyloidosis secondary to familial Mediterranean fever
Mehrnaz Asadi Gharabaghi1, Aram Behdadnia, Mehrnoush Asadi Gharabaghi
1Department of Pulmonary Medicine, Tehran University of Medical Sciences, Iran. asadi_m@tums.ac.ir
Abstract:
Amyloidosis is a common complication of poorly controlled familial Mediterranean fever (FMF). A variety of organs including kidneys, heart, liver, thyroid and adrenal glands may be clinically affected. However, involvement of adrenal glands leading to significant inefficiency is rarely seen in FMF patients with amyloidosis. The impairment of neuroendocrine immune system in FMF together with proteinuria in renal amyloidosis is a challenge while interpreting adrenal function tests. Here we present a case report of a 42-year-old man with FMF and renal failure due to amyloidosis whose disease course was complicated by adrenal insufficiency.
Insights
Familial Mediterranean fever (FMF) amyloidosis can affect adrenal glands, causing rare adrenal insufficiency. This case highlights challenges in diagnosing adrenal function in FMF patients with renal amyloidosis.
Area of Science:
- Endocrinology
- Nephrology
- Rheumatology
Background:
- Familial Mediterranean fever (FMF) is an autoinflammatory disease.
- Amyloidosis is a known complication of FMF, often affecting the kidneys.
- Adrenal gland involvement in FMF-associated amyloidosis is uncommon.
Observation:
- A 42-year-old male with FMF and renal failure due to amyloidosis presented with complications.
- The patient exhibited signs suggestive of adrenal insufficiency.
- Interpreting adrenal function tests was challenging due to FMF's neuroendocrine-immune aspects and proteinuria from renal amyloidosis.
Findings:
- The case report details a rare instance of adrenal insufficiency in a patient with FMF and renal amyloidosis.
- This presentation underscores the potential for widespread organ involvement in FMF complications.
- Diagnostic challenges in assessing adrenal function in this context were noted.
Implications:
- Highlights the importance of considering adrenal insufficiency in FMF patients with amyloidosis, especially with renal involvement.
- Suggests a need for refined diagnostic approaches for adrenal function in FMF patients with complex comorbidities.
- Emphasizes the systemic impact of FMF and its complications on multiple organ systems.
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