Akademik Veri Yönetim Sistemi
RHEUMATOLOGY INTERNATIONAL, cilt.45, sa.11, 2025 (SCI-Expanded, Scopus)
The most important complication of familial Mediterranean fever (FMF) are AA amyloidosis and amyloid nephropathy. Amyloid nephropathy typically follows a slow, indolent course. However, "amyloid storm" is a recently described acute condition, characterized by sudden onset of severe proteinuria and rapidly progressive renal failure with markedly acute-phase reactants, progressing over days to weeks into end-stage renal disease (ESRD). Amyloid storm has been rarely reported in the literature. Most cases have occurred in patients with known FMF-related AA amyloid nephropathy. However, it has also been reported as the initial presentation of AA amyloidosis. No standardized treatment guidelines exist, but management has included supportive care, colchicine, and interleukin (IL)-1 blockade. This report presents the first case of amyloid storm associated with ulcerative colitis in the literature. The patient exhibited impaired oral intake and fatigue, alongside acute kidney injury, spot urine proteinuria of approximately 100 g/day, and elevated C-reactive protein levels (CRP 118 mg/L). The diagnosis of AA amyloidosis was made after a kidney biopsy. In addition to starting haemodialysis, the patient was treated with anakinra and colchicine given the clinical presentation indicative of an amyloid storm. Subsequently, the acute-phase reactants decreased and the patient's general health improved, but haemodialysis was still required. After the patient discontinued anakinra treatment, he was re-hospitalized with complaints of fatigue and weakness but died on day 71 after the amyloid storm diagnosis. In our literature review, we identified a total of 36 patients with amyloid storm, 29 of whom were from two separate original studies. According to our review, the frequency of FMF-associated amyloid storm is 5.5%. The majority of patients were male, and while FMF was the most common cause of AA amyloidosis, ankylosing spondylitis, non-FMF autoinflammatory diseases, and ulcerative colitis were among the other causes. Our analysis revealed that infections were the most common triggering factor of an amyloid storm. ESRD rates were found to be up to 67%, and mortality rates ranged from 33 to 67%. This case report aims to highlight the relatively new and little-known concept of amyloid storm, which, despite its rarity, discusses its clinical features, laboratory findings, treatment options, and outcomes based on our case as well as a few cases in the literature.