From ulcerative colitis to amyloid storm: a case-based review.

Area of Science:

• Nephrology
• Rheumatology
• Internal Medicine

Background:

• Familial Mediterranean fever (FMF) complications include AA amyloidosis and amyloid nephropathy.
• Amyloid storm is an acute presentation of AA amyloidosis with rapid renal failure.
• It is rarely reported, often associated with FMF, but can be the initial manifestation of AA amyloidosis.

Purpose of the Study:

• To report the first case of amyloid storm associated with ulcerative colitis.
• To review the literature on amyloid storm, including causes, clinical features, and outcomes.
• To highlight this rare but severe condition.

Main Methods:

• Case presentation of a patient with ulcerative colitis and amyloid storm.
• Literature review of 36 patients with amyloid storm.
• Analysis of causes, triggering factors, end-stage renal disease (ESRD) rates, and mortality.

Main Results:

• The reported case of amyloid storm was associated with ulcerative colitis, presenting with acute kidney injury and high proteinuria.
• Literature review identified FMF as the most common cause (5.5%), but other conditions like ulcerative colitis, ankylosing spondylitis, and autoinflammatory diseases were noted.
• Infections were the most common trigger; ESRD rates reached 67%, and mortality ranged from 33% to 67%.

Conclusions:

• Amyloid storm is a critical, acute complication of AA amyloidosis with significant morbidity and mortality.
• Ulcerative colitis is identified as a potential underlying cause.
• Prompt recognition and management, including supportive care and IL-1 blockade, are crucial, though outcomes remain poor.