Clinical and Endoscopic Features of Amyloidosis Secondary to Crohn's Disease: Diagnostic Value of Duodenal Observation and Biopsy

Abstract

The incidence of AMY as a complication of CD was low (2.5%). However, because this complication adversely affects patients' prognoses, it is important to check for the presence of AMY, particularly in the duodenum, in patients for whom more than 10 years have elapsed since the development of CD.

Crohn’s disease-specific mortality: a 30-year cohort study at a tertiary referral center in Japan

“…A recent study from the USA showed that mortality increases in the presence of secondary amyloidosis in hospitalized CD patients [ 41 ]. A report by Miyaoka from Japan stated that secondary amyloidosis does not occur frequently in CD (2.5%), but that 40% of these patients died, indicating that the prognosis is poor with secondary amyloidosis [ 40 ]. In addition, a long-term outcome report by Weterman et al showed that 4 of 64 deaths (6.3%) were due to amyloidosis [ 22 ], indicating that this is not rare.…”

“…In addition, a long-term outcome report by Weterman et al showed that 4 of 64 deaths (6.3%) were due to amyloidosis [ 22 ], indicating that this is not rare. Patients are diagnosed with amyloidosis between 12.0 and 25.0 years after the diagnosis of CD [ 38 , 40 , 43 ], and they die of complications such as kidney failure. Thus, death caused by amyloidosis may only be captured by performing long-term follow-up, as demonstrated in the present study.…”

“…The prevalence of comorbid secondary amyloidosis in patients with CD has been reported to be 2.5-5.3% in Japan and 0.9-5.6% in Western countries [38][39][40][41][42]. It is considered that, when amyloidosis develops in conjunction with CD, serious complications including kidney failure ensue, ultimately increasing mortality [42].…”

Clinical Presentation of Amyloid A Amyloidosis

“…Most of clinical symptoms are caused by distortion of the normal tissue architecture. The patients can present with organ enlargement such as hepatomegaly, splenomegaly, renomegali, enlarged thyroid, rarely hypertophy of lymph nodes by massive amyloid deposition, easy bruising by weakening of the vascular walls (65,70), proteinuria, renal failure and malabsorbtion (4,19,20,61,65). Unexplained kidney, heart, or systemic disease, hepatomegaly and splenomegaly are among suspicious for amyloidosis.…”

“…Clinical signs of amyloidosis generate according to its locations, and most of them are not specific. Kidney, liver, spleen, heart, intestinal and respiratory tract are the main involved organs or systems in AA amyloidosis (4,19,20,55,(60)(61)(62)(63)(64)(65)(66). Adrenal and thyroid glands, testes, skin, synovial membrane and bone marrow are other sites of involvement and less common presentations (67)(68)(69).…”