Fatal Acute Pancreatitis with Cystic Formation in Reactive Systemic AA Amyloidosis Secondary to Rheumatoid Arthritis

Matsuda, Masayuki; Sakurai, Shunpei; Suzuki, Akio; Kadoya, Masumi; Ikeda, Shu‐ichi

doi:10.2169/internalmedicine.42.888

Cited by 6 publications

(3 citation statements)

References 9 publications

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“…Both hemorrhage and inflammatory changes were regarded as secondary to amyloidosis due to the chemoattractant features of serum amyloid AA and increased vascular permeability. Similar to the present finding, acute pancreatitis has also been reported as a rare complication of generalized amyloidosis in human beings and it has been demonstrated that this complication might be fatal because generalized amyloidosis usually involves dysfunction in multiple organs (Matsuda et al, 2003). Although the question remains open as to the cause of adhesions in the intra-abdominal organs, there is, however, little doubt that chronic inflammation and altered vascular permeability give rise to serosal adhesions.…”

supporting

confidence: 88%

Generalized AA amyloidosis and fibrino-hemorrhagic pancreatitis in a Gazelle subgutturosa: a case report

Çeribaşı¹,

Eröksüz²,

Çeribaşı³

et al. 2009

Vet. Med. - Czech

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Generalized AA Amyloidosis and fibrino-hemorrhagic pancreatitis were diagnosed in a 6-year-old, male Gazelle subgutturosa submitted for necropsy from a Wild Animal Production Station in Malatya, a province from eastern Turkey. Adhesions between the visceral surfaces of abdominal organs including the liver, pancreas, diaphragma and spleen were observed in the necropsy. Microscopically, the mass consisted of fibrino-hemorrhagic pancreatic tissue. Chronic inflammatory reactions characterized by mononuclear cell infiltration and fibrous connective tissue proliferation were found on the serosal surface in the liver. Amyloid depositions were detected and confirmed by Congo red stained sections of the pancreas, liver, kidneys, and spleen viewed under a polarized light microscope. Generalized AA amyloidosis was thought to responsible for a chronic inflammation characterized by adhesions in Gazelle subgutturosa. Generalized AA amyloidosis along with pancreatic involvement and fibrinohemorrhagic changes are described for the first time in the Gazelle subgutturosa species.

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supporting

confidence: 88%

Generalized AA amyloidosis and fibrino-hemorrhagic pancreatitis in a Gazelle subgutturosa: a case report

Çeribaşı¹,

Eröksüz²,

Çeribaşı³

et al. 2009

Vet. Med. - Czech

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“…Severe AAa may involve blood vessels of different calibers of the pancreas and should be regarded as important factors in the pathogenesis of aRelLnP [74]. The role of severe AAa in pathogenesis of fatal acute pancreatitis is confirmed by others as well [75][76][77].…”

Section: Discussionmentioning

confidence: 86%

β-cell Related Amyloidosis Localized to the Islets of Langerhans, Type II Diabetes Mellitus and Liponecrotic Pancreatitis in Rheumatoid Arthritis: A Postmortem Clinicopathologic Statistical Study of 234 Autopsy Patients

Bély¹,

Apáthy²

2019

IDCD

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The aim of this study was to determine the prevalence of systemic AA amyloidosis (AAa), islet amyloidosis (IA) and liponecrotic pancreatitis (LnP) including acute liponecrotic (aLnP), acute relapsing liponecrotic (aRelLnP), and chronic liponecrotic pancreatitis (chrLnP) in rheumatoid arthritis (RA), and to analyse the possible relationship between them.Patients and methods: At the National Institute of Rheumatology 11558 patients died between 1969 and 1998; among them 234 with RA, and all of them were autopsied. RA was confirmed clinically according to the criteria of the American College of Rheumatology (ACR). The diagnosis of DM was based on clinical data. Tissue samples of pancreas were available for histologic evaluation in 164 of 234 patients. AAa, IA and LnP were diagnosed histologically. Demographics of different patient cohorts were compared with the Student (Welch) t probe. The relationships between AAa and IA, furthermore between IA and DM or LnP (including aLnP, aRelLnP, chrLnP) were analyzed by Pearson’s chi-squared (c2) test.Results: AAa complicated RA in 42 (25.61%) of 164 patients. IA localized to the islets of Langerhans was observed in 16 (9.76%) of 164 pancreases. Clinically diagnosed DM was associated with RA in 31 (18.90%) of 164 patients. Pancreatitis with multiple liponecrotic foci (LnP) was found in 19 (11.58%) of 164 patients; aLnP existed in 9 (47.37%), aRelLnP in 4 (21.05%), and liponecrotic foci in combination with chronic fibrotic pancreatitis (chrLnP) in 6 (31.58%) of these 19 patients.Discussion and conclusions: There was no significant difference between female and male RA patients associated with AAa, IA, DM and LnP. The age, sex and onset of disease did not influence basically the prevalence of AAa, IA, DM and LnP except male patients with IA, whose mean age at death was significantly higher than the general RA population. IA (fibrillar amyloid IAPP deposits -AIAPP) is related to the activity of b cells and may presumably be a faulty product of b-cells (normal islets of Langerhans do not contain IA deposits). The progressive deposition of IAPP prohormon fragments inhibits the function of b-cells because of their toxic effect and/or blocking mechanically the blood supply of b-cells and they “die in their own product”. The significant correlation between IA and DM refers to a close connection between them, but not necessarily a direct cause and effect relationship; it may be an indirect result of damaged (apoptotic) b-cells. The early stage of IA is characterized by minimal IAPP deposits involving only a few islets, which represents a clinically latent DM, and the advanced stage of IA is characterized by massive IAPP deposits involving most of the islets, which correspond to clinically manifest DM. Based on the positive and significant correlation between IA and clinically not diagnosed DM, IA may be a good indicator of potential DM in the latent stage of disease. Therefore we recommend that all biopsy material and surgical specimens of pancreas to be tested for IA or IAPP deposition.

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“…In the present patient, systemic amyloidosis might have led to a more severe and aggressive attack of pancreatitis. In fact, perivascular amyloid deposit with pancreatic ischemia and circulatory impairment as well as amyloidosis-related deformity of the duodenal papilla and pancreatic and biliary ducts are proposed as possible mechanisms for pancreatitis in patients with systemic amyloidosis (4-6). As an example, a fatal acute pancreatitis has been reported in a patient with rheumatoid arthritis and secondary systemic AA amyloidosis (4).…”

mentioning

confidence: 99%

Fulminant Acute Pancreatitis in a Patient with Familial Mediterranean Fever on CAPD: What Caused the Pancreatitis?

et al. 2009

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Fatal Acute Pancreatitis with Cystic Formation in Reactive Systemic AA Amyloidosis Secondary to Rheumatoid Arthritis

Cited by 6 publications

References 9 publications

Generalized AA amyloidosis and fibrino-hemorrhagic pancreatitis in a Gazelle subgutturosa: a case report

Generalized AA amyloidosis and fibrino-hemorrhagic pancreatitis in a Gazelle subgutturosa: a case report

β-cell Related Amyloidosis Localized to the Islets of Langerhans, Type II Diabetes Mellitus and Liponecrotic Pancreatitis in Rheumatoid Arthritis: A Postmortem Clinicopathologic Statistical Study of 234 Autopsy Patients

Fulminant Acute Pancreatitis in a Patient with Familial Mediterranean Fever on CAPD: What Caused the Pancreatitis?

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