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Abdominal syndrome in monogenic autoinflammatory disease – is it just a familial Mediterranean fever?

Authors :
S. O. Salugina
E. S. Fedorov
N. G. Volf
Source :
Современная ревматология, Vol 14, Iss 4, Pp 144-149 (2020)
Publication Year :
2020
Publisher :
IMA-PRESS LLC, 2020.

Abstract

Gastrointestinal (GI) manifestations, such as abdominal pain, nausea, vomiting, and diarrhea, are common autoinflammatory disease (AID) symptoms. The abdominal symptomatology reflecting serositis is one of the most important classification and diagnostic criteria for the classic monogenic AID (MAID) – familial Mediterranean fever (FMF). Failure to timely diagnose FMF frequently leads to unjustified surgical interventions. Other periodic fevers may also present as abdominal symptoms; however, the latter are outside their diagnostic features. These diseases include, first of all, tumor necrosis factor receptor-associated periodic syndrome (TRAPS). Interleukin 1 (IL1) inhibitors serve as the major targeted drugs for the treatment of TRAPS. Russia has registered the IL1 inhibitor canakinumab that prevents the development of organ damages, including those in the GI tract. The paper describes a clinical case of the classic manifestations of TRAPS (fever, rash, periorbital edema, arthritis, and elevated levels of acutephase inflammatory markers) concurrent with severe abdominalgia during attacks and with the development of severe peritoneal adhesions, which led to bowel perforation and emergency surgical intervention. The prolonged persistence of inflammatory attacks before the initiation of therapy, as well as violation of the IL1 inhibitor administration regimen facilitated the development of an urgent exacerbation. Thus, TRAPS should be included in the differential diagnostic circle for patients with severe gastrointestinal manifestations characterized by an attack-like course. These patients need timely prescription of targeted therapy, strict adherence to the dosing and intervals between drug administrations, and careful monitoring to prevent serious complications with the visceral organs, including the gastrointestinal tract, and their immediate correction.

Details

Language :
Russian
ISSN :
19967012 and 2310158X
Volume :
14
Issue :
4
Database :
Directory of Open Access Journals
Journal :
Современная ревматология
Publication Type :
Academic Journal
Accession number :
edsdoj.60e7c6526f843afb4a3c13395d43e1b
Document Type :
article
Full Text :
https://doi.org/10.14412/1996-7012-2020-4-144-149