IgG4-related Sclerosing Disease of the Small Bowel Presenting as Necrotizing Mesenteric Arteritis and a Solitary Jejunal Ulcer

Abstract

Since first described in the mid 1990s, there has been burgeoning literature on IgG4-related sclerosing disease. The number of sites that may be involved is ever increasing, with the pancreas, salivary glands, and lymph nodes being the most commonly affected organs. There are no well-documented cases arising in the gastrointestinal tract. In this report, we present the first case to our knowledge of IgG4-related sclerosing disease involving the small bowel with a distinctly unusual clinicopathologic presentation. A previously well 46-year-old woman presented with a 2-year history of intermittent abdominal pain with recent worsening due to small bowel obstruction. Following imaging, which showed jejunitis with surrounding mesenteric inflammatory changes, she proceeded to a segmental small bowel resection. The resected jejunum revealed an isolated, stenosing chronic ulcer associated with a necrotizing mesenteric arteritis. A transmural inflammatory infiltrate rich in IgG4 plasma cells was seen in the wall of the bowel and mesenteric artery. Abundant IgG4 interfollicular plasma cells were also identified in a mesenteric lymph node. The serum IgG4 level was elevated at >800 mg/dL (reference range 8 to 140 mg/dL). Although phlebitis is an almost constant feature of this disease, arteritis is not described other than in the lung and aorta. In this report, we also discuss the diagnostic pitfalls and the differential diagnoses that should be considered when this condition arises in the gastrointestinal tract.

IgG4-related Disorders of the Gastrointestinal Tract

“…40 Stomach Overview IgG4-related gastropathy is a well-characterized entity. The endoscopic features of IgG4-related gastropathy are nonspecific, although chronic gastric ulceration has been reported.…”

Rare Non-Tumor Diseases of the Pharynx and Larynx

“…Симптомы зависят от ор-гана-мишени. Наиболее часто поражаются под-желудочная железа (с развитием аутоиммунного панкреатита) [1], билиарная система (склерози-рующий холангит) [2], слезные и слюнные желе-зы (болезнь Микулича) [3], элементы орбиты [4], щитовидная железа (тиреоидит Риделя) [5], поч-ки (тубулоинтерстициальный нефрит) [6], забрю-шинное пространство (ретроперитонеальный фиброз) [7], аорта (аортит) [8], кишечник (ме-зентерит), предстательная и молочная железы, легкие, перикард, мозговые оболочки, гипофиз, лимфатические узлы и кожа. Обширная инфиль-трация IgG4 клетками плазмы и Т-лимфоцитами пораженного органа или органов нарушает фор-му и функции тканей, а также инициирует ин-тенсивную воспалительную реакцию [9].…”

An Immunoglobulin G4-Related Sclerosing Disease of the Small Bowel: CT and Small Bowel Series Findings

“…Few studies have been done on IgG4-related sclerosing diseases involving the gastrointestinal tract. So far, there has been only one report of IgG4-related sclerosing diseases arising in the small bowel, and it is mainly described in the histopathologic findings (3). In this paper, we report a case of histologically proven IgG4-related sclerosing disease involving the ileum with imaging findings on CT and small bowel series.…”