Is There IgA from Gut Mucosal Origin in the Serum of Children with Henoch–Schönlein Purpura?

Moja, Ph.; Quesnel, Anne; Resseguier, V.; Lambert, Claude; Freyçon, F; Berthoux, F; Genin, Christian

doi:10.1006/clin.1997.4493

Cited by 23 publications

(14 citation statements)

References 41 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…IgA1 has a hinge region of the heavy chains, absent in IgA2, which is glycosylated. IgA1 levels in HSP are elevated [7]. In addition, this molecule is deficient in sialic acid [8], perhaps due to sialidase (also called neuraminidase) [9] produced by common pathogens implicated as triggers of HSP.…”

Section: Pathogenesis Of Hspmentioning

confidence: 99%

Gastrointestinal Manifestations of Henoch-Schonlein Purpura

2008

View full text Add to dashboard Cite

Henoch-Schonlein Purpura (HSP) is the most common systemic vasculitis in childhood. The diagnostic criteria include palpable purpura with at least one other manifestation -- abdominal pain, IgA deposition, arthritis or arthralgia, or renal involvement. Immune complex deposits result in necrosis of the wall of small- and medium-sized arteries with infiltration of tissue by neutrophils and deposition of nuclear fragments, a process called leukocytoclastic vasculitis (LCV). It is often associated with infections, medications, or tumors. It may coexist with or mimic Crohn's disease. Periumbilical and epigastric pain worsens with meals, from bowel angina. Bleeding is usually occult or, less commonly, associated with melena. Intussusception, the most common surgical complication, is usually ileo-ileo or ileo-colic. Perforations, usually ileal, may occur spontaneously or be associated with intussusception. Ultrasound, recommended as the first diagnostic test, and CT scans may show intussusception and asymmetric bowel wall thickening mainly involving the jejunum and ileum. There are a range of endoscopic findings including gastritis, duodenitis, ulceration, and purpura, with the second portion of the duodenum characteristically being involved more than the bulb. Intestinal biopsies show IgA deposition and LCV in the submucosal vessels. Superficial biopsies may show inflammation, ulceration, edema, hemorrhage, and vascular congestion, presumably due to vasculitis-induced mucosal ischemia. The efficacy of corticosteroids in preventing severe complications or relapses is controversial. The majority of patients, however, improve spontaneously.

show abstract

Section: Pathogenesis Of Hspmentioning

confidence: 99%

Gastrointestinal Manifestations of Henoch-Schonlein Purpura

2008

View full text Add to dashboard Cite

show abstract

“…This predisposes to circulating IgA immune complexes, usually deposited in kidney, skin or gut [20]. The antigenic content of these complexes suggests possible derivation from gut contents, including dietary proteins such as gliadin [14]. Gastrointestinal lesions in HSP are characterised by focal vascular IgA deposition [12,24], as in this case, and the formation of IgA anti-endothelial antibodies may occur [20].…”

Section: Discussionmentioning

confidence: 91%

Massive gastrointestinal haemorrhage in isolated intestinal Henoch-Schonlein purpura with response to intravenous immunoglobulin infusion

et al. 2006

View full text Add to dashboard Cite

There is increasing recognition that Henoch-Schonlein purpura may present in an atypical form in which gastrointestinal symptoms may predominate, and classic cutaneous changes may be delayed or absent. This may lead to significant diagnostic delay. We report the case of a 9-year-old girl who presented acutely with life-threatening gastrointestinal haemorrhage from multiple intestinal sites, with no skin rash and only mild evidence of renal involvement. Henoch-Schonlein purpura was confirmed by finding IgA deposition on vessels within gastric and duodenal mucosa, while immunohistochemistry also identified dense focal T cell infiltration in gastric mucosa and within duodenal epithelium. After initial stabilisation, the patient became shocked due to further gastrointestinal haemorrhage. Isotope bleeding scan identified multiple bleeding sites. Her endoscopically confirmed gastritis was sufficiently severe to preclude corticosteroids, and she was thus treated with intravenous immunoglobulin. This therapy induced prompt and sustained resolution of symptoms, and she has remained well since. Our patient's response concords with previous reports in corticosteroid-resistant cases to suggest that severe intestinal Henoch-Schonlein purpura may respond preferentially to intravenous immunoglobulin (IVIG) therapy. In severe cases where there is significant gastritis, IVIG provides an effective alternative to corticosteroids that may be employed as first-line therapy.

show abstract

“…Increased polymeric IgA production by the mucosal immune system in response to a mucosally presented antigen has been hypothesized to be a mechanism for the development of HSP (3,11,12). IgA is the main immunoglobulin directed against external viral and bacterial antigens.…”

Section: Discussionmentioning

confidence: 99%