Protein Losing Enteropathy as a Manifestation of Henoch‐Schönlein Purpura

Reif, Shimon; Jain, Apurva; Santiago, José; Rossi, Thomas M.

doi:10.1111/j.1651-2227.1991.tb11888.x

Cited by 38 publications

(16 citation statements)

References 17 publications

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“…Protein-losing enteropathy can be diagnosed by an elevated fecal alpha 1 antitrypsin level [83]. Mesenteric vasculitis is uncommon in HSP, but it has a high risk of bowel necrosis and massive GI hemorrhage [84].…”

Section: Gi Features Of Hspmentioning

confidence: 99%

Gastrointestinal Manifestations of Henoch-Schonlein Purpura

2008

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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.

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Section: Gi Features Of Hspmentioning

confidence: 99%

Gastrointestinal Manifestations of Henoch-Schonlein Purpura

2008

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“…Severe skin lesions may require oral corticosteroids 13,37 -39) , which may also improve abdominal pain and protein -losing enteropathy 37) . Severe gastrointestinal complications may occasionally require surgical intervention 30) .…”

Section: Treatment Of Hspnmentioning

confidence: 99%

HENOCH-SCH^|^Ouml;NLEIN PURPURA NEPHRITIS IN CHILDHOOD: PATHOGENESIS, PROGNOSTIC FACTORS AND TREATMENT

Kawasaki

Ono

Ohara

et al. 2013

FJMS

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: Henoch -Schönlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries and the deposition of IgA immune complexes. Renal involvement is the principal cause of morbidity and mortality in children with HSP. Thus, it is important to clarify the onset mechanism as well as the prognostic factors of Henoch -Schönlein purpura nephritis (HSPN) and to identify the most appropriate treatment. We herein review the pathogenesis, the prognostic factors and treatment of HSPN. As to the pathogenesis, several studies suggest that galactose -deficient IgA1 (Gd -IgA1) is recognized by anti -glycan antibodies, leading to the formation of circulating immune complexes and their mesangial deposition, thereby inducing renal injury. With regard to the prognostic factors, a number of factors have been suggested including nephrotic syndrome, decreased factor XIII activity, hypertension, severe renal injury, high renal accumulation of activated macrophage, alpha -smooth muscle actin, and high serum myeloid -related protein levels. For the treatment of severe HSPN, aggressive therapies including multiple drug combination therapy and plasmapheresis have been shown to be effective in ameliorating proteinuria and histological severity. Nevertheless, detailed investigation into the pathogenesis of HSPN and double -blind randomized control studies on children with HSPN are still necessary.

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“…While the etiology and significance of low C3 and C4 in our patient is not clear, the kidney biopsy was not representative of a post-infectious or lupus-like phenomenon. It is possible that gastrointestinal protein losses contributed to the low complement values [23].…”

Section: Discussionmentioning

confidence: 99%

Henoch-Schönlein purpura and pulmonary hemorrhage: a report and literature review

Vats

Kim

et al. 1999

Pediatric Nephrology

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Pulmonary hemorrhage, a rare complication of Henoch-Schönlein purpura (HSP) reported primarily in adults and adolescents, is associated with significant mortality. Although it has been suggested that pulmonary hemorrhage also occurs in children with HSP, the few cases reported lack a clear differentiation from pauci-immune vasculitis. We report a prepubertal child with HSP, pulmonary hemorrhage, and immunofluorescence-documented IgA deposits on renal biopsy. Aggressive supportive management and steroid therapy led to successful recovery. A review of the current literature is presented. Because other conditions clinically mimic HSP, appropriate serological studies and a kidney biopsy to confirm the diagnosis should be performed in severely affected patients with renal disease.

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Protein Losing Enteropathy as a Manifestation of Henoch‐Schönlein Purpura

Cited by 38 publications

References 17 publications

Gastrointestinal Manifestations of Henoch-Schonlein Purpura

Gastrointestinal Manifestations of Henoch-Schonlein Purpura

HENOCH-SCH^|^Ouml;NLEIN PURPURA NEPHRITIS IN CHILDHOOD: PATHOGENESIS, PROGNOSTIC FACTORS AND TREATMENT

Henoch-Schönlein purpura and pulmonary hemorrhage: a report and literature review

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