Henoch-Schönlein purpura presenting with multiplex gastrointestinal manifestations and massive nephrotic syndrome in adulthood — a case report

Abstract Background Henoch–Schönlein purpura (HSP) is a systemic small-vessel vasculitis that commonly affects gastrointestinal tract. The video capsule endoscopy (VCE) characteristics of pediatric HSP patients were rarely investigated. Methods Patients diagnosed with HSP by VCE examination at our hospital from February 2010 to January 2019 are analyzed. The clinical features, laboratory findings, and the characteristics of VCE findings are studied. Results There are 30 patients enrolled in this investigation from February 2010 to January 2020. The mean age of these patients is 96.9±35.8 months, and the most frequent finding of VCE is mucosal erosion, which account for 69 % of the patients, and followed by mucosal erythema or petechia accounted for 79.3 % of the patients. Regarding to the disease location detected by endoscopy, jejunum is the most common involved part of the gastrointestinal tract in pediatric HSP patients. All the patients had jejunum involved except in one patient the VCE did not pass through the pylorus. One third of the patients involved the descent part of duodenum. No side effects are observed in this study. Conclusions VCE is safe and effective in the diagnosis of gastrointestinal involved HSP patients with or without typical skin purpura. Jejunum is the most common involve location in gastrointestinal.

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Henoch-Schonlein Purpura

Pediatric Care Online ◽

10.1542/aap.ppcqr.396172 ◽

2020 ◽

Keyword(s):

Chronic Kidney Disease ◽

Nephrotic Syndrome ◽

Joint Pain ◽

Laboratory Tests ◽

Immunoglobulin A ◽

Small Vessel Vasculitis ◽

Henoch Schonlein Purpura ◽

Key Points ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Key Points Henoch-Schönlein purpura (HSP) is the most common form of vasculitis in children.It is a small-vessel vasculitis mediated by immunoglobulin A–containing immune complexes and characterized by nonthrombocytopenic purpura, abdominal pain, arthralgia, and renal disease.Diagnosis of HSP is clinical, and no laboratory tests are specific for HSP.Treatment is supportive. Patients with severe abdominal and joint pain may be treated with steroids.Steroids do not prevent renal disease in patients with HSP.Patients with nephritic or nephrotic syndrome have a much higher risk of developing chronic kidney disease and should be referred to a nephrologist.

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Henoch-Schonlein Purpura—A Case Report and Review of the Literature

Gastroenterology Research and Practice ◽

10.1155/2010/597648 ◽

2010 ◽

Vol 2010 ◽

pp. 1-7 ◽

Cited By ~ 32

Author(s):

Amit B. Sohagia ◽

Srinivas Guptha Gunturu ◽

Tommy R. Tong ◽

Hilary I. Hertan

Keyword(s):

Early Recognition ◽

Leukocytoclastic Vasculitis ◽

Systemic Disease ◽

Renal Involvement ◽

Symptomatic Treatment ◽

Adolescent Male ◽

Small Vessel Vasculitis ◽

Henoch Schonlein Purpura ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

We describe a case of an adolescent male with Henoch-Schonlein purpura (HSP), presenting with cutaneous and gastrointestinal manifestations. Endoscopy revealed diffuse ulcerations in the stomach, duodenum, and right colon. Biopsies revealed a leukocytoclastic vasculitis in the skin and gastrointestinal tract. Steroid therapy led to complete resolution of the symptoms. HSP is the most common childhood vasculitis, and is characterized by the classic tetrad of nonthrombocytopenic palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement. It is a systemic disease where antigen-antibody (IgA) complexes activate the alternate complement pathway, resulting in inflammation and small vessel vasculitis. Mild disease resolves spontaneously, and symptomatic treatment alone is sufficient. Systemic steroids are recommended for moderate to severe HSP. The prognosis depends upon the extent of renal involvement, which requires close followup. Early recognition of multiorgan involvement, especially outside of the typical age group, as in our adolescent patient, and appropriate intervention can mitigate the disease and limit organ damage.

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The characteristics of video capsule endoscopy in pediatric Henoch–Schönlein purpura with gastrointestinal symptoms

Pediatric Rheumatology ◽

10.1186/s12969-020-00471-4 ◽

2020 ◽

Vol 18 (1) ◽

Author(s):

Youhong Fang ◽

Kerong Peng ◽

Hong Zhao ◽

Jie Chen

Keyword(s):

Gastrointestinal Tract ◽

Capsule Endoscopy ◽

Gastrointestinal Symptoms ◽

Video Capsule Endoscopy ◽

Small Vessel Vasculitis ◽

Henoch Schonlein Purpura ◽

Laboratory Findings ◽

Abdominal Symptoms ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Abstract Background Henoch–Schönlein purpura (HSP) is a systemic small-vessel vasculitis also named IgA vasculitis that commonly affects the gastrointestinal tract. The video capsule endoscopy (VCE) characteristics of pediatric HSP patients are rarely reported. Methods Patients diagnosed with HSP and analyzed by VCE examination at our hospital from February 2010 to January 2019 are enrolled. The clinical features, laboratory findings, and the characteristics of VCE findings are studied. Results There are 30 patients enrolled in this investigation from February 2010 to January 2020. The mean age of these patients is 96.9 ± 35.8 months, and the most frequent finding of VCE is mucosal erosion, which account for 79.3% of the patients, and followed by mucosal erythema or petechia accounted for 69% of the patients. Regarding the disease location detected by endoscopy, jejunum is the most common involved part of the gastrointestinal tract in pediatric HSP patients. All the patients had the jejunum involved except in one patient the VCE did not pass through the pylorus. One third of the patients involved the descending portion of duodenum. No side effect is observed in this study. Conclusions VCE may be an excellent adjust tool for evaluation of the gastrointestinal tract in children with abdominal symptoms without typical purpura in suspected pediatric HSP patients. VCE appears to be superior to esophagogastroduodenoscopy in detecting small intestinal lesions of HSP and has an excellent safety profile.

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Henoch-Schönlein purpura in children: an updated review

Current Pediatric Reviews ◽

10.2174/1573396316666200508104708 ◽

2020 ◽

Vol 16 ◽

Author(s):

Alexander K. C. Leung ◽

Benjamin Barankin ◽

Kin Fon Leong

Keyword(s):

English Literature ◽

Renal Involvement ◽

Clinical Manifestations ◽

Current Evidence ◽

Small Vessel Vasculitis ◽

Henoch Schonlein Purpura ◽

Diffuse Abdominal Pain ◽

Excellent Outcome ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Background: Henoch-Schönlein purpura (HSP) is an IgA-mediated systemic small-vessel vasculitis with a predilection for the skin, gastrointestinal tract, joints, and kidneys. It is the most common form of systemic vasculitis in children. Objective: To familiarize physicians with the etiopathogenesis, etiology, clinical manifestations, evaluation, and management of children with Henoch-Schönlein purpura. Methods : A PubMed search was conducted in January 2020 in Clinical Queries using the key terms “Henoch-Schönlein purpura” OR “IgA vasculitis” OR “anaphylactoid purpura”. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews published within the past 10 years. Only papers published in the English literature were included in this review. This paper is based on, but not limited to, the search results. Results: Globally, the incidence of HSP is 10 to 20 cases per 100,000 children per year. Approximately 90% of cases occur in children between 2 and 10 years of age, with a peak incidence at 4 to 7 years. The diagnosis should be based on the finding of palpable purpura in the presence of at least one of the following criteria, namely, diffuse abdominal pain, arthritis or arthralgia, renal involvement (hematuria and/or proteinuria), and a biopsy showing predominant IgA deposition. Most cases are self-limited. The average duration of the disease is 4 weeks. Long-term complications are rare and include persistent hypertension and end-stage kidney disease. Therapy consists of general and supportive measures as well as treatment of the sequelae of the vasculitis. Current evidence does not support universal treatment of HSP patients with corticosteroids. Oral corticosteroids may be considered for HSP patients with severe gastrointestinal pain and gastrointestinal hemorrhage. Conclusions: Most cases of HSP have an excellent outcome, with renal involvement being the the most important prognostic factor in determining morbidity and mortality. Unfortunately, early steroid treatment does not reduce the incidence and severity of nephropathy in children with HSP. In HSP children who have severe nephritis or renal involvement with proteinuria of greater than 3 months, an angiotensin-converting enzyme inhibitor or angiotensin receptor blocker should be considered in addition to corticosteroids to prevent and/or limit secondary glomerular injury.

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SEVERE GASTROINTESTINAL MANIFESTATIONS COMPLICATING HENOCH-SCHÖNLEIN PURPURA IN ADULT: A RARE CASE IN INDONESIA

Asian Journal of Pharmaceutical and Clinical Research ◽

10.22159/ajpcr.2018.v11i11.28911 ◽

2018 ◽

Vol 11 (11) ◽

pp. 11

Author(s):

William Ray Cassidy ◽

Ketut Suryana ◽

Dewi Catur Wulandari

Keyword(s):

Systemic Vasculitis ◽

Renal Involvement ◽

Immunoglobulin A ◽

Henoch Schonlein Purpura ◽

Gastrointestinal Manifestations ◽

Patient Morbidity ◽

Schonlein Purpura ◽

Term Monitoring ◽

Henoch Schönlein Purpura

Henoch-Schönlein purpura (HSP), also known as immunoglobulin A vasculitis, is a self-limiting, small vessel systemic vasculitis rarely found in adults characterized by palpable purpura, arthritis or arthralgia, gastrointestinal, and renal involvement. We report a case of a 31-year-old male with HSP complicated with severe gastrointestinal manifestations mimicking acute abdomen. He was successfully managed with conservative approach. Meticulous diagnosis, treatment, and long-term monitoring are important to reduce patient morbidity.

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Terminal Ileitis: A Rare Complication of Henoch-Schönlein Purpura in Children

Gazeta Médica ◽

10.29315/gm.v1i1.487 ◽

2021 ◽

Author(s):

Ana Lia Costa Mano ◽

Mafalda Casinhas Santos ◽

Sara Limão ◽

Florbela Cunha

Keyword(s):

Rare Complication ◽

Renal Involvement ◽

Gastrointestinal Symptoms ◽

Abdominal Ultrasound ◽

Good Prognosis ◽

Lower Limbs ◽

Henoch Schonlein Purpura ◽

Terminal Ileitis ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Henoch–Schönlein purpura (HSP) is characterized by nonthrombocytopenic palpable purpura, arthritis or arthralgia, and gastrointestinal and/or renal involvement. Gastrointestinal symptoms are reported in 50%-75% and they are related to a previous group A streptococcal infection in 40%. A healthy 5-year-old girl presented with a three-week history of a recurrent purpuric rash on the lower limbs, arthralgia and angioedema, without renal involvement. During the third relapse, she had severe, diffuse and persistent abdominal pain and bloody stools. An abdominal ultrasound revealed transmural edema of the last ileal segment, compatible with ileitis. She received prednisolone for five days, with full clinical recovery. Antistreptolysin O titer was elevated. The remaining laboratory tests were normal (antinuclear, anti-neutrophil and anti-Saccharomyces cerevisiae antibodies; rheumatoid factor; stool cultures, parasitological examination and viral antigen tests). One month later, an abdominal ultrasound revealed no abnormalities. Terminal ileitis is a very rare complication of HSP in children but has a good prognosis.

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The characteristics of video capsule endoscopy in pediatric Henoch–Schönlein purpura with gastrointestinal symptoms

10.21203/rs.3.rs-25324/v2 ◽

2020 ◽

Author(s):

Youhong Fang ◽

Kerong Peng ◽

Hong Zhao ◽

Jie Chen

Keyword(s):

Gastrointestinal Tract ◽

Capsule Endoscopy ◽

Gastrointestinal Symptoms ◽

Video Capsule Endoscopy ◽

Small Vessel Vasculitis ◽

Henoch Schonlein Purpura ◽

Laboratory Findings ◽

Pass Through ◽

Schonlein Purpura ◽

Henoch Schönlein Purpura

Abstract Background Henoch–Schönlein purpura (HSP) is a systemic small-vessel vasculitis that commonly affects gastrointestinal tract. The video capsule endoscopy (VCE) characteristics of pediatric HSP patients were rarely investigated. Methods Patients diagnosed with HSP by VCE examination at our hospital from February 2010 to January 2019 are analyzed. The clinical features, laboratory findings, and the characteristics of VCE findings are studied. Results There are 30 patients enrolled in this investigation from February 2010 to January 2020. The mean age of these patients is 96.9±35.8 months, and the most frequent finding of VCE is mucosal erosion, which account for 69 % of the patients, and followed by mucosal erythema or petechia accounted for 79.3 % of the patients. Regarding to the disease location detected by endoscopy, jejunum is the most common involved part of the gastrointestinal tract in pediatric HSP patients. All the patients had jejunum involved except in one patient the VCE did not pass through the pylorus. One third of the patients involved the descent part of duodenum. No side effects are observed in this study. Conclusions VCE is safe and effective in the diagnosis of gastrointestinal involved HSP patients with or without typical skin purpura. Jejunum is the most common involve location in gastrointestinal.

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