Plasma exchange therapy for severe gastrointestinal involvement of Henoch Schonlein purpura in children

Başaran, Özge; Acar, Banu; Uncu, Nermin; Çaycı, Fatma Şemsa; Kara, Abdurrahman; Taktak, Aysel; Çakar, Nilgün

doi:10.1186/1546-0096-12-s1-p357

Cited by 14 publications

(11 citation statements)

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“…Similarly, a small case series identified patients with severe HSP nephritis who were treated with steroids in combination with plasmapheresis with mixed outcomes [9]. Knowledge about treatment of severe gastrointestinal manifestations come mainly from case reports and series in children, in which immunosuppression and plasmapheresis have been used in the event of steroid failure [10]. …”

Section: Discussionmentioning

confidence: 99%

Henoch-Schönlein Purpura Presenting as Severe Gastrointestinal and Renal Involvement with Mixed Outcomes in an Adult Patient

Raj

Ramakrishnan

Vollmar

et al. 2017

Cureus

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Henoch-Schönlein purpura (HSP) is typically seen as a self-limiting disease in children, but can present more severely in adults, especially when there is renal involvement. Management of HSP in adults also remains a controversial topic with very few studies evaluating available therapies. In this case, HSP presenting as a combination of severe gastrointestinal involvement and a rapid decline in renal function in an adult patient directed our therapy.The patient was a 48-year-old Caucasian male with no known past medical history, who presented with a combination of purpuric rash over the lower extremities, severe abdominal pain with upper gastrointestinal bleeding and a rapidly increasing serum creatinine, with hematuria. He initially underwent a skin biopsy, along with investigation for other possible causes, including autoimmune and infectious etiologies, which were negative. He was started on therapy for presumed HSP with intravenous methylprednisolone. The skin biopsy, however, was not conclusive, and the patient had no improvement in his clinical status. He then underwent a kidney biopsy that was consistent with HSP nephritis (immunoglobulin A (IgA) predominant glomerulonephritis with crescents), and esophagogastroduodenoscopy (EGD) that showed mucosal inflammation, ulcerations, and stigmata of bleeding—findings that were consistent with ischemia. Cyclophosphamide was added to the regimen at this time. However, he had worsening abdominal pain, continued gastrointestinal bleeding, now with hematochezia, and also worsening renal function that required dialysis. Plasmapheresis was then initiated on days alternating with dialysis. This resulted in the improvement of his gastrointestinal symptoms, but no recovery was seen of his renal function, and the patient required outpatient dialysis.This case report exhibits the unique presentation of severe gastrointestinal (GI) manifestations and rapid progression to renal failure in an adult patient with partial resolution of his severe manifestation after therapy was escalated as above. There was no established protocol that guided this therapy, which reflects the need for more studies on adult HSP.

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Section: Discussionmentioning

confidence: 99%

Henoch-Schönlein Purpura Presenting as Severe Gastrointestinal and Renal Involvement with Mixed Outcomes in an Adult Patient

Raj

Ramakrishnan

Vollmar

et al. 2017

Cureus

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“…The use of high dose steroids may be considered in HSP patients presenting with severe gastrointestinal (GI) symptoms (i.e., abdominal pain and hematochezia) or persistent proteinuria. If severe GI symptoms or nephrotic syndrome persist despite steroid treatment, then other treatment modalities such as intravenous immunoglobulin (IVIG), immunosuppressive drugs, or plasmapheresis may be used [ 12 13 14 15 16 17 ]. In this study, we investigated the epidemiologic characteristics and clinical courses of children hospitalized with HSP at a tertiary hospital between 2004 and 2015.…”

Section: Introductionmentioning

confidence: 99%

Henoch-Schonlein Purpura in Children Hospitalized at a Tertiary Hospital during 2004-2015 in Korea: Epidemiology and Clinical Management

Lee

Kim

Koo

et al. 2016

Pediatr Gastroenterol Hepatol Nutr

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PurposeTo investigate the epidemiology, clinical manifestations, investigations and management, and prognosis of patients with Henoch-Schonlein purpura (HSP).MethodsWe performed a retrospective review of 212 HSP patients under the age of 18 years who were admitted to Inje University Sanggye Paik Hospital between 2004 and 2015.ResultsThe mean age of the HSP patients was 6.93 years, and the ratio of boys to girls was 1.23:1. HSP occurred most frequently in the winter (33.0%) and least frequently in the summer (11.3%). Palpable purpura spots were found in 208 patients (98.1%), and gastrointestinal (GI) and joint symptoms were observed in 159 (75.0%) and 148 (69.8%) patients, respectively. There were 57 patients (26.9%) with renal involvement and 10 patients (4.7%) with nephrotic syndrome. The incidence of renal involvement and nephrotic syndrome was significantly higher in patients with severe GI symptoms and in those over 7 years old. The majority of patients (88.7%) were treated with steroids. There was no significant difference in the incidence of renal involvement or nephrotic syndrome among patients receiving different doses of steroids.ConclusionIn this study, the epidemiologic features of HSP in children were similar to those described in previous studies, but GI and joint symptoms manifested more frequently. It is essential to carefully monitor renal involvement and progression to chronic renal disease in patients ≥7 years old and in patients affected by severe GI symptoms. It can be assumed that there is no direct association between early doses of steroids and prognosis.

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“…Several studies have confirmed low plasma factor XIII levels in IgA vasculitis patients with severe abdominal symptoms, but the underlying mechanisms for the decreased factor XIII activity are unknown. Finally, in cases of IgA vasculitis resistant to corticosteroids and IVIg, plasma exchange and leukocytapheresis have been used [ 27 , 28 ].…”

Section: Discussionmentioning

confidence: 99%

Intravenous γ Globulin for Intractable Abdominal Pain due to IgA Vasculitis

Naifa

Totikidis

Alexiadou

et al. 2020

Case Reports in Pediatrics

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IgA vasculitis (formerly known as Henoch–Schönlein purpura or anaphylactoid purpura) is a usually benign vasculitis that affects children of school age. The disease is characterized by the tetrad of palpable purpura, arthralgia/arthritis, abdominal pain, and hematuria. Treatment of IgA vasculitis is mainly supportive, with administration of simple analgesics. Corticosteroids have been shown to reduce and/or ameliorate the occurrence of abdominal pain which may be severe. We present two children with IgA vasculitis and severe abdominal pain despite corticosteroid administration, who responded promptly to intravenous γ globulin (IVIg) with complete resolution of their symptoms and review of the relevant medical literature. Given the toxicity and/or need for long-term administration of other second-line immunosuppressive therapies in corticosteroid-resistant IgA vasculitis, such as rituximab, cyclosporine, cyclophosphamide, azathioprine, or colchicine, we propose that IVIg may be a useful and safe treatment option, although randomized controlled clinical trials are needed in order to clarify its role in the treatment of abdominal pain in IgA vasculitis.

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Plasma exchange therapy for severe gastrointestinal involvement of Henoch Schonlein purpura in children

Cited by 14 publications

References 0 publications

Henoch-Schönlein Purpura Presenting as Severe Gastrointestinal and Renal Involvement with Mixed Outcomes in an Adult Patient

Henoch-Schönlein Purpura Presenting as Severe Gastrointestinal and Renal Involvement with Mixed Outcomes in an Adult Patient

Henoch-Schonlein Purpura in Children Hospitalized at a Tertiary Hospital during 2004-2015 in Korea: Epidemiology and Clinical Management

Intravenous γ Globulin for Intractable Abdominal Pain due to IgA Vasculitis

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