The Interaction between Circulating Complement Proteins and Cutaneous Microvascular Endothelial Cells in the Development of Childhood Henoch-Schönlein Purpura

Yang, Yao‐Hsu; Tsai, I-Jung; Chang, Chung‐Cheng; Chuang, Ya Hui; Hsu, Hui-Yao; Chiang, Bor‐Luen

doi:10.1371/journal.pone.0120411

Cited by 44 publications

(31 citation statements)

References 37 publications

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“…It is a much more common diagnosis in children younger than 10 years, with mean age 6 years old, as found in the great majority of literature on this subject. [1][2][3][4][5][6][7] There have been some studies describing equal incidence between genders, similar to what we have found. 6 There was an upper airway infection in more than half of the cases, consistent with it being the most commonly pointed trigger.…”

Section: Discussionsupporting

confidence: 82%

Henoch-Schönlein Purpura: What to expect

Gaia¹,

Capela²,

Borges³

et al. 2020

pjnh

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Henoch-Schönlein purpura (HSP) is the most common childhood vasculitis. 1 It is a systemic IgA-mediated leukocytoclastic small-vessel vasculitis, that typically presents with a classic tetrad of features including palpable purpura, arthritis or arthralgia, gastrointestinal complaints and renal disease. 2,3 It has an estimated incidence of 10-20 children per 100,000 per year. 1,4 A male predominance of 1.2-1.5:1 has been described 2,5 , although other studies have found equal predominance between males and females. 6 Most of the cases occur in children less than 10 years old. 4,6,7 HSP is most commonly a self-limited disease with excellent prognosis, although some cases with concomitant nephritis may have serious complications. 1,6 The diagnosis is based on clinical criteria developed by EULAR/PRINTO/ PRES (European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society), presented in 2008, with sensitivity of 100% and specificity of 87% (Table 1). These criteria include a mandatory criterion of purpura or petechiae with lower limb predominance with a minimum of one additional criterion from the following: diffuse abdominal pain with acute onset, histopathology showing leukocytoclastic vasculitis or proliferative glomerulonephritis with predominant IgA deposits, arthritis or arthralgia of acute onset or renal involvement in the form of proteinuria or haematuria. 8 The treatment, which is usually symptomatic, may sometimes demand the use of steroids or other immunomodulators, depending on the severity and organ involvement. 1 The aim of this study was to analyze the clinical, epidemiologic and prognostic factors of children with this diagnostic in our institution. METHODS The authors' institution is a level II Hospital, with a hospital catchment area serving 334 081 inhabitants (population of Vila Nova de Gaia and Espinho), 61 225 of them in the pediatric age range. This was an observational longitudinal retrospective study based on information collected from the clinical records of children with the diagnosis

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Section: Discussionsupporting

confidence: 82%

Henoch-Schönlein Purpura: What to expect

Gaia¹,

Capela²,

Borges³

et al. 2020

pjnh

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“…More than 90% of patients are under 10 years of age, with a mean age of 6 years (1,2). HSP is a leukocytoclastic vasculitis involving small vessels (3). Its clinical presentation includes cutaneous palpable purpura, joint pain, renal involvement, colicky abdominal pain and gastrointestinal bleeding.…”

mentioning

confidence: 99%

Henoch-Schönlein Purpura: A Literature Review

Hetland¹,

Susrud²,

Lindahl³

et al. 2017

Acta Derm Venerol

193

164

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Henoch-Schönlein purpura is the most common childhood vasculitis, but may also affect adults. This article reviews the literature since 2011 on advances in diagnosis, clinical disease manifestations, pathophysiology and treatment of Henoch-Schönlein purpura. The clinical manifestations are thought to arise from IgA depositions in blood vessel walls in the affected organs, mostly skin, gastrointestinal tract, joints and kidneys. Corticosteroids may be effective in rapid resolution of renal manifestations and treating joint and abdominal pain, but they are not proven effective for treating organ manifestations and complications, such as glomerulonephritis, bowel infarction or intussusception. Mycophenolate mofetil or cyclosporine A may be better treatment choices in case of renal involvement. Other immunosuppressive and immunomodulating drugs, such as rituximab and dapsone, are promising, but larger studies are needed to confirm these findings. Cancer screening should be considered in older males diagnosed with Henoch-Schönlein purpura.

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“…In our review it was difficult to make a comparison with these results, since most of our patients had no immunoglobulin analytical data, and in the 6 patients with the data only 2 showed elevated levels. It has also been reported that complement activation by alternative pathways could be related to IgAV pathogenesis, which would be reflected by higher serum levels of these proteins in affected patients [12]. In our review, data concerning this point were collected for only 5 patients, and one case showed a reduction of serum complement levels.…”

Section: Discussionmentioning

confidence: 95%

Rituximab treatment for IgA vasculitis: A systematic review

Chamorro

Carbonell

Canelo³

et al. 2019

Preprint

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Objetives: Immunoglobulin A vasculitis (IgAV) is an inflammatory disease with a controversial treatment based in corticosteroids as first line. To review cases of patients with IgA vasculitis (IgAV) treated with rituximab (RTX) and assess disease characteristics, treatment efficacy and safety.Methods: We conducted a systematic literature review according to PRISMA guidelines. We searched Pubmed, Web of Science, Embase and Scopus, selecting articles with information on IgAV and RTX treatment up to February 2019, with no language limitations. We extracted data on patient characteristics, disease evolution, treatment safety and efficacy. We created a database and analyzed it using statistical software package SPSS v 22.0.Results: We extracted clinical data for 43 IgAV patients treated with RTX. Distribution by sex was similar, and the median age at diagnosis was 16 (range 2 months to 70 years). The majority of patients were diagnosed at a pediatric age (24 patients under 18, 55.81%). The time of disease evolution until RTX administration greatly varied. An important number of patients suffered renal complications (86%) before RTX treatment.The frequency of adverse effects from RTX was low (7%, hypersensitivity with no treatment interruption). In terms of disease evolution, 41 patients (95.3%) presented clinical improvement, 15 patients had recurrence after initial remission (34.9%) and 34 patients (79.1%) achieved full remission after completing treatment. None of the patients treated with RTX and included in our systematic review died.Conclusions: RTX is efficacious in patients with IgAV. A high percentage of patients achieved complete remission with a favorable safety profile.PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist, and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.

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The Interaction between Circulating Complement Proteins and Cutaneous Microvascular Endothelial Cells in the Development of Childhood Henoch-Schönlein Purpura

Cited by 44 publications

References 37 publications

Henoch-Schönlein Purpura: What to expect

Henoch-Schönlein Purpura: What to expect

Henoch-Schönlein Purpura: A Literature Review

Rituximab treatment for IgA vasculitis: A systematic review

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