Henoch-Schonlein purpura

Tizard, E. Jane

doi:10.1136/adc.80.4.380

Cited by 114 publications

(104 citation statements)

References 31 publications

(22 reference statements)

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“…Generally, IgA abnormalities suggest an immunological basis for the pathogenetic mechanisms underlying HSP. 41,42 It may be related to increased production of abnormally glycosylated IgA, which is not sufficiently cleared by the liver and leads to the formation of IgA macromolecules. These accumulate in the circulation and are subsequently deposited on the vessel walls in cutaneous small vessels.…”

Section: Mechanisms Of Primary Cutaneous Vasculitis Anca-associated Cmentioning

confidence: 99%

New algorithm (KAWAKAMI algorithm) to diagnose primary cutaneous vasculitis

Kawakami

2010

The Journal of Dermatology

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Palpable purpura tends to indicate involvement of small vessel vasculitis in the upper dermis. Livedo racemosa, nodular lesion and skin ulceration are indicative of involvement of small to medium-sized vessel vasculitis in the lower dermis to subcutaneous fat. We set out to establish a new algorithm (KAWAKAMI algorithm) for primary cutaneous vasculitis based on the Chapel Hill Consensus Conference classification and our research results, and apply to the diagnosis. The first step is to measure serum antineutrophil cytoplasmic antibodies (ANCA) levels. If myeloperoxidase-ANCA is positive, Churg-Strauss syndrome or microscopic polyangiitis can be suspected, and if the patient is positive for proteinase 3-ANCA, Wegener's granulomatosis is most likely. Next, if cryoglobulin is positive, cryoglobulinemic vasculitis should be suspected. Third, if direct immunofluorescence of the skin biopsy specimen reveals immunoglobulin A deposition within the affected vessels, Henoch-Schö nlein purpura is indicated. Finally, the presence of anti-phosphatidylserine-prothrombin complex antibodies and ⁄ or lupus anticoagulant and histopathological necrotizing vasculitis in the upper to middle dermis (leukocytoclastic vasculitis) indicates cutaneous leukocytoclastic angiitis, whereas if necrotizing vasculitis exists in the lower dermis and ⁄ or is associated with the subcutaneous fat, cutaneous polyarteritis nodosa is indicated. The KAWAKAMI algorithm may allow us to refine our earlier diagnostic strategies and allow for efficacious treatment of primary cutaneous vasculitis. In cutaneous polyarteritis nodosa, warfarin or clopidogrel therapies should be administrated, and in cases that have associated active inflammatory lesions, corticosteroids or mizoribine (mycophenolate mofetil) therapy should be added. We further propose prophylactic treatment of renal complications in patients with Henoch-Schö nlein purpura.

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Section: Mechanisms Of Primary Cutaneous Vasculitis Anca-associated Cmentioning

confidence: 99%

New algorithm (KAWAKAMI algorithm) to diagnose primary cutaneous vasculitis

Kawakami

2010

The Journal of Dermatology

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“…Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis in childhood [1, 2]. The vasculitis may affect several organ systems, including mainly skin, joints, gastrointestinal tract and kidneys.…”

Section: Introductionmentioning

confidence: 99%

Henoch-Schönlein Nephritis: A Nationwide Study

et al. 2009

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Background/Aim: The aim of this retrospective study was to evaluate the presentation, clinical and pathological manifestations and outcome of the Henoch-Schönlein purpura (HSP) nephritis in children. Methods: Clinical and laboratory data of 443 children with HSP nephritis aged between 3 and 16 years from 16 pediatric nephrology reference centers were analyzed retrospectively. The biopsy findings were graded according to the classification developed by the International Study of Kidney Disease in Children (ISKDC). Results: Renal biopsy was performed in 179 of the patients with HSP nephritis. The most common presenting clinical finding in patients who were biopsied was nephrotic range proteinuria (25%) which was followed by nephritic-nephrotic syndrome (23.5%). The biopsy findings according to the ISKDC were as follows: class I: 8.3%; II: 44.1%; III: 36.3%; IV: 6.7%; V: 3.3%; VI: 1.1%. All of the patients who developed end-stage renal disease had nephritic-nephrotic syndrome at presentation. Of 443 patients, 87.2% had a favorable outcome and 12.8% had an unfavorable outcome. The overall percentage of children who developed end-stage renal disease at follow-up was 1.1%. Logistic regression analysis did not show any association of initial symptoms and histology with outcome. Conclusion: In the presented cohort, the presence of crescents in the first biopsy or presenting clinical findings did not seem to predict the outcome of HSP nephritis in children. We conclude that children with HSP nephritis even with isolated microscopic hematuria and/or mild proteinuria should be followed closely.

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“…O envolvimento sistêmico é mais comum na PHS (dois terços dos pacientes apresentam manifestações gastrointestinais e articulares, 20% a 100% cursam com acometimento renal) e possui duração média de 30 dias e recidivas freqüentes (ocorrendo em até 50% dos casos) (10,11) . Histologicamente, raramente ocorre necrose fibrinóide e a IFD revela depósitos de IgA (1,4,11,12) .…”

Section: Discussionunclassified