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

Kawasaki, Yukihiko; Ono, Atsushi; Ohara, Shinichiro; Suzuki, Yuichi; Suyama, Kazuhide; Suzuki, Junzo; Hosoya, Mitsuaki

doi:10.5387/fms.59.15

Cited by 60 publications

(54 citation statements)

References 46 publications

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“…Further, the capillary walls have varying degrees of involvement [14]. The present study showed immune complex depositions in NP group (mainly IgA immune deposits, followed by IgA + IgM immune deposits).…”

Section: Discussionmentioning

confidence: 61%

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A single-center analysis of Henoch-Schonlein purpura nephritis with nephrotic proteinuria in children

et al. 2017

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BackgroundIn children with Henoch-Schonlein purpura nephritis (HSPN), the degree of proteinuria has been proven to be not only a sign of kidney damage, but also an accelerator of kidney disease progression. Nephrotic proteinuria at disease onset has been proposed as a predictor of a poor renal outcome. This study aims to assess the clinical and pathological features of HSPN with nephrotic proteinuria in a single center.MethodsOne hundred thirty-seven patients with HSPN who visited Shanghai Children’s Hospital from January 2009 to December 2013 were retrospectively reviewed. The patients were divided into 2 groups based on the 24-h urinary protein levels: nephrotic proteinuria group (NP group: 24-h urinary protein ≥50 mg/kg) and non-nephrotic proteinuria group (NNP group: 24-h urinary protein <50 mg/kg). In addition, data regarding their sex, age, clinical features, renal pathology, and prognosis were collected.Results(1) There were 34 boys and 20 girls in the NP group with a mean age of 8.39 ± 2.85 years. The peak age of incidence was 6 to 11 years (72.22%). (2) There were 8 cases (14.81%) with joint symptoms and 9 cases (16.67%) with gastrointestinal symptoms in the NP group. According to the analysis of the laboratory test results, the serum albumin and IgG levels of the NP group were significantly lower than that of the NNP group (35.04 ± 8.45 in the NP group vs. 41.55 ± 4.46 in the NNP group, P < 0.0001; 7.68 ± 3.12 in the NP group vs. 9.53 ± 2.74 in the NNP group, P < 0.001, respectively); their blood urea nitrogen and cystatin C levels increased significantly (P < 0.05). (3) The majority of the pathological changes in the NP group were above the International Study of Kidney Disease in Children (ISKDC) grade III (62.97%). The NP group patients with tubulointerstitial injurie with grade 2 and above (50%) were prioritized. Immune complex deposition in the NP group was dominated by IgA. (4) The prognosis of the NP group was in complete remission (A), and their cases did not develop into end-stage renal disease; their prognosis was also associated with clinical classification (P < 0.01) but was not related to pathologic grading and tubulointerstitial injury (P > 0.05).ConclusionThe serum albumin and IgG levels of the NP group were significantly lower; however, their blood urea nitrogen and cystatin C levels were higher. The ISKDC grades were mainly above grade III. The prognosis of the NP group was associated with clinical classification and improved after a timely and early treatment.

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Section: Discussionmentioning

confidence: 61%

“…In our study, the choice of treatment for HSPN depended on both the histological and clinical severity of the patient conditions [13, 14, 17]. All patients received ACE-I and/or ARB, and dipyridamole.…”

Section: Discussionmentioning

confidence: 99%

A single-center analysis of Henoch-Schonlein purpura nephritis with nephrotic proteinuria in children

et al. 2017

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“…In HSP, renal involvement is the most important prognostic factor for the determination of morbidity and mortality and, in contrast to gastrointestinal and joint involvements, it may manifest as a delayed sequel [11][12][13][14]. Generally, the first urinary abnormality occurs after other symptoms [15]. Microscopic hematuria, with or without proteinuria, is the most common renal symptom [15].…”

Section: Discussionmentioning

confidence: 99%

The correlation between cutaneous IgM deposition and renal involvement in adult patients with Henoch-Schönlein purpura

Belli¹,

Derviş²

2014

European Journal of Dermatology

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Background: Henoch-Schönlein purpura (HSP) is a systemic and immune complex-related leukocytoclastic vasculitis. A positive correlation has been found between IgM deposition along with IgA in skin lesions diagnosed by direct immunofluorescence (DIF) and renal involvement with HSP. Objectives: We sought to investigate the relationship between the systemic associations of the disease and the severity of the skin lesions, as well as the immune deposition findings of the lesional skin provided by DIF in patients with HSP. Materials & Methods: Between 2001 and 2012, 47 patients with HSP were investigated at our dermatology clinic. Epidemiologic data, laboratory findings, localizations of the palpable purpura, existence of bulla or necrosis in the skin lesions, accompanying systemic involvement, and DIF findings of skin biopsy specimens were recorded. Results: Of the 47 patients 22 were men and 25 were women (age range 16-88 years, mean 42.2). Among all cases with skin involvement, we found 22 (46.8%) articular involvement, 20 (42.6%) renal involvement and 12 (25.5%) gastrointestinal tract involvement. The frequency of bulla and/or necrosis in the skin lesions was significantly high in patients with gastrointestinal and renal involvement (p<0.05). The risk of renal involvement was significantly high in patients whose DIF examination revealed IgM deposition (p<0.05). No association was found between systemic involvement and IgG, complement 3 (C3) and fibrinogen deposition. Conclusion: Patients with HSP, having bullous and/or necrotic lesions and whose skin DIF investigations reveal IgM deposition along with IgA, should be surveyed for the risk of renal involvement.

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“…Hastalık başlangıcında inatçı döküntü ve şiddetli karın ağrısı görülen olgularda böbrek tutulumu daha sıktır. Böbrek tutulumu geçici izole mikroskopik hematüriden hızlı ilerleyen glomerülonefrite kadar değişen klinik spektrumda karşımıza çıkabilir (11). Hematüri nefritli olguların tamamında görülürken, proteinüri, nefrotik sendrom, HT ve ABY daha az sıklıkta…”

Section: Discussionunclassified

Crescentic Henoch-Schonlein Purpura Nephritis and p-ANCA Positivity in a Case with Advanced Age

Yıldız¹,

Ünlü²,

Çavdar³

et al. 2016

Turk Neph Dial Transpl

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ÖzHenoch-Schönlein purpurası (HSP) sıklıkla çocuklarda görülen, purpurik döküntü, artrit, nefrit ve gastrointestinal semptomlar ile karakterize sistemik küçük damar vaskülitidir. Yazıda perinükleer anti-nötrofil sitoplazmik antikor (p-ANCA) pozitifliği saptanan ileri yaş kresentik HSP nefriti olgusu sunulmuştur.Olgu 78 yaşında ve erkek idi. İdrarda azalma, nefes darlığı, karın ağrısı, eklem ağrısı ve ödem yakınmaları ile hastanemize başvurdu. Fizik bakıda gluteal bölgede ve her iki alt ekstremitede purpura tarzında döküntüler vardı. Böbrek yetmezliği, proteinüri ve hematüri saptandı. Nefroloji kliniğine yatırıldı ve hemodiyaliz tedavisine başlandı. Cilt biyopsisinde damar duvarlarında granüler IgA depolanması ve böbrek biyopsisinde kresentik IgA saptandı. Kresentik HSP nefriti düşünüldü. Serum kompleman düzeyleri normal sınırlarda ölçülen olguda p-ANCA pozitifliği saptandı. Plazmaferez tedavisi ile birlikte damardan yüksek doz metilprednizolon ve siklofosfamid tedavisi uygulandı.HSP sistemik bir küçük damar vasküliti olmasına rağmen serum kompleman düzeyleri genellikle normaldir ve ANCA negatiftir. Literatürde çok nadir de olsa ANCA pozitifliği saptanan HSP olguları bildirilmiştir. HSP'de saptanan ANCA pozitifliğinin nedeni tam olarak bilinmemektedir. Kresentik HSP nefriti olgularında genellikle yüksek doz kortikosteroidlerle kombine edilen immünsüpresif ajanlar ve plazmaferez tedavisi önerilmektedir.anahTaR SÖzCÜklER: Henoch-Schönlein purpurası, Kresentik glomerülonefrit, P-ANCA aBSTRaCT Henoch-Schönlein purpura (HSP) is a vasculitis characterized by purpuric rash, arthritis, nephritis and gastrointestinal symptoms and it is generally seen in children. We report here a case of crescentic HSP nephritis with perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) positivity.Our case was 78-year-old man. He had admitted to hospital with complaints of oliguria, dyspnea, abdominal pain, arthralgia and edema. Purpuric eruptions were observed on the gluteal region and lower extremities on physical examination. He had renal failure, proteinuria and hematuria. Hemodialysis treatment was begun after hospitalisation. Granular deposits of IgA were found in vessel walls of the skin and crescentic IgA nephritis was detected in biopsy. He was considered to be suffering from crescentic HSP nephritis. Complement levels were normal, but p-ANCA was positive. High doses of methylprednisolone and cyclophosphamide were administered in addition to plasmapheresis.Although HSP is a small vessel vasculitis, serum complement levels are generally normal and ANCA is negative. Only a few cases of HSP with ANCA positivity have been reported in the literature. The reason of the ANCA positivity in patients with HSP is not exactly known.High-dose corticosteroids in combination with immunosuppressive agents and plasmapheresis are generally recommended in cases of crescentic HSPN nephritis.kEY wORdS: Henoch-Schönlein purpura, Crescentic glomerulonephritis, P-ANCA

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

Cited by 60 publications

References 46 publications

A single-center analysis of Henoch-Schonlein purpura nephritis with nephrotic proteinuria in children

A single-center analysis of Henoch-Schonlein purpura nephritis with nephrotic proteinuria in children

The correlation between cutaneous IgM deposition and renal involvement in adult patients with Henoch-Schönlein purpura

Crescentic Henoch-Schonlein Purpura Nephritis and p-ANCA Positivity in a Case with Advanced Age

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