Management of intestinal bleeding with single‐dose cyclophosphamide in Henoch–Schönlein purpura

In these case series, we report on six children (3 girls, 3 boys) aged 5–13 years with Henoch–Schönlein purpura (HSP) who developed severe gastrointestinal (GI) bleeding resistant to both 2 mg/kg or pulse (10–30 mg/kg) i.v. methylprednisolone. All patients responded to single‐dose (500 mg/m²) i.v. cyclophosphamide (CPA) and none of them developed new GI bleeding after CPA treatment. No patients required surgical intervention. Single high‐dose CPA may be beneficial in HSP with severe GI involvement, in which bleeding is non‐responsive to high‐dose steroids.     

Hemophagocytic lymphohistiocytosis secondary to Varicella zoster infection in a child with Henoch–Schönlein purpura

Hemophagocytic lymphohistiocytosis (HLH) is a fatal, hyper‐inflammatory syndrome that is characterized by untimely activation of macrophages, and manifests as cytopenia, organ dysfunction, and coagulopathy. Secondary HLH can be associated with infection, drugs, malignancy, and transplantation, and is mostly triggered by infection. Herein, we report the case of a patient with Henoch–Schönlein purpura (HSP) who developed severe HLH secondary to Varicella zoster infection.     

Mechanism of onset and exacerbation of chronic glomerulonephritis and its treatment

Immunoglobulin A nephropathy (IgAN) is one of the most common causes of chronic glomerulonephritis (CGN) in the world. The proliferative and crescentic forms of IgA are found in up to 30% of cases and are associated with nephritic‐range proteinuria, accelerated hypertension, and accelerated decline toward end‐stage renal disease. On the other hand, Henoch–Schönlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis involving the capillaries and the deposition of IgA immune complexes. Renal involvement is the principal cause of morbidity and mortality in children with HSP. Two entity diseases are important as renal diseases in childhood. We herein review the mechanism of the onset and exacerbation of IgAN and HSP nephritis (HSPN) and its treatment. As to the pathogenesis, we found that CB4 provoked exacerbation of renal pathologic findings in hyper IgA mice via endothelial injury, γ‐interferon production, and dysfunction of the mesangial pathway and could possibly become one of the factors involved in the mechanism of the onset or evolution of human IgAN. As to the treatment of IgAN and HSPN, we evaluated the efficacy of multidrug combination therapy (prednisolone, warfarin, and dipyridamole, including mizoribine) for diffuse IgAN and the efficacy of methylprednisolone and urokinase pulse therapy plus immunosuppressive drugs for severe HSPN in children. These therapies were effective in ameliorating the proteinuria and histologic severity of patients with IgAN or HSPN. In future, detailed investigations into the pathogenesis of CGN and double‐blind randomized control studies on children with IgAN or HSPN will be necessary.     

Haemorrhagic bullous lesions in a 3-year-old girl with Henoch-Schölein purpura

Henoch–Schönlein purpura (HSP) is the most common vasculitis in children and is characterized by cutaneous purpura, arthritis, abdominal pain and nephritis. We report a case involving a 3‐year‐old girl with HSP who displayed rapidly evolving haemorrhagic bullae from primary purpuric lesions during systemic corticosteroid therapy. Conclusion: The bullae disappeared within 7 days of systemic corticosteroid therapy, although some scarring of the skin occurred. Also, bullae should not be considered as a poor prognostic factor of renal outcome in HSP.     

Scrotal involvement in childhood Henoch-Schönlein purpura

Aim: Henoch‐Schönlein purpura (HSP) is a common childhood systemic vasculitis involving the skin, gastrointestinal tract, joint, kidneys and even scrotum. Methods: We retrospectively reviewed the clinical and laboratory data of 120 male patients with HSP and also evaluated the risk factors for scrotal involvement and the relation between scrotal involvement and other clinical features. Twenty‐six out of 120 boys (21.7%) diagnosed with HSP had scrotal involvement. Results: Scrotal symptoms manifested as swelling in 88.5% and pain (or tenderness) in 69.2% of HSP patients with scrotal involvement. Neurologic symptoms, mainly headache and localized edema among various manifestations and high serum C3 level of laboratory profiles were more frequently observed in scrotal‐involved group than in those of non‐involved group. However, there was no difference in the outcomes of scrotal symptoms according to therapeutic modalities and the occurrence of scrotal involvement had no correlation with renal involvement from acute to chronic phase. Conclusions: We found that neurologic symptoms, localized edema and high serum C3 level show a significant relation with scrotal involvement in male HSP patients. Because scrotal involvement in male HSP patients is not rare, the accurate early diagnosis of HSP is mandatory by the early notification of purpura and imaging evaluations in order to avoid unnecessary procedures.     

Gastrointestinal manifestations in Henoch-Schönlein purpura: a review of 261 patients

Aim: Henoch‐Schönlein purpura is an IgA‐mediated autoimmune vasculitis of children. It often presents with symptoms including purpuric rash, abdominal pain, renal involvement or arthritis. Abdominal pain is a frequent symptom in children with HSP and raises the suspicion of intussusception or perforation. We sought to evaluate abdominal pain via stool occult blood and image studies. Methods: A retrospective study of 261 patients diagnosed with Henoch‐Schönlein purpura from December 1991 to December 2001 was conducted. Image studies, including abdominal echo, abdominal CT and panendoscopy, were performed for patients who suffered from abdominal pain. Results: Of the 261 patients, 151 (58%) had abdominal pain, and 46 (17.6%) suffered either overt gastrointestinal bleeding or had positive stool occult blood. Seven patients had gross bloody stools. One acute intussusception and one bowel perforation were noted. One patient suffered from hypovolemic shock due to massive gastrointestinal bleeding. When stool occult blood was 3+ or 4+, the incidence of a positive image finding was high. Conclusion: We found that stool occult blood and image studies may be necessary regarding severe gastrointestinal involvement. Ultrasonography is an important tool when intussusception or bowel perforation is suspected. Monitoring the vital signs is important, especially in patients with massive gastrointestinal bleeding.     

Methylenetetrahydrofolate reductase C677T polymorphism in patients with Henoch‐Schönlein purpura

Aim: Associations between several vascular diseases such as Kawasaki disease, venous and arterial thromboembolism, cardiovascular disease, diabetic nephropathy, focal segmental glomerulosclerosis and methylenetetrahydrofolate reductase (MTHFR) C677T polymorphism have been reported. This is a clinical study designed to investigate the possible effects of MTHFR C677T polymorphism on the development of Henoch‐Schönlein purpura (HSP). Methods: Forty‐one patients with HSP (25 male/16 female) with a mean age of 7.8 ± 2.9 years were included in the study. The control group consisted of 50 healthy children. MTHFR genotypes were determined by polymerase chain reaction and by Hindf I restriction enzyme analysis and subsequent 3% agarose gel electrophoresis techniques. Results: No significant differences were observed in the distribution of MTHFR genotypes or allele frequencies in the HSP cases versus controls. Plasma homocysteine levels and vitamin B₁₂ levels were almost comparable in the HSP patients and control group without a significant difference. Folic acid levels were within normal limits in the HSP cases and the control group, HSP patients' levels being significantly higher than the control group. No significant relationship was present with the MTHFR genotype and plasma homocysteine, vitamin B₁₂ and folic acid levels in HSP patients. Conclusion: No association with MTHFR gene polymorphism and homocysteine plasma levels could be found in patients with HSP. The results of this study indicate that other mechanisms should be operative in the development of HSP.     

Henoch-Schönlein purpura with hypocomplementemia in children

BACKGROUND: The clinical course and prognosis of Henoch-Schonlein purpura (HSP) associated with hypocomplementemia are not clear. METHODS: The clinical findings of 10 children with HSP and hypocomplementemia were studied. RESULTS: Purpuric rash in all patients, abdominal pain in five, and arthralgia in nine were noted. The findings in HSP were not different from others with HSP. In eight patients, infection preceded hypocomplementemia. Serum levels of CH50, C3 or C4 were depressed variously. Complement levels returned to normal within 5 weeks in all patients. Antistreptolysin-O (ASO) titer was elevated in all patients and nephritis occurred in eight patients. Six patients had generalized edema and hypertension. Macroscopic hematuria occurred in two patients and heavy proteinuria in five patients. One patient was diagnosed as having poststreptococcal acute glomerulonephritis (PSAGN) combined with HSP nephritis according to renal biopsy findings. In three of eight patients with nephritis, abnormal urinary findings continued for more than 1 year. CONCLUSIONS: Hypocomplementemia in children with HSP was transient and was not related to severity of HSP. Incidences of elevated ASO titer and nephritis were high. The nephritis resembled PSAGN during the acute stage and long-term clinical courses varied. These findings suggest PSAGN may be associated with HSP nephritis.     

过敏性紫癜肾脏损害的临床因素分析

目的　探讨过敏性紫癜 (HSP)肾脏损害的临床因素。方法　过敏性紫癜初发患儿 10 0例 ,根据尿常规检查 ,分为尿检正常组 (5 1例 )及紫癜肾组 (4 9例 ) ;紫癜肾组分为一过性尿异常组 (2 7例 )及持续性尿异常组(2 2例 )。观测临床指标 ,并行统计学分析。结果　紫癜肾组年龄、皮疹分布范围、腹痛及消化道出血的发生率及严重程度均高于尿检正常组 ,有显著性差异 (P均 <0 .0 5 )。持续性尿异常组皮疹持续时间、血尿并蛋白尿发生率、2 4h尿蛋白定量均高于一过性尿异常组 ,有显著性差异 (P均 <0 .0 5 )。结论　年龄增长、皮疹分布范围广泛、腹痛、消化道出血是HSP肾脏受累的危险因素 ,而皮疹持续、血尿并蛋白尿、尿蛋白大于 1.0 g/d ,与肾脏持续受累有关     

儿童过敏性紫癜325例回顾性分析

目的 了解儿童过敏性紫癜(HSP)的临床特点,以提高对本病的认识,并指导临床诊治。方法 收集2012 年6 月至2014 年6 月诊断为HSP 的325 例住院患儿的临床资料进行回顾性分析。结果 325 例患儿中,春季和冬季发病人数较多,分别占33.8% 和27.4%;感染为诱发HSP 的主要因素(57.2%);紫癜伴发腹部症状和紫癜伴发关节及腹部症状的患儿肾脏损害发生率分别为60.3% 和48.9%,与单纯紫癜患儿相比差异有统计学意义(P+ 降低。结论 HSP 发病以冬春季节好发,感染为主要诱因,合并消化道症状者更易发生肾脏损害,肾脏损害的病理分级以Ⅲ a 和Ⅲ b 居多。     

Protein Losing Enteropathy as a Manifestation of Henoch-Schönlein Purpura

ABSTRACT. Gastrointestinal manifestations of Henoch-Schönlein purpura (HSP) commonly include abdominal pain and gastrointestinal bleeding. Hypoproteinemia and edema could be related to renal involvement. We report a 14-year-old boy with classical features of HSP manifestated with edema due to severe intestinal protein loss, measured by elevated fecal alpha 1 antitrypsin secretion. The protein losing enteropathy subsided with corticosteroid therapy.     

Henoch-Schönlein purpura nephritis in children: risk factors, prevention and treatment

Aim:  To identify risk factors for a child with Henoch-Schönlein purpura (HSP) either to develop nephritis (HSPN) or to contract progressive course and to obtain the currently available evidence on the efficacy of treatment options in both preventing and treating the established renal disease.
Method:  Review of the literature published over the last two decades.
Results:  Persistent or recurrent purpura, severe abdominal symptoms and an older age proved as the most significant risk factors for later HSPN. The risks of long-term renal impairment are the highest in children having at presentation nephritic/nephrotic syndrome and/or more than 50% of glomeruli occupied by large crescents or sclerosing lesions. Randomized controlled trials (RCT) do not support short course prednisone at presentation of HSP in preventing persistent renal disease. Many uncontrolled studies using various treatment regimens have reported outcomes considered better than expected. However, the data from RCTs are sparse and no treatment options for the established renal disease can be currently recommended based on RCTs.
Conclusion:  Severity and/or duration of extrarenal HSP symptoms and an older age are the most significant risk factors for developing HSPN, whereas clinical and histological severity at HSPN onset are in general predictive of a long-term renal impairment. The existing evidence does not support of short course prednisone in preventing persistent renal disease. A well-designed RCTs are needed in children with moderately severe or rapidly progressive (crescentic) HSPN.     

儿童腹型过敏性紫癜研究进展

过敏性紫癜是儿童时期最常见的一种以小血管炎为主要病变的血管炎性疾病。以消化道症状为主要临床表现的称为腹型过敏性紫癜,部分患儿在皮肤紫癜前出现腹部症状,甚至病程中始终无皮肤紫癜,这部分患儿极易误诊。现就腹型过敏性紫癜的临床诊治进展作一综述。     

过敏性紫癜患儿血清高迁移率族蛋白B1表达及意义

目的　测定过敏性紫癜（HSP）患儿血清高迁移率族蛋白B1（HMGB1）水平, 分析血清HMGB1与实验室指标的相关性, 初步探讨HMGB1与HSP发病及肾损害的关系。方法　纳入2017年12月至2019年12月在重庆医科大学附属儿童医院初诊为HSP患儿83例, 其中45例无肾脏受累（A组）、 38例合并肾脏损害（B组）, 恢复期HSP患儿18例（C组）, 健康对照组20例（D组）, 采用酶联免疫吸附法测定患儿血清HMGB1水平。比较A组、 B组、 C组、 D组之间以及不同肾脏受累程度患儿血清HMGB1水平, 分析血清HMGB1与实验室指标的相关性。结果　（1）A组、B组、 C组、 D组患儿的血清HMGB1水平分别为： 10.71（6.81～16.03） μg/L、 13.77（10.02～22.72） μg/L、 7.58（5.73～10.83） μg/L、 4.96（3.97～5.43） μg/L, A组与B组、 C组、 D组之间的差异均有统计学意义（P＜0.05）。（2）急性期孤立性血尿型（12例）、 孤立性蛋白尿型（13例）、 血尿和蛋白尿型（13例）患儿血清HMGB1水平分别为： 10.98（8.07～16.38） μg/L、 12.81（8.45～20.89） μg/L、 17.39（13.02～27.79） μg/L, 3组之间的差异无统计学意义（P＞0.05）。（3）HSP患儿血清HMGB1水平与白细胞计数、 中性粒细胞百分比、 血清IgA浓度、 D-二聚体水平、 24 h尿蛋白定量之间呈线性正相关, 与淋巴细胞百分比呈线性负相关（P＜0.05）。结论　血清HMGB1表达与HSP肾脏损害有关, HMGB1可能参与HSP急性炎症。     

Early prednisone therapy in Henoch-Schönlein purpura: a randomized, double-blind, placebo-controlled trial

OBJECTIVE: To evaluate the efficacy of early prednisone therapy in preventing renal and treating extrarenal and renal symptoms in Henoch-Sch?nlein purpura (HSP) in a placebo-controlled trial. STUDY DESIGN: A total of 171 patients (84 treated with prednisone and 87 receiving placebo) were included and followed up for 6 months. The endpoints were renal involvement at 1, 3, and 6 months and healing of extrarenal symptoms. The analyses were performed on an intent-to-treat basis. RESULTS: Prednisone (1 mg/kg/day for 2 weeks, with weaning over the subsequent 2 weeks) was effective in reducing the intensity of abdominal pain (pain score, 2.5 vs 4.8; P = .029) and joint pain (4.6 vs 7.3; P = .030). Prednisone did not prevent the development of renal symptoms but was effective in treating them; renal symptoms resolved in 61% of the prednisone patients after treatment, compared with 34% of the placebo patients (difference = 27%; 95% confidence interval = 3% to 47%; P = .024). CONCLUSIONS: The general use of prednisone in HSP is not supported, but patients with disturbing symptoms may benefit from early treatment, because prednisone reduces extrarenal symptoms and is effective in altering (but not preventing) the course of renal involvement.