Henoch-Schönlein purpura nephritis: an update

Henoch-Sch?nlein purpura (HSP) is a form of systemic vasculitis characterised by vascular wall deposits of predominally IgA typically involving small vessels in skin, gut and glomeruli and associated with purpura, colic, haematuria and arthralgia or arthritis. HSP nephritis (HSPN) leads to chronic renal failure in up to 20% of paediatric patients after 20 years of follow-up in selected series. The risk is related to the initial clinical presentation and the percentage of glomeruli presenting with epithelial crescents. The pathogenesis of HSPN might be related to an increased production of abnormally glycosylated IgA, which is not sufficiently cleared by the liver and leads to the formation of IgA macromolecules, accumulating in the circulation with subsequent deposition in vessel walls and in the glomerular mesangium. HSPN is related to IgA nephropathy. These two diseases can be encountered consecutively in the same patient, have been described in identical twins and bear similar pathological and biological abnormalities. No consensus about treatment has been reached up to now. Recent studies indicate that early treatment with methylprednisolone or a combination of steroids and cytotoxic drugs might prevent evolution to chronic renal failure. CONCLUSION: Despite numerous studies, the pathogeny of Henoch-Sch?nlein nephritis remains incompletely elucidated and controlled therapeutic trials are still needed.     

IgA nephropathy and Henoch-Schönlein purpura nephritis

PURPOSE OF REVIEW: IgA nephropathy and Henoch-Sch?nlein purpura nephritis are common glomerular disorders in pediatrics that can potentially progress to end-stage renal disease in some patients. This review summarizes our current understanding of the pathogenesis of these closely related conditions and discusses the rationale for development of diagnostic tests and prognostic markers. The review also presents the best data for long-term outcome, clinical markers of prognosis, and the results of randomized controlled trials. RECENT FINDINGS: Our understanding of the defective galactosylation of O-linked glycans in the hinge region of human IgA1 and its role in the pathogenesis of IgA nephropathy and Henoch-Sch?nlein purpura nephritis has evolved over the past decade. This review discusses studies that suggest that demonstration of galactose-deficient IgA1 in the serum may become an important diagnostic tool for these conditions. Proteomic techniques for development of biomarkers for diagnosis and prognosis show promise. Although data from randomized controlled trials have failed to support the use of immunosuppressive agents in pediatric IgA nephropathy and Henoch-Sch?nlein purpura nephritis, recent data indicate that angiotensin converting enzyme inhibitor therapy is indicated for reduction of proteinuria. SUMMARY: Childhood IgA nephropathy and Henoch-Sch?nlein purpura nephritis have the potential for serious morbidity, either during childhood or later in adulthood. In the future clinical tests will be used for noninvasive diagnosis and as markers for judging response to treatment, particularly in those individuals at highest risk for eventual progression to end-stage renal disease.     

Encephalopathy in Henoch-Schönlein purpura

Background

Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. Severe central nervous system (CNS) involvement is rare in HSP.

Case characteristics

Three children with features of HSP presented with seizures and CNS dysfunction.

Observation

All three children had abnormalities on neuroimaging; 2 had complete remission but one was left with severe neurological damage.

Message

HSP patients may rarely present with CNS involvement with a prolonged course requiring aggressive treatment.

     

Clinical predictors of self-limited urinalysis abnormality in childhood Henoch-Schönlein purpura nephritis

Background and aim: The majority of patients (85–95%) with Henoch‐Schönlein purpura nephritis (HSPN) suffer from a prolonged course of urinalysis abnormality. We sought to identify favourable prognostic factors predicting a self‐limited urinalysis abnormality within 1 y. Methods: Fifty‐eight HSPN patients admitted to the University Hospital between 1990 and 2003 were retrospectively analysed. Detailed information on clinical and laboratory manifestations on admission and sequential follow‐up clinics were recorded. The χ² or Fisher's exact test were used for univariate analysis, and multiple logistic regression was used for multivariate analysis. Results: The study cohort included 31 boys and 27 girls, with a mean age of onset of 8.0±4.3 y and a median follow‐up duration of 5.9 y (range 1 to 25). Of 58 patients, 39 (67.3%) had a self‐limited urinalysis abnormality within 1 y. On multivariate analysis, onset age less than 9 (p=0.038), low‐grade proteinuria (p=0.044) and interval between purpura onset and renal manifestations less than 2 wk (p=0.005) predicted self‐resolved urinalysis abnormality within 1 y. With two or more predictive factors, the sensitivity for short‐term course was 84.6%, the specificity was 73.7%, and the positive predictive value was 84.8%. Conclusion: A small number of variables were important for detecting a favourable short‐term course of urinalysis abnormality.     

The elevated markers of hypercoagulability in children with Henoch-Schönlein purpura

Twenty-eight children with HSP and 79 healthy children were entered into study. Activities of protein C, free-protein S and antithrombin, activated protein C resistance, levels of fibrinogen. D-dimer, thrombin-antithrombin complex (TAT), prothrombin fragments 1 + 2 (PF(1+2)), and von Willebrand factor antigen (vWAg) and its activity (RiCof) were investigated in acute and recovery phases of HSP and controls. Fibrinogen, D-dimer, TAT, PF(1+2), vWAg, and RiCof levels in patients with HSP during the acute phase were significantly higher than those of recovery phase and of the controls. A significant correlation was detected between severity of disease and TAT, PF(1+2), vWAg, and D-dimer levels.     

Henoch-Schönlein purpura in a child following varicella

Henoch-Sch?nlein purpura (HSP) is one of the most common vasculitis of childhood. It is characterized by nonthrombocytopenic palpable purpura, arthritis, renal and gastrointestinal system (GIS) involvement. HSP is usually triggered by an antigenic stimulus including infectious agents, drugs, cold, insect bite or food. HSP is rarely triggered by Varicella zoster infection. We herein presented a case with HSP following varicella.     

Henoch-Schönlein purpura in north-eastern Turkey

AIM: To evaluate the epidemiological and clinical findings in children with Henoch-Sch?nlein purpura (HSP) admitted during a 10-year period, 1995 to 2004, and to compare them with series from other parts of the world. METHODS: The medical records of all children aged 17 years or less admitted with a diagnosis of HSP to the Department of Pediatrics of Karadeniz Technical University were evaluated retrospectively for epidemiological and clinical features. RESULTS: Of 116 children, 73 (63%) were boys. The mean (SD) age at presentation was 8.9 (3.7) years and one-third of them were older than 10 years of age. Over half the cases presented between September and January. All patients had the typical skin rash. Gastro-intestinal manifestations were seen in 64 (55.1%) and joint manifestations, common during the early course of the disease, in 73 (62.9%). Two patients required laparatomy, one for acute abdomen and the other for bowel resection owing to intussusception. Renal manifestations were observed in 36 (31%), all within 3 months of initial symptoms, and one patient (0.8%) with nephritic syndrome progressed to end-stage renal disease. Five patients had hypertension without urinary findings. Symptoms recurred in eight patients (6.9%) over a period ranging from 2 to 5 months after complete resolution of symptoms. There was a history of a preceding upper respiratory tract infection in 16 (13.7%) and a streptococcal infection was confirmed by throat culture in 12 of the 42 (28.5%) children at presentation. CONCLUSION: HSP is generally benign and self-limiting. Hypertension may be seen during the course of the disease without urinary findings. In this area, it seems to affect older children and there is a relatively lower incidence of renal manifestations.     

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.     

Primary antiphospholipid syndrome presenting as complicated Henoch-Schönlein purpura

A child showing signs of Henoch-Sch?nlein purpura developed a right tibiofibular vascular thrombosis. Antiphospholipid antibody tests were positive for both lupus anticoagulant and anticardiolipin antibodies. This suggests that an antiphospholipid syndrome should be considered in cases of Henoch-Sch?nlein purpura and antiphospholipid antibodies should be measured to determine whether prophylactic antithrombotic measures are needed to prevent thrombotic manifestations.