Efficacy and Safety of Plasma Exchange as an Adjunctive Therapy for Rapidly Progressive IgA Nephropathy and Henoch-Schönlein Purpura Nephritis: A Systematic Review.

Patients with IgA nephropathy (IgAN), including Henoch-Schönlein purpura nephritis (HSP), who present with rapidly progressive glomerulonephritis (RPGN) have a poor prognosis despite aggressive immunosuppressive therapy. The utility of plasmapheresis/plasma exchange (PLEX) for IgAN/HSP is not well established. This systematic review aims to assess the efficacy of PLEX for IgAN and HSP patients with RPGN. A literature search was conducted using MEDLINE, EMBASE, and through Cochrane Database from inception through September 2022. Studies that reported outcomes of PLEX in IgAN or HSP patients with RPGN were enrolled. The protocol for this systematic review is registered with PROSPERO (no. CRD42022356411). The researchers systematically reviewed 38 articles (29 case reports and 9 case series articles) with a total of 102 RPGN patients (64 (62.8%) had IgAN and 38 (37.2%) had HSP). The mean age was 25 years and 69% were males. There was no specific PLEX regimen utilized in these studies, but most patients received at least 3 PLEX sessions that were titrated based on the patient's response/kidney recovery. The number of PLEX sessions ranged from 3 to 18, and patients additionally received steroids and immunosuppressive treatment (61.6% of patients received cyclophosphamide). Follow-up time ranged from 1 to 120 months, with the majority being followed for at least 2 months after PLEX. Among IgAN patients treated with PLEX, 42.1% (n = 27/64) achieved remission; 20.3% (n = 13/64) achieved complete remission (CR) and 18.7% (n = 12/64) partial remission (PR). 60.9% (n = 39/64) progressed to end-stage kidney disease (ESKD). Among HSP patients treated with PLEX, 76.3% (n = 29/38) achieved remission; of these, 68.4% (n = 26/38) achieved CR and 7.8% achieved (n = 3/38) PR. 23.6% (n = 9/38) progressed to ESKD. Among kidney transplant patients, 20% (n = 1/5) achieved remission and 80% (n = 4/5) progressed to ESKD. Adjunctive plasmapheresis/plasma exchange with immunosuppressive therapy showed benefits in some HSP patients with RPGN and possible benefits in IgAN patients with RPGN. Future prospective, multi-center, randomized clinical studies are needed to corroborate this systematic review's findings.

[IgA vasculitis - similarities and differences to IgA nephropathy]. / Die IgA-Vaskulitis.

IgA vasculitis - similarities and differences to IgA nephropathy Abstract. IgA vasculitis (IgAV), formerly called Henoch-Schönlein Purpura (HSP) is an immune complex-mediated vasculitis of small vessels typically affecting the skin, gastrointestinal tract, and the kidneys. Based on distinct histopathological and pathophysiological commonalities of IgAV and IgA nephropathy (IgAN) they are viewed as part of a spectrum of IgA-mediated inflammatory syndromes. While the disease course in children is most often benign, IgAV has a high risk of renal and gastrointestinal complications when first appearing in adulthood. Acute morbidity and mortality in IgAV are determined by gastrointestinal complications such as intussusception or perforation, while chronic morbidity depends on renal involvement. Nephritis symptoms can appear, subclinically, many weeks after the initial manifestation of IgAV, and can therefore be missed. Continuous monitoring of renal parameters and blood pressure is therefore recommended even after apparent remission of the disease. As there are no data from randomized controlled trials available, the treatment of IgAV is currently based on consensus-based expert opinions.

[Cutaneous Vasculitides - Clinical Manifestations, Diagnosis, and Aetiology]. / Kutane Vaskulitiden.

Cutaneous Vasculitides - Clinical Manifestations, Diagnosis, and Aetiology Abstract. Vasculitides are a heterogeneous group of diseases that are classified differently, for example according to the size of the affected vessel or according to primary and secondary causes. The skin is most frequently affected; it can be involved both as single organ vasculitis and in the context of systemic forms. The combination of skin lesions, their anatomical location and information on the time course provide clues for a differential diagnosis. Purpura, blisters, necrosis, ulcerations and possibly a livedo are characteristic manifestations. Constitutional symptoms such as weight loss, exhaustion, fever, and arthralgias are indicative of a systemic form. It is important to differentiate vasculitides from vasculopathies, which can manifest similarly. The most common form in adults is cutaneous leukocytoclastic angiitis, in children IgA vasculitis (Schönlein-Henoch purpura). Various triggers are possible: infections, drugs, autoimmune diseases, and malignancies, whereby up to 50% remain etiologically unexplained. Skin biopsies and laboratory parameters, if necessary supplemented with imaging, are important steps in the clarification process. Treatment is primarily directed at the elimination of a possible triggering cause. Idiopathic cutaneous leukocytoclastic angiitis usually resolves spontaneously; treatment is symptomatic. In more severe cases, topical corticosteroids or calcineurin antagonists are primarily used. In case of therapeutic resistance, systemic immunosuppressants are recommended.

[Selection of enteral nutrition regimens for children with abdominal Henoch-Schönlein purpura].

OBJECTIVE: To explore the reasonable and effective enteral nutrition regimen for children with abdominal Henoch-Schönlein purpura (HSP). METHODS: A retrospective analysis was performed on the medical data of children with abdominal HSP who were hospitalized from August 2013 to August 2018. According to the starting time of enteral nutrition after abdominal pain relief, the children were divided into three groups: < 24 hours (n=68), 24-48 hours (n=64), and 48-72 hours (n=60). According to the type of enteral nutrition, they were divided into another three groups:amino acid-based formula (n=53), extensively hydrolyzed lactoprotein formula (n=67), and normal diet (n=72). The recurrence rate of clinical symptoms and degree of satisfaction among family members were compared between groups. Based on the retrospective analysis, 166 children with abdominal HSP were enrolled in a prospective study. They were given extensively hydrolyzed lactoprotein formula after abdominal pain relief. According to the feeding time after abdominal pain relief, they were divided into three groups: < 24 hours (n=52), 24-48 hours (n=59), and 48-72 hours (n=55). The three groups were compared in terms of the recurrence rates of abdominal pain, rash, and hematochezia, the rate of use of parenteral nutrition and intravenous steroids, and the incidence rate of weight loss at discharge. RESULTS: The retrospective analysis showed that the children who were given extensively hydrolyzed lactoprotein formula for enteral nutrition at 24-48 hours after abdominal pain relief had a lower recurrence rate of clinical symptoms and the highest degree of satisfaction among their family members (P < 0.0167). The prospective study showed that the children who were given extensively hydrolyzed lactoprotein formula for enteral nutrition at 24-48 hours after abdominal pain relief had lower recurrence rates of rash and abdominal pain, a lower rate of use of parenteral nutrition, and a lower incidence rate of weight loss at discharge (P < 0.05). CONCLUSIONS: It is reasonable and effective to start the feeding with extensively hydrolyzed lactoprotein formula at 24-48 hours after abdominal pain relief in children with abdominal HSP.

Rituximab in severe immunoglobulin-A vasculitis (Henoch-Schönlein) with aggressive nephritis.

OBJECTIVES: Immunoglobulin-A vasculitis (IgAV) is a systemic small-vessel vasculitis in which renal involvement indicates severity of illness, and chronic kidney disease represents the most serious long-term complication. No treatment at present is specifically recommended for IgAV. Recently, rituximab (RTX) has been shown to be effective in case series of adults with IgAV. However, long term results are lacking. Aim of the study is to evaluate the effectiveness of RTX as induction therapy and maintenance of remission in adults with severe IgAV and aggressive glomerulonephritis. METHODS: This study included 12 adult-onset patients, 8 males and 4 females, mean age 45.1 years (range 19-75) with a mean follow-up duration of 33.7 months (range 6-144). All patients had a severe IgAV with biopsy proven crescentic nephritis. RTX was given for the treatment of a refractory disease or because of definite contraindications to standard therapies. RESULTS: Eleven patients (91.7%) achieved a clinical response at 6 months. Ten patients had a complete response (CR) while one had a partial response and was given an additional dose of RTX after 12 months for persistent proteinuria (1gr/24 hrs) despite systemic remission. He achieved a CR 6 months later. One patient was considered unresponsive to RTX and was switched to MMF. Among the 10 patients with CR, 1 needed maintenance doses of RTX every 6 months for iterative relapsing of severe purpura, 1 relapsed after 15 months and received a new induction course showing a CR again. A significant decrease in BVAS (p=0.031) and 24-hour-proteinuria (p=0.043) from RTX initiation through the last follow-up has been detected. One patient, who had a CR with RTX alone died after 6 months for therapy-unrelated cardiovascular cause. CONCLUSIONS: RTX proved to be effective and safe for induction and maintenance of long-lasting remission in severe IgAV with aggresive renal involvement. Data also suggest that RTX can be indicated not only for refractory cases, but can be also proposed as a first line therapy.

Henoch-Schönlein Purpura (IgA Vasculitis): Rapid Evidence Review.

Henoch-Schönlein purpura, now called immunoglobulin A (IgA) vasculitis, is a systemic, immune complex-mediated, small-vessel leukocytoclastic vasculitis characterized by nonthrombocytopenic palpable purpura, arthritis, and abdominal pain. It is the most common vasculitis in children but can also occur in adults. Diagnostic testing is required only to exclude other etiologies of purpura, to identify renal involvement, and, if indicated, to determine its extent with biopsy. Imaging or endoscopy may be needed to assess organ complications. IgA vasculitis spontaneously resolves in 94% of children and 89% of adults, making supportive treatment the primary management strategy. However, a subset of patients experience renal involvement that can persist and relapse years later. Additional complications can include gastrointestinal bleeding, orchitis, and central nervous system involvement. Systematic reviews have shown that steroids do not prevent complications and should not be used prophylactically. However, randomized trials have demonstrated success with high-dose steroids, cyclosporine, and mycophenolate in treating glomerulonephritis and other complications. Long-term prognosis depends on the extent of renal involvement. Six months of follow-up is prudent to assess for disease relapse or remission.

Changes of inflammatory mediators and oxidative stress indicators in children with Henoch-Schönlein purpura and clinical effects of hemoperfusion in the treatment of severe Henoch-Schönlein purpura with gastrointestinal involvement in children.

BACKGROUND: To explore the changes of inflammatory and oxidative stress responses in Henoch-Schönlein purpura (HSP) children, and further analyzed the therapeutic effects and mechanisms of hemoperfusion (HP) on HSP with severe gastrointestinal (GI) involvement. METHODS: There were 200 children with HSP were divided into three groups according to their clinical manifestations: 60 in HSP without GI and renal involvement group, 60 in HSP with GI involvement group, and 80 in HSPN group. The HSP with GI involvement group was subdivided into conventional treatment (n = 30) and HP (n = 30) groups. Thirty children who visited the department of children healthcare for healthy physical examinations from January to December 2017 were set as healthy control group. The IL-6 and TNF-α levels were detected by chemoluminescence method. The MDA, SOD and T-AOC levels were determined by thiobarbituric acid colorimetric method, hydroxylamine method and chemical colorimetry. RESULTS: Compared with healthy group, IL-6, TNF-α and MDA levels in HSP were increased in each group, while SOD and T-AOC were decreased (P = 0.000). IL-6, TNF-α and MDA levels in the HSPN group were the highest, SOD and T-AOC levels were the lowest (P = 0.000). Compared with those before treatment, IL-6, TNF-α and MDA levels in the conventional and HP groups were decreased and SOD and T-AOC levels were increased (P = 0.000). The changes in HP group were more significant than those in conventional group (P < 0.047). Compared with conventional group, glucocorticoid dosage and the occurrence rate of hematuria and/or proteinuria within 3 months were lower in HP group. (P = 0.000, 0.004). CONCLUSIONS: Inflammatory and oxidative stress may be involved in the acute phase of HSP children. The intensity of inflammatory and oxidative stress responses were related to the degree of renal involvement. HP can reduce glucocorticoid dosage and the rate of renal involvement in children with severe HSP with GI involvement. The mechanism may be related to the fact that HP can effectively remove IL-6, TNF-α, MDA in HSP children.

Successful treatment of hemorrhagic bullous Henoch-Schonlein purpura with intravenous immunoglobulins.

Henoch-Schonlein purpura (HSP) is the most common systemic vasculitis in childhood. There is no consensus about the management for isolated cutaneous manifestations in HSP. We describe a case of HSP presenting with severe skin lesions that did not respond to standard therapy with corticosteroids. The 11-year-old child was treated with intravenous immunoglobulins, which induced rapid and persistent resolution of symptomatology.

[Clinical effect and mechanism of hemoperfusion in treatment of children with severe abdominal Henoch-Schönlein purpura].

OBJECTIVE: To study the clinical effect and mechanism of hemoperfusion (HP) in the treatment of children with severe abdominal Henoch-Schönlein purpura (HSP). METHODS: A total of 24 children with severe abdominal HSP were divided into two groups: conventional treatment and HP (n=12 each). Ten healthy children who underwent physical examination were enrolled as the control group. Before and after treatment, chemiluminescence was used to measure the serum levels of interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α); thiobarbituric acid colorimetry was used to measure the plasma level of malondialdehyde (MDA); the hydroxylamine method was used to measure the plasma level of superoxide dismutase (SOD); chemical colorimetry was used to measure the plasma level of total anti-oxidant capability (T-AOC). RESULTS: Compared with the control group, the conventional treatment and HP groups had significantly higher IL-6, TNF-α, and MDA levels and significantly lower SOD and T-AOC levels before treatment (P<0.05), but there were no significant differences between the conventional treatment and HP groups (P>0.05). After treatment, the conventional treatment and HP groups had significant reductions in IL-6, TNF-α, and MDA levels and significant increases in SOD and T-AOC levels (P<0.05). The HP group had significantly greater changes than the conventional treatment group; however, there were still significant differences in these indices between the HP and control groups (P<0.05). Compared with the HP group, the conventional treatment group had a significantly lower percentage of children with disappearance of digestive tract symptoms at 4 days after treatment and significantly longer time to disappearance of rash and digestive tract symptoms (P<0.05). Compared with the conventional treatment group, the HP group had a significantly lower amount of glucocorticoid used during treatment and a significantly lower percentage of children who experienced hematuria and/or proteinuria within 6 months of the disease course (P<0.05). There were no significant differences between the two groups in length of hospital stay and recurrence rates of rash and abdominal pain within 6 months of the disease course. CONCLUSIONS: HP can reduce the amount of glucocorticoid used during treatment and the incidence rate of kidney injury in children with severe abdominal HSP, possibly by eliminating IL-6, TNF-α, and MDA.

Evolution of IgA nephropathy into anaphylactoid purpura in six cases--further evidence that IgA nephropathy and Henoch-Schonlein purpura nephritis share common pathogenesis.

BACKGROUND: As the morphological and immunohistochemical manifestations of immunoglobulin A (IgA) nephropathy and Henoch-Schonlein purpura nephritis (HSPN) are very similar, they are considered to share a common pathogenesis. Although HSPN usually develops after the appearance of anaphylactoid purpura, we have encountered patients whose renal symptoms preceded purpura. METHODS: We reviewed the clinical courses of patients who were first diagnosed with IgA nephropathy, but developed purpura later, at the National Center for Child Health and Development in Tokyo, Japan. RESULTS: Of the 53 patients who were diagnosed with primary IgA nephropathy at our institute during the study period (March 2002 to July 2015), six (11 %) developed anaphylactoid purpura after the diagnosis of primary IgA nephropathy and therefore met the inclusion criteria. Duration between the onset of nephritis and subsequent appearance of purpura ranged from 5 months to 14 years. One patient reached end-stage renal failure due to IgA nephropathy and developed purpura after renal transplantation. All renal biopsies performed before the appearance of purpura showed mesangial proliferation with predominant IgA deposits. Urinary findings deteriorated in three patients after the appearance of purpura, including one patient who developed rapidly progressive glomerulonephritis. Renal biopsy findings worsened in two patients. At the last observation, two patients showed mild renal insufficiency. CONCLUSIONS: Our clinical experience and previous reports support the argument that IgA nephropathy and HSPN are different manifestations of a single disease. Hence, it is acceptable to consider that they are variants of a single disease.

Successful treatment of recurrent Henoch-Schönlein purpura nephritis in a renal allograft with tonsillectomy and steroid pulse therapy.

We report a case of recurrent Henoch-Schönlein purpura nephritis (HSPN) treated successfully with a tonsillectomy and steroid pulse therapy in a kidney transplant patient. A 29-year-old woman was admitted to our hospital for an episode biopsy; she had a serum creatinine (S-Cr) of 1.0 mg/dL and 1.34 g/day proteinuria 26 months after kidney transplantation. Histological examination revealed increased amounts of mesangial matrix and mesangial hypercellularity with IgA deposition. Of note, one glomerulus showed focal endocapillary proliferation and tuft necrosis. We diagnosed active recurrent HSPN. Considering both the histological findings and refractory clinical course of the native kidney, she was treated for 3 consecutive days with steroid pulse therapy and a tonsillectomy. The patient's proteinuria decreased gradually to less than 150 mg/day 6 months later. A second biopsy 6 years after kidney transplantation showed an excellent response to treatment and revealed a marked reduction in both the mesangial matrix and mesangial hypercellularity, with trace IgA deposition. We conclude that a tonsillectomy and steroid pulse therapy appeared to be useful in this patient with active recurrent HSPN. This paper is the first to report a tonsillectomy and steroid pulse therapy as a therapeutic option for active recurrent HSPN. Further studies are needed to elucidate the efficacy and mechanisms of tonsillectomy with recurrent HSPN in kidney transplant patients.

Acute Genitourinary Swelling and Erythema as Presenting Symptoms of Henoch-Schonlein Purpura.

Henoch-Schonlein purpura (HSP) may present in a variety of ways, most commonly with joint pain or the distinctive palpable purpura. Genitourinary manifestations of HSP are less common and may precede the classic signs and symptoms of HSP, making the diagnosis difficult. We report a case of a 19-month-old boy with penile and scrotal erythema and swelling at presentation who was later diagnosed with HSP.

[Significance of detection of biomarker fecal bile acids in the diagnosis and treatment of childhood Henoch-Schönlein purpura].

OBJECTIVE: To investigate the changes and clinical significance of biomarker fecal bile acids (BA) in children with Henoch-Schönlein purpura (HSP). METHODS: Nineteen children with HSP and twenty-seven healthy children were enrolled in this study. The stool samples were obtained at the acute and remission phases. Fecal BA levels were measured by high performance liquid chromatography mass spectrometry (HPLC-MS). RESULTS: The fecal cholic acid level in the HSP remission group was significantly higher than in the HSP acute group and the healthy control group (P<0.016). The fecal chenodeoxycholic acid level in the HSP remission group was significantly higher than in the healthy control group (P<0.016). The levels of fecal secondary colonic bile acids, deoxycholic acid and lithocholic acid, in the HSP acute and remission groups were significantly lower than in the healthy control group(P<0.05, P<0.016 respectively). No significant differences were found in the levels of fecal urosodeoxycholic acid among the three groups (P>0.05). CONCLUSIONS: Fecal secondary colonic bile acids, deoxycholic acid and lithocholic acid, are in decrease in children with HSP at the acute stage, which may be involved in the pathogenesis and treatment outcomes of HSP.

Plasma exchange therapy for severe gastrointestinal involvement of Henoch Schönlein purpura in children.

OBJECTIVES: The aim of this report is to evaluate the plasma exchange as a choice for the management of life-threating gastrointestinal system (GIS) involvement in Henoch-Schönlein purpura (HSP) when refractory to conventional therapies. METHODS: We retrospectively reviewed the medical records of HSP patients who had plasma exchange therapy due to massive GIS involvement. We reported age, gender, initial HSP presentation, etiological or triggering factors and disease course. Treatment modalities, side effects and their outcomes were noted. RESULTS: We reported 7 cases of childhood HSP with severe gastrointestinal involvement refractory to common immunosuppression with systemic steroid and cytotoxic therapy. All patients gave inadequate response to pulse methyl prednisolone or oral prednisolone therapy with ongoing GIS bleeding and severe abdominal pain. Therefore, pulse cyclophosphamide was added to the treatment. Two patients received additional intravenous immunoglobulin (IVIG) therapy. Gastrointestinal manifestations continued and plasma exchange was performed. All patients improved after plasma exchange treatment. CONCLUSIONS: Treatment of GI involvement in HSP with plasma exchange has been mainly based on case reports. According to our data, we propose that, plasma exchange may be a safe and efficient management choice in paediatric HSP patients with massive GIS involvement that are refractory to other therapies.

IgA-Nephropathie und Purpura Schönlein-Henoch - ein breites Spektrum.

IgA nephropathy is the most common glomerulonephritis in Europe. The disease has been discovered in 1968 in Paris by Jean Berger at the Necker-Children's Hospital. Diagnosis is made by kidney biopsy and requires the presence of mesangial deposits of IgA. This form of glomerulonephritis can be seen in children and adults. In childhood, it most frequently presents within the context of Schoenlein-Henoch purpura. In adulthood, the most common form is limited to the kidney. Schoenlein-Henoch purpura can be seen in adults and manifests as a very aggressive vasculitis, usually in the context of a specific drug intake. The underlying pathophysiological concept today is an insufficient glycosylation of the IgA1 hinge region triggering the formation of autoantibodies against this site. Therapeutic options for the disease are limited. Important is optimal blood pressure control. Selected patients will profit from steroid therapy.

[VASCULITIDES IN CHILDHOOD: A RETROSPECTIVE STUDY IN A PERIOD FROM 2002 TO 2012 AT THE DEPARTMENT OF PAEDIATRICS, UNIVERSITY HOSPITAL CENTRE ZAGREB]. / VASKULITISI U DJECJOJ DOBI: RETROSPEKTIVNA STUDIJA U RAZDOBLJU OD 2002. DO 2012. GODINE U KLINICI ZA PEDIJATRIJU KLINICKOG BOLNICKOG CENTRA ZAGREB.

The aim of our study was to analyze clinical features, laboratory findings, treatment, course and outcome of different types of vasculitis in children. All children aged up to 18 years that have been diagnosed with a vasculitis disorder from 2002. to 2012. at the Department of Paediatric, University Hospital Centre Zagreb according to EULAR/PRES/PRINTO criteria were included in the study. Vasculitis was diagnosed in 180 children, 101 girls and 79 boys, mean age 7.19 ± 3.7 years, with an average follow-up of 5.58 ± 3.28 years. Most of the children (155 or 86%) were diagnosed with Henoch-Shönlein purpura (HSP), polyarteritis nodosa (PAN) was diagnosed in 6 children (3.3%), isolated cutaneous leukocytoclastic vasculitis in 5 (2.8%), Takayasu arteritis (TA) and Kawasaki disease in 2 (1.1%) respectively, hypocomplementemic urticarial vasculitis in one patient (0.5%) and other types of vasculitis in 10 (5.5%) patients (vasculitides in systemic connective tissue disorders in 7 and unclassified vasculitides in 3 patients). All patients had elevated inflammatory markers (C-reactive protein and erythrocyte sedimentation rate). Anti-neutrophil cytoplasmatic antibodies (ANCA) were positive only in one patient, suffering from microscopic polyangiitis. Treatment modality in most patients were NSAIDs, while children with kidney or gastrointestinal system affection were treated with glucocorticoids and/or immunosuppresive drugs. Biological therapy (anti-CD20, rituximab) was used in patients with most severe symptoms. One child (0.56%), suffering from microscopic polyangiitis, died due to kidney failure during the follow-up. Forty patients (22.6%) had one disease relapse, while 6 (3.4%) had two relapses. In conclusion, we found some differences in laboratory parameters (e.g. lower incidence of elevated antistreptolysin O titer in HSP) and epidemiological data (e.g. higher prevalence of PAN in female children) in comparison to data from available studies, while other clinical features, laboratory findings, disease outcome and treatment were similar.

[Effects of hemoperfusion treatment on serum IL-23 and IL-17 levels in children with Henoch-Schönlein purpure].

OBJECTIVE: To study the effects of hemoperfusion treatment on serum interleukin-17 (IL-17) and IL-23 levels in children with Henoch-Schönlein purpura (HSP). METHODS: Eighty-seven children who were diagnosed with HSP and who had received hemoperfusion treatment between January 2011 and December 2012 were enrolled. Twenty-seven sex- and age-matched healthy children were recruited as normal controls. Enzyme-linked immunosorbent assay (ELISA) was used to measure serum concentrations of IL-17 and IL-23. RESULTS: The serum IL-23 and IL-17 levels in the HSP group were significantly higher than in the control group (P<0.05). After hemoperfusion treatment, the serum IL-23 and IL-17 levels in the HSP group were significantly reduced to the levels of the control group. Serum serum IL-17 level was positively correlated with serum IL-23 level (P<0.05) in children with HSP. CONCLUSIONS: Hemoperfusion treatment can reduce serum IL-23 and IL-17 levels in children with HSP, suggesting that the treatment may be effective for HSP.

Immunoglobulin A nephropathy in horseshoe kidney: case reports and literature review.

Horseshoe kidney is the most common congenital renal fusion anomaly. Immunoglobulin A nephropathy is a common glomerulonephritis worldwide. However, the co-occurrence of these diseases had not been reported in the literature. We report the first two cases with the occurrence of immunoglobulin A nephropathy in horseshoe kidney. The first case was a 26-year-old male with hypertension and proteinuria (1.4 g/24 h), his pathological finding was primary immunoglobulin A nephropathy. The second case was a 15-year-old female who presented with recurrent peliosis on bilateral lower extremities, haematuria and proteinuria (1.7 g/24 h). Her renal biopsy finding was Henoch-Schonlein purpura nephritis (secondary immunoglobulin A nephropathy). In both cases, renal biopsy was performed by experienced doctors under ultrasonic guidance at the renal upper pole and no postoperative complications were observed. After they were treated based on the renal pathological findings for 6 months, urine protein excretion decreased significantly and blood pressure and serum creatinine stabilized. It is possible that immunoglobulin A nephropathy occurs in a horseshoe kidney patient. Renal biopsy may be valuable and viable for horseshoe kidney patients with heavy proteinuria to identify pathologic type of glomerulopathy and to guide treatment, if renal biopsy is performed by experienced doctors at the renal upper pole under renal ultrasonic guidance.