Possible pathogenic role of IgE in Henoch-Schönlein purpura

The incidences of clinical and biological markers of atopy were investigated in 16 children with IgA nephropathy (IgAN) (group A) and in 22 with Henoch-Schönlein purpura nephritis (HSPN) (group B). The incidence of increased plasma IgE levels according to age-matched normal values was significantly higher in group B (17/22, 77%) than in group A (7/16, 44%) (P<0.05). Although not significant, the incidences of positive RAST tests and of a history of typical atopic symptoms were also higher in group B [10/22 (45%) and 11/22 (50%), respectively] than in group A [4/16 (25%) and 5/16 (31%), respectively]. Moreover, IgE deposits were demonstrated by a peroxidase/anti-peroxidase method on cutaneous Langerhans and mast cells in 4 of 6 patients with HSPN. Thus immunoallergy might account, in some cases, for the cutaneous, intestinal and pulmonary signs observed in HSPN, but not in IgAN. We postulate stimulation of IgE-sensitized mast cells by specific antigens in the presence of IgA circulating immune complexes (CIC), release of vasoactive substances, increased capillary permeability and perivascular deposition of IgA CIC.     

Role of β2 integrins in glomerular leucocytes in Henoch-Schoenlein purpura nephritis: an age-matched comparative study with immunoglobulin A nephropathy

SUMMARY: A comparative immunohistological study was performed for the glomerular deposition of complements (C1q and C3c), fibrin/fibrinogen‐related antigen (FRA), the expression of intercellular adhesion molecule‐1 (ICAM‐1), and the infiltration of leucocytes bearing β₂ integrins (leucocyte function associated antigen‐1 (LFA‐1), complement receptor 3 (CR3) and complement receptor 4 (CR4)) on renal biopsy specimens from 49 cases with Henoch‐Schoenlein purpura nephritis (HSPN), and 49 age‐matched cases with immunoglobulin A nephropathy (IgAN). the glomerular expression of ICAM‐1 was signifcantly correlated with the glomerular infiltration of leucocyte function associated antigen (LFA)‐1⁺ leucocytes in both diseases, and with that of CR3⁺ leucocytes in HSPN. the expression of ICAM‐1 was closely localized with the infiltration of LFA‐1⁺ leucocytes in the study with double immunostaining. the incidence and intensity of glomerular deposition of FRA were significantly higher in HSPN than in IgAN (P< 0.001), and those of C3c were significantly lower in HSPN than in IgAN (P< 0.001). the glomerular deposition of FRA was significantly correlated with the glomerular infiltration of CR4⁺ leucocytes in HSPN (P<0.05) but not in IgAN. In contrast, the glomerular deposition of C3c was significantly correlated with the glomerular infiltration of CR4⁺ leucocytes in IgAN (P<0.05), but not in HSPN. Studies with double immunostaining revealed a close association of CR4⁺ leucocytes with FRA deposition in HSPN and with C3c deposition in IgAN, respectively. the number of glomerular leucocytes bearing β₂ integrins was significantly correlated with urinary protein at the time of renal biopsy in both diseases. These results suggested the differential roles of β₂ integrins in the induction of glomerular injury in HSPN and IgAN. the ICAM‐1/LFA‐1 interaction may commonly be involved in the glomerular infiltration of leucocytes in both diseases. the ICAM‐1/CR3 interaction may be involved only in HSPN. Complement receptor 4 may function as a fibrin/fibrinogen receptor in HSPN, while CR4 may function as a complement receptor in IgAN.     

What is the difference between IgA nephropathy and Henoch-Schönlein purpura nephritis?

Henoch-Sch?nlein purpura nephritis (HSPN) and IgA nephropathy (IgAN) are considered to be related diseases since both can be encountered consecutively in the same patient, they have been described in twins, and bear identical pathological and biological abnormalities. Apart from the presence of extrarenal clinical signs found only in HSPN, other differences are noticed between the two diseases. The peak age ranges between 15 and 30 years for a diagnosis of IgAN, whereas HSPN is mainly seen in childhood. Nephritic and/or nephrotic syndromes are more often seen at presentation in HSPN. In contrast to IgAN, HSPN has been described in association with hypersensitivity. Endocapillary and extracapillary inflammations as well as fibrin deposits in the glomerulus are more frequent in HSPN. No major biological differences have been found between the two illnesses, except for a larger size of circulating IgA-containing complexes (IgA-CC) and a greater incidence of increased plasma IgE levels in HSPN. As tissue infiltration by leukocytes is a major feature of HSPN vasculitis, a possible role of a more potent activation of the latter cells by IgA-CC and/or circulating chemokines in HSPN should be considered. Further studies are required to elucidate this possible mechanism as well as the role of hypersensitivity in HSPN.     

Clinical manifestations of Henoch–Schönlein purpura nephritis and IgA nephropathy: comparative analysis of data from the Japan Renal Biopsy Registry (J-RBR)

Background

The clinical presentation of Henoch–Schönlein purpura nephritis (HSPN) has not been thoroughly investigated among patients of different ages. We therefore compared the features of HSPN and IgA nephropathy (IgAN) based on data from the Japan Renal Biopsy Registry (J-RBR).

Methods

This cross-sectional study analyzed data from patients who were registered in the J-RBR between 2007 and 2012. Clinico-pathological findings at diagnosis were compared among children (aged ≤18 years), adult (aged 19–64 years) and elderly (aged ≥65 years) patients with HSPN (n = 513) and IgAN (n = 5679).

Results

The age at diagnosis considerably differed between HSPN and IgAN; HSPN peaked at 1–19 and at 60–69 years, whereas IgAN peaked at 30–39 years. The clinical features were significantly more severe for HSPN than IgAN, especially proteinuria (children, 1.28 vs. 0.57; adult, 1.95 vs. 1.05; elderly patients, 2.71 vs. 1.64 g/day), and low albumin levels (children, 3.72 vs. 4.13; adults, 3.62 vs. 3.99; elderly patients, 3.07 vs. 3.57 g/dL). The rate (%) of histologically classified endocapillary proliferative or crescentic glomerulonephritis was higher in patients with HSPN than with IgAN. Multiple regression analysis revealed that low albumin level and high BP were independent factors associated with decreased estimated glomerular filtration rates in adult and elderly patients with HSPN.

Conclusions

Age at HSPN diagnosis was bimodally distributed, and the clinical features of HSPN were more severe than those of IgAN across all age groups.

     

Aberrant glycosylation of IgA1 is inherited in both pediatric IgA nephropathy and Henoch-Schönlein purpura nephritis

Serum galactose-deficient immunoglobulin A1 (Gd-IgA1) is an inherited risk factor for adult IgA nephropathy (IgAN). In this paper, we determined the heritability of serum Gd-IgA1 levels in children with IgAN and Henoch-Sch?nlein purpura nephritis (HSPN), two disorders with clinical phenotypes sharing common pathogenic mechanisms. Serum Gd-IgA1 concentrations were quantified using a Helix aspersa-lectin-based enzyme-linked immunosorbent assay. As a group, 34 children with either disorder (20 with HSPN and 14 with IgAN) had significantly higher Gd-IgA1 levels compared with 51 age- and ethnicity-matched pediatric controls. Serum levels of Gd-IgA1 were also elevated in a large fraction of 54 first-degree relatives of pediatric IgAN and HSPN patients compared with 141 unrelated healthy adult controls. A unilineal transmission of the trait was found in 17, bilineal transmission in 1, and sporadic occurrence in 5 of 23 families when both parents and the patient were analyzed. There was a significant age-, gender-, and household-adjusted heritability of serum galactose-deficient IgA1 estimated at 76% in pediatric IgAN and at 64% in HSPN patients. Thus, serum galactose-deficient IgA1 levels are highly inherited in pediatric patients with IgAN and HSPN, providing support for another shared pathogenic link between these disorders.     

Evaluation of IgA1 O-glycosylation in Henoch-Schönlein Purpura Nephritis Using Mass Spectrometry

BackgroundGlomerular deposition of IgA1 is a common feature of Henoch-Schönlein purpura nephritis (HSPN) and is indistinguishable from that seen in IgA nephropathy (IgAN). Serum IgA1 is abnormally O-glycosylated in IgA nephropathy, which may contribute to the development of glomerular injury. Abnormal O-glycosylated IgA1 was also detected in HSPN using lectin enzyme-linked immunosorbent assay; however, this method cannot provide the exact structural information of O-glycans. Mass spectrometry is an effective means of quantification of O-glycans, and there is no report to evaluate IgA1 O-glycans in HSPN using mass spectrometry.Materials and MethodsWe investigated O-glycosylation profile in serum IgA1 from 7 HSPN recipients, 26 IgAN recipients, 25 recipients with other kidney diseases (OKDs), and 26 normal healthy donors using mass spectrometry.ResultsOf the 14 GalNac-Gal combinations detected using mass spectrometry, the percentage of the only 6GalNAc-2Gal combination was significantly different between HSPN and IgAN. The percentage of GalNAc 3 in HSPN recipients was significantly higher than that in OKDs recipients and healthy donors (P = .0027 and P < .0001, respectively). Inversely, the percentage of GalNAc 5 in HSPN recipients was significantly lower than that in OKDs recipients and healthy donors (P = .0008, P < .0001, respectively). Moreover, the Gal content and the Gal/GalNAc ratio of HSPN recipients were significantly lower than OKDs recipients and healthy donors.ConclusionsExamination of Henoch-Schönlein purpura recipients revealed that the number of GalNAc fell and the Gal attachment to GalNAc was reduced compared to other kidney diseases and healthy donors. The IgA1 O-glycosylation profile of HSPN was very similar to that of IgAN.     

Spectrum of biopsy-proven kidney diseases in northwest China: a review of 30 years of experiences

Purpose

Large-scale, contemporary studies assessing the spectrum of kidney diseases in northwest China are lacking. Therefore, we aimed to assess the profile of 30-year temporal changes in biopsy-proven kidney diseases in northwest China.

Methods

This cross-sectional study included all patients with a native kidney biopsy specimen in the First Affiliated Hospital of Xi'an Jiaotong University between 1989 and 2018. Data on demographic characteristics and pathological diagnosis were extracted from medical records and pathological reports. Changing patterns of kidney diseases over the study period and disease distributions in different gender and age groups were examined.

Results

This study included 13,620 patients with a mean age of 38.5?±?16.5 years and included 58.2% of men. Primary glomerulonephritis (PGN), second glomerulonephritis (SGN), tubulointerstitial disease, and other renal diseases accounted for 79.1, 18.3, 2.4, and 0.2% of all kidney diseases, respectively. In PGN, IgA nephropathy (IgAN) (25.1%) was the most common type, followed by non-IgA mesangial proliferative glomerulonephritis (MsPGN) (24.9%) and membranous nephropathy (MN) (17.4%). The frequency of MN dramatically increased (p?<?0.001) over the course of the study. Lupus nephritis (6.2%) and Henoch-Schönlein purpura nephritis (HSPN) (4.9%) were leading SGN diagnosis. The frequencies of IgAN, non-IgA MsPGN, and HSPN declined, while those of ANCA/pauci-immune glomerulonephritis and diabetic nephropathy significantly increased.

Conclusion

PGN continues to be the predominant kidney disease in northwest China, and IgAN is the most common type. The frequencies of MN and diabetic nephropathy significantly increased. These findings might be explained by behavioral and environmental exposures and provide implications on future hypothesis-driven research.

     

Latex-related allergy in hemodialysis patients

Abstract

Background: Incidence of allergic reactions is increased in hemodialysis (HD) patients. However, the prevalence of latex allergy is not exactly known in HD patients. The aim of this present study is to determine the prevalence of latex allergy in HD patients. Methods: A total of 205 adult HD patients were included in the study. Questionnaires were completed during patient interviews, and their consents were provided. Latex prick test and latex specific IgE test (HY-TEC, K82) were performed. Mean age of the patients was 52?±?14 (25–79) years, and 61% was male. Mean dialysis duration was 38 months, and 21% of them were diabetics. Only five patients had allergic complaints in their histories, and none of them had severe anaphylaxis history. Latex prick test was positive in two patients. Latex specific IgE test was positive in five patients. Overall latex sensitivity was defined as 3.4%. Conclusion: No increased prevalence in latex allergy was determined in HD patients. Latex allergy incidence may be increased in atopic HD patients. Therefore, the use of latex products should be avoided as much as possible especially in atopic HD patients.     

Clinicopathological study of IgA nephropathy in adults: The influence of onset age on clinical and renal histological findings and on the effect of steroid therapy

Background. The prognosis of IgA nephropathy (IgAN) varies according to the patient's age. It usually affects children or young adults. However, the onset age for IgAN may be at middle-age or older. The influence of onset age on the clinical and renal histological findings of adult IgAN was investigated. Methods. We selected 39 IgAN patients in whom renal biopsy was performed within 2 years from the onset. The patients were divided into two groups according to onset age; early-onset group (under 35 years; group E) and late-onset group (over 35 years; group L). The clinical and histological findings and the response to steroid therapy were compared in the two groups. Results. (1) Clinically, the levels of proteinuria and hematuria in groups E and L were not different, but the creatinine clearance was lower in group L than in group E. Hypertension was frequent in group L (66.7%), but not in group E. (2) Histologically, the rate of glomerular obsolescence and the grades of interstitial fibrosis and arterio- and arteriolosclerosis were more advanced in group L than in group E. However, there were no differences in the grade of mesangial cell proliferation or mesangial matrix increase, nor were the rates of glomerular crescent and tuft adhesion formation different between the two groups. (3) The reduction of proteinuria and hematuria after 1-year steroid therapy was similar in the two groups. Conclusion. Onset age does not affect the severity of glomerular lesions and the effect of steroid therapy in the early phase of adult IgAN. However, advanced interstitial fibrosis and arterio- and arteriolosclerosis, which may be related to hypertension or the aging process, lead to impaired renal function in late-onset IgAN patients. Received: March 18, 1998 / Accepted: October 29, 1998     

Characteristics of patients with coexisting IgA nephropathy and membranous nephropathy

Background: Coexistence of IgA nephropathy (IgAN) and membranous nephropathy (MN) in the same patient is rare. Few studies have reported the clinical and pathological features of patients with combined IgAN and MN (IgAN–MN).

Methods: The clinico-pathological features, levels of galactose-deficient IgA1 (Gd-IgA1) and autoantibodies against M-type transmembrane phospholipase A₂ receptor (anti-PLA₂R) in sera were compared among IgAN–MN, IgAN, and MN patients.

Results: Twenty-six patients with biopsy-proven IgAN–MN were enrolled. The mean age at biopsy was 43.6?±?15.9?years, and 65.4% were male. Proteinuria and estimated glomerular filtration rate (eGFR) levels in patients with IgAN–MN were similar to that of MN patients. Compared with the IgAN patients, IgAN–MN patients showed a higher median proteinuria level (4.3 vs. 1.2?g/day, p?2, p?p?=?.801). Percentage of IgAN–MN patients with detectable serum levels of anti-PLA₂R was lower than that of MN patients (38.5% vs. 68.6%, p?=?.011).

Conclusions: IgAN–MN patients display similar clinical features to MN patients and milder pathological lesions than IgAN patients. IgAN–MN patients have similar levels of Gd-IgA1 to those of IgAN patients, and a lower proportion of anti-PLA₂R than MN patients.     

Clinicopathological correlations in nephrotic children with IgA nephropathy

Background. The prognostic significance of nephrotic syndrome (NS) in children with IgA nephropathy (IgAN) is unclear. Methods. NS was found in eight children with IgAN (mean onset age, 9.3 years). The clinicopathological findings of these eight children were investigated. Results. Five patients presented with macroscopic hematuria, while the remaining three were discovered in a school urinary screening program or by chance urinalysis. Six patients developed NS at the onset, and two developed NS later in the course of IgAN. All patients were treated with corticosteroids. At the end of follow-up, heavy proteinuria persisted in four children, one of whom had renal dysfunction at the onset of NS and developed end-stage renal failure, and two of whom developed NS after the onset of IgAN. Proteinuria decreased to less than 1 g/day 3 months after NS in four patients, two of whom showed disappearance of proteinuria afterward. Renal biopsy specimens revealed mesangial proliferation and crescent formation in all patients. The degree of persisting proteinuria was correlated with the presence of glomerular sclerosis, fibrous crescents, tubulo-interstitial changes on light microscopy, and depositions of C3 on immunofluorescence microscopy. Conclusions. Children who developed NS after the onset of IgAN developed renal dysfunction; the prognosis of those who showed chronic histopathological changes on renal biopsy specimens was poor, even in these young children. Received: April 17, 2000 / Accepted: July 4, 2000     

Evidence-based assessment of treatment options for children with IgA nephropathies

We present an evidence-based evaluation of published data on therapy for children with various presentations of the IgA nephropathies--idiopathic IgA nephropathy (IgAN) and Henoch-Schonlein purpura nephritis (HSPN). Particular attention has been paid to the outcome markers used in the studies reviewed, with the best evidence provided by markers highly associated with progressive renal failure. No treatment modality for either IgAN or HSPN in pediatric patients has been shown to be effective by a properly designed and administered randomized controlled trial (i.e., the highest level of evidence--level 1). Lower levels of evidence support the use of a variety of corticosteroid regimens, often in combination with other agents, although there are some conflicting studies in this area. No convincing evidence has been published to date to support the use of fish oil, angiotensin-converting enzyme inhibitors or tonsillectomy for the treatment of children with IgAN or HSPN. Well designed randomized controlled trials in children with the IgA nephropathies need to be undertaken.     

Atopy phenotype in subjects with variants of the beta subunit of the high affinity IgE receptor

BACKGROUND: Fc epsilon RI plays a central role in atopy, thus genetic variants of Fc epsilon RI-beta may alter receptor function to enhance atopic responses and may manifest as a more severe atopic phenotype and more symptomatic atopic disease. The immunological and clinical features of atopy in children with and without the Leu 181 variant of Fc epsilon RI-beta were compared. METHODS: Sixty British nuclear families, including 10 families with the Fc epsilon RI-beta variant Leu 181, recruited via a young proband with atopic asthma were analysed for atopic parameters including total IgE, specific IgE, and clinical atopic disorder. RESULTS: Compared with other children (combined atopic and non-atopic subjects), maternally inherited Leu 181 was associated with increased levels of total IgE (odds ratio (OR) 4.82, 95% confidence interval (CI) 1.02 to 27.66, p < 0.01) and a positive IgE response to grass pollen allergen (OR 7.45, 95% CI 1.56 to 35.52, p < 0.005) but not wheeze (OR 1.97, 95% CI 0.56 to 7.69), asthma (OR 2.25, 95% CI 0.65 to 7.85), or required medications (OR 0.95, 95% CI 0.29 to 3.14). There were trends for each atopic parameter to be more marked in atopic children with maternally inherited Leu 181 than in atopic children without Leu 181. Children with maternal Leu 181 had significantly raised eosinophils but there was no difference in basophil levels compared with other atopic children. CONCLUSIONS: The Leu 181 variant of Fc epsilon RI-beta, or another identified variant in linkage disequilibrium, may promote the development of atopy.




     

Does thin basement membrane disease prolong the urinaryabnormality in minimal change IgA nephropathy?

Background. The renal prognosis of minimal change IgA nephropathy (MC IgAN) is generally a favorable one. It can also be associated with thin basement membrane disease (TBMD). However, occasionally, a persistent urinary abnormality is observed in some MC IgAN patients. This study was conducted to determine whether the complications of TBMD lead to a prolongation of the urinary abnormality in MC IgAN. Methods. We examined serial biopsy specimens from 991 adult patients (aged 16 years and older) with MC IgAN, 481 patients with TBMD, and 266 patients with MC IgAN + TBMD. The characteristics and duration of the urinary abnormality were compared among the three groups using the Kaplan-Meier method. Results. No significant difference was found in the prolongation of urinary abnormality between the MC IgAN and MC IgAN + TBMD groups. The reasons were twofold: The urinary abnormality in TBMD disappeared at an early stage (P < 0.01), and the complication rate of TBMD diffuse type (which sometimes has prolonged urinary abnormality) with MC IgAN was very low in comparison with that in TBMD diffuse type alone (P < 0.0001). Aging and hypertension were identified as factors inducing prolonged urinary abnormality in MC IgAN and TBMD. Conclusion. TBMD excluding the diffuse type, did not have an effect on the abnormal urinalysis of MC IgAN in the clinical course. Received: October 23, 1998 / Accepted: March 2, 1999     

Peripheral blood IgA bearing cells in children with Henoch-Sch?nlein purpura nephritis and IgA nephropathy. Relationship between IgA bearing cells and clinico-pathological findings or T alpha 4 cells]

It has been reported that patients with Henoch-Sch?nlein purpura nephritis (HSPN) and IgA nephropathy (IgAN) showed an familial increase of IgA bearing peripheral blood lymphocyte. To elucidate the relationship between IgA bearing cells and clinico-pathological findings or T cell subsets, especially IgA specific helper T cells (T alpha 4 cells), 20 patients with HSPN and 33 patients with IgAN were studied. The results demonstrated as follows; 1) IgA bearing cells were significantly increased in patients with both HSPN and IgAN (p < 0.001). 2) The increase of IgA bearing cells was well correlated to the degree of patients' proteinuria and hematuria (p < 0.05), and also correlated with the severity of patients' renal pathological findings in both diseases. 3) With relation to the T cell subsets in patients with both diseases, only the CD4+ Leu8- cells (helper T cells) and T alpha 4 cells were significantly increased, in addition, positive correlation between IgA bearing cells and CD4+ Leu8- cells or T alpha 4 cells was observed. 4) The increase of IgA bearing cells seemed to be transient in HSPN, but remained elevated in IgAN. In conclusion, it was indicated that patients with HSPN and IgAN could have IgA related immunological abnormalities, which may be reflected in the increase of IgA bearing cells and T alpha 4 cells. It was also suggested that determination of IgA bearing cells could be a useful parameter which may be reflected in the disease activity of HSPN and IgAN.     

Analysis of infiltrating cells in IgA nephropathy with acute clinical onset

Background. The prognosis of IgA nephropathy (IgAN) patients is worse than that of poststreptococcal acute glomerulonephritis (PSAGN) patients. Some IgAN patients with acute clinical onset exhibit severe glomerular inflammation similar to that seen in patients with PSAGN, although most IgAN has an insidious onset. The aim of this study was twofold: (1) to differentiate glomerular inflammatory responses between IgAN and PSAGN and (2) to differentiate IgAN by its clinical manifestations. Methods. We examined 31 IgAN patients; 10 with acute clinical onset (AIgAN), 6 with acute exacerbation associated with chronic IgAN (ACIgAN), 8 with insidious onset (IOIgAN), and 7 with acute clinical onset who were anti-streptolysin-O positive (ASLOIgAN). We also examined 11 patients with PSAGN. Conventional pathological examinations were done using immunohistopathologic evaluation of infiltrating cells. Results. (1) Injuries to the glomerular basement membrane (GBM) were common and more severe in AIgAN than in PSAGN. The number of activated monocytes/macrophages (BerMac-3) was significantly greater in the glomeruli of ASLOIgAN than in those of AIgAN (P < 0.05). The numbers of T cells (CD45RO), granulocytes (CD15), and myeloperoxidase (MPO)-positive cells were significantly greater in the glomeruli of PSAGN than those of AIgAN in the other two groups (P < 0.05). (2) In regard to clinical manifestations, injury to the glomerular basement membrane (GBM) was most severe in ACIgAN. The number of MPO-positive cells was significantly greater in the glomeruli of AIgAN than in those of ACIgAN or IOIgAN. Conclusion. In AIgAN, glomerular damage was accompanied by severe GBM injury, but this injury was unexplained by the analysis of cell subclasses, in contrast to findings in PSAGN. Regarding the clinical manifestations of IgANs, MPO-positive cells may play some role in the glomeruli of AIgAN. Received: July 27, 1998 / Accepted: October 13, 1998     

A comparison of corticosteroid and warfarin therapy in IgA nephropathy with crescent formation: preliminary trial

Background. No satisfactory treatment exists for IgA nephropathy (IgAN), especially in patients with severe histologic damage. Several trials using steroids combined with other therapies such as warfarin have demonstrated unremarkable results. We investigated the renoprotective effects of warfarin and steroids in IgAN patients with crescent formation. Methods. Fifteen Japanese patients with IgAN were followed for up to 3 years. Crescent formation was recognized in over half of their glomeruli from renal biopsy specimens. Treatments consisted of either 0.5 mg/kg per day of prednisolone, or warfarin monotherapy. Blood pressure was controlled with long-acting calcium channel blockers and alpha-beta blockers. Serum creatinine and urinary protein excretion were evaluated at least every 2 months for 36 months. Results. Ten of the 15 patients completed the study. The serum creatinine levels had increased in both groups by 3 years, but significantly more so in the group treated with warfarin. However, they were not significantly different between the two groups as measured at the beginning and end of the study. Blood pressure for all patients in the study was maintained below 130/85 mmHg. Excluded from the study were 5 patients who experienced either peptic ulcers (n = 2, steroid group) or bleeding problems (n = 3, warfarin group). Conclusions. These results suggest that corticosteroid therapy may assist in preventing deterioration of renal function in patients with IgAN accompanied by crescent formation. However, further study would be required to decide its usefulness. Received: October 3, 2001 / Accepted: October 29, 2002 Correspondence to:H. Suzuki     

The role of sulfatides in autoimmunity in children with various glomerular disease]

Previous studies have suggested that autoimmunity to a number of kidney antigens may exist in glomerular disease. Our own work suggested that sulfatide which is one of the major acidic glycolipids of human kidney may be antigenic. Glycolipids were isolated from lipid extract of human kidney using thin-layer chromatography (TLC). As the major acidic glycolipids, sulfatide, CDH-sulfate, GM3, GD3 were identified. Acidic fraction of lipid extract were chromatographed and then tested for antigen by immunostaining. Sera from patients with IgA nephropathy (IgAN) and Henoch-Sch?nlein purpura nephritis (HSPN) contained antibody to the sulfatide of human kidney as determined by the direct binding of antibody to TLC. In addition, we measured the presence of sulfatide antibodies by enzyme linked immunosorbent assay (ELISA) in sera of patients with various glomerular disease: IgAN, HSPN, mesangial proliferative glomerulonephritis, membranoproliferative glomerulonephritis (MPGN), focal and segmental glomeruosclerosis (FSGS), membranous nephropathy (MN), minimal change nephrotic syndrome (MCNS), acute post streptococcal glomerulonephritis (PSAGN), and lupus nephritis (LN). IgM class sulfatide antibody were demonstrated in many cases of them. The incidence of IgA class sulfatide antibody in HSPN and IgAN was significantly high, and also the high incidence of IgG class sulfatide antibody occurred in IgAN. On the other hand, we evaluated cellular hypersensitivity to sulfatide in IgAN, HSPN, and FSGS using an active E-rosette assay. Positive results occurred in IgAN and HSPN. It was suggested that delayed hypersensitivity to sulfatide may generate an autoimmune inflammatory process. It has been reported that laminin binds specifically to sulfatide. Autoimmunity to sulfatide may disturb the laminin binding and consequently interfere with renal function. These results suggested sulfatide antigen may play important role in occurrence and aggravation of glomerular disease.     

Heparin calcium treated Henoch–Schönlein purpura nephritis in children through inhibiting hyperfibrinolysis

Abstract

Aim: To explore the underlying mechanism of low-molecular-weight heparin calcium therapy on Henoch–Schönlein purpura nephritis (HSPN). Methods: Eighty-nine children with severe HSPN were randomized into control group (treated with conventional therapy, n?=?45) and treatment group (treated with conventional therapy plus low-molecular-weight heparin calcium, n?=?44). The concentrations of plasma fibrinogen (Fg), d-dimer and fibrin degradation products (FDPs) were detected before and after treatment. The urinary red blood cell (RBC) and 24?h proteinuria were determined weekly for assessing the children’s kidney function. Results: Two groups were well-matched at baseline. After 8 weeks of treatment, the clinical outcomes of HSPN and outcome of proteinuria of the treatment group were better than the control group (p?<?0.05); the content of Fg, d-dimer and FDP in plasma of the treatment group were lower than the control group (p?<?0.05); but there was no difference about the curative effect of hematuria and the coagulation function between the two groups (p?>?0.05). Conclusions: Fibrinolytic system may participate in the kidney injury of HSPN children and low-molecular-weight heparin calcium could correct blood hypercoagulability through inhibiting hyperfibrinolysis, and thus improving the blood supply of kidney.     

Fungus-specific IgG and IgE in allergic fungal rhinosinusitis.

OBJECTIVE: Our study goal was to study fungus-specific immunoglobulins G (sIgG) and E (sIgE) in polypoid rhinosinusitis with and without evidence of allergic fungal rhinosinusitis (AFS). STUDY DESIGN AND SETTING: A prospective analysis was conducted of fungal sIgG and sIgE using a 9-mold RAST panel in 13 AFS, 11 AFS-like, and 27 non-AFS polypoid rhinosinusitis patients. Nonpolyp controls included 17 volunteers with allergic rhinitis and 11 with no atopic history. RESULTS: All groups had elevated fungal sIgG levels. Polyps, increasing polyp severity, and AFS were associated with elevated fungal sIgG to a greater number of molds. The AFS group had sIgE elevations (>or=class II) to an average of 5 molds versus only 0.1 in the non-AFS polyp group. Total IgE was 971 U/mL versus 64 U/mL, respectively. CONCLUSIONS: Multiple elevations of fungal sIgE are adequate diagnostic evidence of these fungi when fungal cultures and histologic examinations are negative in diagnosing AFS. The significance of increased fungal sIgG remains unclear. SIGNIFICANCE: Early recognition of AFS may be facilitated by screening polypoid rhinosinusitis patients with total serum IgE and RAST testing.