Crescentic IgA Nephropathy Research Articles

Clinical features of IgA nephropathy with serum ANCA positivity: a retrospective case–control study

BackgroundThe coexistence of IgA nephropathy (IgAN) and antineutrophil cytoplasmic autoantibodies (ANCAs) is relatively rare. Only a few studies have reported the features of these patients.MethodsWe studied the clinical and histological features of 20 ANCA-positive IgAN patients. They were compared with ANCA-negative IgAN patients (n = 40) and ANCA-associated systemic vasculitis (AASV) patients (n = 40) with a randomly selected and matched proportion of crescentic glomeruli. Furthermore, 9 ANCA-positive crescentic IgAN patients out of the 20 cases were compared with two control groups with crescentic nephritis.ResultsANCA-positive IgAN patients showed older age, lower haemoglobin and higher inflammatory indicator levels at baseline, and a higher percentage of general symptoms and pulmonary involvement, compared with ANCA-negative IgAN patients, and were comparable to AASV patients. Histologically, there was a significantly higher percentage of fibrinoid necrosis in glomeruli in ANCA-positive IgAN patients and in AASV patients compared with ANCA-negative IgAN patients (35, 25 and 0%, respectively, P = 0.003). After immunosuppressive therapy, ANCA-positive crescentic IgAN patients were more likely to withdraw from dialysis (75 versus 9.1%, P = 0.03) and not to reach end-stage renal disease within 6 months (11.1 versus 66.7%, P = 0.01) compared with ANCA-negative crescentic IgAN patients.ConclusionsIgAN patients with ANCA positivity showed more severe clinical and histological features when compared with ANCA-negative IgAN patients and were comparable to AASV patients. However, renal prognosis was relatively better in ANCA-positive crescentic IgAN patients after aggressive immunosuppressive therapy in the short term, compared with ANCA-negative patients.

Use of eculizumab in crescentic IgA nephropathy: proof of principle and conundrum?

IgA nephropathy (IgAN) is characterized by a variable clinical course and multifaceted pathophysiology. There is substantial evidence to suggest that complement activation plays a pivotal role in the pathogenesis of the disease. Therefore, complement inhibition using the humanized anti-C5 monoclonal antibody eculizumab could be a rational treatment. We report here a 16-year-old male with the vasculitic form of IgAN who failed to respond to aggressive conventional therapy including high-dose steroids, cyclophosphamide and plasma exchange and who was treated with four weekly doses of 900 mg eculizumab followed by a single dose of 1200 mg. He responded rapidly to this treatment and has had a stable creatinine around 150 µmol/L (1.67 mg/dL) for >6 months. However, proteinuria was unabated on maximal conventional anti-proteinuric treatment, and a repeat renal biopsy 11 months after presentation revealed severe chronic changes. We believe this case provides proof of principle that complement inhibition may be beneficial in IgAN but also that development of chronicity may be independent of complement.

Tubular reabsorption of high, middle and low molecular weight proteins according to the tubulo-interstitial damage marker N-acetyl-β-D-glucosaminidase in glomerulonephritis.

Proteinuria, the hallmark of glomerular diseases, is an independent predictor of end-stage renal disease (ESRD) progression. Proteinuria is a mixture of proteins of different molecular weight (MW) dependent on alterations of glomerular filtration barrier (GFB) and reabsorption impairment by proximal tubular epithelial cells (PTECs). We aimed to evaluate the excretion of different-MW proteins according to the tubulo-interstitial damage marker N-acetyl-β-D-glucosaminidase (NAG) in glomerulonephritides (GNs). In 189 patients [idiopathic membranous nephropathy (IMN) n = 84, primary focal segmental glomerulosclerosis (FSGS) n = 48, crescentic IgA nephropathy (CIgAN) n = 37, minimal change disease (MCD) n = 20] several urinary proteins were measured at biopsy: α2-macroglobulin/creatinine ratio; fractional excretion of IgG, transferrin, albumin and α1-microglobulin, and the NAG/creatinine ratio divided by estimated glomerular filtration rate (eGFR) (NAG/C/eGFR), as NAG excretion is dependent on functioning nephron mass. Protein excretion was compared between 4th vs. 1st quartile of NAG/C/eGFR. In IMN, FSGS and CIgAN high-MW proteins excretion (α2-macroglobulin, IgG) was greater than that of middle- (transferrin, albumin) and low-MW proteins (α1-microglobulin) in 4th vs. 1st quartile of NAG/C/eGFR; the mean fold excretion increase of high-MW proteins in 3 GNs was 74.9, higher than that of middle- (34.8) and low-MW proteins (12.0). Higher excretion of high-MW proteins may be dependent on lower reabsorption by PTECs. By contrast, in MCD the difference in excretion of different-MW proteins is probably due to high GFB selectivity. High-MW protein excretion is dependent on GFB alteration and reduced reabsorption; its prognostic significance is ominous because in several glomerular diseases progression is associated with high-MW protein excretion.

Update on the management of IgA nephropathy

Abstract IgA nephropathy is the commonest primary glomerular disease worldwide. A high prevalence has been noted in Asia including India. The clinical course has a wide spectrum of presentation varies from isolated microscopic hematuria to crescentic glomerulonephritis. The approach of the treatment has to be decided as per the clinical and histopathological manifestation of the disease. Risk assessment is important to determine management and also to balance between the risks of therapy by the selection of patients. Clinical features appear to be the stronger prognostic indicators however certain renal histopathological findings have been associated with an increased risk of progressive disease. There is no definitive therapeutic approach despite of better understanding of pathogenic mechanism of the disease. The expected outcome of therapy is slowing the deterioration in kidney function as well as a reduction in proteinuria and control of blood pressure by suppression of angiotensin II with ACE inhibitors or angiotensin II-receptor blockers (ARBs). The indications for the use of corticosteroid alone or in combination with other immunosuppressive agents e.g. Azathioprine or cyclophosphamide are not well defined. Different regimens have been used, consisting of corticosteroids alone or in combination with other immunosuppressive agents. Despite retrospective studies in IgA nephropathy supporting the use of immunosuppressive therapy other than corticosteroid, few randomized control trials have demonstrated a benefit. Corticosteroid combined with cyclophosphamide or azathioprine can be considered in patients with rapidly progressive disease with crescentic IgA nephropathy. Fish oil can be used in the treatment of IgA nephropathy with proteinuria above 1 g/day despite 3–6 months of optimized therapy with ACE inhibitors or ARBs and blood pressure control.

Prediction of Outcomes in Crescentic IgA Nephropathy in a Multicenter Cohort Study

Crescentic IgA nephropathy (IgAN), defined as >50% crescentic glomeruli on kidney biopsy, is one of the most common causes of rapidly progressive GN. However, few studies have characterized this condition. To identify risk factors and develop a prediction model, we assessed data from patients ≥ 14 years old with crescentic IgAN who were followed ≥ 12 months. The discovery cohort comprised 52 patients from one kidney center, and the validation cohort comprised 61 patients from multiple centers. At biopsy, the mean serum creatinine (SCr) level ± SD was 4.3 ± 3.4 mg/dl, and the mean percentage of crescents was 66.4%± 15.8%. The kidney survival rates at years 1, 3, and 5 after biopsy were 57.4%± 4.7%, 45.8%± 5.1%, and 30.4%± 6.6%, respectively. Multivariate Cox regression revealed initial SCr as the only independent risk factor for ESRD (hazard ratio [HR], 1.32; 95% confidence interval [CI], 1.10 to 1.57; P=0.002). Notably, the percentage of crescents did not associate independently with ESRD. Logistic regression showed that the risk of ESRD at 1 year after biopsy increased rapidly at SCr>2.7 mg/dl and reached 90% at SCr>6.8 mg/dl (specificity=98.5%, sensitivity=64.6% for combined cohorts). In both cohorts, patients with SCr>6.8 mg/dl were less likely to recover from dialysis. Analyses in additional cohorts revealed a similar association between initial SCr and ESRD in patients with antiglomerular basement membrane disease but not ANCA-associated systemic vasculitis. In conclusion, crescentic IgAN has a poor prognosis, and initial SCr concentration may predict kidney failure in patients with this disease.

Effective management of crescentic IgA nephropathy

IgA nephropathy is a common pattern of glomerulonephritis defined by mesangial IgA deposits. Rapidly progressive renal failure is unusual in IgA nephropathy; it may result from acute tubular necrosis or superimposed crescentic nephritis. In this uncommon situation, the risk–benefit balance is most strongly in favour of intense immunosuppressive therapy because if crescentic IgA nephritis is not treated, there will almost inevitably be rapid progression to end-stage renal disease. We report the clinical and histological features of a patient with crescentic IgA nephropathy treated successfully with intensive immunosuppression.

A Rare Case of Aeromonas Hydrophila Catheter Related Sepsis in a Patient with Chronic Kidney Disease Receiving Steroids and Dialysis: A Case Report and Review of Aeromonas Infections in Chronic Kidney Disease Patients

Aeromonas hydrophila (AH) is an aquatic bacterium. We present a case of fifty-five-year-old gentleman with chronic kidney disease (CKD) due to crescentic IgA nephropathy who presented to us with fever. He was recently pulsed with methyl prednisolone followed by oral prednisolone and discharged on maintenance dialysis through a double lumen dialysis catheter. Blood culture from peripheral vein and double lumen dialysis catheter grew AH. We speculate low immunity due to steroids and uremia along with touch contamination of dialysis catheter by the patient or dialysis nurse could have led to this rare infection. Dialysis catheter related infection by AH is rare. We present our case here and take the opportunity to give a brief review of AH infections in CKD patients.

A case of rapidly progressive IgA nephropathy in a patient with exacerbation of Crohn’s disease

BackgroundIgA nephropathy has been reported as a renal involvement in Crohn’s disease. Crescentic IgA nephropathy, which accounts for fewer than 5% of cases of IgA nephropathy, has a poorer prognosis than other forms of crescentic glomerulonephritis. We recently experienced a case of rapidly progressive IgA nephropathy concurrent with exacerbation of Crohn’s disease.Case presentationAn 18-year-old male diagnosed with Crohn’s disease underwent a hemicolectomy 2 years prior previously. He had maintained a state of Crohn’s disease remission with 5-aminosalicylic acid treatment. Four months prior to referral to the nephrology clinic, he experienced non-bloody diarrhea. He simultaneously developed proteinuria and microscopic hematuria with deterioration of renal function. Based on renal biopsy findings, the patient was diagnosed with crescentic IgA nephropathy. Immunostaining for interleukin-17 in renal tissue and previous exacerbated colonic ulcers was positive. Steroid pulse therapy was administered, followed by high-dose glucocorticoid and oral cyclophosphamide therapy. The patient’s renal function recovered and his gastrointestinal symptoms were alleviated.ConclusionsWe report a case of crescentic IgA nephropathy presenting with exacerbation of Crohn’s disease, and present a review of the literature focusing on the pathophysiologic relationship between these two conditions.

Persistent or New Onset Microscopic Hematuria in Patients with Small Vessel Vasculitis in Remission: Findings on Renal Biopsy

Hematuria is considered a sign of active renal disease in patients with small-vessel vasculitis. In patients who are in apparent clinical remission, presence of persistent or new-onset microscopic hematuria may reflect active vasculitis, damage, or other glomerular pathology. We identified 74 patients from the Johns Hopkins Renal Pathology database between 1995 and 2009 with the diagnosis of pauciimmune glomerulonephritis (GN). Among them we identified 9 who were in clinical remission and underwent a renal biopsy for evaluation of persistent or new-onset hematuria. Nine patients with small-vessel vasculitis, 8 antineutrophil cytoplasmic antibody (ANCA)-positive and 1 ANCA-negative, underwent a renal biopsy at variable time periods after remission of vasculitis (6 to 164 months) for persistent microscopic hematuria (n = 6) or new-onset microscopic hematuria (n = 3). All patients were in apparent clinical remission at the time of renal biopsy. Of the 3 patients presenting with new-onset hematuria, 2 had crescentic IgA nephropathy and 1 had healed crescentic pauciimmune GN. Of the 6 patients with persistent hematuria, 2 had arteriosclerosis, 2 had focal segmental glomerulosclerosis, and 2 had global and segmental glomerulosclerosis and healed crescentic GN, and none had active vasculitis. Microscopic hematuria in patients with renal vasculitis otherwise in remission could represent chronic glomerular injury from prior episode of vasculitis or may represent new glomerular pathology. Renal biopsy should be considered in these patients to guide therapy.

A New Biomarker with High Outcome Prediction in IgA Nephropathy

Biomarkers able to predict progression and treatment responsiveness are of paramount importance in glomerulonephritis. In IgA Nephropathy (IgAN) at present the most powerful prognostic predictor is the level of proteinuria; the predictive value of proteinuria may be enhanced by measurement of its high molecular weight components which are reliable markers of alteration of glomerular filtration barrier. Aim of the review is to summarize recent literature on predictive value of functional outcome in IgAN patients of the new biomarker FE IgG/SG: Fractional Excretion of IgG divided by non obsolescent glomeruli defined Surviving Glomeruli (SG). In 132 non-crescentic IgAN patients, FE IgG/SG < vs ≥ 0.00010 predicts progression (ESRD and eGFR reduction ≥50%) in 2% vs 87.5% of 68 untreated and in 0%vs36% of 64 patients treated with ACE-inhibitors. In patients with SG < 80% (57 + 14%) progression rate is 100% vs 0% if FE IgG/SG is ≥ vs < 0.00010. In 34 crescentic IgAN patients FE IgG/SG < vs ≥ 0.00034 predicts progression in 5%vs83% and in 0%vs89% of 23 patients treated with Steroids and Cyclophosphamide. In patients with FE IgG/SG ≥ 0.00034 and sCr ≥ 1.74 mg/dL progression prediction is 100%. In conclusion this biomarker based on combination of Fractional Excretion of the high MW protein IgG with nephron loss is a powerful outcome predictor in IgAN patients and represents a validation in a human disease of some progressive mechanisms hypothesized by the “remnant kidney” progression theory. The review also outlines some recent patents on the biomarkers for nephropathy. Keywords: Fractional excretion of IgG, IgA nephropathy, outcome prediction, surviving glomeruli (SG)

Typhoid fever presenting with type II MPGN

Typhoid fever with classical features presents no difficulty in recognition. However, when it presents atypically in the guise of one of its rare complications, diagnosis becomes difficult and appropriate treatment is delayed. Typhoid glomerulonephritis is one such rare complication. Mesangial proliferative glomerulonephritis, IgA nephropathy, acute diffuse proliferative glomerulonephritis and crescentic IgA nephropathy have been reported in the past. Membranoproliferative glomerulonephritis (MPGN) type II in association with typhoid fever has never been reported. We report a 13-year-old boy presenting with high grade fever, hematuria, proteinuria and Salmonella typhi bacteremia. Kidney biopsy showed MPGN type II. He had a complete recovery of his renal abnormality following treatment for his typhoid fever.

Behcet’s disease and IgA nephropathy

Although Behçet's disease (BD) is a kind of systemic disease, renal involvement is rare, especially IgA nephropathy (IgAN). Renal manifestations in BD range from mild urinary abnormalities to glomerulonephritis with persistent renal failure, which includes minimal change disease, proliferative glomerulonephritis, rapidly crescentic glomerulonephritis, renal amyloidosis and IgA nephropathy. Amyloidosis seems to be the most common type of renal lesion in BD, and several cases of nephrotic syndrome secondary to amyloidosis have been documented. Co-occurrence of BD and IgA nephropathy has only been reported in only few cases. We describe two patients with the rare association of BD and IgAN. We suggested that it is important to periodically perform renal function assessment in patients with BD, through urinalysis and measurement of serum creatinine for detecting any abnormality and providing an early adequate treatment.

Renal histology in the elderly: indications and outcomes

Renal disease is being increasingly diagnosed in the elderly. However, reports on biopsy-confirmed renal disease in this population are limited. The aim of this study was to give an overview of the most important indications, diagnoses and outcomes of renal biopsies in the elderly in our center. This was a retrospective review of all elderly renal biopsies over 5 years. Patients were eligible for inclusion if they were aged ≥65 years and had had a native kidney biopsy performed. The data recorded included age, sex, indications for biopsy, histological diagnoses and outcomes. During this time, 1,372 native renal biopsies were performed. Of these, 236 (17%) were in patients aged ≥65 years; 150 male (64%) and 86 female (36%). The most common indications for biopsy were acute renal failure and nephrotic syndrome. Common diagnoses included pauci-immune crescentic glomerulonephritis, tubulointerstitial nephritis, membranous nephropathy, IgA nephropathy and chronic thrombotic microangiopathy. Long-term follow-up of 3 years was available for 102 patients; median serum creatinine at the time of biopsy was 427 µmol/L (interquartile range 204-702) and at 3 years post biopsy had fallen to 192 µmol/L (interquartile range 152-408). In our center, 17% of native kidney biopsies are performed in elderly patients aged ≥65 years. In our experience, this procedure was safe and had a 97% diagnostic rate. The available follow-up data of patients suggest that renal histology is not only of benefit in diagnosis but also of potential value in terms of prognosis and treatment.

Cellular components of crescents in four common types of crescentic glomerulonephritis

To examine the cellular components at different stages of the crescent formation in four most common types of human crescentic glomerulonephritis (CGN), including anti-GBM disease (GBM-CGN), crescentic IgA nephropathy (IgA-CGN), ANCA associated pauci-immune CGN (ANCA-CGN) and crescentic lupus glomerulonephritis (LN-CGN). Renal biopsy specimens of patients with GBM-CGN (n = 10), IgA-CGN (n = 12), ANCA-CGN (n = 12), and LN-CGN (n = 11) were selected. Immunohistochemistry was adopted to identify the cellular components using different cell markers including cytokeratin (PEC), CD68 (macrophage), nestin (podocyte), podocalyxin (podocyte), CD3 (lymphocyte), CD15 (neutrophil) and PCNA. There were different subtypes of cell components identified during the formation of a cellular crescent in 4 different types of human CGN. Mainly of PEC 11.4 (0.0, 95.0)%, macrophage 8.0 (0.0, 35.0)% and podocyte 5.5 (0.0, 22.0)% and their constitutive percentages were different among various CGNs (P < 0.01). In all the CGNs studied, there were 50% of cells were negative to all the cell markers adopted for this expeiment. Podocalyxin positive cells 0.5 (0.0, 9.6)% were significantly less than nestin positive cells 5.5 (0.0, 22.0)% in all CGNs. PCNA positive cells were 44.7 (16.7, 83.3)% in the cellular crescent of all CGNs and co-localized with nestin (38/45 cases), CK (42/45 cases) or CD68 (24/45 cases). PEC, macrophage and podocyte might play important roles in the formation of crescents. The staining disparity of nestin and podocalyxin indicates that podocyte dedifferentiation may occur during the crescent formation. PEC, podocytes and macrophages may participate in the formation of crescent in common CGNs through active cellular proliferation.

The spectrum of acute renal failure in IgA nephropathy

Background: Acute renal failure rarely complicates the course of IgA nephropathy. In this study, we have tried to define the mode of presentation, the spectrum of morphology, and the prognostic factors for renal outcome. Methods: Twenty patients with biopsy-proven IgA nephropathy who developed acute renal failure were identified from 2000 to 2009 at a medical center in Taiwan. The patients' records were retrospectively reviewed with respect to clinical presentation, morphology of renal biopsy, and outcomes. Results: On histology, glomerular crescents were present in 11 patients (55%), acute tubular necrosis was identified in 11 patients (55%), acute interstitial nephritis was seen in 4 patients (20%), and extensive tubular red blood cell casts were present in 4 patients (20%). At the end of follow-up, 2 patients (10%) had died, 11 patients (55%) were in remission, and 7 patients (35%) developed end-stage renal disease. The prognostic factors for renal outcome were peak serum creatinine, dialysis support requirement, morphology (prominent glomerular/tubular injury), percentage of glomeruli affected by crescents, and interstitial infiltration (p = 0.04, <0.001, 0.013, 0.05, 0.02, respectively). Conclusions: Our findings suggested that there were four pathogenic mechanisms involved in IgA nephropathy with acute renal failure including (1) crescentic IgA nephropathy; (2) acute tubular necrosis associated with microhematuria and red blood cell casts occluding tubules; (3) acute tubular necrosis not related to microhematuria; and (4) acute interstitial nephritis, apparently induced by drugs. In general, patients with prominent tubular injury had a much higher remission rate than patients with prominent glomerular injury.

Is Presence of ANCA in Crescentic IgA Nephropathy a Coincidence or Novel Clinical Entity? A Case Series

There are few anecdotal reports of circulating antineutrophil cytoplasmic autoantibodies (ANCAs) in patients with immunoglobulin A (IgA) nephropathy. Retrospective case series. We studied 8 patients with crescentic IgA nephropathy associated with ANCAs against myeloperoxidase (n = 5) and proteinase 3 (n = 3) followed up for 2.4 +/- 1.7 years. They were compared with 26 patients with IgA nephropathy with > 10% crescentic glomeruli, but negative for ANCAs. We analyzed clinical and histologic features of patients and their response to treatment. Screening for ANCAs was performed using indirect immunofluorescence, and positive results were verified using enzyme-linked immunosorbent assay. All patients with crescentic IgA nephropathy and positive for ANCAs, compared with only one-third of ANCA-negative patients, presented with the clinical syndrome of rapid progressive glomerulonephritis. ANCA-positive patients reached a higher peak serum creatinine level within the first 3 months (4.2 +/- 2.2 vs 2.5 +/- 1.9 mg/dL; estimated glomerular filtration rate, 19.3 +/- 10.2 vs 45.9 +/- 30.1 mL/min/1.73 m(2)). ANCA-positive patients with IgA nephropathy had a higher percentage of crescentic glomeruli (54.3% +/- 18%) compared with ANCA-negative patients with crescentic IgA nephropathy (34.5% +/- 26%). ANCA-positive patients were treated using cyclophosphamide and corticosteroids. Kidney function improved in all these patients: serum creatinine level decreased from the peak of 4.2 +/- 2.2 to 1.7 +/- 0.7 mg/dL at the end of follow up (estimated glomerular filtration rate, 19.3 +/- 10.2 to 44.6 +/- 11.1 mL/min/1.73 m(2)). In contrast, no significant improvement was achieved in ANCA-negative patients. Patients with IgA nephropathy, crescents, and positive for ANCAs represent a clinical entity with a diverse more exaggerated clinical and histologic picture. However, disease in these patients responded well to aggressive immunosuppressive therapy.

In Crescentic IgA Nephropathy, Fractional Excretion of IgG in Combination with Nephron Loss Is the Best Predictor of Progression and Responsiveness to Immunosuppression

The aim of this study was to evaluate the relationship between proteinuric markers (urinary excretion of IgG, alpha2-macroglobulin, alpha1-microglobulin) and serum creatinine (sCr), histologic lesions, progression, and immunosuppression responsiveness in crescentic IgA nephropathy. Fractional excretion of IgG (FEIgG) and of alpha1-microglobulin and urinary excretion of alpha2-macroglobulin were evaluated in 37 patients, 23 treated with steroids and cyclophosphamide. For assessment of the effective tubular load of proteins in surviving nephrons, new markers that take into account not only the absolute excretion value but also nephron loss were obtained dividing proteinuric markers for percentage of nonobsolescent glomeruli (surviving glomeruli [SG]). For each parameter, low- and high-risk groups were defined according to cutoffs with the highest sensitivity and specificity for progression (ESRD/doubling sCr) assessed by receiver operating characteristic analysis; follow up was 60 +/- 40 mo. FEIgG/SG is the most powerful progression predictor: 5 versus 83% in all patients; in treated patients, 0 versus 89%, increased to 0 versus 100% by sCr and FEIgG/SG in combination (low risk: both markers or only one below cutoff (n = 15); high risk: both markers above cutoff (n = 8). The nonprogressors showed at last observation 65% proteinuria reduction and 10% sCr reduction. In crescentic IgA nephropathy, FEIgG/SG, which evaluates altered size selectivity in relation to nephron loss, is the best progression predictor. In treated patients, progression prediction was increased by FEIgG/SG and sCr in combination. Treatment may be restricted to low-risk patients.

Anti-neutrophil cytoplasmic antibody associated crescentic IgA nephropathy in hematopoietic stem cell transplantation

Membranous glomerulonephritis and minimal change disease are the most common forms of glomerular diseases noted in patients with graft versus host disease after hematopoietic stem cell transplantation. Herein, we report a patient who developed anti-neutrophil cytoplasmic antibody associated crescentic IgA nephropathy within 3 months after autologous hematopoietic stem cell transplantation. He was treated with intravenous pulse steroids and monthly intravenous cyclophosphamide for 6 months followed by cyclophosphamide every 3 months and tapering dose of steroids. His proteinuria resolved and renal function remained stable. Two cases of crescentic IgA nephropathy have been reported in patients who underwent allogenic hematopoietic stem cell transplantation. The etiology of IgA nephropathy developing after hematopoietic stem cell transplantation is unclear and larger registry-based studies are needed to further explore this condition.

Recurrence of IgA Nephropathy With Crescents in Kidney Transplants

Crescentic IgA nephropathy is an uncommon finding in native kidneys (3%–5%) and in renal transplants. This study was performed to determine the frequency of relapsing crescentic IgA nephropathy after kidney transplantation. Over a 15-year period, 42 patients (25 men, 17 women) of age range 17 to 59 years with biopsy-proven IgA nephropathy in their native kidneys were entered into this retrospective study, because they had undergone kidney transplantation and had sequential allograft biopsies during their follow-up. Mean follow-up after transplantation was 8.9 years (range, 1–15 years). In their native kidneys, 5 patients (12%) had more than 20% crescents, and only 2 (5%) had more than 50% of glomeruli involved. As expected, 52.4% of recipients showed recurrent mesangial IgA deposits in their kidney grafts. The 2 patients with diffuse crescentic IgA nephropathy in their native kidneys experienced acute graft dysfunction at 15 and 47 months. Graft biopsy showed recurrent IgA deposits with cellular crescents in 30% and 20% of glomeruli, respectively. Despite corticosteroid pulse therapy, graft failures occurred 2 and 27 months later. No crescentic proliferation was observed during follow-up in any other case. Only 5 other grafts failed because of chronic allograft nephropathy, without any relationship to the relapse of IgA deposits. These data suggested for the first time that only diffuse crescentic IgA nephropathy in the native kidneys was associated with the occurrence of crescents in the kidney transplants, a finding that raises the possibility of a particular subgroup of IgA nephropathies.

Prevalence of hepatitis B and C markers in glomerular diseases

To study the prevalence of hepatitis B and C viruses in patients with glomerulonephritis (Gn). Material and methods. This was a retrospective study of 89 patients (36 females, 53 males) diagnosed with Gn. Infection with hepatitis B and C was studied by means of serological methods; if patients presented hepatitis C antibodies, the presence of viral RNA in serum was detected by means of quantitative polymerase chain reaction. The control group comprised 59,546 first-time blood donors. None of the patients were positive for hepatitis B surface antigen (HBsAg). In the control group there were 168 HBsAg positives. The prevalence of HBsAg in the control group (0.28%) was not significantly different (p = 0.614) from that in the patient group. Four patients with Gn were positive for hepatitis C virus (HCV) antibodies, and in three of these RNA HCV was also positive. The histological diagnoses in the four cases with HCV antibodies were: focal and segmental glomerulosclerosis; crescentic IgA nephropathy; diffuse proliferative Gn; and membranous Gn. The first three patients also presented other pathologies potentially linked to Gn, namely left renal agenesis, heavy alcohol intake/chronic liver disease and HIV seropositivity, respectively. Only the patient with membranous Gn, in whom other causes were disregarded, received antiviral treatment, although RNA HCV remained positive. In the control group, 141 cases were positive for HCV antibodies (prevalence 0.24%). The prevalence in the study group was significantly higher (p < 0.001). HCV is more prevalent in patients with Gn than in those without, and this is the opposite of the situation with HBsAg.