Outcome Of Idiopathic Membranous Nephropathy Research Articles

The ratio of neutrophils to lymphocytes effectively predicts clinical outcomes in idiopathic membranous nephropathy.

Systemic inflammatory indicators are important in the prognoses of various diseases. Such indicators, including the neutrophil-to-lymphocyte ratio (NLR), can be meaningful in predicting the clinical outcome in patients diagnosed with idiopathic membranous nephropathy (IMN). 112 IMN patients diagnosed by renal biopsy were recruited retrospectively. The endpoint was defined as a combination of partial and complete remission. Statistical analysis determined the independent factors associated with clinical remission and the predictive utility of NLR. Within the 12-month follow-up period, 72 patients achieved clinical remission after treatment. Univariate analysis identified significant differences in serum albumin, estimated glomerular filtration rate (eGFR), proteinuria, neutrophil count, and NLR between the remission group and the non-remission group (all p < 0.05). Cox proportional hazards indicated that elevated eGFR (HR 1.022, 95% CI (1.009-1.035), p = 0.001), lower NLR (HR 0.345, 95% CI (0.237-0.501), p = 0.0001), and decreased proteinuria (HR 0.826, 95% CI (0.693-0.984), p = 0.032) were protective elements for remission. With an optimal cut-off value of 2.61, the pre-treatment NLR had an excellent ability to identify the remission (area under the curve (AUC), 0.785). Participants were separated into low- and high-NLR groups by using 2.61. Kaplan-Meier survival curves revealed significantly higher remission rates in the lower group (p < 0.0001). The NLR is an effective indicator for predicting clinical remission in patients with IMN.

Effect of immunosuppressive agents on clinical outcomes in idiopathic membranous nephropathy.

Few comparative studies on the effects of immunosuppressants in patients with idiopathic membranous nephropathy have been conducted. Data from 489 patients who received conservative treatment or immunosuppressants were retrospectively analyzed by propensity score matching. Primary outcomes were complete or partial remission (CR or PR) of proteinuria, and secondary outcomes were renal survival and infection. Of the 489 patients, 357 (73.0%) received immunosuppressants. Propensity score matching identified 82 patients from the conservative group and 82 patients in the immunosuppressant group. CR or PR at 12 months was significantly higher in the immunosuppressant group compared with the conservative group for the total population (p = 0.002) and the propensity score-matched population (p = 0.02). The use of immunosuppressants was significantly more effective with respect to achieving a CR or PR at 12 months in patients from the total population who were aged <65 years or female, or who had a proteinuria level of ≥4.0 g/g or an estimated glomerular filtration rate of ≥60 mL/min/1.73 m2 (p < 0.05). Renal survival was similar between patients receiving immunosuppressants and conservative treatment in both the total and matched populations. The immunosuppressant group (21.8%) had a significantly higher incidence of infections compared with the conservative group (13.6%) for the total population (p = 0.03), but statistical significance disappeared in the matched population (p > 0.99). The remission rate was significantly higher in the immunosuppressant group than in the conservative group, particularly in the subgroup of patients who were young or female, or those with heavy proteinuria loads or good renal function.

Non-glomerular Tip Lesion Focal Segmental Glomerulosclerosis as a Negative Predictor in Idiopathic Membranous Nephropathy

ObjectiveTo assess the significance of focal segmental glomerulosclerosis (FSGS) variants on clinicopathological characteristics and short-term outcomes in idiopathic membranous nephropathy (IMN) patients.MethodsThe clinicopathological data of 146 IMN patients diagnosed between December 2016 and March 2019 in our center were collected and analyzed. These patients were divided into the pure IMN group, IMN with glomerular tip lesion (GTL) group, and IMN with non-GTL FSGS group.ResultsThe IMN with non-GTL FSGS and IMN with GTL groups both had higher proportions of patients with hypertension, lower serum albumin, and severe proteinuria, while the IMN with non-GTL FSGS group additionally showed higher blood pressure and serum cholesterol, and lower serum IgG than the IMN group (all P<0.05). As for pathology, the IMN with non-GTL FSGS group had higher proportions of patients with acute tubular injury and moderate to severe chronic injuries than the IMN group (all P<0.05). In the IMN, IMN with GTL, and IMN with non-GTL FSGS groups, the overall one-year remission rates were 81.6%, 76%, and 58.8%, respectively. Furthermore, the IMN with non-GTL FSGS group showed the lowest cumulative incidence to reach remission within one year. Multivariate Cox logistic analysis demonstrated that higher level of serum anti-M-type phospholipase A2 receptor antibody and the existence of non-GTL FSGS lesion were independent predictors for no remission in IMN patients.ConclusionThe non-GTL FSGS lesion was a novel negative predictor in IMN and should be taken into account in the management of IMN.

Profile and primary treatment outcomes in membranous nephropathy.

To assess the clinical profile and primary treatment response and outcomes in idiopathic membranous nephropathy (IMN) patients. This study was carried out between December 2013 and January 2019 in a tertiary care hospital in North India on 2 years retrospective and 3 years prospective renal biopsy proven patients with IMN presenting with nephrotic syndrome. Basic baseline investigations carried out were urinary proteins, serum albumin, serum creatinine, other special tests wherever necessary or possible (including phospholipase A2 receptor antibodies), and different treatment regimens were offered for treatment. The patients were followed up for a minimum period of 6 months after administration of treatment. The study was carried out in 120 patients with mean age of 43±14.6 years and male female ratio of 1.65:1. Hypertension was noted in 36%, microscopic hematuria in 13%, and mean 24 hours urinary proteinuria 10.5±3.1 gm. Complete or partial response at 6 months was observed in 57% and 34% cases to cyclophosphamide, 60% and 40% to modified Ponticelli treatment, 81% and 19% to tacrolimus, and 40% and 36% cases to rituximab. Relapse was observed in 6% of cyclophosphamide and 13% in tacrolimus groups. Our results show a good and comparable response to cyclophosphamide, tacrolimus, and rituximab at 6 months of follow up. The cases which achieved complete remission had significantly lower baseline proteinuria compared to those who did not respond.

Association of serum mannose-binding lectin, anti-phospholipase A2 receptor antibody and renal outcomes in idiopathic membranous nephropathy and atypical membranous nephropathy: a single center retrospective cohort study

Objectives Idiopathic membranous nephropathy (iMN) is a major cause of nephrotic syndrome. Atypical membranous nephropathy (aMN) is a new type of nephropathy in China, characterized by a ‘full-house’ on immunofluorescent examination, that is IgG, IgA, IgM, C3, C1q positive, but without clinical evidence of a secondary cause. Phospholipase A2 receptor (PLA2R) was the major target antigens in iMN patients. Activation of the mannose-binding lectin (MBL) pathway plays a vital role in the development of MN. Our objective was to investigate the role of PLA2R and MBL in the pathogenesis of iMN and aMN. Methods We conducted a retrospective observational study using propensity score matching by age, gender, and eGFR. All clinical, laboratory data, and follow-up data of the patients were collected. Serum levels of anti-PLA2R antibodies and MBL were tested. Results Finally, 30 iMN patients and 30 aMN patients were included, and 20 healthy controls were retrospectively collected in this study. The 24 h proteinuria level was higher and serum albumin was lower in anti-PLA2R (+) patients than in anti-PLA2R (−) patients in both iMN and aMN groups. In aMN patients, MBL levels were significantly higher in anti-PLA2R (+) patients than in anti-PLA2R (−) patients (p = .045). The serum level of anti-PLA2R positively correlated with no-remission in both iMN and aMN groups. Conclusions The complement lectin pathway has an association with the development of MN, especially in patients with positive anti-PLA2R antibodies. Serum MBL cannot differentiate between the two diseases. Serum MBL levels are not associated with clinical manifestations, nor with prognosis.

The association of anti-PLA2R with clinical manifestations and outcomes in idiopathic membranous nephropathy: a meta-analysis.

The meta-analysis aims to investigate the relationship between anti-phospholipase A2 receptor autoantibody (anti-PLA2R) and clinical characteristics and adverse outcomes of idiopathic membranous nephropathy (IMN). Related studies published before February 2020 were systematically retrieved from foreign and domestic databases, RevMan5.3 software was used for meta-analysis and subgroup analysis, and STATA 15.0 statistical software was used for its heterogeneity and testing publication bias. Twenty studies were included, involving 2224 patients with IMN. Our results showed that a significant correlation existed between the expression of serum anti-PLA2R and age (MD = 2.91, 95% CI = 2.15-3.67, P<0.00001), total serum cholesterol (MD = 35.52, 95% CI = 9.52-61.52, P = 0.007), urine protein by creatinine ratio (UPCR) (MD = 2.15, 95% CI = 1.86-2.44, P<0.00001), serum albumin (MD = -0.40, 95% CI = -0.56 to -0.23, P<0.00001), eGFR (MD = -10.44, 95% CI = -12.19 to -8.68, P<0.00001), unremission rate (RR = 1.76, 95% CI = 1.37-2.27, P<0.0001). In addition, the high titer in seropositive group was closely correlated with serum albumin (MD = -0.40, 95% CI = -0.74 to -0.05, P = 0.03), eGFR (MD = -12.40, 95% CI = -16.29 to -8.52, P<0.00001) and unremission rate (RR = 2.52, 95% CI = 1.79-3.55, P<0.00001). No significant correlation was found between glomerular anti-PLA2R and clinical manifestations except for serum cholesterol (MD = 0.81, 95% CI = 0.21-1.41, P = 0.008). However, in terms of prognosis, glomerular anti-PLA2R showed a significant relevance to recurrence rate (RR = 2.25, 95% CI = 1.07-4.72, P = 0.03). Compared with glomerular anti-PLA2R, serum anti-PLA2R may better reflect the activities of IMN disease, while the glomerular anti-PLA2R might be connected with the recurrence of the disease.

Focal segmental glomerulosclerosis, excluding atypical lesion, is a predictor of renal outcome in patients with membranous nephropathy: a retrospective analysis of 716 cases

BackgroundFocal segmental lesions (FSLs) are not uncommon in idiopathic membranous nephropathy (IMN). The reported percentage of IMN patients with focal segmental glomerulosclerosis (FSGS) lesions varies widely between studies. The objective of this study was to differentiate atypical FSL (aFSL) from typical FSGS in IMN and to analyse the clinicopathological predictors of primary outcome of IMN patients.MethodsA total of 716 patients with biopsy-proven IMN between January 1, 2007 and December 31, 2017 were enrolled in the study. An atypical focal segmental lesion was defined as pure synechia, segmental hyperplasia of podocytes or thickening of the GBM accompanied by proliferation of the mesangial matrix, and absence of typical FSGS. The patients were divided into three groups: patients without FSL (FSL−), patients with typical FSGS (FSGS+), and patients with aFSL (aFSL+).The primary outcome was a 50% decline in the initial estimated glomerular filtration rate or end-stage renal disease (ESRD) incidence. Secondary outcomes included all-cause death and ESRD.ResultsFSGS was present in 174 patients, while aFSL was noted in 161 patients. Systolic blood pressure was higher in both aFSL+ group and FSGS+ groups compared with the FSL− group. IMN patients without FSL and with aFSL had lower serum creatinine levels than IMN patients with FSGS. Both the FSGS+ and aFSL+ groups had higher levels of proteinuria and lower albumin levels than the FSL− group. Renal tissue lesions, including tubulointerstitial fibrosis, glomerular obsolescence, and vascular sclerosis were significantly more severe in the FSGS+ group. Cox multivariate analysis showed that older age ≥ 60 years, eGFR< 60 ml/(min·1.73m2), tubulointerstitial fibrosis area ≥ 15% and FSGS at biopsy were independent risk factors for the primary outcome.ConclusionsNo significant difference in outcome was found between the FSL− and aFSL+ groups, although the patients with aFSL had lower levels of serum albumin and eGFR, higher level of urinary protein, more severe renal lesions with proliferation of the mesangial area,tubulointerstitial fibrosis and vascular sclerosis. FSGS, excluding atypical lesions, was an independent predictor of the primary outcome.

Analysis of different treatments and their outcomes in idiopathic membranous nephropathy: a single-center observational study

Background Although there are multiple treatment modalities for idiopathic membranous nephropathy (IMN) which have been studied in different streams of populations, there is no single convincing answer for the best accepted modality. In the study presented, we have made an effort to compare the different treatment regimens and analyze the results obtained. Patients and methods This was a retrospective observational study. A total number of 54 patients with idiopathic membranous nephropathy were included in the study. Five different treatment groups were made, which included modified Ponticelli regime, oral cyclophosphamide, oral mycophenolate mofetil, oral tacrolimus, and intravenous rituximab therapy, and were followed up for 1 year. Urine protein to creatinine ratio, serum albumin, creatinine, and estimated glomerular filtration rate were noted at 1, 3, 6, 9, and 12 months. Results Among the 54 patients, 10 of 16 patients in modified Ponticelli group, 9 of 18 in oral cyclophosphamide group, 4 of 9 in oral mycophenolate mofetil group, 3 of 6 in oral tacrolimus group, and 3 of 5 in intravenous rituximab group achieved remission. There was statistically significant improvement in renal function in modified Ponticelli group (P=0.02) and oral cyclophosphamide group (P=0.01) as compared with the other groups. Conclusion Patients who received modified Ponticelli regime had superior outcomes in terms of improvement in renal function and renal survival as compared with other different therapies. Large studies with long-term follow ups are needed to support the outcome.

Pathological predictors of renal outcomes in nephrotic idiopathic membranous nephropathy with decreased renal function

The outcome of idiopathic membranous nephropathy (IMN) in adults with nephrotic-range proteinuria and decreased renal function has seldom been described and the predictive value of pathological features is debated. This study aimed to describe the clinical course of this patient subgroup and to identify independently predictive pathological features. We evaluated 129 adults with biopsy-proven IMN diagnosed from 2002 to 2011. All patients had chronic kidney disease (CKD) stages 2-4 and nephrotic-range proteinuria (≥3.5 g/day). Primary outcomes were a 20 or 50 % decline in renal function, progression to end-stage renal disease (ESRD), or all-cause mortality. Of 129 patients, 38 (30 %) presented with proteinuria ≥8.0 g/day and 37 (29 %) with CKD stages 3-4. Thirteen (10 %) presented with segmental sclerosis, 97 (75 %) with arteriosclerosis, 42 (33 %) with moderate-to-severe tubulointerstitial injury, and 86 (67 %) with C3 deposition. Over a median follow-up of 34 months (range 12-135), 51 patients (40 %) had a 20 % decline in renal function, 27 (21 %) a 50 % decline, 14 (11 %) developed ESRD, and 19 (15 %) died. Segmental sclerosis and tubulointerstitial injury but not arteriosclerosis or C3 deposition were independent risk factors for 20 and 50 % renal function decline and progression to ESRD. Segmental sclerosis and tubulointerstitial injury predict renal outcomes independent of clinical data in nephrotic IMN patients with decreased renal function.

Long-Term Outcomes in Idiopathic Membranous Nephropathy Using a Restrictive Treatment Strategy

Recently published Kidney Disease Improving Global Outcomes (KDIGO) guidelines recommend limiting the use of immunosuppressive drugs in idiopathic membranous nephropathy to patients at the highest risk of kidney failure. However, recommendations are based on natural history rather than direct assessment of a restrictive treatment strategy. Here, we describe the long-term outcomes of treating a large cohort of patients with idiopathic membranous nephropathy according to a restrictive treatment policy. We analyzed data for 254 patients who visited our outpatient clinic between 1995 and 2009. All patients were treated with angiotensin-converting enzyme inhibitors or angiotensin-receptor blockers. Immunosuppressive therapy was recommended in cases of deteriorating renal function or untreatable nephrotic syndrome. Primary outcomes for the present study were renal replacement therapy and death. Secondary outcomes included adverse events during follow-up and remission of proteinuria. In total, 124 patients (49%) received immunosuppressive therapy, which predominantly consisted of cyclophosphamide combined with steroids. Ten-year cumulative incidence rates were 3% for renal replacement therapy and 10% for death. Partial remission rates were 39%, 70%, and 83% after 1, 3, and 5 years, respectively; complete remission rates were 5%, 24%, and 38% at 1, 3, and 5 years, respectively. A serious adverse event occurred in 23% of all patients. The most notable complications were infections (17%), leukopenia (18%), cardiovascular events (13%), and malignancies (8%). In conclusion, the use of a restrictive treatment strategy in this cohort of patients with idiopathic membranous nephropathy yielded favorable outcomes while limiting the number of patients exposed to toxic drugs. These results support current KDIGO guidelines.

Predicting outcomes in idiopathic membranous nephropathy

Several immunosuppressive treatments have demonstrated their efficacy in idiopathic membranous nephropathy, but spontaneous remission does develop in more than 30% of patients. The availability of a validated biomarker that is easy to measure and able to accurately predict long-term outcomes would be very helpful for tailoring treatment of the disease to the individual.

Outcome of idiopathic membranous nephropathy using targeted stepwise immunosuppressive treatment strategy

The natural course of idiopathic membranous nephropathy (IMN) is variable and the role of immunosuppressive therapy is controversial. In our centre, the strategy has been conservative: the immunomodulating treatment (glucocorticoids and/or cyclosporine A) has been targeted to patients at high risk of developing progressive renal disease and the cytotoxic drugs have been used cautiously. The aim of this retrospective observational study was to evaluate the efficacy of this strategy. We evaluated the clinical course and outcome of IMN patients diagnosed between 1993 and 2003. Risk assessment was done during an observation period of ≥6 months after the initial renal biopsy. Patients were followed up until death, the development of end-stage renal disease (ESRD) or the last clinical visit (before December 2006). Treatments and their side effects were recorded. One hundred and forty-two patients with membranous nephropathy were diagnosed of which 81 were idiopathic. The clinical course of 76 IMN patients (38 high risk and 38 low risk) were followed up [mean duration 66 ± 40 (median 59) months]. Thirty-five patients were treated with immunosuppressive drugs, and at last follow-up, 71% of them were in complete or partial remission. The overall response rate of this therapy was 83%. 11% of the high-risk patients had reached ESRD. For the high-risk patients, 10-year survival (alive with glomerular filtration rate >10 mL/min/1.73 m(2)) was 79%. No major side effects were observed. This study suggests that targeted, stepwise, cytotoxic drug-sparing immunosuppressive treatment in IMN was associated with favourable renal, as well as overall survival among patient at risk of developing ESRD.

Rituximab in idiopathic membranous nephropathy: a one-year prospective study.

Currently available monoclonal antibodies against the B cell surface antigen CD20 have been employed to explore whether specific inhibition of B cells may help improve the outcome of idiopathic membranous nephropathy (IMN) and may avoid the side effects of steroids and immunosuppressants. This prospective, observational study evaluated the 1-yr outcome of eight IMN patients with persistent (>6 mo) urinary protein excretion > 3.5 g/24 h given four weekly infusions of the anti-CD20 antibody rituximab (375 mg/m(2)). At 3 and 12 mo, proteinuria significantly decreased from mean (+/- SD) 8.6 +/- 4.2 to 4.3 +/- 3.3 (-51%, P < 0.005) and 3.0 +/- 2.5 (-66%, P < 0.005) g/24 h, albumin fractional clearance from 2.3 +/- 2.1 to 1.2 +/- 1.7 (-47%, P < 0.05) and 0.5 +/- 0.6 (-76%, P < 0.003), and serum albumin concentration increased from 2.7 +/- 0.5 to 3.1 +/- 0.3 (+21%, P < 0.05) and 3.5 +/- 0.4 (+41%, P < 0.05) mg/dl. At 12 mo, proteinuria decreased to < or =0.5 g/24 h or < or =3.5 g/24 h in two and three patients, respectively. Proteinuria decreased in the remaining patients by 74%, 44%, and 41%, respectively. Body weight, diastolic BP, and serum cholesterol progressively decreased in parallel with an improvement of edema in all patients. Renal function stabilized (Delta1/creatinine: +0.002 +/- 0.007). CD20 B lymphocytes fell below normal ranges up to study-end. No patient had major drug-related events or major changes in other laboratory parameters. Rituximab thus promotes sustained disease remission in patients otherwise predicted to progress to ESRD, and it is safe. The long-term risk/benefit profile of this novel, disease-specific approach seems much more favorable to that of commonly employed immunosuppressive drugs.

Clinical features and treatment outcome of idiopathic membranous nephropathy in Chinese patients.

We retrospectively studied the clinical course and treatment outcome of idiopathic membranous nephropathy (IMN) amongst 38 Chinese patients (25 male, 13 female, age 51.6 +/- 14.6 years, follow-up duration 58.2 +/- 51.1 months) who presented over a 10-year review period. Eight never received any form of specific treatment (group I), seven received oral corticosteroid alone for 6-9 months (group II), 17 were given corticosteroid plus cyclophosphamide for 6-12 months (group III), and six were treated with methylprednisolone alternating with chlorambucil every other month for 6 months (group IV). No untoward effect from drugs sufficient to alter the dosage used was recorded. After 6 months of treatment, over 50% of patients went into remission: a significant reduction in proteinuria (p = 0.01, 0.01, 0.02) with a corresponding rise in serum albumin levels (p = 0.01, 0.01, 0.04) was observed in groups II, III, and IV, respectively, but not in group I. During follow-up, one patient in each of groups I, III, IV, and two of group II developed renal function deterioration, which correlated with an abnormal presenting serum creatinine. In six group I and eight group III patients who have been followed for at least 5 years, there was progressive reduction in proteinuria in group III (p < 0.05), but not in group I: serum creatinine has remained unchanged in both groups. IMN runs a benign course in Chinese patients in Hong Kong, with 2.6% of patients going into end-stage renal failure during the study period. Contrary to reports in Caucasians, there is similar treatment response to steroid alone or a combination of steroid and cytotoxic agents.

Idiopathic nephrotic syndrome in the elderly

The most frequent primary glomerular diseases (PGD) associated with nephrotic syndrome (NS) in the elderly are membranous nephropathy (MN), minimal change nephropathy (MCN), and focal and segmental glomerulosclerosis (FSGS). In older patients MN may be secondary to drugs or neoplasia in 20 to 25% of cases. The natural renal outcome of idiopathic MN is similar in elderly patients and in those of the second age. However, elderly patients are more exposed to the extra-renal complications of NS. Corticosteroids alone do not seem to modify the course of the disease. A 6-month regimen with corticosteroids alternated to chlorambucil, which has proven to improve the outcome of MN in adults, may increase the chances of remission and protect renal function also in the elderly patients but side effects increase with age. Elderly patients with MCN are more prone than younger adults to the complications of the NS and to the development of renal failure. Only 60% of older patients enter remission with an 8-week course of prednisone, but about 80% can achieve complete remission with corticosteroids if treatment is prolonged to 12–16 weeks. Relapses are more rare in the elderly. In patients with contraindications to prolonged corticosteroid therapy, a course of 12 weeks with a cytotoxic agent may obtain stable remission in most cases. Little information is available about the natural course and the management of idiopathic FSGS in the elderly. A recent report showed that more than 40% of older patients may obtain stable remission after an initial treatment with corticosteroids for 6 months. For those patients who do not respond or have contraindications to steroid therapy, a cautious trial with cyclophosphamide may be tried.

Clinical course and outcome of idiopathic membranous nephropathy in Japanese children.

We retrospectively studied 12 Japanese children (8 boys, 4 girls) with idiopathic membranous nephropathy (IMN), aged 2.9-15.8 (mean 7.7) years at onset. All patients were identified through either screening or a routine urinalysis; proteinuria was present in all, haematuria, which was macroscopic in 4, in 11. Three had nephrotic syndrome (NS) at or soon after onset. Stages on electron microscopy, performed in 10 patients, were I in 3, II in 5 and III in 2. Steroids alone or with cyclophosphamide were administered to 5 patients, including the 3 patients showing NS. Complete remission of proteinuria occurred in 8 patients 0.3-1.6 (mean 0.6) years after onset, and proteinuria did not recur. After a follow-up of 1.6-11.6 (mean 5.9) years, these 8 patients were in complete remission and the remaining 4 had only mild proteinuria; none had hypertension or impaired renal function. Thus, we infer that IMN in Japanese children may have a better course and outcome than IMN in non-Japanese children. Based on a comparative study of Japanese (previously reported cases added to ours) and non-Japanese (mostly Caucasian) children with IMN, this was confirmed; it is possible that steroid therapy in Japanese patients is more effective in inducing remission of NS and preserving renal function.

Long-Term Outcome of Idiopathic Membranous Nephropathy With Nephrotic Syndrome

We retrospectively studied 82 consecutive patients with idiopathic membranous nephropathy accepted at two separate Renal Services. These patients had nephrotic syndrome at presentation and had been followed up for at least 10 years. The duration of the disease before renal biopsy ranged between 1 and 18 months. Forty-nine patients never received treatment (non-treated group) whereas 33 patients (treated group) received corticosteroids (and cytotoxic agents). The two groups were age and sex matched. There was no difference in clinical findings or in outcome between the two Renal Services. A complete and sustained remission of proteinuria was observed in about 25% of patients, and there was a significant difference (P less than 0.01) between the treated and non-treated group (mean value 39.1% vs 14.3%). Forty-four per cent of the non-treated patients and 24.2% of the treated ones developed chronic renal failure or renal death during a mean potential follow-up of 14 years. Non-renal death was recorded in 19.5% of the patients. The staging of the capillary wall lesion represented a very important prognostic index. We confirm, therefore, that idiopathic membranous nephropathy with nephrotic syndrome is frequently a progressive disease. Corticosteroids (and cytotoxic drugs) seem to be of some benefit in interfering with the natural course of the disease.

Genetic Factors in the Outcome of Idiopathic Membranous Nephropathy

Genetic Factors in the Outcome of Idiopathic Membranous Nephropathy

Haematological features of primary myelodysplastic syndromes (PMDS) at initial presentation: a study of 118 cases.

The haematological features of 118 cases of primary myelodysplastic syndromes (PMDS) were reviewed to see how these could be related and classified according to the recent FAB proposals. A majority of the cases were elderly who presented with a macrocytic or normocytic anaemia and reticulocytopenia. Cases of acquired idiopathic sideroblastic anaemia (AISA) usually had normal leucocyte and platelet counts, erythroid hyperplasia, marked dyserythropoiesis and more than 20% ringed sideroblasts. Cases of refractory anaemia with excess of blasts (RAEB) had frequent neutropenia and thrombopenia usually with prominent dysgranulopoiesis and dysthrombopoiesis. Refractory anaemia or refractory cytopenia appeared morphologically to be a heterogeneous group. Leukaemic transformation did not occur in any of these 16 cases of AISA whereas six of the 34 cases of RAEB transformed into acute leukaemia. It appears that the cases of PMDS present with well defined haematological features which permit recognition of different groups; these latter groups appear to be morphologically and prognostically distinct.