Positive C1q staining associated with poor renal outcome in membranoproliferative glomerulonephritis.

BACKGROUND: Pathogenesis and clinical prognosis of membranoproliferative glomerulonephritis (MPGN) has not yet been established. METHODS: We conducted a retrospective study of 41 patients with MPGN (type I and III) and examined the renal survival. In addition, factors contributing to survival time were analyzed. RESULTS: Fourteen patients (34 %) were classified into the renal death group. Patients with nephrotic syndrome and positive C1q staining of glomerular deposits showed a particularly poor prognosis. Significantly higher frequency of nephrotic syndrome and higher urinary protein excretion were observed in the renal death group (p = 0.0002, p = 0.0002) than in the renal survival group. The intensity of C1q staining was positively correlated with the severity of the proteinuria (p = 0.004). Factors that influenced the survival time were positive C1q staining of glomerular deposits (p = 0.003), presence of nephrotic syndrome (p = 0.004), serum albumin (p = 0.02), and proteinuria (p = 0.04). CONCLUSIONS: C1q staining in glomerular deposits and nephrotic syndrome were important factors influencing the prognosis and outcome in MPGN patients. C1q deposition may play a key role in the pathogenesis of MPGN, as evidenced by numerous observations, such as induction of proteinuria.

Rituximab treatment for adult patients with focal segmental glomerulosclerosis.

We present two cases with steroid-resistant nephrotic syndrome (SRNS) and two cases with steroid-dependent nephrotic syndrome (SDNS) due to focal segmental glomerulonephritis (FSGS) who were treated with a single dose of rituximab (375 mg/m(2)). Although the two cases with SRNS showed no response, the two cases with SDNS achieved complete remission. The patients in whom the peripheral B-cell counts subsequently increased after the administration of rituximab demonstrated a relapse. Rituximab may be an effective treatment agent for SDNS with FSGS and the peripheral B-cell count may be a useful marker in such patients for preventing disease relapse.

Severity of nephrotic IgA nephropathy according to the Oxford classification.

BACKGROUND: IgA nephropathy with nephrotic syndrome (nephrotic IgAN) is a rare form of IgAN. Its prognosis and response to steroid therapy are still controversial because the differential diagnosis between nephrotic IgAN and minimal change nephrotic syndrome with IgA depositions is sometimes confused. METHODS: In this retrospective cohort analysis, we accurately diagnosed 42 cases of nephrotic IgAN (4.4%) from 954 IgAN patients, according to the Oxford classification. We analyzed the clinical and histological data, prognosis, and response to steroid therapy. RESULTS: In nephrotic IgAN, mean estimated glomerular filtration rate (eGFR) was 51.1 ± 24.6 ml/min, proteinuria was 5.71 ± 2.56 g/day, and urinary red blood cells were 51.0 ± 37.8 high power field. Both active and chronic histological lesions were observed. Cumulative renal survival rate was significantly lower in nephrotic IgAN than in non-nephrotic IgAN (the control group consisted of 47 non-nephrotic IgAN patients diagnosed between 1995 and 1996) (log-rank test: P < 0.0001). The cases with steroid therapy significantly improved their prognosis, though their male-to-female ratio and blood pressure level measured at renal biopsy were significantly lower than in the cases without steroid therapy. Steroid therapy was particularly effective in cases with low-grade tubular atrophy and interstitial fibrosis (T-grade in Oxford classification). Without steroid therapy, lower eGFR and higher T-grade were independent risk factors for severe outcome by multivariate Cox regression. CONCLUSION: Nephrotic IgAN is a very severe form of IgAN, with renal dysfunction, massive hematuria, and active and chronic histopathological lesions. Renal outcome is severe; however, steroid therapy can improve prognosis in cases with higher eGFR and lower T-grade, according to the Oxford classification.

Comparison of inhibitors of renin-angiotensin-aldosterone system (RAS) and combination therapy of steroids plus RAS inhibitors for patients with advanced immunoglobulin A nephropathy and impaired renal function.

BACKGROUND: The adaptation of steroid therapy and the effect of renin-angiotensin-aldosterone system inhibitors (RASIs) for advanced immunoglobulin A nephropathy (IgAN) patients with impaired renal function are still controversial. METHODS: We divided 63 IgAN patients with an estimated glomerular filtration rate (eGFR) of <60 ml/min/1.73 m(2) and proteinuria ≥ 0.5 g/day into two groups: the RASI group (RASI, n = 33), treated with RASIs alone; and the combination group (COMBI, n = 30), treated with corticosteroids and RASIs. We analyzed the clinical and histological background, renal survival rate, and the risk factors for progression. RESULTS: Renal function (mean eGFR: COMBI 46.4 vs. RASI 47.0 ml/min/1.73 m(2)), the amount of proteinuria (median: COMBI 1.39 vs. RASI 1.17 g/g creatinine) and histological backgrounds were not significantly different between the groups, but urinary red blood cells (U-RBCs) were significantly higher in the COMBI group than in the RASI group (median: COMBI 30.0 vs. RASI 10.0 counts/high-power field, P = 0.0171). The serial change in proteinuria did not differ until 5 years after treatment, but U-RBCs were significantly decreased in both groups (P < 0.0001), and eGFR was significantly decreased in the RASI group (P < 0.001) but not in the COMBI group. The results for each year after treatment did not differ significantly between both groups. The renal survival rate was not significantly different between the groups. There was no independent risk factor for progression by Cox regression analysis. CONCLUSION: Combination therapy with steroids and RASIs was not superior to monotherapy with RASIs for advanced IgAN with impaired renal function.

A case of pulmonary dialysis-related amyloidosis with reticular opacity of the lung in a patient undergoing long-term dialysis.

Dialysis-related amyloidosis (DRA) is one of the most important complications in long-term dialysis patients. Pulmonary involvement in patients with DRA has been rarely described, and lung radiographic findings have not yet been reported. The most common chronic lung disease process in chronic dialysis patients is interstitial fibrosis. This is the first case report of DRA presenting in the lung in a manner resembling interstitial pneumonia. This case study suggests that interstitial pneumonia as a result of DRA should be considered when dyspnoea and reticular opacity of the lung are observed in patients undergoing long-term dialysis.

[Case of lupus peritonitis with thrombus in the right atrium].

A 24-year-old woman with systemic lupus erythematosus (SLE) was admitted to our hospital because of diarrhea, vomiting, and epigastralgia. When she was diagnosed as SLE 5 years earlier, her renal function was normal and her urine protein excretion was 0.15 g/day. Renal biopsy revealed class V lupus nephritis, and she was treated with intravenous steroid (1 g methylprednisolone per day) for 3 days. The prednisolone dose was then reduced from 30 mg/day to 5 mg/day and maintained at that level until she was admitted to our hospital. Her abdominal X-ray, and CT scan showed massive ascites and excessive colonic gas. She was diagnosed as having ileus resulting from lupus peritonitis. The dose of prednisolone was increased up to 50 mg/day. After 1 week, the ascites disappeared and serum albumin and complement levels, lymphocyte count, and urine protein level returned to the normal range. When the prednisolone dose was reduced to 40 mg, however, UCG and an abdominal CT scan revealed thrombus in her right atrium, and inferior vena cava. Urokinase, argatroban and heparin were administered intravenously and warfarin was administered thereafter. Her thrombus gradually disappeared and she was discharged. These findings suggest that anticoagulation therapy is crucial for SLE patients with multiple complications receiving high-dose steroids.