Paraneoplastic nephrotic syndrome can be diagnosed from its clinical and immunological features. The development of several types of glomerular injury in patients with cancer have been recognized, and are considered as paraneoplastic syndrome. Most prominent are the occurrence of membranous glomerulonephritis in patients with carcinomas. We report a case of a 60-year-old-man with small cell lung cancer presenting as nephrotic syndrome. A renal biopsy revealed membranous glomerulonephritis. Six lots of chemotherapy were administerd, which led to a complete tumor response with total resolution of the nephrotic syndrome following treatment.
Biopsy ; Drug Therapy* ; Glomerulonephritis, Membranous* ; Humans ; Nephrotic Syndrome ; Paraneoplastic Syndromes ; Small Cell Lung Carcinoma*

Glomerulonephritis, Membranous ; drug therapy ; Humans ; Male ; Middle Aged ; Rituximab ; therapeutic use

The paraneoplastic nephrotic syndrome can be diagnosed by clinical and immunologic features. We have had a case of paraneoplastic nephrotic syndrome in the patients with aadeno-carcinoma of the lung, whose diagnosis was made by excluding other causes of nephrotic syndrome. The type of renal lesion was membranous glomerulopathy which commonly occurs in carcinoma. The quantity of proteinuria in this patient had decreased according to the improvement of lung cancer with combination chemotherapy. After fourth chemotherapy he was refractory to treatment, and unfortunately he had passed away with cardiac tamponade.
Adenocarcinoma* ; Cardiac Tamponade ; Diagnosis ; Drug Therapy ; Drug Therapy, Combination ; Glomerulonephritis, Membranous* ; Humans ; Lung Neoplasms ; Lung* ; Nephrotic Syndrome ; Paraneoplastic Syndromes ; Proteinuria

The effect of captopril on proteinuria was evaluated in twenty patients with various glomerular diseases excreting heavy proteinuria (> 3.0 g/day). Captopril in a daily dose of 37.5 mg was administered orally three times a day to all patients and they were followed for eight weeks. Twenty-four hour urinary excretion of protein, creatinine, sodium, selective protein index (SPI), and blood chemistry including serum electrolytes were measured every two weeks. Twenty-four hour urinary protein excretion per gram creatinine started to fall within two weeks of captopril administration and became nearly stable after four weeks of therapy (p< 0.05). Mean 24-hour urinary protein excretion decreased significantly from a pretreatment value of 9.0 +/- 6.0 gm/gm of cr. to 4.4 +/- 3.5 gm/gm of cr. after eight weeks of captopril treatment. The serum albumin level increased progressively at six and eight weeks after the captopril treatment period and was significantly higher than the pretreatment value (p< 0.05). The decrease in proteinuria did not coincide with a fall in blood pressure or any changes in creatinine clearance. We conclude that captopril does have a significant antiproteinuric effect in patients excreting heavy proteinuria with various glomerular diseases. However, the long term therapeutic efficacy and any renal protective effect of this drug remain to be proven.
Adult ; Aged ; Blood Pressure/drug effects ; Captopril/*therapeutic use ; Female ; Glomerulonephritis/*drug therapy ; Glomerulonephritis, IGA/*drug therapy ; Glomerulonephritis, Membranous/*drug therapy ; Human ; Male ; Middle Age ; Proteinuria/*drug therapy ; Sodium/urine ; Support, Non-U.S. Gov't

Kaposi's sarcoma is a neoplasm of multifocal origin which manifests primarily as vascular nodules in the skin and other organs. Kaposi's sarcoma in iatrogenically immunocompromised patients is the result of immunosuppressive therapy in organ-transplant recipients, autoimmune disease patients and cytotoxic chemotherapy in cancer patients. We report a case of Kaposi's sarcoma in a 50-year-old man who developed well-defined purple-colored papules and plaques on both hands and feet after prednisolone treatment for underlying membranous glomerulonephritis. The histologic finding showed abnormally proliferated and dilated vessels, vascular slits, spindle-shaped cells and extravasated erythrocytes in the dermis. He was treated with cryotherapy, and the dosage of prednisolone was reduced. Improvement was seen in 6 weeks
Autoimmune Diseases ; Cryotherapy ; Dermis ; Drug Therapy ; Erythrocytes ; Foot ; Glomerulonephritis, Membranous* ; Hand ; Humans ; Immunocompromised Host ; Middle Aged ; Prednisolone ; Sarcoma, Kaposi* ; Skin

BACKGROUNDThe efficacy and safety of immunosuppression for idiopathic membranous nephropathy (IMN) are still controversial. Recent studies showed tacrolimus is effective in the treatment of IMN. To evaluate the efficacy and safety of tacrolimus (TAC) for IMN, we conducted a meta-analysis of published medical literatures.

METHODSStudies addressing the effect of tacrolimus in IMN were searched on PUBMED, EMBASE, The Cochrane Library, and ClinicalTrials.gov (March 2013). Trials comparing tacrolimus with corticosteroid versus control group (cyclophosphamide with corticosteroid) were included. The quality of the studies was assessed using Jadad method. Statistical analyses were performed using Review Manager 5.2 and the results were summarized by calculating the risk ratio (RR) for dichotomous data or the mean difference (MD) for continuous data with 95% confident interval (CI).

RESULTSA total of four studies (259 patients) were included. It was shown that therapy with tacrolimus plus corticosteroid had a higher complete remission rate compared to therapy with cyclophosplamide plus corticosteroid (RR = 1.53, 95% CI: 1.05-2.24, P < 0.05), but not significant on total remission, partial remission and adverse effects. Also, no significant alterations were observed in proteinuria and serum albumin level between the two groups. During the entire follow-up period, serum creatinine level remained stable in both groups without = 50% increase in its level.

CONCLUSIONSTAC is more effective than cyclophosphamide (CTX) by achieving complete remission in patients with IMN. Multi-ethnic RCTs are needed to evaluate its long-term efficacy and safety.

Adrenal Cortex Hormones ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Glomerulonephritis, Membranous ; drug therapy ; Humans ; Immunosuppressive Agents ; therapeutic use ; Tacrolimus ; therapeutic use

Objective: To assess the correlation of glomerular C1q or IgA deposition with clinical and pathological features of primary membranous nephropathy (PMN) in children. Methods: The clinical and pathological manifestations including (phospholipase A2 receptor, PLA2R) and IgG subclasses staining in renal biopsies, serum anti-PLA2R antibody and therapeutic response of 33 children diagnosed with PMN in Peking University First Hospital from December 2012 to December 2020 were retrospectively summarized and analyzed. According to results of PLA2R test and findings renal pathological, the patients were divided into PLA2R-related group and non-PLA2R-related group, typical MN group and atypical MN group, C1q deposit group and non-C1q deposit group, as well as IgA deposit group and non-IgA deposit group respectively. T-test, Mann-Whitney U test and Fisher's exact probability test were used for comparison between the groups. Results: Among the 33 children with PMN, there were 20 males and 13 females, of that the age of onset was 11 (8, 13) years, and 32 patients had nephrotic level proteinuria. Renal biopsies were performed at 4.6 (2.1, 11.6) months after onset, and 28 patients (85%) received glucocorticoid or immunosuppressive therapy prior to renal biopsy. There were 20 cases (61%) with PLA2R-related MN and 13 cases (39%) with non-PLA2R-related MN. Compared with the non-PLA2R-related group, the PLA2R-related group had an older age of onset (12 (10, 13) vs. 7 (3, 12) years, Z=-2.52, P=0.011), a lower preceding infection rate (45% (9/20) vs. 11/13, P=0.032) and lower spontaneous remission rate (0 vs. 4/13, P=0.017). Renal PLA2R positivity was significantly associated with predominant or co-deposition of IgG4 (13/17 vs. 5/15, P=0.031) and low albumin levels at renal biopsy ((25±6) vs. (29±7) g/L, t=2.14, P=0.041). There were 12 patients with typical PMN and 21 patients with atypical PMN, and no significant difference in clinical and pathological manifestations was found between these 2 groups (all P>0.05). There were 10 cases (32.3%) with glomerular C1q deposition, and their disease course before renal biopsy was significantly shorter than those without C1q deposition (1.8 (0.8, 5.9) vs. 6.0 (2.5, 22.3) months, Z=-2.27, P=0.023). Twelve cases (36.4%) had glomerular IgA deposition, and their course of disease,clinical and pathological manifestations were not significantly different from those without IgA deposition (all P>0.05). Conclusion: Glomerular C1q or IgA deposition may not affect the clinical manifestations, glomerular PLA2R and IgG subclasses staining pattern, or the response to treatment of PMN in children.
Autoantibodies ; Child ; Complement C1q/metabolism* ; Female ; Glomerulonephritis, Membranous/drug therapy* ; Humans ; Immunoglobulin A/immunology* ; Immunoglobulin G ; Kidney Glomerulus ; Male ; Retrospective Studies

No abstract available.
Antiviral Agents/*therapeutic use ; Female ; Glomerulonephritis, Membranous/*drug therapy/*etiology ; Hepatitis B, Chronic/*complications/*drug therapy ; Humans ; Lamivudine/*therapeutic use ; Male

OBJECTIVETo investigate retrospectively the efficacy of cyclosporine A (CsA) in the treatment of membranous lupus nephropathy (MLN).

METHODSTwenty-four patients with systemic lupus erythematosus (SLE) and biopsy-proven MLN were treated with CsA in combination with prednisone. CsA was given at a starting dosage of 5 mg x kg(-1) x d(-1) for 3 months, with a 1 mg x kg(-1) x d(-1) reduction every month and then maintained at a dosage of 2 mg x kg(-1) x d(-1). The dosage of oral prednisone differed from person to person according to levels of extra-renal activity. Clinical efficacy and adverse reactions were retrospectively analyzed. Complete remission was defined as having a urinary proteinuria level (Upr) of < 0.4 g/d, and normal serum albumin and serum creatinine (SCr) levels, without SLE activity. Partial remission was defined as having a UPr decrement > 50% of baseline value and a serum albumin value of 30 - 35 g/L, without SLE activity. No response was defined as having a Upr decrement < 50% of baseline value and > 2.0 g/d, or as a deterioration of renal function, or as having active SLE.

RESULTSOne patient could no longer undergo follow-up, and the other 23 patients were treated with CsA and followed up for 6 - 36 months (mean 16.8 +/- 8.4 months). The mean starting dosage of CsA was 4.7 +/- 0.5) mg x kg(-1) x d(-1) and the trough level of the whole blood CsA was 248 +/- 110) micro g/L. Twelve patients (52.2%) achieved complete remission, 10 patients (43.3%) achieved partial remission after CsA treatment, and one patient showed no response. At different CsA treatment timepoint, the complete remission rates were 17.4% (3rd month), 21.7% (6th month), 40% (12th month), 88.9% (18th month) and 100% (24th month) respectively. SCr elevation, when within a normal limit was not observed in most patients during early CsA administration, and at the end of the follow-up all the patients had a normal SCr. Relapse occurred in 33.3% of the patients after withdrawing CsA for 4 - 24 months. No chronic CsA renal toxicity was observed in 4 patients who had a repeat renal biopsy after CsA treatment for 6 - 24 months.

CONCLUSIONSCsA could be regarded as an effective therapy for patients with membranous lupus nephropathy, but its adverse effects, especially its nephrotoxicity, should be carefully monitored during CsA treatment.

Adolescent ; Adult ; Cyclosporine ; administration & dosage ; therapeutic use ; Female ; Glomerulonephritis, Membranous ; drug therapy ; Humans ; Lupus Nephritis ; drug therapy ; Male ; Prednisone ; administration & dosage ; Retrospective Studies ; Treatment Outcome

BACKGROUNDAccording to the renal phospholipase A2 receptor (PLA2R) immunohistochemistry, idiopathic membranous nephropathy (iMN) could be categorized into PLA2R-associated and non-PLA2R-associated iMN. This study aimed to examine whether the non-PLA2R-associated iMN had any difference in clinical features compared with PLA2R-associated iMN.

METHODSA total of 231 adult patients diagnosed as iMN were recruited to this retrospective study. Renal PLA2R expression was examined by immunofluorescence. Among these patients, 186 (80.5%) with complete baseline clinical data were used for further study. Urinary protein excretion, serum albumin, and creatinine were analyzed. For those patients with follow-up longer than 1 year, the relationship between PLA2R and response to immunosuppressants were analyzed. The t-test was used for parametric analysis and the Mann-Whitney U-test was used for nonparametric analysis. Categorical variables were described as frequencies or percentages, and the data were analyzed with Pearson's Chi-square test or Fisher's exact test.

RESULTSOf the 231 iMN patients, 189 showed renal detectable PLA2R expression (81.8%). The baseline serum creatinine, serum albumin, and urine protein excretion were not significantly different between PLA2R-associated (n = 145) and non-PLA2R-associated iMN patients (n = 41). However, about 1/3 of the non-PLA2R-associated iMN had abnormal serological tests, significantly more common than PLA2R-associated iMN (31.7% vs. 8.3%, P = 0.000). The non-PLA2R-associated iMN had lower C4 levels compared with PLA2R-associated iMN (P = 0.004). The non-PLA2R-associated iMN patients also showed a better response to immunosuppressants (complete remission [CR] 42.9%; partial remission [PR] 14.3%) compared with PLA2R-associated iMN (CR 3.2%; PR 48.4%, P = 0.004) at the 3rd month.

CONCLUSIONSThere were no significant differences in serum creatinine, albumin, and urine protein excretion between PLA2R-associated and non-PLA2R-associated iMN, while the non-PLA2R-associated iMN patients showed more abnormal serological tests. The non-PLA2R-associated iMN seemed to respond more quickly to the immunosuppressive therapy compared with PLA2R-associated iMN.

Adult ; Autoantibodies ; metabolism ; Female ; Glomerulonephritis, Membranous ; drug therapy ; metabolism ; pathology ; urine ; Humans ; Immunosuppressive Agents ; therapeutic use ; Kidney ; metabolism ; pathology ; Male ; Middle Aged ; Receptors, Phospholipase A2 ; metabolism ; Retrospective Studies