Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Type II mixed cryoglobulinemia (MC) is a systemic vasculitis, associated in most cases with hepatitis C virus (HCV) infection, sustained by proliferation of oligoclonal cells. Systemic B cell depletion and clinical remission can be achieved in non-Hodgkin lymphoma by human/mouse chimeric monoclonal antibody that specifically reacts with the CD20 antigen (rituximab). Similar effects could be expected in type II MC. Twelve patients, mean age 61.9 years (range 37-76), 11 with HCV infection genotype 2a2c (4 cases) or 1b (6 cases) and 3 (1 case) and symptomatic type II MC with systemic manifestations, including renal involvement, marrow clonal restriction, large necrotizing ulcers, and polyneuropathy, were considered eligible for rituximab therapy because of resistance or intolerance to conventional therapy or important bone marrow infiltration. Rituximab was administered intravenously at a dose of 375 mg/m2 on days 1, 8, 15, and 22. Two more doses were administered 1 and 2 months later. No other immunosuppressive drugs were added. Response was evaluated by assessing the changes in clinical signs, symptoms, and laboratory parameters. Levels of proteinuria, hematuria, erythrocyte sedimentation rate, cryocrit, rheumatoid factor, and IgM decreased while C4 values increased and HCV viral load remained stable during short- and medium-term observation. Bone marrow abnormalities were found to reverse to normal. Constitutional symptoms disappeared or ameliorated. No acute or delayed side effects were seen. Based on this experience and a number of reports published in the last 5 years, Rituximab appears to be a safe and effective therapeutic option in symptomatic patients with HCV-associated MC with signs of systemic vasculitis.
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PMID:Rituximab as a therapeutic tool in severe mixed cryoglobulinemia. 1827 Aug 64

A 13-year-old girl with obesity and hyperinsulinism developed steroid-resistant nephrotic syndrome due to collapsing glomerulopathy with dominant C1q-containing mesangial immune deposits (CG/C1qN). She became overtly diabetic while receiving alternate-day prednisone and tacrolimus, requiring insulin injections. Despite the addition of mycophenolate mofetil to the treatment regimen, renal function subsequently declined. Rituximab (four weekly doses of 375 mg/m2) was tried 6 months after initial presentation and 3 months after weaning all glucocorticoids. Glomerular filtration rate (GFR) and proteinuria improved. Unexpectedly, blood sugar control normalized 6 weeks after antibody infusion. Rituximab was readministered 20 months after the first course because of deteriorating renal function, but the effect on GFR and proteinuria was modest. A retrospective analysis revealed that tubulointerstitial infiltrates present in the biopsies prior to treatment with rituximab contained numerous CD20+ and CD3+ (CD4 > CD8) lymphocyte aggregates. Rebiopsy 10 weeks after repeat rituximab therapy demonstrated the elimination of B-cell infiltrates and the apparent decrease of interstitial T-cell infiltrates, yet persistent, advanced global glomerulosclerosis, interstitial fibrosis and tubular atrophy. In conclusion, CG/C1qN was associated with B- and T-cell-rich tubulointerstitial infiltrates. B-cell-directed therapy delayed clinical progression during early disease but failed to prevent or ameliorate chronic changes, despite effective tissue B-cell clearance. The incidental resolution of diabetes was noted after rituximab treatment.
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PMID:Rituximab treatment of collapsing C1q glomerulopathy: clinical and histopathological evolution. 1835 94

We describe a 10-year-old severe hemophilia B boy with a stop codon mutation of exon 2 in the factor IX gene who developed high inhibitor of 70 Bethesda units (BU) from 12 months of age after exposure to prothrombin complex concentrate for 14 days. The inhibitor spontaneously disappeared within 3 months. The patient, however, exhibited anaphylactic reaction to the administration of prothrombin complex concentrate and factor IX concentrate at ages 15 and 23 months, respectively. Although recombinant activated factor VII was alternatively given, he suffered from progressive hemophilic arthropathy. At the age of 10 years, the boy underwent desensitization to factor IX concentrate and could tolerate factor IX concentrate of 40 U/kg administered on day 9 of desensitization. Unfortunately, the inhibitor of 16 BU was detected on day 6 and rapidly increased to 180 BU on day 9 of desensitization. Rituximab 375 mg/m2 per week was therefore immediately initiated on day 10 and a total of four doses were given. The inhibitor gradually decreased to 21.5 BU after the fourth dose of rituximab. The daily factor IX concentrate administration of 40 U/kg was continued for 1 month and decreased to three times per week for another month, and then to once to twice per week for the remaining 14 months of desensitization. The patient was able to attend regular school and the most recent inhibitor ranged from 4.4 to 10 BU. No proteinuria or alteration of renal function was found. In conclusion, rituximab is a helpful adjuvant to immune tolerance therapy in a hemophilia B boy with inhibitor and anaphylaxis to factor IX concentrate.
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PMID:The use of rituximab as an adjuvant for immune tolerance therapy in a hemophilia B boy with inhibitor and anaphylaxis to factor IX concentrate. 1838

The incidence of nephrotic syndrome co-existing with chronic lymphocytic leukemia (CLL) is a rare condition. Almost any glomerular pathology may accompany CLL. The most frequent of all is membranoproliferative glomerulonephritis (MPGN). Moreover, in 5 - 10% of patients with CLL, monoclonal gammopathy may be detected in serum and/or urine samples. There are no well-established treatment protocols for those CLL patients with accompanying nephrotic syndrome. In this case report, we present a 55-year-old female patient diagnosed with CLL, developing nephrotic syndrome, renal dysfunction and IgG k-type monoclonal gammopathy in the follow-up. The renal biopsy revealed glomerular and tubular deposits of k-chain and histopathology of membranoproliferative glomerulonephritis. Rituximab along with CVP (cyclophosphamide - vincristine - prednisolone) chemotherapy regimen was initiated. At the end of 6 courses of treatment, the patient was on "nephrologic" partial remission as the serum creatinine and albumin levels had returned to normal and proteinuria decreased by more than 50%. The patient was also in partial remission for CLL. In conclusion, in patients with CLL and nephrotic syndrome, presence of MPGN along with light-chain nephropathy is rarely reported. Several different treatment protocols are discussed for these patients. Among these regimes, R-CVP is an acceptable alternative for CLL patients with MPGN.
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PMID:Membranoproliferative glomerulonephritis and light-chain nephropathy in association with chronic lymphocytic leukemia. 1904 12

Immunoglobulin M (IgM) nephropathy is an idiopathic glomerulonephritis characterized by mesangial deposits of IgM. IgM nephropathy presenting with proteinuria, especially nephrotic syndrome, frequently is steroid dependent or steroid resistant and associated with reaching end-stage renal disease after a 15-year follow-up. Because no long-term effective treatment is known for patients with IgM nephropathy, there is a clear need for therapeutic alternatives. We describe a patient who reached end-stage renal disease 20 years after IgM nephropathy was diagnosed at the age of 3 years. IgM nephropathy recurred after kidney transplantation, leading to microscopic hematuria and proteinuria. High-dose steroid therapy was not effective, and kidney function slowly decreased. Three years after transplantation, 2 doses of rituximab were administered, leading to complete remission of the IgM nephropathy. One year after rituximab treatment, the patient has stable kidney function, normal urinary sediment, and no proteinuria. Rituximab may be a valuable novel therapeutic drug for the treatment of patients with IgM nephropathy.
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PMID:Resolution of IgM nephropathy after rituximab treatment. 1908 9

Resistance to steroids and immunosuppression in pediatric nephrotic syndrome may be related to focal segmental glomeruloslerosis (FSGS). Rituximab, monoclonal anti-B-CD20-cell antibody is currently regarded as novel effective drug in selected cases. We describe the case of 8-years-old male pediatric patient, resistant to combined immunosuppression and presenting renal insufficiency (GFR 32.8 ml/min/1.73 m2). Patient was given overall 5 doses of rituximab [375 mg/m2/dose]. Nevertheless significant decrease of proteinuria, the further progress of renal disease was unaffected and patient developed end-stage renal failure. The efficacy of rituximab in nephrotic syndrome must be verified in controlled trials. Late onset of therapy in course of renal insufficiency might be the one of the reasons of treatment failure in our case.
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PMID:[No effect of rituximab in pediatric case of severe nephrotic syndrome and focal segmental glomeruloslerosis accompanied by renal insufficiency]. 1920 86

Corticoresistant idiopathic nephrotic syndrome (INS) is a glomerulopathy of unknown etiology whose original aspect is its recurrence after kidney transplantation in 30 to 50% of patients with end-stage renal disease. This suggests the involvement of circulating factors that would alter the glomerular filtration barrier, but whose nature remains elusive. Although a T cell immune origin has been suggested, the actual role of these cells in INS recurrence is still unclear. Here we present an 8-year-old patient with corticoresistant INS who developed a recurrence of her initial disease after kidney transplantation. Rituximab therapy was proposed 11 months after transplantation; although no immediate effect was induced, a slow but persistent decrease in proteinuria began a few months after Rituximab infusions despite cessation of plasma exchanges and steroid therapy. The pathophysiology of INS and the putative mechanisms of action of Rituximab are discussed.
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PMID:New insights into the pathophysiology of idiopathic nephrotic syndrome. 1941 May 18

A 43-year-old black male was brought to hospital with complaints of confusion and fever. He was noted to have petechial lesions, thrombocytopenia (platelet count 7,200/ml), schistocytes on peripheral smear, and serum creatinine 1.7 mg/dl (150.28 micromol/L). He was diagnosed to have thrombotic thrombocytopenic purpura (TTP) and started on high dose IV steroids and plasmapheresis. Attempts at steroid withdrawal following plasmapheresis were unsuccessful as his platelet count started to decrease. He subsequently was started on rituximab given as 4 weekly infusions. The platelet count normalized after 2 doses of rituximab. A kidney biopsy performed to evaluate proteinuria (10.24 gms/24 hr) revealed membranous nephropathy (MN), with organized obliterative arteriopathy consistent with thrombotic microangiopathy. Upon completion of the treatment, proteinuria decreased to 1.67 gm/24hr. Recent studies indicate that patients with TTP have an inhibitory (auto) antibody to von Willibrand factor cleaving protease (ADAMTS 13). Considerable evidence also exists that idiopathic membranous nephropathy is an autoimmune disease. Rituximab, a monoclonal chimeric antibody directed against CD20 antigen present on B cells, selectively depletes B cells and has been used with success in both diseases. Though evidence for a direct pathogenetic relationship between TTP and MN is lacking, the two entities are more likely related to autoantibodies induced by activation of B cells. For our patient with this rare disease combination rituximab therapy was one treatment solution to two diseases.
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PMID:Membranous nephropathy and thrombotic thrombocytopenic purpura treated with rituximab. 1966 13

Posttransplantation B-lymphoproliferative (PTBL) disease is a severe complication of organ transplantation, which requires reduction of immunosuppressive treatment. The use of the anti-CD20 monoclonal antibody, Rituximab, improves the survival of these patients. In this setting, maintenance immunosuppressive therapy may represent a challenge. The mammalian target of rapamycin (m-TOR) inhibitor Rapamycin has antiproliferative effects that makes it a safe, efficient option to avoid graft rejection and reduce the malignancy risk. We studied 6 renal recipients (4 men and 2 women) of overall mean age of 50.66 +/- 15.89 years who were diagnosed with lymphoma at a mean time of graft function of 137.0 +/- 68.00 months. All of the patients were Epstein-Barr-negative. Four received a combination of Rituximab and cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP), and 2 received Rituximab only. In all cases complete remission persisted during follow-up of 21.83 +/- 8.34 months. The immunosuppressive treatment was switched to the m-TOR inhibitor Rapamycin at therapeutic trough blood levels of 5-8 ng/dL. The mean time of Rapamycin treatment was 15.5 +/- 8.96 months. Notably, we observed neither acute rejection nor relapse episodes. Renal function remained stable with no significant proteinuria. The serum creatinine level before switching to Rapamycin was 1.06 +/- 0.16 mg/dL and 0.9 +/- 0.14 mg/dL 12 months later. However, 1 patient had to stop Rapamycin treatment due to pneumonitis. Our study suggests that immunosuppressant monotherapy with Rapamycin is safe and efficient for renal recipients who develop lymphoma because of its antitumor effects without nephrotoxicity.
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PMID:Monotherapy rapamycin in renal transplant recipients with lymphoma successfully treated with rituximab. 1971 44

Membranous nephropathy (MN) recurs posttransplant in 42% of patients. We compared MN recurrence rates in a historical cohort transplanted between 1990 and 1999 and in a current cohort diagnosed by protocol biopsies, we analyzed the progression of the disease and we assessed the effects of anti-CD20 antibodies (Rituximab) on recurrent MN. The incidence of recurrent MN was similar in the historical (53%) and the current cohorts (41%), although in the later the diagnosis was made earlier (median, 4[2-21] months vs. 83[6-149], p = 0.002) and the disease was clinically milder. Twelve out of 14 patients (86%) with recurrent MN in the current cohort had progressive increases in proteinuria. Eight recipients were treated with Rituximab after their proteinuria increased from median, 211 mg/day (64-4898) at diagnosis to 4489 (898-13 855) (p = 0.038). Twelve months post-Rituximab, 75% of patients had either partial (PR) or complete remission (CR). After 24 months 6/7 (86%) had PR/CR and one patient relapsed. Posttreatment biopsies showed resorption of electron dense immune deposits in 6/7 cases and were negative for C3 (4/7) and IgG (3/7). Protocol biopsies allow early diagnosis of subclinical recurrent MN, which is often progressive. Treatment of recurrent MN with Rituximab is promising and should be evaluated in a prospective randomized controlled trial.
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PMID:Recurrent idiopathic membranous nephropathy: early diagnosis by protocol biopsies and treatment with anti-CD20 monoclonal antibodies. 2019 9


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