BACKGROUND Telitacicept reduces B cell activation and abnormal immunoglobulin A(IgA)antibody production by inhibiting the activity of B lymphocyte stimulator(BLyS)and a proliferation-inducing ligand(APRIL),thereby dec...BACKGROUND Telitacicept reduces B cell activation and abnormal immunoglobulin A(IgA)antibody production by inhibiting the activity of B lymphocyte stimulator(BLyS)and a proliferation-inducing ligand(APRIL),thereby decreasing IgA deposition in the glomeruli and local inflammatory response.This ultimately protects the kidneys from damage.This mechanism suggests that Telitacicept has potential efficacy in the treatment of IgA nephropathy.CASE SUMMARY We present the case of a 24-year-old female who was diagnosed with IgA nephropathy due to significant proteinuria and mild renal impairment.Pathologically,she exhibited focal proliferative glomerulonephritis.Treatment with angiotensin II receptor blocker,hormones,and mycophenolate mofetil did not lead to a significant improvement in her condition.However,upon the addition of telitacicept,the patient’s renal function recovered and her proteinuria rapidly reduced.Hormones were swiftly tapered and discontinued,with no occurrence of severe infections or related complications.CONCLUSION Telitacicept combined with hormones and mycophenolate mofetil may be a safe and effective induction therapy for IgA nephropathy.展开更多
BACKGROUND The co-occurrence of Anti-phospholipase A2 receptor-associated membranous nephropathy(anti-PLA2R-MN)and human immunodeficiency virus(HIV)infection is a rare clinical scenario,presenting significant challeng...BACKGROUND The co-occurrence of Anti-phospholipase A2 receptor-associated membranous nephropathy(anti-PLA2R-MN)and human immunodeficiency virus(HIV)infection is a rare clinical scenario,presenting significant challenges in terms of management and treatment.CASE SUMMARY A 32-year-old Chinese male diagnosed with HIV infection presented with a clinical history of proteinuria persisting for over two years.A kidney biopsy demonstrated subepithelial immune complex deposition and a thickened glomerular basement membrane,indicative of stage I-II membranous nephro-pathy.Immunofluorescence staining revealed granular deposition of PLA2R(3+)along the glomerular capillary loops,corroborated by a strongly positive anti-PLA2R antibody test(1:320).Initial treatment involving losartan potassium,rivaroxaban,tacrolimus,and rituximab was discontinued due to either poor effec-tiveness or the occurrence of adverse events.Following a regimen of weekly subcutaneous injections of telitacicept(160 mg),a marked decline in the 24 h urine protein was observed within a three-month period,accompanied by a rise in serum albumin level.No significant reductions in peripheral blood CD3+CD4+T and CD3+CD8+T cell counts were detected.The patient's physical and psychological conditions showed significant improvements,with no adverse events reported during the treatment course.CONCLUSION Telitacicept might offer a potential therapeutic avenue for patients diagnosed with anti-PLA2R-MN concomitant with HIV infection.展开更多
Introduction:Thrombocytopenia,a common noncriteria manifestation of antiphospholipid syndrome(APS),is severe in approximately one-third of patients with APS.However,there are no guidelines for treating such thrombocyt...Introduction:Thrombocytopenia,a common noncriteria manifestation of antiphospholipid syndrome(APS),is severe in approximately one-third of patients with APS.However,there are no guidelines for treating such thrombocytopenia.B-cell-targeting therapy may be an option in refractory cases;however,its efficacy has not been firmly established.Here,we report on two patients with refractory antiphospholipid antibodies(aPLs)-associated thrombocytopenia treated with telitacicept.Case Description:Case 1,a 39-year-old woman,presented with systemic lupus erythematosus(SLE)and APS with diffuse alveolar hemorrhage,persistent thrombocytopenia,and recurrent miscarriages.The thrombocytopenia had been refractory to multiple lines of treatments,the most recent being mycophenolate mofetil and prednisone(5 mg/day).After receiving telitacicept(160 mg/week)for 3 months,she had presented with decreased titers of aPLs and a slight increase in platelet counts(14×10^(9)to 35×10^(9)/L).Case 2,a 51-year-old woman,presented with SLE and APS with refractory thrombocytopenia.She had been diagnosed with pulmonary tuberculosis 4.5 years ago and received antituberculosis therapy for 2 years.She had also undergone pulmonary lobectomy 4 years ago for lung adenocarcinoma.Her thrombocytopenia relapsed(lowest 14×10^(9)/L)when prednisone was tapered to<10mg/day.After adding telitacicept(160 mg/week)to cyclosporin A(150 mg/day)and hydroxychloroquine(400 mg/day)for 5 months,aPLs decreased and platelet counts increased(78×10^(9)to 183×10^(9)/L),enabling halving of her dose of prednisone from 15 to 7.5 mg/day.Neither patient had any adverse effects.Conclusion:Telitacicept can safely reduce aPLs titers and improve refractory thrombocytopenia in patients with secondary APS.Randomized-controlled trials to assess its effectiveness are urgently required.展开更多
Immunoglobulin(Ig)A nephropathy is the most common type of primary glomerulonephritis globally.It typically manifests with microscopic hematuria and a spectrum of proteinuria,although rapidly progressive glomeruloneph...Immunoglobulin(Ig)A nephropathy is the most common type of primary glomerulonephritis globally.It typically manifests with microscopic hematuria and a spectrum of proteinuria,although rapidly progressive glomerulonephritis may occur in rare instances.Deposition of IgA in the mesangium seems to be the underlying disease mechanism.Despite current treatment,IgA nephropathy may progress into end-stage renal disease,indicating the necessity for the development of new therapeutic agents.Lifestyle modifications and anti-proteinuric treatment are recommended,and steroids have shown to be beneficial to high risk groups.Nevertheless,other conventional immunosuppressive agents,such as cyclophosphamide and mycophenolate mofetil,may be considered,despite the lack of sufficient evidence to support their efficacy.A considerable proportion of cases remain unresponsive to these treatments,underscoring the need for novel therapeutic approaches.There are several promising immunosuppressive drugs,such as B-cell lineage depleting agents or complement system inhibitors,that are currently undergoing clinical trials.These therapies may be considered for use in selected cases.展开更多
文摘BACKGROUND Telitacicept reduces B cell activation and abnormal immunoglobulin A(IgA)antibody production by inhibiting the activity of B lymphocyte stimulator(BLyS)and a proliferation-inducing ligand(APRIL),thereby decreasing IgA deposition in the glomeruli and local inflammatory response.This ultimately protects the kidneys from damage.This mechanism suggests that Telitacicept has potential efficacy in the treatment of IgA nephropathy.CASE SUMMARY We present the case of a 24-year-old female who was diagnosed with IgA nephropathy due to significant proteinuria and mild renal impairment.Pathologically,she exhibited focal proliferative glomerulonephritis.Treatment with angiotensin II receptor blocker,hormones,and mycophenolate mofetil did not lead to a significant improvement in her condition.However,upon the addition of telitacicept,the patient’s renal function recovered and her proteinuria rapidly reduced.Hormones were swiftly tapered and discontinued,with no occurrence of severe infections or related complications.CONCLUSION Telitacicept combined with hormones and mycophenolate mofetil may be a safe and effective induction therapy for IgA nephropathy.
文摘BACKGROUND The co-occurrence of Anti-phospholipase A2 receptor-associated membranous nephropathy(anti-PLA2R-MN)and human immunodeficiency virus(HIV)infection is a rare clinical scenario,presenting significant challenges in terms of management and treatment.CASE SUMMARY A 32-year-old Chinese male diagnosed with HIV infection presented with a clinical history of proteinuria persisting for over two years.A kidney biopsy demonstrated subepithelial immune complex deposition and a thickened glomerular basement membrane,indicative of stage I-II membranous nephro-pathy.Immunofluorescence staining revealed granular deposition of PLA2R(3+)along the glomerular capillary loops,corroborated by a strongly positive anti-PLA2R antibody test(1:320).Initial treatment involving losartan potassium,rivaroxaban,tacrolimus,and rituximab was discontinued due to either poor effec-tiveness or the occurrence of adverse events.Following a regimen of weekly subcutaneous injections of telitacicept(160 mg),a marked decline in the 24 h urine protein was observed within a three-month period,accompanied by a rise in serum albumin level.No significant reductions in peripheral blood CD3+CD4+T and CD3+CD8+T cell counts were detected.The patient's physical and psychological conditions showed significant improvements,with no adverse events reported during the treatment course.CONCLUSION Telitacicept might offer a potential therapeutic avenue for patients diagnosed with anti-PLA2R-MN concomitant with HIV infection.
基金Peking University People's Hospital Research and Development Funds,Grant/Award Numbers:RDY 2019-04,RDJP2022-12National Natural Science Foundation of China,Grant/Award Numbers:81801615,81871289。
文摘Introduction:Thrombocytopenia,a common noncriteria manifestation of antiphospholipid syndrome(APS),is severe in approximately one-third of patients with APS.However,there are no guidelines for treating such thrombocytopenia.B-cell-targeting therapy may be an option in refractory cases;however,its efficacy has not been firmly established.Here,we report on two patients with refractory antiphospholipid antibodies(aPLs)-associated thrombocytopenia treated with telitacicept.Case Description:Case 1,a 39-year-old woman,presented with systemic lupus erythematosus(SLE)and APS with diffuse alveolar hemorrhage,persistent thrombocytopenia,and recurrent miscarriages.The thrombocytopenia had been refractory to multiple lines of treatments,the most recent being mycophenolate mofetil and prednisone(5 mg/day).After receiving telitacicept(160 mg/week)for 3 months,she had presented with decreased titers of aPLs and a slight increase in platelet counts(14×10^(9)to 35×10^(9)/L).Case 2,a 51-year-old woman,presented with SLE and APS with refractory thrombocytopenia.She had been diagnosed with pulmonary tuberculosis 4.5 years ago and received antituberculosis therapy for 2 years.She had also undergone pulmonary lobectomy 4 years ago for lung adenocarcinoma.Her thrombocytopenia relapsed(lowest 14×10^(9)/L)when prednisone was tapered to<10mg/day.After adding telitacicept(160 mg/week)to cyclosporin A(150 mg/day)and hydroxychloroquine(400 mg/day)for 5 months,aPLs decreased and platelet counts increased(78×10^(9)to 183×10^(9)/L),enabling halving of her dose of prednisone from 15 to 7.5 mg/day.Neither patient had any adverse effects.Conclusion:Telitacicept can safely reduce aPLs titers and improve refractory thrombocytopenia in patients with secondary APS.Randomized-controlled trials to assess its effectiveness are urgently required.
文摘Immunoglobulin(Ig)A nephropathy is the most common type of primary glomerulonephritis globally.It typically manifests with microscopic hematuria and a spectrum of proteinuria,although rapidly progressive glomerulonephritis may occur in rare instances.Deposition of IgA in the mesangium seems to be the underlying disease mechanism.Despite current treatment,IgA nephropathy may progress into end-stage renal disease,indicating the necessity for the development of new therapeutic agents.Lifestyle modifications and anti-proteinuric treatment are recommended,and steroids have shown to be beneficial to high risk groups.Nevertheless,other conventional immunosuppressive agents,such as cyclophosphamide and mycophenolate mofetil,may be considered,despite the lack of sufficient evidence to support their efficacy.A considerable proportion of cases remain unresponsive to these treatments,underscoring the need for novel therapeutic approaches.There are several promising immunosuppressive drugs,such as B-cell lineage depleting agents or complement system inhibitors,that are currently undergoing clinical trials.These therapies may be considered for use in selected cases.
