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Article ; Online: Complement involvement in sickle cell disease.

Meuleman, Marie-Sophie / Roumenina, Lubka T / Grunenwald, Anne

Presse medicale (Paris, France : 1983)

2023 Volume 52, Issue 4, Page(s) 104205

Abstract: Sickle Cell Disease (SCD) is a hereditary blood disorder characterized by the presence of abnormal hemoglobin, leading to the formation of sickle-shaped red blood cells, causing vaso-occlusion. Inflammation is a key component of the pathophysiology of ... ...

Abstract	Sickle Cell Disease (SCD) is a hereditary blood disorder characterized by the presence of abnormal hemoglobin, leading to the formation of sickle-shaped red blood cells, causing vaso-occlusion. Inflammation is a key component of the pathophysiology of SCD, contributing to the vascular complications and tissue damage. This review is centered on exploring the role of the inflammatory complement system in the pathophysiology of SCD. Our goal is to offer a comprehensive summary of the existing evidence regarding complement activation in patients with SCD, encompassing both steady-state conditions and episodes of vaso-occlusive events. Additionally, we will discuss the proposed mechanisms by which the complement system may contribute to tissue injury in this pathology. Finally, we will provide an overview of the available evidence concerning the effectiveness of therapeutic interventions aimed at blocking the complement system in the context of SCD and discuss the perspective of complement inhibition.
MeSH term(s)	Humans ; Anemia, Sickle Cell/complications ; Anemia, Sickle Cell/drug therapy ; Vascular Diseases/etiology ; Inflammation/complications
Language	English
Publishing date	2023-11-14
Publishing country	France
Document type	Review ; Journal Article
ZDB-ID	120943-7
ISSN	2213-0276 ; 0032-7867 ; 0755-4982 ; 0301-1518
ISSN (online)	2213-0276
ISSN	0032-7867 ; 0755-4982 ; 0301-1518
DOI	10.1016/j.lpm.2023.104205
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Zs.A 794: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
Ua VI Zs.87: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular ab Jg. 2022: Lesesaal (EG)

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Article ; Online: Complement C3-targeted therapy in C3 glomerulopathy, a prototype of complement-mediated kidney diseases.

Meuleman, Marie-Sophie / Grunenwald, Anne / Chauvet, Sophie

Seminars in immunology

2022 , Page(s) 101634

Abstract: C3 glomerulopathy (C3G) is a rare and complex kidney disease that primarily affects young adults. Renal outcomes remain poor in the absence of specific treatment. C3G is driven by uncontrolled overactivation of the alternative complement pathway, which ... ...

Abstract	C3 glomerulopathy (C3G) is a rare and complex kidney disease that primarily affects young adults. Renal outcomes remain poor in the absence of specific treatment. C3G is driven by uncontrolled overactivation of the alternative complement pathway, which is mainly of acquired origin. Functional characterization of complement abnormalities (i.e., autoantibodies targeting complement components and variants in complement genes) identified in patients and experimental models of the disease improved the understanding of the disease, making C3G a prototype of complement-mediated diseases. The contribution of C3 convertase, as well as C5 convertase, in disease occurrence, phenotype, and severity is now well established, offering various potential therapeutic interventions. However, the lack of sufficient efficiency in anti-C5 therapy highlights the extreme complexity of the disease and the need for new therapeutic approaches based on C3 and C3 convertase axis inhibition. Here, we provide an overview of the complement activation mechanism involved in C3G and discuss therapeutic options based on complement inhibitors, with a specific focus on C3 inhibition.
Language	English
Publishing date	2022-07-08
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	1018141-6
ISSN	1096-3618 ; 1044-5323
ISSN (online)	1096-3618
ISSN	1044-5323
DOI	10.1016/j.smim.2022.101634
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Article: Renal Diseases Associated with Hematologic Malignancies and Thymoma in the Absence of Renal Monoclonal Immunoglobulin Deposits.

Morel, Antoine / Meuleman, Marie-Sophie / Moktefi, Anissa / Audard, Vincent

Diagnostics (Basel, Switzerland)

2021 Volume 11, Issue 4

Abstract: In addition to kidney diseases characterized by the precipitation and deposition of overproduced monoclonal immunoglobulin and kidney damage due to chemotherapy agents, a broad spectrum of renal lesions may be found in patients with hematologic ... ...

Abstract	In addition to kidney diseases characterized by the precipitation and deposition of overproduced monoclonal immunoglobulin and kidney damage due to chemotherapy agents, a broad spectrum of renal lesions may be found in patients with hematologic malignancies. Glomerular diseases, in the form of paraneoplastic glomerulopathies and acute kidney injury with various degrees of proteinuria due to specific lymphomatous interstitial and/or glomerular infiltration, are two major renal complications observed in the lymphoid disorder setting. However, other hematologic neoplasms, including chronic lymphocytic leukemia, thymoma, myeloproliferative disorders, Castleman disease and hemophagocytic syndrome, have also been associated with the development of kidney lesions. These renal disorders require prompt recognition by the clinician, due to the need to implement specific treatment, depending on the chemotherapy regimen, to decrease the risk of subsequent chronic kidney disease. In the context of renal disease related to hematologic malignancies, renal biopsy remains crucial for accurate pathological diagnosis, with the aim of optimizing medical care for these patients. In this review, we provide an update on the epidemiology, clinical presentation, pathophysiological processes and diagnostic strategy for kidney diseases associated with hematologic malignancies outside the spectrum of monoclonal gammopathy of renal significance.
Language	English
Publishing date	2021-04-15
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2662336-5
ISSN	2075-4418
ISSN	2075-4418
DOI	10.3390/diagnostics11040710
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Article ; Online: Ex Vivo Complement Activation on Endothelial Cells: Research and Translational Value.

Meuleman, Marie-Sophie / Fremeaux-Bacchi, Veronique / Roumenina, Lubka T / Chauvet, Sophie

Trends in molecular medicine

2021 Volume 27, Issue 5, Page(s) 418–421

Abstract: The spectrum of human diseases with complement contribution is ever increasing. Tools to study the complement contribution and the potential interest of novel complement inhibitors in clinical practice are lacking. Here we discuss a functional ex vivo ... ...

Abstract	The spectrum of human diseases with complement contribution is ever increasing. Tools to study the complement contribution and the potential interest of novel complement inhibitors in clinical practice are lacking. Here we discuss a functional ex vivo assay to monitor complement activation on endothelial cells, which can answer to this need.
MeSH term(s)	Complement Activation/physiology ; Complement Inactivating Agents ; Complement System Proteins/immunology ; Complement System Proteins/physiology ; Endothelial Cells/immunology ; Endothelial Cells/physiology ; Humans ; Immunity, Innate
Chemical Substances	Complement Inactivating Agents ; Complement System Proteins (9007-36-7)
Language	English
Publishing date	2021-02-26
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	2036490-8
ISSN	1471-499X ; 1471-4914
ISSN (online)	1471-499X
ISSN	1471-4914
DOI	10.1016/j.molmed.2021.01.008
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Article ; Online: Ex Vivo

Meuleman, Marie-Sophie / Duval, Anna / Fremeaux-Bacchi, Véronique / Roumenina, Lubka T / Chauvet, Sophie

Frontiers in immunology

2022 Volume 13, Page(s) 860689

Abstract: As part of the innate immune system, the complement system plays a key role in defense against pathogens and in host cell homeostasis. This enzymatic cascade is rapidly triggered in the presence of activating surfaces. Physiologically, it is tightly ... ...

Abstract	As part of the innate immune system, the complement system plays a key role in defense against pathogens and in host cell homeostasis. This enzymatic cascade is rapidly triggered in the presence of activating surfaces. Physiologically, it is tightly regulated on host cells to avoid uncontrolled activation and self-damage. In cases of abnormal complement dysregulation/overactivation, the endothelium is one of the primary targets. Complement has gained momentum as a research interest in the last decade because its dysregulation has been implicated in the pathophysiology of many human diseases. Thus, it appears to be a promising candidate for therapeutic intervention. However, detecting abnormal complement activation is challenging. In many pathological conditions, complement activation occurs locally in tissues. Standard routine exploration of the plasma concentration of the complement components shows values in the normal range. The available tests to demonstrate such dysregulation with diagnostic, prognostic, and therapeutic implications are limited. There is a real need to develop tools to demonstrate the implications of complement in diseases and to explore the complex interplay between complement activation and regulation on human cells. The analysis of complement deposits on cultured endothelial cells incubated with pathologic human serum holds promise as a reference assay. This
MeSH term(s)	Atypical Hemolytic Uremic Syndrome/diagnosis ; Complement Activation/physiology ; Complement System Proteins ; Endothelial Cells/metabolism ; Endothelium/metabolism ; Female ; Humans ; Male ; Pregnancy
Chemical Substances	Complement System Proteins (9007-36-7)
Language	English
Publishing date	2022-04-12
Publishing country	Switzerland
Document type	Journal Article ; Review ; Research Support, Non-U.S. Gov't
ZDB-ID	2606827-8
ISSN	1664-3224 ; 1664-3224
ISSN (online)	1664-3224
ISSN	1664-3224
DOI	10.3389/fimmu.2022.860689
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Article ; Online: Usefulness and analytical performances of complement multiplex assay for measuring complement biomarkers in plasma.

Meuleman, Marie-Sophie / Duval, Anna / Grunenwald, Anne / Rezola Artero, Mikel / Dermani, Mohamed / Peliconi, Julie / Revel, Margot / Vieira-Martins, Paula / Courbebaisse, Marie / Parfait, Béatrice / Lebeaux, David / Friedlander, Gérard / Roumenina, Lubka / Chauvet, Sophie / Frémeaux-Bacchi, Véronique / Dragon-Durey, Marie-Agnès

Clinica chimica acta; international journal of clinical chemistry

2024 Volume 554, Page(s) 117750

Abstract: Introduction: The complement system is involved in numerous diseases, through diverse mechanisms and degree of activation. With the emergence of complement targeting therapeutic, simple and accessible tools to evaluate the extent of complement ... ...

Abstract	Introduction: The complement system is involved in numerous diseases, through diverse mechanisms and degree of activation. With the emergence of complement targeting therapeutic, simple and accessible tools to evaluate the extent of complement activation are strongly needed. Methods: We evaluated two multiplex panels, measuring complement activation fragments (C4a, C3a, C5a, Bb, Ba, sC5b9) and intact components or regulators (C1q, C2, C3, C4, C5, FD, FP, FH, FI). The specificity of each measurement was assessed by using complement proteins depleted sera and plasma collected from patients with complement deficiencies. Normal values distribution was estimated using 124 plasma samples from healthy donors and complement activation profile was assessed in plasma collected from 31 patients with various complement-mediated disorders. Results: We observed good inter-assay variation. All tested protein deficiencies were accurately detected. We established assay-specific reference values for each analyte. Except for C3, C4 and C4a, the majority of the measurements were in good agreement with references methods or published data. Conclusion: Our study substantiates the utility of the Complement Multiplex assay as a tool for measuring complement activation and deficiencies. Quantifying complement cleavage fragments in patients exhibiting classical or alternative pathway activation allowed evaluating the activation state of the whole cascade.
MeSH term(s)	Humans ; Complement System Proteins ; Complement Activation ; Biomarkers ; Plasma
Chemical Substances	Complement System Proteins (9007-36-7) ; Biomarkers
Language	English
Publishing date	2024-01-02
Publishing country	Netherlands
Document type	Journal Article
ZDB-ID	80228-1
ISSN	1873-3492 ; 0009-8981
ISSN (online)	1873-3492
ISSN	0009-8981
DOI	10.1016/j.cca.2023.117750
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Ud II Zs.173: Show issues

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Article ; Online: Monoclonal gammopathy of unknown significance in kidney transplanted patients: novel insights into long-term outcomes.

Meuleman, Marie-Sophie / Mouyabi, Steven / Gueguen, Juliette / Vicca, Stéphanie / Divard, Gillian / Aubert, Olivier / Bienaimé, Frank / Arnulf, Bertrand / Anglicheau, Dany / Bridoux, Frank / Cohen, Camille

Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association

2023 Volume 39, Issue 1, Page(s) 64–73

Abstract: Background: Because of increased access to kidney transplantation in elderly subjects, the prevalence of monoclonal gammopathies of unknown significance (MGUS) in kidney transplantation (KT) is growing. However, little is known about the consequences of ...

Abstract	Background: Because of increased access to kidney transplantation in elderly subjects, the prevalence of monoclonal gammopathies of unknown significance (MGUS) in kidney transplantation (KT) is growing. However, little is known about the consequences of MGUS on long-term outcomes. Methods: We identified 70 recipients with MGUS present at transplantation (KTMG) and 114 patients with MGUS occurring after KT (DNMG), among 3059 patients who underwent a KT in two French kidney transplantation centers. We compared outcomes of KTMG with those of matched controls. Results: Baseline characteristics were similar except for an older age in KTMG compared with the DNMG group (62 vs 57 years, P = .03). Transient MGUS occurred more frequently in DNMG patients (45% vs 24%, P = .007). When compared with matched controls without MGUS, KTMG patients showed higher frequency and earlier post-transplant solid cancers (15% vs 5%, P = .04) and a trend for more bacterial infections (63% vs 48%, P = .08), without difference regarding patient and graft survival, rejection episodes or hematological complications. KTMG patients with an abnormal kappa/lambda ratio and/or severe hypogammaglobulinemia at the time of KT experienced shorter overall survival. Conclusions: MGUS detection at the time of KT is neither associated with a higher occurrence of graft rejection, nor adversely affects graft or overall survival. MGUS should not contraindicate KT. However, MGUS at the time of KT may be associated with higher risk of early neoplastic and infectious complications and warrants prolonged surveillance. Measurement of serum free light chain should be performed before transplant to refine the risk evaluation of KTMG patients and propose personalized follow-up and immunosuppression.
MeSH term(s)	Humans ; Aged ; Kidney Transplantation/adverse effects ; Monoclonal Gammopathy of Undetermined Significance/complications ; Monoclonal Gammopathy of Undetermined Significance/epidemiology ; Multiple Myeloma/complications ; Immunosuppression Therapy/adverse effects ; Kidney
Language	English
Publishing date	2023-07-04
Publishing country	England
Document type	Journal Article
ZDB-ID	90594-x
ISSN	1460-2385 ; 0931-0509
ISSN (online)	1460-2385
ISSN	0931-0509
DOI	10.1093/ndt/gfad144
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Zs.A 2198: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: C3 Glomerulopathy With Concurrent Thrombotic Microangiopathy: Clinical and Immunological Features.

American journal of kidney diseases : the official journal of the National Kidney Foundation

2023 Volume 82, Issue 3, Page(s) 279–289

Abstract: Rationale & objective: C3 glomerulopathy (C3GN) and atypical hemolytic uremic syndrome (aHUS) are 2 distinct rare kidney diseases caused by dysregulation of the alternative complement pathway. Patients with C3GN and concurrent kidney lesions of ... ...

Abstract	Rationale & objective: C3 glomerulopathy (C3GN) and atypical hemolytic uremic syndrome (aHUS) are 2 distinct rare kidney diseases caused by dysregulation of the alternative complement pathway. Patients with C3GN and concurrent kidney lesions of thrombotic microangiopathy (TMA) have been rarely reported. We characterized the clinical features and underlying immunological abnormalities in these patients. Study design: Case series. Setting & participants: Patients with C3GN and concomitant TMA lesions on biopsy registered from 2009 to 2019 in the French National Registry of C3GN. Findings: Among 278 registered patients with C3GN, 16 (6%) had biopsy-proven glomerular and/or vascular TMA lesions. Their median age at diagnosis was 39 years (range, 7-76), and 59% were female. Fourteen of the 16 patients (88%) had an estimated glomerular filtration rate of<30mL/min/1.73m Limitations: Small retrospective case series with a limited number of biopsies including electron microscopy. Conclusions: Concomitant C3GN and TMA is extremely rare and is associated with poor kidney outcomes. Genetic or acquired abnormalities of the alternative complement pathway are common as is the presence of monoclonal gammopathy, which may inform the selection of treatment approaches.
MeSH term(s)	Humans ; Female ; Child ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Aged ; Male ; Retrospective Studies ; Kidney ; Atypical Hemolytic Uremic Syndrome/drug therapy ; Thrombotic Microangiopathies/therapy ; Thrombotic Microangiopathies/complications ; Paraproteinemias/complications
Language	English
Publishing date	2023-04-14
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	604539-x
ISSN	1523-6838 ; 0272-6386
ISSN (online)	1523-6838
ISSN	0272-6386
DOI	10.1053/j.ajkd.2022.12.020
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Zs.A 1669: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Complement Terminal Pathway Activation and Intrarenal Immune Response in C3 Glomerulopathy.

Meuleman, Marie-Sophie / Petitprez, Florent / Pickering, Matthew C / Le Quintrec, Moglie / Artero, Mikel Rezola / Duval, Anna / Rabant, Marion / Gilmore, Alyssa / Boyer, Olivia / Hogan, Julien / Servais, Aude / Provot, François / Gnemmi, Vivianne / Eloudzeri, Maeva / Grunenwald, Anne / Buob, David / Boffa, Jean-Jacques / Moktefi, Anissa / Audard, Vincent /

Goujon, Jean-Michel / Bridoux F, Frank / Thervet, Eric / Karras, Alexandre / Roumenina, Lubka T / Bacchi, Véronique Frémeaux- / Duong Van Huyen, Jean-Paul / Chauvet, Sophie

Journal of the American Society of Nephrology : JASN

2024

Abstract: Background: C3 glomerulopathy is a rare disease resulting from an overactivation of the complement alternative pathway. Although there is also evidence of terminal pathway activation, its occurrence and consequences on the disease have been poorly ... ...

Abstract	Background: C3 glomerulopathy is a rare disease resulting from an overactivation of the complement alternative pathway. Although there is also evidence of terminal pathway activation, its occurrence and consequences on the disease have been poorly studied. Methods: We retrospectively studied a cohort of 42 patients diagnosed with C3 glomerulopathy. We performed centralized extensive characterization of histological parameters. Kidney C5b-9 staining was performed as a marker of terminal pathway activation, intra-renal immune response was characterised through transcriptomic analysis. Results: Eighty-eight percent of biopsies showed C5b-9 deposits in glomeruli. Biopsies were grouped according to the amount of C5b-9 deposits (no or low n=15/42, 36%, intermediate n=15/42, 36%, and high n=12/42, 28%). Patients with high C5b-9 deposits significantly differed from the 2 other groups patients and were characterized by a significant higher histological chronicity score (p=0.005) and lower outcome-free survival (p=0.001). In multivariable analysis, higher glomerular C5b-9 remained associated with poor kidney prognosis after adjustment. One third of the 847 studied immune genes were upregulated in C3 glomerulopathy biopsies compared to controls. Unsupervised clustering on differentially expressed genes identified a group of kidney biopsies enriched in high glomerular C5b-9 with high immune and fibroblastic signature and showed high chronicity scores on histological examination. Conclusions: In a cohort of patients with C3 glomerulopathy, intra-renal terminal pathway activation was associated with specific histological phenotype and disease prognosis.
Language	English
Publishing date	2024-05-06
Publishing country	United States
Document type	Journal Article
ZDB-ID	1085942-1
ISSN	1533-3450 ; 1046-6673
ISSN (online)	1533-3450
ISSN	1046-6673
DOI	10.1681/ASN.0000000000000373
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Article ; Online: Complement Activation and Thrombotic Microangiopathy Associated With Monoclonal Gammopathy: A National French Case Series.

American journal of kidney diseases : the official journal of the National Kidney Foundation

2022 Volume 80, Issue 3, Page(s) 341–352

Abstract: Rationale & objective: Hemolytic uremic syndrome (HUS), a thrombotic microangiopathy (TMA) with kidney involvement, is a rare condition in patients with monoclonal gammopathy. In the absence of known causes of TMA, the role of complement activation in ... ...

Abstract	Rationale & objective: Hemolytic uremic syndrome (HUS), a thrombotic microangiopathy (TMA) with kidney involvement, is a rare condition in patients with monoclonal gammopathy. In the absence of known causes of TMA, the role of complement activation in endothelial injury in patients with monoclonal gammopathy remains unknown and was the focus of this investigation. Study design: Case series. Setting & participants: We studied the 24 patients in the French national registry of HUS between 2000 and 2020 who had monoclonal gammopathy without other causes of secondary TMA. We provide the clinical histories and complement studies of these patients. Findings: Monoclonal gammopathy-associated TMA with kidney involvement is estimated to be 10 times less frequent than adult atypical HUS (aHUS) in the French national registry. It is characterized by severe clinical features, with 17 of 24 patients requiring dialysis at disease onset, and with median renal survival of only 20 months. TMA-mediated extrarenal manifestations, particularly cutaneous and neurological involvement, were common and associated with poor overall prognosis. Complement studies identified low C3, normal C4, and high soluble C5b-9 levels in 33%, 100%, and 77% of tested patients, respectively, indicating a contribution of the alternative and terminal complement pathways in the pathophysiology of the disease. Genetic abnormalities in complement genes known to be associated with aHUS were found in only 3 of 17 (17%) who were tested. Limitations: Retrospective study without comparison group; limited number of patients, limited available blood samples. Conclusions: Within the spectrum of TMA, TMA associated with monoclonal gammopathy represents a distinct subset. Our findings suggest that HUS associated with monoclonal immunoglobulin is a complement-mediated disease akin to aHUS.
MeSH term(s)	Adult ; Antibodies, Monoclonal, Humanized ; Atypical Hemolytic Uremic Syndrome/epidemiology ; Atypical Hemolytic Uremic Syndrome/genetics ; Complement Activation ; Complement System Proteins ; Humans ; Paraproteinemias/complications ; Paraproteinemias/epidemiology ; Retrospective Studies ; Thrombotic Microangiopathies/epidemiology ; Thrombotic Microangiopathies/etiology
Chemical Substances	Antibodies, Monoclonal, Humanized ; Complement System Proteins (9007-36-7)
Language	English
Publishing date	2022-02-22
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	604539-x
ISSN	1523-6838 ; 0272-6386
ISSN (online)	1523-6838
ISSN	0272-6386
DOI	10.1053/j.ajkd.2021.12.014
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