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Article ; Online: The case of complement inhibitors.

Noris, Marina

2021 Volume 81, Page(s) 100822

Abstract: Severe COVID-19 is characterized by lung and multiorgan inflammation and coagulation in the presence of overactivation of the complement system. Complement is a double edged-sward in SARS-Cov-2 infection. On one hand, it can control the viral infection ... ...

Abstract	Severe COVID-19 is characterized by lung and multiorgan inflammation and coagulation in the presence of overactivation of the complement system. Complement is a double edged-sward in SARS-Cov-2 infection. On one hand, it can control the viral infection in milder cases, on the other hand in cases with severe and prolonged infection massive complement activation occurs, which can intensify lung and systemic inflammation and promote a procoagulant and prothrombotic state. Several uncontrolled studies and controlled clinical trials with different complement inhibitors have been performed and others are ongoing. Results are promising in some but negative in others. Further studies are required to elucidate the benefit to risk profile of complement inhibitors in COVID-19 patients at different stages of the disease and to clarify the best targets in the complement cascade.
MeSH term(s)	COVID-19/blood ; Complement Activation/drug effects ; Complement Inactivating Agents/therapeutic use ; Complement System Proteins/metabolism ; Humans ; Lung/metabolism ; Lung/pathology ; SARS-CoV-2/metabolism ; Severity of Illness Index ; COVID-19 Drug Treatment
Chemical Substances	Complement Inactivating Agents ; Complement System Proteins (9007-36-7)
Language	English
Publishing date	2021-08-19
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	2667413-0
ISSN	2212-4934 ; 2212-4926
ISSN (online)	2212-4934
ISSN	2212-4926
DOI	10.1016/j.jbior.2021.100822
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Article ; Online: Shiga Toxin-Producing Escherichia coli and the Hemolytic-Uremic Syndrome.

Noris, Marina / Remuzzi, Giuseppe

The New England journal of medicine

2023 Volume 389, Issue 26, Page(s) 2499

MeSH term(s)	Humans ; Shiga-Toxigenic Escherichia coli ; Hemolytic-Uremic Syndrome
Language	English
Publishing date	2023-12-29
Publishing country	United States
Document type	Letter ; Comment
ZDB-ID	207154-x
ISSN	1533-4406 ; 0028-4793
ISSN (online)	1533-4406
ISSN	0028-4793
DOI	10.1056/NEJMc2312844
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Article ; Online: C3G and Ig-MPGN-treatment standard.

Noris, Marina / Remuzzi, Giuseppe

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

2023 Volume 39, Issue 2, Page(s) 202–214

Abstract: Among the broad spectrum of membranoproliferative glomerulonephritis (MPGN), immunofluorescence distinguishes C3 glomerulopathy (C3G), with predominant C3 deposits, and immunoglobulin-associated MPGN (Ig-MPGN), with combined C3 and Ig. However, there are ...

Abstract	Among the broad spectrum of membranoproliferative glomerulonephritis (MPGN), immunofluorescence distinguishes C3 glomerulopathy (C3G), with predominant C3 deposits, and immunoglobulin-associated MPGN (Ig-MPGN), with combined C3 and Ig. However, there are several intersections between C3G and Ig-MPGN. Primary C3G and Ig-MPGN share the same prevalence of low serum C3 levels and of abnormalities of the alternative pathway of complement, and patients who present a bioptic pattern of Ig-MPGN at onset may show a C3G pattern in a subsequent biopsy. There is no specific therapy for primary C3G and Ig-MPGN and prognosis is unfavourable. The only recommended indications are inhibitors of the renin-angiotensin system, lipid-lowering agents and other renoprotective agents. The other drugs used currently, such as corticosteroids and mycophenolate mofetil, are often ineffective. The anti-C5 monoclonal antibody eculizumab has been tested in several patients, with mixed results. One reason for the uncertainty is the extremely variable clinical course, most likely reflecting a heterogeneous pathogenesis. An unsupervised clustering analysis that included histologic, biochemical, genetic and clinical data available at onset in patients with primary C3G and Ig-MPGN identified four clusters characterized by specific pathogenic mechanisms. This approach may facilitate accurate diagnosis and development of targeted therapies. Several trials are ongoing with drugs targeting different molecules of the complement cascade, however it is important to consider which component of the cascade may be the most appropriate for each patient. We review the current standards of treatment and discuss novel developments in the pathophysiology, diagnosis, outcome prediction and management of C3G and Ig-MPGN.
MeSH term(s)	Humans ; Glomerulonephritis, Membranoproliferative ; Complement C3 ; Immunoglobulins ; Complement Activation ; Fluorescent Antibody Technique
Chemical Substances	Complement C3 ; Immunoglobulins
Language	English
Publishing date	2023-08-21
Publishing country	England
Document type	Review ; Journal Article
ZDB-ID	90594-x
ISSN	1460-2385 ; 0931-0509
ISSN (online)	1460-2385
ISSN	0931-0509
DOI	10.1093/ndt/gfad182
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Article ; Online: The complement alternative pathway and hemostasis

Noris, Marina / Galbusera, Miriam

Immunological Reviews 2023 Jan., v. 313, no. 1, p. 139-161

2023 , Page(s) 139–161

Abstract: The complement and hemostatic systems are complex systems, and both involve enzymatic cascades, regulators, and cell components—platelets, endothelial cells, and immune cells. The two systems are ancestrally related and are defense mechanisms that limit ... ...

Abstract	The complement and hemostatic systems are complex systems, and both involve enzymatic cascades, regulators, and cell components—platelets, endothelial cells, and immune cells. The two systems are ancestrally related and are defense mechanisms that limit infection by pathogens and halt bleeding at the site of vascular injury. Recent research has uncovered multiple functional interactions between complement and hemostasis. On one side, there are proteins considered as complement factors that activate hemostasis, and on the other side, there are coagulation proteins that modulate complement. In addition, complement and coagulation and their regulatory proteins strongly interact each other to modulate endothelial, platelet and leukocyte function and phenotype, creating a potentially devastating amplifying system that must be closely regulated to avoid unwanted damage and\or disseminated thrombosis. In view of its ability to amplify all complement activity through the C3b‐dependent amplification loop, the alternative pathway of complement may play a crucial role in this context. In this review, we will focus on available and emerging evidence on the role of the alternative pathway of complement in regulating hemostasis and vice‐versa, and on how dysregulation of either system can lead to severe thromboinflammatory events.
Keywords	coagulation ; complement ; hemostasis ; leukocytes ; phenotype ; thrombosis
Language	English
Dates of publication	2023-01
Size	p. 139-161.
Publishing place	John Wiley & Sons, Inc
Document type	Article ; Online
Note	REVIEW
ZDB-ID	391796-4
ISSN	1600-065X ; 0105-2896
ISSN (online)	1600-065X
ISSN	0105-2896
DOI	10.1111/imr.13150
Database	NAL-Catalogue (AGRICOLA)

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Article ; Online: The complement alternative pathway and hemostasis.

Noris, Marina / Galbusera, Miriam

Immunological reviews

2022 Volume 313, Issue 1, Page(s) 139–161

Abstract: The complement and hemostatic systems are complex systems, and both involve enzymatic cascades, regulators, and cell components-platelets, endothelial cells, and immune cells. The two systems are ancestrally related and are defense mechanisms that limit ... ...

Abstract	The complement and hemostatic systems are complex systems, and both involve enzymatic cascades, regulators, and cell components-platelets, endothelial cells, and immune cells. The two systems are ancestrally related and are defense mechanisms that limit infection by pathogens and halt bleeding at the site of vascular injury. Recent research has uncovered multiple functional interactions between complement and hemostasis. On one side, there are proteins considered as complement factors that activate hemostasis, and on the other side, there are coagulation proteins that modulate complement. In addition, complement and coagulation and their regulatory proteins strongly interact each other to modulate endothelial, platelet and leukocyte function and phenotype, creating a potentially devastating amplifying system that must be closely regulated to avoid unwanted damage and\or disseminated thrombosis. In view of its ability to amplify all complement activity through the C3b-dependent amplification loop, the alternative pathway of complement may play a crucial role in this context. In this review, we will focus on available and emerging evidence on the role of the alternative pathway of complement in regulating hemostasis and vice-versa, and on how dysregulation of either system can lead to severe thromboinflammatory events.
MeSH term(s)	Humans ; Endothelial Cells ; Hemostasis ; Blood Coagulation ; Blood Platelets/metabolism ; Thrombosis ; Complement System Proteins/metabolism
Chemical Substances	Complement System Proteins (9007-36-7)
Language	English
Publishing date	2022-10-22
Publishing country	England
Document type	Journal Article ; Review ; Research Support, Non-U.S. Gov't
ZDB-ID	391796-4
ISSN	1600-065X ; 0105-2896
ISSN (online)	1600-065X
ISSN	0105-2896
DOI	10.1111/imr.13150
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Article ; Online: Challenges in Understanding Acute Postinfectious Glomerulonephritis: Are Anti-Factor B Autoantibodies the Answer?

Noris, Marina / Remuzzi, Giuseppe

Journal of the American Society of Nephrology : JASN

2020 Volume 31, Issue 4, Page(s) 670–672

MeSH term(s)	Autoantibodies ; Child ; Complement C3 ; Glomerulonephritis/etiology ; Humans
Chemical Substances	Autoantibodies ; Complement C3
Language	English
Publishing date	2020-03-06
Publishing country	United States
Document type	Editorial ; Research Support, Non-U.S. Gov't ; Comment
ZDB-ID	1085942-1
ISSN	1533-3450 ; 1046-6673
ISSN (online)	1533-3450
ISSN	1046-6673
DOI	10.1681/ASN.2020020168
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Article ; Online: HUS and TTP: traversing the disease and the age spectrum.

Donadelli, Roberta / Sinha, Aditi / Bagga, Arvind / Noris, Marina / Remuzzi, Giuseppe

Seminars in nephrology

2023 Volume 43, Issue 4, Page(s) 151436

Abstract: Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenia purpura (TTP) are rare diseases sharing a common pathological feature, thrombotic microangiopathy (TMA). TMA is characterized by microvascular thrombosis with consequent thrombocytopenia, ... ...

Abstract	Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenia purpura (TTP) are rare diseases sharing a common pathological feature, thrombotic microangiopathy (TMA). TMA is characterized by microvascular thrombosis with consequent thrombocytopenia, microangiopathic hemolytic anemia and/or multiorgan dysfunction. In the past, the distinction between HUS and TTP was predominantly based on clinical grounds. However, clinical presentation of the two syndromes often overlaps and, the differential diagnosis is broad. Identification of underlying pathogenic mechanisms has enabled the classification of these syndromes on a molecular basis: typical HUS caused by Shiga toxin-producing Escherichia coli (STEC-HUS); atypical HUS or complement-mediated TMA (aHUS/CM-TMA) associated with genetic or acquired defects leading to dysregulation of the alternative pathway (AP) of complement; and TTP that results from a severe deficiency of the von Willebrand Factor (VWF)-cleaving protease, ADAMTS13. The etiology of TMA differs between pediatric and adult patients. Childhood TMA is chiefly caused by STEC-HUS, followed by CM-TMA and pneumococcal HUS (Sp-HUS). Rare conditions such as congenital TTP (cTTP), vitamin B12 metabolism defects, and coagulation disorders (diacylglycerol epsilon mutation) present as TMA chiefly in children under 2 years of age. In contrast secondary causes and acquired ADAMT13 deficiency are more common in adults. In adults, compared to children, diagnostic delays are more frequent due to the wide range of differential diagnoses. In this review we focus on the three major forms of TMA, STEC-HUS, aHUS and TTP, outlining the clinical presentation, diagnosis and management of the affected patients, to help highlight the salient features and the differences between adult and pediatric patients which are relevant for management.
MeSH term(s)	Adult ; Humans ; Child ; Infant ; Purpura, Thrombotic Thrombocytopenic/diagnosis ; Purpura, Thrombotic Thrombocytopenic/therapy ; Thrombotic Microangiopathies/diagnosis ; Thrombotic Microangiopathies/etiology ; Atypical Hemolytic Uremic Syndrome/diagnosis ; Diagnosis, Differential ; Mutation
Language	English
Publishing date	2023-11-10
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	604652-6
ISSN	1558-4488 ; 0270-9295
ISSN (online)	1558-4488
ISSN	0270-9295
DOI	10.1016/j.semnephrol.2023.151436
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Article ; Online: Atypical hemolytic uremic syndrome associated with a factor B genetic variant and fluid-phase complement activation: an exception to the rule?

Noris, Marina / Remuzzi, Giuseppe

Kidney international

2020 Volume 98, Issue 5, Page(s) 1084–1087

Abstract: Gain-of-function variants in CFB encoding factor B (FB), a component of the alternative pathway C3 convertase, have been reported in a minority of patients with aHUS and result in massive C3 activation. Zhang et al. describe the functional ... ...

Abstract	Gain-of-function variants in CFB encoding factor B (FB), a component of the alternative pathway C3 convertase, have been reported in a minority of patients with aHUS and result in massive C3 activation. Zhang et al. describe the functional characterization of a novel FB variant (p.Ser367Arg) that they identified in 2 unrelated aHUS pedigrees who had undetectable C3 levels. The mutant FB caused strong C3 cleavage in fluid-phase but also C3 deposition on cell surface. This commentary addresses the implications of these findings for understanding the complexity of complement-related genetic renal diseases.
MeSH term(s)	Atypical Hemolytic Uremic Syndrome/genetics ; Complement Activation/genetics ; Complement Factor B/genetics ; Complement Pathway, Alternative/genetics ; Humans ; Mutation
Chemical Substances	Complement Factor B (EC 3.4.21.47)
Language	English
Publishing date	2020-10-30
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Comment
ZDB-ID	120573-0
ISSN	1523-1755 ; 0085-2538
ISSN (online)	1523-1755
ISSN	0085-2538
DOI	10.1016/j.kint.2020.06.026
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Article ; Online: Terminal complement effectors in atypical hemolytic uremic syndrome: C5a, C5b-9, or a bit of both?

Noris, Marina / Remuzzi, Giuseppe

Kidney international

2019 Volume 96, Issue 1, Page(s) 13–15

Abstract: The role of the terminal complement pathway as the cause of atypical hemolytic uremic syndrome (aHUS) is widely recognized, but the relative contribution of the effectors C5a/C5aR1 and C5b-9 to disease pathogenesis has not been defined. Using ... ...

Abstract	The role of the terminal complement pathway as the cause of atypical hemolytic uremic syndrome (aHUS) is widely recognized, but the relative contribution of the effectors C5a/C5aR1 and C5b-9 to disease pathogenesis has not been defined. Using FH
MeSH term(s)	Animals ; Atypical Hemolytic Uremic Syndrome ; Complement Factor H/genetics ; Complement Membrane Attack Complex ; Mice ; Mutation ; Thrombosis
Chemical Substances	Complement Membrane Attack Complex ; Complement Factor H (80295-65-4)
Language	English
Publishing date	2019-06-19
Publishing country	United States
Document type	Journal Article ; Comment
ZDB-ID	120573-0
ISSN	1523-1755 ; 0085-2538
ISSN (online)	1523-1755
ISSN	0085-2538
DOI	10.1016/j.kint.2019.02.038
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Article ; Online: Membranoproliferative glomerulonephritis: no longer the same disease and may need very different treatment.

Noris, Marina / Daina, Erica / Remuzzi, Giuseppe

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

2021 Volume 38, Issue 2, Page(s) 283–290

Abstract: Membranoproliferative glomerulonephritis (MPGN) is a pattern of glomerular injury that may be primary or secondary to infections, autoimmune diseases and haematological disorders. Primary C3G and IC-MPGN are rare and the prognosis is unfavourable. Based ... ...

Abstract	Membranoproliferative glomerulonephritis (MPGN) is a pattern of glomerular injury that may be primary or secondary to infections, autoimmune diseases and haematological disorders. Primary C3G and IC-MPGN are rare and the prognosis is unfavourable. Based on immunofluorescence findings, MPGN has been classified into complement-mediated C3 glomerulopathy (C3G) and immune complex-mediated MPGN (IC-MPGN). However, this classification leaves a number of issues unresolved. The finding of genetic and acquired complement abnormalities in both C3G and IC-MPGN indicates that they represent a heterogeneous spectrum rather than distinct diseases. An unsupervised hierarchical clustering in a cohort of patients with primary C3G and IC-MPGN identified four distinct pathogenetic patterns, characterized by specific histologic and clinical features, and genetic and acquired complement abnormalities. These results provide the groundwork for a more accurate diagnosis and the development of targeted therapies. The drugs that are currently used, such as corticosteroids and immunosuppressants, are frequently ineffective in primary C3G and IC-MPGN. Eculizumab, an anti-C5 monoclonal antibody, has been used occasionally in single cases or small series. However, only a few patients have achieved remission. This heterogeneous response could be related to the extent of terminal complement activation, which may vary substantially from patient to patient. Several drugs that target the complement system at different levels are under investigation for C3G and IC-MPGN. However, clinical trials to test new therapeutics will be challenging and heavily influenced by the heterogeneity of these diseases. This creates the need to characterize each patient to match the specific complement abnormality with the type of intervention.
MeSH term(s)	Humans ; Glomerulonephritis, Membranoproliferative/diagnosis ; Glomerulonephritis, Membranoproliferative/etiology ; Glomerulonephritis, Membranoproliferative/therapy ; Kidney Glomerulus/pathology ; Complement Activation ; Kidney Diseases ; Glomerulonephritis
Language	English
Publishing date	2021-09-28
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	90594-x
ISSN	1460-2385 ; 0931-0509
ISSN (online)	1460-2385
ISSN	0931-0509
DOI	10.1093/ndt/gfab281
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