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Expert consensus identifies 60 to 90 incident C3G cases annually in Italy with 94% agreementExperts Reach Consensus on C3 Glomerulopathy Diagnosis and Treatment

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Key Takeaway
Note that expert consensus identifies significant unmet needs in rapid diagnosis and specific treatment for C3G.

This guideline was developed using a Delphi consensus process involving 17 Italian nephrologists with expertise in C3G. The scope of the consensus included epidemiology, patient characteristics, disease outcomes, and treatment strategies. The study aimed to establish a common clinical framework for managing C3G within the Italian healthcare context.

Key findings from the consensus include an estimated 60 to 90 incident patients per year in Italy, reaching 94% agreement among experts. The consensus estimated approximately 2700 prevalent cases of C3G in Italy with 82% agreement. Regarding clinical outcomes, the experts identified the stabilization of eGFR and improvement in proteinuria as primary goals.

Experts identified the most critical unmet needs as the availability of specific and effective treatments and the necessity for rapid and correct disease diagnosis. The study is intended to improve clinical knowledge and stimulate research on C3G. Because this is a consensus-based Delphi study, the findings reflect expert agreement rather than direct clinical trial data or large-scale observational evidence.

How this fits prior evidence

This consensus addresses a gap in local epidemiology and clinical management for C3G. It builds upon prior coverage of complement-targeted therapies showing clinical activity and a 68% reduction in proteinuria, and a review of complement pathways in kidney diseases. While the previous evidence focused on the efficacy of specific inhibitors, this consensus focuses on the Italian clinical landscape, including an estimated 60 to 90 incident cases annually and 2700 prevalent cases.

A group of 17 Italian nephrologists with expertise in C3 glomerulopathy participated in a Delphi consensus study. This type of study is used to reach a common agreement among experts rather than testing a new drug or treatment on patients. The experts discussed the current state of the disease, its prevalence, and the best ways to manage it.

The experts reached a consensus on the numbers of people living with this condition in Italy. They estimated that about 2,700 people currently have the disease, with 60 to 90 new cases appearing each year. They also identified key goals for patient care, which include stabilizing kidney function and improving the amount of protein in the urine.

The study highlights that there are still significant gaps in care. The experts noted that the most important needs are faster, more accurate diagnoses and the availability of more specific treatments. Because this was a consensus study among experts and not a clinical trial, the results reflect professional opinions rather than new medical evidence for patients.

What this means for you:
Experts agree that faster diagnosis and more specific treatments are the most important needs for C3 glomerulopathy.

Common questions

How many people in Italy have C3 glomerulopathy?

Experts reached a consensus that there are about 2,700 prevalent cases of C3 glomerulopathy in Italy. They also estimated that between 60 and 90 new cases occur each year. These numbers help experts understand the scale of the condition in the region.

What are the main goals for treating this condition?

The primary goals for managing the disease include the stabilization of eGFR (kidney function) and an improvement in proteinuria (protein in the urine). These are the key outcomes that experts identified as important for patient care.

What are the biggest challenges for patients with C3 glomerulopathy?

The experts identified two main unmet needs: the need for a rapid and correct diagnosis and the availability of specific and effective treatments. These areas are currently seen as the most important priorities for improving patient outcomes.

Study Details

Study typeGuideline
EvidenceLevel 5
PublishedOct 2026
View Original Abstract ↓
BackgroundC3 glomerulopathy (C3G) is a chronic kidney disease previously included within the broader spectrum of membranoproliferative glomerulonephritis. Information on various aspects of C3G, including epidemiology, management, and patient needs, is extremely scarce at the international and particularly at a national level.MethodsBetween April 2024 and February 2025, we conducted an online iterative modified Delphi study involving Italian nephrologists with expertise in C3G. The Delphi study was performed according to the best practices for defining consensus and was reported following the ACcurate COnsensus Reporting Document (ACCORD) guidelines. Five main areas of investigation of C3G were explored, i.e., epidemiology, patient characteristics, disease outcomes, treatment, and other mixed aspects.ResultsOf the 23 invited clinicians, 2 (8.7%) did not reply and 4 (17.4%) did not satisfy the inclusion criteria defined by the Steering Committee. Thus, the voting panel included 17 Italian experts. Two iterations were required to complete the Delphi study. All 17 panellists participating to the first iteration completed the second Delphi round. Agreement was achieved on an estimated number of 60–90 incident patient per year (94% agreement) and about 2700 prevalent cases of C3G (82% agreement) in Italy. The stabilization of eGFR and an improvement in proteinuria emerged as key disease and treatment outcomes. All experts agreed that the availability of specific and effective treatments and rapid and correct disease diagnosis are currently the most important unmet needs related to C3G.ConclusionsThis investigation provided relevant information for improving our knowledge of C3G, stimulate discussion and enhance research on this ultra-rare disease in Italy.
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