Illustration representing C3 glomerulopathy treatment in children.

C3 Glomerulopathy in Children: When to Use Anti-Cellular Immunosuppression?

Avery Sinclair in Health & Wellness December 2025 • 4 min read.

"Navigating treatment options for C3GP, a rare kidney disease in children, and the ongoing debate about anti-cellular immunosuppression."

C3 glomerulopathy (C3GP) is a rare and complex kidney disease where the complement system, part of the immune system, abnormally attacks the kidneys. This leads to damage within the glomeruli, the kidney's filtering units. Diagnosis is confirmed through a kidney biopsy with specific staining techniques.

Managing C3GP is challenging, with varied treatment approaches used by doctors. These range from supportive care to powerful immunosuppressant drugs and targeted therapies like eculizumab. The optimal approach remains a subject of debate.

A new study sheds light on the complexities of C3GP treatment in children, examining the use of anti-cellular immunosuppression, medications that dampen the immune system's activity. By reviewing data from 11 children with C3GP, researchers aimed to contribute to the limited evidence base guiding treatment decisions.

Understanding C3 Glomerulopathy (C3GP): What the Study Revealed

Illustration representing C3 glomerulopathy treatment in children.

Researchers at a university medical center retrospectively reviewed 307 kidney biopsies performed on 240 children between January 2004 and July 2018. From this group, 11 children were identified as having C3GP based on specific diagnostic criteria.

The study meticulously analyzed the children's medical records, focusing on how the disease presented, findings from kidney biopsies, treatment approaches used, and patient outcomes. Different forms of microscopy were also conducted to provide a more comprehensive analysis.

Incidence: C3GP was found in 4.6% of the biopsied children.
Presentation: All children had protein in their urine (proteinuria), and most also had blood in their urine (hematuria). Some experienced nephrotic syndrome (a condition causing swelling and protein loss) or kidney insufficiency.
Biopsy Findings: Microscopic examination revealed different patterns of kidney damage, with membranoproliferative glomerulonephritis (MPGN) being the most common.
Complement Abnormalities: All patients had low levels of C3, a key protein in the complement system.

The children in the study received a variety of treatments, reflecting the lack of a single standard approach. These included medications to suppress the immune system (anti-cellular immunosuppression), plasma therapy, and eculizumab, a drug that targets a specific part of the complement system. The outcomes varied, with some children achieving remission (either total or partial), while others showed no response to treatment.

Key Takeaways and Future Directions

The study highlights the complexity of C3GP in children and the need for individualized treatment strategies. While anti-cellular immunosuppression can be effective for some, it's not a universal solution. Further research is needed to identify which patients are most likely to benefit from specific therapies and to develop more targeted approaches for managing this challenging condition. A trial of classical immunosuppression before eculizumab is still worth trying.

About this Article -

This article was crafted using a human-AI hybrid and collaborative approach. AI assisted our team with initial drafting, research insights, identifying key questions, and image generation. Our human editors guided topic selection, defined the angle, structured the content, ensured factual accuracy and relevance, refined the tone, and conducted thorough editing to deliver helpful, high-quality information.See our About page for more information.

This article is based on research published under:

DOI-LINK: 10.1111/nep.13522, Alternate LINK

Title: C3 Glomerulopathy In Children: Is There Still A Place For Anti-Cellular Immunosuppression?

Subject: Nephrology

Journal: Nephrology

Publisher: Wiley

Authors: Nika Kojc, Alenka Bahovec, Tanja Kersnik Levart

Published: 2019-01-29

Everything You Need To Know

What is C3 Glomerulopathy (C3GP), and why is it important?

C3 Glomerulopathy (C3GP) is a rare kidney disease where the complement system, a part of your immune system, mistakenly attacks the kidneys. This attack damages the glomeruli, which are the kidney's filtering units. The diagnosis of C3GP is confirmed by a kidney biopsy and specific staining techniques. The significance lies in its potential to lead to kidney damage and the need for effective treatment strategies to manage the disease and preserve kidney function.

What is the role of the complement system in C3GP?

The complement system plays a critical role in the body's immune response. In the context of C3GP, the complement system malfunctions, leading to the abnormal activation and attack on the kidneys. In the study, all patients exhibited low levels of C3, a key protein in the complement system. This highlights the central role of the complement system in the pathogenesis of C3GP, and targeting the complement system, such as with the drug eculizumab, is a key strategy for intervention.

What is anti-cellular immunosuppression, and why is it used for C3GP?

Anti-cellular immunosuppression involves using medications to suppress the activity of the immune system. In the study, it was one of the treatment approaches used for children with C3GP. While some patients responded well to this approach, it was not effective for everyone. The implications are that doctors must carefully consider the potential benefits and risks of anti-cellular immunosuppression. Individualized treatment plans are needed. Furthermore, it suggests that anti-cellular immunosuppression may be worth trying before exploring other options like eculizumab.

What did the study reveal about the treatment of C3GP in children?

The study analyzed data from 11 children diagnosed with C3GP. The study examined the presentation of the disease, findings from kidney biopsies, treatment approaches used, and patient outcomes. The study's findings indicate that C3GP can present with a range of symptoms. The study also showed that outcomes varied among patients. This highlights the need for personalized treatment strategies and further research to improve the management of C3GP in children.

What are the key takeaways from the study regarding C3GP?

The study revealed several key findings. C3GP was found in 4.6% of biopsied children. Common presentations included proteinuria and hematuria. Kidney biopsies showed different damage patterns. All patients had low C3 levels. These findings emphasize the diagnostic criteria, the disease's impact on kidney function, and the variability in its presentation. This data is very significant because it can guide physicians in identifying, diagnosing, and managing C3GP in children. The lack of a standard treatment approach is also a very important finding. A trial of classical immunosuppression before eculizumab is still worth trying.