Rare Disease Show Episode 21: Understanding C3 Glomerulopathy with Dr Anil Kumar B T

Medical Dialogues presents a rare disease series, delving into the realm of uncommon medical conditions. Rare diseases are medical conditions that affect only a small percentage of the population.

Throughout this series, we'll discuss these rare diseases in various episodes, with medical experts providing clear explanations and insights.

Rare Disease Show Episode 21: In today's show we will cover C3 Glomerulopathy: Dense Deposit Disease and C3 Glomerulonephritis.

C3 Glomerulopathy is a rare group of kidney disorders caused by abnormal activation of the complement system, a part of the immune system. It includes two main types: Dense Deposit Disease (DDD) and C3 Glomerulonephritis (C3GN).

Both conditions lead to the deposition of complement protein C3 in the glomeruli, the kidney's filtering units, causing inflammation and damage. Symptoms may include blood in urine (haematuria), protein in urine (proteinuria), swelling, and high blood pressure.

Over time, they can progress to chronic kidney disease or kidney failure. Diagnosis involves kidney biopsy, laboratory tests, and imaging studies. Treatment focuses on managing symptoms, slowing disease progression, and addressing underlying immune dysfunction, often using medications like corticosteroids or complement inhibitors.

In this episode, Dr Anil Kumar B T, HOD and Sr Consultant - Nephrologist and Transplant Physician at Gleneagles BGS Hospital, Kengeri, Bengaluru shares his insights by addressing questions asked by the Medical Dialogues team-

1. Could you explain what C3 Glomerulopathy is and how it differs from other kidney diseases, most importantly why does this classify as a rare disease?

2. What is the underlying pathophysiology of Dense Deposit Disease (DDD) and C3 Glomerulonephritis (C3GN)? How do these diseases impact the kidney’s ability to function?

3. What are the common symptoms and signs that might indicate a person has DDD or C3GN?

4. How is C3 Glomerulopathy diagnosed? What tests and biomarkers are most helpful in diagnosing these conditions?

5. What are the typical progression patterns of DDD and C3GN? How quickly do they typically lead to kidney failure?

6. How does the presence of C3 Glomerulopathy influence the prognosis after kidney transplantation?

7. How are C3 Glomerulopathy patients typically managed before they need a transplant?

8. What role do complement inhibitors (like eculizumab) play in the treatment of DDD and C3GN?

9. What are the challenges in performing kidney transplants in patients with C3 Glomerulopathy?

10. Are there any specific protocols you follow to minimize the risk of recurrence in kidney transplant recipients with these conditions?

11. What are the long-term monitoring and follow-up requirements for kidney transplant patients with a history of C3 Glomerulopathy?

12. Are there any promising clinical trials or new therapies that could potentially improve outcomes for patients with these conditions?