Overview

Fibrillary glomerulonephritis is a rare and severe disease affecting the kidneys' filtering units called glomeruli. In this disease, a type of unusual protein accumulates in the walls of the glomeruli, resulting in the swelling of the kidneys. It mostly affects people between 41 and 80 years old and results in symptoms like blood and protein in the urine, loss of kidney function, swelling in the legs, high blood pressure, etc. Even though the treatment is majorly symptomatic, in patients with end-stage kidney damage, transplantation of the kidney is a viable option to prolong life. 

Introduction

The kidney helps filter blood in our body. The filtration unit in the kidney is called a nephron, and each nephron has a filter known as glomeruli. Fibrillary glomerulonephritis is caused by the deposition of organised, randomly oriented, nonbranching fibres with a diameter of 20 nm (range 15–25 nm) in the walls of glomeruli. As a result, the filtration process in the kidneys gets affected, resulting in the swelling of the kidneys. 

Fibrillary glomerulonephritis can be diagnosed by kidney biopsies. It is estimated that 0.5% to 1.4%  of kidney biopsies show positive results for fibrillary glomerulonephritis. The exact cause of this disease is still unknown. Some studies suggest a familial association in rare instances. Studies also show that fibrillary glomerulonephritis is associated with cancer, autoimmune disorders, HIV, and hepatitis C viral infections.¹

Symptoms of fibrillary glomerulonephritis

Since it affects the glomeruli, the first symptoms are usually associated with blood elements of the kidney. Then, as the damage progresses, the function of the kidneys is lost, resulting in permanent damage to the kidneys and associated symptoms. The common symptoms are

• Hematuria: Blood in the urine
• Proteinuria: Protein in the urine that results in foamy urine
• High blood pressure
• Anaemia due to the lack of erythropoietin (hormone required to form red blood cells) produced by the kidney
• Kidney failure
• Swelling in the leg
• Nausea
• Fatigue
• Loss of appetite
• Unusual taste in the mouth
• Shortness of breath
• Heart palpitations

Kidney transplantation

Kidney transplantation is a surgery which involves transplanting a healthy kidney from a donor to a patient with kidney damage. It is done to prolong and improve the life of patients with end-stage kidney diseases. Studies show a 95% one-year and a 93% and 90% five-year survival rate for kidney transplant patients. Patients with end-stage kidney diseases, when subjected to kidney transplantation, show better long-term survival than patients on dialysis. They also lead a better quality of life and may survive 10 years more than dialysis patients.  

Kidney transplantation involves two surgeries, the donor and the recipient and can be an open surgery or a minimally invasive one. The minimally invasive procedures include laparoscopic and robotic surgeries. The implantation in the recipient is usually done by an open surgery. Kidney transplantation is an excellent treatment option for patients with complete kidney failure. 

Transplantation is a multidisciplinary approach and involves many processes. The initial consultation is done by a nephrologist and a hepatologist. Then, the patient is referred to a transplant surgeon. The patient is then placed on the transplant list, and once the donor is available, the necessary tests are conducted to identify the matching of the organs. The patient is then admitted for the surgery, which involves a team of healthcare professionals, each specialised in their field.²

Criteria for candidacy of kidney transplantation 

• End-stage kidney disease patients on dialysis
• Patients with advanced chronic kidney diseases with a glomerular filtration rate of less than 30 mL/min/1.73 m (stage IV or V)
• Patients with 20% or less kidney function
• Patients with irreversible kidney failure²  

Benefits and risks of kidney transplantation

The main advantages include a longer and improved quality of life. Patients do not need dialysis after transplantation, and there are fewer restrictions on diet. However, kidney transplant surgery is a complex procedure and may involve complications like bleeding, infection, organ rejection, etc. It is also found that there may be an increased risk of heart problems, diabetes and infections in kidney recipients. The recent advances in immunosuppressive drugs and surgical and monitoring protocols have led to improved outcomes with minimal complications in kidney transplantation, which makes it more promising.³

Kidney transplantation in fibrillary glomerulonephritis patients

Various therapies like corticosteroids, cytotoxic drugs, cyclosporin A and plasmapheresis have been tried in patients with fibrillary glomerulonephritis. In the majority of the cases, at the time of diagnosis, the disease will be at an advanced stage, thus having a poor response to the treatment.⁴  Since it is a rare disease, the data available about the post-transplantation results in fibrillary glomerulonephritis patients is limited. Fibrillary glomerulonephritis can progress to end-stage kidney disease within 2 to 4 years of diagnosis. Kidney transplantation is a viable option for such patients to prolong their lives.

In a study done by the Australia and New Zealand Dialysis and Transplant Registry (ANZDATA),  the survival rate of kidney transplant patients in fibrillary glomerulonephritis is found to be comparable to those with other causes of end-stage kidney disease (67% vs 76%).⁵

In a mean follow-up of 51 months in kidney transplant patients at the Mayo Clinic, 36% showed a recurrence of fibrillar glomerulonephritis. A recent report from Australia and New Zealand using the ANZDATA registry reported that 1 in 13 patients with fibrillary glomerulonephritis who had kidney transplants showed recurrence and organ failure in 4.6 years.⁶ 

This data shows that kidney transplantation is a possible treatment option in fibrillary glomerulonephritis; however, the risk of recurrence is not negligible. Some studies suggest that kidney transplantation should be done in fibrillary glomerulonephritis patients who show a better prognosis in the associated blood disorders. Close monitoring of proteinuria and kidney function is essential to diagnose the recurrence early in such patients.⁷

Management and follow-up care

After a kidney transplant, proper care and maintenance of a healthy lifestyle are mandatory. Keep in mind that kidney transplantation does not cure kidney disease, and long-term success depends on follow-up and care.

• Continue the regular medications and immunosuppressive therapy to prevent organ rejection
• Continue the regular monitoring and checkups with the transplant team
• Follow a healthy lifestyle
• Keep a healthy weight
• Avoid smoking and alcohol
• Include a healthy diet
• Exercise regularly

Managing potential complications

Recurrence in transplanted kidneys

The recurrence rate of fibrillary glomerulonephritis in kidney transplant patients is not negligible. However, studies suggest that the recurrence is rather benign (less harmful), thus making the prolonged survival of the transplant possible.⁸ Follow-up and proper care are necessary after the transplantation, and if any symptoms of recurrence are noted, discuss it with the health care team immediately.

Dealing with rejection 

Since the transplanted kidney is a foreign object, the body might react to it and try to reject it. Hence, it is important to take the anti-rejection medicines, which are the immunosuppressants prescribed by your healthcare professional, regularly. Rejection can be acute—immediately after surgery or chronic—slowly after a longer time. 

Common signs of rejection include:

• Flu-like symptoms
• Body temperature higher than 38°C 
• Decreased urine output
• Sudden gain in weight
• Pain over the kidneys
• Unexplained tiredness

Hence, lifelong follow-up and maintenance are necessary to minimise organ rejection and optimise the outcome.⁹

FAQs

What is the prognosis for fibrillary glomerulonephritis?

Fibrillary glomerulonephritis has a comparatively poor prognosis, with half the patients reaching end-stage kidney disease within a few years of diagnosis.

Is fibrillary glomerulonephritis hereditary?

The exact cause of fibrillary glomerulonephritis is still unknown. But in rare instances, a familial association has been reported.

What are the signs and symptoms of fibrillary glomerulonephritis?

Hematuria, proteinuria, high blood pressure and kidney failure are the common symptoms of fibrillary glomerulonephritis.

Is fibrillary glomerulonephritis an autoimmune disease?

The exact cause is still unknown, but fibrillary glomerulonephritis is associated with autoimmune disorders, HIV, cancer and hepatitis C viral infections.

Summary

Fibrillary glomerulonephritis is a rare disease that affects the kidneys. It occurs as a result of the deposition of a fibrillary protein within the walls of the glomeruli of the kidney. The disease is reported to have a poor prognosis, resulting in end-stage kidney disease within a few years of diagnosis. Kidney transplantation can be done in patients suffering from end-stage kidney disease due to fibrillary glomerulonephritis. Studies suggest that the outcome is promising to prolong and improve the quality of life of the patients. However, the transplantation also carries a risk of recurrence and organ rejection. Hence, proper follow-up and care are required to assess the post-transplant kidney function and minimise the complications.