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Collapsing Glomerulopathy - Causes, Symptoms, and Treatment

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Collapsing glomerulopathy is characterized by the segmental collapse of the glomerular capillaries present in the kidney. Read this article to know more.

Written by

Dr. Sameeha M S

Medically reviewed by

Dr. Manzoor Ahmad Parry

Published At December 16, 2022
Reviewed AtApril 26, 2023

Introduction

Kidneys play a vital role in maintaining overall health. They are responsible for the filtration and removal of waste products from the body, thus maintaining an acid-base balance, electrolyte concentration, blood pressure, hormone secretion, and hemostasis. Unfortunately, as age increases, the efficiency of kidneys decreases gradually due to developing kidney diseases and other pathologies. Nearly 850 million people worldwide are diagnosed with kidney diseases, with a rapid rise in the number of new cases yearly.

Each kidney contains millions of nephrons that serve as the structural and functional unit of the kidneys by filtering wastes, excess fluids, and toxins from the blood. These nephrons contain a network of blood vessels enclosed in a sac known as the glomerulus. Glomerulopathy affects the glomeruli of the nephrons and ends up in kidney malfunction. The affected individuals may present with conditions like lower concentration of protein in the blood, presence of blood in the urine (hematuria), reduced amount of urine, high protein concentration in urine (proteinuria), and generalized swelling in the body (edema).

What Is Glomerulopathy?

Glomerulopathy is a general term to describe a group of conditions that affect the renal glomerulus and alter renal function. It may occur due to inflammatory or non -inflammatory causes. The renal impairment (injury to endothelium and glomerular filtration barrier) that occurs due to glomerulopathies leads to proteinuria, edema, hyperlipidemia, and hematuria. Glomerulonephritis results in damage and dysfunction of glomeruli due to inflammatory changes in the kidney cells. It may occur due to sudden (acute) or gradual (chronic) inflammatory changes in the glomeruli.

What Is Collapsing Glomerulopathy?

Collapsing glomerulopathy (CG) is a pathological condition characterized by hyperplasia (abnormal increase in the number of cells)and hypotrophy (abnormal increase in the size of cells) of specialized epithelial cells (podocytes) present in the glomerular capillaries along with segmental damage or global collapse of the glomerulus. Collapsing glomerulopathy (CG) is a histological variant of focal segmental glomerulosclerosis (FSGS). It is associated with rapid destruction in the functional cells of the kidney and impaired renal functions. Clinical features of collapsing glomerulopathy include generalized swelling (edema), renal insufficiency, proteinuria, and rapid development of end-stage renal disease (ESRD).

What Are the Causes of Collapsing Glomerulopathy?

  • Gene Mutation - The most significant risk factor associated with the incidence of collapsing glomerulopathy is gene mutation. APOL1 gene located on chromosome 22 (long arm) is responsible for the pathogenesis of collateral glomerulopathy. Usually, the APOL1 gene codes for signals responsible for the formation of immune reactions against infections and the formation of high-density lipoproteins. Genetic mutations in the APOL1 gene result in several cell damage, lysosomal damage, mitochondrial damage, and the formation of cell pores. Other gene mutations involving the COQ2 gene (code for mitochondrial proteins) and MHY9 (code for filaments that maintain podocyte structure) are also responsible for renal diseases, including collateral glomerulopathy.

  • Viral Infections - Infectious diseases associated with hepatitis -B virus, human immunodeficiency virus (HIV), cytomegalovirus, hepatitis - C virus, T-cell lymphotropic virus, parvovirus, Epstein -Barr virus, and Zika virus have shown renal cell damage leading to collateral glomerulopathy.

  • Autoimmune Diseases - Collapsing glomerulopathy (CG) may also occur due to the pathological changes associated with autoimmune diseases like adult Still's disease, systemic lupus erythematosus (SLE), mixed connective tissue disease, temporal arteritis, and Behcet's disease.

  • Drug-induced - Collateral glomerulopathy may occur due to renal toxicity and cell damage induced by the use of drugs like anabolic steroids, bisphosphonates, valproic acid, opioids, and anthracyclines.

  • Neoplastic Conditions - Abnormal neoplastic conditions like acquired hemophagocytic syndrome, multiple myeloma, natural killer cell leukemia, and acute monoblastic leukemia are considered etiological factors for inducing renal impairment and collapsing glomerulopathy.

  • Ischemic Diseases - Collapsing glomerulopathy may occur due to ischemic conditions such as thromboembolism, atheroembolic disease, thrombotic microangiopathy, and sickle cell anemia.

  • Other Renal Conditions - Collapsing glomerulopathy may occur in association with other renal conditions like membranous glomerulopathy, immunoglobulin A nephropathy, and other histological variations of focal segmental glomerulosclerosis (FSGS).

What Are the Symptoms of Collapsing Glomerulopathy?

The most significant and common clinical feature of collateral glomerulopathy is the presence of high concentrations of protein in the urine (proteinuria). Other features like hypertension, blood in the urine (hematuria), and high lipid content in the blood (lipiduria) may also occur in the affected individuals. Most patients develop renal failure immediately after collapsing glomerulopathy, rapidly worsening the condition and progressing to end-stage renal disease (ESRD).

How Can We Treat Collapsing Glomerulopathy?

  • Targeted Therapy - It consists of targeted management of conditions like edema, hypertension, and dyslipidemia due to glomerulopathy. Edema can be managed through fluid restriction, a low-sodium diet, diuretic therapy, and hemodialysis. High blood pressure can be controlled using medications that act as angiotensin II receptor blockers. Dyslipidemia associated with collateral glomerulopathy can be managed using lipid-lowering agents. Lifestyle changes, including weight loss, diet modification, smoking cessation, and increased physical activity, must be encouraged in patients to improve the treatment outcome.

  • Treatment of Underlying Disease - When collateral glomerulopathy occurs due to underlying etiologies such as viral infections, autoimmune diseases, genetic mutations, and drug-induced causes, the patients must be given specific treatment to manage the underlying diseases. In patients with drug-induced glomerulopathy, the offending drug is discontinued as early as possible and replaced with other medications. Viral infections can be managed with specific antimicrobials to reduce the impact and further complications. Collateral neuropathy associated with human immunodeficiency virus (HIV) is treated using specific antiretroviral therapy (ART).

  • Immunosuppressive Therapy - Several immunosuppressant drugs, including oral corticosteroids, Cyclophosphamide, Mycophenolate mofetil, and Rituximab, are given to patients with collateral glomerulopathy to improve the condition.

Conclusion

Collateral glomerulopathy is a pathological condition that may occur due to multiple factors, including genetic mutation, viral infections, autoimmune diseases, neoplastic conditions, ischemic diseases, and other renal disorders. Affected individuals usually present with severe renal impairment leading to altered renal functions and side effects. In most cases, renal impairment rapidly progresses into end-stage renal disease (ESRD). Therefore, timely identification and diagnosis of collateral neuropathy are essential in preventing the patient from stepping into advanced stages and renal failure.

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Dr. Manzoor Ahmad Parry
Dr. Manzoor Ahmad Parry

Nephrology

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