Primary Immune Complex-Membranoproliferative Glomerulonephritis

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Synonyms

IC-MPGN, Membranoproliferative Glomerulonephritis (MPGN) with immune complex deposition, Mesangiocapillary glomerulonephritis, Primary MPGN, Immune Complex-Membranoproliferative Glomerulonephritis,

Overview

IC-MPGN, also known as MPGN, is a type of glomerular disease where the glomeruli, responsible for filtering waste and excess fluid from the blood, become inflamed and damaged. This damage is caused by immune complexes, which are essentially clumps of antibodies and antigens, that get stuck in the glomeruli. The activation of the complement system, a part of the immune system, by these immune complexes leads to further inflammation and damage.

Symptoms

Symptoms: Early symptoms of IC-MPGN can include:
  • Hematuria: Blood in the urine, which may appear pink or brown. 
  • Proteinuria: Excess protein in the urine, which can cause foamy urine. 
  • Edema: Swelling, particularly in the ankles, feet, or face. 
  • High Blood Pressure: Elevated blood pressure due to kidney dysfunction

Causes

Causes: While the exact cause of IC-MPGN is unknown in some cases (idiopathic), it is often associated with other conditions like:
  • Autoimmune Diseases: Conditions where the body’s immune system attacks its own tissues, such as lupus or rheumatoid arthritis. 
  • Infections: Chronic infections like hepatitis C can trigger IC-MPGN. 
  • Monoclonal Gammopathies: Certan blood disorders

Diagnosis

The diagnosis of IC-MPGN requires a comprehensive approach utilizing
⤷ KIDNEY BIOPSY,
⤷ COMPLEMENT WORKUP,
⤷ GENETIC TESTING, AND
⤷ EVALUATION OF NONCOMPLEMENT ETIOLOGIES,
particularly in the context of IC-MPGN.

Prognosis

The prognosis of IC-MPGN is variable and depends on factors such as the type of genetic mutation and the presence of autoantibodies, the degree of kidney damage, and how well the disease responds to treatment. Early diagnosis, careful monitoring, and appropriate treatment may help slow disease progression and improve long-term outcomes.

Treatment

Treatment for IC-MPGN depends on the underlying cause and may include:
  • Treating Underlying Conditions:

    If the disease is secondary to another condition, addressing that condition is crucial. 

  • Immunosuppressive Therapy:

    Medications like corticosteroids or other immunosuppressants can help reduce the immune response and inflammation. 

  • Complement Inhibitors:
    Newer treatments target the complement system directly, aiming to reduce its overactivity and prevent further kidney damage, according to the European Rare Kidney Disease Reference Network