Membranoproliferative glomerulonephritis (type II)

Membranoproliferative Glomerulonephritis Type II (MPGN II), also known as Dense Deposit Disease (DDD), is a rare form of glomerulonephritis, a disease affecting the glomeruli within the kidney. MPGN II is characterized by the abnormal accumulation of dense deposits within the glomerular basement membrane, leading to a thickening of the membrane. This condition can result in significant kidney damage and is associated with the complement system, a part of the immune system that helps the body fight infections.

Etiology and Pathogenesis

The exact cause of MPGN II is not fully understood, but it is closely related to dysregulation of the complement system, specifically the alternative complement pathway. Genetic factors may play a role, with mutations in complement regulatory proteins being implicated. Environmental factors have not been clearly identified.

Clinical Presentation

Patients with MPGN II often present with symptoms indicative of kidney dysfunction, including proteinuria (excessive protein in the urine), hematuria (blood in the urine), hypertension (high blood pressure), and reduced kidney function. In some cases, patients may progress to end-stage renal disease (ESRD), requiring dialysis or kidney transplantation.

Diagnosis

Diagnosis of MPGN II involves a combination of clinical evaluation, laboratory tests, and kidney biopsy. Laboratory tests may show low complement levels, particularly C3. A kidney biopsy is essential for diagnosis, with light microscopy revealing thickening of the glomerular basement membrane and electron microscopy showing dense deposits within the membrane.

Treatment

Treatment of MPGN II is challenging and focuses on controlling symptoms and slowing the progression of kidney damage. Therapeutic options may include immunosuppressive medications, plasmapheresis, and eculizumab, a monoclonal antibody that inhibits the complement system. Management of hypertension and proteinuria with medications such as ACE inhibitors or angiotensin II receptor blockers is also important.

Prognosis

The prognosis of MPGN II varies, with some patients experiencing stable kidney function for years, while others may progress to ESRD. Early diagnosis and treatment are crucial in improving outcomes.

Epidemiology

MPGN II is a rare disease, with a higher prevalence in children and young adults. The incidence and prevalence rates vary globally, and due to its rarity, large-scale epidemiological studies are limited.

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