Rapidly progressive glomerulonephritis history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Despite the wide variety of diseases that cause RPGN, all types of RPGN are characterized by glomeruluar injury and the formation of crescents. Severe injury and GBM rupture leads to the leakage of plasma proteins through the GBM. Of these proteins, fibrin is thought to contribute most strongly to crescent formation. Epithelial cells lining the Bowman capsule respond to the leaked fibrin and proliferate. Infiltrating white blood cells such as monocytes and macrophages may also proliferate. These proliferating cells surround and compress the glomerulus, forming a crescent-shaped scar that is readily visible on light microscopy of a renal biopsy.

History and Symptoms

Most types of RPGN are characterized by severe and rapid loss of kidney function featuring:

• Severe hematuria (blood in the urine)
• Red blood cell casts in the urine
• proteinuria (protein in the urine)

Some patients also experience:

• Hypertension (high blood pressure)
• Edema (fluid retention)

Severe disease is characterized by pronounced

• Oliguria
• Anuria

When the cause of RPGN is Goodpasture syndrome or vasculitis that involves the lungs (such as Wegener granulomatosis), the lungs and upper airway may be involved. Patients with such underlying diseases may present with:

• Cough
• Hemoptysis
• Dyspnea
• Nasal bleeding, obstruction, or sinusitis.