Pauci-immune glomerulonephritis

Pauci-immune glomerulonephritis is a type of rapidly progressive kidney inflammation. It causes severe kidney problems over days or weeks by damaging the small blood vessels in the kidneys. The condition often leads to symptoms such as blood in the urine and high blood pressure, and it can quickly lead to kidney failure. In many cases, pauci-immune glomerulonephritis is part of a broader process involving systemic small vessel vasculitis. This means that other parts of the body can be affected, and people with this condition might experience symptoms in the joints, lungs, or nervous system.

People with this condition may have blood in the urine, high blood pressure, and kidney failure that develops quickly. They might also experience systemic signs of vasculitis, such as joint pain, fever, seizures, mononeuritis, or lung involvement.

The condition is linked to a process of small blood vessel inflammation. In many instances, it is associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCAs). Pauci-immune glomerulonephritis may be seen as part of a systemic small vessel vasculitis including microscopic polyangiitis, granulomatosis with polyangiitis, or eosinophilic granulomatosis with polyangiitis. There is also a rare form that is limited to the kidneys.

Information about treatment is currently limited for this condition.

The condition is serious and can lead to kidney failure rapidly over days or weeks. Early diagnosis is important, as the damage to the kidneys can progress quickly. Overall, the outlook depends on how fast treatment begins once the disease is recognized.

Information about current research is currently limited for this condition.