Pauci-immune

Pauci-immune
Synonyms	Pauci-immune glomerulonephritis
Pronounce	N/A
Specialty	N/A
Symptoms	Hematuria, proteinuria, hypertension, edema
Complications	Chronic kidney disease, end-stage renal disease
Onset	Variable
Duration	Chronic
Types	N/A
Causes	Autoimmune disease
Risks	ANCA-associated vasculitis
Diagnosis	Kidney biopsy, serology
Differential diagnosis	Lupus nephritis, IgA nephropathy, Goodpasture syndrome
Prevention	N/A
Treatment	Immunosuppressive therapy, plasmapheresis
Medication	N/A
Prognosis	Variable, depends on response to treatment
Frequency	Rare
Deaths	N/A

Pauci-immune is a term used in medicine to describe a condition where there is a minimal amount of immune deposits found in the walls of small blood vessels. This condition is often associated with vasculitis, a group of rare diseases that involve inflammation of the blood vessels.

Overview

Pauci-immune conditions are often characterized by the absence of antibodies or immune complexes in the walls of the blood vessels. This is in contrast to other forms of vasculitis, where these immune deposits are often present. The most common diseases associated with pauci-immune conditions are granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). These diseases are also known as ANCA-associated vasculitis as they are often associated with anti-neutrophil cytoplasmic antibodies (ANCA).

Symptoms

The symptoms of pauci-immune conditions can vary widely depending on the specific disease and the organs affected. However, common symptoms can include fever, fatigue, weight loss, and muscle pain. In severe cases, these conditions can lead to kidney failure or lung disease.

Diagnosis

Diagnosis of pauci-immune conditions often involves a combination of blood tests, imaging studies, and biopsies. Blood tests can detect the presence of ANCA, while imaging studies such as CT scans or MRI can help identify any damage to the organs. Biopsies can confirm the diagnosis by showing the absence of immune deposits in the blood vessels.

Treatment

Treatment for pauci-immune conditions typically involves immunosuppressive therapy to reduce inflammation and prevent further damage to the organs. This can include medications such as corticosteroids and cyclophosphamide. In severe cases, plasma exchange may be used.

See also

• Vasculitis
• Granulomatosis with polyangiitis
• Microscopic polyangiitis
• Eosinophilic granulomatosis with polyangiitis