Rapidly progressive glomerulonephritis (RPGN) causes rapid decrease in kidney function when glomerular filtration rate drops up to 50% over days to 3 months. 

It is serious condition can cause acute kidney failure if untreated promptly. In 1970s patients fit criteria for rapidly progressive glomerulonephritis, but exact cause is not established. 

Cases displayed pauci-immune rapidly progressive glomerulonephritis due to lack of antibody deposition in biopsy specimens. 

RPGN linked to severe glomerular injury, inflammation, and damage. Inflammation causes crescents to form in Bowman’s space around glomeruli. 

It is classified into three categories as:  

Anti-glomerular basement membrane antibody disease 

Immune complex disease 

Pauci-immune disease 

Prevalence of ANCA disease is unknown, but rapidly progressive glomerulonephritis incidence is reported 7 cases per 1 million individuals each year. 

UK shows 2 cases per 100000 individuals. In US, white people impacted more than blacks as 7:1 ratio. 

The male-to-female ratio is approximately 1:1. The peak incidence occurs in the middle of the sixth decade of life. 

The correlation between ANCAs and ANCA-associated disease development is uncertain. ANCAs are thought to trigger early neutrophil activation causes enzyme release and oxygen metabolites at injury sites. 

ANCAs directly contribute to the development of pauci-immune vasculitis and glomerulonephritis. 

In vitro findings show these autoantibodies activate human leukocytes, while ANCAs target neutrophil granules and monocyte lysosomes. 

cANCA shows central accentuation in alcohol-fixed neutrophils. Nonspecific pANCA found in autoimmune/inflammatory diseases without MPO specificity. 

The cause of ANCA-associated disease is unknown. Granulomatosis with polyangiitis patients with Z alpha1-antitrypsin phenotype have aggressive disease. 

Study shows ANCA-activated neutrophils attack endothelial cells. 97% of patients with flu-like prodrome indicate viral cause. 

Early treatment leads to complete or partial remission for most with rapidly progressive crescentic glomerulonephritis.  

Higher serum creatinine indicates worse outcomes. Major cause of death in ANCA-associated disease is pulmonary hemorrhage. 

Granulomatosis with polyangiitis presents with upper airway lesions, glomerulonephritis, hemorrhage, and kidney failure. 

The history of ANCA vasculitis includes malaise, fever, myalgias, anorexia, and weight loss. 

>90% patients experience these diseases within days to months of vasculitis onset. 

• Respiratory examination  
• Cardiovascular examination  
• Neurological examination  
• Musculoskeletal examination  

• Clinical symptoms as: 
• Edema 
• Hypertension 
• Acute Kidney Injury 
• Hematuria and Proteinuria 
• Fatigue and Malaise 

Treatment includes corticosteroids and cyclophosphamide for ANCA-associated disease. 

Steroid treatment triples relapse risk. Serum creatinine at diagnosis predicts kidney survival.  

Intravenous preparation decreases cumulative toxicity risk with lower total dose in medications. 

Methotrexate replaces cyclophosphamide in initial granulomatosis with polyangiitis treatment for mild cases and post-induction therapy for severe cases. 

Plasmapheresis can help treat severe kidney failure or disease progression despite therapy in patients. 

Rituximab helps kidney outcomes in ANCA vasculitis, while T-cell therapy also improves outcomes. 

Nephrology

Patients should take a low-sodium diet control to reduce blood pressure and edema. 

Patients should not take any over-the-counter NSAIDs and other nephrotoxic drugs. 

Patients should record their blood pressure at home regularly and follow good hygiene practices to reduce the risk of infections. 

Proper awareness about RPGN should be provided and its related causes with management strategies. 

Appointments with a nephrologist and preventing recurrence of disorder is an ongoing life-long effort. 

Nephrology

Cyclophosphamide: 

It interferes with the growth of normal and neoplastic cells that involve cross-linking of DNA. 

Nephrology

Methylprednisolone: 

It decreases inflammation to suppress migration of PMN leukocytes and capillary permeability. 

Nephrology

Azathioprine: 

It decreases proliferation of immune cells to inhibit synthesis of DNA, RNA, and proteins.  

Methotrexate: 

It inhibits purine and thymidylic acid synthesis to interferes with DNA synthesis, and repair. 

Nephrology

A renal biopsy is performed to diagnose RPGN disease. 

Dialysis is performed in patients with severe acute kidney injury that causes uremia. 

Nephrology

In the initial stabilization phase, the goal is to prevent further kidney damage and diagnose any life-threatening complications in the patient. 

Pharmacologic therapy is effective in the treatment phase as it includes use of antineoplastics, corticosteroids, and immunosuppressants agent. 

In supportive care and management phase, patients should receive required attention such as lifestyle modification and intervention therapies. 

The regular follow-up visits with the nephrologist are scheduled to check the improvement of patients along with treatment response.