Rapidly progressive glomerulonephritis
Rapidly progressive glomerulonephritis | |
ICD-10 | N00-N08 with .7 suffix |
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DiseasesDB | 3165 |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords:: Crescentic glomerulonephritis; RPGN
Overview
Rapidly progressive glomerulonephritis (RPGN) is one of the few nephrological emergency. However, it fortunately only affects 1-4% of all cases of glomerulonephritis. It is a clinical syndrome that includes signs and symptoms of glomerulonephritis, including hematuria, proteinuria, and edema with signs of renal failure and diffuse crescent formation on histopathology. Without appropriate treatment, RPGN progresses into end-stage renal disease within several days to only a few months yielding a very poor prognosis and renal outcome. RPGN is classified based on the presence of absence of anti-neutrophil cytoplasmic antibody (ANCA) and anti-GBM antibodies. Due to its rarity, the pathogenesis of RPGN is poorly understood and most probably is related to the type of RPGN and the circulating antibodies associated with each type. Evidence on treatment options for RPGN is poor; but the use of glucocorticoids and cyclophosphamide is currently recommended. Basic research and clinical data are currently emerging to better understand the disease pathogenesis and optimal therapeutic options.
Classification
Pathophysiology
Causes
Differentiating Rapidly progressive glomerulonephritis from other Diseases
Epidemiology and Demographics
Risk Factors
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Criteria | History and Symptoms | Physical Examination | Laboratory Findings | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies
Treatment
Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies