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
Old Classification
Following its initial description in 1914, crescenteric glomerulonephritis was first classified by Couser in 1988 based on features of immunofluorescence.[1]
Type I: Anti-GBM glomerulonephritis: 20% of patients
Presence of linear staining of glomerular basement membrane (GBM)
Type II: Pauci-immune glomerulonephritis: 50% of patients
Absent or minimal immune deposits
Type III: Immune complex-mediated glomerulonephritis: 30% of patients
Presence of granular patterns of immune deposits within the glomerulus. Immune deposition may be associated with any of the following conditions:
- Infections
- Systemic illnesses
- Other primary glomerular diseases
New Classification
Upon the detection of new serological markers such as anti-GBM antibodies and anti-neutrophil cytoplasmic antibodies (ANCA)[2], the classification of RPGN has changed to involve several types of primary glomerulonephritis that correspond to the quantity and quality of such findings in patients’ sera. ANCA and anti-GBM may co-exist in approximately 20% of the patients.[3]
Type I: Anti-GBM Disease
- Anti-GBM antibody-mediated without pulmonary involvement
- Goodpasture’s disease: Anti-GBM antibody-mediated with pulmonary hemorrhage
Type II: Immune Complex-Mediated Disease
Type III: Pauci-Immune Disease
ANCA positive
- Idiopathic renal-limited vasculitis / renal-limited necrotizing crescenteric glomerulonephritis (NCGN)
- Granulomatosis with polyangiitis (formerly “Wegener’s granulomatosis")
- Microscopic polyangiitis
- Churg-Strauss syndrome
Type IV: Mixed Anti-GBM and ANCA Associated Disease
Type V: Pauci-Immune
ANCA negative
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
Case Studies
References
- ↑ Couser WG (1988). "Rapidly progressive glomerulonephritis: classification, pathogenetic mechanisms, and therapy". Am J Kidney Dis. 11 (6): 449–64. PMID 3287904.
- ↑ Hricik DE, Chung-Park M, Sedor JR (1998). "Glomerulonephritis". N Engl J Med. 339 (13): 888–99. doi:10.1056/NEJM199809243391306. PMID 9744974.
- ↑ Short AK, Esnault VL, Lockwood CM (1995). "Anti-neutrophil cytoplasm antibodies and anti-glomerular basement membrane antibodies: two coexisting distinct autoreactivities detectable in patients with rapidly progressive glomerulonephritis". Am J Kidney Dis. 26 (3): 439–45. PMID 7544065.