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crescentic glomerulonephritis (rapidly progressive glomerulonephritis)

Etiology: (see expanded etiology) 1) idiopathic 2) ANCA-associated vasculitis a) Wegener's granulomatosis b) microscopic polyangiitis 3) anti-glomerular basement membrane (GBM) disease - Goodpasture's syndrome 4) IgA nephropathy 5) idiopathic membranoproliferative glomerulonephritis 6) post-infectious glomerulonephritis 7) lupus nephritis Epidemiology: - mean age 55 years Pathology: 1) distinctive crescents from by proliferation of parietal cells & migration of monocytes & macrophages into Bowman's space 2) fibrin stands between layers of cells in crescents 3) disruption of glomerular basement membrane (EM) 4) immune deposits by immunofluorescence if infectious etiology (3 patterns): a) type I, linear IgG (Goodpasture's syndrome anti-GBM) b) type II, granular immune complexes (D-penicillamine) c) type III, negative immunofluorescence (pauci-immune) 5) alveolar hemorrhage is mediated by deposition of immunecomplexes Clinical manifestations: 1) subacute renal failure (days to weeks to months) 2) nephrotic syndrome may be present 3) oliguria is not uncommon 4) hematuria 5) hypertension 6) edema 7) preceding viral illness may be noted 8) pulmonary-renal syndrome is frequent - alveolar hemorrhage may occur in the absence of anti- glomerular basement membrane antibody 9) type III may present as isolated glomerulonephritis or in association with vasculitis Laboratory: 1) urinalysis a) proteinuria b) microscopy: hematuria, RBC casts, granular casts 2) 24 hour urine may show nephrotic range proteinuria 3) chemistry panel with eGFR - increasing serum creatinine - >= 50% decline in GFR over short period of time 3) anti-neutrophil cytoplasmic antibody (ANCA) may be positive a) especially in type III (pauci-immune) b) may be p-ANCA or c-ANCA c) value of titers to follow course of disease is controversial* 4) serum complement levels are normal 5) >= 50% decline in GFR over short period of time * it appears that MKSAP19 endorses following titers [3] Special laboratory: - renal biopsy [3,6] Management: 1) high-dose glucocorticoids plus cyclophosphamide standard therapy [6] a) methylprednisolone (Solumedrol) 30 mg/kg up to 3 grams IV every 4 hours for 3 doses, followed by prednisone 2 mg/kg PO QOD for 2 months; taper over 4 months b) cyclophosphamide (Cytoxan) 2 mg/kg PO QD (necrotizing glomerulonephritis) 2) plasmapheresis is helpful in Goodpasture's syndrome 3) prognosis a) 40% progress to chronic renal insufficiency b) 35% develop end-stage renal disease c) mortality 25% d) poor prognositic indicators: - serum creatinine > 7 mg/dL - old age

Interactions

disease interactions

Related

etiology of crescentic glomerulonephritis (rapidly progressive glomerulonephritis)

Specific

ANCA-associated glomerulonephritis

General

glomerulonephritis (GN, nephritic syndrome)

References

  1. Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 269
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 605, 785
  3. Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 17, 19. American College of Physicians, Philadelphia 1998, 2012, 2015, 2021 - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  4. Jabur WL, Saeed HM. ANCA-positive pauci-immune rapidly progressive glomerulonephritis and the nephrotic syndrome. Saudi J Kidney Dis Transpl. 2010 May;21(3):526-30. PMID: 20427883
  5. Rutgers A, Sanders JS, Stegeman CA, Kallenberg CG. Pauci-immune necrotizing glomerulonephritis. Rheum Dis Clin North Am. 2010 Aug;36(3):559-72 PMID: 20688250
  6. Moroni G, Ponticelli C. Rapidly progressive crescentic glomerulonephritis: Early treatment is a must. Autoimmun Rev. 2014 Jul;13(7):723-9. Review. PMID: 24657897