Rapidly progressive glomerulonephritis natural history, complications and prognosis: Difference between revisions
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==Overview== | |||
==Natural history, complications and prognosis== | |||
The prognosis of RPGN is poor due to the rapid deterioration of kidney function into end-stage renal disease (ESRD) within a few weeks or months. Early diagnosis and management are both important factors to improve prognosis and avoid progression into ESRD.<ref name="pmid8808106">{{cite journal| author=Hogan SL, Nachman PH, Wilkman AS, Jennette JC, Falk RJ| title=Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. | journal=J Am Soc Nephrol | year= 1996 | volume= 7 | issue= 1 | pages= 23-32 | pmid=8808106 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8808106 }} </ref><ref name="pmid8944617">{{cite journal| author=Lal DP, O'Donoghue DJ, Haeney M| title=Effect of diagnostic delay on disease severity and outcome in glomerulonephritis caused by anti-neutrophil cytoplasmic antibodies. | journal=J Clin Pathol | year= 1996 | volume= 49 | issue= 11 | pages= 942-4 | pmid=8944617 | doi= | pmc=PMC500837 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8944617 }} </ref><ref name="pmid3826068">{{cite journal| author=Heilman RL, Offord KP, Holley KE, Velosa JA| title=Analysis of risk factors for patient and renal survival in crescentic glomerulonephritis. | journal=Am J Kidney Dis | year= 1987 | volume= 9 | issue= 2 | pages= 98-107 | pmid=3826068 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3826068 }} </ref> Histopathologically, the extent of fibrous crescents is associated with poorer renal survival.<ref name="pmid1244562">{{cite journal| author=Whitworth JA, Morel-Maroger L, Mignon F, Richet G| title=The significance of extracapillary proliferation. Clinicopathological review of 60 patients. | journal=Nephron | year= 1976 | volume= 16 | issue= 1 | pages= 1-19 | pmid=1244562 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1244562 }} </ref> The degree of renal impairment at diagnosis is also a significant prognostic factor. Serum creatinine > 6 mg/dL is associated with very poor renal outcomes in patients with anti-GBM disease.<ref name="pmid8203364">{{cite journal| author=Mokrzycki MH, Kaplan AA| title=Therapeutic plasma exchange: complications and management. | journal=Am J Kidney Dis | year= 1994 | volume= 23 | issue= 6 | pages= 817-27 | pmid=8203364 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8203364 }} </ref> | |||
The prognosis of RPGN also depends on the exact diagnosis within subtypes of RPGN. The amount of studies that studied the natural history and prognosis of RPGN and its subtypes are very rare, especially because the disease itself is uncommon. Appropriate therapy in patients with anti-GBM disease and Goodpasture’s disease was shown to significantly alter prognosis, with survival rates reaching as high as 70-90%; but only 40% of patients do not require dialysis after 1 year.<ref name="pmid9241619">{{cite journal| author=Levy JB, Pusey CD| title=Still a role for plasma exchange in rapidly progressive glomerulonephritis? | journal=J Nephrol | year= 1997 | volume= 10 | issue= 1 | pages= 7-13 | pmid=9241619 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9241619 }} </ref> | |||
Another study that enrolled patients with microscopic polyangiitis and glomerulonephritis and positive ANCA.<ref name="pmid8808107">{{cite journal| author=Nachman PH, Hogan SL, Jennette JC, Falk RJ| title=Treatment response and relapse in antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. | journal=J Am Soc Nephrol | year= 1996 | volume= 7 | issue= 1 | pages= 33-9 | pmid=8808107 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8808107 }} </ref> Following appropriate therapy, more than 70% of the patients were in remission; almost half of which continued to be disease-free after 4 years. Similarly, renal function at diagnosis is associated with renal outcome in patients with positive ANCA.<ref name="pmid8808107">{{cite journal| author=Nachman PH, Hogan SL, Jennette JC, Falk RJ| title=Treatment response and relapse in antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. | journal=J Am Soc Nephrol | year= 1996 | volume= 7 | issue= 1 | pages= 33-9 | pmid=8808107 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8808107 }} </ref> | |||
Patients with poststreptococcal RPGN generally have better outcomes compared to those with primary RPGN. But these findings were observed in the adult population, and may not be true for pediatric patients.<ref name="pmid5476201">{{cite journal| author=Leonard CD, Nagle RB, Striker GE, Cutler RE, Scribner BH| title=Acute glomerulonephritis with prolonged oliguria. An analysis of 29 cases. | journal=Ann Intern Med | year= 1970 | volume= 73 | issue= 5 | pages= 703-11 | pmid=5476201 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5476201 }} </ref><ref name="pmid7360526">{{cite journal| author=Cunningham RJ, Gilfoil M, Cavallo T, Brouhard BH, Travis LB, Berger M et al.| title=Rapidly progressive glomerulonephritis in children: a report of thirteen cases and a review of the literature. | journal=Pediatr Res | year= 1980 | volume= 14 | issue= 2 | pages= 128-32 | pmid=7360526 | doi=10.1203/00006450-198002000-00012 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7360526 }} </ref> | |||
==References== | ==References== | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:
Overview
Natural history, complications and prognosis
The prognosis of RPGN is poor due to the rapid deterioration of kidney function into end-stage renal disease (ESRD) within a few weeks or months. Early diagnosis and management are both important factors to improve prognosis and avoid progression into ESRD.[1][2][3] Histopathologically, the extent of fibrous crescents is associated with poorer renal survival.[4] The degree of renal impairment at diagnosis is also a significant prognostic factor. Serum creatinine > 6 mg/dL is associated with very poor renal outcomes in patients with anti-GBM disease.[5]
The prognosis of RPGN also depends on the exact diagnosis within subtypes of RPGN. The amount of studies that studied the natural history and prognosis of RPGN and its subtypes are very rare, especially because the disease itself is uncommon. Appropriate therapy in patients with anti-GBM disease and Goodpasture’s disease was shown to significantly alter prognosis, with survival rates reaching as high as 70-90%; but only 40% of patients do not require dialysis after 1 year.[6]
Another study that enrolled patients with microscopic polyangiitis and glomerulonephritis and positive ANCA.[7] Following appropriate therapy, more than 70% of the patients were in remission; almost half of which continued to be disease-free after 4 years. Similarly, renal function at diagnosis is associated with renal outcome in patients with positive ANCA.[7]
Patients with poststreptococcal RPGN generally have better outcomes compared to those with primary RPGN. But these findings were observed in the adult population, and may not be true for pediatric patients.[8][9]
References
- ↑ Hogan SL, Nachman PH, Wilkman AS, Jennette JC, Falk RJ (1996). "Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis". J Am Soc Nephrol. 7 (1): 23–32. PMID 8808106.
- ↑ Lal DP, O'Donoghue DJ, Haeney M (1996). "Effect of diagnostic delay on disease severity and outcome in glomerulonephritis caused by anti-neutrophil cytoplasmic antibodies". J Clin Pathol. 49 (11): 942–4. PMC 500837. PMID 8944617.
- ↑ Heilman RL, Offord KP, Holley KE, Velosa JA (1987). "Analysis of risk factors for patient and renal survival in crescentic glomerulonephritis". Am J Kidney Dis. 9 (2): 98–107. PMID 3826068.
- ↑ Whitworth JA, Morel-Maroger L, Mignon F, Richet G (1976). "The significance of extracapillary proliferation. Clinicopathological review of 60 patients". Nephron. 16 (1): 1–19. PMID 1244562.
- ↑ Mokrzycki MH, Kaplan AA (1994). "Therapeutic plasma exchange: complications and management". Am J Kidney Dis. 23 (6): 817–27. PMID 8203364.
- ↑ Levy JB, Pusey CD (1997). "Still a role for plasma exchange in rapidly progressive glomerulonephritis?". J Nephrol. 10 (1): 7–13. PMID 9241619.
- ↑ 7.0 7.1 Nachman PH, Hogan SL, Jennette JC, Falk RJ (1996). "Treatment response and relapse in antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis". J Am Soc Nephrol. 7 (1): 33–9. PMID 8808107.
- ↑ Leonard CD, Nagle RB, Striker GE, Cutler RE, Scribner BH (1970). "Acute glomerulonephritis with prolonged oliguria. An analysis of 29 cases". Ann Intern Med. 73 (5): 703–11. PMID 5476201.
- ↑ Cunningham RJ, Gilfoil M, Cavallo T, Brouhard BH, Travis LB, Berger M; et al. (1980). "Rapidly progressive glomerulonephritis in children: a report of thirteen cases and a review of the literature". Pediatr Res. 14 (2): 128–32. doi:10.1203/00006450-198002000-00012. PMID 7360526.