Rapidly progressive glomerulonephritis natural history, complications and prognosis: Difference between revisions

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==Overview==  
==Overview==  
==Natural history, complications and prognosis==
==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>
=== Natural history ===
* Patients with RPGN present with initial flu like symptoms and then progress to nephritc syndrome in 2-3 months if not treated in time.
* Symptoms include malaise, arthralgias, fever, anorexia, weight loss, haematuria, edema, hypertension and  abdominal pain.
* Patients with respiratory system involvement as in Goodpasture syndrome and Churg strauss, have symtopms of asthma, atopy and haemoptysis as well.
* RPGN can lead to ARF when renal symptoms develop.
* Increase in serum creatinine and fall in GFR are used to measure the severity of the disease.
* 70-80% Patients with RPGN will progress to end stage renal disease if not treated in time.
* Serum creatinine >4.6 mg/dl and GFR less than 50% denote end stage renal disease and requires dialysis.  
* Histopathologically, the presence of fibrous crescents indicate irreversible renal damage and poor prognosis.


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>
=== Complications ===
* Nephritic syndrome
* Nephrotic syndrome
* Acute kidney injury
* Chronic renal failure
* End stage renal disease


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>
=== Prognosis ===
* The prognosis of rapidly progressive glomerulonephritis is poor due to rapid deterioration of kidney function in a few weeks.
 
* Prognosis depends on :
** Age
** Serum creatinine
** Presence of pulmonary symptoms at the onset of disease
** Race
** Presence of ANCA
** Presence of fibrous cresecnts on histopathology.
* Factors that are indicators of poor prognosis :
** Age >60 years
** Serum creatinine > 7.5 mg/dl.
** Oliguric renal failure
** Pauci immune RPGN
** White race
** 75 % fibrous cresecents in the glomerulus
* 70-80% of patients with RPGN develop end stage renal disease and require dialysis for a long time.


==References==
==References==

Revision as of 15:11, 23 July 2018

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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

Natural history

  • Patients with RPGN present with initial flu like symptoms and then progress to nephritc syndrome in 2-3 months if not treated in time.
  • Symptoms include malaise, arthralgias, fever, anorexia, weight loss, haematuria, edema, hypertension and abdominal pain.
  • Patients with respiratory system involvement as in Goodpasture syndrome and Churg strauss, have symtopms of asthma, atopy and haemoptysis as well.
  • RPGN can lead to ARF when renal symptoms develop.
  • Increase in serum creatinine and fall in GFR are used to measure the severity of the disease.
  • 70-80% Patients with RPGN will progress to end stage renal disease if not treated in time.
  • Serum creatinine >4.6 mg/dl and GFR less than 50% denote end stage renal disease and requires dialysis.
  • Histopathologically, the presence of fibrous crescents indicate irreversible renal damage and poor prognosis.

Complications

  • Nephritic syndrome
  • Nephrotic syndrome
  • Acute kidney injury
  • Chronic renal failure
  • End stage renal disease

Prognosis

  • The prognosis of rapidly progressive glomerulonephritis is poor due to rapid deterioration of kidney function in a few weeks.
  • Prognosis depends on :
    • Age
    • Serum creatinine
    • Presence of pulmonary symptoms at the onset of disease
    • Race
    • Presence of ANCA
    • Presence of fibrous cresecnts on histopathology.
  • Factors that are indicators of poor prognosis :
    • Age >60 years
    • Serum creatinine > 7.5 mg/dl.
    • Oliguric renal failure
    • Pauci immune RPGN
    • White race
    • 75 % fibrous cresecents in the glomerulus
  • 70-80% of patients with RPGN develop end stage renal disease and require dialysis for a long time.

References

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