Rapidly progressive glomerulonephritis natural history, complications and prognosis: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Rapidly progressive glomerulonephritis}} | {{Rapidly progressive glomerulonephritis}} | ||
{{CMG}} {{AE}} | {{CMG}} {{AE}} {{JSS}} | ||
==Overview== | ==Overview== | ||
Patients with RPGN present with flu like symptoms initially and then develop nephritic syndrome with proteinuria in some cases as well. In type III RPGN, systemic features of vasculitis are present in some cases.Pulmonary symptoms are also present in Goodpastures syndrome and Churg Strauss syndrome.The prognosis is usually poor due to rapid deterioration of renal function and is dependent on age, presence of pulmonary symptoms, serum creatinine levels and presence of ANCAs. | Patients with RPGN present with [[flu]] like symptoms initially and then develop [[nephritic syndrome]] with [[proteinuria]] in some cases as well. In type III RPGN, systemic features of [[vasculitis]] are present in some cases.[[Lung|Pulmonary]] symptoms are also present in [[Goodpasture syndrome|Goodpastures syndrome]] and [[Churgs strauss syndrome|Churg Strauss syndrome]].The prognosis is usually poor due to rapid deterioration of [[renal function]] and is dependent on age, presence of pulmonary symptoms, serum [[creatinine]] levels and presence of [[Anti-neutrophil cytoplasmic antibody|ANCAs]]. | ||
==Natural history, complications and prognosis== | ==Natural history, complications and prognosis== | ||
=== Natural history === | === Natural history === | ||
* Patients with RPGN present with initial flu like symptoms and then progress to | * Patients with RPGN present with initial flu like symptoms and then progress to [[nephritic syndrome]] in 2-3 months if not treated in time<ref name="pmid3287904">{{cite journal| author=Couser WG| title=Rapidly progressive glomerulonephritis: classification, pathogenetic mechanisms, and therapy. | journal=Am J Kidney Dis | year= 1988 | volume= 11 | issue= 6 | pages= 449-64 | pmid=3287904 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3287904 }} </ref><ref name="pmid9507491">{{cite journal| author=Couser WG| title=Pathogenesis of glomerular damage in glomerulonephritis. | journal=Nephrol Dial Transplant | year= 1998 | volume= 13 Suppl 1 | issue= | pages= 10-5 | pmid=9507491 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9507491 }} </ref>. | ||
* Symptoms include malaise, arthralgias, fever, anorexia, weight loss, haematuria, edema, hypertension and abdominal pain. | * Symptoms include [[malaise]], [[Arthralgia|arthralgias]], [[fever]][[Anorexia|, anorexia]], weight loss[[Haematuria|, haematuria]], [[edema]][[Hypertension|, 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. | * Patients with respiratory system involvement as in Goodpasture syndrome and Churg strauss, have symtopms of [[asthma]], [[atopy]] and [[Hemoptysis|haemoptysis]] as well. | ||
* RPGN can lead to ARF when renal symptoms develop. | * RPGN can lead to [[Acute kidney injury|ARF]] when renal symptoms develop. | ||
* Increase in serum creatinine and fall in GFR are used to measure the severity of the disease. | * Increase in serum [[creatinine]] and fall in [[Glomerular filtration rate|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. | * 70-80% Patients with RPGN will progress to [[Chronic renal failure|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. | * 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. | * Histopathologically, the presence of fibrous crescents indicate irreversible renal damage and poor prognosis. | ||
=== Complications === | === Complications === | ||
* Nephritic syndrome | * [[Nephritic syndrome]] | ||
* Nephrotic syndrome | * [[Nephrotic syndrome]] | ||
* Acute kidney injury | * [[Acute kidney injury]] | ||
* Chronic renal failure | * [[Chronic renal failure]] | ||
=== Prognosis === | === Prognosis === | ||
* The prognosis of rapidly progressive glomerulonephritis is poor due to rapid deterioration of kidney function in a few weeks. | |||
=== Prognosis === | |||
* The prognosis of rapidly progressive glomerulonephritis is poor due to rapid deterioration of [[Renal function|kidney function]] in a few weeks. | |||
* Prognosis depends on<ref name="pmid11774098">{{cite journal| author=Zäuner I, Bach D, Braun N, Krämer BK, Fünfstück R, Helmchen U et al.| title=Predictive value of initial histology and effect of plasmapheresis on long-term prognosis of rapidly progressive glomerulonephritis. | journal=Am J Kidney Dis | year= 2002 | volume= 39 | issue= 1 | pages= 28-35 | pmid=11774098 | doi=10.1053/ajkd.2002.29874 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11774098 }} </ref><ref name="pmid16825335">{{cite journal| author=de Lind van Wijngaarden RA, Hauer HA, Wolterbeek R, Jayne DR, Gaskin G, Rasmussen N et al.| title=Clinical and histologic determinants of renal outcome in ANCA-associated vasculitis: A prospective analysis of 100 patients with severe renal involvement. | journal=J Am Soc Nephrol | year= 2006 | volume= 17 | issue= 8 | pages= 2264-74 | pmid=16825335 | doi=10.1681/ASN.2005080870 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16825335 }} </ref> | * Prognosis depends on<ref name="pmid11774098">{{cite journal| author=Zäuner I, Bach D, Braun N, Krämer BK, Fünfstück R, Helmchen U et al.| title=Predictive value of initial histology and effect of plasmapheresis on long-term prognosis of rapidly progressive glomerulonephritis. | journal=Am J Kidney Dis | year= 2002 | volume= 39 | issue= 1 | pages= 28-35 | pmid=11774098 | doi=10.1053/ajkd.2002.29874 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11774098 }} </ref><ref name="pmid16825335">{{cite journal| author=de Lind van Wijngaarden RA, Hauer HA, Wolterbeek R, Jayne DR, Gaskin G, Rasmussen N et al.| title=Clinical and histologic determinants of renal outcome in ANCA-associated vasculitis: A prospective analysis of 100 patients with severe renal involvement. | journal=J Am Soc Nephrol | year= 2006 | volume= 17 | issue= 8 | pages= 2264-74 | pmid=16825335 | doi=10.1681/ASN.2005080870 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16825335 }} </ref> | ||
** Age | ** Age | ||
** Serum creatinine | ** Serum [[creatinine]] | ||
** Presence of pulmonary symptoms at the onset of disease | ** Presence of pulmonary symptoms at the onset of disease | ||
** Race | ** Race | ||
** Presence of ANCA | ** Presence of [[Anti-neutrophil cytoplasmic antibody|ANCA]] | ||
** Presence of fibrous cresecnts on histopathology. | ** Presence of fibrous cresecnts on histopathology. | ||
* Factors that are indicators of poor prognosis : | * Factors that are indicators of poor prognosis : | ||
** Age >60 years | ** Age >60 years | ||
** Serum creatinine > 7.5 mg/dl. | ** Serum creatinine > 7.5 mg/dl. | ||
** Oliguric renal failure | ** [[Renal insufficiency|Oliguric renal failure]] | ||
** Pauci immune RPGN | ** Pauci immune RPGN | ||
** White race | ** White race | ||
** 75 % fibrous cresecents in the glomerulus | ** 75 % fibrous cresecents in the glomerulus | ||
* 70-80% of patients with RPGN develop end stage renal disease and require dialysis for a long time. | * 70-80% of patients with RPGN develop end stage renal disease and require [[dialysis]] for a long time and some may require [[Kidney transplantation|kidney transplant]]. | ||
==References== | ==References== | ||
Latest revision as of 19:41, 24 July 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Jogeet Singh Sekhon, M.D. [2]
Overview
Patients with RPGN present with flu like symptoms initially and then develop nephritic syndrome with proteinuria in some cases as well. In type III RPGN, systemic features of vasculitis are present in some cases.Pulmonary symptoms are also present in Goodpastures syndrome and Churg Strauss syndrome.The prognosis is usually poor due to rapid deterioration of renal function and is dependent on age, presence of pulmonary symptoms, serum creatinine levels and presence of ANCAs.
Natural history, complications and prognosis
Natural history
- Patients with RPGN present with initial flu like symptoms and then progress to nephritic syndrome in 2-3 months if not treated in time[1][2].
- 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
Prognosis
Prognosis
- The prognosis of rapidly progressive glomerulonephritis is poor due to rapid deterioration of kidney function in a few weeks.
- Prognosis depends on[3][4]
- 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 and some may require kidney transplant.
References
- ↑ Couser WG (1988). "Rapidly progressive glomerulonephritis: classification, pathogenetic mechanisms, and therapy". Am J Kidney Dis. 11 (6): 449–64. PMID 3287904.
- ↑ Couser WG (1998). "Pathogenesis of glomerular damage in glomerulonephritis". Nephrol Dial Transplant. 13 Suppl 1: 10–5. PMID 9507491.
- ↑ Zäuner I, Bach D, Braun N, Krämer BK, Fünfstück R, Helmchen U; et al. (2002). "Predictive value of initial histology and effect of plasmapheresis on long-term prognosis of rapidly progressive glomerulonephritis". Am J Kidney Dis. 39 (1): 28–35. doi:10.1053/ajkd.2002.29874. PMID 11774098.
- ↑ de Lind van Wijngaarden RA, Hauer HA, Wolterbeek R, Jayne DR, Gaskin G, Rasmussen N; et al. (2006). "Clinical and histologic determinants of renal outcome in ANCA-associated vasculitis: A prospective analysis of 100 patients with severe renal involvement". J Am Soc Nephrol. 17 (8): 2264–74. doi:10.1681/ASN.2005080870. PMID 16825335.