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

Jump to navigation Jump to search
VisualWikitext
No edit summary
No edit summary
 
(4 intermediate revisions by the same user not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{Rapidly progressive glomerulonephritis}}
{{Rapidly progressive glomerulonephritis}}
{{CMG}} {{AE}}  
{{CMG}} {{AE}} {{JSS}}
 
==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.[[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]].


==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 [[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]], [[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 [[Hemoptysis|haemoptysis]] as well.
* RPGN can lead to [[Acute kidney injury|ARF]] when renal symptoms develop.
* 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 [[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]].
* Histopathologically, the presence of fibrous crescents indicate irreversible renal damage and poor prognosis.
 
=== Complications ===
* [[Nephritic syndrome]]
* [[Nephrotic syndrome]]
* [[Acute kidney injury]]
* [[Chronic renal failure]]
 
=== 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>
=== Prognosis ===
* The prognosis of rapidly progressive glomerulonephritis is poor due to rapid deterioration of [[Renal function|kidney function]] in a few weeks.


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 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
** Serum [[creatinine]]
** Presence of pulmonary symptoms at the onset of disease
** Race
** Presence of [[Anti-neutrophil cytoplasmic antibody|ANCA]]
** Presence of fibrous cresecnts on histopathology.
* Factors that are indicators of poor prognosis :
** Age >60 years
** Serum creatinine > 7.5 mg/dl.
** [[Renal insufficiency|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 transplantation|kidney transplant]].


==References==
==References==

Latest revision as of 19:41, 24 July 2018

Rapidly progressive glomerulonephritis Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Rapidly progressive glomerulonephritis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray Findings

CT-scan Findings

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

Case #1

Rapidly progressive glomerulonephritis natural history, complications and prognosis On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Rapidly progressive glomerulonephritis natural history, complications and prognosis

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Rapidly progressive glomerulonephritis natural history, complications and prognosis

CDC on Rapidly progressive glomerulonephritis natural history, complications and prognosis

Rapidly progressive glomerulonephritis natural history, complications and prognosis in the news

Blogs on Rapidly progressive glomerulonephritis natural history, complications and prognosis

Directions to Hospitals Treating Rapidly progressive glomerulonephritis

Risk calculators and risk factors for Rapidly progressive glomerulonephritis natural history, complications and prognosis

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

  1. ↑ Couser WG (1988). "Rapidly progressive glomerulonephritis: classification, pathogenetic mechanisms, and therapy". Am J Kidney Dis. 11 (6): 449–64. PMID 3287904.
  2. ↑ Couser WG (1998). "Pathogenesis of glomerular damage in glomerulonephritis". Nephrol Dial Transplant. 13 Suppl 1: 10–5. PMID 9507491.
  3. ↑ 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.
  4. ↑ 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.

Template:WH Template:WS

Retrieved from "https://www.wikidoc.org/index.php?title=Rapidly_progressive_glomerulonephritis_natural_history,_complications_and_prognosis&oldid=1484801"