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==Overview==
==Medical therapy==
===Blood Work-Up===
* Complete blood count (CBC) differential and platelet count
 
* Serum electrolytes
 
* Creatinine and blood urea nitrogen (BUN)
 
* Lactate dehydrogenase (LDH)
 
* Creatine phosphokinase (CPK)
 
* Liver function tests
 
* Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)
 
* Antinuclear antibody (ANA)
 
* Anti-GBM antibodies
 
* ANCA
 
* Cryoglobulins
 
* HBV and HCV titers
 
* Complement C3 and C4 levels
 
* Serum protein electrophoresis
 
Anemia is common among patient with RPGN, mostly due to renally impaired production of erythropoietin or GI bleeding. Eosinophilia may be seen in a subset of patients with Churg-Strauss disease.
 
Patients with RPGN may show formation of immune complexes and cryoglobulins. Complement C3 levels is usually low in immune-complex mediated RPGN. The presence of ANCA and anti-GBM is variable; their presence is important for classification of disease and further management planning. Anti-GBM levels is However, anti-GBM antibody level is not prognostic and is not associated with disease activity.<ref name="pmid9744974">{{cite journal| author=Hricik DE, Chung-Park M, Sedor JR| title=Glomerulonephritis. | journal=N Engl J Med | year= 1998 | volume= 339 | issue= 13 | pages= 888-99 | pmid=9744974 | doi=10.1056/NEJM199809243391306 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9744974 }} </ref> On the contrary, literature regarding ANCA-associated glomerulonephritis suggests that levels of ANCA is associated with disease activity and may be used as an index for such purposes.<ref name="pmid2857806">{{cite journal| author=van der Woude FJ, Rasmussen N, Lobatto S, Wiik A, Permin H, van Es LA et al.| title=Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener's granulomatosis. | journal=Lancet | year= 1985 | volume= 1 | issue= 8426 | pages= 425-9 | pmid=2857806 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2857806 }} </ref><ref name="pmid2684074">{{cite journal| author=Tervaert JW, van der Woude FJ, Fauci AS, Ambrus JL, Velosa J, Keane WF et al.| title=Association between active Wegener's granulomatosis and anticytoplasmic antibodies. | journal=Arch Intern Med | year= 1989 | volume= 149 | issue= 11 | pages= 2461-5 | pmid=2684074 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2684074 }} </ref><ref name="pmid2221646">{{cite journal| author=Falk RJ, Hogan S, Carey TS, Jennette JC| title=Clinical course of anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and systemic vasculitis. The Glomerular Disease Collaborative Network. | journal=Ann Intern Med | year= 1990 | volume= 113 | issue= 9 | pages= 656-63 | pmid=2221646 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2221646 }} </ref>
 
ESR and CRP may be elevated and are correlated with the level of inflammation and thus activity of the disease.
 
===Urine Work-Up===
*Urinalysis
*Urinary protein electrophoresis
 
Patients with RPGN do not usually have a full-blown picture of nephrotic syndrome; Nephrotic syndrome only occurs in less than 30% of cases.<ref name="pmid9744974">{{cite journal| author=Hricik DE, Chung-Park M, Sedor JR| title=Glomerulonephritis. | journal=N Engl J Med | year= 1998 | volume= 339 | issue= 13 | pages= 888-99 | pmid=9744974 | doi=10.1056/NEJM199809243391306 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9744974 }} </ref> Proteinuria, if present, is usually mild to moderate. Hematuria of glomerular type with dysmorphic red blood cells is usually present on urinalysis and associated with red cell casts. Mild to moderate leukocyturia may be seen and other casts, such as epithelial cell leukocyte, or fatty casts. Urinary findings in RPGN are important features that not only favor diagnostic work-up, but also follow-up and therapeutic effectiveness.  


==References==
==References==

Revision as of 20:28, 7 May 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Medical therapy

Blood Work-Up

  • Complete blood count (CBC) differential and platelet count
  • Serum electrolytes
  • Creatinine and blood urea nitrogen (BUN)
  • Lactate dehydrogenase (LDH)
  • Creatine phosphokinase (CPK)
  • Liver function tests
  • Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)
  • Antinuclear antibody (ANA)
  • Anti-GBM antibodies
  • ANCA
  • Cryoglobulins
  • HBV and HCV titers
  • Complement C3 and C4 levels
  • Serum protein electrophoresis

Anemia is common among patient with RPGN, mostly due to renally impaired production of erythropoietin or GI bleeding. Eosinophilia may be seen in a subset of patients with Churg-Strauss disease.

Patients with RPGN may show formation of immune complexes and cryoglobulins. Complement C3 levels is usually low in immune-complex mediated RPGN. The presence of ANCA and anti-GBM is variable; their presence is important for classification of disease and further management planning. Anti-GBM levels is However, anti-GBM antibody level is not prognostic and is not associated with disease activity.[1] On the contrary, literature regarding ANCA-associated glomerulonephritis suggests that levels of ANCA is associated with disease activity and may be used as an index for such purposes.[2][3][4]

ESR and CRP may be elevated and are correlated with the level of inflammation and thus activity of the disease.

Urine Work-Up

  • Urinalysis
  • Urinary protein electrophoresis

Patients with RPGN do not usually have a full-blown picture of nephrotic syndrome; Nephrotic syndrome only occurs in less than 30% of cases.[1] Proteinuria, if present, is usually mild to moderate. Hematuria of glomerular type with dysmorphic red blood cells is usually present on urinalysis and associated with red cell casts. Mild to moderate leukocyturia may be seen and other casts, such as epithelial cell leukocyte, or fatty casts. Urinary findings in RPGN are important features that not only favor diagnostic work-up, but also follow-up and therapeutic effectiveness.

References

  1. 1.0 1.1 Hricik DE, Chung-Park M, Sedor JR (1998). "Glomerulonephritis". N Engl J Med. 339 (13): 888–99. doi:10.1056/NEJM199809243391306. PMID 9744974.
  2. van der Woude FJ, Rasmussen N, Lobatto S, Wiik A, Permin H, van Es LA; et al. (1985). "Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener's granulomatosis". Lancet. 1 (8426): 425–9. PMID 2857806.
  3. Tervaert JW, van der Woude FJ, Fauci AS, Ambrus JL, Velosa J, Keane WF; et al. (1989). "Association between active Wegener's granulomatosis and anticytoplasmic antibodies". Arch Intern Med. 149 (11): 2461–5. PMID 2684074.
  4. Falk RJ, Hogan S, Carey TS, Jennette JC (1990). "Clinical course of anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and systemic vasculitis. The Glomerular Disease Collaborative Network". Ann Intern Med. 113 (9): 656–63. PMID 2221646.

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