eMedicine Specialties > Nephrology > Glomerular Diseases

Glomerulonephritis, Rapidly Progressive: Follow-up

Author: James W Lohr, MD, Fellowship Program Director, Professor, Department of Internal Medicine, Division of Nephrology, State University of New York at Buffalo
Coauthor(s): Kerry C Owens, MD, Consulting Staff, Department of Internal Medicine, Section of Nephrology, Integris Baptist Medical Center of Oklahoma City
Contributor Information and Disclosures

Updated: Sep 4, 2008

Follow-up

Prognosis

  • Close follow-up care is extremely important in any patient with active vasculitis. The therapies for ANCA-associated vasculitides are not proven in large, randomized, controlled trials but are the standard of care according to consensus. The same can be said for the definitions of relapse, response, and treatment failure.
  • The following criteria were established by Nachman et al before they performed a randomized control trial comparing immunosuppression regimens in patients diagnosed with microscopic polyangiitis or isolated pauci-immune rapidly progressive glomerulonephritis only (patients with Wegener granulomatosis were excluded).4
    • Remission
      • Stabilization or improvement of renal function (as measured by serum creatinine value), resolution of hematuria, and resolution of extrarenal manifestations of systemic vasculitis
      • Persistence of proteinuria not considered indicative of persistent disease activity
    • Remission by therapy - Achievement of remission while still receiving immunosuppressive medication or corticosteroids (prednisone dose or equivalent at >7.5 mg/d)
    • Treatment resistance
      • Progressive decline in renal function with the persistence of an active urine sediment
      • Persistence or new appearance of any extrarenal manifestation of vasculitis despite immunosuppressive therapy
    • Relapse (at least one of the following)
      • Rapid rise in serum creatinine concentration, accompanied by an active urine sediment
      • Renal biopsy findings demonstrating active necrosis or crescent formation
      • Hemoptysis, pulmonary hemorrhage, or new or expanding nodules without evidence for infection
      • Active vasculitis of the respiratory or GI tracts as demonstrated by findings from endoscopic biopsy
      • Iritis or uveitis
      • New mononeuritis multiplex
      • Necrotizing vasculitis identified based on findings from biopsy specimen of any tissue

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • A delay in diagnosis may lead to the patient progressing to end-stage renal disease (ESRD).

Special Concerns

 


More on Glomerulonephritis, Rapidly Progressive

Overview: Glomerulonephritis, Rapidly Progressive
Differential Diagnoses & Workup: Glomerulonephritis, Rapidly Progressive
Treatment & Medication: Glomerulonephritis, Rapidly Progressive
Follow-up: Glomerulonephritis, Rapidly Progressive
References
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References

  1. Davies DJ, Moran JE, Niall JF, et al. Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology?. Br Med J (Clin Res Ed). Aug 28-Sep 4 1982;285(6342):606. [Medline].

  2. Hall JB, Wadham BM, Wood CJ, et al. Vasculitis and glomerulonephritis: a subgroup with an antineutrophil cytoplasmic antibody. Aust N Z J Med. Jun 1984;14(3):277-8. [Medline].

  3. Falk RJ, Hogan S, Carey TS, et al. Clinical course of anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and systemic vasculitis. The Glomerular Disease Collaborative Network. Ann Intern Med. Nov 1 1990;113(9):656-63. [Medline].

  4. Nachman PH, Hogan SL, Jennette JC, et al. Treatment response and relapse in antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol. Jan 1996;7(1):33-9. [Medline].

  5. Villa-Forte A, Clark TM, Gomes M, et al. Substitution of methotrexate for cyclophosphamide in Wegener granulomatosis: a 12-year single-practice experience. Medicine (Baltimore). Sep 2007;86(5):269-77. [Medline].

  6. Andrassy K, Kuster S, Waldherr R, et al. Rapidly progressive glomerulonephritis: analysis of prevalence and clinical course. Nephron. 1991;59(2):206-12. [Medline].

  7. Bacani RA, Velasquez F, Kanter A, et al. Rapidly progressive (nonstreptococcal) glomerulonephritis. Ann Intern Med. Sep 1968;69(3):463-85. [Medline].

  8. Couser WG. Rapidly progressive glomerulonephritis: classification, pathogenetic mechanisms, and therapy. Am J Kidney Dis. Jun 1988;11(6):449-64. [Medline].

  9. de Lind van Wijngaarden RA, Hauer HA, Wolterbeek R, et al. Chances of renal recovery for dialysis-dependent ANCA-associated glomerulonephritis. J Am Soc Nephrol. Jul 2007;18(7):2189-97. [Medline].

  10. Hogan SL, Nachman PH, Wilkman AS, et al. Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol. Jan 1996;7(1):23-32. [Medline].

  11. Hotta O, Ishida A, Kimura T, et al. Improvements in treatment strategies for patients with antineutrophil cytoplasmic antibody-associated rapidly progressive glomerulonephritis. Ther Apher Dial. Oct 2006;10(5):390-5. [Medline].

  12. Jayne DR, Gaskin G, Pusey CD, et al. ANCA and predicting relapse in systemic vasculitis. QJM. Feb 1995;88(2):127-33. [Medline].

  13. [Best Evidence] Jayne DR, Gaskin G, Rasmussen N, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol. Jul 2007;18(7):2180-8. [Medline].

  14. Jennette JC. Renal involvement in systemic vasculilits. In: Jennette JC, Olson JL, Schwartz MM, Silva FG, eds. Hepinstall's Pathology of the Kidney. 5th ed. Philadelphia: Lippincott-Raven; 1998:1059-94.

  15. Pusey CD, Rees AJ, Evans DJ, et al. Plasma exchange in focal necrotizing glomerulonephritis without anti-GBM antibodies. Kidney Int. Oct 1991;40(4):757-63. [Medline].

  16. Savige J, Davies D, Falk RJ, et al. Antineutrophil cytoplasmic antibodies and associated diseases: a review of the clinical and laboratory features. Kidney Int. Mar 2000;57(3):846-62. [Medline].

  17. Stilmant MM, Bolton WK, Sturgill BC, et al. Crescentic glomerulonephritis without immune deposits: clinicopathologic features. Kidney Int. Feb 1979;15(2):184-95. [Medline].

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Further Reading

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Keywords

rapidly progressive glomerulonephritis, RPGN, kidney disease, renal disease, kidney disorder, renal disorder, Wegener granulomatosis, WG, Wegener's granulomatosis, Churg-Strauss disease, CS, Churg-Strauss syndrome, CSS, CSD, microscopic polyangiitis, MPA, circulating antineutrophil cytoplasmic antibody associated glomerulonephritis, ANCA disease, ANCA-associated disease, ANCA glomerulonephritis, ANCA-associated vasculitis, fibrinoid necrosis, extensive crescent formation, pauci-immune disease, pauci immune disease, renal-limited necrotizing crescentic glomerulonephritis, NCGN, systemic lupus erythematosus, SLE, inflammatory bowel disease, IBD, sclerosing cholangitis, autoimmune hepatitis, rheumatoid arthritis, RA, Felty syndrome, Felty's syndrome, ANCA-associated vasculitides

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Contributor Information and Disclosures

Author

James W Lohr, MD, Fellowship Program Director, Professor, Department of Internal Medicine, Division of Nephrology, State University of New York at Buffalo
James W Lohr, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Nephrology, and Central Society for Clinical Research
Disclosure: Nothing to disclose.

Coauthor(s)

Kerry C Owens, MD, Consulting Staff, Department of Internal Medicine, Section of Nephrology, Integris Baptist Medical Center of Oklahoma City
Kerry C Owens, MD is a member of the following medical societies: American College of Physicians, American Medical Association, American Society of Nephrology, International Society of Nephrology, Oklahoma State Medical Association, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

F John Gennari, MD, Director, Division of Nephrology, Associate Chair for Academic Affairs, Robert F and Genevieve B Patrick Professor, Department of Medicine, University of Vermont College of Medicine
F John Gennari, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society of Nephrology, and International Society of Nephrology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

George R Aronoff, MD, Director, Professor, Departments of Internal Medicine and Pharmacology, Section of Nephrology, Kidney Disease Program, University of Louisville School of Medicine
George R Aronoff, MD is a member of the following medical societies: American Federation for Medical Research, American Society of Nephrology, Kentucky Medical Association, and National Kidney Foundation
Disclosure: Nothing to disclose.

CME Editor

Rebecca J Schmidt, DO, FACP, FASN, Professor of Medicine, Section Chief, Department of Medicine, Section of Nephrology, West Virginia University School of Medicine
Rebecca J Schmidt, DO, FACP, FASN is a member of the following medical societies: American College of Osteopathic Internists, American College of Physicians, American Medical Association, American Society of Nephrology, International Society of Nephrology, National Kidney Foundation, Renal Physicians Association, and West Virginia State Medical Association
Disclosure: Abbott Grant/research funds Speaking and teaching; Genzyme Honoraria Consulting; Roche Honoraria Consulting

Chief Editor

Vecihi Batuman, MD, FACP, FASN, Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Medicine Service, Southeast Louisiana Veterans Health Care System
Vecihi Batuman, MD, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, and International Society of Nephrology
Disclosure: Nothing to disclose.

 
 
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