eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Wegener Granulomatosis: Follow-up

Author: Rudolph P Valentini, MD, Director of Dialysis Services, Associate Professor, Department of Pediatrics, Division of Pediatric Nephrology, Wayne State University; Vice Chief of Staff, Children's Hospital of Michigan
Coauthor(s): Debbie S Toder, MD, Director of Cystic Fibrosis Center, Department of Pediatrics, Division of Pulmonary Medicine, Assistant Professor, Wayne State University and Children's Hospital of Michigan
Contributor Information and Disclosures

Updated: Mar 5, 2009

Follow-up

Further Inpatient Care

  • Consider follow-up renal biopsy at time of transition off of cyclophosphamide (CYP) in patients with Wegener granulomatosis (WG).

Further Outpatient Care

  • Perform weekly CBC counts for the first 1-2 months in patients on oral CYP.
  • Measure CBC counts 10-14 days after IV CYP to assess nadir WBC and decide on next CYP IV dosage.
  • Urinalysis, serum creatinine, CBC count, urine protein and creatinine ratios, and erythrocyte sedimentation rates are checked monthly for the first several months.
  • More time is gradually allowed between follow-up visits if the patient is stable.
  • Antineutrophil cytoplasmic autoantibody (ANCA) titers are screened every 3-6 months or more often if clinical relapse is apparent.

Inpatient & Outpatient Medications

Complications

  • Subglottic stenosis
  • Nasal deformity
  • Permanent hearing loss
  • Visual loss
  • Chronic renal insufficiency
  • Steroid adverse effects (eg, weight gain, hypertension, cataracts, avascular necrosis of the hip)
  • Serious infections
  • Increased risk of infertility
  • Increased risk of malignancy (especially bladder cancer and lymphoma)
  • Death

Prognosis

  • Remission rates of 87% are reported by Rottem et al (1993).10 Relapses occurred in 53% of patients.
  • Earlier disease recognition and more aggressive treatment regimens will hopefully improve both remission and relapse rates.
  • Permanent morbidity from disease or its treatment occurs in 87% of patients. Newer regimens should improve outcome.
  • Chronic renal insufficiency occurs in 35% of patients.
  • Nasal deformity occurs in 48% of patients, and fixed subglottic stenosis occurs in 35% of patients.
  • Of children with Wegener granulomatosis, 2 of 23 died 2.2 and 15 years after disease onset.

Patient Education

  • Educate patients to contact their physicians for symptoms of sinusitis, cough, fever, swelling, or gross hematuria. These could be symptoms of either a disease relapse or an infection in an immunocompromised host.
  • For excellent patient education resources, visit eMedicine's Procedures Center. Also, see eMedicine's patient education article Bronchoscopy.

Miscellaneous

Medicolegal Pitfalls

  • Failure to make a diagnosis

Special Concerns

  • Females of childbearing age are discouraged from engaging in sexual activity. Many are placed on oral contraceptives with close monitoring of their blood pressure.
 


More on Wegener Granulomatosis

Overview: Wegener Granulomatosis
Differential Diagnoses & Workup: Wegener Granulomatosis
Treatment & Medication: Wegener Granulomatosis
Follow-up: Wegener Granulomatosis
Multimedia: Wegener Granulomatosis
References
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References

  1. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. Feb 1994;37(2):187-92. [Medline].

  2. Breda L, Nozzi M, De Sanctis S, Chiarelli F. Laboratory Tests in the Diagnosis and Follow-Up of Pediatric Rheumatic Diseases: An Update. Semin Arthritis Rheum. Feb 24 2009;[Medline].

  3. Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. Jan 1983;98(1):76-85. [Medline].

  4. Hogan SL, Satterly KK, Dooley MA, Nachman PH, Jennette JC, Falk RJ. Silica exposure in anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and lupus nephritis. J Am Soc Nephrol. Jan 2001;12(1):134-42. [Medline].

  5. Schlieben DJ, Korbet SM, Kimura RE, Schwartz MM, Lewis EJ. Pulmonary-renal syndrome in a newborn with placental transmission of ANCAs. Am J Kidney Dis. Apr 2005;45(4):758-61. [Medline].

  6. Cotch MF, Hoffman GS, Yerg DE, Kaufman GI, Targonski P, Kaslow RA. The epidemiology of Wegener's granulomatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources. Arthritis Rheum. Jan 1996;39(1):87-92. [Medline].

  7. US Renal Data System. Annual Data Report. In: Atlas of End-Stage Renal Disease in the United States. Vol 2. Bethesda, MD: National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases; 2008.

  8. North American Pediatric Renal Trials and Collaborative Studies 2007. Annual Report. Dialysis Access Data. NAPRTCS. Available at https://web.emmes.com/study/ped/. Accessed February 12, 2009.

  9. Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. Mar 15 1992;116(6):488-98. [Medline].

  10. Rottem M, Fauci AS, Hallahan CW, et al. Wegener granulomatosis in children and adolescents: clinical presentation and outcome. J Pediatr. Jan 1993;122(1):26-31. [Medline].

  11. Ellis EN, Wood EG, Berry P. Spectrum of disease associated with anti-neutrophil cytoplasmic autoantibodies in pediatric patients. J Pediatr. Jan 1995;126(1):40-3. [Medline].

  12. Valentini RP, Smoyer WE, Sedman AB, Kershaw DB, Gregory MJ, Bunchman TE. Outcome of antineutrophil cytoplasmic autoantibodies-positive glomerulonephritis and vasculitis in children: a single-center experience. J Pediatr. Feb 1998;132(2):325-8. [Medline].

  13. Hattori M, Kurayama H, Koitabashi Y. Antineutrophil cytoplasmic autoantibody-associated glomerulonephritis in children. J Am Soc Nephrol. Jul 2001;12(7):1493-500. [Medline].

  14. Luqmani RA, Bacon PA, Moots RJ, et al. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM. Nov 1994;87(11):671-8. [Medline].

  15. Sneller MC. Rituximab and Wegener's granulomatosis: are B cells a target in vasculitis treatment?. Arthritis Rheum. Jan 2005;52(1):1-5. [Medline].

  16. [Best Evidence] Jayne DR, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L. 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].

  17. Keogh KA, Ytterberg SR, Fervenza FC, Carlson KA, Schroeder DR, Specks U. Rituximab for refractory Wegener's granulomatosis: report of a prospective, open-label pilot trial. Am J Respir Crit Care Med. Jan 15 2006;173(2):180-7. [Medline].

  18. Nowack R, Gobel U, Klooker P, Hergesell O, Andrassy K, van der Woude FJ. Mycophenolate mofetil for maintenance therapy of Wegener's granulomatosis and microscopic polyangiitis: a pilot study in 11 patients with renal involvement. J Am Soc Nephrol. Sep 1999;10(9):1965-71. [Medline].

  19. Joy MS, Hogan SL, Jennette JC, Falk RJ, Nachman PH. A pilot study using mycophenolate mofetil in relapsing or resistant ANCA small vessel vasculitis. Nephrol Dial Transplant. Dec 2005;20(12):2725-32. [Medline].

  20. Stegeman CA, Tervaert JW, de Jong PE, Kallenberg CG. Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener's granulomatosis. Dutch Co-Trimoxazole Wegener Study Group. N Engl J Med. Jul 4 1996;335(1):16-20. [Medline].

  21. Wegener's Granulomatosis Etanercept Trial (WGET) Research Group. Etanercept plus standard therapy for Wegener's granulomatosis. N Engl J Med. Jan 27 2005;352(4):351-61. [Medline].

  22. Patel AM, Lehman TJ. Rituximab for severe refractory pediatric Wegener granulomatosis. J Clin Rheumatol. Oct 2008;14(5):278-80. [Medline].

  23. Gottlieb BS, Miller LC, Ilowite NT. Methotrexate treatment of Wegener granulomatosis in children. J Pediatr. Oct 1996;129(4):604-7. [Medline].

  24. Hoffman GS, Leavitt RY, Kerr GS, Fauci AS. The treatment of Wegener's granulomatosis with glucocorticoids and methotrexate. Arthritis Rheum. Nov 1992;35(11):1322-9. [Medline].

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

  26. Jagiello P, Aries P, Arning L, et al. The PTPN22 620W allele is a risk factor for Wegener's granulomatosis. Arthritis Rheum. Dec 2005;52(12):4039-43. [Medline].

  27. Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. Jul 3 2003;349(1):36-44. [Medline].

  28. Kist-van Holthe JE, Ho PL, Stablein D, Harmon WE, Baum MA. Outcome of renal transplantation for Wilms' tumor and Denys-Drash syndrome: a report of the North American Pediatric Renal Transplant Cooperative Study. Pediatr Transplant. Jun 2005;9(3):305-10. [Medline].

  29. Lamprecht P, Voswinkel J, Lilienthal T, et al. Effectiveness of TNF-alpha blockade with infliximab in refractory Wegener's granulomatosis. Rheumatology (Oxford). Nov 2002;41(11):1303-7. [Medline].

  30. Lynch JP 3rd, Hoffman GS. Wegener's granulomatosis: controversies and current concepts. Compr Ther. Sep 1998;24(9):421-40. [Medline].

  31. Pediatric end-stage renal disease. USRDS. United States Renal Data System. Am J Kidney Dis. Aug 1997;30(2 Suppl 1):S128-44. [Medline].

  32. Sneller MC, Fauci AS. Pathogenesis of vasculitis syndromes. Med Clin North Am. Jan 1997;81(1):221-42. [Medline].

  33. Taketomo CK, Hodding JH, Kraus DM. Pediatric Dosage Handbook. 4th ed. Hudson, Ohio: Lexi-comp Inc; 1997.

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Further Reading

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Keywords

Wegener granulomatosis, WG, Wegener's granulomatosis, ANCA disease, antineutrophil cytoplasmic autoantibodies disease, ANCA-associated vasculitis, ANCA-associated glomerulonephritis, ANCA-associated GN, antineutrophil cytoplasmic autoantibodies–associated vasculitis, antineutrophil cytoplasmic autoantibodies–associated glomerulonephritis, antineutrophil cytoplasmic autoantibodies–associated GN, pulmonary renal syndrome, necrotizing granulomatous lesions of the upper or lower respiratory tract, generalized necrotizing vasculitis involving both arteries and veins, focal necrotizing glomerulonephritis, microscopic polyangiitis, Churg-Strauss syndrome, microscopic polyangiitis, MPA, end-stage renal disease, ESRD, sinusitis, otitis media, subglottic stenosis, pulmonary infiltrates, pulmonary nodules, hemoptysis, hematuria, proteinuria, arthralgia, arthritis, rhinorrhea, epistaxis, pharyngitis, congestive heart failure, CHF

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Contributor Information and Disclosures

Author

Rudolph P Valentini, MD, Director of Dialysis Services, Associate Professor, Department of Pediatrics, Division of Pediatric Nephrology, Wayne State University; Vice Chief of Staff, Children's Hospital of Michigan
Rudolph P Valentini, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and American Society of Pediatric Nephrology
Disclosure: Nothing to disclose.

Coauthor(s)

Debbie S Toder, MD, Director of Cystic Fibrosis Center, Department of Pediatrics, Division of Pulmonary Medicine, Assistant Professor, Wayne State University and Children's Hospital of Michigan
Debbie S Toder, MD is a member of the following medical societies: American Academy of Pediatrics and American Thoracic Society
Disclosure: Nothing to disclose.

Medical Editor

Girish D Sharma, MD, Associate Professor, Department of Pediatrics, Rush University Medical Center, Rush Children's Hospital; Director of Pediatric Pulmonary Section and Rush Cystic Fibrosis Center
Girish D Sharma, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society, and Royal College of Physicians of Ireland
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Heidi Connolly, MD, Associate Professor of Pediatrics and Psychiatry, University of Rochester; Director, Pediatric Sleep Medicine Services, Strong Sleep Disorders Center
Heidi Connolly, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

CME Editor

Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital
Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians
Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Chief Editor

Michael R Bye, MD, Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center
Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society
Disclosure: Merck Honoraria Speaking and teaching

 
 
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