eMedicine Specialties > Rheumatology > Vasculitis

Wegener Granulomatosis: Follow-up

Author: Patricia J Papadopoulos, MD, Fellow, Department of Rheumatology, Walter Reed Army Medical Center, Washington, DC
Coauthor(s): Robert John O'Brian, MD, Staff Rheumatologist, National Naval Medical Center
Contributor Information and Disclosures

Updated: Aug 4, 2009

Follow-up

Further Outpatient Care

  • Patients with Wegener granulomatosis (WG) should have regularly scheduled follow-up visits with the physician primarily responsible for managing his or her disease. Since recurrences occur frequently, patients should be monitored closely clinically, with radiological studies and laboratory examinations to include renal function, ESR, ANCA levels, and urinalysis.
  • Visits should also focus on untoward effects of therapeutic agents.
  • Infection is a major contributor to morbidity and mortality in WG and often mimics a disease flare or manifests as atypical features caused by treatment-related immunosuppression. Providers should have a low threshold for treating suspicious symptoms with antibiotics.
  • Prophylaxis against Pneumocystis pneumonia is essential while patients are receiving conventional therapy for WG. This can be achieved with TMP-SMX single-strength daily or double-strength formulation three times per week. Dapsone 100 mg daily can be used in sulfa-allergic patients.
  • All patients who require long-term glucocorticoid treatment are at risk for glucocorticoid-induced osteoporosis. Baseline bone mineral density should be evaluated. If the density is normal, patients should take daily calcium and vitamin D supplementation. If diminished at baseline or if long-term glucocorticoid use is anticipated, bisphosphonate therapy should be added.

Deterrence/Prevention

No known deterrents prevent development of WG.

Complications

  • In a longitudinal cohort consisting of 158 patients with WG from the National Institutes of Health, 86% of patients experienced permanent damage caused by their disease.48
  • Permanent damage includes end-stage renal disease, chronic pulmonary dysfunction, hearing loss, destructive sinus disease, saddle nose deformities, perforation of the nasal septum, proptosis, and blindness.48
  • Many patients in the cohort (42%) also experienced permanent treatment-associated morbidity, including hemorrhagic cystitis, osteoporotic fractures, bladder cancer, myelodysplasia, and avascular necrosis.48
  • The extent of renal disease is related to most of the complications.
  • Respiratory problems may result from upper-airway obstruction (eg, subglottic stenosis) or pulmonary involvement (eg, pleural effusion, dyspnea, DAH).

Prognosis

  • The remission rate in WG ranges from 30%-93% depending on the definition of remission and the remission induction therapy used.49
  • With aggressive therapy for active disease, more than 50% of patients with WG recover renal function and are able to become dialysis-independent.50,51
  • Relapse is common in WG. Typically, up to half of patients with WG experience relapse within 5 years.16
    • The rate (18%-40% at 24 mo) and time to first relapse (15-29 mo) varies.49
    • Factors associated with relapse include treatment (<10 g of cyclophosphamide in the first 6 mo and/or maintaining a high dose of prednisone [>20 mg/d] for <2.75 mo), ANCA status at diagnosis, and target organ involvement (lung involvement, cardiac involvement, renal involvement, chronic nasal carriage of S aureus).49
    • Rising PR3-ANCA (C-ANCA) titers may correlate with disease activity in approximately two-thirds of patients. However, the relationship is unreliable; thus, negative PR3-ANCA results do not necessarily exclude the possibility of relapse.16 As significant adverse effects are associated with immunosuppressive therapy, especially cyclophosphamide, ANCA persistence or reappearance should be used as a warning signal rather than an indication to escalate therapy.
  • Poorer survival is associated with older age, target organ involvement, and target organ damage. Renal involvement has been consistently shown to confer a poorer prognosis. An absence of renal involvement is associated with a 100% 5-year survival rate compared to approximately 70% in individuals with renal disease.9
  • The overall 10-year survival rate ranges from 75%-88%.9

Patient Education

  • Patients with WG and their families must be educated on the serious nature of this disease.
  • Potential risks and adverse effects of immunosuppressive medications should be detailed.

Miscellaneous

Medicolegal Pitfalls

  • Clinician awareness is important to prevent delayed or missed diagnosis. Recurrent respiratory infection in an adult may be an indicator of Wegener granulomatosis (WG).
  • Whether tissue diagnosis is always required for WG remains controversial. As the therapy for severe WG is not benign, tissue diagnosis is recommended if a biopsy site is available, given that the patient understands the risks of the procedure.
  • Treatment should be tailored to appropriately treat WG manifestations while minimizing long-term toxicities to the patient.

Special Concerns

  • P jiroveci pneumonia (formerly known as Pneumocystis carinii pneumonia)
    • Pneumocystis pneumonia has an annual incidence of 1% but is a potentially deadly complication of immunosuppressive therapy in patients with WG, especially with prolonged lymphocytopenia.
    • Prophylaxis with TMP-SMX (800/160 mg 3 times weekly or 400/80 mg daily) increases life expectancy and reduces medical costs in patients receiving immunosuppressive therapy. Dapsone 100 mg daily is an alternative in sulfa-allergic patients.
 
Acknowledgments

  • The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Sat Sharma, MD, to the development and writing of this article.
  • The views expressed in this article are those of the authors and do not reflect the official policy of the Department of the Army, Department of the Navy, Department of Defense, or the U.S. Government.



More on Wegener Granulomatosis

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

Wegener granulomatosis, WG, Wegener's granulomatosis, Wegener's disease, Wegener disease, systemic vasculitis, systemic necrotizing angiitis, necrotizing glomerulonephritis, necrotizing granulomatous inflammation of the respiratory tract, rapidly progressive glomerulonephritis, ANCA-associated vasculitis, ANCA, AAV, PR3-ANCA, alveolar capillaritis, diffuse alveolar hemorrhage, pauci-immune complex, glomerulonephritis

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Contributor Information and Disclosures

Author

Patricia J Papadopoulos, MD, Fellow, Department of Rheumatology, Walter Reed Army Medical Center, Washington, DC
Patricia J Papadopoulos, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and American College of Rheumatology
Disclosure: Nothing to disclose.

Coauthor(s)

Robert John O'Brian, MD, Staff Rheumatologist, National Naval Medical Center
Robert John O'Brian, MD is a member of the following medical societies: American College of Rheumatology
Disclosure: Nothing to disclose.

Medical Editor

Robert E Wolf, MD, PhD, Professor Emeritus, Department of Medicine, Louisiana State University Health Sciences Center at Shreveport; Chief, Rheumatology Section, Medical Service, Overton Brooks Veterans Administration Medical Center of Shreveport
Robert E Wolf, MD, PhD is a member of the following medical societies: American College of Rheumatology, Arthritis Foundation, and Society for Leukocyte Biology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Elliot Goldberg, MD, Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine
Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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