Pediatric Wegener Granulomatosis Follow-up

  • Author: Rudolph P Valentini, MD; Chief Editor: Michael R Bye, MD   more...
 
Updated: Apr 20, 2011
 

Further Inpatient Care

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

  • See Treatment and Medication.
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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
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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.
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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.
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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.

Specialty Editor Board

Girish D Sharma, MD  Associate Professor of Pediatrics, Rush Medical College; Director, Section of Pediatric Pulmonology and Rush Cystic Fibrosis Center, Rush University Medical 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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

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.

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: Nothing to disclose.

References

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Shown is a chest radiograph of an 11-year-old girl who presented with an upper respiratory tract infection, myalgias, and arthralgias for 1 month followed by an abrupt presentation with pallor, hemoptysis, and hypertension. Her bilateral fluffy infiltrates are suggestive of a pulmonary hemorrhage. She had an antineutrophil cytoplasmic autoantibodies (ANCA)–positive pauci-immune necrotizing and crescentic glomerulonephritis (GN) associated with her pulmonary hemorrhage. Supportive therapy consisted of mechanical ventilation and hemodialysis along with immunosuppressive therapy. Her anti–glomerular basement membrane antibody test result was negative. Nearly 2 years later, she had a serum creatinine of 0.7 mg/dL and no residual pulmonary disease.
An 11-year-old girl presented with an upper respiratory tract infection, myalgias, and arthralgias for 1 month followed by an abrupt presentation with pallor, hemoptysis, and hypertension. She had an antineutrophil cytoplasmic autoantibodies (ANCA)–positive pauci-immune necrotizing and crescentic glomerulonephritis (GN) associated with her pulmonary hemorrhage. A follow-up chest radiograph obtained several days later shows a complete resolution of her pulmonary infiltrates. This rapid resolution is more consistent with hemorrhage than with pneumonia.
A renal biopsy specimen from a 13-year-old girl with antineutrophil cytoplasmic antibody (cANCA)–positive pulmonary renal syndrome. Seven weeks after presenting with sinusitis, she presented with an acute abdomen, pulmonary hemorrhage, and acute renal failure (creatinine 4.9 mg/dL). This biopsy specimen shows a necrotizing and crescentic glomerulonephritis (Silver stain).
 
 
 
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