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Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood
David A Cabral1, América G Uribe, Susanne Benseler
1Division of Rheumatology, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. dcabral@cw.bc.ca
Insights
The European League Against Rheumatism/Pediatric Rheumatology European Society (EULAR/PRES) criteria slightly improved diagnosis of Wegener's granulomatosis (WG) in children with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs). Delays in diagnosing childhood WG and varied initial treatments were noted.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Vasculitis Diagnosis
Background:
- Wegener's granulomatosis (WG), now known as Granulomatosis with Polyangiitis, is a rare autoimmune disease affecting children.
- Accurate classification criteria are crucial for timely diagnosis and effective management of pediatric WG and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs).
- Existing criteria, such as those from the American College of Rheumatology (ACR), may require comparison with newer international standards like EULAR/PRES.
Purpose of the Study:
- To compare the diagnostic performance of ACR and EULAR/PRES classification criteria for WG in a pediatric cohort with AAVs.
- To describe the time to diagnosis, clinical presentation, and initial treatment strategies for WG in children.
- To evaluate the sensitivity and specificity of both sets of criteria within the spectrum of childhood AAVs.
Main Methods:
- A cohort of 117 children diagnosed with AAVs since 2004 was retrospectively analyzed.
- Site rheumatologists' diagnoses (MD diagnosis) served as the reference standard.
- Sensitivity and specificity of ACR and EULAR/PRES WG criteria were calculated; descriptive analyses focused on ACR-classified WG patients.
Main Results:
- The EULAR/PRES criteria demonstrated slightly higher sensitivity (73.6%) and specificity (73.2%) for WG compared to ACR criteria (68.4% and 68.3%, respectively).
- Two additional children with WG were identified using EULAR/PRES criteria.
- For ACR-classified WG patients (n=65), the median age at diagnosis was 14.2 years, with a median diagnostic delay of 2.7 months. Common features included constitutional, pulmonary, ENT, and renal involvement. Initial treatment predominantly involved corticosteroids and cyclophosphamide.
Conclusions:
- The EULAR/PRES criteria offer a minimal but notable improvement in diagnostic accuracy for WG within a select group of pediatric AAVs.
- Diagnostic delays in childhood WG highlight the need for better characterization and awareness of the disease in this age group.
- Significant variability exists in initial therapeutic approaches for pediatric WG across different treatment centers.
Objective:
To compare the criteria for Wegener's granulomatosis (WG) of the American College of Rheumatology (ACR) with those of the European League Against Rheumatism/Pediatric Rheumatology European Society (EULAR/PRES) in a cohort of children with WG and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs), and to describe the interval to diagnosis, presenting features, and initial treatment for WG.
Methods:
Eligible patients had been diagnosed by site rheumatologists (termed the "MD diagnosis") since 2004. This diagnosis was used as a reference standard for sensitivity and specificity testing of the 2 WG classification criteria. Descriptive analyses were confined to ACR-classified WG patients.
Results:
MD diagnoses of 117 patients (82 of whom were female) were WG (n = 76), microscopic polyangiitis (n = 17), ANCA-positive pauci-immune glomerulonephritis (n = 5), Churg-Strauss syndrome (n = 2), and unclassified vasculitis (n = 17). The sensitivities of the ACR and EULAR/PRES classification criteria for WG among the spectrum of AAVs were 68.4% and 73.6%, respectively, and the specificities were 68.3% and 73.2%, respectively. Two more children were identified as having WG by the EULAR/PRES criteria than by the ACR criteria. For the 65 ACR-classified WG patients, the median age at diagnosis was 14.2 years (range 4-17 years), and the median interval from symptom onset to diagnosis was 2.7 months (range 0-49 months). The most frequent presenting features by organ system were constitutional (89.2%), pulmonary (80.0%), ear, nose, and throat (80.0%), and renal (75.4%). Fifty-four patients (83.1%) commenced treatment with the combination of corticosteroids and cyclophosphamide, with widely varying regimens; the remainder received methotrexate alone (n = 1), corticosteroids alone (n = 4), or a combination (n = 6).
Conclusion:
The EULAR/PRES criteria minimally improved diagnostic sensitivity and specificity for WG among a narrow spectrum of children with AAVs. Diagnostic delays may result from poor characterization of childhood WG. Initial therapy varied considerably among participating centers.
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