Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood

Abstract

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

Diagnostic approach and current treatment options in childhood vasculitis

“…Less commonly, findings of musculoskeletal, gastrointestinal, opthalmic, cutaneous and neurological involvement are observed. Cardiac involvement has not been found in any patient (39). In the study conducted by Bohm et al (40), the otorhinolaryngological findings were reported to include septum perforation, saddle nose, auricular chondritis, subglottic stenosis, coarse voice and stridor.…”

“…Characteristic findings related with involvement of these organs have been identified in studies conducted with children (39,40). The most common findings at the time of onset of the disease include fatigue, wieght loss and fever and otorhinolaryngological, respiratory system and renal findings are the most commonly observed findings.…”

Management of Vasculitic Glomerulonephritis

“…No clinical trials have been conducted in pediatric AAV, and almost all knowledge about optimal treatment and outcomes has been extrapolated from adult studies or collected from small case series. Pediatric patients are often given remission-induction treatment with corticosteroids and cyclophosphamide [33]. Rituximab is recommended in refractory or relapsing disease [33].…”

“…Pediatric patients are often given remission-induction treatment with corticosteroids and cyclophosphamide [33]. Rituximab is recommended in refractory or relapsing disease [33]. Methotrexate, mycophenolate mofetil, or azathioprine is often used in maintenance therapy [33,34].…”

“…Rituximab is recommended in refractory or relapsing disease [33]. Methotrexate, mycophenolate mofetil, or azathioprine is often used in maintenance therapy [33,34]. Drug dosages need to be adjusted by the child's weight and renal impairment if present.…”