Wegener granulomatosis in pediatric patients

Hall, Sue; Miller, Laurie C.; Duggan, Eileen; Mauer, S. Michael; Beatty, Eugene C.; Hellerstein, Stanley

doi:10.1016/s0022-3476(85)80346-2

Cited by 89 publications

(29 citation statements)

References 14 publications

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“…The reason for the high prevalence of generalized disease at diagnosis in our patients is unclear but similar findings have been reported previously. All 6 patients under 18 years of age in the study by Stegmayr et al (12) and all 4 in the report by Hall et al (10) had renal involvement at presentation. Loss of renal function in our patients was common: at last followup, 40% had sustained elevation of serum creatinine and 12% had end-stage renal failure, figures comparable with those in the study by Rottem et al (35% and 9%, respectively) (4).…”

Section: Discussionmentioning

confidence: 76%

Clinical features and outcome of pediatric Wegener's granulomatosis

Akikusa

Schneider

Harvey

et al. 2007

Arthritis & Rheumatism

167

153

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Objective. Wegener's granulomatosis (WG) is a predominantly small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs). There are few reports describing its clinical features and outcome in children. We report on the experience at a single tertiary referral center over 21 years. Methods. We conducted a retrospective chart review of all patients diagnosed with WG at The Hospital for Sick Children between 1984 and 2005. Results. Twenty-five patients were identified. Median age at diagnosis and median followup were 14.5 years and 32.7 months, respectively. Male-to-female ratio was 1:4. Median duration of symptoms before diagnosis was 2 months. Of 22 patients, 21 were ANCA positive during their disease course (classic ANCA 78.9%). Constitutional symptoms were the most common clinical feature at presentation (24 of 25). Glomerulonephritis was present in 22 patients at presentation. Only 1 of 11 patients who presented with or developed renal impairment had normalization of serum creatinine. Upper airway involvement occurred in 21 patients at presentation and 24 over followup; only 1 had subglottic stenosis. Twenty patients had initial pulmonary involvement, most commonly nodules (44%) and pulmonary hemorrhage (44%). Five patients required ventilation for pulmonary hemorrhage. Four patients (16%) had venous thrombotic events (VTEs). Treatment included prednisone (100%), cyclophosphamide (76%), azathioprine (40%), and methotrexate (32%). Conclusion. Pediatric WG typically presents in adolescence and has a female predominance. Glomerulonephritis and pulmonary disease are common at diagnosis and frequently present as a pulmonary-renal syndrome. Loss of renal function is common and rarely completely reversible. As in adults, children with WG are at risk of VTEs.

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Section: Discussionmentioning

confidence: 76%

Clinical features and outcome of pediatric Wegener's granulomatosis

Akikusa

Schneider

Harvey

et al. 2007

Arthritis & Rheumatism

167

153

View full text Add to dashboard Cite

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“…This case showed a normal ANA level, indicating a less than 0.14% chance of having SLE 6 and his complement levels were normal. Even though Wegener's granulomatosis is so difficult to differentiate from HSP during early stages of disease 7 , it could be excluded based on the normal ANCA level and the presence of renal Ig A deposits.…”

Section: Discussionmentioning

confidence: 99%

A Case of Pulmonary Hemorrhage Associated with Henoch-Schönlein Purpura

Sim

Choi

et al. 2009

Tuberc Respir Dis

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Henoch-Schönlein purpura (HSP) is an immunologically mediated systemic vasculitis of small blood vessels that primarily involves the skin, gastrointestinal tract, joints and kidneys. HSP is a common vasculitic syndrome in children who, in most cases, achieve complete recovery. Pulmonary hemorrhage is a very rare manifestation of HSP. The authors present a case of a 46-year-old male presenting with pulmonary hemorrhage and renal involvement and the diagnosis of HSP. The patient responded to prednisolone therapy.

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“…WG is rare in childhood, 8,9 and orbital involvement has only been reported twice previously at the ages of 7 and 9 years. 10,12 To our knowledge, this is the youngest case in the literature and demonstrates that WG should be considered in the differential diagnosis of orbital disease even in very young children.…”

Section: Discussionmentioning

confidence: 99%

“…7 The disease typically manifests in the fourth and fifth decades, although its occurrence at ages ranging from 7 to 75 years has been reported. [8][9][10] We report the case of a 5-year-old girl presenting with unilateral upper-lid oedema, conjunctival injection, and restriction of eye movements, who was subsequently diagnosed as having WG. To our knowledge, this is the youngest reported case of WG in the literature.…”

Section: Sir Wegener's Granulomatosis Of the Orbit In A 5-year-old Cmentioning

confidence: 99%