Clinical features in 17 paediatric patients with Wegener granulomatosis

Abstract

The aim of this report was to describe childhood patients with Wegener granulomatosis (WG) from one centre, to analyse the variety of clinical manifestations seen and compare the data with other published paediatric and adult series. The records of 17 patients with WG who were under the care of Great Ormond Street Hospital for Children (GOSH) from 1981 to 1998 were reviewed. We analysed presenting features before admittance to GOSH and the clinical signs observed whilst the children were under the care of the hospital. Of 17 patients, 13 were females and there was a male/female ratio of 1:3.25. Among the patients there were 2 sisters. The age of the patients at disease onset varied from 2 weeks to 14 years. The median/mean age was 6/6.3 years. American College of Rheumatology criteria for diagnosing WG were fulfilled in 11 of 17 patients. The frequency of different system involvement was: respiratory 87%, kidneys 53%, sinuses 35%, joints 53%, eyes 53%, nervous system 12%, skin 53%. cANCA was positive in 10 patients (59%), but pANCA was negative in all measured sera. Kidneys were involved in 2 of 8 patients (25%) with the disease onset from 0 to 5 years and in 7 of 9 patients (78%) with the disease onset from 6 to 14 years ( P<0.05). cANCA was positive in 7 of 9 patients with kidney disease (78%) and in 2 of 8 patients (25%) without kidney involvement ( P<0.05). Colchicine as a supplement to prednisolone and cytotoxic/immunosuppressant drugs was used effectively in 5 patients.

Airway manifestations in childhood granulomatosis with polyangiitis (Wegener's)

“…Due to our identity as a quaternary care center, the prevalence of airway disease may be overestimated. Despite this possible referral bias, our findings are similar to those prior published data (4,6,8,10). Additionally, our cohort is small compared to adult studies on GPA, but this is a relatively large sample given the rarity of this disease in pediatric patients and our short study duration (7 years) (4 -8).…”

“…GPA involves the respiratory epithelium, extending from the nasal mucosa to the bronchi; therefore, involvement of both the upper and lower airways is common (4,6,8,10). This inflammatory process leads to airway edema, erythema, ulceration/hemorrhage, stenosis, nodules, and polyps.…”

“…Details regarding airway manifestations in childhood GPA have been limited in prior studies (4 -9). Differences between adult and childhood GPA include statistically higher prevalences of nasal deformity and subglottic stenosis (SGS) (4,6,8,10,11). Additionally, it has been proposed that GPA may encompass 2 diseases: limited and generalized (12).…”

Long-term remission in pediatric Wegener granulomatosis following allo-SCT after reduced-intensity conditioning

“…In children, a survival rate between 85 and 100% is reported. [1][2][3] Marmont and VanBekkum were the first to discuss the potential role of allo-SCT for refractory autoimmune diseases. 4 Here, we report the successful treatment of a pediatric WG by allo-SCT following reduced-intensity conditioning.…”

Vasculitis: do we know more to classify better?

“…Thus, any sign of ANCA positivity was added to the group of criteria for GPA (WG) classification. The presence of subglottic, tracheal or endobronchial stenosis was also considered to be a new criterion, since it is especially common in pediatric GPA (WG) patients [43,44]. Furthermore, the evolving technology in imaging using Computed tomography (CT) scan results was acknowledged in the definition of pulmonary involvement.…”