Clinical features of childhood granulomatosis with polyangiitis (wegener’s granulomatosis)

Abstract

BackgroundGranulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis (WG), belongs to the group of ANCA-associated necrotizing vasculitides. This study describes the clinical picture of the disease in a large cohort of GPA paediatric patients.Children with age at diagnosis ≤ 18 years, fulfilling the EULAR/PRINTO/PRES GPA/WG classification criteria were extracted from the PRINTO vasculitis database. The clinical signs/symptoms and laboratory features were analysed before or at the time of diagnosis and at least 3 months thereafter and compared with other paediatric and adult case series (>50 patients) derived from the literature.FindingsThe 56 children with GPA/WG were predominantly females (68%) and Caucasians (82%) with a median age at disease onset of 11.7 years, and a median delay in diagnosis of 4.2 months. The most frequent organ systems involved before/at the time of diagnosis were ears, nose, throat (91%), constitutional (malaise, fever, weight loss) (89%), respiratory (79%), mucosa and skin (64%), musculoskeletal (59%), and eye (35%), 67% were ANCA-PR3 positive, while haematuria/proteinuria was present in > 50% of the children. In adult series, the frequency of female involvement ranged from 29% to 50% with lower frequencies of constitutional (fever, weight loss), ears, nose, throat (oral/nasal ulceration, otitis/aural discharge), respiratory (tracheal/endobronchial stenosis/obstruction), laboratory involvement and higher frequency of conductive hearing loss than in this paediatric series.ConclusionsPaediatric patients compared to adults with GPA/WG have similar pattern of clinical manifestations but different frequencies of organ involvement.

Clinical presentation and outcome of pediatric ANCA-associated glomerulonephritis

“…Similar to other childhood chronic diseases, children with AAV differ from adults in that they have a longer anticipated life span over which their disease must be managed. The peak age of onset of AAV in the pediatric population is during late childhood and adolescence, a critical time for growth as well as emotional, physical and reproductive development [4][5][6].…”

Bilateral Facial Paralysis and Otitis Media as the First Presentations of Wegener’s Granulomatosis: A Case Report

“…3 Wegener's characteristic features are involvement of the granulomatous vasculitis in upper and lower airways with glomerulonephritis. Also, diffusive vasculitis can be seen in various degrees in smaller veins and arteries.…”

“…2 In rare cases (such as in the current patient), it develops with unusual symptoms such as hearing loss and bilateral involvement of the facial nerve at an unusual age. 3 Thus, early diagnosis with rapid and wise treatment accompanied by rule out of any important differential.…”

“…However, it can also engage other organs, such as the eyes, ears, skin, jaws, and joints, and it spreads throughout the days and months. 3 In 90% of cases, the disease begins with respiratory involvement and is often controlled by implementing proprietary treatment protocols. Delayed treatment and control of the inflammatory processes endangers life.…”

Estenosis subglótica como manifestación inicial de granulomatosis de Wegener en una adolescente. Caso clínico

“…La incidencia se estima en 0,1 cada 100 000 niños. 2 La ES se presenta en el 8%-23% de los pacientes con GW, principalmente, en menores de 20 años y en el sexo femenino. [2][3][4][5] Puede manifestarse en el debut o en estadios avanzados de la enfermedad.…”