From: Clinical features of childhood granulomatosis with polyangiitis (wegener’s granulomatosis)
Paediatric cohorts | Adult cohorts | ||||
---|---|---|---|---|---|
Signs/symptoms* | Current cohort 2010 (N = 56] | Cabral et al., 2009[6] (N = 65] | Koldingsnes, 2000[10] (N = 55] | Mahr, 2008[11] (N = 180] | Stone, 2010[7] (N = 197) |
Female | 38 (68%) | 41 (63%) | 21 (38%) | 99 (50%) | |
Constitutional | 50 (89%) | 120 (61%) | |||
Malaise | 45/55 (82%) | 58 (89%) | 47 (85%) | ||
Fever | 35 (62%) | 35 (54%) | |||
Weight loss | 30/52 (58%) | 28 (43%) | 12 (7%) | ||
Mucosa and skin | 36 (64%) | 23 (35%) | 17 (31%) | ||
Eye | 19 (34%) | 24 (37%) | 21 (38%) | ||
Red eye: conjunctivitis, keratitis, blepharitis | 14/55 (25%) | 6 (9%) | 36 (20%) | ||
Ears, nose, throat | 51 (91%) | 52 (80%) | 44 (80%) | 115 (58%) | |
Oral/nasal ulcerations | 28 (50%) | 6 (9%) | 26 (14%) | ||
Nasal discharge or recurrent epistaxis/crusts/granulomata | 41 (73%) | 42 (65%) | 122 (68%) | ||
Nasal septum perforation/saddle nose/auricular chondritis | 9 (16%) | ||||
Chronic or recurrent sinus nasal inflammation | 34 (61%) | 39 (60%) | 98 (54%) | ||
Chronic or recurrent otitis media/aural discharge | 12/55 (22%) | 9 (14%) | 7 (4%) | ||
Conductive hearing loss | 6/54 (11%) | 7 (11%) | 52 (29%) | ||
Subglottic stenosis/hoarseness/stridor | 11 (20%) | 9 (14%) | 22 (12%) | ||
Respiratory | 44 (79%) | 52 (80%) | 33 (60%) | 104 (53%) | |
Tracheal/endobronchial stenoses, obstruction | 9 (16.1%) | 11 (6%) | 13 (7%) | ||
Haemoptysis, alveolar haemorrhage | 14/55 (25%) | 29 (45%) | 35 (19%) | 50 (25%) | |
Pleuritis | 7 (13%) | 15 (23%) | 16 (9%) | 17 (9%) | |
Pulmonary nodules or cavities | 17 (30%) | 26/62 (42%) | 67 (37%) | 45 (23%) | |
Fixed lung infiltrates | 26/55 (47%) | 14/62 (23%) | |||
Musculoskeletal | 33 (59%) | ||||
Muscle pain/tenderness | 33 (59%) | 37 (57%) | 35 (64%) | ||
Arthritis | 16/55 (29%) | 13 (20%) | |||
Arthralgia | 29 (52%) | ||||
Other | |||||
Central nervous system | 8 (14%) | 16 (25%) | 7 (13%) | 39 (20%) | |
Gastro-intestinal system | 9 (16%) | 27 (42%) | 3 (5%) | ||
Renal | 38 (68%) | 49 (75%) | 42 (76%) | 130 (66%) | |
Laboratory/biopsy | |||||
ELISA MPO-ANCA | 13/50 (26%) | 15 (23%) | 65 (33%) | ||
ELISA PR3-ANCA | 34/51 (67%) | 44 (68%) | 131 (67%) | ||
Haematuria or red blood cells casts (RBC/hpf) | 35 (63%) | 77 (43%) | |||
Glomerulonephritis by renal biopsy | 23/28 (82%) | 34 (94%) |