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Table 1 Frequency of signs/symptoms (number of patients/%) at the time of diagnosis: comparison with published series

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%)

   
  1. Childhood ANCA, Cytoplasmic Anti-Neutrophil Cytoplasmic Antibodies; MPO-ANCA, myeloperoxidase-specific Anti-Neutrophil Cytoplasmic Antibodies; PR3-ANCA, proteinase 3-specific Anti-Neutrophil Cytoplasmic Antibodies; RBC/hpf, red blood cells per high power field.
  2. Blank cells refers to data not available in the related publication.
  3. *Blank fields refers to not available data.