Table 3

Baseline signs/symptoms, individually or in combination, for the 1124 patients considered in the final analysis (see also figure 1)

Signs/symptomsHSP (N=827)c-PAN (N=150)c-WG (N=60)c-TA (N=87)p Values
General
 Malaise255 (31)89 (59)49 (82)61 (70)<0.0001
 Fever238 (29)112 (75)37 (62)46 (53)<0.0001
Mucosa and skin
 Oral/nasal ulceration5 (0.6)8 (5)29 (48)2 (2)<0.0001
 Purpura (palpable) with lower limb predominance739 (89)23 (15)9 (15)0 (0)<0.0001
 Purpura (palpable) diffuse163 (20)49 (33)5 (8)0 (0)<0.0001
Purpura lower limb predominance/diffuse combined827 (100)68 (45)14 (23)0 (0)<0.0001
 Livedo reticularis8 (1)51 (34)8 (13)2 (2)<0.0001
 Skin nodules9 (1)84 (56)9 (15)2 (2)<0.0001
Livedo/nodules combined17 (2)98 (65)15 (25)4 (5)<0.0001
 Superficial skin infarctions19 (2)19 (13)4 (7)2 (2)<0.0001
 Deep skin infarctions0 (0)16 (11)5 (8)1 (1)<0.0001
Livedo/nodules/skin infarction combined30 (3.6)104 (69)18 (30)7 (8)<0.0001
Upper airway involvement
 Nasal discharge or recurrent epistaxis/crusts/granulomata5 (0.6)3 (2)42 (70)1 (1)<0.0001
 Nasal septum perforation/saddle nose/auricular chondritis0 (0)0 (0)9 (15)0 (0)<0.0001
 Chronic or recurrent sinus nasal inflammation3 (0.4)1 (0.7)35 (58)2 (2)<0.0001
Nasal discharge/perforation/sinus inflammation combined7 (0.8)3 (2)50 (83)3 (3)<0.0001
Laryngo-tracheo-bronchial involvement
 Subglottic stenoses/hoarsness/stridor0 (0)1 (0.7)11 (18)0 (0)<0.0001
 Tracheal/endobronchial stenoses, obstruction1 (0.1)0 (0)9 (15)0 (0)<0.0001
Subglottic/tracheal/bronchial stenoses combined1 (0.1)1 (0.7)13 (22)0 (0)<0.0001
Cardiovascular
 Bruits over aorta and/or its major branches0 (0)1 (0.7)(0)50 (57)<0.0001
 Decreased peripheral artery pulse(s)0 (0)2 (1)0 (0)62 (71)<0.0001
 Claudication of extremities1 (0.1)7 (5)1 (2)33 (38)<0.0001
Decreased pulses/claudication combined1 (0.1)7 (5)1 (2)65 (75)<0.0001
 Discrepancy on 4 limb blood pressure >10 mm Hg in any limb0 (0)4 (3)0 (0)54 (62)<0.0001
Neurological
 Motor mononeuritis multiplex1 (0.1)27 (18)0 (0)1 (1)<0.0001
 Sensory peripheral neuropathy1 (0.1)31 (21)0 (0)0 (0)<0.0001
Mono- or polyneuropathy combined1 (0.1)39 (26)0 (0)1 (1)<0.0001
Gastrointestinal
 Abdominal pain500 (60)75 (50)9 (15)46 (41)<0.0001
Genital
 Testicular pain or tenderness68/419 (16)15/75 (20)0/19 (0)0/21 (0)0.019
Musculoskeletal
 Muscle pain or tenderness109 (13)108 (72)22 (37)27 (31)<0.0001
 Arthritis349 (42)65 (43)18 (30)10 (11)<0.0001
 Arthralgia558 (67)100 (67)31 (52)26 (30)<0.0001
Arthritis/arthralgia combined645 (78)114 (76)33 (55)28 (32)<0.0001
  • Variables in italics indicate combination of signs/symptoms.

  • Data for the remaining 59 patients with other c-vasculitides are not shown. Only data that are relevant for the proposed criteria are shown. The numbers in parentheses indicate the percentage of subjects with a particular diagnosis who had the feature in question. Denominators refer to the top of each column.

  • c-PAN, childhood polyarteritis nodosa; c-TA, c-Takayasu arteritis; c-WG, c-Wegener granulomatosis; HSP, Henoch–Schönlein purpura.