Table 1.

Demographics and disease manifestations of patients with granulomatosis with polyangiitis (GPA) with and without cardiac involvement.

DemographicsGPA with Cardiac InvolvementGPA without Cardiac Involvementp
Total, n17500
Female, n (%)7 (41)262 (52)0.36
Mean age at diagnosis, yrs (± SD)43.7 (16.5)45.9 (20.3)0.70
Race, n (%)0.24
  White17 (100)442 (88)
  Nonwhite*058 (12)
Time from symptom onset to diagnosis, mos (± SD)21 (28)14 (36)0.48
Duration of followup, mos (± SD)97 (67)106 (71)0.68
ANCA-positive, n (%)**16 (94)407 (81)0.33
Anti-PR3–positive, n (%)#11 (65)338 (68)0.68
Any history of flare9 (53)325 (65)0.31
Disease manifestations
Constitutional14 (82)400 (80)1.0
Musculoskeletal10 (59)334 (67)0.49
Skin8 (47)158 (32)0.12
Mucus membranes1 (6)57 (11)1.0
  Eyes1 (6)163 (33)0.03
  Ear, nose, and throat13 (76)431 (86)0.28
  Lung14 (82)351 (70)0.42
Gastrointestinal07 (1)1.0
Kidney involvement10 (69)275 (55)0.55
Nervous system involvement4 (24)109 (22)0.76
  • * Asian/Black/African American/Unknown race/mixed race or American Indian/Native Alaskan.

  • ** Antineutrophil cytoplasmic antibody (ANCA), including cytoplasmic ANCA or perinuclear ANCA.

  • # Antiproteinase 3 antibody.

  • Any history of flare defined as disease activity after a period of remission, either prior to enrollment in the Vasculitis Clinical Research Consortium (VCRC) or during subsequent VCRC followup.