Table 3

Demographic and disease characteristics of the derivation and validation cohorts*

Derivation cohort
(n=486)†
Validation cohort 1
(n=908)†
Validation cohort 2
(n=485)
IgG4-RD272 (56)493 (54)321 (66)
Mimickers‡214 (44)415 (46)164 (34)
 Vasculitis26 (12)106 (26)34 (21)
 Malignancy51 (24)31 (7)36 (22)
 Sjögren’s syndrome13 (6)59 (14)8 (5)
 Other pancreatitis5 (2)15 (4)7 (4)
 Other119 (56)204 (49)79 (48)
Male sex319 (66)503 (55)288 (59)
Age at diagnosis, mean±SD, years58.2±14.555.5±16.556.4±16.8
Select organ involvement
 Salivary glands153 (31)278 (31)151 (31)
 Orbit101 (21)188 (21)146 (30)
 Pulmonary128 (26)173 (19)75 (15)
 Lymph nodes176 (36)262 (29)95 (20)
 Aorta52 (11)97 (11)37 (8)
 Retroperitoneal fibrosis78 (16)108 (12)50 (10)
 Pancreas132 (27)269 (30)160 (33)
 Biliary75 (15)149 (16)91 (19)
 Renal90 (19)137 (15)74 (15)
No of organs involved, median
(IQR)
2 (1–4)2 (1–3)2 (1–3)
  • *In validation cohort 1, the judgement of a case as being IgG4-RD or as being an IgG4-RD mimicker was ‘confident’ or ‘very confident’ in 771 cases (84.9% of all cases and mimickers included in that cohort). In validation cohort 2, the judgement of a case as being IgG4-RD or as being an IgG4-RD mimicker was ‘confident’ or ‘very confident’ in 431 cases (88.9% of all cases and mimickers included in that cohort). Except where indicated otherwise, values are the number (%).

  • †Includes all submitted cases and mimickers.

  • ‡Mimicker conditions are listed in online supplementary table 5.

  • IgG4-RD, IgG4-related disease.