Feature | IgG4+ MOLPS | Typical SS | Japanese, % | p |
---|---|---|---|---|
No. of Patients | 64 | 31 | ||
Xerophthalmia | 32.8 (21) | 93.5 (29) | < 0.001 | |
Xerostomia | 37.5 (24) | 87.1 (27) | < 0.001 | |
Arthralgia | 15.6 (10) | 48.4 (15) | 0.001 | |
Allergic rhinitis | 40.6 (26) | 6.5 (2) | 5–10 | 0.001 |
Bronchial asthma | 14.1 (9) | 3.2 (1) | 3–5 | 0.158 |
Sclerosing pancreatitis | 17.2 (11) | 0 (0) | < 0.001 | 0.014 |
Interstitial nephritis | 17.2 (11) | 6.5 (2) | < 0.005 | 0.210 |
Interstitial pneumonitis | 9.4 (6) | 32.3 (10) | < 0.005 | 0.008 |
RF | 26.6 (17) | 87.1 (27) | < 0.001 | |
ANA | 23.4 (15) | 90.3 (28) | < 0.010 | |
A-SSA | 1.6 (1) | 100 (31) | < 0.001 | |
A-SSB | 0 (0) | 100 (31) | < 0.001 | |
Low CH50 | 57.8 (37) | 48.4 (15) | 0.510 | |
IgG, mg/dl | 2960.1 (1.7) | 2473.4 (1.4) | 870–1700 | 0.042 |
IgG1, mg/dl | 1155.3 (1.6) | 1437.1 (1.5) | 320–748 | 0.039 |
IgG2, mg/dl | 786.5 (1.5) | 566.6 (1.6) | 208–754 | 0.001 |
IgG3, mg/dl | 57.6 (2.8) | 81.9 (1.8) | 6.6–88.3 | 0.047 |
IgG4, mg/dl | 697.7 (2.6) | 23.5 (2.1) | 4.8–105 | < 0.001 |
IgA, mg/dl | 194.7 (1.80) | 389.7 (1.7) | 110–410 | < 0.001 |
IgM, mg/dl | 63.0 (2.0) | 147.3 (1.7) | 35–220 | < 0.001 |
IgE, IU/ml | 307.4 (4.0) | 15.3 (1.4) | < 173 | 0.005 |
P values are for comparisons of all IgG4+ MOLPS with typical SS. MOLPS: multiorgan lymphoproliferative syndrome; SS: Sjögren’s syndrome; RF: rheumatoid factor; ANA: antinuclear antibody. Japanese: Incidence rates of the entire Japanese study population for allergic rhinitis, bronchial asthma, sclerosing pancreatitis, interstitial nephritis, interstitial pneumonitis, and ranges of normal laboratory values of total IgG, IgG1, IgG2, IgG3, IgG4, IgA, IgM, and IgE. IgE was measured in 50 patients (not all), and IgG1, IgG2, and IgG3 were measured in 58 patients (not all), with IgG4+ MOLPS. Geometric means (geometric SD) are shown for IgG, IgG1, IgG2, IgG3, IgG4, IgE, IgA, and IgM concentrations. Patients with typical SS fulfilled both Japanese8 and European9 SS criteria, and were positive for both anti-SSA/Ro and anti-SSB/La antibodies.