Table 2.

Comparison of symptoms, complaints, and laboratory findings in IgG4+ MOLPS and typical SS. Data are percentage (number) unless stated otherwise. Incidence rates (numbers of positive patients) are shown for xerophthalmia, xerostomia, arthralgia, allergic rhinitis, bronchial asthma, sclerosing pancreatitis, interstitial nephritis, interstitial pneumonitis, RF, ANA, A-SSA, A-SSB, and low CH50. Masaki Y, et al7. Ann Rheum Dis 2009; 68:1310–5. Adapted with permission.

FeatureIgG4+ MOLPSTypical SSJapanese, %p
No. of Patients6431
Xerophthalmia32.8 (21)93.5 (29)< 0.001
Xerostomia37.5 (24)87.1 (27)< 0.001
Arthralgia15.6 (10)48.4 (15)0.001
Allergic rhinitis40.6 (26)6.5 (2)5–100.001
Bronchial asthma14.1 (9)3.2 (1)3–50.158
Sclerosing pancreatitis17.2 (11)0 (0)< 0.0010.014
Interstitial nephritis17.2 (11)6.5 (2)< 0.0050.210
Interstitial pneumonitis9.4 (6)32.3 (10)< 0.0050.008
RF26.6 (17)87.1 (27)< 0.001
ANA23.4 (15)90.3 (28)< 0.010
A-SSA1.6 (1)100 (31)< 0.001
A-SSB0 (0)100 (31)< 0.001
Low CH5057.8 (37)48.4 (15)0.510
IgG, mg/dl2960.1 (1.7)2473.4 (1.4)870–17000.042
IgG1, mg/dl1155.3 (1.6)1437.1 (1.5)320–7480.039
IgG2, mg/dl786.5 (1.5)566.6 (1.6)208–7540.001
IgG3, mg/dl57.6 (2.8)81.9 (1.8)6.6–88.30.047
IgG4, mg/dl697.7 (2.6)23.5 (2.1)4.8–105< 0.001
IgA, mg/dl194.7 (1.80)389.7 (1.7)110–410< 0.001
IgM, mg/dl63.0 (2.0)147.3 (1.7)35–220< 0.001
IgE, IU/ml307.4 (4.0)15.3 (1.4)< 1730.005
  • 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.