Table 1.

Clinical manifestations and histopathological/laboratory/serological features in the 2 groups of patients. Values are n (%).

VariablespSS non-MALT, n = 57pSS-MALTδ, n = 19p
Clinical manifestations
  Oral dryness (subjective)46/57 (81)18/19 (95)0.274
  Ocular dryness (subjective)43/57 (75)17/19 (89)0.330
  SGE13/57 (23)11/19 (58)0.009
  Raynaud phenomenon13/57 (23)7/19 (37)0.243
  Palpable purpura5/57 (9)2/19 (10)1.000
  Arthralgias/myalgias35/55 (64)14/19 (73)0.576
  Arthritis10/57 (17)4/19 (21)0.740
  Lymphadenopathy3/57 (5)5/19 (26)0.020
Histopathological/laboratory/serological features
  MSG biopsy focus score ≥ 129/57 (51)17/19 (89)0.003
  MSG biopsy Tarpley score > 217/57 (30)17/19 (89)< 0.001
  Leukopenia*6/56 (11)2/19 (10)1.000
  ANA-positive36/57 (63)16/19 (84)0.153
  C4 hypocomplementemia27/51 (53)9/17 (53)1.000
  Anti-Ro/SSA- and/or anti-La/SSB-positive36/57 (63)14/19 (74)0.235
  RF-positive26/57 (46)16/19 (84)0.001
  Monoclonal gammopathy0/44 (0)5/17 (29)0.001
  • δ pSS-MALT: pSS patients with MALT lymphoma, matched 1:3 for age, sex, and disease duration with pSS non-MALT patients.

  • * Defined as white blood cells < 3000/mm3 on 3 separate occasions.

  • Titers > 1:160.

  • Defined as serum C4 levels < 20 mg/dl on 3 separate occasions. Values in bold face are statistically significant. pSS: primary Sjögren syndrome; MALT: mucosa-associated lymphoid tissue; SGE: salivary gland enlargement; MSG: minor salivary gland; ANA: antinuclear antibodies; RF: rheumatoid factor.