Characteristics of the patients studied
| Features | Non-SS | Patients with SS | ||
| Sialadenitis (n=8) | SSwo (n=27) | SSpL (n=17) | SSL (n=35) | |
| General | ||||
| Age (years), median (range) | 61.5 (53–74) | 55 (30–76) | 35 (24–75) | 43.5 (26–79) |
| Men/women | 2/6 | 0/27 | 1/16 | 4/31 |
| Duration (years) of sicca symptoms, median (range) | 0.65 (0.1–5.0) | 3 (0.5–10.0) | 5.7 (1.0–13.0) | 8.0 (0.5–36.0) |
| Histological (MSG biopsy) | ||||
| Biopsy focus score (number of lymphocytic foci/4mm2), median (range) | 0.42 (0.0–4.4) | 1.45 (1.0–4.0) | 4.44 (1.0–11.5) | 5.14 (1.0–9.23) |
| Germinal center formation, n (%) | 0 (0) | 3 (11.1) | 8 (47.1) | 16 (45.7) |
| Clinical | ||||
| Arthralgias, n (%) | 0 (0) | 8 (29.6) | 5 (29.41) | 12 (34.3) |
| Arthritis, n (%) | 0 (0) | 3 (11.1) | 1 (5.9) | 3 (8.6) |
| SG enlargement, n (%) | 0 (0) | 5 (18.5) | 9 (52.9) | 26 (74.3) |
| Raynaud’s phenomenon, n (%) | 0 (0) | 6 (22.2) | 5 (29.4) | 6 (17.1) |
| Parenchymal organ involvement, n (%) | 0 (0) | 1 (3.7) | 1 (5.9) | 4 (11.4) |
| Lung involvement, n (%) | 0 (0) | 1 (3.7) | 1 (5.9) | 4 (11.4) |
| Renal involvement, n (%) | 0 (0) | 0 (0) | 0 (0) | 0 (0) |
| Liver involvement, n (%) | 0 (0) | 0 (0) | 0 (0) | 0 (0 |
| Indicative of vasculitic involvement, n (%) | 0 (0) | 0 (0) | 7 (41.2) | 18 (51.4) |
| Palpable purpura, n (%) | 0 (0) | 0 (0) | 7 (41.2) | 18 (51.4) |
| Vasculitis (%), n (%) | 0 (0) | 0 (0) | 3 (17.6) | 4 (11.4) |
| Glomerulonephritis, n (%) | 0 (0) | 0 (0) | 1 (5.9) | 4 (11.4) |
| Peripheral neuropathy, n (%) | 0 (0) | 0 (0) | 2 (11.8) | 6 (17.1) |
| ESSDAI score, median (range) | NA | 1 (0–4) | 9 (1–14) | 10 (4–19) |
| Laboratory | ||||
| Anti-Ro/SSA and/or La/SSB positive, n (%) | 0 (0) | 17 (62.96) | 11 (64.7) | 28 (80) |
| Anti-Ro/SSA positive, n (%) | 0 (0) | 17 (63.0) | 11 (64.7) | 28 (80) |
| Anti-La (SSB) positive, n (%) | 0 (0) | 3 (11.1) | 8 (47.1) | 20 (57.1) |
| Rheumatoid factor positive, n (%) | 1 (12.5) | 9 (33.3) | 12 (70.6) | 28 (80) |
| C3 levels, median (range) | 84 (79–118) | 105 (74–139) | 87.5 (65–191) | 98.2 (36–191) |
| C4 levels, median (range) | 24.5 (13–36.0) | 22.0 (4.0–47.3) | 10.4 (1.0–28.0) | 10.4 (1.0–30.0) |
| C4 hypocomplementaemia, n (%) | 1 (12.5) | 7 (25.9) | 10 (58.8) | 24 (68.6) |
| Cryoglobulinaemia, n (%) | 0 (0) | 0 (0) | 4 (23.5) | 11 (31.4) |
| Hypergammaglobulinaemia, n (%) | 0 (0) | 2 (7.4) | 10 (58.8) | 22 (62.9) |
| Leucopenia, n (%) | 0 (0) | 2 (7.4) | 1 (5.9) | 4 (11.4) |
| Treatment, n (%) | ||||
| Corticosteroids | 0 (0) | 0 (0) | 2 (11.8) | 3 (8.5) |
| Hydroxychloroquine | 0 (0) | 2 (7.4) | 2 (11.8) | 2 (5.7) |
| Cyclophosphamide | 0 (0) | 0 (0) | 1 (5.9) | 1 (2.9) |
| Rituximab | 0 (0) | 0 (0) | 1 (5.9) | 5 (14.3) |
ESSDAI, EULAR SS disease activity index; MSG, minor salivary gland; NA, not applicable; SG, salivary gland; SS, Sjögren’s syndrome; SSL, SS-associated lymphoma; SSpL, pre-lymphoma; SSwo, without lymphoma.