Patient no | Age/sex | Presentation | Autoantibodies | Treatment and clinical outcome (follow-up period in months) | IgG4-/IgG-positive cell ratio (%) |
1 | 43/M | Fatigue, systemic lymphadenopathy, thrombocytosis | ANA (borderline) RF (+) | Follow-up; stable disease (10) | 45.2 |
2 | 68/M | No subjective symptoms, systemic lymphadenopathy, thrombocytosis | ANA (borderline) RF (–) Anti-SS-A/anti-SS-B (–) Anti-dsDNA (–) | Follow-up; stable disease (11) | 54.7 |
3 | 47/F | Fatigue, skin purpura, renal dysfunction, systemic lymphadenopathy, thrombocytosis | ANA (+) RF (+) Anti-SS-A/anti-SS-B (+) Anti-dsDNA (+) | Steroid; partial remission, worsening of symptoms on reduced dose of steroid (26) | 46.5 |
4 | 50/M | Fever, renal dysfunction, systemic lymphadenopathy, multiple lung nodules, bone marrow plasmacytosis, thrombocytosis | ANA (+) Anti-dsDNA (+) | Steroid; complete remission, disapperance of lymph node swelling and multiple lung nodules, negative test results for autoantibodies (31) | 48.8 |
5 | 65/M | No subjective symptoms, incidental finding of systemic lymphadenopathy since several years | ANA (borderline) | Follow-up; stable disease (10) | 76.6 |
6 | 54/M | No subjective symptoms, incidental finding of systemic lymphadenopathy since 15 years, multiple lung nodules, thrombocytosis | ANA (–) RF (–) Anti-SS-A/anti-SS-B (–) Anti-Sm (–) | Tocilizumab; complete remission, disappearance of lymph node swelling and multiple lung nodules, improvement in laboratory data (13) | 43.4 |
ANA, antinuclear antibody; dsDNA, double-stranded DNA; F, female; Ig, immunoglobulin; M, male; RF, rheumatoid factor; SS, Sjögren syndrome; Sm, Smith antigen; Tocilizumab, anti-human interleukin-6 receptor monoclonal antibody.