Table 1

Clinical findings and IgG4-/IgG-positive cell ratio in six patients with multicentric Castleman's disease

Patient noAge/sexPresentationAutoantibodiesTreatment and clinical outcome (follow-up period in months)IgG4-/IgG-positive cell ratio (%)
143/MFatigue, systemic lymphadenopathy, thrombocytosisANA (borderline)
RF (+)
Follow-up; stable disease (10)45.2
268/MNo subjective symptoms, systemic lymphadenopathy, thrombocytosisANA (borderline)
RF (–)
Anti-SS-A/anti-SS-B (–)
Anti-dsDNA (–)
Follow-up; stable disease (11)54.7
347/FFatigue, skin purpura, renal dysfunction, systemic lymphadenopathy, thrombocytosisANA (+)
RF (+)
Anti-SS-A/anti-SS-B (+)
Anti-dsDNA (+)
Steroid; partial remission, worsening of symptoms on reduced dose of steroid (26)46.5
450/MFever, renal dysfunction, systemic lymphadenopathy, multiple lung nodules, bone marrow plasmacytosis, thrombocytosisANA (+)
Anti-dsDNA (+)
Steroid; complete remission, disapperance of lymph node swelling and multiple lung nodules, negative test results for autoantibodies (31)48.8
565/MNo subjective symptoms, incidental finding of systemic lymphadenopathy since several yearsANA (borderline)Follow-up; stable disease (10)76.6
654/MNo subjective symptoms, incidental finding of systemic lymphadenopathy since 15 years, multiple lung nodules, thrombocytosisANA (–)
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.