Age at pSS diagnosis | 75 | 52 | 69 | 54 | 67 | 66 | 59 | 50 | 52 | 53 | 25 | 57 |
Age at lymphoma/myeloma onset | 76 | 58 | 72 | 61 | 78 | 73 | 71 | 57 | 65 | 59 | 33 | 67 |
| | 61 | | | | | | | | | | |
Death/age at death | + | + | − | − | + | + | − | + | − | + | + | − |
| 82 | 62 | | | 79 | 74 | | 57 | | 61 | 39 | |
Symptom duration before pSS diagnosis | 10 | 16 | 21 | 12 | 18 | 0 | 6 | 18 | 25 | 11 | 2 | 4 |
Sex | Female | Male | Male | Female | Female | Female | Female | Female | Female | Female | Female | Female |
Lymphoma type (WHO) | Small lymphocytic B cell/chronic lymphatic leukaemia | Small lymphocytic B cell+Myeloma | Follicular B cell | Small lymphocytic B cell = Waldenström MG | Diffuse large B cell | Diffuse large B cell | Diffuse large B cell** | Anaplastic large T cell | Diffuse large B cell | Diffuse large B cell | Diffuse large B cell | Myeloma + diffuse large B cell |
Grade | Low | Low | Low | Low | High | High | High | High | High | High | High | (High) |
Primary localisation | BM, LNs | BM, LNs | Salivary glands | BM | LN groin | Right knee hollow | Salivary gland? LNs neck, intra-abdominal, BM | LNs, liver spleen, BM | Lung parenchyma | BM, LNs | Salivary glands,LNs | LNs |
Salivary gland swelling | −* | +* | −* | +† | +* | +* | +* | −* | +† | +† | +* | −* |
Palpable purpura/skin vasculitis | −* | +* | −* | −* | −* | −* | +* | +* | +† | +† | +* | +† |
Peripheral neuropathy | −* | +* | −* | −* | +† | +† | −* | +† | +† | +† | +† | −* |
Enlarged LN or spleen | −* | −* | −* | −* | −* | −* | −* | +† | +† | + | −* | +* |
| | | | | +† | | | | | | | |
Concomitant lymphoma predisposing condition | Squamous cell cancer* | Psoriasis† | Psoriasis* H pylori† Chronic gastritis | – | Basal cell cancer† | Hashimoto† | H pyloriSquamous + basal cell cancer† | – | Coeliac disease* H pylori† Hashimoto* | – | Sibling with psoriasis | Basal cell cancer† |
RF | +* | +* | −* | +* | +† | +* | +* | −* | +* | − | +* | +* |
SSA/SSB | −† | +* | +† | +* | +* | +† | −* | +† | +† | +* | +* | +* |
ANA | +* | +* | +* | +* | +* | +* | +* | +† | +* | +* | +* | +* |
Cryoglobulinaemia | ND | +† | ND | ND | ND | ND | −* | −* | +* | ND | +* | ND |
Lymphopenia | −* | −* | −* | −* | −* | +* | +* | +† | +* | −* | +* | −* |
Anaemia | −* | −* | −* | −* | −* | +* | −* | −† | +* | −* | +* | −* |
C3 (g/l) | 0.70* | 0.55* | 0.98* | 0.83* | 0.84* | 0.75* | 0.50* | 0.83* | 0.78* | 1.01*0.81† | 0.73* | 1.06† |
C4 (g/l) | 0.36* | 0.13* | 0.27* | 0.29* | 0.14* | 0.16* | 0.01* | 0.16* | 0.18* | 0.37*0.12† | 0.06* | 0.23† |
CD4-penia§ | ND | +* | +* | −* | +* | +* | −† | +* | +* | −* | +* | +* |
IgG (g/l) | 8.2* | 14.0* | 11.9* | 13.8* | 16.0* | 27.0* | 11.3* | 23.0* | 34.1* | 14.4* | 36.0* | 24.6* |
IgA (g/l) | 1.72* | 2.20* | 2.20* | 2.14* | 2.82* | 1.38* | 1.29* | 1.00* | 0.13* | 2.30* | 3.31* | 2.98* |
IgM (g/l) | 0.63* | 2.30* | 1.76* | 1.54* | 0.86* | 2.03* | 1.11* | 1.03* | 1.33* | 0.40* | 1.98* | 1.51* |
Monoclonality | −* | +* IgG | −* | +† IgM | +† IgM | −*, oligo† | −* | −* | −* | −* | +* IgM | Oligo* |
Salivary gland biopsy¶ | +* | +* | ‡ | +* | +* | +* | +* | +* | +* | +* | +* | ND |