Table 3

Clinical and laboratory findings in patients with primary Sjögren's syndrome and subsequent malignant lymphoproliferation and in one patient with suspected malignant lymphoproliferation

Patient no; sex; age at SS diagnosis (years)Diagnostic criteriaExtraglandular manifestations and clinical featuresLaboratory findingsTime between diagnoses of SS and lymphomaType of lymphoma and cause of death
At baselineAt follow up
1. Female, 56Schirmer 2/5 mm, RB+/+Parotid swellings, arthralgiasS-β2m 9.25.4 mg/l2 yearsMalignant lymphocytic lymphoma, large, non-cleaved, stage IVA
Xerostomia +Raynaud's, purpura, lymphadenopathyS-IgG 25.009.25 g/l
LSG grade 4Liver and spleen enlargement, pulmonary fibrosisS-IgA 4.401.98 g/lCause of death: generalised lymphoma
Latex +, Wa-Ro +Pleurisy and pericarditis (in terminal phase)S-IgM 3.300.14 g/l
ANA +Oculomotorius and peroneal paresisS-γ globulin 22.510.5 g/l
(4/4 criteria)Pulmonary pseudolymphomaESR 5638 mm/1st h
2. Female, 55Schirmer 4/3 mm, BUT 3/2 sec,Parotid and submandibular swellingsS-β2m 4.15.0 mg/l10 yearsIleum, malignant lymphoma, T cell type
RB−/−ArthralgiasS-IgG 35.7337.85 g/l
Xerostomia +Liver enlargementS-IgA 4.677.98 g/lCause of death: ileal perforation caused by lymphoma
LSG grade 3–4Coeliac diseaseS-IgM 2.323.53 g/l
Latex +, Wa-Ro +HypothyroidismS-γ globulin 33.4 g/l
RNP +, SSA +, SSB +ESR 4990 mm/1st h
(3/4 criteria)
3. Female, 71Schirmer 4/8, RB NDArthralgias and arthritisS-β2m 2.9 mg/l4 yearsMalignant lymphoma, diffuse centroblastic centrocytic
Xerostomia +LymphadenopathyS-IgG 12.5010.39 g/l
LSG grade 3Pleurisy (associated with pneumonia)S-IgA 3.733.06 g/lCause of death: pneumonia and staphylococcal septicaemia
RF 0, ANA +, SSA +Dizziness, lesio vestibularis trunchi cerebriS-IgM 3.982.99 g/l
(3/4 criteria)ESR 4223 mm/1st h
4. Female, 64Schirmer ND, RB−/+Submandibular swellingsS-β2m 8.012.4 mg/l0 yearsNodal biopsy? (to be taken)
Xerostomia +Arthralgias, Raynaud'sS-IgG 23.97 g/lCause of death: SS and CHF (no autopsy)
LSG grade 4Trigeminal neuralgiaS-IgA 2.27 g/l
Latex 0, Wa-Ro 0Liver enlargementS-IgM 0.55 g/l
ANA +Massive lymphadenopathy and abdominal tumourS-γ globulin 18.6 g/l
(3/4 criteria)ESR 58 mm/1st h
  • RB = Rose-Bengal test; LSG = labial salivary gland biopsy; Wa-Ro = Waaler-Rose test; ANA = antinuclear antibodies; S-β2m = serum β2 microglobulin; ESR = erythrocyte sedimentation rate; SS = Sjögren's syndrome; CHF = congestive heart failure; ND = not done; RNP = ribonucleoprotein; RF = rheumatoid factor.