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 criteria		Extraglandular manifestations and clinical features		Laboratory findings		Time between diagnoses of SS and lymphoma	Type of lymphoma and cause of death
Patient no; sex; age at SS diagnosis (years)		Diagnostic criteria		Extraglandular manifestations and clinical features		At baseline	At follow up	Time between diagnoses of SS and lymphoma	Type of lymphoma and cause of death
1. Female, 56	Schirmer 2/5 mm, RB+/+	Parotid swellings, arthralgias	S-β₂m 9.2	5.4 mg/l	2 years	Malignant lymphocytic lymphoma, large, non-cleaved, stage IVA
	Xerostomia +	Raynaud's, purpura, lymphadenopathy	S-IgG 25.00	9.25 g/l
	LSG grade 4	Liver and spleen enlargement, pulmonary fibrosis	S-IgA 4.40	1.98 g/l		Cause of death: generalised lymphoma
	Latex +, Wa-Ro +	Pleurisy and pericarditis (in terminal phase)	S-IgM 3.30	0.14 g/l
	ANA +	Oculomotorius and peroneal paresis	S-γ globulin 22.5	10.5 g/l
	(4/4 criteria)	Pulmonary pseudolymphoma	ESR 56	38 mm/1st h

2. Female, 55	Schirmer 4/3 mm, BUT 3/2 sec,	Parotid and submandibular swellings	S-β₂m 4.1	5.0 mg/l	10 years	Ileum, malignant lymphoma, T cell type
	RB−/−	Arthralgias	S-IgG 35.73	37.85 g/l		Ileum, malignant lymphoma, T cell type
	Xerostomia +	Liver enlargement	S-IgA 4.67	7.98 g/l		Cause of death: ileal perforation caused by lymphoma
	LSG grade 3–4	Coeliac disease	S-IgM 2.32	3.53 g/l
	Latex +, Wa-Ro +	Hypothyroidism	S-γ globulin 33.4 g/l	—	—
	RNP +, SSA +, SSB +		ESR 49	90 mm/1st h

	(3/4 criteria)

3. Female, 71	Schirmer 4/8, RB ND	Arthralgias and arthritis	S-β₂m 2.9 mg/l	—	4 years	Malignant lymphoma, diffuse centroblastic centrocytic
	Xerostomia +	Lymphadenopathy	S-IgG 12.50	10.39 g/l		Malignant lymphoma, diffuse centroblastic centrocytic
	LSG grade 3	Pleurisy (associated with pneumonia)	S-IgA 3.73	3.06 g/l		Cause of death: pneumonia and staphylococcal septicaemia
	RF 0, ANA +, SSA +	Dizziness, lesio vestibularis trunchi cerebri	S-IgM 3.98	2.99 g/l
	(3/4 criteria)		ESR 42	23 mm/1st h

4. Female, 64	Schirmer ND, RB−/+	Submandibular swellings	S-β₂m 8.0	12.4 mg/l	0 years	Nodal biopsy? (to be taken)
	Xerostomia +	Arthralgias, Raynaud's	S-IgG 23.97 g/l			Cause of death: SS and CHF (no autopsy)
	LSG grade 4	Trigeminal neuralgia	S-IgA 2.27 g/l
	Latex 0, Wa-Ro 0	Liver enlargement	S-IgM 0.55 g/l
	ANA +	Massive lymphadenopathy and abdominal tumour	S-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.