Table 1.

Papers included in the systematic review

References	Study type	Country of origin	N of pSS patients	Criteria for inclusion of patients	Mean age	Female: male ratio	JBI criteria fulfilled (out of 9)
Male studies
Molina et al. [16]	Cross-sectional study	US	36	Personala	50 (at diagnosis)	1.91:1	5
Anaya et al. [17]	Case-control study	US	13	ECSG	39.4 (at diagnosis)	2:1	4
Horvath et al. [18]	Cross-sectional study	Hungary	60	AECG	56.18 (at study)	7:1	6
					47.83 (at diagnosis)
Gondran et al. [19]	Consecutive case series—multicentre study	France	42	AECG	56.9 (at diagnosis)	10.1:1	6
Cervera et al. [20]	Consecutive case series study	Spain	19	ECSG	55 (at onset)	10.7:1	5
Diaz-Lopez et al. [21]	Cross-sectional study	Spain	28	ECSG	63.5 (at study)	18.6:1	6
Drosos et al. [22]	Case selection for male patients, consecutive case series for female patients	Greece	12	ECSG	45.3 (at onset)	2.5:1	5
Paediatric studies
Yokogowa et al. [23]	Retrospective case-control study	USA	26	Personalb	12.4 (at diagnosis)	24:2	4
Cimaz et al. [24]	Multicentre case series study	Italy	40	Personalc	10.6 (at onset)	35:5	7
Drosos et al. [22]	Case selection for paediatric patients, consecutive case series for female patients	Greece	13	ECSG	9.4 (at onset)	11:2	5
Stiller et al. [25]	Retrospective comparative study	Berlin	11	EULAR	9.5 (at onset)	10:1	5
Tomiita et al. [26]	Cross-sectional epidemiological study	Japan	42	Personald	10.7 (at onset)	–	6

AECG American-European Consensus Group, ECSG European Community Study Group, EULAR European League Against Rheumatism, JBI Joanna Briggs Institute

^aSicca syndrome + 2 of the following: (1) abnormal Schirmer’s test, (2) KCS by Rose Bengal dye test and (3) positive biopsy (Greenspan Grade 3 or 4)

^bDiagnosis of pSS by a paediatric rheumatologist and followed for a minimum of 1 year with no subsequent alternative diagnosis

^cDiagnosis of pSS by referring clinician + onset <16 years

^dHistopathological changes in salivary gland (>3 on Greenspan scale)