Table 1.
Main characteristics of included studies.
| Study | Design | Country | n | pSS Classification Criteria |
F, % | ILD, n (%) | HRCT | NSIP, n (%) | UIP, n (%) | PFTs | DLCO (mmol/min kPa) |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Constantopoulos et al., 1985 [17] |
Prospective | Greece | 36 | * | 91.7% | 9 (25%) | - | - | - | - | |
| Papathanasiou et al., 1986 [18] |
Prospective | Greece | 40 | * | 100% | 15 (37.5%) | - | - | - | yes | 91.23 ± 18.86 |
| Papiris et al., 1999 [19] |
Prospective | Greece | 61 | 1993 ECCC | 95% | 19 (31%) | yes | - | - | yes | 85 ± 17.7 |
| Cervera et al., 2000 [24] |
Prospective | Spain | 223 | 1993 ECCC | 91.5% | 19 (8.5%) | - | - | - | - | |
| Taouli et al., 2002 [41] |
Retrospective | France | 35 | 1993 ECCC | 80% | 12 (34.3%) | yes | yes | 71.9 ± 15.7 | ||
| Lin et al., 2010 [29] |
Retrospective | China | 522 | 2002 AECG | 91% | 116 (22.2%) | yes | - | - | - | - |
| Botsios et al., 2011 [27] |
Retrospective | Italy | 336 | 2002 AECG | 96% | 5 (1.5%) | yes | - | - | - | - |
| Ter Borg et al., 2014 [25] |
Prospective | Netherlands | 83 | 2002 AECG | 89% | 6 (7.2%) | yes | 4 (4.8%) | - | - | |
| Kvarnstrom et al., 2015 [21] |
Prospective | Sweden | 199 | 2002 AECG | 93% | 2 (1%) | - | - | - | - | - |
| Li et al., 2015 [30] |
Retrospective | China | 315 | 2002 AECG | 96% | 66 (21%) | yes | yes | - | ||
| Zhao et al., 2015 [31] |
Retrospective | China | 483 | 2002 AECG | 94% | 59 (12.2%) | yes | - | - | - | - |
| Manfredi et al., 2017 [26] |
Prospective | Italy | 77 | 2002 AECG | 88% | 13 (16.9%) | yes | - | 7 (9%) | yes | - |
| Ramirez Sepulveda et al., 2017 [22] |
Prospective | Sweden/Norway | 967 | 2002 AECG | 93% | 48 (5%) | - | - | - | - | - |
| Roca et al., 2017 [42] |
Retrospective | France | 263 | 2002 AECG | - | 21 (8%) | yes | 7 (33.3%) | 5 (23.8%) | yes | |
| Strevens Bolmgren et al., 2017 [23] |
Prospective | Sweden | 51 | 2002 AECG | 96% | 9 (17.6%) | - | 6 (11.7%) | 0 | 6.5 ± 1.9 | |
| Ter Borg et al., 2017 [43] |
Retrospective | Netherlands | 140 | 2002 AECG | 89% | 17 (12.1%) | yes | - | - | - | - |
| Dong et al., 2018 [32] |
Retrospective | China | 527 | 2002 AECG/2016 ACR EULAR | 88% | 206 (39%) | yes | 86 (41.7%) | 22 (10.7%) | yes | 54.54 ± 21.25 |
| Gao et al., 2018 [33] |
Retrospective | China | 853 | 2002 AECG | - | 165 (31.7%) | yes | 27 (39.1%) | 11 (15.9%) | yes | 57.5 ± 21.2 |
| Kakugawa et al., 2018 [44] |
Retrospective | Japan | 101 | 2002 AECG | 94% | 32 (1.8%) | yes | 28 (27.7%) | 2 (2.0) | - | - |
| Wang et al., 2018 [28] |
Prospective | China | 201 | 2002 AECG | 88% | 158 (78.6%) | yes | 72 (45.5%) | 16 (10.1%) | yes | 42.9 ± 19.4 |
| Kampolis et al., 2018 [20] |
Prospective | Greece | 384 | 2002 AECG | 94.5% | 7 (1.8%) | yes | - | - | yes | 81.74 ± 17.38 |
| Guisado-Vasco et al., 2019 [45] |
Retrospective | Spain | 102 | 2016 ACR/EULAR | 93% | 36 (35.3%) | yes | 27 (26%) | 6 (5.9%) | yes | - |
| Sogkas et al., 2020 [46] |
Retrospective | Germany | 31 | 2016 ACR/EULAR | 71% | 19 (61%) | yes | 9 (29%) | 13 (42%) | yes | - |
| Shi et al., 2020 [34] |
Retrospective | China | 706 | 2002 AECG | 90.5% | 168 (23.8%) | - | - | - | - | - |
| Ufuk et al., 2020 [38] |
Retrospective | Turkey | 28 | 2016 ACR/EULAR | 86% | 34 (75%) | yes | 21 (75%) | 6 (21.4%) | yes | |
| Gao et al., 2021 [35] |
Retrospective | China | 934 | 2002 AECG | - | 178 (19%) | yes | 57 (44.9%) | 9 (15.0%) | yes | 72.4 ± 20.9 |
| Sahin Ozdemirel et al., 2021 [39] |
Retrospective | Turkey | 35 | 2016 ACR/EULAR | 94% | 1 (3%) | yes | - | - | yes | 91.28 ± 19.70 |
| Lin et al., 2022 [36] |
Retrospective | China | 333 | 2002 AECG | 93.1% | 66 (19.8%) | yes | 42 (63.6%) | 20 (30.3%) | yes | 58.82 ± 21.04 |
| Weng et al., 2022 [37] |
Retrospective | China | 69 | 2016 ACR/EULAR | 90% | 35 (50%) | yes | - | - | yes | - |
| Işik et al., 2022 [40] |
Retrospective | Turkey | 120 | 2016 ACR/EULAR | - | 16 (13.3%) | yes | 10 (62.5%) | 2 (12.5%) | yes | 60.1 ± 20.4 |
* Other criteria before 2002 AECG: xerostomia, sicca syndrome, focal lymphocyte infiltrate on minor salivary gland biopsy. n, number of patients; ILD, interstitial lung disease, UIP, usual interstitial pneumonia, NSIP, non-specific interstitial pneumonia; PFTs, pulmonary function tests. DLco results are presented as mean ± SD or as a percentage.