Table 2.
Glandular and extra-glandular features of pSS in male and paediatric populations
| Glandular | Extra-glandular (EG) | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| References | Dry eyes N (%) | Dry mouth N (%) | Parotitis N (%) | Schirmera N (%) | Any sicca N (%) | Any EG N (%) | Joint Involvement N (%) | Raynaud’s N (%) | Pulmonary N (%) | Neurological N (%) | Renal N (%) | Lymphadenopathy N (%) | Other N (%) |
| Male studies | |||||||||||||
| Molina et al. [16] | 14 (38.9) | – | 10 (27.8) | 19 (52.8) | 36 (100)b | 29 (80.6) | 28 (77.8) | 4 (11.1) | 1 (2.8) | 14 (38.9) | IgA nephropathy—1 (2.8) | 2 (5.6) | Vasculitis—9 (25.0), splenomegaly—3 (8.3), hepatomegaly—3 (8.3), oesophageal dysmotility—3 (8.3), myositis—2 (5.6), |
| Anaya et al. [17] | – | – | – | 3 (23.1) | 13 (100) | 12 (92.3) | 6 (46.2) | 3 (23.1) | 1 (7.7) | Total—3 (23.1), CN—1 (7.7), PNS—1 (7,7), CNS—1 (7.7) | GN—1 (7.7) | – | Hepatitis—2 (15.4), non-Hodgkin’s lymphoma—2 (15.4), pancreatitis—1 (7.7), thyroiditis—1 (7.7), myositis—1 (7.7) |
| Horvath et al. [18] | – | – | – | – | 60 (100) | – | 41 (68.3) | 4 (6.7) | 0 (0.0) | – | GN/RTA—3 (5.0) | 6 (10.0) | Vasculitis—9 (15.0), serositis—5 (8.3), myositis—5 (8.3) |
| Gondran et al. [19] | 38 (90.5) | 40 (95.2) | 15 (35.7) | – | – | 37 (88.1) | 16 (38.1) | 12 (28.6) | 8 (19.0) | 11 (26.2) | 2 (4.8) | 2 (4.8) | Dermatological—9 (21.4), lymphoma—4 (9.5), myositis—4 (9.5), depression—2 (4.8) |
| Cervera et al. [20] | 18 (94.7) | 18 (94.7) | 3 (15.8) | – | – | 10 (52.6) | 4 (21.1) | 0 (0.0) | 3 (15.8) | PN—2 (10.5) | – | – | |
| Diaz-Lopez et al. [21] | – | – | 5 (17.9) | – | – | – | 8 (28.6) | 5 (17.9) | – | – | – | – | Carpal tunnel syndrome—4 (14.3), fibromyalgia—2 (7.1), thyroidopathy—1 (3.6), liver disease—1 (3.6) |
| Drosos et al. [22] | – | – | 2 (16.7) | – | 9 (75.0) | – | 1 (8.3) | 1 (8.3) | – | – | – | 4 (33.3) | Constitutional—4 (33.3), hepatomegaly—2 (16.7) |
| Paediatric studies | |||||||||||||
| Yokogowa et al. [23] | 16 (61.5) | 17 (65.4) | 16 (61.5) | 6/16 (37.5) | 21 (80.8) | 22 (84.6) | 15 (57.7) | – | – | 6 (23.1) | Total—5 (19.2), nephrocalcinosis—2 (7.7), RTA—2(7.7), nephritis—1 (3.8) | 12 (46.2) | Dental caries—15 (57.7), fever—6 (23.1), haematological—4 (15.3), dermatological—4 (15.3), uveitis—2 (7.7) |
| Cimaz et al. [24] | 5 (12.5)c | 5 (12.5)c | 29 (72.5)c | 28 (70.0) | 14 (35.0)c | – | 4 (10.0)c | 4 (10.0)d | – | 2 (5.0) | RTA—3 (7.5)d | 3 (7.5)d | Vulvovaginitis—5 (12.5), hepatitis—4 (10.0)d, fever—4 (10.0) c/3 (7.5)d, fatigue—3 (7.5)c |
| 23 (57.5)d | 25 (37.5)d | ||||||||||||
| Drosos et al. [22] | – | – | 10 (76.9) | – | 3 (23.1) | – | 3 (23.1) | 4 (30.8) | – | – | – | 3 (23.1) | Constitutional—3 (23.1), hepatomegaly—1 (7.8) |
| Stiller et al. [25] | – | – | 6 (54.5) | 8 (72.7) | – | – | – | – | – | – | – | – | – |
| Tomita et al. [26] | – | – | 15 (35.7) | 15 (35.7) | 10 (23.8) | 21 (50.0) | 11 (26.2) | 0 (0.0) | 1 (2.4) | Aseptic meningitis—3 (7.1) | Nephritis—3 (7.1) | 11 (26.2) | Fever—22 (52.4), fatigue—12 (28.6), erythema—11 (26.2), hypothyroidism—2 (4.8), uveitis—2 (4.8), raised AST—1 (2.4), morning stiffness—1 (2.4) |
AST aspartate Aminotransferase, CN cranial neuropathy, CNS central nervous system, EG extra-glandular, GN glomerulonephritis, PN peripheral neuropathy, PNS peripheral nervous system, RTA renal tubular acidosis
aSchirmer’s I test <5 mm/5 min
bSet by inclusion criteria
cAt onset
dAt follow-up