Table 1.
Clinical manifestations and characteristics of patients with SS with childhood onset included in literature reports describing the use of various treatments
| Author, year [reference] | Level of evidence (Oxford criteria) | N (F:M) | Patient classification criteria used | Age at symptoms onset (years)/age at diagnosis (years) [mean (range) for studies where N > 2] | Associated conditions/ comorbidities, n (%) | Cumulative signs and symptoms, n (%, where applicable) |
|---|---|---|---|---|---|---|
| Singer et al., 2008 [11] | 4 | 7 (7:0) | Not specified | NA/14.2 [10–17] | JIA, 1/7 (14) |
|
| Cimaz et al., 2003 [12] | 4 | 40 (35:5) | Variable classification criteria. Not specified | 10.7/12.4 | NA |
|
| Schuetz et al., 2010 [13] | 4 | 8 (7:1) | Not specified (diagnosis based on histological evidence of salivary gland involvement with or without positive autoantibodies) | 6.5 [0.5–12]/10.6 [6–15] |
|
|
| Kobayashi et al., 1996 [14] | 4 | 4 (4:0) | Not specified | 8.75 [7–10]/10.75 [10–12] |
|
|
| Tomiita et al., 2010 [15] | 3B | 5 (5:0) | Japanese SS diagnostic criteria (1999) | NA/13.6 [9–16] |
|
|
| Franklin et al., 1986 [16] | 4 | 5 (4:1) | At least two of the following three criteria: keratoconjunctivitis sicca, histological evidence of salivary gland involvement with SS and association with well-defined connective tissue disorder | NA/12.6 [5–17] |
|
|
| Saad-Magalhães et al., 2011 [17] | 4 | 8 (6:2) | AECG-2002 (only 3/8 patients fulfilled the criteria) | 5–13 years/NA | NA |
|
| Hamzaoui et al., 2010 [18] | 4 | 3 (3:0) | AECG 2002 | 15.66 [15–16]/15.66 [15–16] | NA |
|
| Yang et al., 2009 [19] | 4 | 4 (4:0) | Revised International Classification for SS (2002) | NA/9–17 years | NA |
|
| Hammett et al., 2020 [20] | 4 | 4 (4:0) | A combination of 2017 ACR/EULAR and expert opinion | 16/16 | N/A | Case 1: abnormal behaviour, tremors, insomnia, polyphagia, polyuria, and suicidal ideation |
| 16/12 | Case 2: 4 year history of severe anxiety, OCD, and tic disorder presented with an abrupt and severe worsening of anxiety, OCD and new auditory hallucinations | |||||
| 19/19 (adult- onset) | Case 3: progressively altered behaviour, incoherent speech, insomnia, headache, and tangential thoughts | |||||
| 17/17 | Case 4: new-onset suicidal ideation, paranoia, confusion and emotional lability | |||||
| Pessler et al., 2006 [21] | 5 | 2 (2:0) | Expert opinion | F, 0.7/10 | NA | Case 1: purpura, polyarthritis, uveitis, RTA, sialadenitis |
| F, 6/10 | NA | Case 2: sialadenitis, RTA/GN | ||||
| Tesher et al., 2019 [22] | 5 | 2 (1:1) | Revised International Classification for SS (2002) | F, 15/15 | NA | Case 1: MALT parotid gland |
| M, 15/15 | Case 2: MALT parotid gland, arthritis | |||||
| De Souza et al., 2012 [23] | 5 | 1 (1:0) | Revised International Classification for SS (2002) | F, 8/16 | NA | Dry eyes, dry mouth |
| Houghton et al., 2005 [24] | 5 | 2 (2:0) | Expert opinion | F, 14 | NA | Case 1: parotid swelling, dental caries, keratitis, xerostomia, LIP |
| F, 14 | Case 2: parotid swelling | |||||
| Berman et al., 1990 [25] | 5 | 1 (1:0) | Expert opinion with histological evidence (parotid biopsy) | F, 10 | NMOSD, hypothyroidism | NMOSD (presented with weakness, decreased sensation in right arm, headache, dizziness, vomiting and low-grade fever) |
| Kornitzer et al., 2016 [26] | 5 | 1 (1:0) | Expert opinion with histological evidence (salivary gland biopsy) | F, 6/9 | NMOSD | NMOSD (presented with fever, headache, progressive right-side weakness and altered mental status) |
| Ostuni et al., 1996 [27]; specific details only given for 2 of 10 patients | 4 | 10 (8:2) | Copenhagen criteria | 11(4–14)/14.6 (11–17) |
|
|
| Baszis et al., 2011 [28] | 4 | 4 (3:1) | Not specified | NA/12 [9–17] | NA |
|
| Gmuca et al., 2017 [29] | 5 | 2 (2:0) | Expert opinion with histological evidence (lip biopsy) | F, 11 | NMOSD | NMOSD (presented with optic neuritis), sicca symptoms |
| F, 13 | NMOSD (presented with optic neuritis) | |||||
| Flaitz et al., 2001 [30] | 5 | 1 (1:0) | Expert opinion with histological evidence (labial lip biopsy) | F, 11/14 | NA | Bilateral parotid swelling, dental problems |
| Nathavitharana et al., 1995 [31] | 5 | 1 (0:1) | Expert opinion with histological evidence (salivary gland biopsy) | M, 5 | NA | Tooth decay, fever, weight loss, bilateral parotid enlargement |
| Siamopoulou-Mavridou et al., 1989 [32] | 5 | 2 (1:1) | Expert opinion with histological evidence (labial salivary gland and lip biopsy, respectively) | M, 8/12 | NA | Recurrent parotid swelling enlargement, keratoconjunctivitis sicca |
| F, 3 | JRA | Arthritis, parotic gland enlargement, dry eyes and dry mouth | ||||
| Civilibal et al., 2007 [33] | 5 | 1 (1:0) | Expert opinion with histological evidence (salivary gland biopsy) | F, 9/13 | N/A | Recurrent bilateral swelling, arthralgia |
| Pessler et al., 2006 [34] | 5 | 1 (1:0) | Expert opinion with histological evidence (salivary gland biopsy) | F, 1/11 | RTA | Purpura, polyarthritis, uveitis, severe dental caries |
| De Oliveira et al., 2011 [35] | 5 | 1 (1:0) | American-European Consensus Group classification criteria for SS | F, 2.6 | NA | Xerostomia, xeropthalmia, bilateral parotic gland enlargement |
| Ohlsson et al., 2006 [36] | 5 | 1 (1:0) | Expert opinion | F, 8 | dRTA | Arthritis |
| Nikitakis et al., 2003 [37] | 5 | 1 (1:0) | Expert opinion with histological evidence (parotid biopsy and labial minor salivary glands biopsy) | F, 4 | NA | Bilateral parotid gland enlargement |
| Ohtsuka et al., 1995 [38] | 5 | 1 (1:0) | Japanese criteria (1980–85) | F, 9 | CNS manifestations | SS with CNS involvement (other symptoms included fever, nausea, xerostomia, parotid gland enlargement) |
| Zhang et al., 2007 [39] | 5 | 1 (1:0) | Expert opinion with histological evidence (minor salivary gland biopsy) | F, 6/9 | PHTN | Recurrent parotid enlargement, xerostomia, purpura, exertional dyspnoea |
| Skalova et al., 2008 [40] | 5 | 1 (1:0) | Expert opinion | F, 16 | dRTA | Rapid-onset muscle weakness, dysphagia, dysphonia, significant wasting |
| Moy et al., 2014 [41] | 5 | 1 (1:0) | Expert opinion with histological evidence (labial gland biopsy) | F, 9 | NA | Recurrent parotid swelling |
| Ladino et al. 2015 [42] | 5 | 1 (0:1) | Expert opinion with histological evidence (salivary gland biopsy) | M, 9/12 | NA | Arthralgia |
| Thouret et al. 2002 [43] | 5 | 1 (1:0) | Expert opinion with histological evidence (labial gland biopsy) | F, 9/13 | NA | Bilateral parotid swelling |
| Shahi et al., 2011 [44] | 5 | 1 (1:0) | Expert opinion with histological evidence (salivary minor gland biopsy) | F, 10 | NA | Recurrent arthralgia, foot swelling |
| Sardenberg et al., 2010 [45] | 5 | 1 (0:1) | Expert opinion | M, 10 | NA | Recurrent parotitis, xerostomia, dental caries |
| Bogdanovic et al., 2013 [46] | 5 | 1 (1:0) | Expert opinion with histological evidence (kidney biopsy) | F, 13 | TIN (manifested as dRTA) | Nephrocalcinosis (incidental finding), parotid gland swelling |
| Zhao et al., 2020 [47] | 5 | 1 (1:0) | 2012 ACR criteria | F, 12 | TIN | Arthritis, glucosuria |
| Aburiziza et al., 2020 [48] | 5 | 1 (1:0) | Expert opinion | F, 3/5 | NA | Bilateral parotid gland enlargement, severe teeth decay, painful micturition |
| Gottfried et al., 2011 [49] | 5 | 1 (1:0) | European Classification Criteria (1996) | F, 4/9 | CNS involvement | Bilateral conjunctival injection and ptosis, lip and cheek swelling, parotid gland enlargement, dry eyes and mouth |
| Fidalgo et al., 2016 [50] | 5 | 1 (1:0) | Expert opinion | F, 12 | JRA | Dry mouth, tooth sensitivity, dental pain, recurrent parotic gland enlargement |
| Majdoub et al., 2017 [51] | 5 | 1 (1:0) | Expert opinion with histological evidence (labial gland biopsy) | F, 4/7 | NA | Recurrent parotid gland swelling |
| Vermylen et al., 1985 [52] | 5 | 1 (1:0) | Expert opinion with histological evidence (parotid gland biopsy) | F, 2/2 | NA | Combination of parotid gland enlargement, hyperglobulinaemia and interstitial infiltrations of chest radiography suggestive of SS |
| Marino et al., 2017 [53] | 5 | 1 (0:1) | Expert opinion and evidence of cystic changes on parotid gland MRI | M, 2/1 | NMOSD | Left vision loss, right hemiparesis and lethargy associated with dry mouth |
AECG: American European Consensus Criteria; AIH: autoimmune hepatitis; JRA: juvenile RA; NA: not available.