Validation of New Criteria in “The Guidance for Diagnosis of Sjögren’s Syndrome in Pediatric Patients”

Minako Tomiita¹, Ichiro Kobayashi², Yuzaburo Inoue³, Nami Okamoto⁴, Naomi Iwata⁵, Yukiko Nonaka⁶, Ryoki Hara⁷, Hiroaki Umebayashi⁸, Yasuhiko Itoh⁹ and Masaaki Mori¹⁰, ¹Department of Allergy and Rheumatology, Chiba Children's Hospital, Chiba, Japan, ²Department of Allergy and Rheumatology, Chiba Children’s Hospital, Sapporo, Japan, ³Department of Pediatrics, Chiba University Graduate School of Medicine, Chiba, Japan, ⁴Pediatrics, Graduate School of Medicine, Osaka Medical College, Takatsuki, Japan, ⁵Department of Immunology and Infectious Diseases, Aichi Children’s Health and Medical Center, Obu, Japan, ⁶Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan, Kagoshima, Japan, ⁷Department of Pediatrics, Yokohama City University, Graduate School of Medicine, Yokohama, Japan, ⁸Department of Rheumatics, Miyagi Children’s Hospital, Sendai, Japan, ⁹Department of Pediatrics, Nippon Medical School, Tokyo, Japan, ¹⁰Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: diagnostic criteria and pediatrics, Sjogren's syndrome

Session Information

Date: Monday, November 6, 2017

Title: Sjögren's Syndrome Poster II: Clinical Research

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Sjögren’s syndrome (SS) has been thought rare during pediatric age. Because patients in this age group lack gsicca symptomsh, the diagnosis of SS is often difficult by currently available diagnostic or classification criteria, and accordingly heeds new criteria. Japanese Pediatric Sjögren’s syndrome Study Group has developed new criteria for diagnosis of SS in pediatric patients (Table 1,2). This criteria has been approved by both the board of he Pediatric Rheumatology Association of Japan and the board of the Japanese Society for Sjögren’s syndrome. In this criteria, patients are classified into 5 groups: definite, probable, possible, need follow-up or non-SS. In the present study, we compared the sensitivity of our criteria with 4 major criteria, and evaluated which criteria is most suitable for identifying SS patients in the early stage.

Methods: We enrolled 41 pediatric patients who were diagnosed as having SS with unanimity by 10 pediatric rheumatologists. They were categorized into primary SS (pSS), secondary SS (sSS) and primary to secondary SS (psSS), which was diagnosed as primary SS at diagnosis but developed other collagen diseases during follow-up. We classified those patients into 5 groups according to our criteria, and examined whether each patient fulfilled the major criteria: revised American-European Consensus Group classification criteria (AECG), the revised Japanese diagnostic criteria (JPN), ACR classification criteria (ACR), American College of Rheumatology/European League Against Rheumatism Classification Criteria for primary Sjögren’s syndrome (A/E).

Results: The numbers of patients were as follows: pSS 25, sSS 11 and psSS 5. According to our criteria, patients were classified into definite 33, probable 2 or possible 6 at the first visit, and 38, 3 or 0 at the last visit. No patient was diagnosed as non-SS. In the other 4 criteria, the most sensitive was JPN, followed by A/E and ACR among all patientsf group (Table 3). However, even by using JPN criteria, there was 12% of pSS patients diagnosed as non-SS at the last visit.

Conclusion: Our new criteria is useful for diagnosis of pediatric SS, and make it possible to recognize SS-associated complications at an early stage.

Table 1: Scoring

			Score
Serological score		serum IgG	1 (≥97.5th percentile for age)
		anti-nuclear antibody	1 (1:40~1:80), 2 (1:160), 3 (≥1: 320)
		Rheumatoid factor	3 (≥15U/ml)
		anti-SSA/Ro or SSB/La antibody	6 (positive)
Glandular score	Salivary gland	Labial salivary gland biopsy	1 (<1 focus / 4mm²), 2 (≥1 focus / 4mm²)
		Solography (conventional or MRI)	2 (Rubin-Holt stage ≥1)
		Salivary scintigraphy	1 (Deceased in uptake or secretion)
		Decreased salivary flow (counted if at least one other test is positive)	1 (Saxon test ≤ 2.0g / 2min, or Salivary flow rate ≤1.5ml / 15min, or Gum test ≤10ml / 10min)
	Lacrimal gland		2 (Schirmer test <5mm/5min and Rose-Bengal test van Bijsterveld score ≥3, or Schirmer test <5mm/5min and fluorescein test (+), or ACR score ≥3)

Table 2: Classification

Serological score	Glandular score
Serological score	≥2	1	0
≥6	Definite	Probable	Possible
5	Probable	Probable	Possible
4	Probable	Probable	Possible
3	Probable	Possible	Need follow-up
2	Probable	Possible	Need follow-up
1	Possible	Possible	Need follow-up
0	Need follow-up	Need follow-up	Possibly non-SS

Table 3: Sensitivity of each criteria

		1st visit			Last visit
		pSS	sSS	psSS	pSS	sSS	psSS
AECG fulfilled		20 (%)	18.2	20	36	36.4	20
AECG (objective items) fulfilled		28	–	40	40	–	–
JPN fulfilled		72	90.9	100	88	100	100
ACR fulfilled		52	72.7	100	72	72.7	100
A/E fulfilled		60	–	100	76	–	–
Our criteia	Definite	72	90.9	100	88	100	100
	Probable	8	0	0	12	0	0
	Possible	20	9.1	0	0	0	0

Disclosure: M. Tomiita, None; I. Kobayashi, None; Y. Inoue, None; N. Okamoto, None; N. Iwata, None; Y. Nonaka, None; R. Hara, None; H. Umebayashi, None; Y. Itoh, None; M. Mori, None.

To cite this abstract in AMA style:

Tomiita M, Kobayashi I, Inoue Y, Okamoto N, Iwata N, Nonaka Y, Hara R, Umebayashi H, Itoh Y, Mori M. Validation of New Criteria in “The Guidance for Diagnosis of Sjögren’s Syndrome in Pediatric Patients” [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/validation-of-new-criteria-in-the-guidance-for-diagnosis-of-sjogrens-syndrome-in-pediatric-patients/. Accessed .

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