Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

New Classification Criteria for Childhood Sjgren's Syndrome.

Yokogawa8,  Naoto, Lieberman1,  Scott M., Bout-Tabaku3,  Sharon M., Alawi7,  Faizan, Palazzo6,  Juan P., Guttenberg2,  Martha, Sherry5,  David D.

Children's Hosp of Philadelphia, Philadelphia, PA
Children's Hosp of Philadelphia
Nationwide Children's Hospital, Columbus, OH
Penn Presbyt Med Ctr, Philadelphia, PA
The Children's Hospital of Philadelphia, Philadelphia, PA
Thomas Jefferson Univ.
Univ. of Pennsylvania
Univ. of Pennsylvania/Tokyo Metropolitan Tama Medical Center


To develop new classification criteria for childhood Sjögren's syndrome (SS).


Adult classification criteria for SS have not been validated in children and currently proposed pediatric criteria lack diagnostic sensitivity.


Retrospective review of medical records of 41 patients (pts) was performed at Children's Hospital of Philadelphia and the Penn Sjögren Center to identify cases of suspected pediatric SS. Diagnosis by physician global assessment required 1) symptom onset at age <20 yrs, 2) consensus agreement on diagnosis by pediatric and adult rheumatologists, 3) abnormal serologies and/or lip biopsy. Pts with overlapping autoimmune disease were not excluded. Clinical presentation, serologies, objective tests for sicca and histopathology were reviewed. All available salivary gland biopsies were blindly reviewed by oral pathologists to calculate a focus score (FS) and compared to biopsy findings from non-SS pts. Pts were also classified as childhood SS (score:3 or more) or non SS (score 2 or less) using a novel scoring system (maximum = 8) based on a modification of American-European Consensus Group (AECG) criteria (Vitali et al, 2002).

Sicca symptomsDey eye (I) AND dry mouth/parotid swelling (II)*12
 Dry eye (I) OR dry mouth/parolid swelling (II)1
 No sicca symptoms0
Objective findingsAbnormal eye exams (III) AND abnormal salvary flow (V)2
 Abnormal eye exams (III) OR abnormal salivary flow (V)1
 Normal eye exams and normal salivary flow0
Laboratory findingsPositive SSA and/or SSB (VI)2
 Positive ANA AND Negative SSA/SSB1
 Negative ANA/SSA/SSB0
Biopsy findingsFocus score>= 1 OR characteristic findings of SS*22
 Presence of focus AND Focus Score<11
 Absence of focus0
Childhood SS: total score>=3 points
*1 Include parotid enlargement by radiological study
*2 Parotid biopsy may be sufficient to diagnose SS.


Following global assessment and long term follow-up of the pediatric cohort (41 pts), 35 pts had confirmed childhood SS, 3 pts had suspected but unconfirmed SS, and 3 pts were diagnosed as HIV, sarcoidosis, or IPEX syndrome. Using the novel scoring system, all 35 pts with definite childhood SS were classified as SS and all 3 pts with other final diagnosis were classified as non SS.

Figure 1.

An expert oral pathologist blindly calculated FS in 21 pts. Among 17 pts with definite childhood SS, FS and Classification Score were positively correlated (R=0.67 p<0.005) and 8 pts had FS less than 1.

Figure 2.


Inclusion of the presence of ANAs and any focal lymphocytic sialadenitis (both more clinically significant in children than adults) in classification criteria for childhood SS can be used to maximize diagnostic sensitivity in order to facilitate earlier treatment and prevent long-term complications.

To cite this abstract, please use the following information:
Yokogawa, Naoto, Lieberman, Scott M., Bout-Tabaku, Sharon M., Alawi, Faizan, Palazzo, Juan P., Guttenberg, Martha, et al; New Classification Criteria for Childhood Sjgren's Syndrome. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1907
DOI: 10.1002/art.29672

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