Background/Purpose: Sjögren’s syndrome (SS) is a systemic disease characterized by dry eyes and mouth resulting from immune mediated damage and dysfunction of the lacrimal and salivary glands. Clinical diagnosis often takes 6-10 years, leading to a lag in potential preventive and therapeutic strategies. Research classification is most often based on the American European Consensus Group (AECG) criteria. For classification as primary SS (pSS), the AECG criteria require ≥4/6 components with at least 1 being autoantibodies or abnormal histopathology. A significant number of subjects with sicca manifestations have “incomplete” syndrome (iSS) and exhibit less than 4 AECG criteria. We describe the clinical and serologic features of a subgroup of iSS patients.

Methods In a multidisciplinary sicca clinic, we assessed features of salivary and lacrimal gland dysfunction and autoimmunity as defined by AECG criteria, identifying 573 iSS participants. We compared the features of iSS based on the presence or absence of anti-Ro/SSA autoantibodies (Ro(+) iSS and Ro(-) iSS, respectively.  

Results Of 573 iSS participants, 467 had complete clinical and laboratory data; 19 of them were Ro(+) (4.1%), and 448 were Ro(-) (95.9%). When compared to Ro(-) iSS, Ro(+) iSS patients were younger (43 ± 5.72 vs. 53 ± 13.27 p=0.001) and less often Caucasian (52.6% vs. 95.7%, p=1.95 x 10^-7); had more anti-La/SSB (+) (46.2% vs. 2.1%, p=8.8 x 10E-6), hypergammaglobulinemia and lymphopenia (p=0.02 and p=0.009, respectively). Furthermore, Schirmer’s I test scores, ocular surface staining, and whole unstimulated salivary flow were less abnormal than the Ro(-) iSS participants (19.05 ± 9.85 vs. 14.19 ±10.55, p=0.05; 1.5±1 vs. 2.84 ± 2.25, p<0.0001; and 5.77 ± 3.66 vs. 2.32 ± 2.3, p<0.001 respectively). These differences in age, race, anti-La, hypergammaglobulinemia, and lymphopenia were also statistically significant when comparing the Ro(+) iSS subjects to Biopsy(+) iSS or Ro(-)/Biopsy(-) iSS.   Finally, Ro(+) iSS patients presented a variety of extraglandular manifestations: 6 had hypothyroidism and/or autoimmune thyroid disease, 5 arthritis/arthralgias, 5 leuko and/or lymphopenia, 5 low CH50, 4 hypergammaglobulinemia, 3 interstitial lung disease, 2 Raynaud’s phenomenon, 2 photosensitivity, and 1 neuromyelitis optica.

Conclusion A small percentage of patients with iSS have anti-Ro autoantibodies but do not meet the classification criteria for SS.  It is possible that these patients will remain as a forme frustre of SS or anti-Ro positive undifferentiated connective tissue disease, but given their younger age and multiple manifestations, it is plausible that they may progress to pSS. Of any possible line of autoimmune disease prevention research, understanding the early events of the disease have the strongest potential to lead to improvements in prevention, early diagnosis, and therapeutics. Thus, this group of patients warrants careful follow up to characterize the transition from iSS to full-blown pSS and pinpoint indicators of early autoimmunity that may help identify those at risk for further disease progression.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/anti-rossa-positive-incomplete-sjogrens-syndrome/