Seung-Ki Kwok

doi:10.4078/jrd.2019.26.4.219

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands that results in dry eye and dry mouth [1]. The prevalence of pSS varies, ranging from 0.03%~2.7% [2]. Patients with pSS suffer from sicca symptoms. However, the inflammatory process can extend beyond exocrine glands and therefore they may also experience other organ involvement such as arthralgia, arthritis, Raynaud's phenomenon, cytopenia, interstitial nephritis, and vasculitis. In addition, some of the patients experience severe fatigue [3]. Therefore, it is can be speculated that patients with pSS have reduced health-related quality of life [4].

Clinical features of pSS patients can be divided into two aspects: (1) benign but disabling patients' symptoms such as dryness, pain and fatigue that affect most patients and (2) systemic manifestations that affect 20%~40% of patients [5]. Different indexes were developed for evaluation of both disease facets: (1) for patients' disabling symptoms, such as Profile of Fatigue and Discomfort (PROFAD) [6] and Sicca Symptoms Inventory (SSI) [7] and (2) for systemic features, such as Sjögren's syndrome Disease Activity Index [8] and Sjögren's Systemic Clinical Activity Index [9]. However, theses scores are either non-exhaustive or quite long. Therefore, nowadays theses indexes are rarely used because it is very complicated to use in routine daily clinical practice.

Thus, the European League Against Rheumatism (EULAR) has sponsored an international collaboration aiming to develop both systemic disease activity index and patient reported outcome measurement. As a result, EULAR Sjögren's syndrome disease activity index (ESSDAI) and EULAR Sjögren's syndrome patient reported index (ESSPRI) have been developed [5 10]. Both ESSDAI and ESSPRI have now been validated as outcome measures in independent cohort and have been used in many clinical studies [11]. ESSPRI, which consists of a numerical rating scale for dryness, pain, and fatigue, reflects benign and disabling subjective symptoms of pSS patients [10]. ESSPRI is simple and easy to perform in real clinical practice. In addition, ESSPRI is one of determinant of health-related quality of life [4]. However, no report has investigated serial changes in routine clinical practice for pSS.

A recent paper by Kim et al. [12] published in Journal of Rheumatic Diseases investigated serial changes in ESSPRI of Korean pSS patients in routine clinical practice. They also examined the clinical features associated with favorable ESSPRI changes in pSS patients. At baseline and after a median period of 6.6 years, 41 pSS patients were evaluated using the ESSPRI, ESSDAI, short-form 36 (SF-36), xerostomia inventory (XI), and visual analog scale (VAS) scores for symptoms. Median ESSPRI increased from 4.11 to 5.33 (p<0.05). But, ESSDAI scores was not changed. Favorable subgroup (n=17) whose definition was based on temporal ESSPRI changes exhibited profoundly lower VAS scores for sicca and depression and XI and ESSPRI scores at baseline (all p<0.05) and more lacrimal flow (p<0.05). So, Kim et al. [12] could draw a conclusion that long-term patient patient-derived outcomes might depend on residual exocrine function at pSS diagnosis because the favorable subgroup had more lacrimal flow and less sicca symptoms at baseline.

The study by Kim et al. [12] is meaningful in some aspects. First of all, it is a first paper showing the serial ESSPRI changes for the relatively long follow-up period in Korean pSS patients although the number of patients is small. Additionally, they investigated longitudinal changes in not only ESSPRI but also ESSDAI and clinical features associated with favorable ESSPRI changes in Korean patients with pSS.

There have been several reports showing the utility of ESSPRI in the management of pSS patients [4 11 13]. The study by Kim et al. [12] also demonstrated the utility of ESSPRI especially in routine daily practice. Thus, the rheumatologists could consider the application of ESSPRI in daily routine practice when managing the pSS patients.

Notes

^{CONFLICT OF INTEREST} No potential conflict of interest relevant to this article was reported.

References

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Longitudinal Changes of the European League Against Rheumatism Sjögren's Syndrome Patient Reported Index in Korean Patients with Primary Sjögren's Syndrome

Notes

References