EPIDEMIOLOGICAL AND CLINICAL PROFILE OF PATIENTS WITH SJÖGREN SYNDROME IN A TERTIARY HOSPITAL
Name: Wildner Mardegan Sardenberg Calhau
Type: MSc dissertation
Publication date: 01/03/2019
Advisor:
Name |
Role |
|---|---|
| Valéria Valim Cristo | Advisor * |
Examining board:
| Name |
Role |
|---|---|
| Érica Vieira Serrano | Internal Examiner * |
| SAMIRA TATIYAMA MIYAMOTO | Internal Examiner * |
| Valéria Valim Cristo | Advisor * |
Summary: Introduction: Little is known about the epidemiological and clinical characteristics of primary Sjögren's Syndrome (pSS) in Brazil. There are still gaps in the etiology, epidemiology, diagnosis, prognosis and treatment of the disease, despite frequent studies in the area. Comparison between registries and epidemiological studies in other countries has the limitation of using different classification criteria, diagnostic methods and ethnic differences. Thus, due to difficulties in generalizing the epidemiological and clinical aspects of the disease, this type of study becomes important in our country. In addition to these characteristics, it is also important to evaluate the predictors of mortality and injury. Objective: To describe the epidemiological and clinical profile of pSS patients in a tertiary hospital, including the description of disease activity indexes, damage index and salivary gland biopsy findings, as well as the frequency of new and previous classification criteria; the evaluation of predictors of severity, damage and mortality and the relationship between histological changes of the salivary glands with clinical manifestations, symptoms and damage index. Method: Retrospective cross-sectional study of pSS cases by reviewing charts with epidemiological, clinical and laboratory data and evaluation of salivary gland biopsy slides. Results: A total of 104 patients with pSS were eligible for inclusion in the study. There was a predominance of women (20 women: 1 man), in perimenopausal age, mean age of 51.49 years, married, of brown color, coming from metropolitan region of Espírito Santo, with 5.5 years of follow-up and diagnostic delay of 4 years. All patients completed at least 1 of the 3 most recent pSS classifications - AECG 2002, ACR 2012, ACR-EULAR 2016. This new criterion had the highest sensitivity. At diagnosis, 92.3% had xerophthalmia and 91.3% had xerostomia; 60%, with Schirmer I test ≤ 5 mm; 52.2%, with OSS ≥3; 21.7%, with OSS ≥5; 80% with salivary flow ≤ 01ml / min; 68% with anti-Ro/SSA; 30.3%, with anti-La/SSB; 82% with positive ANA; 37.4% with positive FR ; 73.7% with focal score ≥1.
Most had low current disease activity with 74% at low activity (ESSDAI <5), 17.3% moderate activity (ESSDAI 5 to 13) and 8.7% high activity (ESSDAI> 13). There was a predominance of hematological (22/104 patients) and biological (42/104 patients) domains at the present time and predominance of articular (30/104 patients) and biological domains (28/104 patients) at diagnosis. It was present moderate symptoms indexes (ESSPRI with an average of 5.62 ± 2.56), some damage by the SSDDI index (mean of 1.77 ± 1.25), with 7.7% lung damage and 1.9% lymphoproliferative damage. There were 3 deaths in the study, 2 of which were due to advanced lung disease and 1 by infection. The high disease activity at diagnosis (2.89 ± 0.93 versus 1.86 ± 1.53 versus 1.58 ± 1.07, p = 0.002), disease time (correlation coefficient 0.242, p = 0.018) and initial respiratory distress (correlation coefficient 0.286, p = 0.004) were the predictors of damage and it was demonstrated that the presence of respiratory dominance at the onset of the disease increased the chance of 9-fold damage [OR = 8.996 (1.480-54.694), p = 0.017] regardless of disease duration. Being younger (1.76 ± 1.26 versus 1.14 ± 0.76, p = 0.017), it have a higher focal score (49.62 ± 12.62 versus 54.74 ± 10.61, p = 0.038) and have higher erythrocyte sedimentation rate (ESR) (55.9% versus 31.4%, p = 0.021) at the present time were predictors of severity, as well as presented elevation of gammaglobulin (41.2% versus 15.8%, p = 0.041), IgG (41.3% versus 11.8%, p = 0.022) and ESR (52.7% versus 25%, p = 0.028) at diagnosis. The presence of the articular domain at diagnosis increased the chance of developing higher indexes of current symptoms (55.2% versus 44.8%, p = 0.047), especially with pain ESSPRI (52.5% versus 47.5%, p = 0.021) and dryness ESSPRI (54.9% versus 45.1%, p = 0.035). Higher mortality was associated with greater activity in the initial and current respiratory domain (p <0.05). Most of the biopsies, 73.7% (72/99 biopsies), consisted of focal lymphocytic sialoadenitis with a score focal ≥ 1, with degree of inflammation 4 (58.8%, n = 57/97), some grade of acinar atrophy (70 (86.41%, n = 70/81) and about half with fatty infiltration (48,75%, n=39/80). Focal lymphocytic sialoadenitis with a score focal ≥1 was associated with higher CRP (2.0 ± 5.45 versus 0.43 ± 0.88, p = 0.04) and there was a positive correlation with total SSDDI (0.391, p = 0.09). Acinar atrophy was associated with age (p <0.001), menopause (p = 0.043), dryness symptoms (ESSPRI dryness ≥5) (p = 0.018) and higher disease activity (ESSDAI> 5) (p = 0.017). The adipose infiltration was associated with age (p = 0.016) and lacrimal dysfunction (Schirmer I test ≤ 5) (p <0.05). Conclusion: The study describes the epidemiological and clinical characteristics of a part of patients with SSp in the Brazilian population. It shows that the new ACR-EULAR 2016 qualifying criterion is more sensitive. It confirms that precocious age, greater focal score, elevation of gammaglobulins, IgG and erythrocyte sedimentation rate are markers of greater severity. In an unprecedented way, it shows that joint involvement and respiratory involvement at the onset of the disease are predictors, independent of chronic non-inflammatory pain and damage, respectively.
Key words: Sjögren's syndrome, epidemiological characteristics, clinical characteristics, mortality predictor, damage predictor.
