ABSTRACT
Abstract Background Diagnosis of SS is a complex task, as no symptom or test is unique to this syndrome. The American-European Consensus Group (AECG 2002) and the American-European classification criteria of 2016 (ACR/EULAR 2016) emerged through a search for consensus. This study aims to assess the prevalence of Sjögren's Syndrome (SS) in patients with Systemic Lupus Erythematosus (SLE), according to AECG 2002 and ACR-EULAR 2016 classifications, as well as clinical and histopathological features in this overlap. To date, there is no study that has evaluated SS in SLE, using the two current criteria. Methods This cross-sectional study evaluated 237 SLE patients at the outpatient rheumatology clinic between 2016 and 2018. Patients were submitted to a dryness questionnaire, whole unstimulated salivary flow (WUSF), "Ocular Staining Score" (OSS), Schirmer's test I (ST-I), and labial salivary gland biopsy (LSGB). Results After verifying inclusion and exclusion criteria, a total of 117 patients were evaluated, with predominance of females (94%) and mixed ethnicity (49.6%). The prevalence of SS was 23% according to AECG 2002 and 35% to ACR- EULAR 2016. Kappa agreement between AECG 2002 and ACR-EULAR 2016 were 0.7 (p < 0.0001). After logistic regression, predictors for SS were: anti/Ro (OR = 17.86, p < 0.05), focal lymphocytic sialadenitis (OR = 3.69, p < 0.05), OSS ≥ 5 (OR = 7.50, p < 0.05), ST I positive (OR = 2.67, p < 0.05), and WUSF ≤ 0.1 mL/min (OR = 4.13, p < 0.05). Conclusion The prevalence of SS in SLE was 23% (AECG 2002) and 35% (ACR-EULAR 2016). The presence of glandular dysfunction, focal lymphocytic sialadenitis, and anti/Ro were predictors of SS in SLE. The greatest advantage of the new ACR-EULAR 2016 criteria is to enable an early diagnosis and identify the overlapping of these two diseases. ACR- EULAR 2016 criteria is not yet validated for secondary SS and this study is a pioneer in investigating prevalence based on the new criteria.
ABSTRACT
Introducción: la enfermedad relacionada con IgG4 (ER-IgG4) es una afección inmunomediada, asociada con lesiones fibroinflamatorias que pueden ocurrir en casi cualquier órgano. Ante su sospecha clínica es fundamental realizar el examen histopatológico para excluir malignidad y otras enfermedades. El objetivo principal de este estudio fue describir los hallazgos histológicos de la biopsia de glándulas salivales labiales (GSL) en pacientes con diagnóstico de ER-IgG4 y, posteriormente, comparar estos hallazgos con la histología e inmunohistoquímica de la glándula salival mayor (GSM). Materiales y métodos: estudio retrospectivo, observacional y descriptivo sobre una cohorte de 40 pacientes con diagnóstico de ER-IgG4. Se incluyeron 26 pacientes con diagnóstico de ER-IgG4 que presentaron biopsias de GSL. Resultados: el 73% de la muestra tuvo un infiltrado linfoplasmocitario y el 11%, fibrosis con patrón no estoriforme. En ningún caso se halló arteritis obliterativa ni ninguna forma de flebitis. Al realizar la comparación con los hallazgos de las biopsias de GSM, no hubo significancia estadística. El 65% de los pacientes manifestó compromiso extraglandular y fueron los sistemas pancreato hepato biliar y las adenopatías los más afectados. Conclusiones: la biopsia incisional de glándula submandibular sería más útil y apropiada que la biopsia de GSL para un diagnóstico definitivo de ER-IgG4.
Introduction: IgG4-related disease (IgG4-RD) is an immune-mediated condition associated with fibroinflammatory lesions that can occur in almost any anatomical site. Histopathological examination is essential when suspected to diagnose it to exclude malignancy and other diseases. The objective of this study was to describe the histological findings of the minor salivary gland biopsy (GSL) in patients diagnosed with IgG4-RD, and subsequently to correlate these findings with the histology and immunohistochemistry of the major salivary gland (GSM). Materials and methods: retrospective, observational and descriptive study on a cohort of 40 patients diagnosed with RD-IgG4. Twenty-six patients diagnosed with IgG4-RD who presented SLG biopsies were included. Results: the 73% of the samples presented a lymphoplasmacytic infiltrate and 11% of them fibrosis with a non-storiform pattern. In no case was obliterative arteritis or any form of phlebitis found. When comparing the findings of the GSM biopsies, there was no statistical significance. Sixty-five percent of the patients presented extraglandular involvement; the pancreato-hepato-biliary system and the adenopathies were the most affected. Conclusions: incisional biopsy of the submandibular gland would be more useful and appropriate than LSG for a definitive diagnosis of IgG4-RD.