Background Primary Sjogren Syndrome (pSS) is an autoimmune disease mostly affecting women, characterized by a lymphocyte-mediated infiltration and destruction of several exocrine glands, which causes mucosal dryness. Genital involvement is frequent and characterized by vulvar and vaginal dryness, dyspareunia and pruritus, that significantly impairs sexual function. However, despite the high frequency of genital involvement, few data were published about the histopathology of external genitalia in pSS. The studies performed until now show that vaginal and vulvar dryness are due to the presence of a vulvar inflammatory infiltrate and to the atrophy of minor and major vestibular glands, whose secretions are important for the sexual function.
Objectives To evaluate the presence and the characteristics of histopathological and immunohistochemical changes in vulvar tissues in women with pSS.
Methods Women with pSS (21 patients) underwent vulvar biopsies that have been evaluated for histopathological and immunohistochemical changes and finally compared with those obtained from 26 patients with lichen sclerosus.
Results An inflammatory infiltrate (composed predominantly by T lymphocytes (CD3+), sparse CD20+ B cells and mean CD4:CD8 T-cell ratio of 1.5) was evidenced in all 21 biopsies and classified in mild (10), moderate (11) and severe (0).
No correlation was shown between vulvar inflammatory infiltrate score and salivary Chisholm e Mason score.
No differences were found neither in gynecological symptoms neither in clinical and demographical characteristics between patients with mild and those with moderate vulvar inflammatory score.
A higher prevalence of moderate inflammatory infiltrate was observed in biopsies from women with lichen sclerosus than in pSS.
Conclusions Women with pSS show high prevalence of vulvar inflammation that could explicate the high frequency of gynaecological symptoms, but the severity don't correlate with the grade of histological changes.
The gynaecological evaluation is mandatory in pSS to assess genital involvement and, eventually, to address a therapy targeted to genital symptoms.
Capriello P et al. Sjögren's Syndrome: clinical, cytological, histological and colposcopic aspects in women. Clin Exp Obst Gyn 1988; 15:9–12.
Skopouli FN et al. Obstetric and gynaecological profile in patients with primary Sjögren's syndrome. Ann Rheum Dis. 1994;53:569–73.
Picone O et al. Sjögren syndrome in obstetric and gynecology: literature review. J Gynecol Obstet Biol Reprod (Paris). 2006; 35:169–75.
Sellier S et al. Dyspareunia and Sjögren syndrome. Ann Dermatol Venreol. 2006; 133:17–20.
Mulherin DM et al. Sjögren's syndrome in women presenting with chronic dyspareunia. BR J Obstet Gynaecol. 1997; 104:1019–23.
Maddali Bongi S et al. Gynaecological symptoms and sexual disability in women with primary Sjögren's syndrome and sicca syndrome. Clin Exp Rheumatol 2013;31:683–90.
Priori R et al. Quality of Sexual Life in Women with Primary Sjögren Syndrome. J Rheumatol. 2015 Aug;42(8):1427–31.
Disclosure of Interest None declared
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.