A multisystem chronic autoimmune disorder, Sjogren syndrome, is characterized by lymphocytic infiltrates in the exocrine glands. Keratoconjunctivitis sicca and xerostomia are the two pathognomic findings in Sjogren syndrome. Here, we present a case of a 30-year-oldan old Indian woman presenting with primary Sjogren syndrome. The clinical features, diagnostic modalities, treatment options, and management of complications have been discussed.

Sjogren syndrome (SS) is an autoimmune disease that involves salivary and lacrimal glands. There are rare articles describing hypohidrosis or anhidrosis in SS patients. Little is known about the histologic findings seen in patients with SS who do not report decreased sweating. We analyzed skin samples from 31 patients with SS who had undergone skin biopsies for reasons not related to SS. We assessed the number, distribution and pathologic changes within and around the eccrine ducts, glands and acrosyringia. The composition of the inflammatory infiltrate was additionally accessed utilizing immunohistochemical stains. Overall, in 60% of cases we observed some degree of inflammation. In virtually all cases the inflammatory infiltrate was located around the eccrine ducts at a distance from the non-SS pathology. It was mainly composed of CD3+ T cells with increasing number of B and plasma cells as periductal inflammation intensified. This study is the first histologic analysis of asymptomatic skin in patients with SS. It demonstrates the presence of skin involvement in the majority of SS patients. Our results emphasize the importance of systematic skin evaluation in this category of patients as well as counseling, early preventive measurements and timely treatment when indicated.

Systemic lupus erythematosus (SLE) is a prevalent autoimmune condition, yet its alignment with a specific host immunological pathway remains unclear. The THαβ host immunological pathway, recognized for its defense against viruses and prions, has recently emerged as a response to DNA, RNA, and protein pathogens. SLE patients often produce anti-double strand DNA antibodies and anti-nuclear antibodies, suggesting a potential association with the THαβ host immune reaction. Throughout the course of SLE, elevated levels of type 1 interferons, type 3 interferons, interleukin-10, IgG1, and IgA1 are commonly observed. These cytokines and antibody isotypes are indicative of the THαβ host immunological pathway. Similarly, Myasthenia gravis, Grave’s disease, Graft versus host disease, autoimmune hemolytic anemia, immune thrombocytopenia, dermatomyositis, and Sjogren’s syndrome are also linked to THαβ-related type 2 hypersensitivities. Considering the potential association of these diseases with dysregulated THαβ immune responses, therapeutic strategies such as anti-interleukin-10 or anti-interferons α/β could be explored to manage these disorders effectively.