Systemic sarcoidosis and primitive Gougerot-Sjörgen syndrome: a rare, complex yet intriguing association

Abstract

Abstract Primary Sjogren’s syndrome is a chronic autoimmune disease affecting mainly the exocrine glands, typically presenting with sicca syndrome, but systemic multi-organ manifestations are possible. The diagnosis is based on the 2016 ACR-EULAR criteria. On the other hand, sarcoidosis, which is also a chronic systemic disease, is characterized by tissue infiltration with noncaseating granulomas. The granulomas may occur in any organ, but the most frequently affected sites are the lungs, lymph nodes, skin, eyes, and liver. Traditionally, sarcoidosis is considered to be an exclusion criterion for the diagnosis of primary Sjogren’s syndrome, mainly because of overlapping clinical features. We report the case of a 60-year-old female patient of North African descent, hospitalized initially for chronic dyspnea associated with other systemic manifestations such as sicca syndrome, and in whom we have objectified biological and histological features of both systemic sarcoidosis and systemic primitive Sjogren’s syndrome at the same time. The coexistence of sarcoidosis and Sjogren’s syndrome is not frequent and has been rarely reported. We aim to bring more attention to the possibility of an association of these two systemic diseases despite what is typically recommended. Subsequently, it might be of interest to remove sarcoidosis from the elimination criteria in order to avoid the possibility of misdiagnosis.