Salivary Gland Sarcoidosis: Systematic Review of Case Reports and Case Series

Background: Salivary gland sarcoidosis is a rare manifestation of systemic sarcoidosis that poses a challenge in terms of its diagnosis due to its similarities to disorders such as Sjögren's syndrome, other granulomatous diseases, and infections. Objective: To systematically review reported cases of salivary gland sarcoidosis and summarize clinical presentation, diagnostic methods, treatments, and outcomes. Methods: We conducted a systematic search of PubMed, Scopus, Embase, ScienceDirect, and Medline for case reports and case series published up to April 2025. This review was registered with PROSPERO (CRD42024629263) and was conducted following PRISMA guidelines. Variables assessed included age, sex, presenting symptoms, location, duration of symptoms, treatment approaches, and outcomes. Study quality assessment was assessed using The Joanna Briggs Institute (JBI) Critical Appraisal tools. Results: A total of 28 articles involving 39 participants met the inclusion criteria, with a mean age of 42.7 years. Salivary gland sarcoidosis predominantly affected female patients (66.7%). The parotid gland was the most frequently involved site (82.1%). Common presenting features included glandular swelling that is usually painless, xerostomia, and facial palsy. Sarcoidosis was newly diagnosed in 82.1% of cases, primarily through histopathological examination revealing non-caseating granulomas. Systemic corticosteroids were the most common treatment. Outcomes were favorable in nearly all cases, with complete resolution post treatment or spontaneous remission without treatment. Conclusions: Salivary gland sarcoidosis predominantly affects middle-aged women, typically presenting as a painless parotid swelling and often serving as the initial sign of systemic disease. Diagnosis requires histopathological confirmation via biopsy, as serum ACE levels are insufficient alone. The prognosis is excellent, with most patients responding favorably to corticosteroids or even experiencing spontaneous resolution. This condition must be considered in differential diagnoses for persistent salivary gland swellings to ensure accurate diagnosis and prevent unnecessary interventions.