Abstract
OBJECTIVE: This study reviews the existing literature on patients diagnosed with sarcoidosis isolated to the larynx to improve our understanding of the diagnosis and management of this rare condition. DATA SOURCES: Embase, PubMed, Web of Science. REVIEW METHODS: We conducted a systematic review of published literature pertaining to patients diagnosed with isolated laryngeal sarcoidosis (LS). Demographics, symptomatology, diagnostic evaluation, management, and outcome details were recorded for patients diagnosed with isolated LS. RESULTS: 21 articles with 39 patients (11 male, 28 female, mean age: 33) were included (mean follow-up: 33 months). The most common presentations were dyspnea (74%), dysphonia (67%), and dysphagia (49%). The supraglottic region was most involved (n = 37, 95%). Systemic steroids were the most used initial treatment, leading to symptom resolution in 27% of patients treated as such. 64% of all patients underwent further treatment for refractory symptoms, most commonly surgical excision with intralesional steroid injection. All patients (n = 8) who underwent initial treatment with systemic steroids followed by surgical excision with intralesional steroid injection experienced complete symptom resolution. Emergent tracheostomy was required in 8 (21%) patients during treatment and maintenance therapy was required in 11 (28%) patients. Long-term outcomes (follow-up mean: 33 months; range: 2-192 months) showed 71% complete improvement, 19% partial symptom improvement, and 10% no improvement. CONCLUSION: Isolated LS most commonly affects middle-aged women. Patients primed with a course of systemic steroids before undergoing surgical excision with intralesional steroid injection demonstrated the best outcomes. We recommend long-term follow-up of these patients for surveillance of symptoms and extra-laryngeal sarcoidosis manifestations.