S4333 Multisystem Sarcoidosis Presenting as Oropharyngeal Dysphagia: A Diagnostic Challenge

Introduction: Sarcoidosis is a multisystem granulomatous disease with a higher prevalence among women and African Americans. It involves the lungs and mediastinal lymph nodes in over 90% of cases. Extrapulmonary manifestations can involve the skin, eyes, liver, and heart. However, gastrointestinal involvement is rare (<1%). Even more uncommon is laryngeal sarcoidosis, seen in only 0.5–5% of cases, often presenting with hoarseness and/or dysphagia due to mass effect or vocal cord dysfunction. Case Description/Methods: A 68-year-old African American woman with a history of hypertension presented with progressive dysphagia, dry cough, and unintentional weight loss of 14 pounds over the past 3 months. Initial evaluation included a modified barium swallow, which showed oropharyngeal dysphagia. Computed tomography (CT) of the neck revealed soft tissue prominence in the oropharynx. Further workup included an upper endoscopy, that was unrevealing, and a laryngoscopy, which revealed left oropharyngeal fullness and right vocal cord paralysis, suggestive of neuromuscular dysfunction. Due to significant weight loss and dysphagia, which was initially concerning for malignancy, a CT abdomen and pelvis was ordered. It showed multiple enhancing splenic lesions, the largest measuring 5.7 cm. positron emission tomography/CT showed intense fluorodeoxyglucose uptake in the spleen, gastric wall, mediastinal and hilar lymph nodes, and multiple bones, raising concern for disseminated malignancy. Laboratory studies revealed mildly elevated ACE 56 U/L and CRP 13 mg/L, elevated lysozyme at 20.9 mcg/mL and elevated 1,25 dihydroxy Vitamin D. She had a negative flow cytometry, ANA, Quantiferon® Gold, and negative CEA, CA 125, CA 19-9, CA 27-29, CA 15-3. Iliac bone biopsy revealed non-caseating granulomas. Endobronchial ultrasound-guided biopsies of the mediastinal lymph nodes revealed granulomatous inflammation without malignant cells, confirming a diagnosis of sarcoidosis. Discussion: Laryngeal sarcoidosis presenting as oropharyngeal dysphagia due to laryngeal nerve compression is an extremely rare and often missed diagnosis, with only a few cases reported in the literature. This case highlights the need for a high index of suspicion for sarcoidosis in atypical presentations, especially when imaging points toward malignancy and no overt lung involvement.