Cricoarytenoid Arthritis in a Patient With Sjögren's Syndrome

Abstract

BACKGROUNDPrimary Sjögren's syndrome (pSS) is a slowly evolving chronic inflammatory disease that affects the exocrine glands leading to the sicca syndrome. Extraglandular systemic manifestations are frequent, but laryngeal involvement is very rare. To our knowledge, only four cases in the medical literature have been reported. We report a case of cricoarytenoid arthritis in a patient with pSS. CASE REPORTA 50-year-old patient with pSS presents the following manifestations: sicca syndrome (Schirmer test < 5 mm/5 min in both eyes), symmetric nonerosive polyarthritis, Jaccoud's arthropathy, ANA+, anti-Ro+, anti-La+, anti-CCP-, anti-Sm-, anti-DNA-, C3 consumption. Disease was controlled with the use of methotrexate and artificial lubrication. After 10 years of disease, she began to present progressive dysphonia, dysphagia, odynophagia, and marked weight loss (20 kg in 3 months, body mass index decreased to 12 kg/m²). She had voluntarily stopped taking methotrexate 1 year before. Muscle strength and creatine phosphokinase levels were normal. Gastrointestinal endoscopy without findings. Computed tomography scan of the neck showed only calcification of the salivary glands. Fiberoptic endoscopic evaluation of swallowing showed hyperemia and edema in arytenoids, salivary stasis and paresis of the right vocal fold. Prednisone 1 mg/kg/day was started, resulting in complete resolution of symptoms within 1 month and recovery of 10 kg within 2 months. Methotrexate was restarted. CONCLUSIONLaryngeal involvement in pSS is uncommon and may manifest as dysphagia, dysphonia, dyspnea, and even acute airway obstruction. Important differential diagnoses are infections (especially tuberculosis) and laryngeal carcinoma. Laryngoscopy and/or fiberoptic endoscopic evaluation of swallowing are necessary tests to evaluate this condition. Inflammation without lesions in the laryngeal mucosa is compatible with cricoarytenoid arthritis associated with connective tissue diseases. Corticosteroids have been used successfully in the management of these patients.