Relapsing Polychondritis Associated With Primary Sjögren's Syndrome: A Rare Autoimmune Overlap

Relapsing polychondritis is a rare immune-mediated inflammatory disorder characterized by the recurrent inflammation of cartilaginous structures, particularly the auricular, nasal, and respiratory tract cartilage. Airway involvement represents one of the most severe and potentially life-threatening complications. Approximately one-third of relapsing polychondritis cases are associated with other systemic autoimmune diseases. We report the case of a 50-year-old man presenting with inspiratory dyspnea, progressive dysphonia, bilateral auricular and nasal chondritis, inflammatory polyarthralgia, xerostomia, and xerophthalmia. Cervicothoracic computed tomography demonstrated diffuse circumferential thickening of the trachea. Bronchoscopy confirmed inflammatory tracheal involvement. Immunological testing revealed positive antinuclear antibodies with strongly positive anti-SSA and anti-SSB antibodies. Minor salivary gland biopsy showed grade IV chronic lymphocytic sialadenitis. A diagnosis of relapsing polychondritis associated with primary Sjögren's syndrome was established. The patient was treated with intravenous methylprednisolone followed by oral corticosteroids and hydroxychloroquine, resulting in marked clinical improvement. This case highlights the importance of systematic autoimmune screening in patients with relapsing polychondritis and emphasizes the need for early recognition of airway involvement to prevent irreversible structural damage.