A Case of Undifferentiated Connective Tissue Disease with Bilateral Auricular Polychondritis Manifestations: A Rare Clinical Association.

Abstract

BACKGROUND Undifferentiated connective tissue disease (UCTD) is a heterogeneous autoimmune condition characterized by clinical features of connective tissue involvement without meeting the full classification criteria for a defined connective tissue disease (CTD). It often includes arthralgias, Raynaud's phenomenon, and serologic abnormalities. In rare cases, UCTD presents with features resembling relapsing polychondritis, raising questions about a possible overlap syndrome or a shared immunopathogenic mechanism. This case report describes an atypical presentation of UCTD with bilateral auricular inflammation and its therapeutic management. CASE REPORT A 45-year-old man diagnosed with UCTD initially presented with acral cyanosis and inflammatory arthralgias, which responded well to hydroxychloroquine and low-dose prednisone. Over time, he developed progressive bilateral auricular pain, erythema, and nodular swelling, mimicking polychondritis. In the absence of systemic features of relapsing polychondritis, this was considered an unusual manifestation within the UCTD spectrum rather than a distinct overlap syndrome. His symptoms improved significantly following an increase in corticosteroid therapy. CONCLUSIONS This case highlights the importance of recognizing atypical inflammatory manifestations in UCTD and adjusting treatment accordingly. The findings suggest auricular inflammation is part of the broader UCTD spectrum rather than a separate disease entity. These observations underscore the role of shared autoimmune pathways in connective tissue disorders and the need for individualized treatment approaches.