Relapsing Polychondritis as a Cause of Sudden and Unexpected Death With Central Nervous System Involvement

Abstract

Relapsing polychondritis (RP) is a rare inflammatory disease process that affects cartilaginous tissues throughout the body. Although the pathogenesis remains unknown, RP is thought to be an autoimmune disorder in which host immune cells are conditioned to attack the body's cartilage, such as the ears, nose, eyes, joints, and airways, resulting in inflammation and destruction of otherwise healthy tissues. In rare and unusual cases, neurological involvement has been described.We report a case of a 36-year-old man with a medical history of asthma and suspected seronegative rheumatoid arthritis/RP and panuveitis who was found deceased in his residence. Postmortem examination revealed cartilaginous destruction of the external ear and large airways and meningoencephalitis involving the left medial temporal lobe without an underlying infectious cause.Progressive destruction of airway tissue and increased susceptibility to pulmonary infection is the most common cause of death in RP. Central nervous system involvement is exceedingly rare, presenting with highly variable clinical and pathological manifestations. A review of RP and systemic manifestations will follow. Accurate recognition of this multisystem autoimmune disease as a cause of sudden and unexpected death is critical for proper death certification and to broaden our understanding of this disease.