Relapsing Polychondritis with Central Nervous System Involvement: Experience of Three Different Cases in a Single Center

Abstract

Relapsing polychondritis (RP) is an autoimmune disorder characterized by inflammation in cartilaginous structures including the ears, noses, peripheral joints, and tracheobronchial tree. It rarely involves the central nervous system (CNS) but diagnosis of CNS complication of RP is challenging because it can present with varying clinical features. Herein we report 3 cases of relapsing polychondritis involving CNS with distinct manifestations and clinical courses. The first patient presented with rhombencephalitis resulting in brain edema and death. The second patient had acute cognitive dysfunction due to limbic encephalitis. He was treated with steroid pulse therapy and recovered without sequelae. The third patient suffered aseptic meningitis that presented as dementia, which was refractory to steroid and immune suppressive agents. We also reviewed literature on CNS complications of RP.

Patient Perception of Disease‐Related Symptoms and Complications in Relapsing Polychondritis

“…Despite therapeutic advancements in a number of rheumatologic diseases, patients with RP report substantial glucocorticoid requirements at high doses during times of disease activity. The need for high doses of glucocorticoids to control flares have been reported by others (23,24). While the majority of patients in this study report use of glucocorticoid-sparing therapies, multiple different medications were detailed, highlighting a need for clinical trials and subsequent treatment guidelines in RP.…”

Relapsing polychondritis: state of the art on clinical practice guidelines

“… 37 For articular involvement, Tc-99m bone scintigraphy may reveal active sites of inflammation. 38 Cerebral MRI may be useful if central nervous system involvement is suspected. 39 Otorhinolaryngoscopic examination reveals conductive and sensorineural hearing loss in about 40% of cases, sometimes along with nasal ulcerations, septal perforations, saddle nose deformity, arytenoid swelling and vocal cords deformities.…”

Relapsing Polychondritis with Meningoencephalitis Refractory to Immunosuppressant Therapy

“…Neurologic complications of RP are rare in general; in the literature limbic encephalitis was reported and the patient presented with cognitive dysfunction [ 4 ]. Another case reported rhombencephalitis in which the patient presented with severe occipital headache and this resulted in brain edema and death in two months [ 5 ]. Most of the reported cases in the literature reported vasculitic encephalitis, limbic encephalitis, and rarely aseptic meningoencephalitis.…”