Unusual Presentation of Relapsing Polychondritis in a Patient with Human Immunodeficiency Virus and Reactive Arthritis

Patient: Female, 58-year-old Final Diagnosis: Relapsing polychondritis Symptoms: Ear pain • eye pain Medication: — Clinical Procedure: — Specialty: Rheumatology OBJECTIVE: Rare disease BACKGROUND: Relapsing polychondritis (RP) is an uncommon autoimmune condition that impacts cartilaginous structures involving the ears, nose, respiratory tract, and joints. Its etiology is unknown; however, it may be associated with other systemic autoimmune diseases, malignancy, and rarely with human immunodeficiency virus (HIV) infection. RP has a variable pattern at presentation and may be associated with constitutional symptoms such as fever and arthralgia, in addition to various auricular, ocular, respiratory, and cardiovascular manifestations. Auricular and ocular signs are the most common presenting features; however, idiopathic orbital inflammatory syndrome is considered a rare manifestation of the disease. Systemic corticosteroids are the mainstay of treatment, but immunomodulatory therapy may be required for refractory cases. CASE REPORT: We present a challenging case of RP in a 58-year-old woman with HIV controlled by highly active anti-retroviral therapy (HAART) and stable chronic reactive arthritis on sulfasalazine who developed unilateral auricular chondritis associated with contralateral idiopathic orbital inflammation and scleritis as well as worsening arthralgia. She was initially treated empirically with antibiotics, without clinical improvement. The infectious diseases work-up was unrevealing, and other diagnostic possibilities were meticulously excluded. Clinical suspicion for RP ultimately led to appropriate therapy with corticosteroids and subsequent immunosuppression with methotrexate, resulting in clinical improvement and allowing for gradual tapering of steroids. CONCLUSIONS: RP is an uncommon multisystem disorder that can occur in the setting of other underlying chronic illnesses, as seen in our patient. It has a variable presentation and course, with no diagnostic laboratory tests; therefore, clinical suspicion is imperative for appropriate diagnosis and management.