Treatment
Standardized guidelines for treatment do not exist. Treatments should be chosen based on severity of disease. Mild disease, presenting with recurrent chondritis and arthritis without evidence of systemic involvement, can be treated with nonsteroidal anti-inflammatory drugs, dapsone, or colchicine. Refractory disease often requires high-dose systemic corticosteroids.5
Severe systemic involvement leads to increased mortality and warrants more aggressive treatment.22 Commonly used agents include the immunosuppressants cyclophosphamide, cyclosporine, and methotrexate. Tumor necrosis factor α inhibitors have been the most widely utilized immunomodulatory agent for treatment of RP.25,26 Abatacept and rituximab also have been used with variable efficacy in patients with severe disease. Recently, the IL-6 receptor blocker tocilizumab has been used with some success.27
Prognosis
The prognosis for patients with RP largely depends on the severity of disease and degree of internal involvement. With improved management, largely due to awareness and recognition of disease, the survival rate among RP patients has increased from 55% at 10 years to 94% at the end of 8 years.18 The main cause of death in RP patients is airway complications related to laryngotracheal involvement.10 The second most common cause of death is cardiovascular complications in which valvular disease predominates.5
Concomitant Illness
Thirty-five percent of RP patients have coexisting autoimmune disease, the most common being antineutrophil cytoplasmic antibody–associated vasculitis.5,28 Although this association with autoimmune disease is well described, reports of RP occurring in other states of immune dysfunction are sparse. One case of RP has been reported in a child with common variable immunodeficiency thought to be related to underlying abnormal immune regulation and immunodeficiency.29 Relapsing polychondritis has been described in 4 patients with HIV, 2 of whom had concomitant autoimmune disease.7-9
Human immunodeficiency virus infection is a well-established cause of immune dysregulation and has variable association with autoimmunity. This variability depends largely on the stage of infection. When divided into stages, autoimmune diseases develop predominantly in stage I, during acute infection with an intact immune system; in stage III, with immunosuppression, a low CD4 count, and development of AIDS; and in stage IV, when the immune system is restored with the institution of highly active antiretroviral therapy.6 The interplay between HIV infection and development of autoimmune disease is complex, and pathogenesis remains speculative.
Conclusion
Our patient represents a case of RP in an HIV-positive patient. Additionally, our patient had no other identifiable autoimmune conditions but did have a strong family history of them. It is important for providers to be aware of the potential for development of RP as well as other autoimmune disease in the setting of HIV infection. The implications of a missed diagnosis could be dire because the disease course of RP is progressive and has the potential to decrease survival.