Relapsing polychondritis: A clinical review

Objective: This study comprehensively reviews the literature related to relapsing polychondritis (RP). Methods: A detailed search via MEDLINE (PubMed) was performed using relapsing polychondritis as the key term. Relevant articles were analyzed with a focus on history, epidemiology, etiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of RP. Results: RP is a rare episodic and progressive inflammatory disease of presumed autoimmune etiology first described in 1923, RP affects cartilage in multiple organs, such as the ear, nose, larynx, trachea, bronchi, and joints. In addition, it can affect proteoglycan-rich tissues, such as the eyes, aorta, heart, and skin. The diagnosis of RP is based on the presence of clinical criteria. A standardized therapeutic protocol for RP has not been established. Nonsteroidal anti-inflammatory drugs, dapsone and/or colchicine, may control disease activity in some patients. In other patients, immunosuppressive drugs and prednisone have been effective, RP is a potentially lethal disease; pulmonary infection, systemic vasculitis, airway collapse, and renal failure are the most common causes of death. Earlier studies indicate survival rates between 70% at 4 years and 55% at 10 years. In a recent study, a survival rate of 94% at 8 years may be due to improved medical and surgical management. Conclusions: RP is a rare, multisystemic, and potentially fatal disease. The pathogenesis and optimal therapeutic approach to patients with RP is poorly understood

Optimising drug therapy for non-infectious uveitis

Introduction Uveitis encompasses a wide variety of sight-threatening diseases characterized by intraocular inflammation. It is often classified as infectious and non-infectious uveitis. Unlike infectious uveitis, a distinct infectious agent cannot be identified in non-infectious uveitis and disease origin is usually autoimmune, drug related, or idiopathic. The Issue at Hand Non-infectious uveitis can often have a relapsing-remitting course, making it difficult to treat, and poses a significant challenge to ophthalmologists. The autoimmune nature of non-infectious uveitis warrants the use of anti-inflammatory and immunomodulatory agents for disease control. However, a subset of patients has persistent or recurrent ocular inflammation despite appropriate treatment, stressing the need for newer therapies aimed at more specific inflammatory targets such as tumour necrosis factor (TNF) alpha agents, anti-interleukin agents, and anti-interleukin receptor agents. Objectives This article discusses the various medical options available for the treatment of non-infectious uveitis in the light of the most recent evidence. Conclusion Successful management of non-infectious uveitis requires the clinician carefully balance advantages and disadvantages of each new and old therapy while considering individual circumstances. Counselling regarding the benefits and complications of each therapy can help patients make an informed choice

The use of biologic agents in the management of uveitis

The uveitides are a heterogenous group of ocular inflammatory disorders that account for the third highest cause of blindness worldwide, responsible for 5–10% of visual impairment globally. Up to 35% of patients with uveitis can suffer significant vision loss. To prevent irreversible structural damage and blindness, it is important that the diagnosis and commencement of appropriate therapy occurs promptly. Management includes topical and systemic corticosteroid therapy and conventional immunomodulatory agents, including methotrexate, azathioprine, mycophenolate mofetil and cyclosporin. Significant progress has been made in the past decade in our understanding of the immunopathological pathways that drive intraocular inflammation, allowing the development of targeted therapy with biologic agents. These include TNF-α inhibitors, such as infliximab, adalimumab and etanercept; interleukin blockers, such as tocilizumab and daclizumab; and other targeted therapies, such as rituximab and abatacept. The efficacy of these agents has been studied in cases of severe uveitis that are refractory to conventional immunomodulatory agents and provide exciting results that have revolutionised uveitis management. Though the biologic era has provided a large armamentarium to treat uveitis, ongoing challenges and cases of recalcitrant uveitis remain, posing a challenge to internal medicine physicians. This comprehensive review aims to construct an updated summary on the existing evidence pertaining to the use of biologic agents in the treatment of uveitis. Methods include a systematic search for studies between 2000 and 2018 using PubMed, EMBASE, Ovid MEDLINE and Cochrane libraries