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Adult onset Still's disease (AOSD) is a rare clinical entity with unknown etiology, characterized by arthritis, fever, erythematous rash, and other systemic presentations. We report a case of a 21-year-old male who presented with high spiking fever, dry cough, generalized body ache, arthralgia, and an erythematous rash. He was eventually diagnosed to have AOSD based on the Yamaguchi criteria, after a month of visiting three different healthcare facilities and receiving two misdiagnoses and treatment regimes not specific to his diagnosis. The patient immediately responded to prednisoloneand was healthy upon discharge.

Autoinflammatory diseases are rare group conditions manifested by recurrent fevers, systemic inflammation, and dysfunctions of the innate immune system. These conditions are characterized by overproduction or lack of inhibition of various cytokines, and the advent of biologic drugs that block specific cytokines involved in these conditions have revolutionized their treatment. In this review, I will discuss the most common autoinflammatory conditions of adulthood including Familial Mediterranean Fever, cryopyrin-associated periodic syndrome, mevalonate kinase deficiency/hyperimmunoglobulinemia D Syndrome, TNF receptor-associated autoinflammatory syndrome, and systemic juvenile idiopathic arthritis/adult-onset Still's disease. I will discuss how IL-1, IL-6, IL-18, and TNF play pathogenic roles in these conditions and will review the evidence behind cytokine blockade for these diseases. Throughout the paper, I will reflect on gaps in knowledge of autoinflammatory diseases and will highlight the latest advances and newest drugs in development.

Adult onset Still's disease (AOSD) is a rare inflammatory disorder of unknown etiology. It is a diagnosis of exclusion. We report a case of post-splenectomised man, presented with high grade fever, joint pain and body ache. Since Overwhelming Post Splenectomy Infection (OPSI) was the initial probable diagnosis, empirical antibiotic therapy was initiated. Evaluation to find a septic focus, autoimmune diseases ad malignancies was carried out which showed negative results. Since alternative diagnoses were excluded and the Yamaguchi's criteria for AOSD was fulfilled, the patient was treated with IV steroids which resulted in rapid resolution of his symptoms. AOSD with asplenia is a unique condition because immunosuppressive therapy for AOSD may increase the risk of OPSI in such a patient. This is the first case report of AOSD in an asplenic patient from India.

Adult onset Still's disease (AOSD) is an uncommon systemic inflammatory disease on the clinical spectrum of autoinflammatory disorders. Its presentation and clinical course may result in several well-differentiated phenotypes: from a systemic and highly symptomatic pattern to a chronic articular pattern. Overproduction of numerous pro-inflammatory cytokines is observed in AOSD. Anakinra (ANK), a human interleukin (IL)-1R antagonist, has recently been approved in the EU for the treatment of AOSD. Areas covered: In this review, we discuss the main studies on the efficacy and safety on ANK for the treatment of AOSD. The vast majority of them are retrospective studies and case series. Expert commentary: Overall, ANK is an effective biologic agent for the treatment of AOSD, especially for the systemic pattern and also for those patients who have life-threatening complications, which frequently occur over the course of the disease. The initial dose usually indicated of ANK in adults is 100 mg/day subcutaneously, although dose reduction can be performed in some cases once the disease is under control. The safety profile of ANK is favorable and similar to that described in other rheumatic diseases. In conclusion, ANK is an effective and safe agent for the treatment of AOSD.