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YOUR PATIENT

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A 23-year-old female with a history of juvenile idiopathic arthritis (JIA), currently in remission, presents for contracture release in hands.

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Physical examination shows a well-appearing 55-kg female with decreased motion in hands bilaterally.

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PREOPERATIVE CONSIDERATIONS

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Juvenile idiopathic arthritis, also known as juvenile rheumatoid arthritis, is the most common autoimmune disease of childhood and is characterized by chronic joint inflammation. The etiology and pathogenesis are largely unknown. There is a strong genetic predisposition that is very complex, with multiple genes being responsible for disease onset and manifestations. Humoral and cell-mediated immunity are both involved in the pathogenesis of JIA, and T lymphocytes play a central role in releasing proinflammatory cytokines.

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The incidence of JIA is about 4-14 cases per 100,000 children annually, with a prevalence of 1-86 per 100,000 children. It is more commonly found in girls than in boys by a ratio of 3:1, but systemic onset occurs with equal frequency in girls and boys. JIA, by definition, is arthritis beginning before age 16, but the age of onset is often much earlier, usually around the age of 1-3 years. Advances in pharmacologic therapy for JIA over the past 20 years have dramatically improved the prognosis and quality of life for these children, and most of them can lead productive lives when the disease is in remission.

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JIA is a clinical diagnosis of exclusion, where arthritis must be present in at least one joint for 6 weeks before a diagnosis can be made. The disease onset can be abrupt or insidious and can be characterized by morning stiffness or stiffness after long periods of sitting or inactivity. Children usually do not complain of pain, but parents will notice that the child will stop using the affected joints normally. There are three subsets of JIA. Oligoarticular JIA, accounting for 50% of cases, occurs when fewer than 5 joints are affected, mainly knees. Polyarticular JIA accounts for 20%-40% of JIA cases and affects more than 5 joints, most commonly the small joints of the hands. Systemic-onset JIA, also known as Still’s disease, can involve the cervical spine, jaw, hands, hips, and shoulders and is manifested by spiking fevers with a predictable pattern, occurring 1-2 times a day and around the same time every day. The child appears systemically ill. It is also accompanied by an evanescent rash on the trunk and extremities that lasts for a few hours; this rash is typically nonpruritic, macular, and salmon-colored.

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There are no recognized diagnostic serologic tests available. On physical examination, there is either intra-articular swelling or limited joint motion with pain, warmth, or erythema of the joint. Preoperative assessment mainly focuses on airway management because JIA involvement of the temporomandibular joint (TMJ) and mandibular joint, which occurs in more than 60% of children, limits mouth opening. As a result, the mandibular growth is stunted, and micrognathia results. ...

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