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System: Part XIV: Rheumatic Diseases of childhood

A 14-year-old competitive gymnast presents to your clinic complaining of anterior knee pain. She notes the pain is most significant when she is walking up and down stairs or after prolonged sitting. On examination, there is no joint effusion, warmth, or instability, but you elicit pain with patellar distraction. Which of the following is the most likely diagnosis?

A 5-year-old with systemic juvenile idiopathic arthritis presents with new-onset unremitting fever, hepatosplenomegaly, and confusion. Labs show thrombocytopenia, elevated aspartate transaminase, and hyperferritinemia. The erythrocyte sedimentation rate has paradoxically decreased. In addition to high-dose intravenous glucocorticoids, which targeted therapy is most appropriate for the emergency management of this condition?

In diagnosing fibromyalgia using the 2011 criteria, the Symptom Severity Scale (SSS) is calculated. This score is derived from the patient’s rating of three cardinal symptoms over the past week, plus the presence of three other symptoms over the past six months. Which of the following are the three cardinal symptoms rated for severity?

An 11-year-old girl develops severe, burning pain and allodynia in her right foot several weeks after a minor ankle sprain. The foot appears dusky and is cool to the touch. This presentation is concerning for Complex Regional Pain Syndrome (CRPS). In contrast to the adult form of the disease, which anatomic location is most often affected in childhood CRPS?

A pediatric rheumatologist is giving a lecture on Systemic Lupus Erythematosus (SLE). She emphasizes that the disease presentation and course can differ based on the age of onset. When comparing pediatric-onset SLE to adult-onset SLE, which statement is most accurate?

The parents of a child newly diagnosed with Juvenile Dermatomyositis ask about the cause of the disease. You explain that while the exact etiology is unknown, a genetic predisposition is strongly suspected. Which Human Leukocyte Antigen (HLA) allele is most significantly associated with increased susceptibility to this condition?

A 14-year-old with fibromyalgia is referred for psychological intervention to improve her coping skills. The treatment plan focuses on teaching her to control her behavioral, cognitive, and physiologic responses to pain. Which of the following techniques is a core component of this type of intervention?

A pediatric resident notes that a subset of her patients seems particularly prone to developing chronic idiopathic musculoskeletal pain syndromes. Which patient characteristic or personality trait is often described as a predisposition for the development of these pain syndromes?

A pediatric resident is reviewing the pathogenesis of scleroderma. An initial trigger, such as trauma or infection, is thought to injure vascular endothelial cells, initiating a cascade of events. What is the initial key event in the vasculopathy that immediately follows this injury?

An infant presents in the first few months of life with persistent fevers, systemic inflammation, and a violaceous, ulcerative vasculopathic rash on his nose and fingers. He later develops interstitial lung disease and lung fibrosis. This presentation is characteristic of STING-Associated Vasculopathy with onset in Infancy (SAVI), which is caused by a dominant gain-of-function variant in which gene?

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