Juvenile/ pediatric rheumatology & autoinflamatory diseases Exam with Questions and Solutions

It covers key domains which include; Juvenile Idiopathic Arthritis (JIA), Systemic Autoimmune Rheumatic Diseases (SARDs), Autoinflammatory Diseases and Other Inflammatory and Musculoskeletal Conditions. A 7-year-old child presents with intermittent fever for 2 weeks, evanescent salmon-colored rash, hepatosplenomegaly, and arthritis. Laboratory tests show markedly elevated ferritin and leukocytosis. Which subtype of juvenile idiopathic arthritis best fits this presentation? a. Oligoarticular JIA b. Systemic JIA c. Polyarticular RF-positive JIA d. Enthesitis-related arthritis Correct Answer: b Rationale: Systemic juvenile idiopathic arthritis is characterized by quotidian fever, evanescent rash, hepatosplenomegaly, lymphadenopathy, and systemic inflammation. Very high ferritin and systemic features strongly suggest macrophage-driven inflammation typical of systemic JIA. A 6-year-old girl is diagnosed with oligoarticular juvenile idiopathic arthritis. Which complication requires routine long-term screening even if joint symptoms are mild? a. Interstitial lung disease b. Anterior uveitis c. Renal amyloidosis d. Pulmonary hypertension Correct Answer: b Rationale: Oligoarticular JIA, especially in ANA-positive patients, carries a high risk of chronic anterior uveitis, which can be asymptomatic but may lead to vision loss. Regular slit-lamp ophthalmologic screening is essential.