Juvenile idiopathic arthritis presenting with rare extra-articular manifestations: A report of two cases

Abstract

Juvenile idiopathic arthritis (JIA), a common rheumatic disease of childhood, is a consolidated term used for all chronic childhood arthritis affecting joints and extra-articular structures. Involvement of skin and internal organs such as liver, spleen, intestine, peritoneum, pleura, pericardium and myocardium will cause systemic symptoms leading to disability and mortality. Despite advanced treatment options, significant morbidity still occurs due to an unpredictable clinical course and risk of joint restriction. We describe polyarticular rheumatoid factor-negative JIA with extra-articular manifestations involving pericardium causing chronic constrictive pericarditis in a 16-year-old girl and her sibling a 13-year-old boy. The female patient developed cardiac cirrhosis, portal hypertension and oesophageal varices. Screening evaluation of this patient’s asymptomatic sibling with similar articular manifestations revealed the rare occurrence of chronic constrictive pericarditis without symptoms. Both were treated with pericardiectomy and were relieved of symptoms. Two patients of JIA from the same family presenting with constrictive pericarditis is a rare association.

Keywords

• arthritis
• constrictive pericarditis
• extra-articular manifestations
• juvenile idiopathic
• pericardiectomy