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A CASE REPORT OF A CHILD WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS - A RARE BUT POTENTIALLY DANGEROUS DISEASE

Liliya Raeva, Boris Botev, Paola Kulicheva, Veselin Boyadzhiev, Lachezar Marinov

Abstract

Introduction: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of conditions unified by the presence of chronic childhood arthritis without an identifiable cause. Systemic juvenile idiopathic arthritis (sJIA)  is a rare form of this group characterised by systemic autoinflammation. It contributes to about two-thirds of the total mortality rate of JIA and is frequently complicated by macrophage activation syndrome. Clinical manifestations of the disease can be non-specific in the beginning and as a result the diagnosis can be delayed.

Materials and Methods: We present a 13-year-old boy hospitalized at the St. Marina University Hospital, Varna with complaints of a fever over 38oC accompanied by a rash on the trunk. After physical examination, hyperemic throat, erythematous rash and cervical lymphadenopathy were discovered. The laboratory tests showed leukocytes - 19.32x109/L and CRP -  203.78 mg/l. Echocardiography and computed tomography revealed pericardial effusion and splenomegaly. The patient was diagnosed with acute pericarditis and treated with antibiotics but his condition was not improving. After a month he developed symptoms such as dyspnea, cough and joint pains and was admitted urgently to the Hospital. Further examinations were performed showing hemoglobin- 114 g/l, leukocytes - 23.37x109/L and pleural effusion in addition of the previous objective symptoms.

Results: Due to the clinical presentation, the patient was diagnosed with sJIA. Now he is successfully treated with tocilizumab and methotrexate.  

Conclusion: The clinicians should be aware of sJIA as a disease with various clinical features in the beginning and the resemblance to infectious or malignant diseases. Early diagnosis and effective treatment are essential to achieve the best outcomes for the patients and to prevent fatal complications.


Keywords

systemic juvenile idiopathic arthritis, children, high mortality rate, non-specific clinical manifestation


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