Nonspecific interstitial pneumonia in refractory systemic juvenile idiopathic arthritis responded to tocilizumab treatment
Maynart Sukharomana,1 Kanokporn Udomittipong,2 Ruchira Ruangchira-urai,3 Sirirat Charuvanij1
1 Division of Rheumatology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
2 Division of Pulmonary Medicine, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
3 Department of Pathology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
Abstract
Background: Nonspecific interstitial pneumonia (NSIP) is a rare pulmonary complication in systemic juvenile idiopathic arthritis (SJIA).
Objective: To present a case with NSIP in SJIA
Methods: Case report
Results: We report the case of a 4-year-old boy with SJIA complicated by macrophage activation syndrome (MAS) refractory to conventional therapy, and who later developed NSIP confirmed by high-resolution computerized tomography of the chest and lung histopathology. The patient received tocilizumab, a monoclonal antibody to interleukin-6 receptor, to control his disease. Data relating to tocilizumab treatment of NSIP in refractory SJIA is limited.
Conclusions: The data from this case report suggests that NSIP could be a pulmonary complication of SJIA complicated by MAS that is refractory to conventional therapy. Early initiation of tocilizumab should be considered to achieve disease remission in this pediatric patient population.
Key words: macrophage activation syndrome, nonspecific interstitial pneumonia, refractory systemic juvenile idiopathic arthritis, tocilizumab, interleukin-6