Clinical analysis of systemic onset juvenile idiopathic arthritis complicated with interstitial lung disease / 中华风湿病学杂志

ABSTRACT

Objective:To investigate the clinical characters and prognosis of patients with systemic-onset juvenile idiopathic arthritis associated interstitial lung disease (SoJIA-ILD).Methods:Clinical manifestations, imaging features and prognosis were analyzed in 75 patients with SoJIA between October 2010 and December 2019 in the Second Affiliated Hospital of Wenzhou Medical University.Results:Seventy-five patients with SoJIA were enrolled. Among 12 children with SoJIA-ILD, 6 were male and 6 were female. The mean age of onset was (7.9±2.6) years. All of the 12 patients had fever. Ten patients had arthritis which mainly occurred in large joints. The incidence of arthritis was knee, hip and shoulder from high to low. Two patients had no joint involvement at the onset of the disease and had no joint symptoms during the follow-up. Nine patients (75%) had fever, rash and arthritis at the same time. The clinical features of ILD were mostly nonspecific, including cough in 8 cases (75%), shortness of breath in 7 cases (58%), chest pain in 3 cases (25%), velcro sound in 4 cases (33%) and pulmonary hypertension in 1 case (8%). Inflammatory indicators were all signifi-cantly elevated, among which was CRP (235±112) mg/L, ESR (39±25) mm/1 h, serum ferritin (SF) (1 312±384) ng/ml and serum amyloid A (SAA) (212±101) mg/L. High resolution computed tomography (HRCT) of the chest presented as reticular or line shadows in 12 patients, consolidation in 7 patients, ground interlobular septal thickening in 5 patients, glass opacity in 4 patients and honeycomb lung in 1 patient. ILD occurred in 4 cases (33%) in the early stage of SoJIA (disease course ≤6 months), and 8 cases (67%) in the medium and late stages of the disease course (>6 months), but all appeared in the active status of SoJIA. All of 12 patients received glucocorticoids therapy, 11 patients received high dose of glucocorticoid (>1 mg·kg -1·d -1) and 2 pa-tients received intravenous methylprednisolone pulse therapy. All of 12 patients were treated with glucocorti-coids combined with immunosuppressant or disease modifying antirheumatic drugs and 5 patients needed dual therapy or triple therapy. One case had been treated with biological agents before the occurrence of lung injury and the other 11 cases had not used biological agents before. After the diagnosis of SoJIA complicated with ILD, 4 cases were treated with tocilizumab. Macrophage activation syndrome (MAS) was found in 7 cases and 25% had MAS for two times or more. Ten patients had partial remission or complete remission and 2 patients died of respiratory failure. Conclusion:SoJIA-ILD maybe asymptomatic at the early stage of the disease. It is associated with disease activity of SoJIA. HRCT examination is very important for early diagnosis. Patients with SoJIA-ILD have higher rate of recurrence, death and MAS. It needs to arouse the clinicians' attention.