ABSTRACT
To investigate the clinical significance of soluble tumour necrosis factor receptor (sTNF-R) II/I ratio as an indicator of the diagnosis of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA), we measured the serum sTNF-RI and II levels in 117 patients with s-JIA, including 29 patients with MAS, 15 with Epstein-Barr virus-induced haemophagocytic lymphohistiocytosis (EBV-HLH), 15 with Kawasaki disease (KD) and 28 healthy controls (HCs). We determined their correlation with measurements of disease activity and severity. Furthermore, we measured serum interleukin (IL)-18 levels in patients with EBV-HLH and compared these in levels in patients with MAS. The sTNF-RII/I ratio was elevated significantly in MAS and EBV-HLH patients compared with those in the acute phase of s-JIA and KD patients, whereas there were no significant differences between HCs and those in the acute phase of s-JIA. The sTNF-RII/I ratio increased profoundly as MAS developed and correlated positively with disease activity. Serum IL-18 levels were elevated significantly in MAS patients compared with EBV-HLH patients. The monitoring of serum IL-18 and sTNF-RII/I might be useful for the diagnosis of MAS and the differentiation between MAS and EBV-HLH.
Subject(s)
Arthritis, Juvenile/diagnosis , Epstein-Barr Virus Infections/diagnosis , Herpesvirus 4, Human/immunology , Lymphohistiocytosis, Hemophagocytic/diagnosis , Macrophage Activation Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/diagnosis , Receptors, Tumor Necrosis Factor, Type II/blood , Receptors, Tumor Necrosis Factor, Type I/blood , Acute Disease , Arthritis, Juvenile/complications , Arthritis, Juvenile/immunology , Child , Child, Preschool , Diagnosis, Differential , Disease Progression , Epstein-Barr Virus Infections/immunology , Female , Humans , Interleukin-18/blood , Lymphohistiocytosis, Hemophagocytic/immunology , Macrophage Activation Syndrome/complications , Macrophage Activation Syndrome/immunology , Male , Mucocutaneous Lymph Node Syndrome/immunology , Up-RegulationABSTRACT
A 6-year-old girl who had been diagnosed as hyper immunoglobulin-E syndrome, was admitted to the department of pediatrics of our institute in May 2006, because of pulmonary aspergillosis. The chest X-ray showed bilateral cavities with niveau and fungus ball in the left middle lung field. In spite of medical treatment by antibiotics and antimycotics, the lesions did not improve. Therefore, bilateral lobectomy was done. After surgery, she needed re-operation twice, because of prolonged air leakage. There are few reports of lung surgery for the patient with the hyper immunoglobulin-E syndrome, and we present our case and review previous 2 case reports in the Japanese literature.