The role of indices in predicting disease severity and outcomes of multisystem inflammatory syndrome in children.

BACKGROUND: The aim of this study was to evaluate the role of the systemic immune inflammation index (SII), C-reactive protein/albumin ratio (CAR), the monocyte/lymphocyte ratio (MLR), and the neutrophil/lymphocyte ratio (NLR) in predicting disease severity, treatment, and prognosis in multisystem inflammatory syndrome in children (MIS-C). METHODS: This medical record review retrospectively evaluated the clinical and laboratory findings of 191 MIS-C patients followed in the Department of Pediatric Rheumatology at Ankara City Hospital, Turkey. The patients were grouped by disease severity: mild, moderate, and severe. SII, CAR, MLR, and NLR were calculated for each group. RESULTS: All patients had fever at the time of admission; 153 (80.1%) had gastrointestinal tract involvement, 74 (38.7%) had rash, 63 (33%) had conjunctivitis, 107 (56%) had cardiac involvement, 32 (15.6%) had renal involvement, and 143 (74.9%) had hematological involvement. According to logistic regression analysis, SII, NLR, MLR, and CAR were found to be predictive indexes for disease severity, need for intensive care, need for inotropes, and anakinra treatment in MIS-C. The cut-off values of ≥1605.3 for SII, ≥9.1 for NLR, and ≥3.9 for CAR increased the risk of severe disease by 3.4, 7.1, and 5.7 times, respectively. CONCLUSION: NLR, SII, MLR, and CAR are effective and useful for predicting the severity of MIS-C, the need for intensive care, and the need for anakinra treatment.

A case series of intracardiac thrombi and vascular involvement in pediatric Behçet's disease.

To evaluate the general characteristics of pediatric Behçet's disease (BD) patients with thrombus and to present the clinical features, treatment responses and prognosis of patients with intracardiac thrombus. The clinical characteristics and outcomes of 15 patients with thrombus among 85 pediatric BD patients followed in the Department of Pediatric Rheumatology were evaluated retrospectively. Of the 15 BD patients with thrombus, 12 (80%) were male, 3 (20%) were female. The mean age at diagnosis was 12.9 ± 1.1 years. Thrombus was present at the time of diagnosis in 12 patients (80%), while thrombus developed in three patients within the first three months after diagnosis. The most common site of thrombus was the central nervous system (n = 9, 60%), followed by deep vein thrombus (n = 6, 40%) and pulmonary artery thrombus (n = 4, 26.6%). Three male patients (20%) developed intracardiac thrombus. The overall intracardiac thrombus rate in the 85 patients was 3.5%. Two of the three patients had thrombus in the right, and one had thrombus in the left heart cavity. In addition to steroids, 2 of the 3 patients received cyclophosphamide, while the patient with thrombus localized in the left heart cavity was given infliximab. In the follow-up, the two patients with thrombus in the right heart cavity were switched to infliximab because of resistance to cyclophosphamide. Complete resolution was observed in 2 of the 3 patients on infliximab; a significant reduction in the thrombus of the other patient was achieved. Intracardiac thrombus is a rare presentation of cardiac involvement in BD. It is usually observed in males and in the right heart. Although steroids and immunosuppressive agents such as cyclophosphamide are recommended as first-line treatment, favorable outcomes can be achieved with anti-TNFs in resistant cases.

Intravenous immunoglobulin therapy in immunoglobulin A vasculitis with gastrointestinal tract involvement.

OBJECTIVE: The aim of this study is to evaluate the outcomes of patients who received intravenous immunoglobulin (IVIG) for immunoglobulin A vasculitis (IgAV) with gastrointestinal (GI) tract involvement, and to determine the differences between the groups that responded to IVIG and those that did not. METHODS: This retrospective study comprised 152 patients with IgAV between 2018 and 2022. Sixty-five patients (43%) had GI tract involvement. Patients with IgAV-GI involvement who had been treated with IVIG were evaluated. Patients were classified with IgAV according to the 2008 Ankara-EULAR/PRINTO/PRES. Their demographics, presentation, and management are reported. RESULTS: Twelve (7 boys/5 girls) of these patients were treated with IVIG. The median age was 90.1 (31-177) months. The mean follow-up period was 30.6 ± 9.9 months. All patients had skin involvement, joint involvement (arthralgia or arthritis), and abdominal pain. All 12 patients were given steroids (30 mg/kg/day pulse methylprednisolone for 3-7 days, followed by 2 mg/kg/day steroids) before IVIG. Nine patients received cyclophosphamide treatment (four before IVIG and five after IVIG). Complete remission was achieved in 5 of the patients with IVIG. Four patients were diagnosed with IgAV concomitant familial Mediterranean fever, and colchicine treatment was initiated. CONCLUSIONS: IVIG may be used in steroids and/or immunosuppressive drug resistant IgAV. It can be considered as a treatment option, especially in patients with multi-organ/system involvement, comorbid inflammatory diseases such as familial Mediterranean fever, and in patients with IgAV-GI tract involvement resistant to standard treatment in the advanced pediatric age group.