No abstract

Background: Kawasaki disease (KD) is one of the most common vasculitis in childhood. Intravenous γ-immunoglobulin (IVIG) is recommended to be administrated within 10 days after fever onset. However, some patients didn't have IVIG therapies because of atypical disease presentations or spontaneous defervescence. We aimed to evaluate the coronary outcomes of the KD patients who didn't receive IVIG and had defervescence within 10 days. Methods: We retrospectively reviewed the KD patients in NTUCH between 2008 and 2015. The patients with a diagnosis of KD and had a febrile length between 5 and 10 days were enrolled. Days of fever, clinical symptoms, laboratory data at the acute stage, and series of coronary artery measurements within a minimum of 3 months after disease onset were recorded. Risk factors associated with coronary lesions 1 month after KD onset were also analyzed. Results: Two hundred ninety-three eligible KD patients were enrolled (Male: 55.1%, mean age of onset: 1.8 years old). Thirty-seven patients had spontaneous defervescence without IVIG treatment. The incidence of coronary aneurysms at the 4th week after disease onset was higher in spontaneously defervesced KD patients than those treated with IVIG (18.9% vs. 5.1%, p = 0.002). Interestingly, of the 238 KD patients without coronary lesions at their acute phase, percentages of emerging coronary aneurysms became significantly higher if they didn't have IVIG therapies due to spontaneous defervescence (4/31), compared with those who received IVIG (3/208). Further analysis showed the development of coronary lesions at 1 month after disease onset was associated with younger age (<12 months old, p = 0.024), and leukocytosis (WBC > 17,000/cumm, p = 0.031). Conclusions: 18.9% of KD patients with spontaneous defervescence had coronary aneurysms. Even without initial coronary lesions, such patients were still riskier to develop coronary aneurysms, compared with KD patients who received IVIG therapies. Such findings address the importance of refining the strategy for use of IVIG in the spontaneously defervesced KD patients within 10 days after fever onset, at least in those with age younger than 1 year and those with leukocytosis.

Backgrounds Behçet’s disease (BD) is a rare vasculitic disorder affecting all sizes of vessels. Among BD patients, 4 to 25% of patients with diagnosed age younger than 16 years old are defined as juvenile BD (JBD). This study aimed to evaluate the clinical manifestations and treatments of patients with JBD, with a particular focus on the effectiveness and safety of anti-tumor necrosis factor (TNF)-alpha therapy. Methods We retrospectively reviewed data of all patients diagnosed with JBD at age of 16 years or younger in a tertiary hospital in Taiwan. The clinical manifestations, laboratory data, treatments, disease courses, and clinical outcomes were evaluated. The effectiveness of anti-TNF-alpha therapy was measured based on changes in Behçet’s Disease Current Activity Form (BDCAF) scores, prednisolone dosages and the immunosuppression load scores. Results Fifty-five patients were included in the study. The median age at disease onset was 11 years. The most common clinical presentation was recurrent oral aphthous ulcers (100%), followed by genital ulceration (69.1%), skin lesions (36.4%), gastrointestinal symptoms (29.1%), ocular involvement (27.3%), and arthralgia (27.3%). Ninety-one percent of the patients fulfilled the International Criteria for Behçet’s Disease, and 36.4% met the Paediatric Behçet’s Disease criteria. The most frequently used medications were prednisolone (74.5%) and colchicine (54.5%). Six patients with refractory or severe JBD received anti-TNF-alpha therapy. These patients were diagnosed at a younger age compared with those who did not receive anti-TNF-alpha therapy (7.5 vs 13 years; P = 0.012), the BDCAF scores reduced significantly at the 1st month, the 6th month and 1 year after the treatment. They did not use steroids after the first year of treatment, and, after treatment for 6 months, their immunosuppression load scores reduced significantly. Due to the limited case numbers, literature reviews of anti-TNF-alpha therapy for refractory JBD were conducted, which had a total 18 JBD patients receiving anti-TNF-alpha therapy, of which fifteen patients had favorable outcomes after treatment with minimal side effects. Conclusions Anti-TNF-alpha therapy may be necessary for JBD patients with refractory disease courses. Anti-TNF-alpha therapy was effective and safe in these patients, especially regarding its corticosteroid- and immunosuppressive drug-sparing effects.

No abstract

Systemic lupus erythematosus (SLE) patients are vulnerable to infections. We aim to explore the approach to differentiate active infection from disease activity in pediatric SLE patients. Fifty pediatric SLE patients presenting with 185 clinical visits were collected. The associations between both clinical and laboratory parameters and the outcome groups were analyzed using generalized estimating equations (GEEs). These 185 visits were divided into 4 outcome groups: infected-active (n = 102), infected-inactive (n = 11), noninfected-active (n = 59), and noninfected-inactive (n = 13) visits. Multivariate GEE (generalized estimating equation) analysis showed that SDI, SLEDAI-2K, neutrophil‐to‐lymphocyte ratio (NLR), hemoglobin, platelet, RDW-to-platelet ratio (RPR), and C3 are predictive of flare (combined calculated AUC of 0.8964 and with sensitivity of 82.2% and specificity of 90.9%). Multivariate GEE analysis showed that SDI, fever temperature, CRP, procalcitonin (PCT), lymphocyte percentage, NLR, hemoglobin, and renal score in SLEDAI-2k are predictive of infection (combined calculated AUC of 0.7886 and with sensitivity of 63.5% and specificity of 89.2%). We can simultaneously predict 4 different outcome with accuracy of 70.13% for infected-active group, 10% for infected-inactive group, 59.57% for noninfected-active group, and 84.62% for noninfected-inactive group, respectively. Combination of parameters from four different domains simultaneously, including inflammation (CRP, ESR, PCT), hematology (Lymphocyte percentage, NLR, PLR), complement (C3, C4), and clinical status (SLEDAI, SDI) is objective and effective to differentiate flares from infections in pediatric SLE patients.