Successful treatment of secondary macrophage activation syndrome with emapalumab in a patient with newly diagnosed adult-onset Still's disease: case report and review of the literature.

Here, we present a 22-year-old female patient with adult-onset Still's disease (AOSD) who was newly diagnosed in the setting of secondary macrophage activation syndrome (MAS), a rare, life-threatening inflammatory disease with 50% mortality due to multi-organ failure. She met the diagnostic criteria of AOSD and MAS, while genetic testing excluded primary causes of MAS. She had high fevers, anemia, thrombocytopenia, splenomegaly, hematophagocytosis, and elevated serum ferritin (37,950 ng/mL) and CD25 levels (11,870 pg/mL), which remained unresponsive to corticosteroids and anakinra. Her serum interferon gamma (IFN-γ) levels were elevated (7 pg/mL). She was markedly responsive to IFN-γ blockade with emapalumab that eliminated her fevers and all MAS-associated laboratory abnormalities. This report provides initial evidence for therapeutic efficacy for IFN-γ blockade in AOSD and secondary MAS.

Hydroxychloroquine-Induced Toxic Myopathy Causing Diaphragmatic Weakness and Lung Collapse Requiring Prolonged Mechanical Ventilation.

A 42-year-old woman with juvenile idiopathic arthritis was treated with anakinra, corticosteroids, and hydroxychloroquine when she developed chronic hypoxic respiratory myopathy. She was admitted to the intensive care unit for acute hypercapnic respiratory failure and required prolonged intubation, subsequent tracheostomy, and long-term ventilatory support due to multiple failed spontaneous breathing trials after discontinuation of anakinra and steroids. Muscle biopsy revealed type II fiber atrophy with the accumulation of autophagosomes and vacuoles presenting as curvilinear bodies, elevated MHC class I antigen expression, and infiltration by CD68+ macrophages and CD8+ T cells. Type II fiber atrophy was attributed to corticosteroid use and curvilinear bodies due to blockade of autophagy by hydroxychloroquine. After hydroxychloroquine was discontinued, the patient recovered to her prehospitalization baseline.

Low Vitamin D is Associated With Infections and Proinflammatory Cytokines During Pregnancy.

Vitamin D is known to regulate innate and adaptive immune processes at the cellular level, but the role of vitamin D status on associated inflammatory processes across pregnancy is unclear. Our primary objective was to evaluate the relationships between serum biomarkers of inflammation (interleukin [IL]-6, IL-10, tumor necrosis factor [TNF]-α), acute-phase proteins (C-reactive protein and hepcidin) and vitamin D status, 25-hydroxyvitamin D (25(OH)D) and 1,25-dihydroxyvitamin D (1,25(OH)2D), measured across pregnancy and in the neonate at birth. A second objective was to identify associations between vitamin D status and clinically diagnosed infections. In this study, 158 racially and ethnically diverse pregnant adolescents were recruited from the Rochester Adolescent Maternity Program (RAMP) in Rochester, NY. Serum 1,25(OH)2D was significantly lower in adolescents and neonates with IL-6 concentrations above the 75th percentile at delivery ( P = .04) and at birth ( P = .004), respectively. After adjusting for other potential covariates of inflammation, maternal serum 1,25(OH)2D was significantly positively associated with TNF-α during pregnancy ( P = .02), but at delivery 1,25(OH)2D and TNF-α were inversely associated with one another ( P = .02). Teens with 25(OH)D concentrations <30 ng/mL were more likely to test positive for candida ( P = .002) and bacterial vaginosis ( P = .02) during pregnancy. African Americans exhibited significantly lower TNF-α concentrations at both mid-gestation ( P = .009) and delivery ( P = .001) compared to the Caucasian adolescents. These results suggest that lower maternal vitamin D status may increase risk of infection across gestation.

Prevalence and Risk Factors for Infections in a Pregnant Adolescent Population.

STUDY OBJECTIVE: Our objective was to identify risk factors associated with maternal infections and placental inflammation in pregnant adolescents attending an urban adolescent maternity clinic. DESIGN: This cross-sectional, descriptive study used survey and medical chart data collected at entry and prospectively across gestation. The prevalence of maternal infections and placental inflammation was determined and potential risk factors were identified. SETTING: Rochester Adolescent Maternal Program (RAMP) in Rochester, NY. PARTICIPANTS: Racially and ethnically diverse pregnant adolescents (n = 158 ≤ 18 y at entry) were recruited. INTERVENTIONS AND MAIN OUTCOME MEASURES: Main outcome measures were diagnosis of an infection or inflammatory condition in relation to demographic, anthropometric, dietary, socioeconomic, and health data. RESULTS: The three most prevalent infections diagnosed in this study population were recto-vaginal colonization of group B Streptococcus (GBS) (38%), bacterial vaginosis (BV) (40%) and candida (42%). African-American teens (AOR = 4.6; 95% CI: 1.74-13.02) and those with higher pre-pregnancy BMI (ppBMI; AOR = 1.2; 95% CI: 1.04-1.31) were more likely to test positive for BV across gestation. Older maternal age decreased the likelihood of positive tests for trichomoniasis (OR = 0.51; 95% CI: 0.26-0.92) and gonorrhea (OR = 0.38; 95% CI: 0.16-0.82). Higher mean dietary vitamin D intake (mcg/d) was associated with a lower likelihood of testing positive for recto-vaginal GBS (OR = 0.87; 95% CI: 0.77-0.98). CONCLUSION: Addressing modifiable risk factors associated with dietary intake and pre-pregnancy weight may help reduce health disparities among pregnant minority adolescents. Additionally, targeted sexual health education may greatly benefit younger female adolescents.