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Background Large vessels could be involved in immunoglobulin (Ig)-G4-related disease (IgG4-RD). This study aimed to clarify the clinical features and evaluate the treatment efficacy for IgG4-RD with aortitis/periaortitis and periarteritis (PAO/PA). Methods This study prospectively enrolled 587 patients with IgG4-RD with a follow-up time of more than 6 months. The distribution of IgG4-related PAO/PA was classified into four types: type 1, thoracic aorta; type 2a, abdominal aorta; type 2b, abdominal aorta and iliac artery; type 2c, iliac artery; type 3, thoracic and abdominal aorta; and type 4, other arteries. Patient’s demographic data, clinical characteristics, laboratory parameters, and treatment efficacy were analyzed. Results Of 587 IgG4-RD patients, 89 (15.2%) had PAO/PA. The average age was 58.3 ± 11.1 years, with male predominance (85.4%). Vessels affected were as follows: abdominal aorta (83.1%), iliac artery (70.8%), thoracic aorta (13.5%), and other vessels (13.5%). The most prevalent distribution type of IgG4-related PAO/PA was type 2b, with 74 (83.1%) patients, followed by type 2a, type 2c, type 3, and type 1. Fifty-five (61.8%) PAO/PA patients had hydronephrosis, with renal insufficiency occurring in 43 (48.3%), and 31 (34.8%) PAO/PA patients had D-J stent drainage due to severe ureteral obstruction. After treatment with a glucocorticoid and immunosuppressants, 82% patients achieved remission with shrinking of the perivascular mass by more than 30%. Conclusions IgG4-RD with PAO/PA was distinct from non-PAO/PA in demographic features, organ involvement distribution, inflammatory markers, and serum IgG4 and IgE. The most common affected vessel was the abdominal aorta, and most patients responded well with treatment.

Anemia and the need for transfusion of packed red blood cells (PRBCs) are common in preterm infants. PRBC transfusion increases the oxygen carrying capacity of hemoglobin and may result in higher rates of organ dysfunction. To determine whether PRBC transfusion in preterm infants is associated with an increased incidence of bronchopulmonary dysplasia (BPD), this retrospective study was performed on neonates with birth weights ≤ 1,500 g or gestational age ≤ 32 weeks admitted from August, 2008 to November, 2013. Infants who received PRBC transfusion before the diagnosis of BPD and those who did not receive PRBC transfusion or received PRBC transfusion after diagnosis of BPD were compared for incidence of BPD and other morbidities. Of 231 preterm infants, 137 received PRBC transfusion before BPD was diagnosed (group 1) and 94 did not (group 2). The incidence of BPD was significantly higher in group 1 than in group 2 (37.2% vs. 2.1%, P < 0.00001). After adjusting for potential risk factors, the adjusted odds ratio for BPD was 9.80 (95% confidence interval, 1.70–56.36; P = 0.01). This study demonstrated an association between PRBC transfusion and BPD in preterm infants. A cautious approach to PRBC transfusion in these infants is warranted.

Trehalose is a non-reducing disaccharide and can be accumulated in response to heat or oxidative stresses in Candida albicans. Here we showed that a C. albicans tps1Δ mutant, which is deficient in trehalose synthesis, exhibited increased apoptosis rate upon H2O2 treatment together with an increase of intracellular Ca2+ level and caspase activity. When the intracellular Ca2+ level was stimulated by adding CaCl2 or A23187, both the apoptosis rate and caspase activity were increased. In contrast, the presence of two calcium chelators, EGTA and BAPTA, could attenuate these effects. Moreover, we investigated the role of Ca2+ pathway in C. albicans apoptosis and found that both calcineurin and the calcineurin-dependent transcription factor, Crz1p, mutants showed decreased apoptosis and caspase activity upon H2O2 treatment compared to the wild-type cells. Expression of CaMCA1, the only gene found encoding a C. albicans metacaspase, in calcineurin-deleted or Crz1p-deleted cells restored the cell sensitivity to H2O2. Our results suggest that Ca2+ and its downstream calcineurin/Crz1p/CaMCA1 pathway are involved in H2O2 -induced C. albicans apoptosis. Inhibition of this pathway might be the mechanism for the protective role of trehalose in C. albicans.

Objective The aim of this study was to investigate the role of serum IgE levels in the clinical features and outcomes of IgG4-related disease (IgG4-RD). Methods We retrospectively enrolled 459 newly diagnosed IgG4-RD patients with serum IgE examined at baseline from 2012 to 2019 and compared the clinical features between group A (serum IgE level ≤ 60 KU/L) and group B (serum IgE level > 60 KU/L). Subsequently, 312 patients who had been followed up for ≥ 1 year were further selected to evaluate the correlation between serum IgE level and disease outcome. Results At baseline, the serum IgE level was positively correlated with the serum IgG4 level (r = 0.1779, P = 0.0001), eosinophil count (r = 0.3004, P < 0.0001), and serum IgG level (r = 0.2189, P < 0.0001) in IgG4-RD patients. Compared with group A, group B had more patients with allergic diseases (P = 0.004), more organ involvement (P = 0.003), and higher IgG4-RD responder index scores (P = 0.002). During follow-up, group A patients had a higher remission induction rate than group B patients (88.4% vs. 73.6%, P = 0.035), while group B patients had a higher relapse rate than group A patients (29.0% vs. 16.2%, P = 0.039). Multivariate analysis found that a serum IgE level > 125 KU/L at baseline was a risk factor for disease relapse (hazard ratio [HR], 1.894 [95% confidence interval (CI) 1.022–3.508]; P = 0.042). Cox regression analysis showed that elevation of the eosinophil count was a risk factor for relapse in both group A and group B patients (HR, 8.504 [95% CI 1.071–42.511]; P = 0.009; and HR, 2.078 [95% CI 1.277–3.380]; P = 0.003, respectively), and the involvement of the lacrimal gland (HR, 1.756 [95% CI 1.108–2.782]; P = 0.017), submandibular gland (HR, 1.654 [95% CI 1.037–2.639]; P = 0.035), and kidney (HR, 3.413 [95% CI 1.076–10.831]; P = 0.037) were also risk factors for relapse in group B patients. Conclusion IgG4-RD patients with high serum IgE levels at baseline were more likely to have higher disease activity, and baseline high IgE levels were associated with disease relapse.

We report the identification of a novel autoantibody (anti-eIF2B) in a small number of patients with SSc (∼1%); this autoantibody is closely associated with diffuse cutaneous manifestations and the presence of ILD.

Anti-eIF2B antibodies are cytoplasmic SSc-specific autoantibodies with strong clinical associations with dcSSc and SSc-ILD found in ANA-negative sera. Anti-synthetase autoantibodies, and other recently discovered SSc-specific antibodies such as anti-RuvBL1/2, can also be identified in ANA-negative SSc.

Although extensive studies have focused on the development of acute mountain sickness (AMS), the exact mechanisms of AMS are still obscure. In this study, we used isobaric tags for relative and absolute quantitation (iTRAQ) proteomic analysis to identify novel AMS−associated biomarkers in human plasma. After 9 hours of hypobaric hypoxia the abundance of proteins related to tricarboxylic acid (TCA) cycle, glycolysis, ribosome, and proteasome were significantly reduced in AMS resistant (AMS−) group, but not in AMS susceptible (AMS+) group. This suggested that AMS− individuals could reduce oxygen consumption via repressing TCA cycle and glycolysis, and reduce energy consumption through decreasing protein degradation and synthesis compared to AMS+ individuals after acute hypoxic exposure. The inflammatory response might be decreased resulting from the repressed TCA cycle. We propose that the ability for oxygen consumption reduction may play an important role in the development of AMS. Our present plasma proteomic study in plateau of the Han Chinese volunteers gives new data to address the development of AMS and potential AMS correlative biomarkers.