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BACKGROUND: Pulmonary arterial hypertension consists in an increase of mean pulmonary arterial pressure (PAPm ≥ 25 mmHg), and may lead to right ventricular failure. Pulmonary arterial hypertension can arise in several disorders, encompassing inflammatory conditions and connective tissue diseases. The occurrence of pulmonary arterial hypertension has recently been reported in monogenic interferonopathies and in systemic lupus erythematosus, highlighting the pathogenic role of type I interferons and paving the way to therapies aimed at inhibiting interferon signaling. CASE: We describe a 17-year-old boy with DNase II deficiency, presenting a clinical picture with significant overlap with systemic lupus erythematosus. During treatment with the Janus kinase inhibitor ruxolitinib, he developed pulmonary arterial hypertension, raising the question whether it could represent a sign of insufficient disease control or a drug-related adverse event. The disease even worsened after drug withdrawal, but rapidly improved after starting the drug again at higher dosage. SUMMARY AND CONCLUSION: Pulmonary arterial hypertension can complicate type I interferonopathies. We propose that ruxolitinib was beneficial in this case, but the wider role of Janus kinase inhibitors for the treatment of pulmonary arterial hypertension is not clear. For this reason, a strict cardiologic evaluation must be part of the standard care of subjects with interferonopathies, especially when Janus kinase inhibitors are prescribed.

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In a series of studies it was demonstrated that activating the self is sufficient to increase social comparison tendencies. Treating the relevant constructs as individual differences that can be measured as well as contextual variables that can be manipulated, the authors show that individual differences in self-activation are correlated with interest in social comparison information and that manipulations of self-activation cause changes in interest in social comparison. Self-certainty often has been portrayed as the primary determinant of social comparison interest. The present results suggest that self-activation affects interest in social comparison even when self-certainty is controlled.

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Using cellular automata, the authors show how mutual influences among elements of self-relevant information give rise to dynamism, differentiation, and global evaluation in self-concept. The model assumes a press for integration that promotes internally generated dynamics and enables the self-structure to operate as a self-organizing dynamical system. When this press is set at high values, the self can resist inconsistent information and reestablish equilibrium after being perturbed by such information. A weak press for integration, on the other hand, impairs self-organization tendencies, making the system vulnerable to external information. Paradoxically, external information of a random nature may enhance the emergence of a stable self-structure in an initially disordered system. The simulation results suggest that important global properties of the self reflect the operation of integration processes that are generic in complex systems.

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In a series of four studies, the self-evaluation maintenance (SEM) model is used to predict peoples' self-reported affective responses to doing better or worse than their partners. Both self-protective reactions to comparison (i.e., those predicted by the original SEM model) and empathic reactions to the partner's response (i.e., those predicted by the extended SEM model) were obtained. In addition, as predicted on the basis of both models, comparisons with romantic partners resulted in a different pattern of self-reported affect than did comparisons with strangers. Unexpectedly, it appeared that empathic effects were minimal or absent among dating partners when comparisons were in areas of high self-relevance.

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Relationships among stressful life events, negative affect, and judged quality of intimate relationships were explored. Three studies and a mini-meta-analysis revealed that as negative life events increased, judgments of close relationships gradually became less favorable, jumped back toward positivity, and then, again, gradually became less favorable. The same methods of analysis revealed a relationship between negative life events and negative affect with no evidence of significant discontinuities. Moreover, the correlation between relationship satisfaction and negative affect (with negative events held constant), was maximized at the point of relationship-judgment discontinuity. Although the findings are complex, they are consistent with the authors' theoretical account and represent an initial attempt to conceptualize the effect of negative life events in light of the recent social judgment literature.

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This research project posits a model of repression that incorporates both repressive personality and repressive social behavior. The 1st parameter of the model specifies the motivation for repressors' distancing of themselves from emotional events. Experiment 1 demonstrates that repressors are hypersensitive--in their cognitive attention--to both negative and positive emotional events. The 2nd parameter of the model specifies the conditions under which repressors distance themselves from emotional events. Experiments 2 and 3 demonstrate that repressors psychologically distance themselves when the situation threatens their self-evaluation and provides opportunity for them to attend to and process self-relevant and non-self-relevant information. This 2-factor model extends the current conceptualization of repression in that it identifies motivation (dispositional emotional sensitivity) and context (situational threats to self-evaluation and distraction availability) for repressors' distancing of themselves from negative and positive emotional events.

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It is argued that differences in response heritability may have important implications for the testing of general psychological theories, that is, responses that differ in heritability may function differently. For example, attitudes higher in heritability are shown to be responded to more quickly, to be more resistant to change, and to be more consequential in the attitude similarity attraction relationship. The substantive results are interpreted in terms of attitude strength and niche building. More generally, the implications of heritability for the generality and typicality of treatment effects are also discussed.

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The present study investigated the independence of Self-Evaluation Maintenance (SEM) processes and the larger self-system. SEM processes are activated whenever another's performance is made salient relative to one's own. According to the SEM model, when one is outperformed by a close other on a task high in self-relevance, self-esteem is lowered and a negative affect state is generated. When one is outperformed by a close other on a task low in self-relevance, however, self-esteem and positive affect are actually enhanced, because one can take pride in the other's accomplishments. There are many sources of threat or enhancement to self-esteem, however, that are unrelated to one's performance relative to another's. The present study, using American undergraduates as subjects, examined the impact that these unrelated self-esteem threats or enhancements might have on SEM processes, that is, whether a prior unrelated ego-threatening or ego-enhancing experience modulates the effects of SEM processes. It was expected that a prior ego-threatening experience would augment the impact of SEM variables, whereas an ego-enhancing experience would attenuate the effect. Results, however, indicated little interaction between SEM processes and unrelated threats or enhancements to self-esteem.

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Two studies examined the causal role of emotional arousal in self-evaluation maintenance processes. In previous work, Tesser and Campbell (1982) found that Ss were most charitable in their perception of another's performance when self-relevance was low and the other was close. If emotional arousal mediated this pattern of behavior, then the pattern of behavior should be replicated when arousal is present but attenuated when arousal is misattributed or low. The misattribution hypothesis was tested in Study 1. Study 2 was a correlational study in which physiological arousal and misattribution were measured. The results supported the prediction that SEM processes would be attenuated when arousal was attributed to external sources. The misattribution effect was particularly pronounced among more highly aroused Ss. The results are discussed in terms of their implications for the SEM model and person perception.

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We asked 26 subjects to recall and describe social situations in which either a close or a distant other performed better or worse than the self at an activity that was either high or low in relevance to the self. Subjects then rated the extent to which they experienced each of 18 different emotions in each situation. They also rated each situation on a series of dimensions that Smith and Ellsworth (1985) found to be consequential for differentiating emotions. In a series of analyses guided by intuitive hypotheses, the Smith and Ellsworth theoretical approach, and a relatively unconstrained, open-ended exploration of the data, the situations were found to vary with respect to the emotions of pride, jealousy or envy, pride in the other, boredom, and happiness. We discuss the results in terms of their relevance to emotion theories and to the self-evaluation maintenance model of social behavior.

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A self-evaluation maintenance (SEM) model of social behavior was described. According to the comparison process, when another outperforms the self on a task high in relevance to the self, the closer the other the greater the threat to self-evaluation. According to the reflection process, when another outperforms the self on a task low in relevance to the self, the closer the other the greater the promise of augmentation to self-evaluation. Affect was assumed to reflect threats and promises to self-evaluation. In three studies, subjects were given feedback about own performance and the performance of a close (friend) and distant (stranger) other on tasks that were either low in self-relevance (Study 2) or that varied in self-relevance (Studies 1 and 3). In Study 1 (N = 31), subjects' performance on simple and complex tasks after each feedback trial served as a measure of arousal. Being outperformed by a close other resulted in greater arousal than being outperformed by a distant other. In Study 2 (N = 30), evaluative ratings of words unrelated to task performance served as an indirect measure of affect. Results indicated that when relevance is low, more positive affect is associated with a friend's outperforming the self than either a friend's performing at a level equal to the self or being outperformed by a stranger. In Study 3 (N = 31), subjects received feedback while their facial expressions were monitored. Pleasantness of expression was an interactive function of relevance of task, relative performance, and closeness of comparison other. The results of all three studies were interpreted as being generally consistent with the SEM model.

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