RNF213 p.Arg4810Lys is linked to various vascular diseases, including pulmonary arterial hypertension (PAH); however, its pathogenesis remains unclear. Here, we report the unique features of two cases of severe PAH with this variant: one is the first reported case with stenosis of the thoracic and abdominal aorta, femoral arteries, and subclavian veins. Coexistence of severe and continuous eosinophilic inflammation, which has been suspected to be implicated in the pathogenesis of PAH in previous fundamental studies, was also present in both cases. Further studies are needed to clarify the pathogenetic mechanisms in vascular lesions with this variant.

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BACKGROUND: There is an incomplete understanding of the risk of COVID-19 infection in atopic dermatitis (AD) patients.
OBJECTIVE: To evaluate the risk of COVID-19 infection in AD patients in a large, diverse cohort.
METHODS: A case-control study of the All of Us cohort to analyze the association between AD and COVID-19. Comorbidities and risk factors were compared between cases and controls using multivariable analyses.
RESULTS: In a cohort of 11,752 AD cases with 47,008 matched controls, AD patients were more likely to have a COVID-19 diagnosis (4.2% vs 2.8%, P < .001). AD remained significantly associated with COVID-19 in multivariable analysis (odds ratio, 1.29; P < .001) after adjusting for demographic factors and comorbidities.
CONCLUSIONS: Ascertainment of AD and COVID-19 cases using electronic health records and lack of clinical data on AD severity or therapy and COVID-19 outcomes.
CONCLUSIONS: AD is associated with increased odds of COVID-19 infection even after controlling for common comorbidities.

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Severe eosinophilic asthma is associated with a heavy burden and impact on daily living in patients experiencing uncontrolled symptoms, exacerbations, and treatment side effects. This case study reports a 49-year-old woman who presented to the severe asthma center with uncontrolled severe asthma despite multiple maintenance medications and omalizumab treatment. On presentation, the patient had experienced two to three hospitalizations per year, frequent asthma exacerbations requiring courses of oral corticosteroids, and symptoms that impacted her quality of life. Omalizumab was previously discontinued, and bronchial thermoplasty was also unsuccessful. The patient stabilized on injectable steroids and commenced mepolizumab once available on prescription. Owing to continued exacerbations and an inability to reduce steroid treatment without exacerbating, mepolizumab was discontinued and the patient commenced benralizumab (30 mg subcutaneously every 4 weeks for the first three doses, every 8 weeks thereafter) under the sole care of the severe asthma center. Benralizumab treatment resulted in a reduction in steroid treatment, zero asthma exacerbations, improved asthma control and lung function, and a marked improvement in activity levels that allowed the patient to participate in a long-distance running event. Additionally, 7 months following the initiation of benralizumab treatment, her blood eosinophils were completely depleted. These findings support the use of benralizumab in patients with refractory uncontrolled severe eosinophilic asthma despite previous biologic treatment with omalizumab and mepolizumab, as improvements in clinical and patient outcomes, including quality of life, can be achieved in difficult-to-treat cases.

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