Youssef Mohamed Amin Soliman

Background: Pulmonary hypertension (PH) is a serious and potentially life-threatening complication of systemic lupus erythematosus (SLE). Its etiology in SLE is heterogeneous and includes vasculopathy, interstitial lung disease (ILD), left-sided heart dysfunction, and chronic thromboembolic disease. High-resolution computed tomography (HRCT) plays an important role in defining phenotypes and supporting targeted management. Methods: This analytical cross-sectional study included 60 adults with SLE and echocardiograph-confirmed PH. All patients underwent clinical evaluation and HRCT. Radiologic patterns were evaluated and correlated with pulmonary hypertension subtypes based on the 2022 ESC/ERS classification. We used SPSS v20 to analyze the data, and chi-square and Fisher’s exact tests were used to look for connections. Results: In our cohort of patients with SLE and confirmed pulmonary hypertension, the most prevalent subtype observed was Group 2 PH, attributed to underlying left-sided heart disease (48.3%). This was followed by pulmonary arterial hypertension (25%), PH due to lung disease/hypoxia (20%), and chronic thromboembolic PH (6.7%). HRCT findings differed significantly among PH groups using Fisher’s Exact Test ( p < 0.05). Normal HRCT scans were most frequent in Group 1, lung congestion with septal thickening predominated in Group 2 ( p = 0.049), nonspecific interstitial pneumonia (NSIP)—a pattern of interstitial lung disease—was strongly associated with Group 3 ( p < 0.000001), and all Group 4 patients demonstrated pulmonary embolism on Computed Tomography Pulmonary Angiography (CTPA) ( p < 0.00001). Conclusion: HRCT patterns vary markedly across PH subtypes in SLE and provide important diagnostic clues to the underlying pathophysiological mechanism. HRCT provides important phenotypic information that supports noninvasive classification of SLE-associated pulmonary hypertension and helps guide further diagnostic and management pathways.

Background Pulmonary arterial hypertension (PAH) is a progressive, cureless disease, characterized by increased pulmonary vascular resistance and remodeling, with subsequent ventricular dilatation and failure. New therapeutic targets are being investigated for their potential roles in improving PAH patients’ symptoms and reversing pulmonary vascular pathology. Method We aimed to address the available knowledge from the published randomized controlled trials (RCTs) regarding the role of Rho-kinase (ROCK) inhibitors, bone morphogenetic protein 2 (BMP2) inhibitors, estrogen inhibitors, and AMP-activated protein kinase (AMPK) activators on the PAH evaluation parameters. This systematic review (SR) was registered in the International Prospective Register of Systematic Reviews (PROSPERO) database (CDR42022340658) and followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Results Overall, 5092 records were screened from different database and registries; 8 RCTs that met our inclusion criteria were included. The marked difference in the study designs and the variability of the selected outcome measurement tools among the studies made performing a meta-analysis impossible. However, the main findings of this SR relate to the powerful potential of the AMPK activator and the imminent antidiabetic drug metformin, and the BMP2 inhibitor sotatercept as promising PAH-modifying therapies. There is a need for long-term studies to evaluate the effect of the ROCK inhibitor fasudil and the estrogen aromatase inhibitor anastrozole in PAH patients. The role of tacrolimus in PAH is questionable. The discrepancy in the hemodynamic and clinical parameters necessitates defining cut values to predict improvement. The differences in the PAH etiologies render the judgment of the therapeutic potential of the tested drugs challenging. Conclusion Metformin and sotatercept appear as promising therapeutic drugs for PAH. Clinical Trials Registration This work was registered in PROSPERO (CDR42022340658).

Background: The pandemic had a significant impact on those with underlying chronic health conditions being at risk of developing a more severe disease with rapid progression, significant complications, and with increased risk of mortality.This was also expected in the pulmonary vascular community owing to the vulnerable nature of this population, who are characterized by an increase in the pulmonary vascular resistance leading to right heart failure.This study is aiming to identify the incidence of COVID-19 infection among pulmonary hypertension patients receiving specific therapy as well as the predictors of the COVID-19 disease severity and outcome in those patients.Results: Data analysis of 197 PAH and CTEPH patients, showed that the incidence of SARS-CoV-2 infection is 10.66% (n= 21). Seven patients (33.3%) required hospitalization. Mortality rate is 14.3% (3/21).Severity of COVID19 disease in those patients has statistically significant moderate to strong correlation with higher values of d-dimer (r= 0.821,P= 0.000), ferritin (r= 0.718,p= 0.000), CRP (r= 0.613,p= 0.04), acute renal failure (r= 0.557,p= 0.009), and hypoxemia (r= 0.825,p= 0.000).Mortality from COVID-19 show moderate to strong statistically significant correlations with acute renal failure (r= 0.795,p= 0.000), hypoxemia (r= 0.645,p= 0.002), higher values of ferritin (r= 0.689,p= 0.001) and d-dimer (r= 0.603,P= 0.004).Conclusions: COVID-19 in PAH and CTEPH patients is challenging, higher COVID-19 infection rate is present in those patients and is associated with increased disease severity and higher mortality.

Been considered an important prognostic marker in many respiratory disorders. Aim The aim of this work was to evaluate the relation between RDW and severity of HP. Patients and methods Forty patients with HP were included within one year. The patients were subdivided into two equal groups according to their partial oxygen pressure (PO2) values: 20 nonhypoxemic patients (PO2 ≥60 mmHg) and 20 hypoxemic patients (PO2 <60 mmHg). Patients with primary hematological diseases, cardiac diseases, connective tissue disorders, history of diabetes and patients with other forms of diffuse parenchymal lung diseases were excluded. Demographics’ data, blood count, Dyspnea score, arterial blood gases, high-resolution computed tomography chest scan score, 6-min walk distance, echocardiography, and pulmonary function data were recorded. Results The mean RDW in the hypoxic group was 17.25±3.9, higher than that of the nonhypoxic group (16.02±3.08), with no statistical significance (P=0.174). RDW was positively correlated with dyspnea score, BMI and total disease extent score. Moreover, it showed an inverse relationship with diffusing capacity and 6-min walk distance without statistically significant values. There was a statistically significant correlation between RDW and pulmonary hypertension (P=0.027). Conclusion The RDW is a readily available laboratory test that may provide useful information about severity and prognosis of HP....