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In an era of cost pressure, substituting generic drugs represents one of the main cost-containment strategies of healthcare systems. Despite the obvious financial benefits, in a minority of cases, substitution may require caution or even be contraindicated. In most jurisdictions, to obtain approval, the bioequivalence of generic products with the brand-name equivalent needs to be shown via bioavailability studies in healthy subjects. Rare diseases, defined as medical conditions with a low prevalence, are a group of heterogenous diseases that are typically severe, disabling, progressive, degenerative, and life-threatening or chronically debilitating, and disproportionally affect the very young and elderly. Despite these unique features of rare diseases, generic bioequivalence studies are typically carried out with single doses and exclude children or the elderly. Furthermore, the excipients and manufacturing processes for generic/biosimilar products can differ from the brand products which may affect the shelf-life of the product, its appearance, smell, taste, bioavailability, safety and potency. This may result in approval of generics/biosimilars which are not bioequivalent/comparable in their target population or that meet bioequivalence but not therapeutic equivalence criteria. Another concern relates to the interchangeability of generics and biosimilars which cannot be guaranteed due to the phenomenon of biocreep. This review summarizes potential concerns with generic substitution of orphan drugs and discusses potentially problematic cases including narrow therapeutic index drugs or critical conditions where therapeutic failure could lead to serious complications or even death. Finally, we put forward the need for refining regulatory frameworks, with emphasis on Saudi Arabia, for generic substitution and recent efforts toward this direction. [ABSTRACT FROM AUTHOR]

Abstract Background We assessed the relationship between physiologic parameters, computed tomography patterns, 6 minute walk distance (6MWD) and the distance-saturation product [DSP; defined as the product of the 6MWD and the lowest oxygen saturation during the 6 minute walk test (6MWT)]. In addition, we investigated factors affecting 6MWD in patients with pulmonary sarcoidosis. Methods We performed a retrospective study of patient demographics, treatment, pulmonary function, 6MWT, echocardiography and computed tomography results. Results Fifty nine patients were included in this study. Their mean+standard deviation age was 47.5 years + 12.5 years, and 42 (71.2%) were female. Mean pulmonary function parameters for forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1) and total lung capacity (TLC) results, as percentages of predicted values, were 77.6 ± 22.2, 77.1 ± 22.8 and 78.7 ± 16.1, respectively. Comparison of the DSP with distance walked revealed a significant correlation with factors underlying reduced 6MWD, including gender, pulmonary function indices, partial pressure of oxygen (PaO2), and Borg dyspnea score. Other factors were significantly associated with DSP but not distance; these included lung fibrosis (p = 0.02), pulmonary hypertension (p = 0.01) and systemic therapy (p = 0.04). Backward elimination stepwise multiple regression analysis revealed that gender, and FEV1 were independent predictors of 6MWD, but FEV1 was more strongly related when DSP applied [DSP, R2 = 0.53, p = 0.02; distance, R2 = 0.45, p < 0.0001]. Conclusion Our findings reveal that, compared to 6MWD alone, the DSP is correlated with a greater number of factors associated with reduced 6MWT performance. Therefore, the DSP may be a useful indicator of functional status in patients with sarcoidosis. Additional large-scale studies are warranted to validate our findings.

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This is an updated guideline for the diagnosis and management of asthma, developed by the Saudi Initiative for Asthma (SINA) group, a subsidiary of the Saudi Thoracic Society. The main objective of SINA is to have guidelines that are up to date, simple to understand and easy to use by nonasthma specialists, including primary care and general practice physicians. SINA approach is mainly based on symptom control and assessment of risk as it is the ultimate goal of treatment. The new SINA guidelines include updates of acute and chronic asthma management, with more emphasis on the use of asthma control in the management of asthma in adults and children, inclusion of a new medication appendix, and keeping consistency on the management at different age groups. The section on asthma in children is rewritten and expanded where the approach is stratified based on the age. The guidelines are constructed based on the available evidence, local literature, and the current situation in Saudi Arabia. There is also an emphasis on patient–doctor partnership in the management that also includes a self-management plan. [ABSTRACT FROM AUTHOR]

Pregnancy in pulmonary hypertension (PH) is known to be associated with high morbidity and mortality. The physiological changes occur during normal pregnancy, such as increase blood volume and cardiac output (CO) may be detrimental in PH patients. Several practice guidelines advise against pregnancy and even recommend termination of pregnancy. Occasionally PH may be diagnosed for the first time during pregnancy, as stress of pregnancy can unmask previously undiagnosed PH in an asymptomatic individual. This narrative review provides a detailed discussion about the physiologic parameters associated in pregnancy and their negative effect on the right ventricle. It also gives practical evidence-based recommendations about different management issues in PH pregnant patients. [ABSTRACT FROM AUTHOR]

There is scant published data about pulmonary hypertension (PH) from the developing countries. True prevalence of the disease, its biology, etiology and response to treatment are not well known, and they are likely to be somewhat different from that of the developed countries. In this review, we will discuss the main challenges for managing PH in developing countries and propose real-life recommendations to deal with such difficulties. [ABSTRACT FROM AUTHOR]

Chronic lung diseases are common causes of pulmonary hypertension. It ranks second after the left heart disease. Both obstructive and restrictive lung diseases are know to cause pulmonary hypertension. The pathophysiology of the disease is complex, and includes factors affecting the blood vessels, airways, and lung parenchyma. Hypoxia and the inhalation of toxic materials are another contributing factors. Recent guidelines have further clarified the association between pulmonary hypertension and chronic lung disease and made general guidelines concerning the diagnosis and management. In this article, we will provide a detailed revision about the new classification and give general recommendations about the management of pulmonary hypertension in chronic lung diseases. [ABSTRACT FROM AUTHOR]

Treatment of pulmonary hypertension (PH) patients is challenging and should only be initiated after a comprehensive diagnostic evaluation. Such treatment should ideally be done in specialized centers with full capability for hemodynamic measurements, having access to a broad range of PAH therapies, and adequate experience in the management of critically ill patients. The following discussion is intended to review the general measures and the non-specific (supportive) therapy used in managing PH patients, while the specific therapy will be discussed in a subsequent different article. [ABSTRACT FROM AUTHOR]

Prior to the availability of the pulmonary arterial hypertension (PAH)-specific therapy, PAH was a dreadful disease with a very poor prognosis. Better understanding of the complex pathobiology of PAH has led to a major therapeutic evolution. International regulatory agencies have approved many specific drugs with different pharmacologic pathways and routes of administration. In the year 2013, two new drugs with great potentials in managing PAH have been added to the treatment options, macitentan and riociguat. Additional drugs are expected to come in the near future. A substantial body of evidence has confirmed the effectiveness of pulmonary arterial hypertension (PAH)-specific therapies in improving the patients' symptomatic status and slowing down the rate of clinical deterioration. Although the newer modern medications have significantly improved the survival of patients with PAH, it remains a non-curable and fatal disease. Lung transplantation (LT) remains the only therapeutic option for selected patients with advanced disease who continue to deteriorate despite optimal therapy. [ABSTRACT FROM AUTHOR]

Chronic thromboembolic pulmonary hypertension (CTEPH) is categorized as group IV in the WHO classification for pulmonary hypertension. The disease requires a very low index of suspicion for identification and needs a special diagnostic approach utilizing clinical, radiological, and hemodynamic tools. As CTEPH is potentially curable, all efforts should be consumed to reach the accurate diagnosis and subsequently evaluated for operability. Although pulmonary endarterectomy (PEA) is the only curative tool so far, recent updates concerning medical and interventional therapy have made significant advances in inoperable patients. In this review, we provide a detailed discussion on diagnostic algorithm, surgical operability criteria, PEA, and the medical therapy. [ABSTRACT FROM AUTHOR]

Pulmonary hypertension (PH) due to left heart disease is the most common cause of pulmonary hypertension in the western world. It is classified as WHO PH group II. Different pathophysiologic abnormalities may take place in this condition, including pulmonary venous congestion and vascular remodeling. Despite the high prevalence of WHO group 2 PH, the major focus of research on PH over the past decade has been on WHO group 1 pulmonary arterial hypertension (PAH). Few investigators have focused on WHO group 2 PH; consequently, the pathophysiology of this condition remains poorly understood, and no specific therapy is available. Clinical and translational studies in this area are much needed and have the potential to positively affect large numbers of patients. In this review, we provide a detailed discussion upon the pathophysiology of the disease, the recent updates in classification, and the diagnostic and therapeutic algorithms. [ABSTRACT FROM AUTHOR]

Portopulmonary hypertension (POPH) is defined as pulmonary arterial hypertension (PAH) complicated by portal hypertension, with or without advanced hepatic disease. Significant percentage of patients with cirrhotic liver disease has high cardiac output and subsequently elevated pulmonary arterial pressures (PAP). However, patients with POPH develop a progressive increase in pulmonary vascular resistance (PVR), which is generally lower than that observed in other forms of PAH. The prognosis of untreated patients with POPH is very poor and the outcome of liver transplant (LT) in those patients is determined by the degree of severity of the associated pulmonary hemodynamics. In this narrative review, we describe the clinical presentation of POPH, the pathobiology, and the clinical implication of pulmonary hemodynamics. We also provide evidence-based recommendations for the diagnosic and management approaches of POPH. [ABSTRACT FROM AUTHOR]

The Saudi Association for Pulmonary Hypertension (previously called Saudi Advisory Group for Pulmonary Hypertension) has published the first Saudi Guidelines on Diagnosis and Treatment of Pulmonary Arterial Hypertension back in 2008.[1] That guideline was very detailed and extensive and reviewed most aspects of pulmonary hypertension (PH). One of the disadvantages of such detailed guidelines is the difficulty that some of the readers who just want to get a quick guidance or looking for a specific piece of information might face. All efforts were made to develop this guideline in an easy-to-read form, making it very handy and helpful to clinicians dealing with PH patients to select the best management strategies for the typical patient suffering from a specific condition. This Guideline was designed to provide recommendations for problems frequently encountered by practicing clinicians involved in management of PH. This publication targets mainly adult and pediatric PH-treating physicians, but can also be used by other physicians interested in PH. [ABSTRACT FROM AUTHOR]

The Saudi Thoracic Society (STS) launched the Saudi Initiative for Chronic Airway Diseases (SICAD) to develop a guideline for the diagnosis and management of chronic obstructive pulmonary disease (COPD). This guideline is primarily aimed for internists and general practitioners. Though there is scanty epidemiological data related to COPD, the SICAD panel believes that COPD prevalence is increasing in Saudi Arabia due to increasing prevalence of tobacco smoking among men and women. To overcome the issue of underutilization of spirometry for diagnosing COPD, handheld spirometry is recommended to screen individuals at risk for COPD. A unique feature about this guideline is the simplified practical approach to classify COPD into three classes based on the symptoms as per COPD Assessment Test (CAT) and the risk of exacerbations and hospitalization. Those patients with low risk of exacerbation (<2 in the past year) can be classified as either Class I when they have less symptoms (CAT < 10) or Class II when they have more symptoms (CAT ≥ 10). High-risk COPD patients, as manifested with ≥2 exacerbation or hospitalization in the past year irrespective of the baseline symptoms, are classified as Class III. Class I and II patients require bronchodilators for symptom relief, while Class III patients are recommended to use medications that reduce the risks of exacerbations. The guideline recommends screening for co-morbidities and suggests a comprehensive management approach including pulmonary rehabilitation for those with a CAT score ≥10. The article also discusses the diagnosis and management of acute exacerbations in COPD. [ABSTRACT FROM AUTHOR]

This an updated guidelines for the diagnosis and management of asthma, developed by the Saudi Initiative for Asthma (SINA) group, a subsidiary of the Saudi Thoracic Society. The main objective of SINA is to have updated guidelines, which are simple to understand and easy to use by non-asthma specialists, including primary care and general practice physicians. This new version includes updates of acute and chronic asthma management, with more emphasis on the use of Asthma Control Test in the management of asthma, and a new section on "difficult-to-treat asthma." Further, the section on asthma in children was re-written to cover different aspects in this age group. The SINA panel is a group of Saudi experts with well-respected academic backgrounds and experience in the field of asthma. The guidelines are formatted based on the available evidence, local literature, and the current situation in Saudi Arabia. There was an emphasis on patient-doctor partnership in the management that also includes a self-management plan. The approach adopted by the SINA group is mainly based on disease control as it is the ultimate goal of treatment. [ABSTRACT FROM AUTHOR]

BACKGROUND: Right ventricular (RV) dysfunction has been identified as a poor prognostic indicator in sub-massive pulmonary embolism (SPE). We hypothesized that using selective vasodilator agent is beneficial in improving RV function in patients with this condition. METHODS: We used inhaled prostacyclin analogue (Iloprost, Ventavis®) in five patients with SPE. Helical computerized tomography angiogram was confirmatory for pulmonary embolism and echocardiography was used to evaluate the RV status. All patients received inhaled Iloprost, 2.5 to 5 µg every 4 hours for 3 weeks. RESULTS: Patients were prospectively followed for 3 months. They were assessed at baseline before starting Iloprost treatment and at 3 days, 3 weeks, and 3 months after treatment. All patients showed significant improvement in their functional class, Borg dyspnea score, NT pro-BNP level, and echocardiographic parameters. CONCLUSION: In SPE, directing therapy toward decreasing pulmonary vascular resistance improves the associated pulmonary hemodynamic compromise and improves RV function. [ABSTRACT FROM AUTHOR]

BACKGROUND: Pulmonary hypertension (PH) occurs in many patients with interstitial lung disease, including sarcoidosis. We explored the frequency, clinical characteristics and outcomes of PH in Arab patients diagnosed with pulmonary sarcoidosis. METHODS: A retrospective study in three tertiary hospitals was performed on 96 patients who underwent Doppler echocardiography. Demographic and clinical characteristics, physiological studies and computed tomography (CT) results were collected, and compared between patients with and without PH. RESULTS: Twenty (20.8%) patients were found to have PH. Patients with PH were more likely to be symptomatic (cough, P = 0.008; dyspnea, P = 0.04), to have an advanced radiographic stage (P = 0.001), and to be receiving systemic therapy (P = 0.001), compared to those without PH. Physiological data including pulmonary function test parameters, arterial blood gas levels and oxygen saturation at rest and after exercise were all significantly lower in patients with PH compared to those without PH. Comparison of CT patterns between patients with and without PH showed significant differences in the frequencies of ground-glass opacity (61.5 vs. 28.8%, P = 0.032) and fibrosis (76.9 vs. 44.2%, P = 0.035). In total, four patients died during the study period, including three with evidence of PH. CONCLUSIONS: The frequency of PH in the present study was 20.8%. Clinical, physiologic and radiographic characteristics appeared to differentiate patients with PH from those without PH. The presence of PH contributed to poor outcomes in patients with pulmonary sarcoidosis. [ABSTRACT FROM AUTHOR]

Background: We assessed the relationship between physiologic parameters, computed tomography patterns, 6 minute walk distance (6MWD) and the distance-saturation product [DSP; defined as the product of the 6MWD and the lowest oxygen saturation during the 6 minute walk test (6MWT)]. In addition, we investigated factors affecting 6MWD in patients with pulmonary sarcoidosis. Methods: We performed a retrospective study of patient demographics, treatment, pulmonary function, 6MWT, echocardiography and computed tomography results. Results: Fifty nine patients were included in this study. Their mean+standard deviation age was 47.5 years + 12.5 years, and 42 (71.2%) were female. Mean pulmonary function parameters for forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1) and total lung capacity (TLC) results, as percentages of predicted values, were 77.6 ± 22.2, 77.1 ± 22.8 and 78.7 ± 16.1, respectively. Comparison of the DSP with distance walked revealed a significant correlation with factors underlying reduced 6MWD, including gender, pulmonary function indices, partial pressure of oxygen (PaO2), and Borg dyspnea score. Other factors were significantly associated with DSP but not distance; these included lung fibrosis (p = 0.02), pulmonary hypertension (p = 0.01) and systemic therapy (p = 0.04). Backward elimination stepwise multiple regression analysis revealed that gender, and FEV1 were independent predictors of 6MWD, but FEV1 was more strongly related when DSP applied [DSP, R² = 0.53, p = 0.02; distance, R² = 0.45, p < 0.0001]. Conclusion: Our findings reveal that, compared to 6MWD alone, the DSP is correlated with a greater number of factors associated with reduced 6MWT performance. Therefore, the DSP may be a useful indicator of functional status in patients with sarcoidosis. Additional large-scale studies are warranted to validate our findings. [ABSTRACT FROM AUTHOR]

The Saudi Initiative for Asthma (SINA) provides up-to-date guidelines for healthcare workers managing patients with asthma. SINA was developed by a panel of Saudi experts with respectable academic backgrounds and longstanding experience in the field. SINA is founded on the latest available evidence, local literature, and knowledge of the current setting in Saudi Arabia. Emphasis is placed on understanding the epidemiology, pathophysiology, medications, and clinical presentation. SINA elaborates on the development of patient-doctor partnership, self-management, and control of precipitating factors. Approaches to asthma treatment in SINA are based on disease control by the utilization of Asthma Control Test for the initiation and adjustment of asthma treatment. This guideline is established for the treatment of asthma in both children and adults, with special attention to children 5 years and younger. It is expected that the implementation of these guidelines for treating asthma will lead to better asthma control and decrease patient utilization of the health care system. [ABSTRACT FROM AUTHOR]

The Saudi guidelines on diagnosis and treatment of pulmonary arterial hypertension (PAH) are presented. It discusses the epidemiology, natural history, causes, risk factors, clinical classification, histopathology, and pathophysiology of PAH. Tests used in the diagnosis and classification of PAH include Pulmonary function tests, ventilation and perfusion (V/Q) lung scan, and pulmonary angiography. Pharmacological treatment for PAH include oral anticoagulants, diuretics, and calcium-channel blockers (CCBs).

Objective: The aim of this study was to evaluate asthma control after the introduction of a leukotriene modifier (Montelukast), in addition to the current controller asthma therapies, in patients with inadequately controlled mild-to-moderate persistent asthma. Asthma control and patient perception were assessed prior to, and 4 weeks after, the introduction of Montelukast, and the pre-introduction and post-introduction results were compared. Materials and Methods: A cross-sectional, observational study collected information on 1,490 eligible adult asthmatic patients in Saudi Arabia. The eligibility criteria included patients aged 15 years or more with symptomatic mild-to-moderate persistent asthma despite treatment with inhaled corticosteroids with or without long-acting beta agonist; also, the patient should attend the initial visit and follow-up visits after at least 4 weeks. Results: Of the 1,490 eligible patients, 79.5% received inhaled corticosteroids alone, and the remaining 20.5% received combination of inhaled corticosteroids and long-acting bronchodilator. Despite the treatment with daily controller medications, asthma symptoms persisted in more than two-thirds of the study population. Upon adding Montelukast, more than 80% of patients reported improvement in symptoms, which was consistent in all patients irrespective of corticosteroid type or dose (stratum) or the addition of long-acting β2-agonist. At the follow-up visit, 92.2% of patients reported that they felt better on Montelukast. Conclusion: Leukotriene modifier Montelukast has significant additive benefits in the management of patients who suffer from mild-to-moderate asthma and who are inadequately controlled on inhaled corticosteroids therapy with or without long-acting bronchodilator. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "Novel approach for the management of sub‑massive pulmonary embolism" by Majdy M. Idrees, Enas Batubara and Tarek Kashour in the July 2012 issue.

The article presents information the organization of the Pulmonary Hypertension Association (PHA) and the Saudi Advisory Group for Pulmonary Hypertension (SAPH). Pulmonary hypertension (PH) is characterized by a progressive increase of pulmonary vascular resistance and pulmonary artery pressure that leads to right ventricular failure and death. It relates the objectives of the four patients for organizing the PHA. It presents all the achievements of the SAPH in 2009.

The Saudi Initiative for Asthma 2024 (SINA-2024) is the sixth version of asthma guidelines for the diagnosis and management of asthma for adults and children that was developed by the SINA group, a subsidiary of the Saudi Thoracic Society. The main objective of the SINA is to have guidelines that are up-to-date, simple to understand, and easy to use by healthcare workers dealing with asthma patients. To facilitate achieving the goals of asthma management, the SINA Panel approach is mainly based on the assessment of symptom control and risk for both adults and children. The approach to asthma management is aligned for age groups: adults, adolescents, children aged 5-12 years, and children aged <5 years. SINA guidelines have focused more on personalized approaches reflecting a better understanding of disease heterogeneity with the integration of recommendations related to biologic agents, evidence-based updates on treatment, and the role of immunotherapy in management. The medication appendix has also been updated with the addition of recent evidence, new indications for existing medication, and new medications. The guidelines are constructed based on the available evidence, local literature, and the current situation at national and regional levels. There is also an emphasis on patient-doctor partnership in the management that also includes a self-management plan., Competing Interests: The SINA is fully sponsored by the Saudi Thoracic Society., (Copyright: © 2023 Annals of Thoracic Medicine.)

Asthma control is the main goal of management. Unfortunately, most asthma patients with moderate-severe asthma remain uncontrolled despite receiving standard treatment of inhaled corticosteroids (ICS) with long-acting β2 agonists (LABA). The addition of long-acting antimuscarinic agents (LAMA) has been shown to improve different aspects of asthma control, including symptoms, lung functions, and probably exacerbations. Such an option could be considered for low-T2 asthma phenotype. Umeclidinium and glycopyrronium bromide are other LAMA agents that have been recently made available in combination with ICS and LABA in single-inhaler triple therapy (SITT) devices. Here, we discuss the position of SITT as a new novel therapeutic option in asthma management and its clinical benefits, potential cost saving, and improved compliance., Competing Interests: The authors are members of the Saudi Initiative for Asthma Panel, a subsidiary of the Saudi Thoracic Society., (Copyright: © 2022 Annals of Thoracic Medicine.)

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is uncommon but well-known sequel of venous thromboembolism (VTE). At present, it is the only potential curable subtype of pulmonary hypertension. The aim of this study is to describe the medically treated-CTEPH patients' characteristics in a single specialized PH center in Saudi Arabia., Methods: This study presents demographic, clinical, physiological, and hemodynamic characteristics of medically treated-CTEPH patients in a single PH center, namely Prince Sultan Military Medical City, Riyadh, Saudi Arabia. Both incident and prevalent cases are included., Results: A total of 20 patients with a confirmed diagnosis of CTEPH were included. Mean age at diagnosis was 43 years with a female preponderance of 75%. Most common presenting symptom was dyspnea (100%) followed by syncope (58%). At diagnosis, a mean of 15 ± 10 months had passed since symptoms onset. About 45% of patients were in WHO functional class IV. At baseline, mean 6-min walk distance was 354.3 meters. Overall, VTE was the most frequent risk factor identified (65% of all patients). Nearly 30% of patients had sickle cell disease. 13 out of 20 patients had radiographic (i.e., computed tomography [CT] pulmonary angiogram) features of chronic thromboembolism. About 75% of patients were found to have distal disease on radiographic imaging. At the time of diagnosis, 7 out of 20 (35%) patients demonstrated right ventricular failure on echocardiography. Mean tricuspid annular plane systolic excursion was 17.7 ± 1.20. Median NT-proBNP levels were found to be 688 pg/ml. Mean diffusing capacity for carbon monoxide was 74.8%., Conclusions: Diagnosis of CTEPH was established at a relatively younger age. Majority of patients had advanced but distal disease on radiographic imaging, not amenable to surgery., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Annals of Thoracic Medicine.)

The Saudi Initiative for Asthma 2021 (SINA-2021) is the fifth version of asthma guidelines for the diagnosis and management of asthma for adults and children, which is developed by the SINA group, a subsidiary of the Saudi Thoracic Society. The main objective of the SINA is to have guidelines that are up to date, simple to understand, and easy to use by healthcare workers dealing with asthma patients. To facilitate achieving the goals of asthma management, the SINA panel approach is mainly based on the assessment of symptom control and risk for both adults and children. The approach to asthma management is aligned for age groups: adults, adolescents, children aged 5-12 years, and children aged less than 5 years. SINA guidelines have focused more on personalized approaches reflecting better understanding of disease heterogeneity with the integration of recommendations related to biologic agents, evidence-based updates on treatment, and the role of immunotherapy in management. Medication appendix has also been updated with the addition of recent evidence, new indications for existing medication, and new medications. The guidelines are constructed based on the available evidence, local literature, and the current situation at national and regional levels. There is also an emphasis on patient-doctor partnership in the management that also includes a self-management plan., Competing Interests: The SINA is fully sponsored by the STS., (Copyright: © 2021 Annals of Thoracic Medicine.)

Background: After the emergence of new influenza viruses, the morbidity and mortality of viral pneumonia have received a great attention., Objectives: The objective of this study is to describe the epidemiologic, clinical and laboratory changes, and outcomes of viral pneumonia caused by influenza and the Middle East respiratory syndrome-coronavirus (MERS-CoV) infections., Methods: In a retrospective cohort study, the medical records of all patients diagnosed with viral pneumonia at Prince Sultan Military Medical City, Riyadh, Saudi Arabia, during the period from January 2012 to December 2015 were screened. Cases who were > 18 years old and were confirmed by a respiratory viral panel to have viral pneumonia either MERS-CoV or influenza viruses were included in the analysis. Sociodemographic, clinical, laboratory, and outcome data were extracted from patients' medical files. The data were analyzed descriptively and inferentially to identify the predictors of poor outcome., Results: A total of 448 patients with confirmed viral pneumonia were included, of those, 216 (48.2%) were caused by influenza A (non H1N1)/influenza B, 150 (33.5%) by H1N1, and 82 (18.3%) by MERS-CoV. The majority of patients presented with fever (82%), shortness of breath (64%), and flu-like symptoms (54.9%), particularly in MERS-CoV infected cases (92%). The peak incidence of viral pneumonia was in early spring and autumn. The mortality rate was 13.8%, and it was significantly higher among MERS-CoV cases. The predictors of death were age > 65 years, male gender, and associated comorbidities particularly diabetes mellitus, hypertension, and chronic kidney diseases. The number of comorbid illnesses was directly related to the increase in mortality in this group of patients., Conclusion: Viral pneumonia caused by influenza and MERS-CoV carries a high mortality rate, particularly among MERS-CoV infected cases. Old age, male gender, and comorbid illnesses are predictors of poor outcome. Routine testing for newly emergent viruses is warranted for adults who have been hospitalized with pneumonia., Competing Interests: There are no conflicts of interest.

This is the fourth version of the updated guidelines for the diagnosis and management of asthma, developed by the Saudi Initiative for Asthma (SINA) group, a subsidiary of the Saudi Thoracic Society. The main objective of the SINA is to have guidelines that are up to date, simple to understand, and easy to use by healthcare workers dealing with asthma patients. To facilitate achieving the goals of asthma management, the SINA panel approach is mainly based on the assessment of symptom control and risk for both adults and children. The approach to asthma management is now more aligned for different age groups. The guidelines have focused more on personalized approaches reflecting better understanding of disease heterogeneity with integration of recommendations related to biologic agents, evidence-based updates on treatment, and role of immunotherapy in management. The medication appendix has also been updated with the addition of recent evidence, new indications for existing medication, and new medications. The guidelines are constructed based on the available evidence, local literature, and current situation at national and regional levels. There is also an emphasis on patient-doctor partnership in the management that also includes a self-management plan., Competing Interests: The SINA is fully sponsored by the STS.

Background: Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in patients with systemic sclerosis (SSc). The objective of this study is to describe the clinical characteristics, mortality, and predictors of SSc-PAH in Saudi patients., Methods: Retrospective chart review study of SSc patients who were followed for at least 1 year in three tertiary care centers in Saudi Arabia was conducted. Clinical information, echocardiographic findings, and right heart catheterization (RHC) results were collected. Descriptive statistics were used for demographic and disease characteristics., Results: Fifty-seven patients with SSc were reviewed. PAH was confirmed by RHC in 40 patients (87.5%, females). Their mean age was 45.43 ± 13.48 years. The mean pulmonary artery pressure was 42.9 ± 12.7 mmHg, the pulmonary vascular resistance index was 19.4 ± 7.7 woods unit, and cardiac index was 2.43 ± 0.68 min/m 2 . The median time from symptoms to first assessment was 42.8 ± 115.62 months. Most patients (77.5%) presented with functional Class III or IV and more than half (22.55%) were on dual combination therapy. Ten patients (25%) SSc PAH died over a follow up period of 37 ± 7 months. Compared to SSc patients without PAH, SSc-PAH patients had shorter 6-min walk distance (6MWD) (296.1 ± 116.5 vs. 399.59 ± 40.60 m, P < 0.0001), higher pro-brain natriuretic peptide (1755.8 ± 2123.4 vs. 69.8 ± 44.3 pg/ml P = 0.004), and more frequent Raynaud's phenomenon (RP) (90% vs. 35%, P < 0.0001). Logistic regression showed RP (odds ratio [OR] =48.58, 95% confidence interval [CI]; 3.73-633.10) and 6MWD (OR 1.02: 95% CI; 1.01-1.03) were associated with the development of PAH., Conclusion: Our cohort of Saudi SSc-PAH patients has a younger disease onset and a lower mortality than what is described worldwide despite late presentation and requirement of combination therapy. The presence of RP and lower were associated with the development of SSc-PAH., Competing Interests: There are no conflicts of interest.

Objectives: To evaluate the prevalence and severity of plaque-induced gingivitis among a Saudi adult population in Riyadh region., Methods: Three hundred and eighty-five eligible participants in this cross-sectional study were recruited from routine dental patients attending the oral diagnosis clinic at Al-Farabi College in Riyadh, Saudi Arabia from June 2013 to December 2013. A clinical examination was performed by 2 dentists to measure the gingival and plaque indices of Löe and Silness for each participant., Results: The prevalence of gingivitis was 100% among adult subjects aged between 18-40 years old. Moreover, the mean gingival index was 1.68±0.31, which indicates a moderate gingival inflammation. In fact, males showed more severe signs of gingival inflammation compared with females (p=0.001). In addition, the mean plaque index was 0.875±0.49, which indicates a good plaque status of the participants. Interestingly, the age was not related either to the gingival inflammation (p=0.13), or to the amount of plaque accumulation (p=0.17). However, males were more affected than females (p=0.005)., Conclusion: The results of this study show that plaque accumulation is strongly associated with high prevalence of moderate to severe gingivitis among Saudi subjects.

A 38-year-old male was diagnosed with unrepaired ventricular septal defect associated with severe pulmonary arterial hypertension, cyanosis, and significant exercise intolerance. His echocardiogram showed right ventricular dysfunction and moderate pericardial effusion with no signs of cardiac tamponade. He was treated with an intensive course of inhaled iloprost and sildenafil. He showed a dramatic clinical response; his saturation went up from 60% on admission to 90% on minimal oxygen with significant improvement in his symptoms and signs of heart failure and total resolution of pericardial effusion. On follow up 3 and 6 weeks later, he was stable and could walk 360 meters in a 6 minutes walk test with disappearance of pericardial effusion. With unavailability of intravenous prostacyclin, we have shown in this case that intensive administration of inhaled iloprost could be used intensively as a rescue therapy in severe cases of pulmonary arterial hypertension with excellent results.

Portopulmonary hypertension (POPH) is defined as pulmonary arterial hypertension (PAH) complicated by portal hypertension, with or without advanced hepatic disease. Significant percentage of patients with cirrhotic liver disease has high cardiac output and subsequently elevated pulmonary arterial pressures (PAP). However, patients with POPH develop a progressive increase in pulmonary vascular resistance (PVR), which is generally lower than that observed in other forms of PAH. The prognosis of untreated patients with POPH is very poor and the outcome of liver transplant (LT) in those patients is determined by the degree of severity of the associated pulmonary hemodynamics. In this narrative review, we describe the clinical presentation of POPH, the pathobiology, and the clinical implication of pulmonary hemodynamics. We also provide evidence-based recommendations for the diagnosic and management approaches of POPH.

Pregnancy in pulmonary hypertension (PH) is known to be associated with high morbidity and mortality. The physiological changes occur during normal pregnancy, such as increase blood volume and cardiac output (CO) may be detrimental in PH patients. Several practice guidelines advise against pregnancy and even recommend termination of pregnancy. Occasionally PH may be diagnosed for the first time during pregnancy, as stress of pregnancy can unmask previously undiagnosed PH in an asymptomatic individual. This narrative review provides a detailed discussion about the physiologic parameters associated in pregnancy and their negative effect on the right ventricle. It also gives practical evidence-based recommendations about different management issues in PH pregnant patients.

Prior to the availability of the pulmonary arterial hypertension (PAH)-specific therapy, PAH was a dreadful disease with a very poor prognosis. Better understanding of the complex pathobiology of PAH has led to a major therapeutic evolution. International regulatory agencies have approved many specific drugs with different pharmacologic pathways and routes of administration. In the year 2013, two new drugs with great potentials in managing PAH have been added to the treatment options, macitentan and riociguat. Additional drugs are expected to come in the near future. A substantial body of evidence has confirmed the effectiveness of pulmonary arterial hypertension (PAH)-specific therapies in improving the patients' symptomatic status and slowing down the rate of clinical deterioration. Although the newer modern medications have significantly improved the survival of patients with PAH, it remains a non-curable and fatal disease. Lung transplantation (LT) remains the only therapeutic option for selected patients with advanced disease who continue to deteriorate despite optimal therapy.

Chronic thromboembolic pulmonary hypertension (CTEPH) is categorized as group IV in the WHO classification for pulmonary hypertension. The disease requires a very low index of suspicion for identification and needs a special diagnostic approach utilizing clinical, radiological, and hemodynamic tools. As CTEPH is potentially curable, all efforts should be consumed to reach the accurate diagnosis and subsequently evaluated for operability. Although pulmonary endarterectomy (PEA) is the only curative tool so far, recent updates concerning medical and interventional therapy have made significant advances in inoperable patients. In this review, we provide a detailed discussion on diagnostic algorithm, surgical operability criteria, PEA, and the medical therapy.

Treatment of pulmonary hypertension (PH) patients is challenging and should only be initiated after a comprehensive diagnostic evaluation. Such treatment should ideally be done in specialized centers with full capability for hemodynamic measurements, having access to a broad range of PAH therapies, and adequate experience in the management of critically ill patients. The following discussion is intended to review the general measures and the non-specific (supportive) therapy used in managing PH patients, while the specific therapy will be discussed in a subsequent different article.

Pulmonary hypertension (PH) due to left heart disease is the most common cause of pulmonary hypertension in the western world. It is classified as WHO PH group II. Different pathophysiologic abnormalities may take place in this condition, including pulmonary venous congestion and vascular remodeling. Despite the high prevalence of WHO group 2 PH, the major focus of research on PH over the past decade has been on WHO group 1 pulmonary arterial hypertension (PAH). Few investigators have focused on WHO group 2 PH; consequently, the pathophysiology of this condition remains poorly understood, and no specific therapy is available. Clinical and translational studies in this area are much needed and have the potential to positively affect large numbers of patients. In this review, we provide a detailed discussion upon the pathophysiology of the disease, the recent updates in classification, and the diagnostic and therapeutic algorithms.