Prospective clinical assessment of patients with pulmonary arterial hypertension switched from bosentan to macitentan (POTENT).

Even though pulmonary arterial hypertension (PAH) remains an incurable disease, the combination of PAH-specific therapies allowed evolving from symptom-based strategies to others aiming to move patients to low-risk conditions. Endothelin-1 (ET-1) receptor antagonists emerged as specific-PAH drugs that can be used in combination with other specific therapies. This work aimed to perform a prospective clinical assessment of patients with PAH that switched from bosentan to macitentan (POTENT), due to inadequate response.

Tuberculosis presenting as metastatic lung cancer.

Mycobacterium tuberculosis infection (TB) masquerading as lung tumor is well reported, but its mimicry as metastatic thoracic cancer is rare. We report the case of a young male who presented with clinical and radiological picture of lung cancer but investigations confirmed it as TB. A 35-year-old male, with 18-pack year of smoking history, presented with dry cough, anorexia, weight loss, and lower back and left hip pain.

Genetic basis of pulmonary arterial hypertension: a prospective study from a highly inbred population.

Pulmonary arterial hypertension (PAH), whether idiopathic PAH (IPAH), heritable PAH, or associated with other conditions, is a rare and potentially lethal disease characterized by progressive vascular changes. To date, there is limited data on the genetic basis of PAH in the Arab region, and none from Saudi Arabian patients. This study aims to identify genetic variations and to evaluate the frequency of risk genes associated to PAH, in Saudi Arabian patients.

Role of Fluorodeoxyglucose Positron Emission Tomogram Scan in Sirolimus-Induced Lung Toxicity: A Rare Case Report.

Lung toxicity is a rare but serious side effect of sirolimus, a mammalian target of rapamycin inhibitor used as an immunosuppressive agent in solid-organ transplant recipients. We report a case of 67-year-old man who had living-related renal transplant 12 years previously that was complicated by chronic allograft dysfunction. He presented with fever, cough, and shortness of breath, and his chest imaging showed bilateral patchy and ground glass opacities.

Pulmonary hypertension in Saudi Arabia: First data from the SAUDIPH registry with a focus on pulmonary arterial hypertension.

Background And Objective: This study presents the first results of 'SAUDIPH' registry, aiming to assess patient characteristics, treatment approach and clinical and survival outcomes in patients with PAH.

Methods: The registry enrolled patients with Group 1 and Group 4 PH under clinical management in a specialized tertiary care centre from 2004 to 2018. Changes from baseline to last follow-up visit were assessed.

Chronic thromboembolic pulmonary hypertension in Saudi Arabia: preliminary results from the SAUDIPH registry.

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive vascular disease with poor prognosis if left untreated. This study aims to assess the patient characteristics, treatment approach and clinical and survival outcomes for CTEPH patients enrolled in the Systematic Prospective Follow Up for Better Understanding of Clinical Characteristics of Patients with Pulmonary Hypertension Disease (SAUDIPH) registry.

Methods: This study presents a subanalysis of CTEPH patients enrolled in the SAUDIPH registry.

Superior vena cava stenting in IgG4-associated mediastinal fibrosis.

We report a rare case of IgG4-associated mediastinal fibrosis with complete superior vena cava (SVC) obstruction successfully managed by thrombolysis and stenting in a 33-year-old male. The patient presented with a mediastinal mass lesion with clinical findings of SVC obstruction. Surgical biopsy of the mediastinal mass lesion with histology and immunohistochemistry staining established the diagnosis of IgG4 associated mediastinal fibrosis.

Pulmonary arterial hypertension in Saudi patients with systemic sclerosis: Clinical and hemodynamic characteristics and mortality.

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.

Adherence to COPD treatment in Turkey and Saudi Arabia: results of the ADCARE study.

Background: COPD affects millions of people worldwide. Poor treatment adherence contributes to increased symptom severity, morbidity and mortality. This study was designed to investigate adherence to COPD treatment in Turkey and Saudi Arabia.

Middle Eastern Respiratory Syndrome Corona Virus (MERS CoV): case reports from a tertiary care hospital in Saudi Arabia.

Background And Objectives: Middle Eastern respiratory syndrome caused by novel coronavirus (MERS CoV) has been a major public health challenge since it was first described in 2012 in Saudi Arabia. So far, there is no effective treatment for this serious illness, which features a high mortality rate. We report an initial experience of the use of ribavirin and interferon (IFN)-a2b in the management of MERS CoV at a tertiary care hospital.

Tracheobronchial anomalies in a patient with Schimke immuno-osseous dysplasia (SIOD).

Schimke Immuno-osseous Dysplasia (SIOD) is a rare genetic disorder with multiple systemic manifestations. Pulmonary manifestations have been described but not well characterized. They are believed to be secondary to decreased elasticity, and include emphysema, pulmonary hypertension and bronchiectasis.

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary hypertension associated with hemolytic anemia.

Hereditary hemoglobin disorders affecting the globin chain synthesis namely thalassemia syndromes and sickle cell disease (SCD) are the most common genetic disorders in human. Around 7% of the world population carries genes for these disorders, mainly the Mediterranean Basin, Middle and Far East, and Sub-Saharan Africa. An estimated 30 million people worldwide are living with sickle cell disease, while 60-80 million carry beta thalassemia trait.

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Portopulmonary hypertension.

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.

Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: 2014 updates.

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.

Gastroesophageal reflux disease and its association with bronchiolitis obliterans syndrome in allogeneic hematopoietic stem cell transplant recipients.

Objectives: Bronchiolitis obliterans syndrome is a significant postallogeneic hematopoietic stem cell transplant problem. Recent data in lung transplant patients suggest an association with gastroesophageal reflux disease and bronchiolitis obliterans syndrome. We studied posthematopoietic stem cell transplant patients with bronchiolitis obliterans syndrome for gastroesophageal reflux disease and its response to a proton pump inhibitor.

Portopulmonary hypertension.

Portopulmoanry hypertension (POPH) is a form of pulmonary arterial hypertension (PAH) associated with portal hypertension with or without underlying chronic liver disease. POPH is increasingly recognized and recent evidence suggests that it is one of the leading causes of PAH. The pathophysiology of POPH is poorly understood although the pathological changes in pulmonary vasculature in advanced POPH are similar to those seen in idiopathic pulmonary hypertension.

Effect of obstructive airway disease in patients with non-cystic fibrosis bronchiectasis.

Background: Extensive research has been devoted to cystic fibrosis-related brochiectasis, compared with non-cystic fibrosis bronchiectasis but the latter is more common and results in significant morbidity and mortality. We assessed the relationship between pulmonary function test (PFT) findings and sputum bacteriology, blood gases, number of hospital admissions and mortality in patients with non-cystic fibrosis bonchiectasis (NCFB).

Methods: We conducted a retrospective review of 88 consecutive patients admitted with exacerbation of bronchiectasis over 5 years from 1996 to 2001.

Tuberculosis presenting as endobronchial tumor.

Endobronchial tuberculosis TB can be present in various forms including ulceration, hyperemia and granulation tissue and rarely as mass lesion. We present a case of TB presenting as endobronchial tumor mimicking lung cancer on bronchoscopic examination. The histology of the tumor showed caseating granuloma and bronchial lavage culture was positive for mycobacterium TB.