Pulmonary arterial hypertension (PAH) is a devastating disease that without specific therapy is characterised by a progressive increase in pulmonary vascular resistance (PVR), leading to right ventricular failure and ultimately death. Among the conditions associated with PAH, connective tissue disease-associated PAH (CTD-PAH) is linked with the gravest prognosis (one-year survival of 50%) without appropriate therapy.
Extraordinary advances in PAH treatment have been made in the last 10 years, benefiting not only patients with idiopathic PAH (IPAH), but also those with CTD-PAH. CTD-PAH is easily overlooked, as many of these patients will have breathlessness secondary to interstitial lung disease or myocardial involvement. Thus, PAH should be considered in all patients with CTD presenting with breathlessness in the absence of obvious other cardio-respiratory disease. This review will discuss the evidence base for current therapies and consider the potential role for combination therapy.
Most data in CTD-PAH have been acquired from subanalysis of larger studies. Although scleroderma is a relatively uncommon connective tissue disease, the high prevalence of PAH associated with scleroderma results in systemic sclerosis-associated PAH (SSc-PAH) being the most common form of CTD-PAH. Most available data concerning CTD-PAH are derived from the SSc-PAH population, and these data are used to inform our management of other forms of CTD-PAH. This may not be an entirely valid approach: clinical experience shows us that lupus patients respond better to endothelin receptor antagonist (ERA) therapy than SScPAH patients, and both lupus and mixed connective tissue disease (MCTD) patients are more likely to respond to immunosuppressive therapies than patients with scleroderma. Importantly, with current treatments the one-year survival in SSc-PAH remains around 80%, while in mixed populations with significant numbers of lupus and MCTD patients one-year survival is closer to 90%.
Pulmonary embolism (PE) is a common, treatable, highly lethal emergency, which despite advances in diagnostic testing, remains an under diagnosed killer. PE is currently the third leading cause of death in the United States, with 50,000 to 100,000 estimated deaths per year and an incidence of 0.5 to 1 per 1000. Studies in the Indian subcontinent show a low incidence of Venousthroembolism (VTE). The mortality rate of diagnosed and treated pulmonary embolism ranges from 3-8%, but increases to about 30% in untreated pulmonary embolism. PE is a part of the spectrum of venousthromboembolic disease and most pulmonary emboli have their origin from clots in the iliac, deep femoral, or popliteal veins. Non-specific clinical signs and symptoms with low sensitivity and specificity of routine tests such as arterial blood gas (ABG), chest roentgenogram (CXR) and electrocardiogram (ECG) make the diagnosis of PE very challenging for the clinician. Pulmonary angiography (PA) is the gold standard diagnostic test, but this technique is invasive, expensive, not readily available and labor intensive. Diagnostic strategies have revolved around establishing clinical probabilities based on predictive models, then ruling in or ruling out the diagnosis of PE with various tests. The aim of this article was to review the literature and present an evidence- based medicine approach to diagnosis of pulmonary embolism.
Idiopathic pulmonary fibrosis (IPF) is a devastating disease. Marked by progressive breathlessness, impaired quality of life and functional status, and a median survival of 2.5–5 years, IPF remains resistant to currently available medical therapy. However, recent advances suggest a more promising future: the recognition of its clinical impact and increasing prevalence, the successful completion of multiple controlled, multicenter trials, and fresh basic scientific discoveries have led to a better understanding of IPF’s natural history and molecular underpinnings. The results of a number of currently active clinical studies and basic investigation are hastening the day of at least disease control, if not cure.
Clarinex-D 12 Hour is a new twice-daily antihistamine and decongestant formulation for the relief of nasal and non- nasal symptoms of seasonal allergic rhinitis (outdoor allergies), including nasal congestion, in patients 12 years of age and older.
Clarinex-D 12 Hour is a new twice-daily antihistamine and decongestant formulation for the relief of nasal and non- nasal symptoms of seasonal allergic rhinitis (outdoor allergies), including nasal congestion, in patients 12 years of age and older.
