scholarly journals Pulmonary arterial hypertension in patients treated with interferon

2014 ◽  
Vol 44 (6) ◽  
pp. 1627-1634 ◽  
Author(s):  
Laurent Savale ◽  
Caroline Sattler ◽  
Sven Günther ◽  
David Montani ◽  
Marie-Camille Chaumais ◽  
...  

Isolated cases of pulmonary arterial hypertension (PAH) in patients treated with interferon (IFN) α or β have been reported in the literature.The aim of this study was to describe all consecutive cases of PAH patients with a history of IFN exposure identified in the French reference centre for severe pulmonary hypertension between 1998 and 2012.A total of 53 patients with PAH and a history of IFN therapy were identified. 48 patients had been treated with IFNα for chronic hepatitis C. Most of them had portal hypertension (85%) and 56% had HIV co-infection. Five additional patients had been treated with IFNβ for multiple sclerosis. The diagnosis of PAH was made within 3 years after IFN therapy in 66% of patients. Repeated haemodynamic assessment was available in 13 out of 16 patients exposed to IFN after the diagnosis of PAH. Increased pulmonary vascular resistance >20% was observed in 11 out of 13 cases (median 43% increase; IQR 32–67%). In five of these patients, IFN withdrawal resulted in spontaneous haemodynamic improvement.This retrospective analysis suggests that IFN therapy may trigger PAH. However, most of these patients had other risk factors for PAH. A prospective case–control study is necessary to definitively establish a link between IFN exposure and PAH.

2020 ◽  
Vol 10 (1) ◽  
pp. 204589402090878
Author(s):  
Jessica B. Badlam ◽  
David Badesch ◽  
Evan Brittain ◽  
Shannon Cordell ◽  
Tan Ding ◽  
...  

Pulmonary arterial hypertension (PAH) is a sexually dimorphic disease that for unknown reasons affects women more than men. The role of estrogens, both endogenous and exogenous, and reproductive factors in this female susceptibility is still poorly understood. It has been strongly suggested that sex hormones may influence the development and progression of the disease. We sought to determine whether sex hormone exposures and reproductive factors associate with PAH patients compared to control subjects, using a questionnaire and interview to obtain information regarding these potential risk factors. We conducted a single-center unmatched case–control study. Six hundred and thirty-four women and men with PAH, as well as 27 subjects with BMPR2 mutations but no PAH and 132 healthy population controls were enrolled from the Vanderbilt Pulmonary Hypertension Research Cohort and researchmatch.org. Questionnaires and nurse-led interviews were conducted to obtain information regarding sex hormone exposures and reproductive factors. Additional history was obtained on enrolled patients including disease severity variables and comorbidities. Responses to the questionnaires were analyzed to describe these exposures in this population as well as assess the association between disease severity variables and sex hormone exposures. Reproductive and endogenous factors that determine lifelong estrogen exposure were similar between PAH cases and controls. Patients with associated PAH were significantly more likely to be postmenopausal compared to controls. There were similar rates of “ever-use” and duration of use of oral contraceptive pills and hormone replacement therapy in patients when compared to controls. Disease severity variables were not significantly affected by any exposure after adjusting for PAH sub-group. In contrast to our hypothesis, that a greater exposure to exogenous sources of female sex hormones associates with PAH case status, we found similar rates of endogenous and exogenous sex hormone exposure between PAH patients and unmatched controls.


Author(s):  
Sophie Vergnaud ◽  
David Dobarro ◽  
John Wort

A 16-year-old girl with a diagnosis of diffuse cutaneous systemic sclerosis is referred to a specialist pulmonary hypertension centre with a history of progressive breathlessness, reduced exercise tolerance, and raised pulmonary pressures on transthoracic echocardiogram. She is found to have pulmonary arterial hypertension on right cardiac catheterization and is started on sildenafil, a phosphodiesterase-5 inhibitor, which stabilizes her condition. An endothelin receptor antagonist is added, which provides some initial symptomatic improvement. She continues to deteriorate over a period of 5 years, ultimately requiring intravenous prostanoids, the only treatment to provide a real symptomatic and haemodynamic improvement. This chapter explores the physiology and pathophysiology of pulmonary arterial hypertension, its classification, the means of investigation and diagnosis, who to refer to specialist centres, and the concepts behind current and future treatment strategies.


Author(s):  
Jessica K. Paulus ◽  
Karen M. Switkowski ◽  
Ioana R. Preston ◽  
Nicholas S. Hill ◽  
Kari E. Roberts

2020 ◽  
Author(s):  
Nan Liu ◽  
Ying Xing ◽  
Chen Wang

AbstractPulmonary arterial hypertension (PAH) is a vascular disorder associated with significant morbidity and mortality. The pathophysiology of PAH remains controversial, but the only currently available therapies for PAH are pharmacological pulmonary artery vasodilation, decreasing right ventricular (RV) afterload, and relieving symptoms. By now, there is no therapy being able to minimize vascular remodeling processes and thus to reverse or delay the natural history of the disease. It has been generally thought that reduction of RV preload was detrimental, which deteriorated the systemic hemodynamics. In the present study, however, we repetitively and briefly occluded (RBO) both superior vena cava and inferior vena cava by ligation (occlusion for less than 5 seconds then re-open for 30 seconds and repeated 5 cycles as one sequence, 1 sequence every 6 hours) to intermittently restrict RV preload, for continuous 24 hours, total 5 sequences, in the Sugen 5416 (VEGF receptor blocker) and hypoxia induced PAH rat models and we found this strategy was beneficial for lowering pulmonary vascular resistance (PVR).


2018 ◽  
Vol 17 (2) ◽  
pp. 55-62 ◽  
Author(s):  
Nimaljeet Tarango ◽  
Andrea Gergay Baird

Pulmonary arterial hypertension (PAH) is a serious, chronic, progressive cardiopulmonary disease. PAH is associated with several concomitant conditions, as well as drugs and toxins.12 Methamphetamine abuse is likely associated with the development of PAH.3 Methamphetamine abuse is epidemic in the United States and abroad, with rates of new users escalating since 2012. There are over 100,000 new users annually as young as 12 years old. Treating a patient with a history of methamphetamine abuse poses many challenges for a clinician, including nonadherence, therapeutic treatment selection, complex psychosocial issues, and relapse or continued drug abuse. Patients with methamphetamine-associated PAH (Meth-APAH) have higher mortality rates when compared to idiopathic PAH.3 Having a better understanding of the complexities of addiction and working with a multidisciplinary team that includes a social worker to provide care and counseling to these patients can improve their trajectory. In this article, we will offer insight and background into methamphetamine abuse and addiction, as well as discuss a practical approach for clinicians in treating a patient with Meth-APAH, based on the literature, as well as our personal experiences at University of California, San Francisco Medical Center.


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