Pulmonary arterial hypertension in patients treated with interferon

Savale, Laurent; Chaumais, Marie-Camille; Sitbon, Olivier; Humbert, Marc

doi:10.1183/13993003.01376-2015

Cited by 44 publications

(36 citation statements)

References 13 publications

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“…It is difficult to directly correlate the two as many of these patients had concomitant liver disease and/or HIV, both of which are independently associated with the development of PAH. These patients developed disease approximately 3 years after drug initiation that was largely irreversible with drug discontinuation (125).…”

Section: Interferon Alpha and Betamentioning

confidence: 99%

Drug- and toxin-induced pulmonary arterial hypertension: Current state of the literature

Ramírez

Thomas

Anderson

et al. 2020

gcsp

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Drug- and toxin-induced pulmonary arterial hypertension (PAH) is an increasingly important sub-group of group 1 pulmonary hypertension (PH). In the last 60 years, we have seen the rise and fall of numerous prescription and illicit agents that have caused severe and deadly outbreaks of PAH. Currently, drugs and toxins are classified into “possible” and “definite” risk factors for PAH. While the exact mechanisms and pathogenesis of drug- and toxin-induced PAH are currently unknown, novel clinical and basic science studies are beginning to unravel the biologic factors and genetic underpinnings responsible for disease development. The clinical management of affected patients can be challenging as it is often difficult to identify patients early and demonstrate causality between drugs and PAH. Given the recent trends in drug utilization and development, it is highly likely that we will continue to identify new agents capable of causing pulmonary vascular disease. We must keep a high index of suspicion in order to identify patients and new compounds deemed definite or likely risk factors for PAH. Practicing pharmacovigilance, raising awareness, and working in tandem with PH patient associations and drug regulatory agencies may ultimately be our most effective strategy in preventing the next deadly outbreak of drug- and toxin-induced PAH.

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Section: Interferon Alpha and Betamentioning

confidence: 99%

Drug- and toxin-induced pulmonary arterial hypertension: Current state of the literature

Ramírez

Thomas

Anderson

et al. 2020

gcsp

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“…Τhe Sixth World Symposium on Pulmonary Hypertension, based on recent data, proposed a simplified characterisation of PAH associated with drugs and toxins into two subgroups (definite and possible) to help physicians to identify drugs requiring specific surveillance. According to this framework, and supported by case series and case reports, IFN-β is classified as a drug with possible association with PAH development [2].In the literature, there are several cases of patients with multiple sclerosis (MS) on treatment with IFN-β who were diagnosed with PAH associated with the IFN-β exposure [3][4][5][6][7][8][9]. The need to identify and present related cases has been highlighted [4,5].We report a case of a patient with MS on treatment with IFN-β who subsequently developed PAH.…”

mentioning

confidence: 99%

“…According to this framework, and supported by case series and case reports, IFN-β is classified as a drug with possible association with PAH development [2].In the literature, there are several cases of patients with multiple sclerosis (MS) on treatment with IFN-β who were diagnosed with PAH associated with the IFN-β exposure [3][4][5][6][7][8][9]. The need to identify and present related cases has been highlighted [4,5].We report a case of a patient with MS on treatment with IFN-β who subsequently developed PAH. The initial suspicion of PAH, the subsequent diagnosis and management, and the final outcome are presented in order to underline the reversibility of PAH following IFN-β discontinuation and specific PAH therapy administration, and the emerging need to early detect similar cases.A 32-year-old female was diagnosed with relapsing remitting MS on 2010 with an Expanded Disability Status Scale score of 1 and negative immunological indices.…”

mentioning

confidence: 99%

“…In the literature, there are several cases of patients with multiple sclerosis (MS) on treatment with IFN-β who were diagnosed with PAH associated with the IFN-β exposure [3][4][5][6][7][8][9]. The need to identify and present related cases has been highlighted [4,5].…”

mentioning

confidence: 99%

“…Βased on reported cases [3][4][5][6][7][8][9], IFN-β was discontinued and the patient received glatiramer acetate for 2 years. After a severe optic neuritis, she is currently treated with natalizumab.…”

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confidence: 99%

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Is pulmonary arterial hypertension associated with interferon-β therapy for multiple sclerosis reversible? A case study to explore the complexity

et al. 2020

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Interferon (IFN)-β has been classified as a drug that is possibly associated with development of pulmonary arterial hypertension (PAH), a devastating disease that can lead to right heart failure and premature death [1].Drug-and toxin-induced PAH is a well-known entity in the field of pulmonary hypertension (PH) since the epidemic of idiopathic PAH occurred in the central Europe, between 1967 and 1971, following the use of anorexigens. Τhe Sixth World Symposium on Pulmonary Hypertension, based on recent data, proposed a simplified characterisation of PAH associated with drugs and toxins into two subgroups (definite and possible) to help physicians to identify drugs requiring specific surveillance. According to this framework, and supported by case series and case reports, IFN-β is classified as a drug with possible association with PAH development [2].In the literature, there are several cases of patients with multiple sclerosis (MS) on treatment with IFN-β who were diagnosed with PAH associated with the IFN-β exposure [3][4][5][6][7][8][9]. The need to identify and present related cases has been highlighted [4,5].We report a case of a patient with MS on treatment with IFN-β who subsequently developed PAH. The initial suspicion of PAH, the subsequent diagnosis and management, and the final outcome are presented in order to underline the reversibility of PAH following IFN-β discontinuation and specific PAH therapy administration, and the emerging need to early detect similar cases.A 32-year-old female was diagnosed with relapsing remitting MS on 2010 with an Expanded Disability Status Scale score of 1 and negative immunological indices. She had received therapy with IFN-β for 6 years when she presented to our PH clinic with a 4-month history of dyspnoea on exertion, fatigue, dizziness and episodes of presyncope. She thought her symptoms were MS-related. Her past medical history was otherwise unremarkable, with no known risk factors for PAH.

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Sex, Gender, and Sex Hormones in Pulmonary Hypertension and Right Ventricular Failure

Hester

Ventetuolo

Lahm

2019

Comprehensive Physiology

110

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Pulmonary hypertension (PH) encompasses a syndrome of diseases that are characterized by elevated pulmonary artery pressure and pulmonary vascular remodeling and that frequently lead to right ventricular (RV) failure and death. Several types of PH exhibit sexually dimorphic features in disease penetrance, presentation, and progression. Most sexually dimorphic features in PH have been described in pulmonary arterial hypertension (PAH), a devastating and progressive pulmonary vasculopathy with a 3-year survival rate <60%. While patient registries show that women are more susceptible to development of PAH, female PAH patients display better RV function and increased survival compared to their male counterparts, a phenomenon referred to as the "estrogen paradox" or "estrogen puzzle" of PAH. Recent advances in the field have demonstrated that multiple sex hormones, receptors, and metabolites play a role in the estrogen puzzle and that the effects of hormone signaling may be time and compartment specific. While the underlying physiological mechanisms are complex, unraveling the estrogen puzzle may reveal novel therapeutic strategies to treat and reverse the effects of PAH/PH. In this article, we (i) review PH classification and pathophysiology; (ii) discuss sex/gender differences observed in patients and animal models; (iii) review sex hormone synthesis and metabolism; (iv) review in detail the scientific literature of sex hormone signaling in PAH/PH, particularly estrogen-, testosterone-, progesterone-, and dehydroepiandrosterone (DHEA)-mediated effects in the pulmonary vasculature and RV; (v) discuss hormone-independent variables contributing to sexually dimorphic disease presentation; and (vi) identify knowledge gaps and pathways forward.

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Pulmonary arterial hypertension in patients treated with interferon

Cited by 44 publications

References 13 publications

Drug- and toxin-induced pulmonary arterial hypertension: Current state of the literature

Drug- and toxin-induced pulmonary arterial hypertension: Current state of the literature

Is pulmonary arterial hypertension associated with interferon-β therapy for multiple sclerosis reversible? A case study to explore the complexity

Sex, Gender, and Sex Hormones in Pulmonary Hypertension and Right Ventricular Failure

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