Delphi consensus recommendation for optimization of pulmonary hypertension therapy focusing on switching from a phosphodiesterase 5 inhibitor to riociguat.

Dual combination therapy with a phosphodiesterase-5 inhibitor (PDE5i) and endothelin receptor antagonist is recommended for most patients with intermediate-risk pulmonary arterial hypertension (PAH). The RESPITE and REPLACE studies suggest that switching from a PDE5i to a soluble guanylate cyclase (sGC) activator may provide clinical improvement in this situation. The optimal approach to escalation or transition of therapy in this or other scenarios is not well defined.

Real-world dosing characteristics and utilization of parenteral treprostinil in the outpatient setting.

Real-world dosing and titration of parenteral (subcutaneous, SC; intravenous, IV) prostacyclin, a mainstay of pulmonary arterial hypertension (PAH) treatment, is not always consistent with prescribing information or randomized trials and has yet to be adequately characterized. The current study describes real-world outpatient dosing and titration patterns over time, in PAH patients initiated on SC or IV treprostinil. A longitudinal, cross-sectional analysis of medication shipment records from US specialty pharmacy services between 2009 and 2018 was conducted to determine dosing and titration patterns of SC or IV treprostinil in the outpatient setting beginning with the patient's first shipment.

Participation in pulmonary hypertension support group improves patient-reported health quality outcomes: a patient and caregiver survey.

Support group participation has been shown to be effective in many chronic medical conditions. The evidence for integrating support group into pulmonary hypertension care and its effect on quality of life, physical and psychological well-being is limited. We sought to assess the effect of support group participation on quality of life in patients diagnosed with pulmonary hypertension and their caregivers.

An expert panel delphi consensus statement on patient selection and management for transitioning between oral and inhaled treprostinil.

Treprostinil, a prostacyclin analogue used in the treatment of pulmonary arterial hypertension (PAH), is available for administration by parenteral, oral, or inhaled routes. Transitioning between routes may be beneficial for appropriate patients; however, there is little published data on transitions between oral and inhaled treprostinil. We used a modified Delphi process to develop expert consensus recommendations on transitions between these formulations.

Dosing characteristics of oral treprostinil in real-world clinical practice.

Pharmacokinetic studies with oral treprostinil demonstrate that three times daily (TID) dosing reduces peak-to-trough plasma trepostinil fluctuations compared with twice daily (BID) dosing. TID dosing may allow for faster titration, higher total daily doses, and potentially improve the tolerability of oral trepostinil. This analysis, which looks at the real-world dosing of oral treprostinil, supports the utility of TID dosing.

Recommendations for the use of oral treprostinil in clinical practice: a Delphi consensus project pulmonary circulation.

Oral treprostinil was recently labeled for treatment of pulmonary arterial hypertension. Similar to the period immediately after parenteral treprostinil was approved, there is a significant knowledge gap for practicing physicians who might prescribe oral treprostinil. Despite its oral route of delivery, use of the drug is challenging because of the requirement for careful titration and management of drug-related adverse effects.

Recommendations for the clinical management of patients receiving macitentan for pulmonary arterial hypertension (PAH): A Delphi consensus document.

In patients treated with macitentan (Opsumit®, Actelion Pharmaceuticals Ltd., Basel, Switzerland) for pulmonary arterial hypertension (PAH), prevention and/or effective management of treatment-related adverse events may improve adherence. However, management of these adverse events can be challenging and the base of evidence and clinical experience for macitentan is limited.

Pneumocystis carinii pneumonia with pleural effusion in a non-HIV host.

Pneumocystis carinii pneumonia (PCP) is a life-threatening opportunistic infection that occurs in immunocompromised hosts, especially patients with the acquired immunodeficiency syndrome (AIDS). However, this infection is increasing in frequency in other immunosuppressed patients, including organ transplant recipients and those with malignancy who are treated with chemotherapeutic regimens. It carries a relatively high mortality in the non-human immunodeficiency virus (HIV) population.

Chronic obstructive pulmonary disease: effects beyond the lungs.

Purpose Of Review: This article reviews the systemic effects of chronic obstructive pulmonary disease in general and the musculoskeletal effects and cachexia in particular.

Recent Findings: Shift in muscle fiber type from I to II, reduction of capillary/mitochondria ratio, reduction of oxidative enzymes and oxidative capacity, alteration in amino acid profile, changes in respiratory muscle dynamics, activation of the ubiquitin-proteosome pathway with loss of contractile proteins are some of the factors found to cause muscle dysfunction in chronic obstructive pulmonary disease. Chemotaxis and capability to produce more reactive oxygen species and to cause extracellular proteolysis are enhanced in neutrophils of chronic obstructive pulmonary disease patients.