Pulmonary arterial hypertension (PAH) is a rare, debilitating and rapidly progressive disease. Although there have been important medical advances in PAH management, the search for a cure continues. Despite an increased understanding of the disease, data on the wider effect of PAH on patients and carers, beyond the clinical symptoms, are still limited. In order to explore this, a large-scale international survey investigated four key areas affected by PAH (physical and practical, emotional, social, and information needs) and provides new insight into patients' and carers' experiences of living with the disease. The results from the survey highlight not only the limited ability of patients to carry out everyday tasks, but also the financial impact and social isolation experienced by both patients and carers. The study confirmed that a decline in a patient's World Health Organization functional class, which indicates an increase in clinical severity of the disease, is associated with greater limitations. Results from the survey demonstrate the need for multidisciplinary PAH management and a comprehensive standard of care to assess and improve all aspects of well-being for both patients and carers. In addition, they underline the need for updated PAH guidelines that address these needs. @ERSpublications PAH has a profound impact on numerous aspects of patients' and carers' lives http://ow.ly/pWWYf This article has supplementary material available from err.ersjournals.comFull survey results, along with a call to action, are available at www.phaeurope.org/projects-activities/pah-patient-andcarer-survey

Therapies that target the prostacyclin pathway are considered effective, yet are complex to dose and may cause dose-limiting side effects for patients with pulmonary arterial hypertension (PAH). Careful side effect management and the ability to discern side effects from worsening disease are essential in order for patients to continue, and benefit from, prostacyclin therapy. This manuscript was developed through a collaborative effort of allied health providers with extensive experience in managing patients with PAH who are treated with medications that target the prostacyclin pathway. This article provides an overview of individual prostacyclin pathway therapies approved in the United States, side effects most commonly associated with these therapies, and practical suggestions for side effect management. Most patients will experience significant side effects on prostacyclin therapy. Creating a proactive and careful side effect management program will increase the likelihood that patients are able to stay on therapy and receive the benefits afforded by prostacyclin therapy.

clinicaltrials.gov Identifier: NCT00370214.

BACKGROUND Epoprostenol and treprostinil are intravenous prostacyclin medications used to treat pulmonary arterial hypertension (PAH). This survey explored hospital policies regarding prostacyclin infusions, and investigated the type and frequency of errors that occurred in the inpatient setting. METHODS Information on prostacyclin infusion policies and inpatient errors was obtained through detailed interviews with 18 PAH nurses, and through an electronic survey completed by 97 PAH clinicians. RESULTS The electronic survey respondents reported wide variability in prostacyclin infusion policies, including variability in the use of home vs hospital infusion pumps, and variability in the use and storage of back-up epoprostenol and treprostinil. Serious or potentially serious errors in medication administration were reported by 68% of survey respondents. The most common error types (reported by ≥25%), included: incorrect cassette placed in the pump; inaccurate pump programming; errant drug dosing; and inadvertent cessation of the pump. Nine errors, all at different centers, were believed to have contributed to patient death. In the separate interviews with the PAH nurses, 94% reported serious errors. These errors prompted many of the centers to implement policy changes in an attempt to reduce future errors, improve safety and optimize patient outcomes. CONCLUSIONS These findings suggest that prostacyclin infusion therapy is problematic and that an opportunity exists to improve safety. The development of standardized treatment guidelines should be considered.

Abstract:Objective: B-cell expansion is a key feature of Sjögren's syndrome (SS). Accordingly, several studies have reported the benefits of B-cell depletion with anti-CD20 monoclonal antibody (Rituximab) in the treatment of glandular and extraglandular manifestations of SS. Patients with SS are at increased risk of lymphoma development. B-lymphocyte stimulator (BAFF) is an essential cytokine for the control of B-cell maturation and survival, and high levels of BAFF were described in the serum and salivary glands of SS patients, strongly suggesting a crucial role in the proliferation of B cells in SS. Patient and Methods:we describe the treatments employed, with particular regards to rituximab therapy, and the histopathologic and biologic studies, in particular BAFF levels in serum and in pathologic tissues before and after B-cell depletion therapy, and the characterization of the cultured epithelial cells obtained by the parotid gland MALT-lymphoma, in a case of a 51-year old woman with primary SS and mixed cryoglobulinaemia type II with features of systemic vasculitis, who developed a bilateral parotid MALT-type lymphoma. Rheumatoid factor (RF), cryoglobulins, BAFF levels were assessed monthly up to month +6, then at the end of follow-up (month +12), as well as peripheral blood CD19-positive Bcell level.Results: a significant systemic effect of rituximab on B-cell biomarkers was documented, however, the cryoglobulinemic syndrome did not improve and the parotid enlargement did not decrease confirming the failure of B-cell depletion to affect the parotid lymphoma. BAFF levels decreased only under B-cell depletion associated with high-dose steroids. Tissue studies further documented the persistent overexpression of BAFF in the salivary gland pathologic tissue during the disease course. Conclusion:Tissue and systemic overexpression of BAFF may have contributed to resistance to rituximab therapy, in MALT lymphoproliferation associated with SS. Thus, alternative treatment strategies should be then considered, possibly including BAFF-targeted approaches.