Impact of switching from epoprostenol to IV treprostinil on treatment satisfaction and quality of life in patients with pulmonary hypertension.

BACKGROUND: Transition from intravenous (IV) epoprostenol to IV treprostinil in patients with pulmonary hypertension (PH) has traditionally been performed by gradually decreasing the epoprostenol dose while increasing the treprostinil dose. Preliminary data suggest that this transition can be performed more rapidly without the need for epoprostenol weaning. We conducted a single center, prospective clinical trial to assess the safety, efficacy, and treatment satisfaction of rapidly switching from epoprostenol to IV treprostinil. METHODS: This study included patients with PH who had rapidly transitioned from epoprostenol to IV treprostinil. Data collected included clinical status, adverse events, PH symptoms, and previously validated measures of quality of life and treatment satisfaction. RESULTS: Ten patients were enrolled in this study. Exercise capacity measured by mean 6-min walk distance was maintained from baseline throughout follow-up. Severity of disease as assessed by WHO functional class was maintained or improved for the majority of patients. Adverse events were minimal during the transition, and all patients remained on IV treprostinil throughout the follow-up period. A favorable impact on quality of life and treatment satisfaction measures was observed by eight weeks following the transition from epoprostenol to IV treprostinil. Specifically, time spent on drug preparation activities decreased by 39.5% with treprostinil compared to epoprostenol. CONCLUSIONS: Rapidly switching from epoprostenol to IV treprostinil can be achieved without safety concerns, with minimal patient monitoring and without the need for extended hospitalization, while favorably impacting on patients' quality of life.

Effectiveness and safety of leflunomide for pulmonary and extrapulmonary sarcoidosis.

Leflunomide has been reported as an alternative therapy in sarcoidosis. However, the published data are limited. We performed a retrospective chart review of the tolerance and effects of leflunomide therapy in patients with sarcoidosis. 76 patients were included. The most common reasons for initiation were progression of disease or failure of other immunomodulator therapy. Side-effects attributable to leflunomide were noted in 34% of subjects, prompting discontinuation in 17%. The lungs were a target of therapy in 33 (44%) and extrapulmonary organs were a target in 45 (59%). The mean ± sd change in forced vital capacity in the 6 months prior to leflunomide was -0.1 ± 0.3 L, and it was +0.09 ± 0.3 L in the following 6 months (p=0.01). For extrapulmonary target organ response, 51% had a good response and 32% a partial response. The median corticosteroid dose at initiation was 10 mg (interquartile range 5-20) mg at baseline, and 0 (0-10) mg at the 6-month follow-up (p<0.001). Leflunomide is a viable alternative agent for pulmonary and extrapulmonary sarcoidosis. Leflunomide appears to facilitate reduction of steroid dose and can be considered as monotherapy or as add-on therapy in cases of progressive disease.

Haemosiderin-laden macrophages in the bronchoalveolar lavage fluid of patients with diffuse alveolar damage.

Quantification of haemosiderin-laden macrophages in bronchoalveolar lavage fluid (BALF) has been used to diagnose diffuse alveolar haemorrhage (DAH) but has not been assessed in patients with diffuse alveolar damage (DAD). The present study analysed BALF obtained from 21 patients with DAD diagnosed by surgical lung biopsy. The median age of 21 patients with DAD was 68 yrs (range 18-79 yrs); 14 (67%) were male and 12 (57%) were immunocompromised. The median proportion of haemosiderin-laden macrophages in BALF was 5% (range 0-90%), but was >or=20% in seven (33%) patients, fulfilling the commonly used BALF criterion for DAH. There was a trend toward a positive correlation between the percentage of haemosiderin-laden macrophages in BALF and parenchymal haemorrhage assessed semiquantitatively by histopathological analysis. Patients with >or=20% haemosiderin-laden macrophages in BALF showed a significantly increased mortality rate (p = 0.047) compared to those with <20%. In patients with an acute onset of diffuse lung infiltrates and respiratory distress, >or=20% haemosiderin-laden macrophages in BALF can occur with DAD, and is not necessarily diagnostic of DAH. The finding of >or=20% haemosiderin-laden macrophages in BALF is associated with a worse prognosis in patients with DAD.

Platelet larceny: spurious hypoxaemia due to extreme thrombocytosis.

Rapid oxygen consumption by markedly increased numbers of hypermetabolic leukocytes in leukaemic patients resulting in the apparent diagnosis of hypoxaemia on arterial blood gas analyses is termed leukocyte larceny. In the present report, a case of polycythaemia vera, extreme thrombocytosis, normal leukocyte counts and arterial hypoxaemia in the absence of clinical, radiological or physiological evidence of lung disease is described. This pseudohypoxaemia case was established by pulse oximetry, as well as by incubation of a blood specimen with potassium cyanide, and became less significant after the use of cytoreductive agents showed a proportionate increase in arterial oxygen tension as platelet counts decreased on serial arterial blood gas analyses. The present case report demonstrates spurious hypoxaemia due to extreme thrombocytosis and shows that, beside significant leukocytosis, even markedly elevated platelet counts can cause larceny of arterial blood oxygen.