Pulmonary hemorrhage in patients receiving anti-platelets- a case series and a suggested therapeutic algorithm.

Antiplatelet therapy including aspirin and thienopyridine agents (such as clopidogrel, prasugrel and ticagrelor) are often used in patients with coronary disease. Pulmonary hemorrhage due to antiplatelet therapy although very rare, when excessive, is a life-threatening event. So far, there is lack of specific guidelines for the management of these patients. We report a case series of 5 patients receiving antiplatelet therapy who were admitted to the hospital due to pulmonary hemorrhage related to antiplatelet therapy. We also propose an algorithm on the management of these patients taking into consideration the balance between thrombotic and bleeding risk and the severity of the hemorrhage.

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

The possible causal relationship between interferon-ß exposure and pulmonary arterial hypertension development requires close follow-up of patients on treatment with interferon-ß http://bit.ly/2OPGSVP.

Epidemiology and initial management of pulmonary arterial hypertension: real-world data from the Hellenic pulmOnary hyPertension rEgistry (HOPE).

Pulmonary arterial hypertension (PAH) is a heterogenous clinical entity with poor prognosis, despite recent major pharmacological advances. To increase awareness about the pathophysiology, epidemiology, and management of the disease, large national registries are required. The Hellenic pulmOnary hyPertension rEgistry (HOPE) was launched in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. Baseline epidemiologic, diagnostic, and initial treatment data of consecutive patients with PAH are presented in this article. In total, 231 patients with PAH were enrolled from January 2015 until April 2018. At baseline, about half of patients with PAH were in World Health Organization functional class II. The majority of patients with PAH (56.7%) were at intermediate 1-year mortality risk, while more than one-third were low-risk patients, according to an abbreviated risk stratification score. Half of patients with PAH were on monotherapy, 38.9% received combination therapy, while prostanoids were used only in 12.1% of patients. In conclusion, baseline data of the Greek PAH population share common characteristics, but also have some differences with other registries, the most prominent being a better functional capacity. This may reflect earlier diagnosis of PAH that in conjunction with the increased proportion of patients with atypical PAH could partially explain the preference for monotherapy and the limited use of prostanoids in Greece. Nevertheless, early, advanced specific therapy is strongly recommended.

Mycophenolate mofetil as an alternative treatment in sarcoidosis.

INTRODUCTION: In sarcoidosis although no better drug therapy than corticosteroids (CS) has emerged, alternative immunosuppressive agents are used when indicated. Mycophenolate mofetil (MMF) presents rapid action, a considerable safety profile and absence of lung toxicity. Few data exist so far on its use in patients with sarcoidosis. This is a retrospective study on the effectiveness and safety of MMF in patients with sarcoidosis. MATERIALS AND METHODS: All patients with biopsy proven sarcoidosis treated for at least 1 year with MMF from 2008 to 2017 in our department are evaluated. RESULTS: Eight patients with both pulmonary and extrapulmonary disease are included in the analysis. During follow-up, symptoms and chest radiological findings improved in all. A statistically significant improvement of FEV1 and FVC is reported (p = 0.010 and p = 0.021 respectively). Cardiac and renal disease resolved during treatment while dermal disease significantly improved. MMF permitted CS dose reduction from 15.0 (10.0, 35.0) to 2.5 (0.0, 5.0) mg prednisolone (or equivalent), p = 0.016. All patients but one, tolerated well MMF. CONCLUSION: MMF as an alternative drug in systemic sarcoidosis, proved safe and effective, permitting the reduction of the dose of oral CS and leading to clinical, functional and radiological improvement.

Serum Admission 25-Hydroxyvitamin D Levels and Outcomes in Initially Non-Septic Critically Ill Patients.

INTRODUCTION: To examine whether very low levels of 25-hydroxyvitamin D {25(OH)D} upon admission to the intensive care unit (ICU) are associated with worse outcomes. METHODS: Retrospective observational cohort study of critically ill patients treated in a multidisciplinary ICU. Two hundred twenty seven initially non-septic, critically ill patients, in whom 25-hydroxyvitamin D was measured at ICU admission. An additional group of 192 healthy subjects was also used. Patients were categorized according to their vitamin D levels at admission; the two patient groups were those with severely low 25-hydroxyvitamin D levels (<7 ng/mL, N = 101) and those with vitamin D levels ≥7 ng/mL, N = 126. RESULTS: ICU admission 25-hydroxyvitamin D levels of critically ill patients were much lower than those of healthy subjects (P < 0.0001). The median time to sepsis for the two patient groups did not differ, nor did the length of ICU stay (days). Both groups exhibited similar hospital mortality rates. However, among the fraction of patients who eventually became septic (N = 145), the odds ratio (OR) for developing respiratory infections in patients with admission vitamin levels < 7 ng/mL compared with patients with admission vitamin D levels ≥7 ng/mL was 5.25 {95% confidence interval (CI) 1.5-18.32, P = 0.009}. CONCLUSIONS: Initially non-septic critically ill patients appear to have very low ICU admission 25-hydroxyvitamin D levels. Among critically ill patients, severely low vitamin D levels (<7 ng/mL) at ICU admission do not predict sepsis development, increased risk of in-hospital mortality, or longer stay in the ICU. However, these severely low admission vitamin D levels in patients who will eventually develop sepsis are associated with development of respiratory tract infections.

How common is sleep-disordered breathing in patients with idiopathic pulmonary fibrosis?

BACKGROUND AND AIM: The frequency of obstructive sleep apnea-hypopnea syndrome (OSAHS) in patients with idiopathic pulmonary fibrosis (IPF) remains controversial. The aim of this study was to assess the frequency of OSAHS in newly diagnosed IPF patients and to identify possible correlations with body mass index and pulmonary function testing parameters. MATERIALS AND METHODS: Thirty-four newly diagnosed IPF patients were included. All subjects underwent attended overnight PSG. None of the included subjects was under any of the currently available IPF treatments or nocturnal supplemental oxygen therapy. RESULTS: Total apnea-hypopnea index (AHI) was <5, 5-15, and ≥ 15/h of sleep in 14 (41%), 15 (44%), and five patients (15%), respectively. REM AHI was statistically significant correlated with TLC [Total lung capacity] (p=0.03, r= -0.38). Diffusing capacity of the lung for carbon monoxide was correlated with mean oxygen saturation during sleep (p=0.02, r=0.39). CONCLUSIONS: Sleep-disordered breathing seems frequent, although remains usually under diagnosed in IPF patients. A decrease in TLC, reflecting the severity of pulmonary restriction, might predispose IPF patients in SDB, especially during the vulnerable REM sleep period.

The treatment of lupus pernio: results of 116 treatment courses in 54 patients.

BACKGROUND: Lupus pernio is a disfiguring sarcoidosis skin lesion that is difficult to treat and often causes a major psychosocial impact that may adversely affect the patient's quality of life. We reviewed the treatment outcome of 54 patients with lupus pernio who received 116 individual courses of treatment in our sarcoidosis clinic. METHODS: Lupus pernio patients were identified from an institution-approved database. All patients were assessed at each clinic visit with facial photographs. By examining the photographs, the percentage of face involved (< 10%, 10 to 25%, > 25 to 50%, > 50%) was determined as was the effect of therapy (resolution, near resolution, improvement, no change, worsening). Medications included infliximab-containing regimens; systemic corticosteroids; noninfliximab, noncorticosteroid agents; and corticosteroids plus noncorticosteroid agents. RESULTS: In terms of achieving resolution or near resolution, infliximab regimens were superior to all others (infliximab, 77%; corticosteroids plus noncorticosteroids, 29%; corticosteroids, 20%; noncorticosteroids, 11%; infliximab vs other therapies: corticosteroids plus noncorticosteroids, p = 0.0015; corticosteroids, p = 0.0005; noncorticosteroids, p = 0.0002). The percentage of facial involvement also improved most with infliximab. Evaluating a secondary analysis of achieving resolution, near resolution, or improvement, infliximab (92%) was superior to noncorticosteroids (20%; p < 0.0001) and corticosteroids plus noncorticosteroids (56%; p = 0.0098), but not corticosteroids (72%; p = 0.2456); and noncorticosteroid agents were inferior to all other regimens. CONCLUSIONS: Infliximab appears superior to systemic corticosteroids with or without additional agents for the treatment of lupus pernio. Noninfliximab, noncorticosteroid-containing regimens are of limited use for this condition.