Phenotyping Exercise Limitation in Systemic Sclerosis: The Use of Cardiopulmonary Exercise Testing.

BACKGROUND: Exercise impairment is a common symptom of systemic sclerosis (SSc), a disorder which is frequently complicated by cardiopulmonary involvement. OBJECTIVES: This study's aims were: (a) to define the prevalence and the potential causes of limited exercise capacity and (b) to study potential differences in clinical, radiological and functional characteristics and blood serology among SSc patients with exercise limitation of different etiology. METHODS: Prospectively collected data on SSc patients who had conducted full lung function testing, blood serology, thorax high-resolution computed tomography, Doppler echocardiogram and a maximal cardiopulmonary exercise testing (CPET) were retrospectively analyzed. Using a CPET algorithm, patients were characterized as having normal or subnormal exercise capacity (N), respiratory limitation (RL), left ventricular dysfunction (LVD) or pulmonary vasculopathy (PV). Group comparisons were conducted using either one-way ANOVA or the Kruskal-Wallis test. A p value <0.05 was considered significant. RESULTS: The study population consisted of 78 patients (53.7 ± 13.7 years old; 10.3% male). PV was present in 32.1%, LVD in 25.6% and RL in 10.2%, while 32.1% of the patients constituted the N group. The presence of antisclero-70 antibodies, low anaerobic threshold and low peak exercise capacity measures could discriminate LVD from the other groups. Low end-tidal carbon dioxide pressure and its change from rest to anaerobic threshold could discriminate between the PV, LVD and N groups, while respiratory restriction along with ventilatory inefficiency indices could differentiate the RL group from the rest. CONCLUSIONS: The combined evaluation of CPET gas exchange patterns with baseline measurements could discriminate the causes of exercise limitation among SSc patients.

Lemierre's syndrome presenting to the ED: rapidly fatal sepsis caused by methicillin-susceptible Staphylococcus aureus Staphylococcus protein A type t044.

We describe the case of a fatal septic illness in a previously healthy young man caused by community-acquired methicillin-susceptible Staphylococcus aureus of Staphylococcus protein A (spa) type t044. The patient developed a devastating Lemierre-like syndrome with extensive thrombosis of inferior vena cava and iliac veins with multiple metastatic septic emboli of the lungs. He presented to the emergency department with rapidly progressing sepsis followed by multiple organ dysfunction syndrome. Recognition of such virulent community-acquired strains is of great importance because they could prove to be emerging pathogens for life-threatening diseases.

Repeated episodes of respiratory failure due to bilateral vocal cord paralysis.

BACKGROUND: Bilateral vocal cord paralysis can produce severe airway obstruction, leading to acute respiratory failure. Discriminating the pathology of the upper airway from chronic obstructive diseases of the lower airways often presents a challenge for clinicians in the Emergency Department. OBJECTIVES: To underlie the value of clinical examination and flow-volume loops in the establishment of diagnosis of upper airway obstruction. CASE REPORT: We describe the case of a 55-year-old female ex-smoker who presented with a long history of hoarseness and progressive exertional dyspnea. The patient developed repeated episodes of acute respiratory failure and was supported with noninvasive ventilation. The diagnosis of bilateral vocal cord paralysis was finally established by patient's symptoms and flow-volume loops demonstrating variable extrathoracic obstruction. CONCLUSION: Vocal cord paralysis is a rare and often neglected condition, contributing to repeated episodes of acute respiratory failure. Flow-volume loop is a useful tool when symptoms are suggestive of upper airway obstruction.

SARS-Cov-2 Infection in Severe Asthma Patients Treated With Biologics.

BACKGROUND: At the beginning of the pandemic, there have been considerable concerns regarding coronavirus disease 2019 (COVID-19) severity and outcomes in patients with severe asthma treated with biologics. OBJECTIVE: To prospectively observe a cohort of severe asthmatics treated with biologics for the risk of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and disease severity during the COVID-19 pandemic. METHODS: Physicians from centers treating patients with severe asthma all over Greece provided demographic and medical data regarding their patients treated with biologics. Physicians were also asked to follow up patients during the pandemic and to perform a polymerase chain reaction test in case of a suspected SARS-Cov-2 infection. RESULTS: Among the 591 severe asthmatics (63.5% female) included in the study, 219 (37.1%) were treated with omalizumab, 358 (60.6%) with mepolizumab, and 14 (2.4%) with benralizumab. In total, 26 patients (4.4%) had a confirmed SARS-CoV-2 infection, 9 (34.6%) of whom were admitted to the hospital because of severe COVID-19, and 1 required mechanical ventilation and died 19 days after admission. Of the 26 infected patients, 5 (19.2%) experienced asthma control deterioration, characterized as exacerbation that required treatment with systemic corticosteroids. The scheduled administration of the biological therapy was performed timely in all patients with the exception of 2, in whom it was postponed for 1 week according to their doctors' suggestion. CONCLUSION: Our study confirms that despite the initial concerns, SARS-CoV-2 infection is not more common in asthmatics treated with biologics compared with the general population, whereas the use of biologic treatments for severe asthma during the COVID-19 pandemic does not seem to be related to adverse outcomes from severe COVID-19.

Acute respiratory failure as primary manifestation of antineutrophil cytoplasmic antibodies-associated vasculitis.

The systemic vasculitides are multifocal diseases characterized by the presence of blood vessel inflammation in multiple organ systems. Their clinical presentation is variable extending from self-limited illness to critical complications including diffuse alveolar hemorrhage and glomerulonephritis. Alveolar hemorrhage is a life-threatening manifestation of pulmonary vasculitis that can rapidly progress into acute respiratory failure requiring ventilatory support. We present the case of a 74-year-old patient admitted to the Intensive Care Unit with severe hypoxic respiratory failure and diffuse alveolar infiltrates in chest imaging that was later diagnosed as antineutrophil cytoplasmic antibodies-associated vasculitis. The report highlights the importance of differentiate between alveolar hemorrhage and acute respiratory distress syndrome of other etiology because alveolar hemorrhage is reversible with prompt initiation of treatment.

Chronic thromboembolic pulmonary hypertension: do not miss the chance for an early diagnosis.

BACKGROUND: Chronic thromboembolic pulmonary hypertension most often results from obstruction of the pulmonary vascular bed by nonresolving thromboemboli. Misdiagnosis of the disease is common because patients often present with subtle or nonspecific symptoms. Furthermore, some features in chest imaging may mimic parenchymal lung disease. The most clinically important mimic in high-resolution chest tomography is air trapping, which can be seen in a variety of small airway diseases. CASE REPORT: We present the case of a 45-year-old woman with a long history of dyspnea and exercise intolerance, misdiagnosed with allergic alveolitis. The diagnosis of CTEPH was finally established with computed tomography (CT) angiography and hemodynamics. CONCLUSIONS: Chronic thromboembolism is under-diagnosed and also frequently misdiagnosed in clinical practice. The present report aims to increase the awareness of clinicians towards an accurate diagnosis of the disease, which is necessary for the early referral of CTEPH patients for operability.

A case of pulmonary veno-occlusive disease: diagnostic dilemmas and therapeutic challenges.

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH). Misdiagnosis of the disease is common since PVOD presents with clinical and radiographic features mimicking idiopathic pulmonary arterial hypertension or even PH due to interstitial lung disease. Vasodilators may not be efficacious in PVOD and may in fact worsen hemodynamic status with the development of pulmonary edema. Lung transplantation represents the best treatment option. In the present report we describe the challenging diagnosis of PVOD in a patient with PH referred to our department. Final diagnosis was established by surgical lung biopsy. The patient was offered sequential combination therapy under close monitoring and maintained remarkable clinical stabilization while being on the waiting list for lung transplantation.