Evdokia Sourla

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 studys 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.

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.

A patient who made an impact on how I practice: Severe leptospirosis presenting as ARDS in the ICU.

Severe leptospirosis can be a rare cause of acute respiratory distress syndrome (ARDS) and multiorgan failure. A patient who made an impact on how I practice was a case of severe leptospirosis (Weils disease) that presented as ARDS in the ICU. Leptospirosis is an under-reported infectious disease worldwide and should be considered as a cause of ARDS especially in patients with exposure history complicated with diffuse alveolar bleeding (DAH), icteric hepatitis and renal deterioration. Empiric treatment should be recommended before confirmation of laboratory tests as serological diagnosis is time consuming.

Rheumatoid arthritis associated pulmonary hypertension: Clinical challenges reflecting the diversity of pathophysiology

The present article reports three clinical cases in order to elucidate the diversity of the pathophysiological mechanisms that underlie rheumatoid arthritis associated pulmonary hypertension. The conditions three major causes are: interstitial lung disease, vasculitis, and chronic thromboembolic disease, but it should be noted that the multiple pulmonary manifestations of rheumatoid arthritis, can all contribute to chronic lung disease or hypoxia. The first patient in this report suffered from moderate restriction due to fibrosis and was diagnosed with pulmonary hypertension during an episode of life threatening hypoxia. Early upfront combination therapy prevented intubation and reversed hypoxia to adequate levels. The second presented patient was a case of isolated pulmonary hypertension attributable to vasculopathy. The patient maintained normal lung volumes but low diffusion capacity and echocardiography dictated the need for right heart catheterization. Finally, the third patient presented severe functional limitation due to several manifestations of rheumatoid arthritis, but a past episode of acute pulmonary embolism was also reported although it had never been evaluated. Chronic thromboembolic disease was eventually proved to be one major cause of the patients pulmonary hypertension. The importance of early identification of pulmonary hypertension in patients with rheumatoid arthritis is therefore emphasized, especially since multiple treatment options are available, symptoms can be treated, and right heart failure can be avoided.

Chronic Thromboembolic Pulmonary Hypertension: Do not Miss the Chance for an Early Diagnosis

Patient: Female, 45 Final Diagnosis: Chronic thromboembolic pulmonary hypertension (CTEPH) Symptoms: Dyspnea • fatigue • palpitations Medication: — Clinical Procedure: Right heart catheterization • pulmonary endarterectomy Specialty: Pulmonology Objective: Mistake in 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.

Is it just an infection

A 75-year-old man (body mass index 24.49 kg·m−2) presents to the emergency department with fever, myalgia and dyspnoea, which he has had for the last 4 days. He quit smoking 15 years ago (40 pack-years). His only medical history is hypertension treated with valsartan and amlodipine. He travelled recently and stayed in a motel. On admission, his temperature is 38.7°C, blood pressure 132/67 mmHg, heart rate 102 beats per min and respiratory rate 24 breaths per min. Arterial blood gases (ABG) analysis shows severe hypoxaemia with arterial oxygen tension (PaO2) 47.1 mmHg (6.3 kPa), arterial carbon dioxide tension (PaCO2) 40.9 mmHg (5.5 kPa), pH 7.464, HCO3− 28.4 mmol·L−1 and arterial oxygen saturation (SaO2) 84.2% while breathing room air. Laboratory tests show anaemia (haemoglobin 10.7 g·dL−1), elevated white blood cells (11 420 per μL; 92.0% neutrophils and 4.6% lymphocytes), elevated C-reactive protein (19.97 mg·dL−1) and hyponatraemia with serum Na+ 127 mmol·L−1. The rest of laboratory tests are normal. Chest radiograph showa infiltrates in both lungs. Antibiotic therapy with intravenous ampicillin/sulbactam 3 g per 6 h and antiviral therapy with oral oseltamivir 75 mg per 12 h are initiated. Is it just an infection or something more? http://ow.ly/HEcH30lSO6a

The clinical presentation of obstructive sleep apnea syndrome is different in diabetic patients

Background: Obstructive sleep apnea (OSA) is a highly prevalent disease with different clinical presentations. Diabetes and OSA may coexist. Objectives: To evaluate the possible different clinical presentations of OSA in diabetic patients. Methods: Retrospective analysis of the data of patients visiting a Sleep Clinic. Antropometric data, Epworth Sleepiness Scale (ESS), STOP BANG(SB), Athens Insomnia Scale (AIS) were completed and sleep studies were performed. Results: From 1248 included, 27(2%) had type 1 diabetes and 176(14%) type 2 with no gender difference. Diabetic patients were older (61±10 vs 52.2±14 p 2 =0.002). Significant differences were found in ESS (10.25 ± 5 vs 9.4 ±4.6, P=0.02), SB (5.5 ±1.5 vs 4.4 ±1.6, p Conclusions: Diabetic OSA patients were older, more obese, and sleepier,with more insomnia symptoms and arousals than non diabetic.

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.

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 patients 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.