Severe pulmonary arterial hypertension and massive ascites in a patient with systemic lupus erythematosus and secondary Sjogren's syndrome.

BACKGROUND: Pulmonary arterial hypertension (PAH), is a rare manifestation of systemic lupus erythematosus (SLE), characterized by pulmonary arterial remodeling leading to right ventricular failure and death. To date, optimal management of SLE-associated PAH should be clarified, especially regarding the respective places of immunosuppressants and PAH vasodilator treatments. CASE REPORT: We report the case of a 48-year-old woman with SLE and secondary Sjogren syndrome, associated with severe PAH and lupus peritonitis with massive ascites, who showed a remarkable response, both for SLE flare and PAH, to a treatment combining immunosuppressants and pulmonary arterial vasodilator treatment. CONCLUSION: This observation highlights the interest of combining immunosuppressive therapy in SLE-PAH, whose modalities in association with PAH treatments should be clarified.

Clinical characteristics of and outcomes for patients with COVID-19 and comorbid lung diseases primarily hospitalized in a conventional pulmonology unit: A retrospective study.

BACKGROUND: Scant data are currently available about a potential link between comorbid chronic lung diseases (CLD) and the risk and severity of the coronavirus disease 2019 (COVID-19) infection. METHODS: To describe the clinical characteristics of and outcomes for patients with COVID-19 infection, including patients with comorbid respiratory diseases, who have been primarily hospitalized in the pulmonology department of Strasbourg University Hospital, France. In this retrospective, single-center study, we included all confirmed cases of COVID-19 from March 3 to April 15, 2020. We then compared the symptoms, biological and radiological findings, and outcomes for patients with and without CLD. RESULTS: Of the 124 patients that were enrolled, the median age was 62 years, and 75 patients (60%) were male. Overall, 40% of patients (n=50) had preexisting CLD, including chronic obstructive pulmonary disease (COPD) (n=15, 12%) and asthma (n=19, 15%). Twenty-eight patients were transferred to the intensive care unit (ICU), and six patients died in our unit. CLD were not predictive of ICU hospitalization, but a significantly higher total mortality was observed (17.6% vs. 5.5%, P<0.05) in these patients. CONCLUSIONS: Our results suggest the lack of an over-representation of CLD in COVID-19, representing 40% of patients in this cohort and even within a pulmonology department. CLD were not a risk factor for ICU management. However, a tendency to higher global mortality was observed in COVID-19 patients with CLD. Further studies are warranted to determine the risk of COVID-19 for patients with comorbid CLD.

[Variation in ventilatory response to CO2 in patients treated by non-invasive ventilation]. / Variation de la réponse ventilatoire au CO2 chez des patients appareillés par ventilation non invasive.

INTRODUCTION: There are many mechanisms for improving the clinical and blood gas status of patients with the obesity hypoventilation syndrome (OHS) or chronic obstructive pulmonary disease (COPD) by non-invasive ventilation (NIV) at home. Our objective was to set up a pilot study to evaluate the potential modification of the sensitivity of the respiratory centers to CO2 by NIV in paired new COPD and OHS patients. METHODS: We assessed the sensitivity of the respiratory centers to CO2 by the Read method in 3 COPD patients and 3 OHS patients newly treated by NIV and again 3 months later. We compared their results to those of 6 control subjects. RESULTS: All the patients included had altered ventilatory responses to CO2 with slopes of less of than 1 L.min-1.mmHg-1. Mean coefficients of variation were significantly higher in patients than in healthy subjects (P=0.007). Patients who improved their CO2 sensitivity slope were those most observant of NIV. CONCLUSION: This work showed significant changes in the ventilatory response to hypercapnia in patients with either OHS or COPD after NIV therapy. The significance of these changes deserves to be studied.

Diagnostic Pitfall of Platypnea-Orthodeoxia Syndrome Caused by Atrial Septal Defect after Right Pneumonectomy.

We describe a case of platypnea-orthodeoxia syndrome (POS) due to atrial septal defect (ASD) occurring in the early postoperative course of a right pneumonectomy. Deformation of the atrial septum after right pneumonectomy deviates the blood from the inferior vena cava to ASD during the sitting position creating, a massive right-to-left shunt. Diagnosis can initially be missed by making contrast bubble test through the superior vena cava. The atrial septal defect was then closed using the surgical technique, allowing an instantaneous improvement of hematosis.

[Liver diseases and pulmonary vascular disorders]. / Hépatopathies et maladies vasculaires pulmonaires.

About 70% patients waiting for liver transplantation have a dyspnea. Two pulmonary vascular disorders can be associated with portal hypertension or chronic liver diseases: portopulmonary hypertension (PoPH) related to pulmonary small arteries remodeling and obstruction and hepatopulmonary syndrome (HPS) characterized by pulmonary capillaries dilatations and proliferations. PoPH is defined by the combination of pulmonary arterial hypertension (PAH) (mean pulmonary artery pressure [PAP]≥25mmHg, with normal pulmonary artery wedge pressure≤15mmHg and pulmonary vascular resistance [PVR]>3 Wood units [WU]) and portal hypertension. HPS is a triad of intrapulmonary vascular dilatations, hypoxemia (increased alveolar-arterial oxygen gradient) and liver disease or isolated portal hypertension. The pathophysiology of both syndromes is complex and poorly understood. PoPH and HPS have a negative impact on functional and vital prognosis in patients with portal hypertension. Liver transplantation is the established treatment standard in HPS. PoPH treatment is improved over the years with the use of specific PAH treatment despite the lack of randomized assay in this indication. Liver transplantation could be considered in PoPH leading to stabilization, improvement or recovery in selected patients (mean PAP<35mmHg without severe right ventricular dysfunction and PVR<4 WU).

[SaO2 and PaO2 mismatch: Do not forget hemoglobinopathy]. / Discordance entre SaO2 ­ PaO2 : ne pas oublier les hémoglobinopathies.

INTRODUCTION: Different clinico-biological parameters are used to estimate the amount of oxygen available for the organism. Oxygen saturation measured with pulse oxymetry (SpO2), oxygen saturation of arterial blood (SaO2) and oxygen partial pressure of the arterial blood (PaO2) are the most commonly used. CASE REPORT: We report the case of a patient admitted for investigation of respiratory failure in the context of chronic dyspnea of effort. SpO2 and SaO2 were decreased, though the PaO2 was normal. This mismatch between oxygen saturation and PaO2 led to the diagnosis of hemoglobinopathy (Bassett hemoglobin). CONCLUSION: The diagnosis of hemoglobinopathy should be considered in cases of oxygen desaturation with normal respiratory and cardiac investigations. There are no reasons to prescribe long-term oxygen to these patients.

[Pleural effusion in hereditary haemorrhagic telangiectasia: hemothorax or empyema?]. / Pleurésie dans le cadre d'une maladie de Rendu-Osler: hémothorax ou pyothorax?

INTRODUCTION: In hereditary hemorrhagic telangiectasia the pulmonary arteriovenous malformations frequently lead to complications. In a case of pleural effusion in a patient with known pulmonary arterio-venous malformations, the first diagnosis to consider is a hemothorax even though alternatives such as empyema are possible. CASE REPORT: We report the case of a 35-year-old woman with a known diagnosis of hereditary hemorrhagic telangiectasia with bilateral arteriovenous malformations, who was admitted to the emergency department with subacute dyspnoea and left thoracic pain. The clinical examination suggested a hemothorax. Pulmonary angiography was performed and showed an arteriovenous malformation in the left lower lobe. Embolisation was undertaken and at the same time a chest tube was inserted. Pus was evacuated leading to the final diagnosis of pleural empyema. Progress was good despite an episode acute respiratory distress due to a pulmonary embolism. CONCLUSION: Pleural empyema is rarely described in the context of hereditary hemorrhagic telangiectasia with pulmonary arteriovenous malformations. In the case of pleural effusion this diagnosis should be considered even though hemothorax is more common.

[Muscular metastasis from primary lung cancer: about seven cases]. / Métastases musculaires de cancers bronchiques: à propos de sept cas.

Muscular metastasis from primary bronchial carcinoma are rare. We report seven cases with different clinical features. In most cases, these metastasis first manifested as a painful mass and revealed the cancer. Localisation of muscular metastasis was assessed by various imaging techniques (RMI, CT scan and ultrasonography). All cases were assessed by biopsy and were associated with primary adenocarcinoma or undifferentiated large cell carcinoma of the lung. Three patients underwent wide excision, leading to an improvement of general condition. All patients were treated with platinum-based chemotherapy. With reference to our cases, imaging of the metastases will be illustrated and clinical and therapeutic features will be discussed.

[Pulmonary hypertension due to tumor embolism]. / Hypertension artérielle pulmonaire par embolisation de cellules tumorales.

We report a 46-year-old woman with a metastatic breast cancer who developed a subacute pulmonary hypertension. The final diagnosis was microscopic pulmonary neoplastic emboli. She presented with the typical clinical picture of a pulmonary hypertension with a normal pulmonary angiographic computer tomography and multiple sub-segmental perfusion defects on radionuclide imaging. This serious condition must be known by physicians, because of an opportunity to obtain the diagnosis by the cytologic analysis of sampled microvascular blood obtained with a pulmonary artery catheterization. The prognosis is poor and there is no efficient therapy.

[The Overlap Syndrome: association of COPD and Obstructive Sleep Apnoea]. / L' "Overlap Syndrome": association de bronchopneumopathie chronique obstructive et de syndrome d'apnées obstructives du sommeil.

Chronic obstructive pulmonary disease (COPD) and the obstructive sleep apnoea-hypopnoea syndrome (OSAHS) are both common diseases affecting respectively 10 and 5% of the adult population over 40 years of age. Their coexistence, which is denominated "Overlap Syndrome", can be expected to occur in about 0.5% of this population. Two recent epidemiologic studies have shown that the prevalence of OSAHS is not higher in COPD than in the general population, and that the coexistence of the two conditions is due to chance and not through a pathophysiological linkage. Patients with "overlap" have a higher risk of sleep-related O(2) desaturation than do patients with COPD alone and the same degree of bronchial obstruction. They have an increased risk of developing hypercapnic respiratory failure and pulmonary hypertension when compared with patients with OSAHS alone and with patients with "usual" COPD. In patients with overlap, hypoxaemia, hypercapnia, and pulmonary hypertension can be observed in the presence of mild to moderate bronchial obstruction, which is different from "usual" COPD. Treatment of the overlap syndrome consists of nasal continuous positive airway pressure or nocturnal non-invasive ventilation (NIV), with or without nocturnal O(2). Patients who are markedly hypoxaemic during the daytime (PaO(2)<55-60 mmHg) should be given conventional long-term O(2) therapy in addition to nocturnal ventilation.

[Obesity-hypoventilation syndrome]. / Syndrome obésité-hypoventilation.

INTRODUCTION: The obesity-hypoventilation syndrome (OHS), or alveolar hypoventilation in the obese, has been described initially as the "Pickwickian syndrome". It is defined as chronic alveolar hypoventilation (PaO2<70 mmHg, PaCO2 > or =45 mmHg) in obese patients (body mass index>30 kg/m2) who have no other respiratory disease explaining the hypoxemia-hypercapnia. BACKGROUND: The large majority of obese subjects are not hypercapnic, even in case of severe obesity (>40 kg/m2). There are three principal causes, which can be associated, explaining alveolar hypoventilation in obese subjects: high cost of respiration and weakness of the respiratory muscles (probably the major cause), dysfunction of the respiratory centers with diminished chemosensitivity, long-term effects of the repeated episodes of obstructive sleep apneas observed in some patients. The role of leptin (hormone produced by adipocytes) in the pathogenesis of this syndrome, has been recently advocated. OHS is generally observed in subjects over 50 years. Its prevalence has markedly increased in recent years, probably due to the present "epidemic" of obesity. The diagnosis is often made after an episode of severe respiratory failure. Comorbidities, favored by obesity, are very frequent: systemic hypertension, left heart diseases, diabetes. VIEWPOINT: OHS must be distinguished from obstructive sleep apnea syndrome (OSAS) even if the two conditions are often associated. OSAS may be absent in certain patients with OHS (20% of the patients in our experience). On the other hand obesity may be absent in certain patients with OSAS. CONCLUSION: Losing weight is the "ideal" treatment of OHS but in fact it cannot be obtained in most patients. Nocturnal ventilation (continuous positive airway pressure and mainly bilevel non invasive ventilation) is presently the best treatment of OHS and excellent short and long-term results on symptoms and arterial blood gases have been recently reported.

[Pulmonary hypertension of chronic respiratory disease]. / Hypertension pulmonaire des affections respiratoires chroniques.

Pulmonary hypertension of chronic respiratory disease is defined as elevation of the mean resting pulmonary artery pressure to over 20 mm Hg. It is the commonest cause of pre-capillary pulmonary hypertension on account of the high prevalence of chronic obstructive pulmonary disease. It is primarily due to alveolar hypoxia that leads to remodelling of the distal pulmonary vasculature and consequently to an increase in pulmonary resistance. It is not as severe as idiopathic pulmonary hypertension. The mean pulmonary artery pressure is usually moderately increased (20-35 mm Hg) when patients are seen during a stable period of the disease. There are, however, more severe cases of pulmonary hypertension called, in some cases, disproportionate but these are uncommon. Increased right ventricular afterload can lead to the development of right ventricular failure that is a definite prognostic factor. The clinical symptoms of pulmonary hypertension are at a secondary level compared with those of the causal disease. Non-invasive diagnosis depends on Doppler echocardiography. Currently the most effective treatment is long term oxygen therapy (16-18 hr/24 hr). This improves or, at least stabilises, the pulmonary hypertension.

[Pulmonary hypertension associated with disorders of the respiratory system]. / Hypertension pulmonaire des affections respiratoires chroniques.

Pulmonary hypertension associated with disorders of the respiratory system is defined by a pulmonary artery mean pressure above 20 mmHg at rest in stable disease. The most frequent form of precapillary pulmonary hypertension is associated with chronic obstructive pulmonary disease, because of its high prevalence. Pulmonary vascular remodeling occurs in the small pulmonary arteries and is due mainly to chronic alveolar hypoxia. Pulmonary hypertension associated with disorders of the respiratory system is usually mild to moderate, with resting pulmonary artery mean pressure ranging between 20 and 35 mm Hg. It may increase markedly during sleep, exercise or exacerbation of respiratory failure, however. Abrupt postload elevation can lead to right heart failure, an indisputable indicator of prognosis. Because the symptoms of pulmonary hypertension are minimal relative to those of the chronic hypoxic lung disease, noninvasive diagnosis is difficult, particularly in patients with chronic obstructive pulmonary disease. Oxygen therapy (at least 16 h/day) is currently the best treatment for this type of pulmonary arterial hypertension.

[Physiological functional tests to evaluate pulmonary arterial hypertension]. / Explorations fonctionnelles dans l'hypertension artérielle pulmonaire.

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a rare and complex disease, which requires careful diagnostic evaluation. STATE OF THE ART: Most patients have a mild decrease in lung volumes and a moderate decrease in carbon monoxide transfer factor. Mild to moderate arterial hypoxaemia, is often present, associated with a mild respiratory alkalosis. When hypoxaemia is severe, it may represent an intracardiac shunt. Right heart catheterisation is required to confirm the diagnosis and in most cases shows a significant elevation of pulmonary artery pressure due to an increase in pulmonary vascular resistance. The haemodynamic profile and the response to an acute pulmonary vasodilator challenge are determinants of prognosis. Finally, exercise capacity which is usually assessed by the six minute walk test provides an overall functional measure of disease severity, response to therapy, and progression. PERSPECTIVES: Functional evaluation of the heart using echocardiography will play an increasing role in the evaluation of PAH. CONCLUSIONS: Physiological measurements in PAH have several objectives. They form part of the diagnostic definition, allow the exclusion of other conditions and are tools for the assessment of severity, prognosis and response to therapy.