Right-to-left shunt with hypoxemia in pulmonary hypertension

<p>Abstract</p> <p>Background</p> <p>Hypoxemia is common in pulmonary hypertension (PH) and may be partly related to ventilation/perfusion mismatch, low diffusion capacity, low cardiac output, and/or right-to-left (RL) shunting.</p> <p>Methods</p> <p>To determine whether true RL shunting causing hypoxemia is caused by intracardiac shunting, as classically considered, a retrospective single center study was conducted in consecutive patients with precapillary PH, with hypoxemia at rest (PaO₂< 10 kPa), shunt fraction (Qs/Qt) greater than 5%, elevated alveolar-arterial difference of PO₂(AaPO₂), and with transthoracic contrast echocardiography performed within 3 months.</p> <p>Results</p> <p>Among 263 patients with precapillary PH, 34 patients were included: pulmonary arterial hypertension, 21%; PH associated with lung disease, 47% (chronic obstructive pulmonary disease, 23%; interstitial lung disease, 9%; other, 15%); chronic thromboembolic PH, 26%; miscellaneous causes, 6%. Mean pulmonary artery pressure, cardiac index, and pulmonary vascular resistance were 45.8 ± 10.8 mmHg, 2.2 ± 0.6 L/min/m², and 469 ± 275 dyn.s.cm^-5, respectively. PaO₂in room air was 6.8 ± 1.3 kPa. Qs/Qt was 10.2 ± 4.2%. AaPO₂under 100% oxygen was 32.5 ± 12.4 kPa. Positive contrast was present at transthoracic contrast echocardiography in 6/34 (18%) of patients, including only 4/34 (12%) with intracardiac RL shunting. Qs/Qt did not correlate with hemodynamic parameters. Patients' characteristics did not differ according to the result of contrast echocardiography.</p> <p>Conclusion</p> <p>When present in patients with precapillary PH, RL shunting is usually not related to reopening of patent <it>foramen ovale</it>, whatever the etiology of PH.</p

Effectiveness of cladribine therapy in patients with pulmonary Langerhans cell histiocytosis.

BackgroundPulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder characterised by granulomatous proliferation of CD1a-positive histiocytes forming granulomas within lung parenchyma, in strong association with tobacco smoking, and which may result in chronic respiratory failure. Smoking cessation is considered to be critical in management, but has variable effects on outcome. No drug therapy has been validated. Cladribine (chlorodeoxyadenosine, 2-CDA) down-regulates histiocyte proliferation and has been successful in curbing multi-system Langerhans cell histiocytosis and isolated PLCH.Methods and patientsWe retrospectively studied 5 patients (aged 37¿55 years, 3 females) with PLCH who received 3 to 4 courses of cladribine therapy as a single agent (0.1 mg/kg per day for 5 consecutive days at monthly intervals). One patient was treated twice because of relapse at 1 year. Progressive pulmonary disease with obstructive ventilatory pattern despite smoking cessation and/or corticosteroid therapy were indications for treatment. Patients were administered oral trimethoprim/sulfamethoxazole and valaciclovir to prevent opportunistic infections. They gave written consent to receive off-label cladribine in the absence of validated treatment.ResultsFunctional class dyspnea improved with cladribine therapy in 4 out of 5 cases, and forced expiratory volume in 1 second (FEV1) increased in all cases by a mean of 387 ml (100¿920 ml), contrasting with a steady decline prior to treatment. Chest high-resolution computed tomography (HRCT) features improved with cladribine therapy in 4 patients. Hemodynamic improvement was observed in 1 patient with pre-capillary pulmonary hypertension. The results suggested a greater treatment effect in subjects with nodular lung lesions and/or thick-walled cysts on chest HRCT, with diffuse hypermetabolism of lung lesions on positron emission tomography (PET)-scan, and with progressive disease despite smoking cessation. Infectious pneumonia developed in 1 patient, with later grade 4 neutrocytopenia but without infection.DiscussionData interpretation was limited by the retrospective, uncontrolled study design and small sample size.ConclusionCladribine as a single agent may be effective therapy in patients with progressive PLCH

Systemic scleroderma with interstitial pneumonia

International audienc

Rendu-Osler disease

International audienc

Isolated Recurrent Pleuritis Revealing Familial Mediterranean Fever in Adulthood

International audienc

Pulmonary hypertension therapy and COPD: still many questions to be answered.

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Diffuse bronchiectasis and airflow obstruction in granulomatosis with polyangiitis

International audienceParenchymal lung nodes and diffuse intra-alveolar hemorrhage are the archetypal pulmonary manifestations of Granulomatosis with Polyangiitis (GPA). The occurrence of diffuse bronchiectasis and airflow obstruction during GPA is unusual. We report here 3 patients with GPA who developed diffuse bronchiectasis during follow-up. The airflow obstruction seemed then to evolve independently from the GPA itself and ultimately led to respiratory insufficiency. Bronchiectases promoted the occurrence of opportunistic infections, especially with atypical mycobacteria

Anti-elastin autoantibodies are not present in combined pulmonary fibrosis and emphysema

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Persistent airflow obstruction in asthma of patients with Churg-Strauss syndrome and long-term follow-up

International audienceLittle is known about the long-term outcome of airflow obstruction in asthma of patients with Churg-Strauss syndrome (CSS). We conducted a retrospective study of 24 consecutive patients (aged 41.1 +/- 13.5 years) with CSS in a single center. All had asthma (starting 8.1 +/- 9.5 years prior to the diagnosis of CSS), blood eosinophilia (6.1 +/- 4.4 x 10(9)/l) and systemic manifestations of CSS. Antineutrophil cytoplasmic antibodies were found in 7 of 22 tested patients. Seven patients had smoked (a mean of 10 pack-years). All patients received oral corticosteroids, 11 cyclophosphamide and 23 inhaled corticosteroids. Airflow obstruction was found in 14 patients (70%) at diagnosis, and in 11 of 22 patients (50%) at the time of the clinical remission of CSS. The mean postbronchodilator FEV1/FVC and FEV1 were 69 +/- 12% and 74 +/- 21% of predicted at diagnosis (n = 20); 71 +/- 10% and 92 +/- 19% of predicted at the clinical remission (n = 22); and 64 +/- 13% and 80 +/- 21% at last visit (n = 13), respectively. During follow-up, postbronchodilator FEV1 increased by 30 +/- 28% in six patients with FEV1/FVC < 70% despite inhaled therapy who received higher dose of oral corticosteroids. At last visit, 5 of 13 patients (38%) with more than 3 years of follow-up had persistent airflow obstruction as defined by postbronchodilator FEV1/FVC < 70% and FEV1 < 80% of predicted. Airflow obstruction due to uncontrolled asthma is present despite corticosteroids in many patients at diagnosis and at clinical remission of CSS, and during follow-up. It may be still partly reversible with increased oral corticosteroid treatment

Incontinence urinaire à la toux au cours des pneumopathies interstitielles diffuses [Urinary incontinence due to chronic cough in interstitial lung disease]

INTRODUCTION: Urinary stress incontinence affects 10% to 30% of the female population and may have a major impact on psychosocial health. In interstitial lung disease, chronic cough may lead to development of urinary incontinence, but the prevalence and impact of this symptom are unknown. OBJECTIVES: To determine the rate and impact of urinary stress incontinence among women with chronic cough due to interstitial lung disease. METHODS: 28 female patients with chronic cough secondary to interstitial lung disease and 15 controls were evaluated by questionnaires to determine the prevalence of cough-related urinary incontinence, its severity, and its impact on quality of life. RESULTS: Cough-related urinary incontinence was present in 14/28 patients with interstitial lung disease and chronic cough (50%), but in only 1/15 controls (7%, p=0.005). On a 5-points quality of life scale, the median impact of urinary incontinence was 3 (minimum=1, maximal=5), and the median impact of chronic cough was 3.5. The majority of patients (64%) believed that incontinence was a natural phenomenon due to ageing, all were ashamed by this symptom and 79% were unable to mention it to their caring physician. Only one physician had previously addressed this issue. CONCLUSION: Cough-related urinary incontinence is common in patients with interstitial lung disease and is largely overlooked. It may significantly alter quality of life. A systematic questioning by the physician would allow to promptly refer these patients for appropriate therapeutic interventions, such as perineal training