Showing papers by "Vincent Cottin published in 2009"

Characterisation of severe obliterative bronchiolitis in rheumatoid arthritis

Abstract: The characteristics of patients with rheumatoid arthritis (RA) who develop obliterative bronchiolitis characterised by severe airflow obstruction have been hitherto poorly investigated. A retrospective study of 25 patients with RA and functional evidence of obliterative bronchiolitis (forced expiratory volume in one second (FEV1)/forced vital capacity (FVC) 140% predicted) was conducted. Patients (mean +/- SD age 64 +/- 11 yrs) included 17 never-smokers and eight ex-smokers (10.5 +/- 5.4 pack-yrs). The diagnosis of RA preceded respiratory symptoms in 88% of cases. Dyspnoea on exertion was present in all patients and bronchorrhea in 44%. High-resolution computed tomography findings included: bronchial wall thickening (96%), bronchiectasis (40%), mosaic pattern (40%), centrilobular emphysema (56%), and reticular and/or ground-glass opacities (32%). Pulmonary function tests showed: FEV1 41 +/- 12% pred, FEV1/FVC 49 +/- 14%, FVC 70 +/- 20% pred, RV 148 +/- 68% pred and RV/TLC 142 +/- 34% pred. Lung biopsy, available in nine patients, demonstrated constrictive, follicular and mixed bronchiolitis. Patients were followed for 48.2 +/- 49 months. Treatment was poorly effective. Chronic respiratory failure occurred in 40% of patients, and four patients died. Obliterative bronchiolitis associated with rheumatoid arthritis is a severe and under-recognised condition leading to respiratory failure and death in a high proportion of patients.

Syndrome emphysème des sommets et fibrose pulmonaire des bases combinés (syndrome emphysème/fibrose) : aspects tomodensitométriques et fonctionnels

Abstract: Combined apical emphysema and basal fibrosis syndrome (emphysema/fibrosis syndrome): CT imaging features and pulmonary function tests. Purpose. To describe the high resolution CT (HRCT) imaging and functional features of the emphysema/fibrosis syndrome. Patients and methods. A total of 61 patients were included based on HRCT. We have quantified the extent of fibrosis and emphysema lesions and a combined score was calculated. The scores were correlated to pulmonary function test parameters and specific HRCT features were described. Results. The emphysema and fibrosis scores correlated with functional parameters of obstruction and restriction respectively. The combined score correlated with the reduction in DLCO and degree of pulmonary hypertension. Three HRCT patterns were identified : progressive transition (n = 23, 38%) with diffuse emphysema (centrilobular and/or bullous) and zone of transition between bullae and honeycombing ; paraseptal emphysema (n = 13, 21%) with predominent subpleural bullae of enlarging size at the bases ; separate processes (n = 14, 23%) with independent areas of fibrosis and emphysema. Eleven patients (18%) could not be classified. The HRCT imaging features changed based on TLC (p = 0.04) and FEV1/FVC (p = 0.01). Conclusion. The emphysema/fibrosis syndrome may be associated with different patterns on HRCT corresponding to specific profiles on pulmonary function tests.

Progressive diffuse pulmonary Langerhans cell histiocytosis improved by cladribine chemotherapy

Abstract: Langerhans cell histiocytosis (LCH) is a group of disorders characterised by monoclonal proliferation of histiocytic cells (Langerhans cells) producing tumour-like masses in multiple organs including the bone, skin, lymph nodes and central nervous system. In contrast to multiorgan LCH, pulmonary LCH (PLCH) usually involves only a single organ and presents as an infiltrative lung disease. PLCH is strongly correlated with smoking and presumably reflects reactive Langerhans cell proliferation triggered by some inhaled agent.1 In early cellular PLCH, Langerhans cells aggregate in multiple small bronchiolocentric granulomas which may further cavitate to form inflammatory thick-walled cysts, usually predominating in the upper lobes. With disease progression, PLCH may evolve towards irreversible lung destruction by cicatricial fibrotic thin-walled cysts, respiratory insufficiency and death or …

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

Abstract: Background: Little is known about the long-term outcome of airflow obstruction in asthma of patients with Churg–Strauss syndrome (CSS). Methods: 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 × 109/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. Results: 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. Conclusion: 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.

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

Abstract: To the Editors: Whereas tobacco smoking is a well-established risk factor for the development and progression of chronic obstructive pulmonary disease (COPD), the molecular basis for individual predisposition and disease progression remains largely unknown. Immunological processes and especially autoreactive immune processes have recently been implicated, with anti-elastin antibodies and a T-helper type-1 lymphocyte response identified in patients with COPD/emphysema 1. Immune mechanisms are also implicated in interstitial lung disease and pulmonary fibrosis 2, and smoking-induced autoimmunity has been demonstrated in rheumatoid arthritis 3. We explored whether an autoimmune process implicating anti-elastin autoantibodies and facilitated by tobacco smoking may take place in patients with combined pulmonary fibrosis and emphysema (CPFE), a distinct entity recently defined on the basis of characteristic features of chest imaging, pulmonary function and outcome 4. Patients with CPFE, diagnosed within …

Right-to-left shunt with hypoxemia in pulmonary hypertension

Abstract: 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. 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. 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. PaO2 in room air was 6.8 ± 1.3 kPa. Qs/Qt was 10.2 ± 4.2%. AaPO2 under 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. When present in patients with precapillary PH, RL shunting is usually not related to reopening of patent foramen ovale, whatever the etiology of PH.

Incontinence urinaire à la toux au cours des pneumopathies interstitielles diffuses

Abstract: Introduction L’incontinence urinaire d’effort, dont la prevalence est estimee a 10-30 % dans la population feminine, peut avoir un retentissement psychosocial majeur. Dans les pneumopathies interstitielles diffuses (PID), la toux chronique peut favoriser l’apparition d’une incontinence urinaire, mais la prevalence et les consequences de ce symptome ne sont pas connues. Objectif Determiner la frequence et l’impact de l’incontinence urinaire chez les femmes presentant une toux chronique au cours d’une PID. Methodes Vingt-huit patientes presentant une toux chronique associee a une PID et 15 patientes controles ont ete interrogees a l’aide de questionnaires pour evaluer l’existence d’une incontinence urinaire d’effort, sa severite (questionnaire K. Bo Index), et son retentissement sur la qualite de vie (questionnaire Ditrovie). Resultats Une incontinence urinaire a la toux etait presente chez 14/28 patientes ayant une PID avec toux chronique (50 %) mais chez seulement 1/15 patientes controles (7 %, p = 0,005). Sur une echelle de qualite de vie a 5 points, l’impact median de l’incontinence urinaire etait de 3 (aucun = 1, maximal = 5) et l’impact median de la toux etait de 3,5. La majorite des patientes (64 %) pensaient que l’incontinence etait un phenomene naturel accompagnant le vieillissement, toutes eprouvaient un sentiment de honte vis-a-vis de leur incontinence et 79 % n’osaient pas en parler a leur medecin. Seul un medecin avait aborde ce probleme auparavant. Conclusion L’incontinence urinaire a la toux est frequente au cours des PID et largement meconnue. Elle peut entrainer une alteration importante de la qualite de vie. Sa recherche systematique a l’interrogatoire permettrait d’orienter rapidement les patientes vers un traitement approprie, telle que la reeducation perineale.

Near-fatal haemorrhage from pulmonary arteriovenous malformation in HHT with increased cardiac output

Abstract: To the Editor: We read with interest the report by Montani et al. [1] of fatal rupture of pulmonary arteriovenous malformation (AVM) in a patient with hereditary haemorrhagic telangiectasis (HHT) and severe pulmonary arterial hypertension (PAH) confirmed by right heart catheterisation. The authors hypothesised that bleeding from pulmonary AVM may have been facilitated by increased mean pulmonary artery pressure ( P pa). Here, we report a patient with near-fatal haemorrhage from pulmonary AVM, probably facilitated by increased cardiac output, but without severe PAH. A 35-yr-old female nonsmoker, with a history of obesity and fenfluramide use 10 yrs previously for 2 months, was admitted for exertional dyspnoea during pregnancy. Arterial blood gases showed arterial oxygen tension ( P a,O2) of 8.2 kPa, and arterial carbon dioxide tension of 3.5 kPa, with markedly increased alveolar–arterial oxygen tension difference on 100% oxygen (74 kPa, normally <18.6 kPa). Chest radiograph revealed numerous pulmonary AVMs. Transthoracic contrast echocardiography showed estimated systolic P pa of 47 mmHg with moderate dilatation of the right heart cavities, and extracardiac right-to-left shunting. She had recurrent epistaxis since childhood, numerous characteristic telangiectasiae, and possible familial HHT in her paternal grandmother. HHT was diagnosed according to the Curacao criteria [2]. One deletion was found in exon 9 of the ENG gene, and analysis of the ACVRL1 gene was normal. Long-term oxygen therapy was initiated. Emergency caesarean delivery was performed at 25 weeks of gestation because of …

Unusual bilateral acinar nodules: case for diagnosis.

Abstract: Abdominal ultrasonography was normal. Erythrocyte sedimentation rate was 50 mm/h, and C-reactive protein was 15 mg/l. Peripheral blood neutrophils were 12.4 ! 10 9 /l, lymphocytes 1.6 ! 10 9 /l, eosinophils 2.2 ! 10 9 /l. Serum protein level was elevated (83 g/l), with polyclonal hyper-gamma-globulinemia (15.7 g/l). Serology for Epstein-Barr virus showed absence of IgM antibodies. Serologies for human immunodeficiency virus and hepatitis B and C viruses were negative. Serum lactate dehydrogenase was normal. Antinuclear and antineutrophil cytoplasmic antibodies were negative. The patient underwent videothoracoscopic lung biopsies ( fig. 2 ). What is your diagnosis? Case Report