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J-F Cordier

Publications and source records attributed to J-F Cordier.

15 recordsLinked to original sources

Diagnostic value of serum precipitins to mould antigens in active hypersensitivity pneumonitis.

Serum precipitins have a controversial diagnostic value in hypersensitivity pneumonitis (HP). The present authors' objective was to assess their diagnostic value by developing scores from a panel of specific antigens tested by two techniques (electrosyneresis and double diffusion) to discriminate active HP from other interstitial lung diseases. Consecutive patients presenting with a condition for which HP was considered in the differential diagnosis were included in the study. All patients underwent the same standardised diagnostic procedure, including precipitin tests performed in routine conditions. Clinical manifestations, bronchoalveolar lavage and high-resolution computed tomography defined the presence or absence of HP. Receiver-operating characteristic curves and logistic regression were used to develop the serological scores. A total of 122 patients (including 31 cases of HP) were included in the study. Five antigens from the panel were selected for the serological scores (Absidia corymbifera, Eurotium amstelodami, Wallemia sebi, Saccharopolyspora rectivirgula and mesophilic Streptomyces sp.). Electrosyneresis was more discriminative than the double-diffusion technique. Predictive negative values varied 81-88% and predictive positive values varied 71-75% for prevalence of HP 20-35%. In conclusion, serological scores using a panel of relevant antigens may guide both biological and clinical practice in areas of high prevalence of hypersensitivity pneumonitis.

Adult↗

[Pulmonary MALT lymphoma revealing AIDS].

INTRODUCTION: We report the case of a patient with an isolated pulmonary mucosa associated lymphoid tissue (MALT) lymphoma that revealed an acquired immune deficiency syndrome (AIDS). CASE REPORT: A 30 year old man from Central Africa was admitted to hospital with cough, dyspnoea and general weakness. A diagnosis of HIV infection was made promptly. The thoracic CT scan revealed diffuse bilateral ground glass opacities as well as consolidation of the right upper lobe. After a non-diagnostic endoscopy the diagnosis of a low grade B cell MALT lymphoma (CD20+) was made by lung biopsy and confirmed by the presence of the t(11;18) translocation. No extrathoracic lymphoma was found. Treatment with rituximab and triple anti-retroviral therapy led to a rapid and complete remission that was maintained for 3 years after the diagnosis. CONCLUSION: Pulmonary MALT lymphoma may reveal AIDS. A combination of rituximab and anti-retroviral therapy led to complete remission in this patient.

Acquired Immunodeficiency Syndrome↗

Cryptogenic organising pneumonia.

Organising pneumonia is defined histopathologically by intra-alveolar buds of granulation tissue, consisting of intermixed myofibroblasts and connective tissue. Although nonspecific, this histopathological pattern, together with characteristic clinical and imaging features, defines cryptogenic organising pneumonia when no cause or peculiar underlying context is found. Rapid clinical and imaging improvement is obtained with corticosteroid treatment, but relapses are common after stopping treatment.

Cryptogenic Organizing Pneumonia↗

[Pulmonary manifestations of hereditary hemorrhagic telangiectasia].

INTRODUCTION: Osler-Weber-Rendu disease (hereditary hemorrhagic telangiectasia) is an autosomal dominant genetic disorder with variable penetrance. It is estimated to affect at least one in ten thousand of the population in France. The diagnosis is clinical and depends on the association of epistaxis, telangiectasia, visceral manifestations of the disease, and familial occurrence. STATE OF THE ART: Pulmonary arterio-venous malformations (AVM) which occur in about 15-30% of patients with this condition represent the main visceral complication of the disease. Infectious and ischaemic neurological manifestations due to paradoxical embolism may occur and may be the presenting feature. The high frequency of neurological complications even in asymptomatic patients justifies systematic screening for pulmonary AVMs, using chest radiography, contrast echocardiography, and/or chest CT. Treatment is based on percutaneous transcatheter coil vaso-occlusion of the feeding artery. CONCLUSION: Pulmonary arterial hypertension is rare. It may be due to systemic arteriovenous shunting in the liver increasing cardiac output, or be similar to idiopathic pulmonary hypertension.

Arteriovenous Malformations↗

[Pulmonary vascular manifestations in hereditary hemorrhagic telangiectasia].

Hereditary hemorrhagic telangiectasia (Osler-Rendu-Weber disease) is a genetic disorder with autosomal dominance, variable penetrance, and an estimated prevalence of 1/10,000 inhabitants in France. Diagnosis is based on clinical criteria including epistaxis, telangiectasia, visceral manifestations, and familial occurrence. Pulmonary arteriovenous malformations, present in 15-33% of patients, are its primary visceral complications. The disease may be revealed by infectious and ischemic neurological manifestations due to paradoxical embolism. The high frequency of neurologic complications even in asymptomatic patients justifies systematic screening for pulmonary arteriovenous malformations. Treatment of these malformations is based on percutaneous transcatheter coil embolization of the feeding artery. Pulmonary arterial hypertension is rare in this disease. It may be due to systemic arteriovenous shunting in the liver, which increases cardiac output, or be similar to idiopathic pulmonary hypertension.

Algorithms↗

Eosinophilic pneumonias.

Eosinophilic pneumonias (EP) encompass a wide spectrum of lung diseases characterized by peripheral blood eosinophilia (>1 x 10(9) eosinophils/l) and/or alveolar eosinophilia (>25%). Blood eosinophilia may be lacking, as in the early phase of idiopathic acute EP, or in patients already taking oral corticosteroids. EP may present with varying severity, ranging from almost asymptomatic infiltrates to the acute respiratory distress syndrome necessitating mechanical ventilation. Possible causes of EP must be thoroughly investigated, especially drugs and the variety of parasitic infections (considering history of travel or residence in areas of endemic parasitic infection). However, chronic EP remains idiopathic in many cases. When present, extrathoracic manifestations lead to suspect Churg-Strauss syndrome (CSS) or the hypereosinophilic syndrome (HES), the prognosis of which is dominated by cardiac involvement. Apart from the treatment of specific causes when possible, corticosteroids remain the cornerstone of symptomatic treatment for eosinophilic disorders, usually with a dramatic response, but frequent relapses when tapering or after stopping the treatment. The adjunction of immunosuppressants to corticosteroids is necessary in patients with CSS and poor prognosis factors. Imatinib has recently proven effective in the treatment of the myeloproliferative variant of the HES.

Adrenal Cortex Hormones↗

Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity.

The syndrome resulting from combined pulmonary fibrosis and emphysema has not been comprehensively described. The current authors conducted a retrospective study of 61 patients with both emphysema of the upper zones and diffuse parenchymal lung disease with fibrosis of the lower zones of the lungs on chest computed tomography. Patients (all smokers) included 60 males and one female, with a mean age of 65 yrs. Dyspnoea on exertion was present in all patients. Basal crackles were found in 87% and finger clubbing in 43%. Pulmonary function tests were as follows (mean+/-sd): total lung capacity 88%+/-17, forced vital capacity (FVC) 88%+/-18, forced expiratory volume in one second (FEV1) 80%+/-21 (% predicted), FEV1/FVC 69%+/-13, carbon monoxide diffusion capacity of the lung 37%+/-16 (% predicted), carbon monoxide transfer coefficient 46%+/-19. Pulmonary hypertension was present in 47% of patients at diagnosis, and 55% during follow-up. Patients were followed for a mean of 2.1+/-2.8 yrs from diagnosis. Survival was 87.5% at 2 yrs and 54.6% at 5 yrs, with a median of 6.1 yrs. The presence of pulmonary hypertension at diagnosis was a critical determinant of prognosis. The authors hereby individualise the computer tomography-defined syndrome of combined pulmonary fibrosis and emphysema characterised by subnormal spirometry, severe impairment of gas exchange, high prevalence of pulmonary hypertension, and poor survival.

Adult↗

[Diffuse idiopathic interstitial pneumonias. International multidisciplinary consensus classification by the American Thoracic Society and the European Respiratory Society, principal clinico-pathological entities, and diagnosis].

INTRODUCTION: The classification of the idiopathic interstitial pneumonias includes seven clinico-pathologic entities. The diagnosis is based on a multidisciplinary approach, integrating the clinical evaluation, the high-resolution computerised tomography, and the pathological pattern. STATE OF THE ART: A definitive diagnosis of idiopathic pulmonary fibrosis relies on the association of a suggestive clinico-radiological profile and a pathological pattern of usual interstitial pneumonia. Nonspecific interstitial pneumonia is a recently described clinico-pathologic entity, with a better prognosis than that of idiopathic pulmonary fibrosis. Cryptogenic organising pneumonia has been included in the group of idiopathic interstitial pneumonias because of its idiopathic and multifocal characteristics, although it does not predominate in the lung interstitium. Desquamative interstitial pneumonia and respiratory bronchiolitis with interstitial lung disease are rare entities with predominance in young smoking adults. Lymphoid interstitial pneumonia, usually encountered in the context of Sjögren's syndrome, is very rare in its idiopathic form. Acute interstitial pneumonia is responsible for idiopathic acute respiratory distress syndrome. PERSPECTIVES: The current classification of idiopathic interstitial pneumonias better defines the diagnostic criterias of each clinico-pathologic entity, and is expected to facilitate clinical research. CONCLUSIONS: This classification has clinical implications, with prognostic and therapeutic significance.

Anti-Inflammatory Agents↗

[Renal AA amyloidosis secondary to bronchiectasis: a report of two cases (including one with Mounier-Kuhn syndrome)].

INTRODUCTION: We describe 2 patients with AA renal amyloidosis secondary to bronchiectasis (one patient had tracheobronchomegaly or Mounier-Kuhn syndrome). CASE REPORTS: The time period between the diagnosis of bronchiectasis and the development of renal amyloidosis was 40 years and 30 years respectively. Both patients evolved to end-stage renal failure requiring dialysis. CONCLUSION: Although the incidence of renal amyloidosis secondary to bronchectasis has declined, it remains a dreadful complication.

Aged↗

Thymic carcinoma: a clinicopathological and immunohistological study of 19 cases.

AIMS: To study 19 cases of primary thymic carcinoma in order to define the clinicopathological features and the precise histochemical profile of this rare and heterogeneous group of tumours of the anterior mediastinum. METHODS AND RESULTS: The study group consisted of 13 males and six females, with a mean age of 58.5 years (range 29-75 years). Superior vena cava syndrome and chest pain were the main presenting symptoms. Three patients were asymptomatic. No patient had myasthenia gravis. Six different histological types were identified: neuroendocrine tumours (six patients), epidermoid carcinoma (five patients), sarcomatoid carcinoma (three patients), lymphoepithelioma-like carcinoma (two patients), mucoepidermoid carcinoma, clear cell carcinoma, and undifferentiated carcinoma (one patient each). The clear cell carcinoma was associated with a thymic cyst. No association with thymoma was observed. Surgical resection, performed in 10 cases, was complete in two. Sixteen patients received thoracic radiation, and 11 received systemic chemotherapy. Follow-up information was available in 16 cases; 12 patients presented with local or metastatic relapse, and 10 patients died of their tumour. The overall 5-year survival was 14.5%. CONCLUSION: Primary thymic carcinoma is a very heterogeneous group of tumours of the anterior mediastinum with an aggressive clinical behaviour, and a poor overall prognosis.

Adult↗

[Churg-Strauss syndrome].

Churg-Strauss syndrome is a vasculitis associated with asthma and eosinophilia. Respiratory involvement is marked by generally severe and often steroid-dependent late-onset asthma associated with allergic rhinitis and sometimes nasal polyposis and recurrent sinusitis. Asthma generally precedes the systemic vasculitis by a few years. General signs, eosinophilic gastroenteritis, peripheral multiplex neuropathy, cutaneous vasculitis, nephropathy, or arthromyalgia, predominate. Cardiac involvement is often silent but of severe prognosis. The chest X-ray usually shows irregularly delimited and sometimes labile infiltrates. Perinuclear antineutrophil cytoplasmic autoantibodies (ANCA) are found in two-thirds of the patients and strongly suggest the diagnosis. Clinically, the diagnosis is established by the presence of asthma, peripheral eosinophilia > 1.5 G/L, and systemic vasculitis involving at least two extra-pulmonary organs. Histological confirmation is usually necessary (nerve and muscle biopsy), showing small-vessel eosinophilic vasculitis, tissue infiltration with eosinophils, and eosinophilic granulomas. Treatment includes corticosteroids, which should be associated with immunosuppressive agents (cyclophosphamide) in severe cases.

Churg-Strauss Syndrome↗

[Chronic cough after laparoscopic adjustable gastric banding].

INTRODUCTION: Patients are frequently referred for chronic cough. The causes are various. CASE REPORT: We report two cases of chronic cough that occurred after laparoscopic adjustable gastric banding for treatment of morbid obesity. In both cases, the computed tomography scan showed an important oesophageal dilatation. The cough disappeared after the band deflation. CONCLUSION: Oesophageal dilatation after laparoscopic adjustable gastric banding is a new cause to be included in the aetiology of chronic cough.

Chronic Disease↗

[Pulmonary alveolar proteinosis].

Pulmonary alveolar proteinosis (PAP) is a rare, diffuse infiltrative lung disease of unknown etiology characterized by accumulation of abnormal lung surfactant in the alveoli and distal airways. Although it was first described in 1958, few large scale studies have been published. The diagnosis is conventionally based on lung biopsy and the value of less invasive procedures such as bronchoalveolar lavage (BAL) remains controversial. A multi-center retrospective study was conducted in an attempt to update epidemiological, clinical and long term outcome and to assess the usefulness of chest CT scan and BAL in the management of a large homogeneous series of 41 patients. Inclusion criteria required a clinical picture compatible with PAP and either histological confirmation, or a BAL with a milky macroscopic appearance coupled with positive staining to PAS. Mean age was 48.8 years with a 4: 1 male predominance. The prevalence of smokers (80%) and occupational exposure to various inhaled dusts (39%) was high. The clinical, roentgenographic and functional features were in keeping with previous series. Worsening dyspnea was the most common symptom. Other common manifestations were dry cough, recurrent lung infections, weight loss, finger clubbing, crackles and cyanosis. LDH serum levels were increased in 51% of cases. Lung function tests usually showed a mild restrictive ventilatory defect with hypoxemia and reduced DLCO. Chest X ray revealed bilateral ground glass opacities with consolidation or air bronchogram involving peri-hilar or lower lobes. Chest CT scan, performed in 38 patients, revealed a similar pattern in most of them with diffuse intra-alveolar ground glass opacities (33/38) and interlobular thickening (35/38), resulting in a crazy paving appearance in the typical geographic pattern (32/38). This latter aspect was highly suggestive of the diagnosis. Segmental BAL led to a definitive diagnosis in 62% and lung biopsy was only required in 38% of cases. These data suggest that careful BAL analysis can be sufficient to provide a diagnosis without the need for a lung biopsy. During follow-up, spontaneous remission or improvement was reported in 26%. Segmental (n=3) or whole lung lavage (n=22) was required in 62.5% with a favorable course (complete remission 37%, improvement 34%, stable course 21%) in 92% of the cases. In this series the overall prognosis for PAP remained good, although unpredictable.

Adolescent↗