Relapse of respiratory insufficiency one year after organising pneumonia.
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Aged↗
Biomedical subjects
D Valeyre
Publications and source records attributed to D Valeyre.
[Adult pulmonary Langerhans' cell histiocytosis].
INTRODUCTION: Adult pulmonary Langerhans'cell histiocytosis, also referred to as histiocytosis X, is a disorder of unknown etiology which affects preferentially young smokers. The disease is characterized by granulomatous lesions which progressively invade and destroy distal airways, leading to the formation of characteristic cicatricial kystic lesions. Florid granulomas contain numerous Langerhans'cells, antigen-presenting cells of the dendritic cell lineage, associated with T lymphocytes and eosinophils. The diagnosis rests on the combination of clinical and radiologic data, and particularly on high-resolution CT scan findings showing a typical association of nodular and cystic changes, predominantly in the upper and middle lobes. Further evaluation with surgical lung biopsy is indicated in less typical situations. CURRENT KNOWLEDGE AND KEY POINTS: The pathogenesis of Langerhans'cell histiocytosis is not fully understood, but several arguments suggest that the disease results from an abnormal immune reaction initiated by Langerhans'cells and directed against the bronchial epithelium. Other arguments suggest the presence of genetic abnormalities susceptible, for example, to increase the sensitivity of these cells to cytokines (GM-CSF, or others) known to influence their survival and maturation. FUTURE PROSPECTS AND PROJECTS: These recent advances in the pathogenesis of Langerhans'cell histiocytosis could promote the development of new therapeutic strategies designed to regulate the number and activated state of Langerhans'cells in specific lesions.
Adult↗
Cytokine profiles in idiopathic pulmonary fibrosis suggest an important role for TGF-beta and IL-10.
Modulation of cytokine expression represents a potentially useful approach for the treatment of idiopathic pulmonary fibrosis (IPF). To identify potential targets for such intervention, semi-quantitative reverse transcriptase-polymerase chain reaction was used to compare the expression of messenger ribonucleic acids (mRNAs) coding for 17 cytokines in lung tissue obtained from patients with IPF at the time of diagnosis and control subjects. Some cytokines were also studied at the protein level by immunohistochemical techniques. mRNAs coding for all of the cytokines evaluated were detected in both control and fibrotic lung samples. Only transforming growth factor (TGF)-beta and interleukin (IL)-10 mRNAs were quantitatively increased in lung biopsies from patients with IPF compared with those of controls, results confirmed at the protein level by immunohistochemistry. Although mRNAs for platelet-derived growth factor (PDGF)-BB and keratinocyte growth factor (KGF) were expressed in similar amounts in lungs from patients with IPF and controls, localised accumulation of both factors was also observed in IPF. Hyperplastic alveolar epithelial cells were a prominent source of cytokines, where IL-10, PDGF-BB and KGF were present in increased amounts, although increased accumulation in fibroblasts, smooth-muscle cells and matrix components was also observed (PDGF-BB, TGF-beta). These results offer new insights into the cytokines produced in the lung in idiopathic pulmonary fibrosis and suggest that modulation of the production of transforming growth factor-beta and interleukin-10 may represent a potentially useful therapeutic strategy for this disabling disease.
Female↗
[Isolated pleural metastases from an atypical meningioma].
INTRODUCTION: Less than 2 per 1,000 meningiomas are complicated by extra-cranial metastases. These are most often found in the lung parenchyma, liver or lymph nodes. They almost always occur in anaplastic meningiomas (grade III according to OMS) and much more rarely in atypical meningiomas (grade II). CASE REPORT: We report a case of pleural metastases from a primary frontal atypical meningioma with no other extra-cranial spread. CONCLUSION: Poorly differentiated meningioma presents many morphological and immuno-histochemical similarities to malignant mesothelioma. For this reason the diagnosis of pleural metastase from a meningioma cannot be made without knowledge of the primary meningeal tumour and its histological type.
Aged↗
[Multiple pulmonary nodules with halo sign].
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Adult↗
[Cardiac sarcoidosis].
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Cardiomyopathies↗
[Peritoneal tuberculosis: 27 cases in the suburbs of northeastern Paris].
SETTING: Peritoneal tuberculosis did not disappear from France during the 1990s. OBJECTIVE: To determine the characteristics of peritoneal tuberculosis in the north-eastern suburbs of Paris. METHOD: A retrospective study of cases diagnosed with peritoneal tuberculosis between 1990 and 1998 in five suburban hospitals in the north-east region of Paris. RESULTS: Twenty-seven cases of adult peritoneal tuberculosis were diagnosed. There were nine women and 18 men, with a mean age of 37.5 years, 88.9% of whom were foreign born. General and digestive symptoms--abdominal pain and/or ascites--were present in 96.3% of the cases. The mean delay in treatment was 30 days. Peritoneal involvement was isolated in 25.9% of cases, and associated with pulmonary tuberculosis in 40.7% or hepatic tuberculosis in 25.9%. Co-infection with HIV (human immunodeficiency virus) was present 14.8% of cases. Culture of ascites fluid, laparoscopy and/or laparotomy (n = 17), with directed biopsy, aided in the formal diagnosis of peritoneal tuberculosis in 59.2%. One relapse and one case of multiresistance were observed. The mean duration of treatment was 9 months (range 6-12 months). Three patients received treatment with corticosteroids, and 91.2% of the patients achieved cure without sequelae. CONCLUSION: Peritoneal tuberculosis is not rare in the Paris region. The diagnosis should be suspected in case with ascites and fever, and can be confirmed by laparoscopy with sampling for bacteriology and histology. The methods of treatment need to be standardised.
Abdominal Pain↗
[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↗
[Retinal arteritis in a case of sarcoidosis. A case report].
Retinal vasculitis is a common finding and occurs in 10% - 83% of patients with sarcoidosis. In most cases, there are bilateral, peripheral and nonischemic periphlebitis. In contrast, periarterial involvement is rarely observed. We report a case of retinal vasculitis associated with sarcoidosis and its differential diagnosis. Sarcoidosis should not be excluded in case of periarteritis.
Aged↗
[Sarcoidosis (first part)].
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Anti-Inflammatory Agents↗
[Value of imaging in sarcoidosis].
Imaging plays an important role in identifying thoracic, abdominal and neurological localizations of sarcoidosis as well as ENT and bone localizations. It contributes to disease detection and is helpful for diagnostic, prognostic and therapeutic decision making and useful for therapeutic monitoring. The chest x-ray is particularly important in the initial work-up and to follow well-tolerated disease. Other investigations, depending on the localization, are helpful to follow the course of severe or chronic sarcoidosis.
Chronic Disease↗
[Severe pulmonary sarcoidosis].
Mediastinal and pulmonary localizations are found in 90% of al patients with sarcoidosis. In half the cases, the disease is not severe and is reversible without treatment. In the other half of cases, early or late respiratory complications can be seen. Early complications include subacute respiratory insufficiency by interstitial lung disease or by bronchial airway obstruction. Among late complications, the most frequent is pulmonary fibrosis. Four computed tomography patterns are found with variable functional impairments and course. Chronic obstructive respiratory insufficiency can be the consequence of specific bronchial lesions or pulmonary fibrosis surrounding proximal bronchi. Cor pulmonale is seen in 5% of cases. Aspergilloma seen in fibroemphysematous lesions can be the cause of major hempoptysis. Respiratory complications account for half of the 5% of deaths due to sarcoidosis. Respiratory complications are most often seen in radiographic stage III and IV disease. Treatments, mainly corticosteroids, only exert a suspensive effect but reduce the incidence and severity of respiratory manifestations. The gain obtained by treatment depends on the choice of the best time of institution and on the quality of monitoring. Lung transplantation is useful in most severe cases unresponsive to medical treatment.
Airway Obstruction↗
[Chronic infiltative lung disease and viruses].
A role for viruses in the development or course of the main idiopathic chronic infiltrative lung disease has been suggested for a long time. Viruses that have usually been incriminated in cryptogenic fibrosing alveolitis are hepatitis C virus, whose role has not been accurately proven, and latent viruses including Epstein-Barr virus and adenovirus. These latent viruses might be re-activated in the lung of patients immunocompromised by treatments and might be accountable for disease progression. However, published studies have been very conflicting and the only clinical trial testing ribavirin has failed to demonstrate a beneficial effect. In sarcoidosis, the responsibility of human herpesvirus 6 and 8 and retroviruses has not been proven. Finally, data in the literature do not support a link between Langerhans cell histiocytosis and human herpesvirus 6 and 8. These viruses may act by several mechanisms: viral proteins may be antigens driving an appropriate immune response; they may also behave as transactivating factors to control the expression of various genes involved in immune response, cell growth or synthesis of matrix proteins.
Cell Division↗
[Inflammatory pseudotumors of the lung with severe course].
Pulmonary inflammatory pseudotumors are usually unique lesions of unknown etiology with good prognosis. We report two severe cases with mediastinal invasion, local recurrence, extrathoracic locations, one of them with a fatal evolution. Certain microscopic features, which were present in our cases (increased cellularity, nuclear pleomorphism, mitotic activity, focal necrosis, bizarre giant cells, vascular invasion), may have prognostic relevance in determining an aggressive behavior of these tumors. Surgical resection is the recommended treatment, and incomplete resection, as in our cases, seems to be a risk factor for developing recurrent inflammatory pseudotumor. Immunosuppressive therapy was ineffective as well as radiotherapy in one of our cases. Only high doses of corticosteroids seemed to slow the evolution of the disease.
Adult↗
[Retinal vascularization in sarcoidosis].
OBJECTIVES: To describe the characteristics of retinal vasculitis related to sarcoidosis, and to study the radio-clinical findings of sarcoidosis in case of retinal vasculitis. METHODS: We performed a retrospective study on 33 cases of retinal vasculitis associated with sarcoidosis. Our patients had a complete ophthalmological examination and a systematic fluorescein angiography was performed. Recorded data included clinical, biological and radiological evaluation of sarcoidosis. RESULTS: Retinal vasculitis were and remained asymptomatic in almost a third of the patients (n = 10). In 11 patients (33%) a decreased visual acuity of more than 3 lines was observed. Periphlebitis was observed in all cases, and 75.7% were of non ischemic forms. Thirty patients out of 33 (90.9%) were treated with systemic steroids in whom 17 were treated for ophthalmological reasons. The presence of anterior or posterior uveitis, more than one attack of vasculitis, and significant hyalitis were statistically associated with visual acuity decrease. Ninety percent of patients regained their initial visual acuity, only three patients at the end of the medical follow-up remained with a visual acuity below or equal to 2/10. Thirty one patients (91%) presented with mediastinal and pulmonary signs concordant with sarcoidosis on chest x-rays. Extrapulmonary localisations were common in case of retinal vasculitis (91% of patients). CONCLUSION: Retinal vasculitis associated with sarcoidosis are often asymptomatic. In most cases, there are bilateral, non ischaemic periphlebitis. They seem to have a relatively better prognosis than in other etiologies of vasculitis and they are part of an evolutive multisystemic sarcoidosis. Most of the time treatment with systemic steroids was indicated for ophthalmological or extra ophthalmological reasons.
Administration, Oral↗
[Diagnostic approach in diffuse infiltrative lung diseases].
Infiltrative lung diseases (ILD) include more than 130 diseases. Chronic and acute forms of ILD must be differentiated. In each group, cases with a known cause or with an unknown cause have to be individualized. For the physician, the distinctive sign of ILD is the evidence of diffuse pulmonary opacities on chest X rays or a suggestive pattern on pulmonary function tests. The diagnosis of chronic ILD depends on epidemiologic data ("a priori" probabilities), anamnestic informations, clinical and radiological (radiography and tomodensitometry) findings which make possible to consider a diagnosis of high probability in at least 60% of cases and reduce the gamut of hypothesis in the others. Other investigations (blood, endoscopy, broncho-alveolar lavage and sometimes surgical lung biopsy) lead to the final diagnosis. Most frequent chronic ILD are sarcoidosis, idiopathic pulmonary fibrosis and connective disease associated ILD. In case of acute ILD, diagnostic procedures depend on the severity of the disease and symptomatic treatment is urgent. The main lung lesion is either oedema or alveolitis. Main acute ILD are respectively cardiogenic oedema, lesional oedema, intra-alveolar hemorrhage, infectious ILD and acute eosinophilic pneumonia. The diagnostic approach depends on the presence of an initial orientation. Sometimes, several causes are associated.
Acute Disease↗
Central nervous system sarcoidosis: follow-up at MR imaging during steroid therapy.
PURPOSE: To document the changes observed at sequential magnetic resonance (MR) imaging of sarcoidosis lesions of the central nervous system (CNS) during treatment with corticosteroids. MATERIALS AND METHODS: The abnormalities detected in 24 patients (mean follow-up, 36 months) were compared before and after therapeutic periods (n = 75) that were divided into attack (high-dose), upkeep (decreased-dose), and minimal (low-dose) periods. Parenchymal lesions were classified as type 1 (enhanced with gadolinium), type 2 (demyelinating), or type 3 (lacunar) and were assessed as regressing, stable, or progressing. RESULTS: Seven of the 24 patients had several types of lesions. Isolated type 3 lesions (six patients) were the only lesions not associated with neurologic deficit. Type 1 lesions (13 patients) regressed in 22 of 22 attack periods and progressed in nine of 27 upkeep and minimal periods. MR imaging depicted relapses in patients with multifocal CNS involvement or long-standing CNS impairment or in those who had previously received steroid therapy. Type 2 (seven patients) and type 3 (13 patients) lesions remained stable in 68 of 68 therapeutic periods. Type 1 lesions appeared in three patients with type 2 and type 3 lesions during two upkeep and three minimal periods. Findings at follow-up MR imaging contributed to the reintroduction of high-dose corticosteroid therapy in eight patients. CONCLUSION: MR imaging can be used to differentiate between reversible and irreversible lesions in CNS sarcoidosis. MR imaging can be a useful tool for adjusting treatment to prevent irreversible CNS damage.
Adult↗
Is high-resolution computed tomography a reliable tool to predict the histopathological activity of pulmonary Langerhans cell histiocytosis?
High-resolution computed tomography (HRCT) has proved to be very useful in the diagnosis and follow-up of pulmonary Langerhans cell histiocytosis (PLCH), but the precise relationships between nodules and thin-wall cysts observed by HRCT, and granulomatous or cystic lesions present in lung tissue, remain to be established. The aim of this study was to compare quantitative data obtained by HRCT and those obtained by histopathological examination of corresponding lung tissue specimens in patients with biopsy-proven PLCH. The results demonstrated that the extent of nodular abnormalities was strongly correlated with the density of florid granulomatous lesions in lung tissue. A strong correlation was also found between the extent of cystic abnormalities and the density of cavitary lesions, but the latter included both still inflammatory cavitary granulomas and cicatricial fibrous cysts. Interestingly, small isolated florid granulomas were found in lung tissue from most patients with a predominant cystic CT scan pattern. Taken together, these results demonstrate that HRCT has to be considered with caution to evaluate the histopathological activity of PLCH. Patients presenting with predominant HRCT cystic abnormalities should benefit from a long-term follow-up. Because these patients are susceptible to developing severe respiratory insufficiency, they should also be considered for treatment as soon as an effective therapy for LCH is available.
Adult↗