[Idiopathic pulmonary fibrosis: recent diagnostic and therapeutic advances]. / Fibrose pulmonaire idiopathique: nouveautés diagnostiques et thérapeutiques.

Idiopathic pulmonary fibrosis (IPF) is the most frequent of the idiopathic interstitial pneumonias. It is a progressive disorderwith a poor prognosis. Its diagnosis requires the careful exclusion of potential causes, and a pattern of usual interstitial pneumonia at high-resolution computed tomography or video-assisted surgical lung biopsy. Several recent randomized trials have profoundly modified the therapeutic management of IPF. The combination of prednisone and azathioprine, often prescribed until recently, has been shown to be harmful and is no longer indicated. N-acetylcystein, also used in the past decade, failed to show an efficacy. However, two new antifibrotic drugs, pirfenidone and nintedanib, have for the first time proven effective in slowing disease progression.

[Lung volume reduction (LVR) in severe emphysema: a multidisciplinary approach]. / Réduction de volume pulmonaire dans l'emphysème sévère: importance d'une prise en charge multidisciplinaire.

Most cases of emphysema are managed conservatively. However, in severe symptomatic emphysema associated with hyperinflation, lung volume reduction (LVR) may be proposed to improve dyspnea, exercice capacity, pulmonary functions, walk distance and to decrease long-term mortality. LVR may be achieved either surgically (LVRS) or endoscopically (EVLR by valves or coils) according to specific clinical criteria. Currently, the optimal approach is discussed in a multidisciplinary setting. The latter permits a personalized evaluation the patient's clinical status and allows the best possible therapeutic intervention to be proposed to the patient.

[Cryptogenic organizing pneumonia]. / Pneumopathie organisée cryptogénique.

INTRODUCTION: Organizing pneumonia is a particular type of inflammatory reaction of the lung which gives rise to a clinico-pathological syndrome. It is called "secondary" when a cause such as an infection, a drug toxicity, or a connective tissue disease can be identified, or "cryptogenic" when no cause is identified. The clinical picture is usually characterized by the subacute onset of fever, fatigue, cough and dyspnea, with multiple subpleural areas of consolidation on thoracic imaging. STATE OF THE ART: Organizing pneumonia is characterised by the presence of buds of endoalveolar connective tissue. These result from an injury to the alveolar epithelium, followed by the deposition of fibrin in the alveolar spaces, and the migration of fibroblasts which produce a myxoid endoalveolar matrix. A remarkable feature of organizing pneumonia is the complete disappearance of these endoalveolar buds with corticosteroid treatment, in sharp contrast with what is seen in pulmonary fibrosis. The clinical response to corticosteroids is usually prompt and excellent. Relapses are frequent but usually benign. PERSPECTIVES AND CONCLUSION: As the clinical, imaging and pathological characteristics of organizing pneumonia are now well established, many questions remain unanswered, such as the mechanisms involved in the complete reversibility of the pulmonary lesions, and the role of steroid-sparing treatments such as immunomodulatory macrolides.

Pheochromocytoma as the first manifestation of von Hippel-Lindau disease.

BACKGROUND: von Hippel-Lindau disease is an autosomal dominant disorder characterized by the development of hemangioblastomas in the cerebellum, spinal cord, and retina, renal cell carcinoma and cysts, pancreatic cysts, and pheochromocytoma. METHODS: We have studied a series of 36 French patients affected with von Hippel-Lindau disease pheochromocytoma. Thirty (83%) of them were diagnosed as having von Hippel-Lindau disease because the disease occurred in a familial von Hippel-Lindau disease setting; six (17%) were diagnosed as having von Hippel-Lindau disease because they displayed another characteristic manifestation of that disease. RESULTS: The mean age at pheochromocytoma diagnosis was 29 +/- 14 years (5 to 62 years). Bilateral tumors were documented in 15 (42%) cases, paraganglioma was associated with adrenal pheochromocytoma in four cases, and malignant pheochromocytoma occurred in three cases. Prevalence of pheochromocytoma revealing von Hippel-Lindau disease was 20 (53%) out of 36. In six cases pheochromocytoma was the only manifestation of the disease. CONCLUSIONS: In the interest of the patients themselves and of family members who are at risk, search for von Hippel-Lindau disease must be systematic in the presence of pheochromocytoma. Basic checkup may be completed with familial inquiry, ophthalmoscopy, cerebral magnetic resonance imaging, abdominal ultrasonography, and computed tomography-scan for detection of latent lesions. In the future, after characterization of von Hippel-Lindau disease gene mutations, molecular diagnosis is going to be possible in individual patients.

Iron oxide-enhanced MR lymphography: initial experience.

The detection of nodal metastases is of utmost importance in oncologic imaging. Ultrasmall superparamagnetic iron oxide particles (USPIO) are novel contrast agents specifically developed for MR lymphography. After intravenous administration, they are taken up by the macrophages of the lymph nodes, where they accumulate. They reduce the signal intensity (SI) of normally functioning nodes on postcontrast T2-and T2*-weighted images through the magnetic susceptibility effects on iron oxide. Metastatic nodes, in which macrophages are replaced by tumor cells, show no significant change in SI on postcontrast T2-and T2*-weighted images. Early clinical experience suggests that USPIO-enhanced MR lymphography improves the sensitivity and specificity for the detection of nodal metastases. It also suggests that micrometastases could be detected in normal-sized nodes. This article reviews the physiochemical properties of USPIO contrast agents, their enhancement patterns, and early clinical experience.

High-resolution computed tomography of the airways.

High-resolution computed tomography (HRCT) is the imaging modality of choice to evaluate most bronchial tree lesions, especially those affecting small airways. It can confirm the diagnosis of bronchiectasis with high sensitivity and specificity and may contribute to the investigation of bronchiolitis (particularly diffuse panbronchiolitis and bronchiolitis obliterans) and other inflammatory disorders of the airways. HRCT can also reliably detect obstructing bronchial lesions such as tumors or broncholithiasis. It may also provide useful clues to the diagnosis of bronchial fistula, dehiscence, or rupture as well as permit the study of many pulmonary congenital abnormalities. This article describes the CT techniques recommended in each clinical situation and reviews the HRCT findings in diseases of the airway.

[Variety of pancreatic lesions observed in von Hippel-Lindau disease. Apropos of 8 cases]. / Variété des lésions pancréatiques observées au cours de la maladie de von Hippel-Lindau. Présentation de 8 cas.

Von Hippel-Lindau's disease is a rare genetic disease, with an autosomal dominant mode of inheritance, characterised by the development of several tumours, such as haemangioblastoma of the central nervous system and retina, renal cysts or carcinoma and pheochromocytoma. Several pancreatic lesions, mainly represented by multiple cysts, are also encountered. We report here 8 cases of pancreatic involvement in patients affected with von Hippel-Lindau's disease. It consisted of multiple cysts (3 cases), serous cystadenoma (2 cases), endocrine tumour (1 case), haemangioblastoma (1 case) and ductal adenocarcinoma (1 case). Diagnosis of a rare lesion of the pancreas (multiple cysts, serous cystadenoma or vascularized tumour) in a young patient may lead to search the other lesions of von Hippel-Lindau's disease and to undergo a familial inquiry in order to propose a multidisciplinary approach for patients affected by the disease.

[Subacute neuropathy, multiple cancers, and Gougerot-Sjögren syndrome: contribution of anti-Hu antibody]. / Neuropathie subaiguë, cancers multiples et syndrome de Gougerot-Sjögren: intérêt des anticorps anti-Hu.

We report the case of a patient presenting a subacute, predominantly sensory neuropathy. The work up revealed a Sjögren's syndrome and a breast carcinoma. The presence of anti-Hu antibodies, identified by Western Blot using purified recombinant HuD protein, and the absence of the Hu antigen in the breast carcinoma ruled out the responsibility of the Sjögren's syndrome or breast carcinoma. In this context, the most likely diagnosis was a subacute neuropathy associated with small cell lung cancer, which was indeed discovered 3 years later.

[CT angiography of the pulmonary arteries]. / Angioscanographie des vaisseaux pulmonaires.

CT angiography of the pulmonary arteries recently appeared as an alternative method potentially useful in the diagnosis of pulmonary embolism. Technical aspects, normal aspects and variants, diagnostic criteria of pulmonary embolism as well as potential pitfalls are successively described. The accuracy of CT angiography compared with pulmonary angiography and V-Q scintigraphy has been evaluated in several studies. The method appears accurate for the diagnosis of pulmonary embolism at the level of segmental arteries. The signification of more distal embolism remains however controversed. CT angiography seems to play a major role in the diagnosis of pulmonary embolism, as well as echo-doppler of the legs.

[Comparative evaluation of iopamidol 300 versus 370 during spiral x-ray computed tomographic studies of the thorax]. / Evaluation comparative du iopamidol 300 versus 370 au cours des explorations tomodensitométriques hélicoïdales du thorax.

PURPOSE: A retrospective study was performed to assess the role of iodine concentration on the opacification of different vascular compartments of the chest with a nonionic contrast agent (Iopamidol) during routine spiral CT examination. MATERIAL AND METHOD: 105 injected spiral CTs of the chest were studied. 50 examinations were done with Iopamidol 300 (iodine concentration 300 g/L) (I300). 55 examinations were done with Iopamidol 370 (iodine concentration 370 g/L) (I370). A constant 24- g total iodine dose was delivered with no difference in the injected iodine flow rates (respectively 0.6 and 0.55 g/s). Images were scored for opacification success, opacification quality and for artefacts in each vascular, of the chest. RESULTS: There was no statistically significant difference in opacification success or in opacification quality, in each chest vascular compartment between the group receiving I300 and the group receiving I370. Artefact frequencies on the lower part of superior vena cava were however different, 24% and 41.8% respectively. CONCLUSION: For a constant injected iodine flow rate, a moderate increase in iodine concentration, with decreased injection volume and flow rate, had little or no effect on opacification of the aorta and the pulmonary artery. The presence of artefacts on the lower part of the superior vena cava would suggest that the use of lower concentration contrast media would be advisable for specific vena cava examinations.

[Abdominal echography in colonic diseases]. / Intérêt de l'échographie abdominale en pathologie colique.

In the past few years, several studies have assessed the visualization of digestive diseases by ultrasound, but its clinical accuracy has been rarely defined. Thus we evaluated the contribution of sonographic examination as an initial diagnostic mean in the detection of colonic lesion. During a period of thirty six months (June 85-June 88) when we performed a standard sonographic abdominal examination, we utilized ultrasound to look for thickening or other modifications of the large bowel wall, if the clinical findings suggested a colonic disease. In these conditions we performed 453 US examinations. In our study, 363 cases were considered to be negative and the controls demonstrated colonic wall lesions in 24 cases. Thus the sensitivity was one of 77%. The sonographic examination was positive in 90 cases with 6 false positives. Thus our specificity reached 98.2% and the accuracy achieved was 93.3%. Thus sonographic examination appears to be a rapid, non invasive, mobile, mean of examining the abdominal cavity and also the large bowel in all patients. If this examination is negative, a colonic disease is possible since sensitivity is 77%. On the other hand, if the examination is positive, a colonic disease is very probable, since specificity reached 98.2%. The accuracy of US Colonic examination is 93.3% and with using abdominal plain film, theses percentages may be improved.

[Enteroclysis in chronic radioenteritis. Study of 43 cases]. / L'entéroclyse dans l'entérite radique chronique. Etude de 43 observations.

Enteroclysis were practiced for 43 patients with radiation induced injuries of the small bowel. The radiological data revealed mucosal lesions in 23 cases, submucosa lesions in 21 cases, parietal thickening in 20 cases, mesenteric involvement in 32 cases. Narrowings and stenosis were present in 24 cases and dilated loops in 15 cases. Fistula formation existed in two cases. Twenty four patients were operated and the diagnosis of radiation enteritis was confirmed. In 12 cases, the enteroclysis succeed to a per os technique. The authors showed the superiority of the enteroclysis.

[Enteroclysis in malabsorption syndrome in adults. Apropos of 73 cases]. / L'apport de l'entéroclyse dans les syndromes de malabsorption de l'adulte. A propos de 73 observations.

We retrospectively analyzed the small bowel radiographies, performed by enteroclysis in 73 patients presenting a malabsorption disease. The etiology are: celiac disease (44 cases), abetalipoprotidemia (2 cases), Whipple's disease (7 cases), mastocytosis (1 case), amyloidosis (1 case), ischemia enteritis (4 cases), radiation injury (4 cases), lymphangiectasia (4 cases), Crohn's disease (4 cases) and NH lymphoma (2 cases). With enteroclysis, the radiological diagnosis of malabsorption disease is improved and it is able to differentiate malabsorption diseases based on radiological features: increased or decreased number of folds (celiac disease, mastocytosis, abetalipoprotidemia), nodularity of folds (Whipple's disease) and wall thickness (amyloidosis, lymphangiectasia, ischemic enteritis and radiation injury). It is no possible to differentiate NH lymphoma from Crohn's disease.

[Thoracic infections in immunocompetent patients. The contribution of computed tomography]. / Infections thoraciques chez le sujet immunocompétent. Apport de la tomodensitométrie.

Chest X-Ray is the most accurate method of imaging for infectious diseases in an immunocompetent patient. Computed tomography (CT) may be useful in certain circumstances, particularly in case of atypical findings at the time of diagnosis or in case of complications. CT helps to detect and perform a complete study of the lesions, some aspects being very suggestive of a diagnosis, as in post-primary active tuberculosis. CT may also detect an unknown underlying etiology. Multiplanar reformations with helical CT can be useful for example in case of empyema. In case of non tuberculous bacterial infections, CT is mainly recommended when abscess and empyema are difficult to differentiate or in case of pleural complications with possible percutaneous treatment. In case of tuberculosis, CT may be indicated when clinical and chest X-Ray findings are discordant, in case of mediastinal adenopathies, when reactivation is suspected or in case of complications as hemoptysis. A baseline CT examination could be proposed at the end of a specific treatment to facilitate the diagnosis of reactivation tuberculosis. A nontuberculous mycobacterial infection should finally be suspected in front of peculiar CT findings.

[Does hemangioblastoma exist outside von Hippel-Lindau disease?]. / L'hémangioblastome existe-t-il en dehors de la maladie de von Hippel-Lindau?

Hemangioblastoma may arise in isolation ("sporadic" cases) or as a major manifestation of von Hippel-Lindau (VHL) disease, an autosomal dominant disorder with a prevalence of at least 1/36,000. In addition of central nervous system hemangioblastomas (cerebellum, spinal cord and retina), affected patients may develop renal cysts or carcinomas, pheochromocytomas and pancreatic cysts. A multidisciplinary group including neurosurgeons, geneticists, pathologists and clinicians from all involved specialities has been organized to develop a national registration of all hemangioblastoma and VHL patients. The findings of a preliminary 10-year study (1983-1993) conducted in France are presented. Two hundred thirteen cases of hemangioblastoma were reviewed for their location and genetic features. The majority (77%) of the tumors were located in the cerebellum whereas 23% were located inside the spinal canal. By thorough clinical examination of the patients and systematic genetic inquiry of their family background, it was found that 34.3% of the total (58.7% before age 30) were afflicted with VHL disease. Spinal hemangioblastomas were more often related to VHL disease than infra-tentorial locations (50% versus 36.6%). In addition, mean age at diagnosis in VHL disease was significantly younger than in sporadic cases (33.5 +/- 10 versus 43.6 +/- 15 years). Recent progress in VHL molecular genetics led to the identification of the mutated gene to the distal part of the short arm of chromosome 3 (3p25-3p26), paving the way to presymptomatic diagnosis and, hopefully, to elucidation of pathogenesis, which may offer a further glimpse into tumorigenesis in general. Because of the usually early adulthood onset, accurate presymptomatic diagnosis of affected members would be of great benefit to VHL families. However, the fact that very few mutations in the VHL gene are identified precludes molecular diagnosis of "sporadic" hemangioblastomas. In summary, this study reveals that VHL-related hemangioblastoma is a more common clinical problem that it was previously reported. Thus, all patients with an apparently isolated central nervous system hemangioblastoma should be investigated for evidence of VHL disease.

[Computed tomography in 2000: technique, expected progress, limitations, indications]. / La tomodensitom¿etrie en l'an 2000. Technique, progrès attendus, limites, indications.

Computed tomography (CT) is today a fundamental technique for thoracic imaging, a logical direct extension of the standard chest x-ray. Since the early 90s, volume-acquisition CT has greatly improved the performance of the conventional technique. After replacing lung and bronchial tomography, the technique has greatly contributed to the reduction in the number of diagnostic conventional angiographies. Certain decisional algorithms have been totally changed, particularly for the diagnosis of pulmonary embolism. In addition, technological advances offer new perspectives, particularly in the field of morphofunctional imaging. There are still many indications for CT and new applications can be expected. Possible drawbacks related to radiation dose and injection of contrast agents must be kept in mind.

[Imaging of COPD]. / Imagerie des BPCO.

Standard chest radiographs have been shown to be insensitive for the diagnosis of morphologic abnormalities of airways. Computed tomography is the most sensitive and specific investigation to diagnose emphysema. However, as emphysema may be missed on computed tomography, this investigation cannot be used to definitely rule out the diagnosis. Computed tomography may contribute to the investigation of bronchiolitis, and it is now considered as the gold standard for establishing the diagnosis of bronchiectasis. Imaging may contribute to identify complications such as bronchopulmonary infection, pulmonary hypertension, pneumothorax, cancer of the lung, compressive bullae, and pulmonary embolism.

Imaging of the non-traumatic brachial plexus.

The first line imaging of the non-traumatic brachial plexus is by MRI. Knowledge of the anatomy and commonest variants is essential. Three Tesla imaging offers the possibility of 3D isotropic sequences with excellent spatial and contrast enhancement resolutions, which leads to time saving and quality boosting. The most commonly seen conditions are benign tumor lesions and radiation damage. Gadolinium is required to assess inflammatory or tumour plexopathy. MRI data should be correlated with FDG-PET if tumor recurrence is suspected.