[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.

[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.

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.

[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.

Alveolar lymphocytosis in patients with chronic uveitis: relationship to sarcoidosis.

Bronchoalveolar lavage (BAL) is frequently performed in patients with suspected ocular sarcoidosis. This study describes the immunogenetical, immunological, radiological, and functional features of a subclinical alveolar lymphocytosis unrelated to sarcoidosis in patients with chronic uveitis. Two hundred and ten patients with chronic uveitis of unknown origin, who underwent fiber-optic bronchoscopy with BAL as part of a prospective protocol over a three-year period, were evaluable for retrospective analysis. Sixty-five patients had alveolar lymphocytosis: Sarcoidosis was diagnosed in 13 (6%) patients, whereas alveolar lymphocytosis was considered unrelated to sarcoidosis in 52 (25%). Alveolar lymphocytosis unrelated to sarcoidosis was not associated with radiologically detectable interstitial lung disease or pulmonary function impairment. CD4/CD8 lymphocyte ratio was 3.7 +/- 3.0. Total cell count, total lymphocyte, and CD4 lymphocyte percentage were significantly lower when compared with sarcoidosis-related alveolitis (129,000 +/- 80,000 vs. 218,000 +/- 117,000, p <0.05; 33.1% +/- 13.2 vs. 39.7% +/- 13.2, p <0.05; and 54.3% +/- 18.2 vs. 65.4% +/- 10.1, p <0.05, respectively). Patients with alveolar lymphocytosis unrelated to sarcoidosis were older (47.8 +/- 17.7 years vs. 42.7 +/- 14.2 years, p <0.05) and more likely to carry the HLA-B51 allele (19.7% vs. 7.1%, p <0.01) than patients with chronic uveitis without alveolar lymphocytosis. They did not appear to be at risk of developing clinically apparent interstitial lung disease on followup. We conclude that alveolar lymphocytosis is frequently observed in patients with chronic uveitis. It is generally unrelated to sarcoidosis and may then be associated with a distinctive immunogenetic phenotype.

Pancreatic involvement in von Hippel-Lindau disease. The Groupe Francophone d'Etude de la Maladie de von Hippel-Lindau.

BACKGROUND & AIMS: Pancreatic involvement in von Hippel-Lindau (VHL) disease, a genetic disorder with a dominant mode of inheritance affecting various organs, has rarely been studied. We assessed the prevalence, type of lesions, natural history, and impact of pancreatic involvement in patients with VHL. METHODS: A total of 158 consecutive patients from 94 families with VHL disease were studied in a prospective French collaborative study. All patients underwent systematic screening for VHL lesions, including computerized tomography (CT) scanning of the pancreas reviewed by an experienced radiologist. Clinical data, investigations, and treatments performed were also reviewed. RESULTS: Pancreatic involvement was observed in 122 patients (77.2%) and included true cysts (91.1%), serous cystadenomas (12.3%), neuroendocrine tumors (12.3%), or combined lesions (11.5%). The pancreas was the only organ affected in 7.6% of patients. Patients with pancreatic lesions had fewer pheochromocytomas than those without (14/122 vs. 16/36; P<0.0001), and patients with neuroendocrine pancreatic tumors had renal involvement less often than those without (8/99 vs. 6/20; P = 0.013). None of the patients with neuroendocrine tumors had symptoms of hormonal hypersecretion. Pancreatic lesions evolved in half of patients but required specific treatment in only 10 (8.2%) when they were symptomatic or for the resection of large neuroendocrine tumors. CONCLUSIONS: Pancreatic involvement is seen in most patients with VHL disease. Although symptoms are rare, specific treatment of pancreatic lesions is required in selected patients, mainly those with neuroendocrine tumors.

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.

[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.

Association of small cell lung cancer and the anti-Hu paraneoplastic syndrome: radiographic and CT findings.

OBJECTIVE: The anti-Hu syndrome (bearing the name of the patient in whom the antibody was first discovered) is characterized by a paraneoplastic encephalomyelitis or sensory neuronopathy and the presence of a specific antibody in the serum or CSF and is associated with lung cancer in most patients who have the syndrome. The objective of this study was to determine the relative merits of chest radiography and CT in the imaging of patients with the anti-Hu syndrome. MATERIALS AND METHODS: Chest radiographic and CT findings of 11 patients with anti-Hu syndrome were reviewed and correlated with results of bronchoscopy, surgery, or autopsy. RESULTS: Ten of the 11 patients had small cell lung cancer; in the remaining patient, no cancer was found. Small cell lung cancer was first revealed on radiographs in one patient and solely on CT in the remaining nine patients with small cell lung cancer. In six of these nine patients, the initial CT findings were positive for lung cancer. Tumor was found on follow-up CT in 4-8 months when initial CT findings were negative. Mediastinal adenopathy was present in all 10 cancer patients. Hilar adenopathy was present in four. Parenchymal involvement was seen in three of the 10 patients with cancer. In two patients, the only CT finding of small cell lung cancer was one mediastinal lymph node of 10 mm in each patient. CONCLUSION: Chest CT should be recommended for patients with anti-Hu syndrome, even when chest radiographic findings are interpreted as normal.

Bronchiectasis: comparative assessment with thin-section CT and helical CT.

PURPOSE: To compare thin-section computed tomography (CT) and helical CT in the detection and assessment of the extent of bronchiectasis. MATERIALS AND METHODS: Both thin-section and helical CT scans were obtained in 50 consecutive patients with clinical symptoms suggestive of bronchiectasis. Thin-section CT was performed with 1.5-mm collimation and 10-mm intervals, and helical CT was performed with 3-mm collimation and a pitch of 1.6 during a 24-second breath hold. Three observers evaluated 593 segments on CT scans both independently and in consensus. Radiation dose was measured for both techniques. RESULTS: Bronchiectasis was noted in 77 segments (22 patients) on thin-section CT scans compared with 90 segments (26 patients) on helical CT scans. No findings were positive for bronchiectasis on only thin-section CT scans. Interobserver agreement was statistically significantly better (P < .05) in identification of segments that were positive for bronchiectasis on helical CT scans (kappa = 0.87) than on thin-section CT scans (kappa = 0.71). Total skin dose of radiation delivered with helical CT was 3.4 times greater than that delivered with thin-section CT. CONCLUSION: Helical scanning can improve CT depiction of bronchiectasis but with an increase in radiation exposure to the patient. It should be used in patients considered for surgery or for thin-section CT scans that are difficult to interpret.

[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.

[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.

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.

Late CD8+ lymphocytic alveolitis after allogeneic bone marrow transplantation and chronic graft-versus-host disease.

Late-onset interstitial pneumonitis following allogeneic bone marrow transplantation (BMT) is a rare condition usually caused by a variety of infective agents, although in some cases these are idiopathic. We investigated noninfectious late interstitial pneumonitis with lymphocytic alveolitis in seven allogeneic BMT recipients using bronchoalveolar lavage (BAL), lymphocyte phenotyping analysis, CT lung scans, and pulmonary function tests. The results were compared with those of a control group composed of similar patients with no pulmonary symptoms. Of 65 long-term survivors, seven were included in the study. All had chronic graft-versus-host disease (GVHD) and developed interstitial pneumonitis a median of 210 d (range 120 to 445 d) after BMT. BAL revealed lymphocytosis, with an overall expansion of CD8+ subsets (38 to 90%). Lymphocytic alveolitis was not observed in the control group. Pulmonary function tests revealed a restrictive syndrome, and biopsy samples obtained from 2 patients showed interstitial lymphoid infiltration with fibrosis of the alveolar walls. Of the 7 patients, six were cured by starting immunosuppressive drugs or increasing the dosage with a drastic improvement in respiratory symptoms within 1 mo. These findings suggest that CD8+ alveolitis may be observed in late interstitial pneumonitis in allogeneic BMT recipients and may be a pulmonary manifestation of chronic GVHD.

[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.

[High resolution computed tomography in the study of the respiratory tract]. / La tomodensitométrie en haute résolution dans l'étude des voies aériennes.

High resolution computed tomography (CT) is an imaging technique of choice for the evaluation of most lesions of the bronchial tree, particularly those affecting the small airways. This technique can confirm the diagnosis of bronchiectasis with a high degree of sensitivity and specificity and can contribute to the diagnosis of bronchiolitis (particularly diffuse panbronchiolitis and bronchiolitis obliterans) as well as other inflammatory diseases of the airways. CT can also reliably detect obstructive bronchial lesions such as tumours or broncholithiasis and can provide key information useful for the diagnosis of bronchial fistula, dehiscence or bronchial rupture, in addition to the study of numerous congenital pulmonary malformations. This article describes the computed tomography techniques recommended in each clinical situation and review the computed tomographic signs of all diseases of the airways.