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Resume La maladie de Horton est la plus frequente des vascularites. Elle peut se compliquer par des accidents cardiovasculaires (infarctus du myocarde, accidents vasculaires cerebraux), mais leur incidence reelle et leur risque relatif par rapport a la population generale sont inconnus. Objectif. – Evaluer l'incidence des accidents cardiovasculaires dans une etude en double cohorte prospective de patients avec maladie de Horton par rapport a des temoins, apres ajustement sur les facteurs de risque cardiovasculaire. Patients et methodes. – Quatre cent trente-deux patients nouvellement diagnostiques ont ete inclus sur des criteres preetablis, et apparies par âge et par sexe a des temoins tires au sort dans la population generale. Les facteurs de risque cardiovasculaire majeurs etaient mesures a l'inclusion : tabagisme, maladie atheromateuse, diabete, hypertension arterielle, hypercholesterolemie. Les cas et les temoins ont ete suivis pendant 24 mois avec recueil de la survenue d'accidents cardiovasculaires : infarctus du myocarde, angor, accidents vasculaires cerebraux constitues ou transitoires, arteriopathie obliterante des membres inferieurs. La survenue d'accidents cardiovasculaires au cours du temps a ete etudiee en analyse univariee par un test du log-rank apres stratification sur les facteurs de risque cardiovasculaire et en analyse multivariee par un modele de survie parametrique. Resultats. – Les accidents cardiovasculaires sont globalement plus frequents chez les cas que chez les temoins (RR = 2,15 [1,21–3,81], p = 0,009) ; ils sont lies a l'âge (p = 0,0001), aux antecedents d'accidents cardiovasculaires (p = 0,023) et a la maladie de Horton (p = 0,009). Pris separement, les accidents neurologiques (RR = 2,42 [0,84–7]) et les accidents cardiaques (RR = 1,67 [0,72–3,89]) sont plus frequents sans que les ecarts soient cependant significatifs. Conclusion. – La mise en evidence d'un surrisque d'accidents cardiovasculaires en cas de maladie de Horton doit faire discuter l'adjonction d'un traitement antiagregant preventif au long cours.

Background: Chest physicians have a limited experience of primary pulmonary sarcomas, which represent a particular entity among rare intrathoracic neoplasms. Design: Retrospective review of medical records. Purpose: To study patients with primary sarcomas of the lung diagnosed in our pathology department in order to define their clinical characteristics, treatment, and prognosis. Patients: The study group consisted of 12 patients, with a mean age of 53 years. Results: The main symptoms were chest pain, and cough. Imaging findings consisted of: eight single peripheral opacities, three single parahilar opacities, and one lobar actelectasis. The histologic diagnoses confirmed in all cases by detailed immunohistochemical study were leiomyosarcoma (7), monophasic synovial sarcoma (2), one case each of malignant peripheral nerve sheath tumor (MPNST), epithelioid sarcoma, and malignant fibrous histiocytoma. Thoracic surgery done in nine cases consisted of six lobectomies with further parietal resection in two cases, and three pneumonectomies. Four patients received chemotherapy and two patients had radiation therapy postoperatively. Follow up available on 12 patients ranged from 3 to 144 (mean 42) months. Long term survival up to 3 years was observed in five patients. Median overall survival was 48 months. Overall 5-year survival rate was 38%. Conclusions: Primary sarcomas of the lung are a rare and aggressive malignancy. Treatment and prognosis do not differ from other soft tissue sarcomas.

A series of 90 thymic epithelial tumors were reviewed and classified by histopathologic characteristics into the three major categories (A, B, and C) recognized by the WHO schema. Each tumor type was correlated with patient characteristics and clinical data (age, sex, presence of myasthenia gravis, tumor size and invasiveness, and completeness of resection), and with outcome (survival, recurrence, and metastasis). All tumors were categorized by the WHO schema. Myasthenia gravis was present in 32 patients, mostly young and with type B thymic epithelial tumors. Tumors were invasive in 56% of cases, but resection was total in 67% of patients and only partial in the rest. Five factors were shown by univariate analysis to be associated with a favorable prognosis: presence of myasthenia gravis (p = 0.0389), younger age (p = 0.0022), completeness of resection (p = 0.0001), noninvasiveness (p = 0.0138), and tumor type A or B, as opposed to type C (p = 0.0001). Prognosis for types A and B was not significantly different, suggesting that the subtypes of types A and B thymic epithelial tumors should be regarded as a morphologic continuum rather than as distinct histologic variants.

Primary cardiac lymphoma is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium, extremely rare in immunocompetent patients, and more frequent in immunodepressed patients. We present 3 retrospectives cases of primary cardiac lymphoma in immunocompetent patients and review 35 cases reported in the literature. Two patients were adults and one was a child. Primary cardiac lymphoma presented with constitutional symptoms in two cases and superior vena caval syndrome in one case. Diagnosis of a tumor mass was made in all cases by transthoracic echocardiography. Primary cardiac lymphoma arose in the heart right chambers in two cases. Histological diagnoses, obtained after thoracotomy, were diffuse large B-cell lymphoma in two cases, and Burkitt's lymphoma in one case. All three cases received chemotherapy, combined with radiotherapy in one patient. Of our patients, 2 are alive and asymptomatic 12 months and 33 months after diagnosis. In conclusion, diagnosis of primary cardiac lymphoma is difficult due to non-specific clinical manifestations and should be considered in patients with a cardiac mass sometimes with pericardial effusion. It is confirmed using transthoracic echocardiography and magnetic resonance imaging and certified using cytology or open biopsy. The only effective treatment is chemotherapy, but prognosis remains poor.

Autosomal dominant hereditary amyloidosis with a unique cutaneous and cardiac presentation and death from heart failure by the sixth or seventh decade was found to be associated with a previously unreported point mutation (thymine to cytosine, nt 1389) in exon 4 of the apolipoprotein A1 (apoA1) gene. The predicted substitution of proline for leucine at amino acid position 90 was confirmed by structural analysis of amyloid protein isolated from cardiac deposits of amyloid. The subunit protein is composed exclusively of NH2-terminal fragments of the variant apoA1 with the longest ending at residue 94 in the wild-type sequence. Amyloid fibrils derived from four previously described apoA1 variants are composed of similar fragments with carboxyl-terminal heterogeneity, but contrary to those variants, which all carry one extra positive charge, the substitution Leu90Pro does not result in any charge modification. It is unlikely, therefore, that amyloid fibril formation is related to change of charge for a specific residue of the precursor protein. This is in agreement with studies on transthyretin amyloidosis in which no unifying factor such as change of charge for amino acid residues has been noted.

Objective To assess the role of cardiovascular risk factors, measured at the time of diagnosis, in the pathogenesis of giant cell (temporal) arteritis (GCA). Methods Four hundred new patients with GCA or polymyalgia rheumatica (PMR) and population-based, individually age- and sex-matched controls were included in this multicenter, prospective case-control study. Each participant was evaluated by review of the medical history and by clinical and laboratory assessments. Results Among women, smoking was associated with a 6-fold increase in risk (P = 0.00006, 95% confidence interval [95% CI] 2-17), heavy smoking with a 17-fold increase in risk, and previous atheromatous disease with a 4.5-fold increase in risk (P = 0.0003, 95% CI 2-11) of GCA in both the biopsy-positive and biopsy-negative GCA groups; only smoking appeared to be a risk factor for PMR in women (odds ratio 3.64, 95% CI 1.07-12.40). Among men, no risk factor was found to be significant. Conclusion Smoking and previous arterial disease were independently associated with GCA in women. In order to avoid matching bias, risk factors for diseases with an unbalanced sex distribution should be studied separately in each sex, using a sex-matched, case-control study design.

Nonspecific interstitial pneumonia/fibrosis (NSIP) has recently been individualized within the group of idiopathic interstitial pneumonias mainly based on a pathologic pattern of temporally uniform lesions distinct from usual, desquamative, and acute interstitial pneumonia. We studied 12 consecutive patients with NSIP at lung biopsy done as a diagnostic procedure for idiopathic interstitial lung disease. The patients were six males and six females, aged 52.5 +/- 11.8 yr. In 8 of 12 cases the pathologic lesions consisted of both cellular interstitial inflammation and fibrosis, whereas only cellular inflammation was present in three cases, and fibrosis in one. Dyspnea, cough, inspiratory crackles, and squeaks were the most common symptoms and signs. Six cases were cryptogenic. An associated disorder or a presumed cause was present in the other six patients, including underlying connective tissue disease (n = 3), organic dust exposure (n = 2), and prior acute lung injury (n = 1). Lung function tests found a restrictive ventilatory defect (11/12), impairment of TLCO (11/11), and hypoxemia at rest (7/12). Chest X-ray showed infiltrative opacities in all cases. Computed tomography of the chest in 11 cases mainly showed ground glass opacities (9/11), patchy areas of alveolar consolidation (6/ 11), and thickening of interlobular septas (5/11). All patients were treated with corticosteroids, and seven with immunosuppressive agents. All patients were alive at last follow-up, 50 +/- 40 mo after diagnosis. Ten patients (83%) were clinically improved or stabilized. Thus, despite its heterogeneity, NSIP deserves to be individualized as an original clinicopathologic entity and should be clearly distinguished from usual interstitial pneumonia, especially because of a better prognosis.

Objective. To assess the prevalence and thrombogenic role of anticardiolipin antibodies (aCL) in giant cell arteritis. Methods. Two hundred eighty-four patients with newly diagnosed temporal arteritis or polymyalgia rheumatica and 210 age- and sex-matched controls randomly selected from the general population were included in a multicenter, prospective case-control study. Blood samples were obtained at the time of diagnosis, data on initial clinical features were collected in a questionnaire, and temporal artery biopsy findings were reviewed by an experienced pathologist. Results. Anticardiolipin antibodies were present in 20.7% of patients compared with 2.9% of controls (P = 1.45 x 10 -9 ). The prevalence of aCL was higher in samples found positive for temporal arteritis than in those found negative on biopsy (31.2% versus 16.7%; P = 0.04), and was similarly higher in the biopsy-positive temporal arteritis group compared with the polymyalgia rheumatica and control groups. Although aCL were associated with thrombotic complications in univariate analysis, the positivity of the biopsy findings remained the only predictive variable in stratified analysis. Conclusion. In giant cell arteritis, aCL seem to function as reactive antibodies in relation to endothelial lesions.

We studied 70 patients with biopsy-proven pulmonary non-Hodgkin's lymphomas without extrathoracic involvement or mediastinal adenopathy to determine the clinical, imaging, and endoscopic features of this condition in a homogeneous series. In low-grade (LG) lymphomas, symptoms were cough, dyspnea, chest pain, hemoptysis. Imaging features consisted of localized alveolar opacities, infiltrative diffuse opacities, atelectasis, and pleural effusions. Inflammatory changes of the mucosa were present in some patients, leading to bronchial stenosis in 7; biopsies showed lymphomatous infiltration in 12. Prognosis of LG lymphomas was excellent, with 93.6 percent survival at five years. High-grade lymphomas differed from LG lymphomas principally by a more aggressive course and a worse survival. Inflammatory changes occurred in seven of nine cases leading to stenosis in two, and biopsies showed lymphomatous involvement in five. The profile of primary pulmonary lymphomas in this study could help clinicians consider this condition and prompt them to evaluate new diagnostic tools.

Among the patients showing evidence of cerebral metastases without previously known cancer history, lung cancer has been found 37 times as the primary tumor in our institution. There were 34 men and three women and all but two were heavy smokers. Only one presented at diagnosis with thoracic symptoms but the chest radiograph was abnormal in 34. The histologic type of the primary tumor was obtained in 32 cases as a result of thoracic investigations and in five cases from metastatic tumor tissue. The primary tumor appeared to be non-small cell lung carcinoma in 26 cases and small cell lung carcinoma in 11 cases. These results show that patients treated with surgery (20 cases) have a better survival (median 10 months versus 4.5) than the others, and among surgically treated patients only those treated with bifocal resection (eight patients) are long-term survivors. Also, in four of six patients, objective regression of the neurologic symptoms was seen after radiation therapy alone. Central nervous system relapse was seen in 12 patients, but in none of the patients treated with postoperative radiation therapy. Conventional chemotherapy (11 patients) induced objective responses only in the small cell type and proved to be too toxic when used simultaneously with radiation therapy in inoperable patients.

Objective To assess the prevalence and clinical significance of small-vessel vasculitis (SVV) surrounding an uninflamed temporal artery (TA) in patients diagnosed as having giant cell (temporal) arteritis (GCA) and/or polymyalgia rheumatica (PMR). Methods Patients with GCA and/or PMR (n = 490) were included in this multicenter prospective study. Slides of TA biopsy specimens were reviewed by 2 pathologists who were blinded with regard to clinical information. SVV was defined as aggregates of mononuclear inflammatory cells surrounding a capillary, distant from an uninflamed temporal artery. Clinical and biologic data of patients in the SVV group (n = 35) were compared with data of patients with biopsy-proven GCA (n = 280) and with negative TA biopsy findings (n = 175). Results SVV was diagnosed in 18 women and 17 men (mean ± SD age 74.5 ± 9.4 years). The group of patients with SVV had a higher proportion of men than in the entire GCA series, had systemic symptoms, headache, jaw claudication, and an abnormal temporal artery less frequently at clinical examination, but had symptoms of PMR more often than patients in the biopsy-proven GCA group (P = 2.6 × 10−7, odds ratio 9.17 [95% confidence interval 3.44–24.4]). Levels of inflammation markers were significantly lower in the SVV group. Patients in the SVV group had fever less frequently than patients in the group with negative TA biopsy findings, but otherwise shared the same clinical (including PMR symptoms) and biologic features. Eighteen of the 94 patients with pure PMR (19%) had SVV. Conclusion SVV is often neglected by pathologists, and appears to be strongly associated with PMR symptoms in patients with a clinical diagnosis of GCA and/or PMR. However, SVV as a new diagnostic criterion for PMR must be assessed in prospective studies.

Background:Permanent visual loss (PVL) is the most feared complication of giant cell arteritis (GCA), and its risk factors are still unclear.Objectives:The aim of our study was to assess the pathological features predictive of PVL on temporal artery biopsy (TAB) specimens in patients with GCA.Methods:The slides of 391 TAB specimens from patients with GCA were reviewed by two pathologists without clinical information.Results:A total of 29 patients (26 females and 3 males, mean age 78.3 years) presented with unilateral PVL at the onset of the disease, and 362 patients (258 females, 104 males, mean age 74.7 years), did not. The pathological features strongly predictive for PVL were the presence (p = 0.003), number (p = 0.001) and aggregates of giant cells (p = 0.001), presence of plasmocytes (p = 0.002), thickened intima (p = 0.007), neoangiogenesis (p = 0.001) and degree of arterial occlusion (p = 0.006). Presence of neutrophils, eosinophils, parietal necrosis, calcification in the arterial wall and disruption of the internal elastic membrane were similar in both groups. Total obstruction of the arterial lumen by a thrombus, intensity of the inflammatory cells infiltration and inflammation of small vessels, nerves and veins surrounding the temporal artery were not associated with blindness. In multivariate analysis, only giant cells remained significantly associated with PVL.Conclusion:Giant cells are strongly associated with PVL, with a significant gradient between great risk and large number of giant cells. However, PVL was neither associated with the intensity of the inflammatory infiltrate, nor with the presence of arterial thrombosis.

We studied 77 patients with biopsy-proven WG and pulmonary manifestations, to characterize the nature and frequency of the clinical, imaging and endoscopic features of this condition. Pulmonary symptoms were cough, mild dyspnea, hemoptysis and chest pain. Five patients had no pulmonary symptoms. Imaging features consisted of nodules, infiltrates and pleural opacities. A CT scan proved useful by disclosing cavities in opacities or opacities which were not seen on an x-ray film. Fiberoptic bronchoscopy was performed in 74 patients, and it was macroscopically abnormal in 55 percent (showing bronchial inflammation or stenosis or both or isolated hemorrhage). Six patients presented with alveolar hemorrhagic syndrome. Four patients had a pleural exudate rich in polymorphonuclear leukocytes. The WG was limited to the lung in seven patients. Sixteen patients died because of active disease or iatrogenic complications (two). An improved knowledge of clinical and imaging features of WG could help the clinician reach an earlier diagnosis.

International audience; OBJECTIVES To quantify and study the distribution of innervation of the left atrium and the pulmonary veins in humans. BACKGROUND Damage to cardiac nerves has been hypothesized as the explanation for successful radioftequency ablation of atrial fibrillation. METHODS From January 2003 to September 2003, histologic and quantitative studies of innervation of the left atrium and the pulmonary veins was performed in 43 consecutive necropsied adult hearts (30 men and 3 women; mean age 45.5 +/- 12.4 years). The left atrium was sectioned in 1-cm slices from left to right, with the plane of section perpendicular to the long axis of the heart. Sections of the pulmonary veins at their ostia and sections 1cm away of this structure also were obtained. Nerve fiber density was counted manually for each case and expressed as the mean number per slice. RESULTS Numerous epicardial nerve fibers and ganglia having distinct patterns of distribution in the left atrium were found. Nerve density was significantly higher at the ostia of the four pulmonary veins than in their distal part (7.1 +/- 2.1 vs 5.2 +/- 1.3 for left upper pulmonary vein; 6.3 +/- 1.5 vs 5.2 +/- 1.7 for right upper pulmonary vein; 7.4 - 2 vs 5.9 +/- 2 for left lower pulmonary vein; 6.7 +/- 1.8 vs 3.9 +/- 1.3 for right lower pulmonary vein). The left superior vein was significantly more innervated than the right inferior vein (12.3 +/- 3 vs 10.6 +/- 1.4). Gradients of innervation were found from right to left (9.8 +/- 4.6 vs 18.5 +/- 6.6, P < 05) and from the front to the rear of the atrium (17.2 +/- 6.4 vs 20.7 +/- 6.5, P < 05). The same heterogeneous distribution was observed at the myocardial level but with thinner nerve fibers, making quantification difficult. Only very thin nerve fibers were present in the endocardium. CONCLUSIONS The human left atrium exhibits several gradients of innervation at discrete sites. These findings may have clinical implications for radiofrequency ablation of atrial fibrillation.

Chest physicians have a limited experience of primary pulmonary sarcomas, which represent a particular entity among rare intrathoracic neoplasms.Retrospective review of medical records.To study patients with primary sarcomas of the lung diagnosed in our pathology department in order to define their clinical characteristics, treatment, and prognosis.The study group consisted of 12 patients, with a mean age of 53 years.The main symptoms were chest pain, and cough. Imaging findings consisted of: eight single peripheral opacities, three single parahilar opacities, and one lobar actelectasis. The histologic diagnoses confirmed in all cases by detailed immunohistochemical study were leiomyosarcoma (7), monophasic synovial sarcoma (2), one case each of malignant peripheral nerve sheath tumor (MPNST), epithelioid sarcoma, and malignant fibrous histiocytoma. Thoracic surgery done in nine cases consisted of six lobectomies with further parietal resection in two cases, and three pneumonectomies. Four patients received chemotherapy and two patients had radiation therapy postoperatively. Follow up available on 12 patients ranged from 3 to 144 (mean 42) months. Long term survival up to 3 years was observed in five patients. Median overall survival was 48 months. Overall 5-year survival rate was 38%.Primary sarcomas of the lung are a rare and aggressive malignancy. Treatment and prognosis do not differ from other soft tissue sarcomas.

[DOI:\hrefhttps://dx.doi.org/10.2214/ajr.178.4.178094910.2214/ajr.178.4.1780949] [PubMed:\hrefhttps://www.ncbi.nlm.nih.gov/pubmed/1190688011906880]

The process leading to irreversible fibrotic constriction of the bronchioles was studied in two cases of bronchiolitis obliterans (BO) after bone marrow transplantation. Because lysyl oxidase (LOX) is the main collagen cross-linking enzyme that might account for irreversible fibrosis, its expression was studied together with expression of extracellular matrix (ECM) proteins. Characteristic types of lesions could be distinguished on the basis of histological and immunohistological criteria. An inflammatory stage was characterised by infiltration restricted to the bronchioles by lymphocytes and dendritic cells. A fibro-inflammatory stage was characterised by the coexistence of a persistent immune cellular lesion pattern with further focal modelling of a sub-epithelial neo-synthesised connective matrix. LOX expression was observed at the tips of intra-luminal fibrotic protrusions, together with tenascin and cellular fibronectin. A fibrotic stage was characterised by dense ECM deposits spreading throughout the peri-bronchiolar connective tissue, resulting in bronchiole obliteration and final disappearance. In contrast to reversible cases of fibrosis, persistence of long-term LOX expression reflecting continuing fibrosing activity might account for the irreversible status of BO. Our two cases illustrated that, at inflammatory and fibro-inflammatory stages, BO may be stabilised by immunosuppressive treatment, while the persistence of LOX expression in the fibrotic stage might correspond to a disease that becomes irreversible and fatal.The process leading to irreversible fibrotic constriction of the bronchioles was studied in two cases of bronchiolitis obliterans (BO) after bone marrow transplantation. Because lysyl oxidase (LOX) is the main collagen cross-linking enzyme that might account for irreversible fibrosis, its expression was studied together with expression of extracellular matrix (ECM) proteins. Characteristic types of lesions could be distinguished on the basis of histological and immunohistological criteria. An inflammatory stage was characterised by infiltration restricted to the bronchioles by lymphocytes and dendritic cells. A fibro-inflammatory stage was characterised by the coexistence of a persistent immune cellular lesion pattern with further focal modelling of a sub-epithelial neo-synthesised connective matrix. LOX expression was observed at the tips of intra-luminal fibrotic protrusions, together with tenascin and cellular fibronectin. A fibrotic stage was characterised by dense ECM deposits spreading throughout the peri-bronchiolar connective tissue, resulting in bronchiole obliteration and final disappearance. In contrast to reversible cases of fibrosis, persistence of long-term LOX expression reflecting continuing fibrosing activity might account for the irreversible status of BO. Our two cases illustrated that, at inflammatory and fibro-inflammatory stages, BO may be stabilised by immunosuppressive treatment, while the persistence of LOX expression in the fibrotic stage might correspond to a disease that becomes irreversible and fatal.

OBJECTIVE: To determine the type of lesions observed in young patients infected with human immunodeficiency virus-1 (HIV-1). DESIGN: Examination of coronary networks in corpses of 13 men and two women who had died aged 23-32 years after having been infected with HIV-1 virus, having been seropositive for 2-5 years. Causes of death were infectious complications (five cases), infection with cytomegalovirus leading to gastro-intestinal haemorrhaging (one case), infection with cytomegalovirus and Kaposi's sarcoma (one case), overdoses of drugs (five cases) and sudden death (three cases). METHODS: The pathological analysis was carried out on the proximal and distal coronary networks. In order to characterize the lesions better, the cells and the cytokines involved were characterized by immunohistochemistry. RESULTS: In all 15 cases we observed thickening of intima in the proximal network at least as great as that of the media, caused by a proliferation of secreting cells, phenotypically identified as smooth muscle cells, with exaggerated production of elastic fibres and in association with an increase in the expression of tumor necrosis factor-alpha and interleukin-1 alpha. In nine cases, atherosclerosis had developed from and on the surface of this proliferation and in four cases arteriosclerosis had an unusual appearance, in the form of mamillated vegetations with endoluminal protrusions. A similar proliferation was found in the distal network in four cases, but with a significantly smaller proportion of elastic fibres. CONCLUSIONS: The lesions we examined in these young HIV-1-infected patients presented particular features and were intermediate between the lesions observed during common coronary atherosclerosis and atherosclerosis associated with chronic rejection of cardiac transplants.

The association between giant cell arteritis and thyroid dysfunction remains controversial, but as giant cells are a possible feature of Graves' disease, a common pathway has been suggested. In two series of 101and 98patients, the prevalence of hyperthyroidism was reported to be six times higher in cases of giant cell arteritis than in controls. 1 2 This was not confirmed on smaller series, 3 4 but 15cases of hypothyroidism were reported in 31patients with giant cell arteritis.5 We conducted a multicentre case-control study on cases of giant cell arteritis to investigate this relation. Assuming a prevalence of thyroid dysfunction of 1% in the general population and an odds ratio of 6for hyperthyroidism in the patient group, the sample size requested, with α=0.05 and β=0.2, had been estimated …

Sheep lung adenomatosis is a contagious tumor of the lung. It is present in all continents [1, 2], it can be reproduced by intratracheal inoculation of tumor tissue or fluid and it is caused by a retrovirus (Jaagsiekte sheep retro-virus) that is endogenously present in sheep.

Between July 1st 1988 and Dec 31th 1995, 112 patients presenting terminal pulmonary and infectious pulmonary disease received lung transplants at Louis Pradel. In recent years we have published data concerning functional improvement due to transplantation in Primary Pulmonary Hypertension, Pulmonary Sepsis and Emphysema, etc. We have also addressed lung preservation, viral infection and the use of the Gianturco metal stent in bronchial complications. The aim of the current study is to establish 3 year survival data in 95 patients having received 100 transplants between 1988 and 1994.

International audience; This study of two cases of pulmonary Wegener's granulomatosis (WG) focuses on the ultrastructural aspects of the vascular wall injury and on the immunohistochemical characterization of the perivascular connective matrix. The iterative waves of endothelial cell necrosis and regeneration are demonstrated by the multilamellar appearance of the basal lamina. Neutrophils infiltrate the vessel wall and myofibroblasts are recruited to injured vessels. The perivascular connective matrix associates basement-membrane like and fibrillar material with fibrin deposits. The initiation of the fibrosing process was assessed by the visualization of matrix molecules involved in targeting (p-fibronectin), organizing (cellular fibronectin and tenascin) and stabilizing (lysyl-oxidase) the fibrogenic activity. These elementary lesions affect different levels of the vascular tree, and capillaritis is involved in the extension of the pathological process. Lysyl-oxidase labelling reveals the fibrosing front which is located on the border of dense fibrosis. The markers of fibrosing activity disappear in the areas of fibrosis following vasculitis and/or ischaemic necrosis and/or granulomatosis. Vasculitis plays a major role in both the genesis and progression of the fibrosis observed in the late stage of WG.This study of two cases of pulmonary Wegener's granulomatosis (WG) focuses on the ultrastructural aspects of the vascular wall injury and on the immunohistochemical characterization of the perivascular connective matrix. The iterative waves of endothelial cell necrosis and regeneration are demonstrated by the multilamellar appearance of the basal lamina. Neutrophils infiltrate the vessel wall and myofibroblasts are recruited to injured vessels. The perivascular connective matrix associates basement-membrane like and fibrillar material with fibrin deposits. The initiation of the fibrosing process was assessed by the visualization of matrix molecules involved in targeting (p-fibronectin), organizing (cellular fibronectin and tenascin) and stabilizing (lysyl-oxidase) the fibrogenic activity. These elementary lesions affect different levels of the vascular tree, and capillaritis is involved in the extension of the pathological process. Lysyl-oxidase labelling reveals the fibrosing front which is located on the border of dense fibrosis. The markers of fibrosing activity disappear in the areas of fibrosis following vasculitis and/or ischaemic necrosis and/or granulomatosis. Vasculitis plays a major role in both the genesis and progression of the fibrosis observed in the late stage of WG.

A group of 32 lung (single lung, [n = 14] and double lung [n = 1]) or heart-lung (n = 17) transplant recipients were studied with serial high-resolution computed tomography (HRCT) scans and transbronchial biopsies from the time of surgery. These investigations were carried out routinely every 2 weeks for the first 2 months, every 2 months for a year, every 4 months in the second year, and on any clinical suspicion of acute lung rejection or infection. A total of 190 transbronchial biopsy specimens and concurrent HRCT scans were obtained. Forty (21%) of the biopsy specimens, showed histologic evidence of lung rejection, 111 (58%) were normal, and 39 (21%) were not conclusive. The more frequent HRCT pattern encountered during an acute rejection episode was the presence of patchy "ground-glass" density areas (65%). This finding was sparsely observed during minimal and mild acute rejection episodes. Using histologic diagnosis as a standard for acute rejection, ground-glass opacities on HRCT had a sensitivity of 65% in detecting lung rejection. Although ground-glass opacities were also intermittently observed during cytomegalovirus pneumonia (14%), this finding had a specificity of 85% for detecting occurrence of an acute lung complication. The detection of ground-glass opacities on lung HRCT after lung transplantation, more particularly after the first month after surgery, can aid the decision of when and where to undertake transbronchial lung biopsy.

Thoracic splenosis (post-traumatic autotransplantation of splenic tissue) is rare and generally asymptomatic. We report a patient with thoracic splenosis presenting with repeated hemoptysis. The blood supply of the hypervascular splenic transplants originated from a bronchial and an intercostal artery. Hemoptysis improved after surgical exeresis of splenosis. Recognizing splenosis presenting with hemoptysis is important, since percutaneous embolotherapy could be hazardous because of the risk of ectopic splenic tissue infarction.

We report one case of biopsy-proved large cell arteritis with polyneuropathy and intestinal involvement leading in 15 days to mesenteric ischemia, small bowel perforation, multivisceral failure and death in spite of the administration of high-dose steroids, symptomatic treatment and abdominal surgery.

Bronchiolitis obliterans organizing pneumonia (BOOP) is a pathologic finding common to various injuries to the lung of either definite or idiopathic etiology. Since the presentation of patients with idiopathic BOOP varies, we studied 16 patients with BOOP on pulmonary histology to define more distinct and homogeneous clinical and imaging profiles of idiopathic BOOP. We distinguished three groups of patients: group 1 (n=4), with multiple patchy migratory pulmonary involvement of the pneumonia type. Their clinical course was subacute, with cough, fever, weight loss, mild dyspnea, and increased ESR. Chest x-ray film and CT scan showed multiple alveolar opacities. All patients completely recovered with corticosteroid therapy but relapsed when therapy was stopped too rapidly. Group 2 (n=5) had solitary pulmonary involvement of the pneumonia type occurring in a similar clinical context. Since carcinoma was suspected, they underwent surgical excision of the pneumonic area and recovered without relapse. Group 3 patients (n=7) presented with diffuse pulmonary involvement of the interstitial lung disease type. They had more progressive onset of more severe dyspnea, crackles heard over all lung surfaces, and interstitial opacities with or without alveolar opacities on chest imaging. Improvement with corticosteroid therapy was obtained in only three patients. In all three groups, lung function test results showed a restrictive pattern. The obstructive pattern characteristic of pure bronchiolitis obliterans was found in none. BAL showed a mixed pattern (increase of both lymphocytes and polymorphonuclear cells) in the patients of the first two groups. Thus, we distinguished three characteristic clinical and imaging profiles in patients with idiopathic BOOP: multiple patchy pneumonia, solitary pneumonia, and diffuse interstitial lung disease. These profiles are so different that they should be distinguished in clinical studies of idiopathic BOOP.

Twenty one cases of amyloidosis of the lower respiratory tract were seen at a single center. In three patients, multifocal bronchial amyloid plaques led to stenosis and atelectasis, and in two, small pseudotumor masses were an incidental bronchoscopic finding. Two patients had nodular parenchymal amyloidosis, in one of whom the lesions were progressive and in the other static. Fifteen patients had diffuse parenchymal amyloidosis. Two of these had severe interstitial involvement and died in respiratory failure; eight had congestive cardiac failure, and parenchymal amyloidosis was a post-mortem finding; two had senile cardiorespiratory amyloidosis, also found at autopsy; and in three, the amyloidosis was associated with malignancy. The degree of respiratory embarrassment seemed to be related to the amount of amyloid in the gas diffusion zones, irrespective of the etiology of amyloidosis.

Treize malades atteints d'endocardite infectieuse comportant une localisation tricuspidienne sont etudies. Il s'agit de 7 hommes et 6 femmes âges de 7 mois a 85 ans. Le germe responsable est isole 10 fois et le streptocoque n'est en cause que dans 2 cas. La localisation tricuspidienne est revelee dans 11 cas par l'autopsie, dans 1 cas par l'intervention salvatrice et dans 1 cas par le bilan hemodynamique apres guerison clinique. Dans 4 observations, l'atteinte tricuspidienne est isolee, sur cœur anteieurement sain; la porte d'entree de l'infection, toujours iatrogene, est evidente; iln'y a pas de defaillance hemodynamique liee aux degâts valvulaires, et le pronostic defavorable dans 3 cas depend de l'infection irreductible ou d'une cause myocardique intercurrente. Dans 2 observations, l'endocardite infectieuse touche les deux valves du cœur droit, se developpe sur une cardiopathie congenitale, une fois a l'occasion du post-partum, et evolue vers l'insuffisance cardiaque conduisant a la chirurgie dans 1 cas et au deces dans l'autre. Dans 7 observations, l'endocardite tricuspidienne s'associe a une atteinte mitro-aortique (4 cas), aortique (2 cas) ou mitrale (1 cas). La porte d'entree est indeterminee dans 5 cas, mais dans 6 cas il existe un shunt gauche-droit intracardiaque congenital. L'evolution, constamment defavorable, est sous la dependance des delabrements valvulaires gauches par le biais d'une insuffisance cardiaque irreductible (6 cas) ou d'une embolie systemique (1 cas).

L'etude retrospective presentee cherche a preciser les caracteres cliniques, electrocardiographiques et evolutifs de l'insuffisance aortique (IA) compliquant la spondylarthrite ankylosante (SA) ou le syndrome de Reiter (FLR). Douze observations d'IA par SA ont ete recueillies de 1970 a 1981 parmi 524 IA pures (10 cas = 1,9 p. 100), parmi 373 SA dont 9 FLR (2 cas = 0,5 p. 100) et parmi 36 FLR sans SA (0 cas). Dans 2 cas, les lesions inflammatoires ont ete objectivees par l'etude histologique des sigmoides aortiques. Dans 10 cas, toutes les autres etiologies possibles d'IA ont ete eliminees. - Tous les malades sont de sexe masculin. - Les symptomes articulaires inaugurent l'affection dans 10 cas a un âge moyen de 32,7 ans. L'atteinte est axiale et peripherique dans 6 cas (dont 2 FLR), uniquement axiale dans 6 cas. Les lesions vertebrales et sacro-iliaques sont radiologiquement constantes. L'antigene HLA B27 est retrouve 3 fois sur 5. Une restriction ventilatoire est objectivee 5 fois sur 7. - L'IA est decouverte a un âge moyen de 55 ans; dans 10 cas, 3 a 47 ans apres la SA et dans 2 cas, 1 a 2 ans avant. Huit IA sont initialement asymptomatiques et 4 sont revelees par un trouble hemodynamique ou conductif. Sur 7 IA suivies en moyenne 10 ans en phase prehospitaliere, on note 4 aggravations progressives, 1 aggravation brutale (EI) et 2 stabilisations. Lors du bilan cardiologique, l'insuffisance ventriculaire gauche (IVG) est constante (NYHA II: 4 cas; III: 6 cas; IV: 2 cas). Cliniquement, l'IA est majeure dans 8 cas et moderee dans 4 cas. Les voltages ventriculaires gauches (VG) et le rapport cardio-thoracique sont eleves dans 10 cas. L'echographie TM (6 cas) montre une dilatation des diametres VG dans 6 cas, une reduction de la fraction d'ejection dans 3 cas et une dilatation moderee de la racine de l'aorte dans 4 cas. L'angiographie preoperatoire (5 cas) objective toujours une fuite aortique massive et, dans 2 cas, une alteration de la contractilite VG. - Un trouble de la conduction auriculo-ventriculaire (BAV) est observe dans 8 cas, 3 fois apres la decouverte de l'IA, 2 fois avant et 3 fois simultanement; 3 BAV du 1er degre sont stables; 5 BAV se completent progressivement (3 syncopes; 2 IVG) et sont appareilles. - 5 malades sont asymptomatiques 2 ans en moyenne apres un remplacement valvulaire aortique; 3 malades decedent subitement (1 cas) ou en IVG (2 cas) dans les 2 ans qui suivent les premiers symptomes d'intolerance; 4 malades sont stabilises (3 stade II, 1 stade III) sous traitement medical 6 ans apres la decouverte de l'IA. - L'IA complique exceptionnellement et habituellement de facon tardive la SA. - Il s'agit souvent de fuites graves, car evolutives et susceptibles de se decompenser spontanement ou a l'occasion d'une greffe infectieuse. - L'adjonction tres frequente de troubles de la conduction auriculo-ventriculaire evolutifs semble tres specifique et evocatrice de cette etiologie particuliere d'IA, en raison de la rarete des BAV dans les IA d'autres origines et dans les SA sans IA (5/195 ECG soit 2,6 p. 100). - La symptomatologie rhumatologique n'offre aucune particularite et la restriction ventilatoire n'a pas alourdi le risque operatoire dans cette serie.

Mesenchymal cystic hamartoma of the lung is a rare clinicopathological entity (1), characterized by a cystic pulmonary lesionwith typical pathological features, that slowly increases in size. Fewer than 15 cases have been reported in the literature. Cowden’s disease, or the multiple hamartoma syndrome, is an autosomal dominant tumor syndrome with involvement of various organs. Clinically, it is characterized by multiple hamartomas of ectodermal, endodermal, and mesodermal origin, a high incidence of malignant tumors of the breast, thyroid gland, and female adnexa together with gastrointestinal polyps, various mucocutaneous lesions, macrocephaly, and mild-to-moderate mental retardation (2). Our present observation suggests that Cowden’s disease may include mesenchymal cystic hamartoma of the lung.

Presented is a summary of our experience with 157 patients having a diagnosis of epidermoid cyst, glottic sulcus, or mucosal bridge of the true vocal cord. Each patient in this group was diagnosed and treated jointly by a phoniatrist and microlaryngoscopist and then evaluated for results of surgical and phoniatric therapy. Included in the discussion are methods of examination and diagnosis, microanatomy and histopathology of the lesions, methods of treatment, and results of treatment. An argument for a common etiology for these lesions is advanced.