A delayed immune reconstitution inflammatory syndrome.
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Adult↗
Biomedical subjects
Michel Brauner
Publications and source records attributed to Michel Brauner.
Pulmonary cystic disorder related to light chain deposition disease.
Light chain deposition disease (LCDD) is a rare disorder that very uncommonly affects the lung. We report three cases of severe cystic pulmonary LCDD leading to lung transplantation. Such a presentation has never been previously reported. The three patients present with a progressive obstructive pulmonary pattern associated with numerous cysts diffusely distributed in both lungs. The disease was histologically characterized by non-amyloid amorphous deposits in the alveolar walls, the small airways and the vessels. It was associated with emphysematous-like changes and small airway dilation. Monotypic kappa light chain fixation was demonstrated on the abnormal deposits and along the basement membranes. Electron microscopy revealed coarsely granular electron-dense deposits in the same localizations. Mild extrapulmonary deposits were found in salivary glands in one patient. No immunoproliferative disorder was identified. We conclude that LCDD may primarily affect the lung, present as a pulmonary cystic disorder, and lead to severe respiratory insufficiency.
Adult↗
Magnetic resonance images of diaphragmatic endometriosis treated by polyglactin mesh.
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Adult↗
[Churg-Strauss syndrome and pulmonary fibrosis: an unusual association].
INTRODUCTION: Churg-Strauss syndrome (CSS) is characterized by asthma, hypereosinophilia, and vasculitis involving at least two extrapulmonary organs. CASE: We report a case of a patient with antineutrophilic cytoplasmic antibody-negative CSS who developed pulmonary interstitial fibrosis (PIF). DISCUSSION: The possible relations between CSS and PIF are discussed. Because this case report is the first to describe features of pulmonary fibrosis in a patient with CSS, we cannot know whether this association is causal or fortuitous.
Aged↗
[Interstitial lung disease in systemic sclerosis].
Interstitial lung diseases (ILD) associated with systemic sclerosis (SSc) are mainly encountered in patients with diffuse disease, although they may also be seen in patients with limited cutaneous SSc. ILD screening must be performed regularly, with high-resolution computed tomography and pulmonary function tests (TLCO). Up to 75% of patients with diffuse SSc develop a form of ILD. ILD remains stable in most patients and does not worsen. The nonspecific nature of SSc-associated ILD makes it different from idiopathic ILD and helps to explain its better prognosis. Nonetheless, ILD is one of the two leading causes of death in SSc patients. Treatment of SSc-associated ILD is not yet well codified. Antifibrotic treatments have not proved beneficial, and the efficacy of cyclophosphamide, which has been used to treat this condition for 15 years, has been shown to be very limited against SSc-associated ILD. A subgroup of patients with rapidly progressive ILD might benefit from intravenous cyclophosphamide pulses in association with 15 mg/d prednisone.
Anti-Inflammatory Agents↗
Sinonasal involvement in sarcoidosis: a case-control study of 20 patients.
We conducted a retrospective single-center study to describe the clinical features of sinonasal sarcoidosis (SNS) and to determine whether SNS is associated with a particular clinical phenotype of sarcoidosis. Twenty patients with histologically proven SNS (men/women, 7/13; mean age, 32 +/- 9 yr) were compared with control patients with sarcoid but without sinonasal (SN) involvement. Each patient was matched with 2 controls for the date of admittance in our institution. SN involvement occurred in the course of previously known sarcoidosis in 8 patients, whereas it preceded disease diagnosis in 12 patients. Among these 12 patients, 4 initially presented with strictly isolated SNS and 8 had other associated signs related to sarcoidosis. The most common symptoms were stuffiness (90%), anosmia (70%), and rhinorrhea (70%). Lupus pernio was frequent (50%). Local examination was constantly abnormal and showed hypertrophy (75%) and purplish coloring of the nasal mucosa with granulations (50%) on the septum and/or inferior turbinates. Computed tomography scans showed medial lytic lesions, mainly of the septum and/or the turbinates in about half the cases. All patients had negative antineutrophil cytoplasmic antibodies. Patients with SNS had significantly more frequent and severe involvement of vital organs than controls, had a longer history of sarcoidosis, and required systemic treatment more frequently (100% vs. 57.7%, p < 0.001) and for a longer time (78 +/- 42 mo vs. 29 +/- 18 mo, p < 0.0001). Corticosteroids maintenance dosage was high (10.5 +/- 6 mg daily) and mainly depended on SN involvement. Although rare, SN involvement is a severe and recalcitrant manifestation of sarcoidosis representing a therapeutic challenge.
Adult↗
Increased uptake of 111In-octreotide in idiopathic pulmonary fibrosis.
UNLABELLED: Idiopathic pulmonary fibrosis (IPF) is characterized by an uncontrolled accumulation and activation of lung fibroblasts. A modulation of fibroblast activation has been observed in various systems with octreotide, a synthetic somatostatin analog with strong affinity for the somatostatin receptor subtype 2 (sst2). One aim of our study was to evaluate the expression of somatostatin receptors in the lungs of patients with IPF. A second aim was to evaluate the relationship between 111In-octreotide uptake and the effect of pulmonary fibrosis as assessed by lung function tests and parameters and by radiologic findings. METHODS: We investigated 11 patients with IPF, 6 patients with pulmonary fibrosis associated with systemic sclerosis (SSc), and 19 patients with disease not of the lung (control patients). The expression of somatostatin receptors was evaluated in vivo using 111In-octreotide scintigraphy. We evaluated the relationship between 111In-octreotide uptake and the activity of pulmonary fibrosis as assessed by lung function tests, bronchoalveolar lavage (BAL) cellularity, and high-resolution CT (HRCT) of the chest. Planar images and thoracic SPECT (24 h) were performed after injection of 222 MBq of 111In-octreotide. Lung uptake was quantified using the lung-to-background ratio (L/B). In addition, the expression of sst2 was evaluated in vitro, in frozen lung-tissue samples using autoradiography, and in human cultures of lung fibroblasts using a ligand-binding assay. RESULTS: Compared with lung uptake in control patients (median L/B, 1.25; range, 1.14-1.49), lung uptake was increased in all 11 IPF patients (median L/B, 2.63; range, 1.59-3.13; P < 0.001) and in 4 of 6 SSc patients (median L/B, 1.68; range, 1.42-2.16). The L/B was lower in SSc patients than in IPF patients (P = 0.011). Increased uptake correlated with the alteration of lung function (carbon monoxide diffusing capacity [rho = -0.655; P = 0.038], diffusing capacity for carbon monoxide and alveolar volume ratio [rho = -0.627; P = 0.047], vital capacity [rho = -0.609; P = 0.054], and total lung capacity [rho = -0.598; P = 0.058]) and with the intensity of alveolitis (total BAL cellularity [rho = 0.756; P = 0.045], neutrophil counts [rho = 0.738; P = 0.05]), and HRCT fibrosis score (rho = 0.673; P = 0.007). Autoradiography suggested that vascular structures were a prominent binding site. Lung fibroblasts expressed somatostatin receptors in vitro as measured by binding assay. CONCLUSION: Our preliminary results identified an increased expression of sst2 in (mainly idiopathic) pulmonary fibrosis. Lung uptake correlates with the alteration of lung function and with the intensity of alveolitis.
Adult↗
[Pathophysiology of interstitial lung disease].
Chronic interstitial lung disease (ILD) groups a number of diseases with the common feature of radiological pulmonary infiltration, typical functional syndrome, and diffuse involvement of the deep pulmonary parenchyma identified histologically. Correlations between histological and radiological findings have enabled progress in both fields, leading to better interpretation of the radiological findings and optimizing the etiological diagnosis. Besides the signs themselves, their distribution in relation to the normal lung structures is highly contributive. Function tests can be used to quantify the impact on the respiratory system and assess the effect of treatment. Evidence-based criteria will progressively replace the consensual criteria enabling more effective evaluation of treatment in difficult pathological conditions such as idiopathic pulmonary fibrosis.
Humans↗
[Interstitial lung disease in connective tissue disorders].
Interstitial lung diseases (ILD) associated with connective tissue disorders differ from idiopathic ILD in several aspects, although most of them are comparable. In most patients, ILD occurs during the course, or at the time of diagnosis of connective tissue disease. Opportunistic pulmonary infections, together with adverse effects of treatment should always be discussed. The prevalence of ILD varies among the different connective tissue disorders. Thus, ILD is frequently encountered in patients with systemic sclerosis and to a lesser degree in patients with myositis. As compared to idiopathic ILD, histopathological aspects of ILD associated with connective tissue diseases are more frequently those of non-specific interstitial pneumonia, whereas usual interstitial pneumonia is rare. Other ILD, such as organized pneumonia, interstitial lymphoid pneumonia, diffuse alveolar damage and alveolar hemorrhage are occasionally encountered. ILD must be detected early in the course of collagen disorders by performing computed tomodensitometry and pulmonary function tests. The prognosis of connective tissue associated ILD is better than that of idiopathic ILD. The treatment requires corticosteroids and/or immunosuppressants, depending on the nature of the associated connective tissue disease and ILD progression.
Adrenal Cortex Hormones↗
Endoluminal stenosis of proximal bronchi in sarcoidosis: bronchoscopy, function, and evolution.
AIM: Endoluminal stenosis of proximal bronchi (ESPB) is a potentially severe manifestation of sarcoidosis. Unusual clinical presentation and variable response to medical treatment require specific attention to diagnosis and follow-up. DESIGN: Of 2,500 patients with sarcoidosis seen at our institution, we retrospectively identified 18 patients with stage 1-3 sarcoidosis and ESPB. Clinical manifestations, endoscopic findings, pulmonary function tests, follow-up, and therapeutic response were assessed. RESULTS: Respiratory symptoms were present in 17 patients (94%): cough and dyspnea (89% each), wheezing (83%), and hemoptysis (11%). Generalized symptoms (67%) and extrapulmonary manifestations (72%) of sarcoidosis were frequent. Three bronchoscopic patterns were observed: single stenosis (n = 3), multiple stenoses (n = 12), or diffuse narrowing of the bronchial tree (n = 3). The two former groups accounted for 45 ESPBs located in the left upper lobe (44.5%), the right upper and middle lobes (15.5% each), and the left lower lobe (11%). ESPBs were due to mural thickening of bronchi (n = 16) or associated with extrinsic compression by lymphadenopathy (n = 2). Endobronchial biopsies uniformly confirmed the presence of granulomas. FEV(1)/FVC ratio was < 70% in 12 patients (66.7%), with a correlation between the decrease of FEV(1)/FVC ratio and the number of ESPBs (R(2) = 0.31; p = 0.02). Patients treated with oral corticosteroids (n = 12) or methotrexate (n = 1) within the first 3 months had a good prognosis, whereas patients in whom treatment was delayed by > 3 months (n = 4) or who did not receive any systemic treatment (n = 1) acquired fixed ESPB and persistent ventilatory defects. CONCLUSIONS: ESPB is a rare and serious complication of sarcoidosis. Its clinical hallmarks include multiple respiratory symptoms, multiorgan involvement, and generalized symptoms. Treatment has to be started early to avoid the development of fixed stenotic lesions and irreversible pulmonary function impairment.
Administration, Oral↗
Ground-glass computed tomography pattern in chronic beryllium disease: pathologic substratum and evolution.
Five cases of chronic beryllium disease with predominant or isolated diffuse ground-glass lung opacities on computed tomography are reported with correlation to lung pathology. The ground-glass attenuation was either homogeneous and isolated (n = 1) or patchy and clearly predominant (n = 4) over linear and nodular opacities. In 4 cases, histologic samples showed a prominent diffuse involvement of the alveolar-capillary walls by florid granulomas. Evolution under corticosteroid was remarkable by the appearance of small-sized cysts in 3 cases.
Adrenal Cortex Hormones↗