Rheumatological evaluation of patients with interstitial lung disease.

Objective: Interstitial lung disease (ILD) is a common feature of connective tissue disease (CTD). The diagnosis of CTD-ILD can be challenging and is important for therapeutic decisions. In this study, we aimed to determine whether a systematic rheumatological assessment could help pulmonologists in the diagnosis and care of ILD patients.Method: We conducted an observational single-centre study of patients with ILD. All patients underwent standardized pulmonary and rheumatological evaluations, including clinical evaluation (pulmonary symptoms and musculoskeletal signs), immunological screening, chest high-resolution computed tomography, pulmonary function tests, and ultrasonography (US) of joints and major salivary glands.Results: We included 100 consecutive ILD patients (47% women, mean ± sd age 67 ± 14 years); 15 patients already had CTD. The main extrapulmonary symptoms were joint pain (n = 52), joint swelling (n = 26), and sicca syndrome (n = 33). US of joints revealed synovitis, bone erosion, and tenosynovitis in 37, 17, and 13 patients, respectively. US of major salivary glands detected features associated with Sjögren's syndrome in 13 patients. After rheumatological evaluation, CTD-ILD was confidently diagnosed in 39 patients; diseases were mainly rheumatoid arthritis (n = 20), primary Sjögren's syndrome (n = 17), and inflammatory myopathies (n = 7). The diagnosis of CTD-ILD was associated with the presence of musculoskeletal symptoms and immunological and US abnormalities. The CTD diagnosis led to a therapeutic change in 21 patients.Conclusion: Our findings suggest that musculoskeletal symptoms are frequent in ILD patients, which supports multidisciplinary management, involving the rheumatologist, for evaluating patients with ILD.

Lung ultrasonography in patients with COVID-19: comparison with CT.

AIM: To determine whether findings from lung ultrasound and chest high-resolution computed tomography (HRCT) correlate when evaluating COVID-19 pulmonary involvement. MATERIALS AND METHODS: The present prospective single-centre study included consecutive symptomatic patients with reverse transcription polymerase chain reaction (RT-PCR)-proven COVID-19 who were not in the intensive care unit. All patients were assessed using HRCT and ultrasound of the lungs by distinct operators blinded to each other's findings. The number of areas (0-12) with B-lines and/or consolidations was evaluated using ultrasound and compared to the percentage and classification (absent or limited, <10%; moderate, 10-25%; extensive, 25-50%; severe, 50-75%; critical, >75%) of lung involvement on chest HRCT. RESULTS: Data were analysed for 21 patients with COVID-19 (median [range] age 65 [37-90] years, 76% male) and excellent correlation was found between the ultrasound score for B-lines and the classification (p<0.01) and percentage of lung involvement on chest HRCT (r=0.935, p<0.001). In addition, the ultrasound score correlated positively with supplemental oxygen therapy (r=0.45, p=0.041) and negatively with minimal oxygen saturation at ambient air (r=-0.652, p<0.01). CONCLUSION: The present study suggests that among COVID-19 patients, lung ultrasound and HRCT findings agree in quantifying lung involvement and oxygen parameters. In the context of the COVID-19 pandemic, lung ultrasound could be a relevant alternative to chest HRCT.

[Low-dose methotrexate: Indications and side effects, particularly in cases of diffuse interstitial pneumonia]. / Méthotrexate à faible dose : indications et effets secondaires, en particulier en cas de pneumopathie interstitielle diffuse.

INTRODUCTION: Methotrexate (MTX) is a folate antagonist used as an immunosuppressant in a number of conditions, including rheumatoid arthritis (RA). Low-dose MTX (MTX-LD) is associated with a risk of haematological, hepatic, gastrointestinal and pulmonary toxicity, which may up until now have limited its use. STATE OF THE ART: In RA, data from retrospective cohorts have reported a possible excess risk of methotrexate toxicity in cases of underlying interstitial lung disease (ILD). However, recent prospective and retrospective multicentre studies have found no such increased risk, and have reassuringly concluded that MTX-LD can be prescribed in cases of RA-associated ILD (RA-ILD). PERSPECTIVES AND CONCLUSIONS: Current recommendations are not to delay the introduction of MTX in patients with RA at risk of developing ILD or in the presence of RA-ILD with mild to moderate respiratory impairment.

[Lung imaging in severe asthma]. / Imagerie de l'asthme sévère.

INTRODUCTION: Asthma is a common disease whose diagnosis does not typically rely on the results of imaging. However, chest CT has gained a key place over the last decade to support the management of patients with difficult to treat and severe asthma. STATE OF THE ART: Bronchial wall thickening and mild dilatation or narrowing of bronchial lumen are frequently observed on chest CT in people with asthma. Bronchial wall thickening is correlated to the degree of obstruction and to bronchial wall remodeling and inflammation. Diverse conditions which can mimic asthma should be recognized on CT, including endobronchial tumours, interstitial pneumonias, bronchiectasis and bronchiolitis. Ground-glass opacities and consolidation may be related to transient eosinophilic infiltrates, infection or an associated disease (vasculitis, chronic eosinophilic pneumonia). Hyperdense mucous plugging is highly specific for allergic bronchopulmonary aspergillosis. PERSPECTIVES: Airway morphometry, air trapping and quantitative analysis of ventilatory defects, with CT or MRI, can help to identify different morphological subgroups of patients with different functional or inflammatory characteristics. These imaging tools could emerge as new biomarkers for the evaluation of treatment response. CONCLUSION: Chest CT is indicated in people with severe asthma to search for additional or alternative diagnoses. Quantitative imaging may contribute to phenotyping this patient group.

[Haemothorax revealing thoracic endometriosis]. / Hémothorax spontané révélant une endométriose pleurale.

INTRODUCTION: Thoracic endometriosis (TE) is a rare disorder affecting women during their reproductive years. Manifestations of TE include pneumothorax and haemothorax. Treatment is based on surgical and hormonal therapy that aims at eradicating existing endometrial thoracic plaques and to prevent reseeding from pelvic endometriosis. CASE REPORT: We report the case of a 36 year-old young woman presenting thoracic endometriosis revealed by a recurring spontaneous, large and isolated right haemothorax. Diagnosis, pathogeny and treatment are discussed. CONCLUSION: Thoracic endometriosis needs to be considered as a cause of haemothorax in women of childbearing age.

Rituximab therapy in autoimmune pulmonary alveolar proteinosis.

Idiopathic pulmonary alveolar proteinosis is presumed to be an autoimmune disorder that may lead to pulmonary insufficiency. However, steroids do not appear to be effective and the standard of therapy is whole-lung lavage. We report the first case of successful therapy with rituximab, which addresses the pathogenic mechanism of pulmonary alveolar proteinosis.

Diffuse interstitial pneumonia and pulmonary hypertension: a novel manifestation of chronic granulomatous disease.

The present authors report the case of an adult with chronic granulomatous disease who developed an unusual lung fibrosis associated with severe pulmonary hypertension. Histological analysis of a lung biopsy showed a diffuse infiltration with pigmented macrophages without granulomas, which particularly involved the pulmonary arterial and venular walls. Clinical and histological findings were suggestive of pulmonary veno-occlusive disease. Such a clinical association has not been previously described in the literature and might be due to the persistent expression of gp91phox at a very low level. In conclusion, the present case report illustrates a novel manifestation of chronic granulomatous disease.

[Endoscopic lung volume reduction for emphysema]. / Réduction de volume pulmonaire endoscopique dans l'emphysème.

Lung hyperinflation which is a hallmark of advanced emphysema plays a major role in the exertional dyspnoea experienced by patients. This has led to the development of surgical lung volume reduction which, though effective, is also associated with significant morbidity and mortality. The goal of endoscopic lung volume reduction which has developed over several years is to decrease hyperinflation without exposing patients to the risks of surgery. Several endoscopic techniques have been assessed by high quality controlled studies: airway by-pass, instillation of glue, insertion of coils or unidirectional valves, vapour ablation. The aim of this review is to present the results of these studies in terms of functional benefit and side effects. Based on these studies, an algorithm for the endoscopic management of advanced forms of emphysema is proposed.

[Niemann-Pick disease type B identified following an episode of bronchopneumonia]. / Maladie de Niemann-Pick de type B révélée par une atteinte bronchopulmonaire.

Niemann Pick disease type B (NPD type B) is a rare autosomal recessive lipid storage disorder, characterized by a partial deficiency of sphingomyelinase. We report the case of an adult male patient affected by NPD type B and diagnosed at 39-years-of age. Pulmonary CT scan revealed a cranio-caudal gradient with nodular centrilobular ground glass opacities and thickening of the interlobular septa. Pathological examination of the bronchoalveolar lavage showed foamy alveolar macrophages and vacuolated bronchial epithelial cells on bronchial biopsy. Diagnostic confirmation was achieved by a decrease in cell lysosomal enzyme activity and by the presence of the homozygous DeltaR608 mutation in the acid sphingomyelinase gene (SMPD1).

[Respiratory manifestations during the course of Sjogren's syndrome]. / Manifestations respiratoires au cours du syndrome de Gougerot-Sjögren.

INTRODUCTION: Sjogren's syndrome is a common auto-immune disease. BACKGROUND: Clinically significant pulmonary involvement affects approximately 10% of patients and may be the first manifestation of the disease, putting the respiratory physician in a position to suspect and confirm the diagnosis. Besides interstitial lung disease and bronchial disorders, cough is a common symptom of the disease and particularly difficult to treat. Lung cysts and amyloid deposits, sometimes associated with lymphoma, have recently been described. The development of a primary pulmonary lymphoma, usually from MALT, is a major complication of the disease. VIEWPOINT: Characterisation of the pathophysiology of pulmonary involvement in Sjogren's syndrome and the institution of specific treatment merits the interest of the respiratory physician. CONCLUSION: The respiratory physician should consider the diagnosis of Sjogren's syndrome in many different clinico-pathological situations.

[Haemolytic uremic syndrome and acute mesenteric ischemia caused by interferon-alpha-2b in the treatment of Kaposi's sarcoma in an AIDS patient]. / Syndrome hémolytique et urémique et ischémie mésentérique aiguë lors d'un traitement par interféron alpha-2b pour une maladie de Kaposi associée au VIH.

BACKGROUND: Interferon alpha is approved for the treatment of Kaposi's sarcoma in HIV infected patients. Hemolytic and uremic syndrome (HUS) is a rare side-effect of interferon alpha and has been reported primarily in chronic myelogenous leukemia. CASE REPORT: A 44-year-old HIV-infected woman from Cameroon was admitted for treatment of cutaneous Kaposi's sarcoma. Two days later, she presented severe HUS. Abdominal pains subsequently revealed non-occlusive mesenteric ischemia. The patient rapidly improved after interferon withdrawal. DISCUSSION: To our knowledge this is the first case of HUS induced by interferon alpha given for Kaposi's sarcoma. Further, no cases of acute mesenteric ischemia have been reported with interferon alpha. It is possible that the condition may have been induced or aggravated by HUS or a low infusion rate. Interferon can exert vascular toxicity on both the mesenteric vessels and the renal vessels in a setting of microangiopathy.

[Idiopathic interstitial pneumonias in 2016]. / Actualités dans les pneumopathies interstitielles idiopathiques en 2016.

Idiopathic interstitial pneumonias comprise 8 clinicopathological entities, most of them with a chronic course and various prognosis. Idiopathic pulmonary fibrosis is the most frequent and most severe of these. Computed tomography has an important role for its diagnosis. It can identify the corresponding pathological pattern of usual interstitial pneumonia in about 50 percent of cases. It can suggest differential diagnosis in other cases, most frequently fibrosing nonspecific interstitial pneumonia and chronic hypersensitivity pneumonitis. Imaging features should be integrated to clinical and available pathologic data during multidisciplinary team meetings involving physicians with a good knowledge of interstitial diseases. Some cases may be unclassifiable, but these could later be reclassified as new data may occur or imaging features may change. Surgical lung biopsy is being less frequently performed and an emerging less invasive technique, lung cryobiopsy, is under evaluation. Pleuroparenchymal fibroelastosis is a distinct entity only recently described, with uncertain prevalence and prognosis that seems being quite often associated to another pattern of interstitial pneumonia.

Contribution of magnetic resonance imaging in lung cancer imaging.

Lung cancer is the leading cause of cancer death worldwide. Prognosis and treatment outcomes are known to be related to the disease stage at the time of diagnosis. Therefore, an accurate assessment of the extent of disease is critical to determine the most appropriate therapy. Currently available imaging modalities for diagnosis and follow-up consist of morphological and functional imaging. Morphological investigations are mainly performed with CT-scan and in some cases with MRI. In this review, we describe the contribution of MRI in lung cancer staging focusing on solid pulmonary nodule characterization and TNM staging assessment using chest and whole-body MRI examinations, detailing in each chapter current recommendations and future developments.

[Mediastinal fat necrosis]. / Nécrose de la graisse médiastinale.

Epicardial fat necrosis is a rare cause of benign chest pain. Its physiopathological mechanism is unknown. Diagnosis is easily performed through radiological investigations that show a round opacity of fat density limited by a dense pseudo-capsule in the anterior mediastinum, close to the heart.

Failing arteriovenous hemodialysis fistulas: assessment with magnetic resonance angiography.

RATIONALE AND OBJECTIVES: The authors sought to evaluate prospectively magnetic resonance angiography (MRA) versus fistulography in the detection and characterization of complications associated with malfunctioning hemodialysis access fistulas (arteriovenous fistulas [AVF]). METHODS: Nineteen patients with clinical AVF dysfunction were studied by MRA and fistulography. Data from each study were collected prospectively and analyzed in a blinded manner. RESULTS: The main diagnosis was stenosis in eight patients, thrombosis in five patients (mural thrombosis with preserved flow in one), aneurysm without stenosis in two patients, and normal AVF in four patients. A hazy flow void, assumed to be related to turbulence, was observed in normal arterial anastomoses. When flow void was considered as a criterion of stenosis or thrombosis, one false-positive and one false-negative MRA study were determined, yielding a sensitivity and specificity of 92% and 86%, respectively. CONCLUSIONS: Magnetic resonance angiography is a feasible and sensitive technique with which to portray suspected malfunctioning hemodialysis access fistulas.

MR imaging of coarctation of the aorta and its postoperative complications in adults: assessment with spin-echo and cine-MR imaging.

To evaluate the ability of ECG-gated spin-echo (SE)-MR imaging vs. cine-MR imaging to assess coarctation of the aorta, 11 patients aged 15-45 years, with known or suspected coarctation of the aorta, and five patients suspected of re-stenosis or postoperative false-aneurysms after coarctation repair were examined by multisection SE-MR imaging and single-section multiphase cine-MR imaging on a 1.0 Tesla device. Aortography was performed in 15, and surgery in 14 of these 16 patients. Qualitatively, the location, severity, and length of the coarctation were shown in all cases with MR imaging, as well as the relationship with the arteries arising from the aortic arch. The respective sensitivities and specificities in the assessment of severity of stenoses were 86% and 100% for SE MR images, and 100% and 100% for cine-MR images. Cine-MR imaging was superior to SE imaging in stenosis diameter assessment with contrast angiography as reference, as well as to identify the site of leakage in cases of postoperative pseudoaneurysm. Pre-stenotic dilation or post-stenotic aneurysm, collateral channels, and associated malformations were better identified on SE images. Quantitatively, a better stenosis diameter correlation was found between cine-MR images and angiography than between SE-MR images and angiography (r=0.99 vs. r=0.78; p=0.001 vs. p=0.004), related to overestimation of stenoses with SE-MR imaging. The use of a combination of spin-echo and cine-MR imaging correlates well with conventional angiographic findings in this small series of patients with coarctation of aorta or postoperative pseudoaneurysmal complications. Cine-MRI can provide anatomic information that is equivalent to angiography for surgical planning.