pulmonary manifestation of LES

RATIONALE: Patients with autoimmune lymphoproliferative syndrome (ALPS), a disorder of impaired lymphocyte apoptosis, often undergo radiographic chest imaging to evaluate the presence and progression of lymphadenopathy. These images often lead to parenchymal and interstitial lung findings of unclear clinical significance. OBJECTIVES: To characterize the pulmonary findings associated with ALPS and to determine if lung abnormalities present on computed tomographic (CT) imaging of the chest correlate with infection or functional status...

OBJECTIVE: To evaluate the clinical manifestations, long-term clinical outcome and longitudinal pulmonary function in a large cohort of Spanish patients with anti-Jo1 antibodies. METHODS: We retrospectively analyzed the clinical data and lung function parameters of 148 anti-Jo1 patients recruited from a multicentre registry including 18 Spanish hospitals. A composite endpoint was defined, comprising death due to respiratory failure directly related to antisynthetase syndrome (ASS), the need for long-term oxygen therapy or lung transplantation...

The Author remarks the interstitial lung involvement in systemic lupus erythematosus. This secondary respiratory manifestation is infrequent as well as the consequent pulmonary hypertension making it possible to miss or delay the diagnosis. Therefore the interdisciplinary evaluation of the multisystemic disease lupus erythematosus needs. In this context the role of the pneumologists is relevant for the global treatment of the patients with LES in particular as concerns the early detection of the clinical and functional respiratory symptoms as well as the appropriate treatment plan within their specialistic competence...

BACKGROUND: Mutations in the telomerase complex (TERT and TR) are associated with pulmonary fibrosis and frequent hematologic manifestations. The aim of this study was to characterize the prognosis of lung transplantation in patients with TERT or TR mutations. METHODS: Patients with documented TERT or TR mutations who received a lung transplant between 2007 and 2013 in France were identified via an exhaustive search of the lung transplantation network, one expert genetic laboratory, and the clinical research network on rare pulmonary diseases...

OBJECTIVE: To report the experience in three Brazilian institutions with the use of rituximab in patients with different clinical forms of lupus erythematosus systemic in activity. METHODS: The study consisted of a sample of 17 patients with LES, who were already being treated, but that at some stage of the disease showed refractory symptoms. The patients were subdivided into groups according to the clinical manifestation, and the responses for the use of rituximab were rated as complete, partial or no response...

BACKGROUND: Comparisons of lung manifestations in primary pulmonary vs disseminated nontuberculous mycobacterial disease have not been well described. The clinical, histopathologic, and radiologic disease manifestations of primary pulmonary or disseminated nontuberculous mycobacterial disease were compared in an autopsy series. METHODS: Medical and microbiologic records, autopsy reports, histopathologic slides of the lungs, and chest CT scans were reviewed on patients at the National Institutes of Health with nontuberculous mycobacterial disease who died between 1996 and 2010...

OBJECTIVE: The pediatric interstitial lung disease is a group of poorly understood disease entities. This study aimed to better understand the clinical features, radiological manifestations and pathological patterns of pediatric interstitial lung disease. METHOD: Patients with diffuse lung disease seen in the year 2009 in 7 hospitals were studied by the Pediatric Interstitial Lung Disease Cooperative Group. Nineteen patients underwent lung biopsy, 11 cases were male, 8 were female and their age ranged from 1 year and 4 months to 13 years...

OBJECTIVE: Connective tissue diseases (CTDs) are associated with several interstitial lung diseases. The aim of this study was to describe the recently individualized syndrome of combined pulmonary fibrosis and emphysema (CPFE) in a population of patients with CTD. METHODS: In this multicenter study, we retrospectively investigated data from patients with CTD who also have CPFE. The demographic characteristics of the patients, the results of pulmonary function testing, high-resolution computed tomography, lung biopsy, and treatment, and the outcomes of the patients were analyzed...

Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder characterized by epistaxis, telangiectasia, and visceral vascular manifestations. Infectious and ischemic central nervous system (CNS) manifestations due to embolism through pulmonary arteriovenous malformations (PAVMs) represent the main causes of morbidity. To improve the phenotypic characterization of HHT with PAVM, we conducted a retrospective multicenter study of patients with HHT and at least 1 PAVM detected by chest computed tomography (CT) and/or pulmonary angiography, with particular attention to CNS and infectious manifestations...

The priming of bronchiolitis obliterans organising pneumonia by radiation therapy (RT) to the breast is now a well recognised syndrome. This study describes the occurrence of chronic eosinophilic pneumonia following RT after surgery for breast cancer in five female patients, with a mean age of 68 yrs (range 49-77). All patients had a history of asthma and/or allergy. At the onset of eosinophilic pneumonia, all patients were symptomatic. Chest radiograph showed pulmonary infiltrates, unilateral and limited to the irradiated lung in three patients, and bilateral in two...

Gastroesophageal reflux (GER) is a common situation that can express with digestive, extra-digestive, respiratory or otolaryngologic symptoms. Some chronic pulmonary disorders include in their setting GER as well. This review will address pathogenesis, clinical signs, complications and treatment of GER with a special focus towards the pulmonologist field. GER is a physiological post-prandial phenomenon of limited duration. It is induced by transient lower esophageal sphincter relaxation (tLESR) or by factors that impede LES function by reducing its tone or disrupting its contractions...

Pulmonary alveolar proteinosis (PAP) is a rare, diffuse infiltrative lung disease of unknown etiology characterized by accumulation of abnormal lung surfactant in the alveoli and distal airways. Although it was first described in 1958, few large scale studies have been published. The diagnosis is conventionally based on lung biopsy and the value of less invasive procedures such as bronchoalveolar lavage (BAL) remains controversial. A multi-center retrospective study was conducted in an attempt to update epidemiological, clinical and long term outcome and to assess the usefulness of chest CT scan and BAL in the management of a large homogeneous series of 41 patients...

Microscopic polyangiitis (MPA) is a systemic small-vessel vasculitis primarily associated with necrotizing glomerulonephritis and pulmonary capillaritis. In this retrospective study of 29 patients with MPA and alveolar hemorrhage (AH), we characterized the pulmonary manifestations at presentation and assessed the short- and long-term outcome. AH was diagnosed when bronchoalveolar lavage was macroscopically bloody, or contained hemosiderin-laden macrophages, in the absence of lung infection or pulmonary edema...

Pulmonary lymphangioleiomyomatosis (LAM) is a rare disorder of unknown cause characterized by peribronchial, perivascular, and perilymphatic proliferation of abnormal smooth muscle cells leading to cystic lesions. The hypothesis of hormonal dependence and the effectiveness of hormonal therapy have not yet been demonstrated conclusively, and the prevalence of extrathoracic manifestations and the survival of patients with LAM are somewhat contradictory. A multicentric retrospective study was conducted in an attempt to describe better the initial features, the diagnostic procedures, the associated lesions, and, above all, the management and course of LAM in a large homogeneous series of 69 stringently selected patients, with a majority of cases diagnosed since 1990...