In this retrospective study, we analyzed 17 patients presenting with pulmonary fibrosis and a positive ANCA testing. This group was compared with a control group of 12 patients with IPF and negative ANCA testing. Patients were 15 males and 2 females, with a mean age of 66 years. Eight patients ...

Lung disease has become the leading cause of mortality and morbidity in scleroderma (SSc) patients. The frequency, nature, and progression of interstitial lung disease seen on high-resolution CT (HRCT) scans in patients with diffuse SSc (dcSSc) compared with those with limited SSc (lcSSc) has not been well characterized. Baseline HRCT ...

BACKGROUND: Only limited data exist on the relationship of lung function to patients with extreme obesity. To assess the relationship between lung function tests and clinical characteristics in a cohort of morbidly obese patients undergoing evaluation for bariatric procedures in a university hospital in the United States. METHODS: Consecutive patients ...

OBJECTIVE: The objective of this study was to characterize the clinical features of patients with Niemann-Pick disease type B and to identify efficacy end points for future clinical trials of enzyme-replacement therapy. METHODS: Fifty-nine patients who had Niemann-Pick disease type B, were at least 6 years of age, and manifested ...

BACKGROUND: Systemic sclerosis (SSc) is a vasculopathy with increased tissue deposition of collagen. The aetiology is unknown. Genetic and environmental susceptibility factors have been implicated. It is unknown whether disease presentation varies within Europe. AIMS AND METHODS: The baseline data of all SSc patients entered in the EULAR Scleroderma Trials ...

Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is increasingly recognized in older patients. The differences in disease presentation and outcome between older and younger patients remain controversial. We conducted the current study to analyze the characteristics of patients aged over 65 years with AAV and to compare the younger and older ...

The aim of this study is to evaluate the presence of antibodies to carbonic anhydrase I and/or II (ACAI and ACAII) in patients affected by connective tissue diseases (CTD) and to investigate their association with lung involvement evaluated by High resolution CT scan (HRCT). Ninety-six patients affected by CTD were ...

OBJECTIVE: To define the risk factors associated with a relatively rapid increase in estimated pulmonary arterial systolic pressure (PASP) in patients with systemic sclerosis (SSc). METHODS: SSc patients undergoing screening for pulmonary arterial hypertension (PAH) by echocardiography were identified and their charts were retrospectively reviewed. In all patients, we recorded ...

High-resolution CT scan (HRCT) and its score have an important role in delineating pathological changes and pulmonary functional impairment in patients with bronchiectasis. To assess pulmonary function tests (PFTs) in patients with cystic and cylindrical bronchiectasis. To correlate HRCT score with PFTs and systolic pulmonary artery pressure (SPAP) in both ...

BACKGROUND: Air cysts or blebs in the lungs may predispose pulmonary barotrauma (PBT) by causing air trapping when there is a change in environmental pressure. The changes in the environmental pressure are also seen during hyperbaric oxygen treatments (HBOT). AIM: The aim of this study was to determine how patients ...

Combined interstitial lung disease and the presence of 'mechanic's hands' could be a clinical clue in the early diagnosis of the rare disease, amyopathic antisynthetase syndrome for which anti-Jo-1 antibody is a useful diagnostic tool. The case is reported of a patient who suffered from shortness of breath and dry ...

Systemic sclerosis (SSc) is a relatively rare chronic connective tissue disease characterized by varying degrees of skin fibrosis and visceral organ involvement. Pulmonary compromise, including pulmonary arterial hypertension and interstitial lung disease, is currently the leading cause of death in patients with SSc. Digital ulcers are common complications which lead ...

INTRODUCTION: Development of pulmonary artery hypertension (PAH) worsens the prognosis of systemic sclerosis (SSc). There is paucity of data on PAH in patients with SSc in India. We have attempted to determine the prevalence and predictors of pulmonary artery hypertension in systemic sclerosis using noninvasive cardiopulmonary evaluation. OBJECTIVES: (1) To ...

OBJECTIVE: Behçet's disease (BD) is a multisystem disorder characterized by vasculitis, and consists of a triad of recurrent ulcers of the oral and genital mucosa with relapsing uveitis. The prevalance of pulmonary involvement varies in the range of 1-10% in various studies and its complications are severe and life threatening. ...

BACKGROUND/AIMS: Legal pulmonary disability in Korea is decided for chronic respiratory patients who have been diagnosed for a year or more, and the patients haven't gotten better after more than 2 months of sufficient treatment and they have shown no change in their pulmonary function within the two years after ...

The purpose of this study was to assess the radiographic features of pulmonary fusariosis, an increasingly encountered cause of severe opportunistic mold pneumonia. Pulmonary fusariosis has radiographic manifestations that are suggestive of an angioinvasive mold. Nodules or masses were the most common findings at CT, seen in 82% of patients ...

OBJECTIVE: To estimate predictors and long-term outcome of interstitial lung disease (ILD) in patients with polymyositis (PM) and dermatomyositis (DM). METHODS: We conducted a prospective study in which newly diagnosed PM/DM patients, regardless of clinical symptoms of pulmonary disease, were investigated with repeated chest radiography, high-resolution computed tomography (HRCT) of ...

OBJECTIVE: To examine pulmonary function, dyspnea and exercise capacity in adult Saudi sickle cell disease (SCD) patients. METHODS: The patients were recruited from the hematology clinic at King Khalid University Hospital in Riyadh from January to December 2005. The study involved 39 patients with stable SCD (20 women and 19 ...

In cases of chronic pulmonary tuberculosis (PTB) with negative sputum smears, particularly when they are symptomatic, physicians encounter problems in differentiating the active from the inactive stage of the disease. This study was undertaken to determine the usefulness of technetium-99m hexakis methoxy isobutyl isonitrile ((99m)Tc-MIBI) pulmonary scintigraphy and lactic dehydrogenase ...

Fluid therapy in patients with pulmonary disease is challenging. Although a single set of rules cannot be applied to every patient, the following guidelines can be used when managing patients with pulmonary disease. Euvolemic patients with adequate tissue perfusion should be given sufficient isotonic fluid to balance insensible losses. If ...

OBJECTIVE: To investigate the association between serum levels and clinical signs of lung fibrosis in patients with systemic sclerosis and inflammatory myopathies. METHODS: ELISA tests for a mucin-like glycoprotein KL-6, von Willebrandt factor (vWF), soluble E-selectin (sES) and surfactant protein D (SP-D) were performed in sera of 104 patients with ...

The abnormality of DNA methylation is involved in tumour progression, and thus has a modulating effect on clinical outcome of cancer patients. In this study, we measured the mRNA expression levels of three methylation-regulating genes (DNMT1, DNMT3b, and MBD2) in 148 tumour samples from patients with non-small cell lung cancer ...

BACKGROUND AND OBJECTIVE: A new interpretative strategy for pulmonary function tests (PFT) has been proposed by the American Thoracic Society/European Respiratory Society (ATS/ERS) Task Force. To assess the accuracy of this strategy, clinical diagnosis was compared with the PFT interpretation in patients showing concomitant decreases in FEV(1) and FVC. METHODS: ...

Pulmonary hypertension (PH) adversely affects outcome in sarcoidosis and is an important predictor of mortality in these patients. Early and accurate diagnosis of this complication may improve outcome. The current authors hypothesised that integration of 6-min walk test (6MWT) as part of the evaluation leads to an earlier diagnosis of ...

BACKGROUND: To evaluate the colonisation rate and type in different groups of patients with chronic lung diseases, bronchial lavage (BL) fluid was investigated for bacteria. METHODS: All patients underwent fibre-optic bronchoscopy as part of routine investigation for remote haemoptysis or nodule investigation. The standard procedure included BL and microbiological culture ...

Lung involvement is the leading cause of morbidity and mortality in systemic sclerosis (SSc; scleroderma), and interstitial lung disease (ILD) is the most common pulmonary manifestation. An abnormal profibrotic Th2/Tc2-polarized T cell response is postulated to mediate tissue damage and fibrosis. The aim of this study was to investigate whether ...

BACKGROUND: Pulmonary fibrosis in systemic sclerosis (SSc) involves inflammatory processes in the lower respiratory tract. Analysis of exhaled breath condensate (EBC) is a non-invasive method for studying inflammatory mediators, such as cytokines, which are of interest from both physiological and therapeutic perspectives. The aim of this study was to assess ...

Patients with large left to right shunts as a result of congenital heart disease can develop changes in pulmonary vasculature that are initially reversible. It is critical in these patients to determine whether closure of the defect would reverse some of the changes in the pulmonary vasculature. A comprehensive clinical ...

BACKGROUND: Dyspnea and functional limitation in interstitial lung diseases (ILD) are not always adequately explained by the degree of compromise in pulmonary function alone. Pulmonary hypertension (PH) is felt to be a major contributor to morbidity and mortality in these patients. It is not clear whether treatment with newer vaso-active ...

BACKGROUND: Recent studies have shown strong evidence that bronchiolitis obliterans is the major long-term sequelae of exposure to sulfur mustard (SM). This study is the first to examine the histopathologic spectrum of changes in a large number of surgical lung biopsies from patients exposed to SM. METHOD: Fifteen patients with ...

Chemical warfare agents (CWA) including sulfur mustard (SM) were commonly used in Iran-Iraq war. Respiratory problems are the greatest cause of long-term disability among people who had combat exposure to SM. High-resolution computed tomography (HRCT) has been accepted as the imaging modality of choice in these patients. We used expiratory ...

BACKGROUND AND OBJECTIVE: Coccidioidomycosis is endemic in the south-western USA. This study described the clinicopathological features of pulmonary coccidioidomycosis found in otherwise healthy Japanese individuals. METHODS: Four male patients with pulmonary coccidioidomycosis were assessed by clinical examination, serology, PCR assay, high-resolution computed tomography and histopathological findings. RESULTS: All patients had ...

Bird fancier's lung (BFL) is one of the most common types of hypersensitivity pneumonitis. Nevertheless, the criteria for diagnosing this condition are not standardized. The current study is an in-depth investigation into the clinical characteristics of BFL in the largest series examined for this purpose by a single group, to ...

BACKGROUND AND OBJECTIVE: Vascular endothelial growth factor (VEGF) is known to contribute to the development of pulmonary oedema, and has been suggested to have a protective role against lung injury. To determine the role of VEGF in acute lung injury (ALI) and ARDS, VEGF levels were measured in lung epithelial ...

Bronchiolitis obliterans (BrOb) is a well-recognized complication of allogeneic hematopoietic stem cell transplantation (HSCT). It is associated with substantial morbidity and mortality in adult patients. However, the incidence and morbidity of this complication have not been well described in the pediatric population. We report our experience of BrOb in 216 ...

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is a common disease with several known extrarenal manifestations, although no known pulmonary features. The formation of renal cysts in ADPKD has been attributed to dysfunction of primary cilia and the primary cilia-related proteins polycystin-1 (in 85% of cases) and polycystin-2 in renal ...

Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive lung disease that commonly results in respiratory failure and death. However, the cause of death in these patients has not previously been fully defined. The current study reviews the clinical records and pathological findings of 42 consecutive patients with IPF who underwent ...

OBJECTIVE: Study the clinical features of pulmonary arterial hypertension (PAH) in Thai patients with systemic sclerosis (SSc), and compare these features between those with limited (lc) SSc and diffuse (dc) SSc. MATERIAL AND METHOD: The medical records of SSc patients attending the Division of Rheumatology, Chiang Mai University were reviewed. ...

Abciximab, a platelet glycoprotein (GP) IIb/IIIa inhibitor, has been shown to improve clinical outcomes in patients undergoing percutaneous coronary intervention. However, there is a well-documented increase in bleeding risk associated with the use of this agent. Spontaneous pulmonary hemorrhage is a particularly rare and easily misdiagnosed complication that requires early ...

Smoking is known to be linked to skin ageing and there is evidence for premature senescence of parenchymal lung fibroblasts in emphysema. To reveal whether the emphysema-related changes in cellular phenotype extend beyond the lung, we compared the proliferation characteristics of lung and skin fibroblasts between patients with and without ...

RATIONALE: Steady-state mild pulmonary hypertension is a risk factor for death in adults with sickle cell disease. Acute pulmonary hypertension has been reported during exercise and vasoocclusive pain crisis in these patients. OBJECTIVES: The aim of the present study was to evaluate changes in pulmonary pressures and cardiac biomarkers during ...

Respiratory function tests and arterial blood gas analysis were performed on 59 patients with β-thalassemia major (27 M, 22 F, age range: 18-45 years). All investigations were performed 24 h before the patients received a blood transfusion or when they were in a stable state hematologic condition. Echocardiography was performed ...

Respiratory function tests and arterial blood gas analysis were performed on 59 patients with β-thalassemia major (27 M, 22 F, age range: 18-45 years). All investigations were performed 24 h before the patients received a blood transfusion or when they were in a stable state hematologic condition. Echocardiography was performed ...

Markers of inflammation, coagulation, and fibrinolysis predict an adverse outcome in patients with sepsis. These markers also seem predictive of an adverse outcome in patients with localized infection and inflammation, such as in acute lung injury. Whether this is entirely related to the disease or is also due to ventilation ...

BACKGROUND/PURPOSE: Clinicians need to decide whether to begin isolation and empiric therapy for patients suspected of having infectious tuberculosis (TB). This study aimed to identify the demographic, clinical and radiographic characteristics of acid-fast bacilli (AFB) smear-positive patients and to create a smear-positive TB prediction rule, which clinicians may use to ...

Optic neuropathy has been reported in association with the use of tumor necrosis factor-alpha antagonists such as etanercept, infliximab, and adalimumab. This is a report of a patient who began experiencing decreased vision approximately 1 month after starting infliximab therapy for rheumatoid arthritis. Visual field testing showed bitemporal hemianopic scotomas, ...

The purpose of the current study was to compare the characteristics of lung cancer patients who had exposure to asbestos and asbestos-related radiological findings (ARRF) to the characteristics of patients who had no exposure. Of the 766 lung cancer patients evaluated, 607 had no exposure to asbestos and no ARRF, ...

Abnormal lung sounds in diffuse interstitial pneumonia have been characterized by the presence of crackles. However, few attempts have tried to analyze the sound where crackles are immersed. In this work base lung sounds (BLS) were analyzed by linear and nonlinear techniques to find possible differences between normal subjects and ...

Using recruitment manoeuvres in acute lung injury remains a controversial issue because no convincing outcome data support their general use, although many physiological studies have demonstrated beneficial effects on lung compliance, end-expiratory lung volume and gas exchange. One of the reasons why physiologically meaningful observations do not translate into clear ...

BACKGROUND: Transfusion-Related Acute Lung Injury (TRALI) is an uncommon but potentially fatal reaction that is both temporally and mechanistically related to receiving allogenic blood components. Although many patients with neurological disease receive plasma exchange, TRALI in a patient receiving plasma exchange for a neurological disease has not been previously reported. ...