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At least 19 recordsLinked to original sources
PubMed1986

Breathing pattern during and after maximal exercise in patients with chronic obstructive lung disease, interstitial lung disease, and cardiac disease, and in normal subjects.

Inspiratory muscle fatigue and pulmonary edema are both known to cause rapid shallow breathing. It has been suggested that exercise tolerance in patients with pulmonary disease and cardiac disease may be limited by the development of inspiratory muscle fatigue and pulmonary edema, respectively, at maximal exercise. If these hypotheses are correct, breathing pattern during recovery from maximal exercise in these patients should be rapid and shallow compared with that during exercise. This study was performed to test these hypotheses. Seven patients with chronic obstructive pulmonary disease (COPD), 8 patients with interstitial lung disease (ILD), 7 patients with cardiac disease (CD) (mitral valve disease or left ventricular dysfunction) and 8 normal (NR) subjects each performed maximal incremental exercise on a cycle ergometer. Exercise breathing pattern was compared with that during recovery by calculating the mean difference in tidal volume (at the same levels of minute ventilation) between exercise and recovery for each subject. Recovery breathing pattern was similar to that during exercise for the COPD, ILD, and NR subjects. In contrast, breathing pattern during recovery was rapid and shallow compared with that during exercise for the CD patients; recovery tidal volume was less than that during exercise for the same level of minute ventilation. The fact that rapid shallow breathing does not develop during recovery from maximal exercise in patients with COPD or ILD suggests that inspiratory muscle fatigue does not limit their exercise tolerance. The relative rapid shallow breathing during recovery from maximal exercise in patients with CD is probably due to the development of pulmonary edema at maximal exercise, but further studies are needed to confirm this.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed2001

Lung transplantation in interstitial lung disease.

Interstitial lung disease is a heterogeneous group of illnesses, some of which may progress to a fibrosing stage and cause respiratory failure. For selected candidates, lung transplantation is the ultimate therapeutic option. We review data on lung transplantation for various interstitial lung diseases. We address indications, procedures, and outcomes for patients undergoing transplantation. Unique issues affecting morbidity, mortality, and recurrence of disease are discussed. We review the literature of transplantation for specific interstitial lung diseases and the outcomes of transplantation for interstitial lung diseases. Candidates with idiopathic pulmonary fibrosis experience high mortality on the waiting list, but derive significant survival benefit from lung transplantation. Recurrence is reported for several interstitial lung diseases after lung transplantation. Survival with lung transplantation for interstitial lung diseases is comparable with that attained in recipients with other indications. Lung transplantation is a well-tolerated, effective therapy for respiratory failure in interstitial lung disease.

Histiocytosis, Langerhans-Cell↗
PubMed2000

Smoking-related interstitial lung diseases.

Interstitial lung diseases (ILDs) encompass diverse clinicopathological disease entities ranging from idiopathic interstitial pneumonia (IIP) to interstitial pneumonia accompanied by collagen vascular diseases and diseases related to smoking, drug reactions, occupational environments, infections, and malignancies. Our focus is on the smoking-related interstitial lung diseases. Specifically, we focus on reports suggesting that chronic smoking is deeply involved in the disease pathogenesis and on reports suggesting that chronic smoking may influence the clinical course in terms of either disease severity or progression. Pulmonary Langerhans' cell granulomatosis (PLCG), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis with interstitial lung diseases (RB-ILD), interstitial pneumonia associated with rheumatoid arthritis, acute respiratory distress syndrome (ARDS), and idiopathic pulmonary fibrosis (IPF) are covered.

Comorbidity↗
PubMed2006

[Pulmonary damage during polymyositis and dermatomyositis: interstitial lung disease].

Interstitial lung disease is a serious complication of polymyositis/dermatomyositis and leads to death from chronic respiratory insufficiency in 30 to 66% of cases. It is a criterion of poor prognosis in these disorders. Its onset occurs at variable points in the course of polymyositis/dermatomyositis, and precedes them in half of all cases. Presentation may also vary: acute (25%), insidious (60%), or infraclinical, discovered fortuitously (15%). The examinations of choice for early screening are high-resolution computed tomography (CT) and pulmonary function tests, which should be performed during the initial work-up and during ongoing surveillance. Moreover, high-resolution CT also makes it possible to determine the type of histologic lesions in the interstitial lung disease. Today, diagnosis of this disease does not generally require histological confirmation; nonspecific interstitial lung disease seems to be the most common histologic form of lung damage in polymyositis/dermatomyositis (40 to 80%). Anti-Jo1 antibodies are a sensitive marker of interstitial lung disease during polymyositis/dermatomyositis, and close surveillance of lung function is recommended in patients with these autoantibodies. Systematic testing for them is also justified in patients with apparently idiopathic interstitial lung disease, to rule out underlying polymyositis/dermatomyositis. No clear treatment protocols have been established for interstitial lung disease during polymyositis/dermatomyositis. Corticosteroid treatment is the first choice. Its combination with cyclophosphamide may be most effective in corticosteroid-resistant forms of polymyositis/dermatomyositis, especially when begun early; it may also be appropriate to begin corticosteroids as soon as factors predicting poor prognosis are detected.

Acetylcysteine↗
PubMed2004

Resting and exercise physiology in interstitial lung diseases.

Interstitial lung diseases (ILDs) are a heterogeneous group of parenchymal pulmonary disorders with varying histologic appearances. Pulmonary function tests have gained a prominent role in the diagnosis and management of patients with these disorders. Although their role in the differential diagnosis of the various ILDs is limited, physiologic measurements are pivotal studies providing clues regarding disease severity, prognosis,and response to therapy.

Diagnosis, Differential↗
PubMed2003

[Interstitial lung diseases].

Interstitial lung diseases (ILD) are an heterogeneous group of inflammatory diseases characterized by an anatomical distortion of peripheral airways and interstitium, determined by a first stage of alveolitis and a following stage of fibrosis. Natural history of several ILD is characterized by slow and progressive destruction of alveolar-capillary functional units, often with respiratory failure and death. For their smoldering evolution and not specificity of symptoms (exertional dyspnea and cough) ILD may remain not diagnosed and not treated for a long time.

Adrenal Cortex Hormones↗
PubMed2001

Unusual systemic disorders associated with interstitial lung disease.

Interstitial lung disease often starts as a reaction to a causative insult or antigen, such as an inhaled environmental organic or inorganic dust and autoimmune mediated injury. When no underlying cause can be found, the inflammatory change in the gas exchanging parts of the lungs and associated fibrosis is called idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis). The common causes of pulmonary fibrosis can be classified as granulomatosus, nongranulomatous, neoplastic, and iatrogenic. There are other multisystem disorders not included in this classification that are associated with interstitial lung disease. Amyloidosis, Behcet disease, Kikuchi disease, and Whipple disease are such multiorgan disorders that involve the lungs.

Amyloidosis↗
PubMedDate not supplied

Radiographic appearance of interstitial lung disease.

Interstitial lung disease has many forms, and its appearance can vary widely under radiographic imaging. This article reviews the anatomy and physiology of the lung, then describes the most common radiographic characteristics of ILD-the ground glass effect, consolidation, cysts, honey-combing, pulmonary nodules and interstitial thickening. Also included is a discussion of the advantages of using high-resolution computed tomography to image ILD.

Humans↗
PubMed2002

Pertechnegas lung clearance in different forms of interstitial lung disease.

Interstitial lung diseases (ILD) are characterized by an acute or chronic inflammation of the alveolar capillary membrane, which affects the permeability of this membrane. A possible way to measure the permeability of the membrane is by radionuclide aerosol imaging. Pertechnegas, a gas composed of technetium-labelled carbon particles, has recently been proposed as a new ventilation agent to measure this lung clearance. The clearance by pertechnegas in the four most common forms of ILD (eight patients with connective tissue disease, 10 with hypersensitivity pneumonitis, nine with idiopathic interstitial pneumonia and 10 with sarcoidosis) was measured and compared with 10 nonactive smoking controls. Because forced vital capacity (FVC), total lung capacity (TLC) and carbon monoxide diffusing capacity of the lung (DL,CO) are used in the assessment of functional severity of the ILD, the pertechnegas clearance was correlated with these lung-function indices. It was found that the time to half clearance of pertechnegas of the lung is significantly decreased in idiopathic interstitial pneumonia (p<0.0001), hypersensitivity pneumonitis (p=0.0005) and connective tissue disease (p=0.002) but not in sarcoidosis when compared with 10 nonsmoking controls. A significant correlation is also found between time to half clearance and FVC (r=0.76; p<0.0001), TLC (r=0.63; p<0.0001) and DL,CO (r=0.75; p<0.0001) for all groups together. For all subjects as a group, the time to half clearance is shorter in the upper lung zones than in the lower zones (p<0.0001) and the ratio between both zones is not significantly different between the different types of disease. These results indicate that pertechnegas clearance is increased in idiopathic interstitial pneumonia, hypersensitivity pneumonitis and connective tissue disease, but not in sarcoidosis and is related to the functional severity of the disease.

Adolescent↗
PubMedDate not supplied

Interstitial lung disease.

Interstitial lung disease in children is a complex group of disorders whose etiology and pathogenesis is not entirely clear. Although the basic pathogenesis has been extrapolated from adult studies, its relevance in the pediatric population can be questioned. Several classifications of this condition have been put forward but Liebow's histologic classification is well accepted. Clinical manifestations are non-specific and can vary from no symptoms and a positive chest X-ray to more characteristic signs and symptoms. The triad of tachypnea, intercostal retractions and dry crackles is characteristic. Chest roentgenograms are a useful diagnostic tool and CT scans help to stage disease severity. Spirometry classically shows a restrictive pattern. Lung biopsy is the gold standard for establishing a diagnosis. Corticosteroids are the cornerstone of treatment in this condition. Thus, in view of the multifactorial nature of this disease, more extensive multi-centre prospective studies are required in order to better understand this rather complex group of disorders.

Adult↗
PubMed1998

Exercise and interstitial lung disease.

Interstitial lung disease (ILD) limits exercise capacity through a variety of complex and intriguing mechanisms, including ventilatory limitation, diffusion impairment, and ventilation-perfusion derangement. Resting pulmonary function testing seldom explains the symptoms nor defines the specific pathophysiology of the individual patient. Cardiopulmonary exercise testing can elucidate the relative contributions of these mechanisms and guide therapy. A fundamental problem in ILD is one of inadequate time for lung inflation during intense exercise, resulting in dynamic hypoinflation relative to the ventilatory demand. Pulmonary rehabilitation is underused in ILD. Functional CT imaging may provide insight into the relationship between structural and physiologic abnormalities of regional pulmonary function. Recent advances in nitric oxide research will perhaps further our understanding of the basic pathophysiology of ILD and provide specific treatment for the associated pulmonary vascular disease.

Bronchodilator Agents↗
PubMedDate not supplied

Pulmonary function testing in interstitial lung diseases.

Interstitial lung diseases (ILDs) are functionally characterized by a restrictive ventilatory defect due to a reduced distensibility of the lung parenchyma. ILD patients also show a reduced exercise tolerance, the main factors limiting exercise capacity being ventilatory and gas exchange abnormalities. Functional abnormalities in ILDs are typical, but not specific. Despite the fact that different lung function patterns have been described among ILDs, they overlap and their practical application to differentiate ILDs is poor. Resting pulmonary function and exercise-induced hypoxemia can aid in defining the prognosis of ILDs and in referring patients for lung transplantation. Additionally, spirometry and diffusing capacity are useful to monitor the response of patients to therapy.

Humans↗
PubMed1994

Cardiopulmonary exercise testing in interstitial lung disease.

Interstitial lung disease (ILD) can determine severe lung function impairment both at rest and during exercise. Usually, resting measurements of lung and cardiac function give enough information on the degree of the disease. Thus, exercise testing should be reserved only for particular situations such as presence at the same time of cardiac and respiratory involvement, symptomatic patients with normal spirometry, and to check the response to therapy. A better understanding of the pathophysiology and cardiorespiratory consequences of ILD can give an important contribution in improving methods of exercise testing to assess disability. In this perspective, we analyze the factors limiting exercise performance: the progressive hypoxemia that appears or is worsened by exertion; the ventilatory abnormalities that lead to a rapid shallow breathing pattern with a VE that reach the MVV; and the cardiovascular limitation with low maximum heart rate, low cardiac output and high PVR and PAP.

Exercise Test↗
PubMed1993

Current issues in the assessment of interstitial lung disease.

Interstitial lung diseases (ILDs) are chronic disorders of the lower respiratory tract, characterized by inflammation and derangement of the alveolar walls. The major functional consequence is the loss of alveolar capillary units and, thus, a limitation of oxygen transfer from air to blood. In recent years, new insights into many aspects of alveolitis as the basic pathological substrate of these diseases, the different inflammatory and immune competent effector cells and their role in the mechanisms of injury and repair have changed our concepts about the pathogenesis of ILD. New techniques, such as bronchoalveolar lavage and computed tomography, allow a better assessment of disease activity and extent of the underlying process. This review also discusses the problems of classification of ILD, and concentrates on issues of assessment and markers of disease activity. Treatment is not a topic of this review but better assessment of these disorders is probably a prerequisite of further progress in treatment and prognosis.

Biomarkers↗
PubMed2005

Comparison of disease progression and mortality of connective tissue disease-related interstitial lung disease and idiopathic interstitial pneumonia.

OBJECTIVE: To compare disease progression and mortality between idiopathic interstitial pneumonia (IIP) and interstitial lung disease (ILD) due to connective tissue diseases (CTD) including scleroderma, rheumatoid arthritis, systemic lupus, polymyositis, dermatomyositis, Sjögren's syndrome, and mixed CTD. METHODS: A case-control study of patients with CTD-ILD (n = 46) and IIP controls (n = 51), seen at the University of Michigan between July 1,1998 and June 30,1999 and followed until March 30, 2002, was conducted. Survival analysis and Cox regression were performed to estimate survival, accounting for demographic and clinical parameters, including pulmonary function tests and high resolution computed tomography (HRCT) diagnosis and scoring. RESULTS: Median followup time was 4.4 person-years. Five-year survival in the IIP group was 51.9% (95% confidence interval [95% CI] 30.8-69.4) versus 43.4% (95% CI 21.1-63.9) in the CTD-ILD group. There were no significant differences among HRCT diagnostic categories between IIP and CTD-ILD. A fibrotic score > or = 2 was associated with decreased survival among the entire group. Age at diagnosis and most recent forced vital capacity were significant predictors of mortality when adjusted for IIP versus CTD-ILD diagnosis, sex, and interstitial score. CONCLUSION: Contrary to expectation, CTD-ILD compared with IIP appears to be associated with a worse prognosis when adjusted for age. A higher fibrotic score is suggestive of decreased survival.

Adult↗
PubMed2004

Interstitial lung disease in the patient who has connective tissue disease.

Interstitial lung disease is a common complication of many of the connective tissue diseases. Because the prognosis, degree of reversibility, and optimal therapy differs for each disease presentation, a thorough knowledge of the pulmonary presentations of each connective tissue disease is important. Additionally, the challenge of finding the patient who has occult connective tissue disease in an interstitial lung disease clinic is discussed.

Arthritis, Rheumatoid↗
PubMed1995

[Interstitial lung disease in patients with connective tissue diseases].

Interstitial lung disease is a significant prognostic factor in connective tissue diseases. To further clarify clinical and immunological features of interstitial lung disease, we studied 894 consecutive patients with connective tissue diseases first evaluated between 1970 and 1993. Interstitial lung disease was found in 101 of 181 (56%) patients with systemic sclerosis, 92 of 183 (50%) with overlap syndrome, 35 of 76 (46%) with polymyositis/dermatomyositis, and 13 of 444 (3%) with systemic lupus erythematosus (p < 0.05, systemic lupus erythematosus vs. other connective tissue diseases). The presence of interstitial lung disease correlated with decreased survival in systemic sclerosis and in polymyositis/dermatomyositis, but not in overlap syndrome or in systemic lupus erythematosus. Interstitial lung disease in patients with connective tissue diseases was classified into two types: acute (n = 8) and chronic (n = 233). Among the 8 patients with acute disease, 4 (all with dermatomyositis) died of respiratory failure and 3 (all with systemic lupus erythematosus) responded to corticosteroids. Among the 233 patients with chronic interstitial lung disease, 20 had polymyositis/dermatomyositis with anti-aminoacyl tRNA synthetase antibodies and 40 had overlap syndrome with anti-U1 RNP antibodies. Respiratory failure in these patients was not frequent and occurred late in the course of the disease. As a cause of death, respiratory failure was associated with autoantibodies to topoisomerase I and aminoacyl tRNA synthetase but not anti-U1 RNP. Autoantibodies to aminoacyl tRNA synthetases were detected before the development of interstitial lung disease in 9 patients with polymyositis/dermatomyositis. We conclude that the clinical features of interstitial lung disease associated with connective tissue diseases vary with the type of connective tissue disease, and that analysis of autoantibodies can be useful in establishing a diagnosis and in forecasting the course and outcome.

Adult↗
PubMed2005

[Interstitial lung disease related to systemic connective tissue diseases].

Interstitial lung disease (ILD) is one of the most frequent and most serious complications of connective tissue diseases such as systemic sclerosis, systemic lupus erythematosus, dermato- and polymyositis, Sjogren's syndrome, or mixed connective tissue disease. Diagnosis of ILD is often delayed in patients with connective tissue diseases because of the systemic character of the primary condition, restricted physical activity of the patient and insufficient awareness of the physycian. The aim of the current review is to present the up-to-date information on ethiopathology, classification, diagnosis and treatment of ILD in patients with connective tissue diseases.

Collagen Diseases↗