Smoking-related Interstitial Lung Diseases Research Articles

BOSENTAN-RESPONSIVE SEVERE PULMONARY HYPERTENSION IN A PATIENT WITH PULMONARY LANGERHANS CELL HISTIOCYTOSIS: A CASE REPORT AND LITERATURE REVIEW

INTRODUCTION: Pulmonary langerhans cell histiocytosis (PLCH) is a rare smoking-related interstitial lung disease. Pulmonary hypertension (PH) is a common complication, and is strongly associated with increased mortality. Our patient developed severe PH secondary to an unusual combination of pulmonary arteriopathy and pulmonary veno-occlusive disease (PVOD) associated with PLCH. The PH and the dyspnea improved with Bosentan therapy.

PULMONARY LANGERHANS CELL HISTIOCYTOSIS AND RESPIRATORY BRONCHIOLITIS ASSOCIATED INTERSTITIAL LUNG DISEASE: A CASE REPORT

INTRODUCTION: Cigarette smoke is associated with the development of severe, diffuse interstitial inflammation, resulting in interstitial lung disease (ILD).1 A distinct entity termed smoking-related ILD, occurring exclusively in smokers, includes: Pulmonary Langerhans cell histiocytosis (PLCH), respiratory bronchiolitis (RB)-associated interstitial lung disease (RB-ILD), and desquamative interstitial pneumonia (DIP). These three diseases have overlapping diagnostic features, suggesting that they may represent a spectrum of lung disease from cigarette smoke.

Essential Role of Osteopontin in Smoking-Related Interstitial Lung Diseases

Smoking-related interstitial lung diseases are characterized by the accumulation of macrophages and Langerhans cells, and fibrotic remodeling, which are linked to osteopontin (OPN) expression. Therefore, OPN levels were investigated in bronchoalveolar lavage (BAL) cells in 11 patients with pulmonary Langerhans cell histiocytosis (PLCH), 15 patients with desquamative interstitial pneumonitis (DIP), 10 patients with idiopathic pulmonary fibrosis, 5 patients with sarcoidosis, 13 otherwise healthy smokers, and 19 non-smoking controls. Furthermore, OPN overexpression was examined in rat lungs using adenoviral gene transfer. We found that BAL cells from patients with either PLCH or DIP spontaneously produced abundant amounts of OPN. BAL cells from healthy smokers produced 15-fold less OPN, and those cells from non-smoking healthy volunteers produced no OPN. BAL cells from patients with either idiopathic pulmonary fibrosis or sarcoidosis produced significantly less OPN, as compared with patients with PLCH. These data were confirmed by immunochemistry. Nicotine stimulation increased production of both OPN and granulocyte-macrophage colony stimulating factor by alveolar macrophages from smokers. Nicotinic acetylcholine receptor expression resembled the pattern of spontaneous OPN production and was dramatically increased in both PLCH and DIP. OPN overexpression in rat lungs induced lesions similar to PLCH with marked alveolar and interstitial accumulation of Langerhans cells. Our findings suggest a pathogenetic role of increased OPN production in both PLCH and DIP by promoting the accumulation of macrophages and Langerhans cells.

Concurrent Smoking-Related Interstitial Lung Diseases in a Single Patient

The three smoking-related interstitial lung diseases (ILD) include desquamative interstitial pneumonia (DIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), and pulmonary Langerhans cell histiocytosis (PLCH). They are considered discrete entities, yet it is not unusual to find a mixture of pathologic features rendering the histopathologic diagnosis difficult. It is uncommon to have overlap in the different radiologic findings between these diseases. We present a unique case, in that the extent of DIP and PLCH-like changes were manifested both histologically and on high resolution computed tomography (HRCT) with ground-glass attenuation and upper lobe cystic changes suggestive of both diseases.

Emphysema and smoking-related lung diseases

•. Smoking-related lung diseases are a heterogeneous group of diseases that can overlap, and coexistence of individual disease processes is well recognised. •. The imaging and histopathological findings of each of these entities, excluding lung cancer, are described. Smoking-related lung diseases are a heterogeneous group of diseases that include lung cancer, emphysema, chronic bronchitis, desquamative interstitial pneumonia, respiratory bronchiolitis-related interstitial lung disease, pulmonary Langerhans' cell histiocytosis, acute eosinophilic pneumonia and interstitial fibrosis. High-resolution CT is highly sensitive for the detection of abnormalities in the lung parenchyma and airways. The imaging and histopathological findings in smoking-related interstitial lung disease can overlap, and co-existence of individual disease processes is well recognised. The aim of this paper is to describe the most important aspects of each of these entities, excluding lung cancer.

Tobacco Smoke–Related Diffuse Lung Diseases

Cigarette smoking has been associated with several diffuse lung diseases in which both bronchiolar and interstitial lung inflammation appear to result from chronic tobacco smoke inhalation. These diseases occur primarily in relatively young adult smokers and include desquamative interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, and pulmonary Langerhans cell histiocytosis. Although these diseases are associated with characteristic histopathologic and radiological features, there is significant overlap among these diseases, and some smokers may exhibit features indicative of the broad spectrum of smoking-related interstitial and bronchiolar lung injury patterns. Cigarette smoking has also been associated with acute eosinophilic pneumonia, and it increases the risk of developing other fibrotic interstitial lung diseases such as idiopathic pulmonary fibrosis and rheumatoid arthritis-associated interstitial lung disease. Although relatively uncommon, these smoking-related diffuse lung diseases should be recognized by clinicians as an important subset in the spectrum of interstitial lung diseases in which smoking cessation forms a central part of disease management. With the exception of acute eosinophilic pneumonia, which responds well to corticosteroid therapy, the role of corticosteroid and other immune-suppressive treatments in the management of smoking-related interstitial lung diseases is not entirely clear and is probably of limited utility, particularly in the absence of smoking cessation.

Smoking‐related changes in the background lung of specimens resected for lung cancer: a semiquantitative study with correlation to postoperative course

To assess the pathological findings in lobectomy specimens, to correlate them with smoking history and postoperative course and to compare the findings with those in smoking-related interstitial lung disease. Patients who had undergone lobectomy for lung cancer were reviewed. Subjects included 230 non-smokers and 587 smokers, of whom 572 had a known smoking index (SI). They were classified into mild, moderate and heavy smokers. Centrilobular emphysema (CLE), respiratory bronchiolitis, airspace enlargement with fibrosis (AEF), the presence of foci resembling usual interstitial pneumonia pattern (UIP/P) and the rate of postoperative respiratory failure were assessed. The incidence of AEF was 6.5% in mild smokers, and 17.7% in moderate smokers (P < 0.01) with lower lobe predominance. There were significant correlations (P < 0.01) between AEF and CLE and AEF and UIP/P. The rate of respiratory failure after lobectomy was 6%, and 10% in patients having UIP/P with or without AEF, but was not seen in patients with AEF alone (P < 0.01). AEF is an important smoking-related change in the lung that appears to correlate with the smoking history, and its distinction from UIP/P may be important.

Smoking-related interstitial lung disease

Pulmonary diseases associated with tobacco smoking are a complex group of disorders ranging from chronic obstructive pulmonary disease (COPD) to lung cancer. Interstitial lung diseases (ILDs) have only recently been linked to smoking. The ILDs related to smoking include respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and pulmonary Langerhans cell histiocytosis. The relationship of smoking with each of these entities has been largely established on the weight of epidemiologic evidence. Although they have been retained as distinct and separate conditions in various classifications of interstitial lung diseases, these 3 entities share a number of clinical, radiologic, and pathologic features suggesting that they represent a spectrum of patterns of interstitial lung disease occurring in predisposed individuals who smoke. Evaluation of histologic features, particularly in surgical lung biopsy samples, is important in making the distinction between these disorders. However, even after tissue biopsy, it may sometimes be difficult to clearly separate these entities. The importance of making the distinction between them lies in the different clinical management strategies used. Further experimental evidence, including genetic information, may be important in improving our understanding of these diseases.

47-jähriger Patient mit langjährigem Nikotinabusus, zunehmender Belastungsdyspnoe und diffusen, peripher und basal betonten Milchglastrübungen in der Lunge

The causal relationship between cigarette smoking and a number of interstitial lung diseases continues to evolve. These "smoking-related interstitial lung diseases" (SR-ILD) are a heterogeneous group of entities which have overlapping imaging findings and which can coexist. The presented case of a patient with smoking history and pulmonary ground-glass opacities demonstrates that thorough knowledge of the various manifestations of SR-ILD is essential for a confident diagnosis.

BAL in the diagnosis of smoking‐related interstitial lung diseases: Review of literature and analysis of our experience

The group of interstitial lung diseases (ILDs) is formed by respiratory tract disorders, whose aetiology is unknown in the majority of cases, the clinical course differs and the prognosis is generally serious. Some of the ILDs have a potential relation to tobacco smoking and are known as smoking-related ILDs (sr-ILD). Bronchoalveolar lavage fluid (BALF) examination is one of the initial procedures in the diagnosis of ILD. Despite the fact that histological confirmation is the gold standard in ILD diagnosis in many studies, the number of reported biopsies was low. In this review we present the results of BALF examinations of patients with sr-ILD and discuss their value in the differential diagnosis with other types of ILD. An extremely high total cell count (about 50 x 10(6) cells) with significant predominance of pigmented alveolar macrophages is a characteristic pattern of BALF in sr-ILD. The greatest challenge in BALF cytology interpretation is to distinguish sr-ILD and idiopathic pulmonary fibrosis (IPF). IPF is characterised by an elevated proportion and absolute count of lymphocytes and neutrophils; in addition, BALF lymphocytosis is higher in non-specific interstitial pneumonia than in usual interstitial pneumonia (UIP). The population of alveolar macrophage of patients with sr-ILD differs markedly from the foamy and vacuolated cells that predominate in IPF/UIP. Thus, the absence of pigmented cells rather excludes sr-ILD and indicates other types of ILD. To summarise, the place of BALF in the diagnosis of sr-ILD seems to be established.

Smoking-related Interstitial Lung Disease: Radiologic-Clinical-Pathologic Correlation

Cigarette smoking is a recognized risk factor for development of interstitial lung disease (ILD). There is strong evidence supporting a causal role for cigarette smoking in development of respiratory bronchiolitis ILD (RB-ILD), desquamative interstitial pneumonitis (DIP), and pulmonary Langerhans cell histiocytosis (PLCH). In addition, former and current smokers may be at increased risk for developing idiopathic pulmonary fibrosis (IPF). The combination of lower lung fibrosis and upper lung emphysema is being increasingly recognized as a distinct clinical entity in smokers. High-resolution computed tomography is sensitive for detection and characterization of ILD and may allow recognition and classification of the smoking-related ILDs (SR-ILDs) into distinct individual entities. However, the clinical, radiologic, and histologic features overlap among the different SR-ILDs, and mixed patterns of disease frequently coexist in the same patient. The overlap is most significant between RB-ILD and DIP. Macrophage accumulation is bronchiolocentric in RB-ILD, producing centrilobular ground-glass opacity, and more diffuse in DIP, producing widespread ground-glass changes. The coexistence of upper lung nodules and cysts in a smoker allows confident diagnosis of PLCH. Final diagnosis of an SR-ILD and identification of the specific entity can be achieved with certainty only after the pulmonologist, radiologist, and pathologist have reviewed all of the clinical, radiologic, and pathologic data.

Enfermedades intersticiales relacionadas con el tabaco: hallazgos en TC de alta resolución

To describe the anatomic and radiologic characteristics common to smoking-related interstitial lung diseases use and to evaluate the association between smoking and these diseases. We reviewed the clinical histories of patients with suspected smoking-related interstitial lung diseases from 2001 to 2005 who underwent high-resolution computed tomography and had radiologic findings of desquamative interstitial pneumonia, respiratory bronchiolitis, histiocytosis X, or pulmonary fibrosis. We analyzed the presence of emphysema, ground glass, centrilobar nodules, cysts, honey combing, and overlap among these findings. Fourteen patients were included. In desquamative interstitial pneumonia, the most common finding was ground glass (100%); in respiratory bronchiolitis, it was centrilobar nodules (100%); cysts were the most common finding in histiocytosis (85.7%) and honey combing was the most common finding in fibrosis (100%). Furthermore, over 75% of the patients had emphysema, which supported the relation with tobacco use. The patients with bronchiolitis also had signs common to the other three diseases (ground glass, honey combing, and cysts). There is a relation between smoking and these interstitial diseases, as well as overlapping in their clinical and radiological manifestations.

Choroby Śródmiąższowe Związane z Paleniem Tytoniu

Wśród chorób związanych z paleniem tytoniu znajdują się choroby o ustalonym związku przyczynowo-skutkowym, jak POChP czy rak płuca [...]

Smoking-related Emphysema and Interstitial Lung Diseases

Smoking-related illnesses contribute to a large number of deaths in the industrialized world and their treatment comprises a substantial percentage of total healthcare dollars. The most common and most well-known smoking-related illnesses include chronic obstructive pulmonary disease, bronchogenic carcinoma, and ischemic heart disease. However, the role of cigarette smoking in the pathogenesis of other lung diseases is becoming increasingly apparent. Knowledge of both the histologic and radiographic manifestations of smoking-related lung disease is important to the radiologist as imaging findings can be nonspecific. Finally, correlation of imaging and clinical information may obviate the need for open lung biopsy.

Interstitielle Lungenerkrankungen bei Rauchern

The most important smoking-related interstitial lung diseases (ILD) are respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and Langerhans' cell histiocytosis. Although traditionally considered to be discrete entities, smoking-related ILDs often coexist, thus accounting for the sometimes complex patterns encountered on high-resolution computed tomography (HRCT). Further studies are needed to elucidate the causative role of smoking in the development of pulmonary fibrosis.

Smoking-related interstitial lung diseases: radiologic-pathologic correlation

Smoking-related interstitial lung diseases (SRILD) are a heterogeneous group of entities of unknown cause. These diseases include desquamative interstitial pneumonia (DIP), respiratory-bronchiolitis-related interstitial lung disease (RB-ILD), pulmonary Langerhans' cell histiocytosis (LCH) and idiopathic pulmonary fibrosis (IPF). High-resolution CT is highly sensitive in the detection of abnormalities in the lung parenchyma and airways. Ground-glass attenuation can occur in DIP and RB-ILD. Whereas DIP is histologically characterized by intra-alveolar pigmented macrophages, RB-ILD shows alveolar macrophages in a patchy peribronchiolar distribution. LCH shows nodular infiltrates on histopathological examination containing varying amounts of characteristic Langerhans' histiocytes. The HRCT findings are characteristically bilateral, symmetrical and diffuse, involving the upper lobe zones with sparing of the costophrenic angles. The most prominent CT features are nodules (sometimes cavitary) measuring 1 to 10 mm in diameter, cysts and areas of ground-glass attenuation. Pathologically, IPF is characterized by its heterogeneity with areas of normal lung, alveolitis and end-stage fibrosis shown in the same biopsy specimen. High-resolution CT findings consist of honeycombing, traction bronchiectasis and intralobular interstitial thickening with subpleural and lower lung predominance. Since coexisting lesions in the same cases have been observed, a better understanding of the different smoking-related interstitial lung diseases (SRILD) allows a more confident and specific diagnosis.

Lack of Evidence for an Association Between Neurofibromatosis and Pulmonary Fibrosis

To reassess the association between neurofibromatosis and pulmonary fibrosis. Retrospective single-center study with analysis of patients' chest radiographs, CT scans, and medical records. Tertiary care, referral medical center. One hundred fifty-six adult patients with neurofibromatosis seen over a 6-year period between 1997 and 2002. A review of chest radiographs revealed abnormal findings in 70 patients (44.9%). The most common radiographic abnormalities were extrapulmonary nodules or masses seen in 22 patients (14.1%), followed by skeletal abnormalities in 16 patients (10.3%). Bilateral interstitial infiltrates were noted in only three patients (1.9%), all of whom had potential causes other than neurofibromatosis for their lung infiltrates, including smoking-related interstitial lung disease, rheumatoid lung disease, recurrent pneumonias, and a history of ARDS. CT scans were available in two of these patients and revealed nonspecific patterns of abnormalities with no honeycombing. Six patients had bullae or cystic airspaces demonstrated on chest radiography or CT scan; all of these findings occurred in the context of smoking-related emphysema. Combined together, bilateral interstitial lung infiltrates or cystic airspaces were demonstrated in five patients (3.2%) by chest radiography, and in eight patients (5.1%) by chest radiography or CT scanning; one patient had both findings on the CT scan. We found little evidence to support an association between neurofibromatosis and pulmonary fibrosis or any other form of parenchymal lung disease. Interstitial lung disease and bullae described in association with neurofibromatosis in previous reports may have, in part, represented smoking-induced manifestations.

Imaging of macrophage-related lung diseases

Macrophage-related pulmonary diseases are a heterogeneous group of disorders characterized by macrophage accumulation, activation or dysfunction. These conditions include smoking-related interstitial lung diseases, metabolic disorders such as Niemann-Pick or Gaucher disease, and rare primary lung tumors. High-resolution computed tomography abnormalities include pulmonary ground-glass opacification secondary to infiltration by macrophages, centrilobular nodules or interlobular septal thickening reflecting peribronchiolar or septal macrophage accumulation, respectively, emphysema caused by macrophage dysfunction, and honeycombing following macrophage-related lung matrix remodeling.

Emphysema and smoking-related lung diseases

Pulmonary complications of smoking include lung cancer, chronic obstructive pulmonary disease (COPD) and smoking-related interstitial lung disease.Injury related to cigarette smoking may result in a spectrum of response in the lungs in which several disease entities co-exist.High resolution CT is the imaging modality of choice in patients with suspected emphysema or smoking-related interstitial lung disease.

Respiratory bronchiolitis associated with interstitial lung disease

Respiratory bronchiolitis associated with interstitial lung disease (RB-ILD), first described by Niewoehner et al in an autopsy study of cigarette smokers who died from non pulmonary causes in 1974, is a rare entity that should be distinguished from the other interstitial lung diseases and in particular from desquamative interstitial pneumonia, although the two conditions share a similar histopathological pattern. RB-ILD is clearly connected with tobacco smoking and has been inserted in the "smoking related interstitial lung diseases" together with DIP and Cell histiocytosis of Langerhans; it may also be associated with occupational exposure to machine fumes. The following is a case report of a patient with both smoking and occupational exposure.