Desquamative Interstitial Pneumonia Research Articles

Why making smoking cessation a priority for rare interstitial lung disease smokers?

This review aims to discuss the complex relationship between smoking and interstitial lung diseases (ILDs), emphasizing the significant morbidity and mortality associated with these conditions. While the etiology of ILDs remains multifactorial, cigarette smoking emerges as a prominent modifiable risk factor implicated in their pathogenesis and progression. This narrative review will provide insight into smoking-associated interstitial lung diseases and personalised approaches to smoking cessation. Epidemiological studies consistently link smoking to ILDs such as idiopathic pulmonary fibrosis (IPF), respiratory bronchiolitis-associated ILD (RB-ILD), and desquamative interstitial pneumonia (DIP), highlighting the urgent need for comprehensive tobacco cessation strategies. Despite the established benefits of smoking cessation, adherence to cessation programs remains challenging due to nicotine addiction, psychological factors, and social influences. The modest success rates of smoking cessation in ILD patients, emphasises the importance of tailored interventions and ongoing support is needed to overcome barriers and to improve outcomes of quitting smoking in this category of vulnerable patients.

Perspectives in Smoking Related Interstitial Lung Diseases

Abstract Interstitial lung disease is a generic term encompassing a wide range of unrelated conditions that have in common a tendency to cause shortness of breath and/or cough associated with bilateral abnormal opacity of various types on conventional chest X-rays or high-resolution computed tomography scans, symptoms including dyspnea, wheezing, cough, and/or sputum production. This study aimed to show that cigarette smoke is a powerful inducer of inflammation and the cause-effect relationship between cigarette smoking and interstitial diseases – respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) and desquamative interstitial pneumonia (DIP). We bring to your attention the cases of two patients, heavy smokers with HRCT images suggestive for RB-ILD and DIP. The patients present to the on-call room with dry cough, exertional dyspnea and receive corticosteroid treatment with the recommendation to cease smoking. An approach would be to target high-risk populations, such as older adults with a history of smoking for early diagnosis of parenchymal lung diseases (e.g. ILD and chronic obstructive pulmonary disease) or lung cancer.

Exploring Histologic Emphysema in a Rural Lobectomy Cohort: Insights and a Review Correlations of Histologic Emphysema

Exposure to cigarette smoking is extensive in rural Appalachia where one in four adults’ smokes. The clinical implications of this habit are evident among patients with some of the highest national rates for chronic obstructive pulmonary disease (COPD), lung cancer, and pulmonary fibrosis. Individuals undergoing surgical lung resection for suspicious lung nodules or masses at a major rural academic center in the area demonstrated an excessive burden of histologic emphysema (73.5%). This destructive process of the alveoli was linked to a significant burden of comorbid conditions, various radiologic patterns of interstitial lung diseases and interstitial lung abnormalities, histologic fibrosis, inflammatory processes (respiratory bronchitis, desquamative interstitial pneumonia, peribronchiolar metaplasia), anthracosis, and lung cancer. Physiologically, this combination of injuries imposed substantial limitations. Findings presented may enhance the understanding of concurrent changes occurring in the smoker. The complex inter-relationships and disparities between clinical COPD, radiologic and histologic emphysema are defined. While emphysema remains an irreversible pathology, associated inflammatory and fibrotic conditions are possibly amenable to earlier smoking cessation strategies and available disease-modifying therapies.

Smoking-related interstitial lung disease: A narrative review.

Although smoking-related interstitial lung diseases (SR-ILD) are a relatively rare group of entities, they are a relevant public health problem of growing importance, both because they affect young adults and because of their increasing prevalence in recent years due to increased tobacco consumption. In patients who smoke and have non-specific respiratory symptoms, SR-ILD should be ruled out, a term that encompasses a group of different entities in which the basis for diagnosis is the smoking history together with compatible respiratory functional findings, radiology and/or histology. An association has been established between tobacco smoke and a group of diseases that include respiratory bronchiolitis-associated interstitial lung disease (2%-3% of all ILD), desquamative interstitial pneumonia (<1%), Langerhans cell histiocytosis (3%-5%) and acute eosinophilic pneumonia. Smoking is considered a risk factor for idiopathic pulmonary fibrosis which has also been called combined fibroemphysema (5%-10% of all ILD); however, the role and impact of smoking in its development, remains to be determined. The likely interconnection between the mechanisms involved in inflammation and pulmonary fibrosis in all these processes often results in an overlapping of clinical, radiological, and histological features. In the absence of robust scientific evidence on its management, smoking cessation is the first measure to be taken into account. Although most diseases have a benign clinical course after smoking cessation, some cases may progress to chronic respiratory failure.

Desquamative interstitial pneumonia: If there is smoke there is fire

Desquamative interstitial pneumonia (DIP) is a rare type of idiopathic interstitial lung disease (ILD) predominantly seen in smokers. Connective tissue diseases (CTDs) are one of the important causes of ILD and may have lung involvement antedating their usual manifestations, making the diagnosis difficult. Interstitial pneumonia with autoimmune features (IPAFs) are ILDs having autoimmune features but not fulfilling the criteria for a definite CTD. A small minority of IPAF cases may progress to CTD–related ILD over a period. Usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia are common radiological and histopathological patterns associated with IPAF. DIP is a rare manifestation of IPAF. Patients with IPAF and a non-UIP pattern may benefit from immunomodulation. We present a rare case of DIP in a nonsmoker, who was initially labeled as idiopathic ILD but eventually evolved into IPAF and exhibited a good response to immunosuppressive treatment.

A rare case of childhood interstitial lung disease attributed to desquamative interstitial pneumonia

Desquamative interstitial pneumonia (DIP) is a rare form of interstitial lung disease (ILD) in children that can be idiopathic but is usually associated with an inborn error of surfactant metabolism. We reported DIP in a 13-year-old girl who was referred to our outpatient clinic because of worsening dyspnea. High-resolution computed tomography showed ground-glass attenuation with honeycombing and intralobular, interstitial septal thickening, suggestive of an ILD. Transbronchial lung biopsy was performed, and histopathology findings were consistent with DIP. The patient was started on steroid therapy with oxygen support. Unfortunately, she died a month after being diagnosed due to disease progression.

Sequence of Rare Diagnoses in a Young Patient: Altitude Barotrauma Hemopneumothorax and Desquamative Interstitial Pneumonia.

We present the case of a 35-year-old patient without pathological history who developed hemopneumothorax due to altitude barotrauma during a commercial airline flight. The computed tomography (CT) of the chest identified the presence of right hydropneumothorax and emphysema "blebs" and bubbles. After the therapeutic insertion of a drain tube, the patient returned to the country by land transport. Three weeks later, he was diagnosed with right-sided pleurisy based on a CT scan with contrast material. A surgical intervention was then performed, and three biopsy samples were taken; the histopathological result highlighted suggestive elements for the diagnosis of desquamative interstitial pneumonia (DIP).

Imaging Features of Idiopathic Interstitial Lung Diseases.

Idiopathic interstitial pneumonias (IIPs) are a group of diffuse parenchymal lung diseases of unclear etiology and are distinguished from diffuse parenchymal lung diseases of known cause, such as connective tissue disease-related interstitial lung diseases or hypersensitivity pneumonitis by history, physical exam, imaging, serologic testing, and, when necessary, histopathology. The 2013 American Thoracic Society (ATS)/European Respiratory Society (ERS) guidelines are the most widely accepted classification of IIPs and include the following diagnoses: idiopathic pulmonary fibrosis, idiopathic nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, idiopathic lymphocytic interstitial pneumonia, idiopathic pleuro-parenchymal fibroelastosis, respiratory bronchiolitis-interstitial lung disease, and desquamative interstitial pneumonia. The gold standard for diagnosis of IIP involves multidisciplinary discussion among pulmonologists, radiologists, and pathologists. The focus of this review will be to discuss the imaging features of the most common IIPs and the role of multidisciplinary discussion as the gold standard for diagnosis.

ABCA3-related interstitial lung disease beyond infancy

BackgroundThe majority of patients with childhood interstitial lung disease (chILD) caused by pathogenic variants in ATP binding cassette subfamily A member 3 (ABCA3) develop severe respiratory insufficiency within their first...

СИНДРОМ МУЛЬТИКІСТОЗНОЇ ЛЕГЕНІ: РІДКІСНІ ВИПАДКИ РЕГРЕСІЇ

Multicystic lung syndrome (MLS) is defined by a presence on chest computed tomography of multiple, round parenchymal attenuations, having distinct borders with normal parenchyma and &lt; 2 mm thick wall. Pneumothorax, usually relapsing, and respiratory failry are the major clinical manifestations of MLS. CT findings can make certain contribution into recognition of the disease but diagnosis is largely based on appropriate symptoms, extrapulmonary signs of systemic disease and/or congenital disease. There has been described three major causes of MLS — lymphangioleiomyomatosis — LAM (sporadic or associated with tuberal sclerosis complex), pulmonary Lanherhans cell histiocytosis (LCH) and in recent time — Birt-Hogg-Dube) syndrome, associated with gene FLCN mutations. Other causes of MLS include lymphoid disorders of lung, especially in Sjögren's syndrome, non-amyloid immunoglobulin deposition disease; infections; malignancy, mainly sarcoma metastasis; desquamative interstitial pneumonia, hypersensitivity pneumonitis, cryptogenic organizing pneumonia with atypical cavity forming regression etc. The cases of regression of MLS in other diseases are rare. In most cases according to literature data regression is documented based on functional improvement which is not confirmed by CT data. The article presents the brief description of two clinical cases of MLS with demonstration of positive CT changes. Presented cases of MLS regression confirm the effectiveness of glucocorticosteroid treatment, which help to restore bronchial passability by means of reduction of compression caused by pulmonary infiltration in patient with organizing pneumonia and involution of granulamatous process in patient with hypersensitivity pneumonitis. In both cases the valvular mechanism of cyst genesis has been interrupted. Key words: multicystic lung syndrome caused by organizing pneumonia and hypersensitivity pneumonitis, glucocorticosteroid therapy, regression.

Peribronchiolar Metaplasia: A Marker of Cigarette Smoke-Induced Small Airway Injury in a Rural Cohort.

Peribronchiolar metaplasia (PBM) is considered a reaction to injury characterized by the proliferation of bronchiolar epithelium into immediately adjacent alveolar walls. While an association of PBM with diffuse interstitial lung diseases has been recognized, the clinical significance of PBM remains uncertain. A cohort (n = 352) undergoing surgical resection of a lung nodule/mass in a rural area was retrospectively reviewed. Multivariate logistic regression analysis was performed to determine the association of PBM with clinical, physiological, radiographic, and histologic endpoints. In the total study cohort, 9.1% were observed to have PBM as a histologic finding in resected lung tissue (n = 32). All but one of these patients with PBM were ever-smokers with a median of 42 pack years. Clinical COPD was diagnosed in two-thirds of patients with PBM. Comorbid gastroesophageal reflux disease (GERD) was significantly associated with PBM. All patients with PBM demonstrated radiologic and histologic evidence of emphysema. Measures of pulmonary function were not impacted by PBM. Mortality was not associated with the histologic observation of PBM. In a logistic regression model, centrilobular-ground glass opacity interstitial lung abnormality and traction bronchiectasis on the CT scan of the chest and histologic evidence of fibrosis, desquamative interstitial pneumonia and anthracosis all strongly predicted PBM in the cohort. A constellation of radiologic and histologic smoking-related abnormalities predicted PBM in study cohort. This confirms a co-existence of lung tissue responses to smoking including PBM, emphysema, and fibrosis. Acknowledging the physiologically "silent" nature of small airway dysfunction on pulmonary function testing, our findings support PBM as a histologic marker of small-airway injury associated with cigarette smoking.

The role of computed tomography in a diagnostic approach to cystic lung diseases and their differential diagnosis

In everyday routine, lung cysts are commonly seen in Computed Tomography, as far as manydifferentconditionsanddiseasesareassociatedwithaircysts.Thus,correctdiag-many different conditions and diseases are associated with air cysts. Thus, correct diag-nosisofcysticlungdiseases,whichshowawidespectrum,isachallengeforradiologists.nosis of cystic lung diseases, which show a wide spectrum, is a challenge for radiologists. Fordiagnosisanddifferentialdiagnosis,firstofall,cystsshouldbedistinguishedfromFor diagnosis and differential diagnosis, fi rst of all, cysts should be distinguished from suchotherairfilledlesions,likecavities,bullae,pneumatocele,emphysema,honeycomb-such other air fi lled lesions, like cavities, bullae, pneumatocele, emphysema, honeycomb-ingandcysticbronchiectasis.Second,cystscanbecategorizedassingle/localizedversusing and cystic bronchiectasis. Second, cysts can be categorized as single/localized versus multiple/diffuse.Solitary/localizedcystsincludeincidentalcysts,congenitalcysticdis-multiple/diffuse. Solitary/localized cysts include incidental cysts, congenital cystic dis-easesandcysticcancers.Multiple/diffusecystscanbefurthercategorizedaccordingtoeases and cystic cancers. Multiple/diffuse cysts can be further categorized according to thepresenceorabsenceofassociatedradiologicfindings.Multiple/diffusecystswithoutthe presence or absence of associated radiologic fi ndings. Multiple/diffuse cysts without associatedfindingsincludelymphangioleiomyomatosisandBirt-Hogg-Dubésyndrome.associated fi ndings include lymphangioleiomyomatosis and Birt-Hogg-Dubé syndrome. Multiple/diffusecystsmaybeassociatedwithground-glassopacityorsmallnodules.Multiple/diffuse cysts may be associated with ground-glass opacity or small nodules. Multiple/diffusecystswithnodulesincludeLangerhanscellhistiocytosis,cysticmetas-Multiple/diffuse cysts with nodules include Langerhans cell histiocytosis, cystic metas-tasisandamyloidosis.Multiple/diffusecystswithground-glassopacityincludepneumo-tasis and amyloidosis. Multiple/diffuse cysts with ground-glass opacity include pneumo-cystispneumonia,desquamativeinterstitialpneumoniaandlymphocyticinterstitialpneu-cystis pneumonia, desquamative interstitial pneumonia and lymphocytic interstitial pneu-monia.Thestepwiseradiologicdiagnosticapproachcanbehelpfulinreachingacorrectmonia. The stepwise radiologic diagnostic approach can be helpful in reaching a correct diagnosisforvariouscysticlungdiseases.diagnosis for various cystic lung diseases.

Smoking-related interstitial lung disease

Exposure to smoke is associated with the development of diseases of the airways and lung parenchyma. Apart from chronic obstructive pulmonary disease (COPD), in some individuals, tobacco smoke can also trigger mechanisms of interstitial damage that result in various pathological changes and pulmonary fibrosis. A causal relation has been established between tobacco smoke and a group of entities that includes respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP), Langerhans cell histiocytosis (LCH), and acute eosinophilic pneumonia (AEP). Smoking is considered a risk factor for idiopathic pulmonary fibrosis (IPF); however, the role and impact of smoking in the development of this differentiated clinical entity, which has also been called combined pulmonary fibrosis and emphysema (CPFE) as well as nonspecific interstitial pneumonia (NIP), remains to be determined. The definition of smoking-related interstitial fibrosis (SRIF) is relatively recent, with differentiated histological characteristics. The likely interconnection between the mechanisms involved in inflammation and pulmonary fibrosis in all these processes often results in an overlapping of clinical, radiological, and histological features in the same patient that can sometimes lead to radiological patterns of interstitial lung disease that are impossible to classify. For this reason, a combined approach to diagnosis is recommendable. This combined approach should be based on the joint interpretation of the histological and radiological findings while taking the clinical context into consideration. This paper aims to describe the high-resolution computed tomography (HRCT) findings in this group of disease entities in correlation with the clinical manifestations and histological changes underlying the radiological pattern.

Enfermedades pulmonares intersticiales relacionadas con el tabaco

Exposure to smoke is associated with the development of diseases of the airways and lung parenchyma. Apart from chronic obstructive pulmonary disease (COPD), in some individuals, tobacco smoke can also trigger mechanisms of interstitial damage that result in various pathological changes and pulmonary fibrosis. A causal relation has been established between tobacco smoke and a group of entities that includes respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP), Langerhans cell histiocytosis (LCH), and acute eosinophilic pneumonia (AEP). Smoking is considered a risk factor for idiopathic pulmonary fibrosis (IPF); however, the role and impact of smoking in the development of this differentiated clinical entity, which has also been called combined pulmonary fibrosis and emphysema (CPFE) as well as nonspecific interstitial pneumonia (NIP), remains to be determined. The definition of smoking-related interstitial fibrosis (SRIF) is relatively recent, with differentiated histological characteristics. The likely interconnection between the mechanisms involved in inflammation and pulmonary fibrosis in all these processes often results in an overlapping of clinical, radiological, and histological features in the same patient that can sometimes lead to radiological patterns of interstitial lung disease that are impossible to classify. For this reason, a combined approach to diagnosis is recommendable. This combined approach should be based on the joint interpretation of the histological and radiological findings while taking the clinical context into consideration. This paper aims to describe the high-resolution computed tomography (HRCT) findings in this group of disease entities in correlation with the clinical manifestations and histological changes underlying the radiological pattern.

Diffuse cystic lung diseases: Imaging spectrum and diagnostic approach using high-resolution computed tomography.

The lung cyst is an air-containing lucent area surrounded by thin imperceptible walls. Other lucent lung lesions like centrilobular emphysema, cavity, cystic bronchiectasis, honeycomb cyst, and pneumatoceles are close mimics of a lung cyst on high-resolution computed tomography (HRCT). Various diseases with multiple lung cysts throughout both the lungs are classified as diffuse cystic lung diseases (DCLDs). HRCT is considered the imaging of choice for diagnosis of such diffuse cystic lung diseases. Common DCLDs like lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome (BHS), Langerhans cell histiocytosis (LCH), lymphocytic interstitial pneumonia (LIP), and desquamative interstitial pneumonia (DIP) can be confidently diagnosed on HRCT without further requirement of histopathological confirmation. The imaging also helps in differentiation of uncommon DCLDs and exclusion of the mimics. This review describes a simple algorithmic approach for DCLDs on HRCT based on scrutinizing the cyst's distribution, size, and shape, background parenchymal changes, and its correlation with clinical features and extrapulmonary imaging findings.

Neumonías intersticiales idiopáticas

Idiopathic interstitial fibrosis has nonspecific, insidious clinical symptoms; it is the most common interstitial disease and has the worse known prognosis, with a survival without treatment of two to five years. In 2018, scientific societies updated the diagnostic criteria; the presence of a usual interstitial pneumonia pattern on an HRCT scan and/or a lung biopsy are decisive for establishing a diagnosis. Among idiopathic interstitial pneumonias, the most common is pulmonary fibrosis, but there are other less common entities such as nonspecific interstitial pneumonia (NSIP), respiratory bronchiolitis-associated diffuse interstitial lung disease (RB-DILD), and desquamative interstitial pneumonia (DIP); the latter two are related to tobacco use. There are also those which present in acute or subacute forms, such as cryptogenic organizing pneumonia (COP) and acute interstitial pneumonia (AIP). Lastly, there are the least common forms, such as lymphoid interstitial pneumonia (LIP) and idiopathic pleuroparenchymal fibroelastosis (IPPFE).

The method of probe-based confocal laser endomicroscopy in the diagnosis of idiopathic interstitial pneumonias

The diagnosis of idiopathic interstitial pneumonia (IIP) remains one of the most challenging issues in pulmonology. The article demonstrates the capabilities of the method of probe-based confocal laser endomicroscopy (pCLE) in diagnosing various forms of IIPs and provides comparisons with CT and the histological picture. According to the data obtained, it is difficult to distinguish most IIPs from each other using pCLE. Among the specific signs, a large number of alveolar cells in pCLE in a nonsmoking patient with desquamative interstitial pneumonia is not typical for other IIP in nonsmokers. Also, pCLE can be used to differentiate between IIP and other conditions, in particular, malignant lesions.

Simultaneous pathological findings in biopsy specimens of patients with surgically resected lung carcinoids and their role in survival.

Pulmonary carcinoid tumors are rare, low-grade malignant tumors that constitute 1–2% of all lung tumors. The present study aimed to describe the simultaneous pathological findings in biopsy specimens of patients with surgically resected lung carcinoids and determine their association with survival rates. For this purpose, 108 patients with resected carcinoid lung tumors were followed-up for 96 months and analyzed for simultaneous pathological findings in biopsy specimens. Among these, simultaneous pathological findings were found in 82 patients. The association between these findings and patient survival rates was evaluated. Atelectasis was a simultaneous finding in 52.4% of the patients, desquamative interstitial pneumonia (DIP) in 13.4%, emphysema in 24.4% and bronchiectasis in 9.8%. The survival rate was 100% for the patients with atelectasis, 81.8% for the patients with DIP, 90% for the patients with emphysema and 75% for the patients with bronchiectasis (P<0.05). According to the univariate analysis, the type of carcinoid was associated with patient survival with better survival rates for patients with typical carcinoids, while age, sex, stage and simultaneous pathological findings were not associated with patient survival. On the whole, there was a statistically significant difference in the survival rates of patients with resected lung carcinoids with different simultaneous pathological findings. However, further studies are warranted to assess the role of these findings in the survival of these patients.

Desquamative interstitial pneumonia in association with chronic necrotizing aspergillosis, a case report and literature review

Desquamative interstitial pneumonia (DIP) is a form of interstitial lung disease that is directly related to smoking. In addition to smoking, other factors have been implicated in its etiology, including: systemic disorders, dangerous materials in the environment, drugs and infectious agents. By reviewing the literature, we find that there are very rare cases that indicate infections as causing DIP. Here the author report on a 58-year-old male who was addict and complained of a dry cough with dyspnea for one month. TBLB was performed and pathology result was consistent with DIP. He received prednisolone 5 mg twice a day, but his symptoms persisted. Open-lung biopsy was performed and it illustrated Aspergillus pneumonia (Chronic necrotizing aspergillosis). He was treated with corticosteroids combined with antifungal agents.

Smoking-related interstitial lung diseases

Klinisches ProblemRaucherassoziierte interstitielle Lungenerkrankungen umfassen heterogene pulmonale Pathologien, deren korrekte Diagnostik prognostische und therapeutische Konsequenzen hat. In diesem Artikel werden die gängigsten raucherassoziierten interstitiellen Lungenerkrankungen beschrieben sowie eine strukturierte Herangehensweise präsentiert, welche den diagnostischen Arbeitsprozess erleichtern kann.Empfehlungen für die PraxisDie Computertomographie (CT) besitzt einen hohen Stellenwert in der Diagnose der raucherassoziierten interstitiellen Lungenerkrankungen und kann dazu beitragen, Lungenbiopsien zu verhindern. Um eine hohe diagnostische Genauigkeit zu erreichen, sollten standardisierte Untersuchungsprotokolle sowie eine strukturierte Herangehensweise in der Befundung zur Anwendung kommen. In den entzündlich dominierten Stadien der respiratorischen Bronchiolitis (RB), der respiratorischen Bronchiolitis mit interstitieller Lungenerkrankung (RB-ILD) sowie der desquamativen interstitiellen Pneumonie (DIP) haben die Beendigung des Rauchens sowie Steroide den größten therapeutischen Effekt. Bei fibrotischen Veränderungen (z. B. im Rahmen einer idiopathischen pulmonalen Fibrose [IPF]) können antifibrotische Therapien mit Pirfenidon und Nintedanib zum Einsatz kommen. Patienten mit dem Verdacht auf raucherassoziierte interstitielle Lungenerkrankung sollten in multidisziplinären Boards abgeklärt und behandelt werden.