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Current Opinion in Pulmonary Medicine Sep 2022Pleuroparenchymal fibroelastosis (PPFE) is a clinico-radiologic-pathologic interstitial lung disease (ILD) characterized by fibrosis that has upper lobe and subpleural... (Review)
Review
PURPOSE OF REVIEW
Pleuroparenchymal fibroelastosis (PPFE) is a clinico-radiologic-pathologic interstitial lung disease (ILD) characterized by fibrosis that has upper lobe and subpleural predominance, involving both the visceral pleura and the subjacent subpleural lung parenchyma, and comprises dense fibroelastic changes with prominent elastosis of the alveolar walls together with fibrous thickening of the visceral pleura. The goal of this review is to summarize the state-of-the-art understanding in PPFE.
RECENT FINDINGS
PPFE was described in an increasing number of conditions. The course of disease is heterogeneous. Idiopathic PPFE, cases associated with telomerase-related gene mutations, cases related to a history of chemotherapy, and cases combining PPFE with a pattern of usual interstitial pneumonia, may have a particularly poor prognosis. Well-conducted retrospective studies identified marked PPFE features in approximately 10% of patients with idiopathic pulmonary fibrosis, 11% of patients with systemic sclerosis-associated ILD, 6.5% of patients with rheumatoid arthritis-associated ILD, and 23% of patients with hypersensitivity pneumonitis. Drug therapy has not been evaluated prospectively. A small retrospective study suggests that nintedanib may slow disease progression. However, whether the efficacy of antifibrotics is comparable in PPFE and in other forms of progressive pulmonary fibrosis warrants further evaluation.
SUMMARY
Accumulating data indicate that PPFE features are associated with poor prognosis in fibrosing ILDs. Further research on the management of PPFE is warranted.
Topics: Fibrosis; Humans; Idiopathic Pulmonary Fibrosis; Lung; Lung Diseases, Interstitial; Pleura; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 35855575
DOI: 10.1097/MCP.0000000000000907 -
The New England Journal of Medicine Sep 2021
Review
Topics: Antineoplastic Agents; Combined Modality Therapy; Epigenetic Repression; Humans; Immunotherapy; Mesothelioma, Malignant; Pleura; Pleural Neoplasms; Radiotherapy
PubMed: 34551230
DOI: 10.1056/NEJMra1912719 -
Annals of the American Thoracic Society Nov 2019Pleuroparenchymal fibroelastosis (PPFE) is an unusual pulmonary disease with unique clinical, radiological, and pathological characteristics. Designated a rare... (Review)
Review
Pleuroparenchymal fibroelastosis (PPFE) is an unusual pulmonary disease with unique clinical, radiological, and pathological characteristics. Designated a rare idiopathic interstitial pneumonia in 2013, its name refers to a combination of fibrosis involving the visceral pleura and fibroelastotic changes predominating in the subpleural lung parenchyma. Although a number of disease associations have been described, no single cause of PPFE has been unequivocally identified. A diagnosis of PPFE is most commonly achieved by identifying characteristic abnormalities on computed tomographic scans. The earliest changes are consistently located in the upper lobes close to the lung apices, the same locations where subsequent disease progression is also most conspicuous. When sufficiently severe, the disease leads to progressive volume loss of the upper lobes, which, in combination with decreased body mass, produces platythorax. Once regarded as a slowly progressing entity, it is now acknowledged that some patients with PPFE follow an inexorably progressive course that culminates in irreversible respiratory failure and early death. In the absence of effective medical drug treatment, lung transplant remains the only therapeutic option for this disorder. This review focuses on improving early disease recognition and evaluating its pathophysiological impact and discusses working approaches for its management.
Topics: Bone Marrow Transplantation; Fibrosis; Humans; Immunosuppressive Agents; Lung; Lung Diseases, Interstitial; Lung Transplantation; Pleura; Pulmonary Fibrosis; Tomography, X-Ray Computed
PubMed: 31425665
DOI: 10.1513/AnnalsATS.201902-181CME -
CA: a Cancer Journal For Clinicians Sep 2019Mesothelioma affects mostly older individuals who have been occupationally exposed to asbestos. The global mesothelioma incidence and mortality rates are unknown,... (Review)
Review
Mesothelioma affects mostly older individuals who have been occupationally exposed to asbestos. The global mesothelioma incidence and mortality rates are unknown, because data are not available from developing countries that continue to use large amounts of asbestos. The incidence rate of mesothelioma has decreased in Australia, the United States, and Western Europe, where the use of asbestos was banned or strictly regulated in the 1970s and 1980s, demonstrating the value of these preventive measures. However, in these same countries, the overall number of deaths from mesothelioma has not decreased as the size of the population and the percentage of old people have increased. Moreover, hotspots of mesothelioma may occur when carcinogenic fibers that are present in the environment are disturbed as rural areas are being developed. Novel immunohistochemical and molecular markers have improved the accuracy of diagnosis; however, about 14% (high-resource countries) to 50% (developing countries) of mesothelioma diagnoses are incorrect, resulting in inadequate treatment and complicating epidemiological studies. The discovery that germline BRCA1-asssociated protein 1 (BAP1) mutations cause mesothelioma and other cancers (BAP1 cancer syndrome) elucidated some of the key pathogenic mechanisms, and treatments targeting these molecular mechanisms and/or modulating the immune response are being tested. The role of surgery in pleural mesothelioma is controversial as it is difficult to predict who will benefit from aggressive management, even when local therapies are added to existing or novel systemic treatments. Treatment outcomes are improving, however, for peritoneal mesothelioma. Multidisciplinary international collaboration will be necessary to improve prevention, early detection, and treatment.
Topics: Antineoplastic Agents, Immunological; Asbestos; Australia; Biomarkers, Tumor; Carcinogenesis; Combined Modality Therapy; Diagnostic Errors; Europe; Genetic Predisposition to Disease; Germ-Line Mutation; Global Burden of Disease; Humans; Incidence; Inhalation Exposure; International Cooperation; Mesothelioma; Molecular Targeted Therapy; Occupational Exposure; Pleura; Pleural Neoplasms; Pneumonectomy; Prognosis; Tumor Suppressor Proteins; Ubiquitin Thiolesterase; United States
PubMed: 31283845
DOI: 10.3322/caac.21572 -
The American Journal of the Medical... Apr 2021The subpleural sparing pattern is a common finding on computed tomography (CT) of the lungs. It comprises of pulmonary opacities sparing the lung peripheries, typically... (Review)
Review
The subpleural sparing pattern is a common finding on computed tomography (CT) of the lungs. It comprises of pulmonary opacities sparing the lung peripheries, typically 1cm and less from the pleural surface. This finding has a variety of causes, including idiopathic, inflammatory, infectious, inhalational, cardiac, traumatic, and bleeding disorders. Specific disorders that can cause subpleural sparing patterns include nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), pulmonary alveolar proteinosis (PAP), diffuse alveolar hemorrhage (DAH), vaping-associated lung injury (VALI), cracked lung, pulmonary edema, pneumocystis jirovecii pneumonia (PJP), pulmonary contusion, and more recently, Coronavirus disease 2019 (COVID-19) pneumonia. Knowledge of the many etiologies of this pattern can be useful in preventing diagnostic errors. In addition, although the etiology of subpleural sparing pattern is frequently indistinguishable during an initial radiologic evaluation, the differences in location of opacities in the lungs, as well as the presence of additional radiologic findings, patient history, and clinical presentation, can often be useful to suggest the appropriate diagnosis. We did a comprehensive search on Pubmed and Google Scholar database using keywords of "subpleural sparing," "peripheral sparing," "sparing of peripheries," "CT chest," "chest imaging," and "pulmonary disease." This review aims to describe the primary differential diagnosis of subpleural sparing pattern seen on chest imaging with a strong emphasis on clinical and radiographic findings. We also discuss the pathogenesis and essential clues that are crucial to narrow the differential diagnosis.
Topics: Diagnosis, Differential; Humans; Lung Diseases; Pleura; Radiography, Thoracic; Tomography, X-Ray Computed
PubMed: 33487401
DOI: 10.1016/j.amjms.2021.01.008 -
Respiratory Medicine Jan 2022Pleural effusion is a frequent complication of acute pulmonary infection and can affect its morbidity and mortality. The possible evolution of a parapneumonic pleural... (Review)
Review
Pleural effusion is a frequent complication of acute pulmonary infection and can affect its morbidity and mortality. The possible evolution of a parapneumonic pleural effusion includes 3 stages: exudative (simple accumulation of pleural fluid), fibropurulent (bacterial invasion of the pleural cavity), and organized stage (scar tissue formation). Such a progression is favored by inadequate treatment or imbalance between microbial virulence and immune defenses. Biochemical features of a fibrinopurulent collection include a low pH (<7.20), low glucose level (<60 mg/dl), and high lactate dehydrogenase (LDH). A parapneumonic effusion in the fibropurulent stage is usually defined "complicated" since antibiotic therapy alone is not enough for its resolution and an invasive procedure (pleural drainage or surgery) is required. Chest ultrasound is one of the most useful imaging tests to assess the presence of a complicated pleural effusion. Simple parapneumonic effusions are usually anechoic, whereas complicated effusions often have a complex appearance (non-anechoic, loculated, or septated). When simple chest tube placement fails and/or patients are not suitable for more invasive techniques (i.e. surgery), intra-pleural instillation of fibrinolytic/enzymatic therapy (IPET) might represent a valuable treatment option to obtain the lysis of fibrin septa. IPET can be used as either initial or subsequent therapy. Further studies are ongoing or are required to help fill some gaps on the optimal management of parapneumonic pleural effusion. These include the duration of antibiotic therapy, the risk/benefit ratio of medical thoracoscopy and surgery, and new intrapleural treatments such as antibiotic-eluting chest tubes and pleural irrigation with antiseptic agents.
Topics: Chest Tubes; Drainage; Exudates and Transudates; Fibrinolytic Agents; Humans; Pleura; Pleural Effusion
PubMed: 34896966
DOI: 10.1016/j.rmed.2021.106706 -
American Journal of Clinical Pathology May 2021Peritoneal mesothelial cysts have been reported under various terms, including benign cystic mesothelioma, usually in the form of case reports/series, whereas...
OBJECTIVES
Peritoneal mesothelial cysts have been reported under various terms, including benign cystic mesothelioma, usually in the form of case reports/series, whereas extraperitoneal cases are rarely reported. Our objective was to report the detailed characteristics of cystic lesions of the serosal cavities.
METHODS
We retrospectively examined the clinicopathologic findings of a series of mesothelial cystic lesions (n = 79).
RESULTS
Most cases (n = 68, 86%) concerned the peritoneum, whereas 11 (14%) concerned the pericardium. No pleural cases were found. A total of 51 (64.5%) lesions were solitary, whereas 28 (35.5%) were multiple. Peritoneal lesions harbored a plump eosinophilic mesothelium and a loose connective stroma, whereas pericardial lesions showed a cuboidal/flattened mesothelium, collagenous stroma, intense inflammation, and other tissue types, like adipose and muscle tissue. Solitary peritoneal lesions are usually extrapelvic and found in older patients incidentally during other surgeries, whereas multiple lesions are found in younger patients and usually in the pelvis. The lesions show a benign clinical course with rare recurrences but no malignant transformation.
CONCLUSIONS
Most mesothelial cysts are peritoneal and rarely pericardial. Peritoneal cysts differ from pericardial cysts. Peritoneal solitary lesions differ from multiple lesions, also suggesting their pathogenetic differences.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Cysts; Epithelium; Humans; Male; Mesothelioma; Middle Aged; Neoplasm Recurrence, Local; Pelvis; Pleura; Retrospective Studies; Young Adult
PubMed: 33258870
DOI: 10.1093/ajcp/aqaa189 -
European Journal of Cardio-thoracic... Aug 2022
Topics: Anthracosis; Humans; Lung Neoplasms; Lymphatic Vessels; Pleura
PubMed: 35437587
DOI: 10.1093/ejcts/ezac204 -
Zentralblatt Fur Chirurgie Jun 2022Inflammatory diseases of the lung and pleura in children and adolescents cover a broad spectrum, including complicated pneumonia, tuberculosis, mycoses, and hydatid... (Review)
Review
Inflammatory diseases of the lung and pleura in children and adolescents cover a broad spectrum, including complicated pneumonia, tuberculosis, mycoses, and hydatid disease. Their frequency strongly depends on the geographical origin. The following article gives an overview - from diagnosis to surgical treatment of these diseases in the paediatric population.
Topics: Adolescent; Child; Communicable Diseases; Humans; Lung; Pleura
PubMed: 35104898
DOI: 10.1055/a-1720-2292 -
European Journal of Internal Medicine Feb 2020The functioning of the pleura and the endocrine system are not entirely independent of each other. Some hormones can reach a greater concentration in the pleural exudate... (Review)
Review
The functioning of the pleura and the endocrine system are not entirely independent of each other. Some hormones can reach a greater concentration in the pleural exudate than in the blood. However, the clinical significance of this finding remains unknown. In some circumstances, hormonal changes are responsible for pathological manifestations in the pleura. Hypothyroidism is one of the most common diseases that can cause a pleural effusion, likely resulting from alterations in capillary permeability. The presence of ectopic endometrial tissue within the lung parenchyma, pleura, pericardium or diaphragm is known as thoracic endometriosis and is one of the causes of catamenial pneumothorax and /or catamenial hemothorax, which can affect women of childbearing age and arises within 72 h from the onset of menstruation. Treatment and prevention of recurrent catamenial pneumothorax / hemothorax usually requires an approach that combines surgery and hormone therapy. Malignant pleural effusion from breast cancer may contain estrogen receptor-positive cells. In such a case, endocrine treatment may be effective in reducing the amount of pleural fluid and the associated symptoms. Thyroid cancer and lymphangioleiomyomatosis (LAM) are further hormone-sensitive malignancies in which pleura is frequently involved. The solitary fibrous tumor of pleura (SFPT) is an example of a pleural disease that can cause hormonal balance disorders. It can lead to a rise in the releasing factor for growth hormone (GHRH), human beta chorionic gonadotropin (Beta-hCG), and insulin-like growth factor 2 (IGF2). The consequence of such hormonal imbalance include hypertrophic pulmonary osteoarthropathy, gynecomastia, and refractory hypoglycemia, respectively.
Topics: Endocrine System; Endometriosis; Female; Humans; Male; Pleura; Pleural Diseases; Pneumothorax
PubMed: 31918926
DOI: 10.1016/j.ejim.2019.12.034