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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 Feb 2018
Review
Topics: Diagnosis, Differential; Empyema, Pleural; Exudates and Transudates; Humans; Pleura; Pleural Effusion; Pleural Effusion, Malignant; Pneumothorax
PubMed: 29466146
DOI: 10.1056/NEJMra1403503 -
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 -
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 -
Respiratory Medicine Aug 2015Malignant pleural effusion is a frequent situation in pulmonary medicine. However, it is sometimes difficult to recognize the underlying etiology. The aim of this review... (Review)
Review
BACKGROUND/PURPOSE
Malignant pleural effusion is a frequent situation in pulmonary medicine. However, it is sometimes difficult to recognize the underlying etiology. The aim of this review is to provide the key characteristics of primary and metastatic pleural neoplasms.
METHODS
A review of the recent literature regarding pleural neoplasia is provided.
RESULTS
Malignant pleural mesothelioma (MPM) is the commonest primary pleural epithelial tumor showing remarkable histological heterogeneity often with prognostic significance. Various genetic alterations like changes in INK4 locus, NF2, BAP1 but also epigenetic changes are present in MPM. It should be distinguished from atypical mesothelial hyperplasia, mainly through morphological and clinical criteria, and from other rare primary and metastatic tumors, for which immunohistochemistry is rather important. Solitary fibrous tumor, the commonest primary pleural mesenchymal tumor is characterized by STAT6 overexpression. Other primary tumors, like adenomatoid tumor, well-differentiated papillary mesothelioma, synovial sarcoma, vascular tumors, various other sarcomas, thymic tumors and tumors of uncertain histogenesis are rarely encountered in the pleura. In contrast, metastatic disease is the commonest neoplasia of the pleura, and especially lung, breast and lymphoid malignancies.
CONCLUSION
The basic pathological, immunohistochemical and molecular characteristics of these entities are provided in the current review, along with their differential diagnosis.
Topics: Biomarkers, Tumor; Diagnosis, Differential; Humans; Immunohistochemistry; Lung Neoplasms; Mesothelioma; Mesothelioma, Malignant; Pleura; Pleural Neoplasms; Prognosis
PubMed: 26048082
DOI: 10.1016/j.rmed.2015.05.014 -
Ultraschall in Der Medizin (Stuttgart,... Dec 2023The CME review is intended to explain and discuss the clinical value of lung ultrasound but also to enable a pragmatically oriented approach by analyzing the clinical...
The CME review is intended to explain and discuss the clinical value of lung ultrasound but also to enable a pragmatically oriented approach by analyzing the clinical aspect. This includes knowledge of the pre-test probability, the acuteness of the disease, the current clinical situation, detection and/or characterization, initial diagnosis or follow up assessment and the peculiarities of exclusion diagnosis. Diseases of the pleura and lungs are described using these criteria with their direct and indirect sonographic signs and the specific clinical significance of ultrasound findings. The importance and criteria of conventional B-mode, color Doppler ultrasound with or without spectral analysis of the Doppler signal and contrast-enhanced ultrasound are discussed as well.
Topics: Humans; Lung; Ultrasonography; Pleura; Ultrasonography, Doppler, Color; Thorax
PubMed: 37054729
DOI: 10.1055/a-2010-7282