Cystic Lung Disease

Chest Imaging ◽  
2019 ◽  
pp. 429-433
Author(s):  
Juliana Bueno
Keyword(s):  
Lung Disease ◽  
Langerhans Cell Histiocytosis ◽  
Normal Lung ◽  
Lung Cysts ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Cystic Lung Disease ◽  
Lymphocytic Interstitial Pneumonia ◽  
Cavitary Disease ◽  
Diffuse Lung

A lung cyst is a round parenchymal lucency or low-attenuating area with a well-defined interface with adjacent normal lung and surrounded by an epithelial or fibrous wall that is often less than 2 mm in thickness. Diseases that manifest with lung cysts comprise a limited but well known group of entities. Classic diseases manifesting with diffuse lung cysts include pulmonary Langerhans cell histiocytosis (PLCH), lymphangioleiomyomatosis (LAM), lymphocytic interstitial pneumonia (LIP) and Birt-Hogg-Dubé syndrome. However, it is important to recognize other conditions that may mimic cystic lung disease, such as pneumatoceles, tracheobronchial papillomatosis, cavitary disease (e.g. tuberculosis, fungal infection), bronchiectasis, and emphysema. Differentiation between true cysts and cyst mimics may be difficult, and may rely on identification of ancillary findings.

scholarly journals Multiple cystic lung disease

2015 ◽  
Vol 24 (138) ◽  
pp. 552-564 ◽  
Author(s):  
Flavia Angélica Ferreira Francisco ◽  
Arthur Soares Souza ◽  
Gláucia Zanetti ◽  
Edson Marchiori
Keyword(s):  
Lung Disease ◽  
Open Lung Biopsy ◽  
Diagnostic Challenge ◽  
High Resolution Computed Tomography ◽  
Disease Evolution ◽  
Lung Cysts ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Cystic Lung Disease ◽  
Cystic Metastasis

Multiple cystic lung disease represents a diverse group of uncommon disorders that can present a diagnostic challenge due to the increasing number of diseases associated with this presentation. High-resolution computed tomography of the chest helps to define the morphological aspects and distribution of lung cysts, as well as associated findings. The combination of appearance upon imaging and clinical features, together with extrapulmonary manifestations, when present, permits confident and accurate diagnosis of the majority of these diseases without recourse to open-lung biopsy. The main diseases in this group that are discussed in this review are lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and folliculin gene-associated syndrome (Birt–Hogg–Dubé); other rare causes of cystic lung disease, including cystic metastasis of sarcoma, are also discussed. Disease progression is unpredictable, and understanding of the complications of cystic lung disease and their appearance during evolution of the disease are essential for management. Correlation of disease evolution and clinical context with chest imaging findings provides important clues for defining the underlying nature of cystic lung disease, and guides diagnostic evaluation and management.

scholarly journals Paediatric pulmonary Langerhans cell histiocytosis

Breathe ◽  
2020 ◽  
Vol 16 (2) ◽  
pp. 200003
Author(s):  
Mhairi Barclay ◽  
Rebecca Devaney ◽  
Jayesh. M. Bhatt
Keyword(s):  
Respiratory Failure ◽  
Lung Disease ◽  
Langerhans Cell Histiocytosis ◽  
Multidisciplinary Treatment ◽  
Langerhans Cell ◽  
List Type ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Cystic Lung Disease ◽  
Management Of Complications

Paediatric pulmonary Langerhans cell histiocytosis (pPLCH) is a rare diffuse cystic lung disease. Unlike pulmonary Langerhans cell histiocytosis (LCH) in adults, which is often seen as an isolated condition with smoking being a major risk factor, isolated pPLCH is vanishingly rare in children and it is most often a component of multisystem LCH. Diagnosis should be based on histological and immunophenotypic examination of affected tissue in addition to clinical and radiological features. It should be considered an important differential for diffuse cystic lung disease in paediatric patients. Recent progress in the biological understanding of the disease supports the classification of LCH as an inflammatory myeloid neoplasia. Chemotherapy and specific management of respiratory complications are the mainstays of treatment. The lungs are no longer considered a “risk organ” in LCH as pulmonary involvement is not associated with a worse prognosis than the involvement of other organs. Multidisciplinary treatment approaches are needed.Prognosis can be good but is adversely influenced by multisystem involvement, and complications such as pneumothoraces and respiratory failure can be life threatening. This review aims to give an overview of this condition, with a focus on the diagnosis, monitoring and management of complications such as pneumothoraces and respiratory failure, which can be challenging for the paediatric respiratory specialist.Educational aimsTo give an overview of paediatric pulmonary LCH.To discuss the differential diagnosis of paediatric cystic lung disease.

scholarly journals Lung cysts in chronic paracoccidioidomycosis

2013 ◽  
Vol 39 (3) ◽  
pp. 368-372 ◽  
Author(s):  
Andre Nathan Costa ◽  
Edson Marchiori ◽  
Gil Benard ◽  
Mariana Sponholz Araujo ◽  
Bruno Guedes Baldi ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Retrospective Analysis ◽  
Interstitial Pneumonia ◽  
Langerhans Cell Histiocytosis ◽  
Lung Diseases ◽  
Lung Parenchyma ◽  
Langerhans Cell ◽  
Lung Cysts ◽  
Cystic Lung ◽  
Lymphocytic Interstitial Pneumonia

On HRCT scans, lung cysts are characterized by rounded areas of low attenuation in the lung parenchyma and a well-defined interface with the normal adjacent lung. The most common cystic lung diseases are lymphangioleiomyomatosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. In a retrospective analysis of the HRCT findings in 50 patients diagnosed with chronic paracoccidioidomycosis, we found lung cysts in 5 cases (10%), indicating that patients with paracoccidioidomycosis can present with lung cysts on HRCT scans. Therefore, paracoccidioidomycosis should be included in the differential diagnosis of cystic lung diseases.

scholarly journals Revisiting Cystic Lung Disease - A Review on Diagnostic Approach in High Resolution Computed Tomography Thorax

2021 ◽  
Vol 10 (19) ◽  
pp. 1435-1443
Author(s):  
Rajoo Ramachandran ◽  
Jeffrey Ralph ◽  
Rajeev Pulimi ◽  
Logesh Rajamani ◽  
Prabhu Radhan ◽  
...  
Keyword(s):  
Computed Tomography ◽  
High Resolution ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Ground Glass ◽  
High Resolution Computed Tomography ◽  
Lung Cysts ◽  
Cystic Lung ◽  
Cystic Lung Disease ◽  
Lung Abnormalities

The differential diagnosis of the cystic lung disease is extensive, ranging from an isolated incidental lung cyst to multiple diffuse lung cysts with other varied associated lung abnormalities. High resolution computed tomography (HRCT) thorax is the imaging modality of choice in the evaluation of these diseases, by improving the characterisation of the lung cysts (number, size, shape, distribution, wall thickness and regularity), and associated pulmonary and extrapulmonary abnormalities (like ground glass densities, nodules, interstitial fibrosis, mediastinal lymphadenopathy). After differentiating from the common cyst mimickers (cavity, centrilobular emphysema and cystic bronchiectasis), they should be grouped by location into subpleural cysts (paraseptal emphysema, bulla and honeycombing) and intraparenchymal cysts. Intraparenchymal cysts are in turn categorised into solitary / localised cysts (incidental cyst, congenital pulmonary airway malformations, intrapulmonary bronchogenic cyst and hydatid cyst) and multiple diffuse cysts. The next step will be the categorisation of these multiple diffuse cysts into those without other lung abnormalities (lymphangioleiomyomatosis and Birt-Hogg-Dube syndrome) and those with other lung abnormalities such as predominant ground glass densities (Pneumocystis jirovecii pneumonia, desquamative interstitial pneumonia, lymphoid interstitial pneumonia, subacute hypersensitivity pneumonitis and pneumatoceles) or predominant nodules (pulmonary Langerhans cell histiocytosis, amyloidosis, light chain deposition disease, follicular bronchiolitis, recurrent respiratory papillomatosis, cystic tuberculosis, cystic lung metastasis, sarcoidosis, and pulmonary mesenchymal cystic hamartomas). We conclude that this orderly radiologic approach in a given HRCT chest study of a cystic lung disease (in addition to correlation with clinical and laboratory findings), can lead us to a specific diagnosis in majority of instances, thereby optimising their treatment management, without the need of an invasive biopsy. KEY WORDS HRCT Thorax, Lung Cysts, Ground Glass Densities, Lung Nodules

scholarly journals Diffuse cystic lung diseases: differential diagnosis

2017 ◽  
Vol 43 (2) ◽  
pp. 140-149 ◽  
Author(s):  
Bruno Guedes Baldi ◽  
Carlos Roberto Ribeiro Carvalho ◽  
Olívia Meira Dias ◽  
Edson Marchiori ◽  
Bruno Hochhegger
Keyword(s):  
Lung Diseases ◽  
Laboratory Findings ◽  
Lung Cysts ◽  
Lung Lobe ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Pulmonary Cysts ◽  
Lymphocytic Interstitial Pneumonia ◽  
Definition Of ◽  
Airway Walls

ABSTRACT Diffuse cystic lung diseases are characterized by cysts in more than one lung lobe, the cysts originating from various mechanisms, including the expansion of the distal airspaces due to airway obstruction, necrosis of the airway walls, and parenchymal destruction. The progression of these diseases is variable. One essential tool in the evaluation of these diseases is HRCT, because it improves the characterization of pulmonary cysts (including their distribution, size, and length) and the evaluation of the regularity of the cyst wall, as well as the identification of associated pulmonary and extrapulmonary lesions. When combined with clinical and laboratory findings, HRCT is often sufficient for the etiological definition of diffuse lung cysts, avoiding the need for lung biopsy. The differential diagnoses of diffuse cystic lung diseases are myriad, including neoplastic, inflammatory, and infectious etiologies. Pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, lymphocytic interstitial pneumonia, and follicular bronchiolitis are the most common diseases that produce this CT pattern. However, new diseases have been included as potential determinants of this pattern.

scholarly journals Three’s company: A woman with rash, uterine mass and cystic lungs

2020 ◽  
Vol 90 (1) ◽  
Author(s):  
Laura Miranda ◽  
Sarwat Gilani ◽  
Liying Han ◽  
Oleg Epelbaum
Keyword(s):  
Autoimmune Disease ◽  
Lung Disease ◽  
Endometrial Carcinoma ◽  
Uterine Cancer ◽  
Lung Cysts ◽  
Cystic Lung ◽  
Cystic Lung Disease ◽  
Instructive Case ◽  
Three Components

Cystic lung disease encompasses a wide variety of clinical entities, the diagnosis of which is sometimes straightforward and other times obscure.  To narrow the list of possibilities, it behooves the physician to consider the context in which the cystic lung disease is uncovered. Clues to the diagnosis might be provided by findings that are not initially obvious and are not located in the thorax. We describe an instructive case of a woman with cystic lungs detected during a search for malignancy prompted by a diagnosis of dermatomyositis. Malignancy was indeed uncovered in the form of endometrial carcinoma, the management of which eventually also established the etiology of cystic lung disease. In the discussion we attempt to connect the patient’s autoimmune disease, uterine cancer, and lung cysts. The potential interplay among these three components of her presentation makes for intriguing mechanistic speculation.

Sign in / Sign up

Export Citation Format

Share Document