Multiple Cystic Lung Diseases

Pulmonary Langerhans cell histiocytosis is a rare diffuse cystic interstitial pneumonia of unknown etiology that occurs selectively in young smokers of both genders. The multicenter studies conducted by the reference center have better defined the short and medium terms natural history of the disease and the clinical management of patients. A substantial proportion of patients experience a dramatic decline in their lung function soon after diagnosis. Importantly, smoking cessation is associated with a decreased risk of subsequent deterioration. Cladribine, a purine analogue, chemotherapy may dramatically improve lung function in patients with progressive pulmonary Langerhans cell histiocytosis, but this treatment should be used only in the setting of clinical research. Specific pulmonary hypertension therapies (anti-endothelin receptors, inhibitors of phosphodiesterases) may be used with caution in specialized centres for patients with severe pulmonary hypertension, and seem to be well tolerated. The recent identification of the V600E mutation of the BRAF oncogene in approximately half of the Langerhans cell histiocytosis lesions, including pulmonary granulomas, represents an important step forward in the understanding of the pathogenesis of Langerhans cell histiocytosis. Potentially it opens the way to targeted therapies.

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“…Chez ces patients, les lésions pulmonaires prédominent souvent dans les bases pulmonaires. [4,[37][38][39][40][41][42].…”

Section: Histopathologie Pulmonaireunclassified

“…Dans la majorité des cas, elle survient chez l'adulte jeune, le plus souvent sous une forme isolée, et représente la forme clinique rencontrée en pneumologie. Elle fait partie des pneumopathies infiltrantes diffuses kystiques [4].…”

Section: Introductionunclassified

Manifestations pulmonaires de l’histiocytose langerhansienne

Obert

Tazi

2015

Revue des Maladies Respiratoires

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“…( 1 ) The most common cystic lung diseases are lymphangioleiomyomatosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. ( 1 - 3 ) Paracoccidioidomycosis, however, does not currently feature on the list of differential diagnoses of cystic parenchymal lung diseases.…”

mentioning

confidence: 99%

“…These mechanisms would include vascular occlusion followed by ischemia and necrosis, bronchial dilatation, smooth muscle cell proliferation, and even a check-valve mechanism in small airways, which, because of inflammatory cell infiltration and subsequent centrilobular fibrosis, would lead to bronchial obstruction and dilatation downstream of the lesion. ( 2 , 3 , 20 ) It can be speculated that, in paracoccidioidomycosis, centrilobular fibrosis, with involvement of the small airways and small vessels ( 9 , 16 ) would cause bronchial obstruction and distal airway dilatation, similarly to the check-valve mechanism identified in bronchiolitis. ( 21 ) Another possible explanation would be the presence of peribronchial nodules or granulomas associated with mycosis, promoting dilatation of a small airway, with the consequent formation of lung cysts, similarly to what is observed in Langerhans cell histiocytosis.…”

mentioning

confidence: 99%

“…( 21 ) Another possible explanation would be the presence of peribronchial nodules or granulomas associated with mycosis, promoting dilatation of a small airway, with the consequent formation of lung cysts, similarly to what is observed in Langerhans cell histiocytosis. ( 2 , 3 ) Another plausible explanation would be that the cysts or pneumatoceles would result from an inflammatory process leading to central necrosis and elastic recoil of the adjacent lung tissue, with localized air expansion, whether in the airways or in the lung interstitium, as occurs in other infectious diseases, such as staphylococcal pneumonia. ( 3 ) …”

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confidence: 99%

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Lung cysts in chronic paracoccidioidomycosis

et al. 2013

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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.

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Association of Pulmonary Cysts and Nodules in a Young Female Patient

Dias

Nascimento

Carvalho

et al. 2016

Chest

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A 27-year-old female patient was referred to our outpatient clinic with a 1-year history of shortness of breath when walking fast on level ground or when climbing stairs. Symptoms worsened after a second episode of spontaneous left pneumothorax, when a chest tube was placed in another hospital for complete lung expansion. During this hospitalization, an open lung biopsy was performed. There was no history of rhinorrhea, nasal congestion, cough, hemoptysis, wheezing, or expectoration.

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Multiple Cystic Lung Diseases

Cited by 7 publications

References 153 publications

Manifestations pulmonaires de l’histiocytose langerhansienne

Manifestations pulmonaires de l’histiocytose langerhansienne

Lung cysts in chronic paracoccidioidomycosis

Association of Pulmonary Cysts and Nodules in a Young Female Patient

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