Multiple cystic lung disease

Francisco, Flávia Angélica Ferreira; Souza, Arthur Soares; Zanetti, Gláucia; Marchiori, Edson

doi:10.1183/16000617.0046-2015

Cited by 55 publications

(42 citation statements)

References 115 publications

(196 reference statements)

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“…Infiltration and obstruction of lymphatics and small airways is described in lymphangioleiomyomatosis and thought to cause cysts through a “ball‐valve” effect 3. While lymphatic and small airway obstruction was not evident at autopsy in this case, obstruction by mucin filled alveoli may have contributed to a similar effect.…”

Section: Discussionmentioning

confidence: 64%

Metastatic pancreatic carcinoma masquerading as cystic lung disease: a rare presentation

Stern

Huseini

Kuok

et al. 2017

Respirology Case Reports

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This 52‐year‐old male ex‐smoker presented with a six‐month history of progressive breathlessness and weight loss. He deteriorated acutely, and was admitted with severe type 1 respiratory failure. Apart from diffuse coarse crackles on chest auscultation, physical examination was unremarkable. High‐resolution computed tomography (HRCT) showed diffuse cystic changes throughout the lungs. A diagnosis of pulmonary Langerhans cell histiocytosis (PLCH) was considered. Further workup identified a coincidental pancreatic lesion of uncertain significance, which remained indeterminate on magnetic resonance imaging (MRI) and on positron emission tomography (PET). Transbronchial biopsy revealed enteric differentiated adenocarcinoma exhibiting lepidic spread, and autopsy later confirmed primary pancreatic malignancy. This case demonstrates that metastatic pancreatic malignancy can present with severe respiratory failure and masquerade as cystic lung disease.

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Section: Discussionmentioning

confidence: 64%

Metastatic pancreatic carcinoma masquerading as cystic lung disease: a rare presentation

Stern

Huseini

Kuok

et al. 2017

Respirology Case Reports

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“…Cystic lesions predominate in the upper lobes, sparing the region of the costophrenic sinuses (Figure 1C). Pneumothorax can occur in up to 15% of cases (Figure 1D) 3 6 It is speculated that the cysts are formed by airway dilatation due to bronchiolar wall inflammation, nodule cavitation, or air space enlargement secondary to traction from fibrosing lesions.…”

Section: Pulmonary Langerhans Cell Histiocytosismentioning

confidence: 99%

Diffuse cystic lung diseases: differential diagnosis

et al. 2017

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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.

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“…The diagnosis can be missed in some occasions when the rare causes of cystic lung diseases (like metastasis) are overlooked. Different mechanisms have been implicated in the pathogenesis of lung cysts, these include (1) check-valve obstruction (allows air flow in one direction) with distal overinflation, (2) infiltration and obstruction of small vessels and capillaries supplying the terminal bronchioles which lead to necrosis and ischaemic dilation of small airways and alveoli, progressing to cyst formation, (3) lung remodelling due to connective tissue degradation and elastolysis by matrix Metalloproteinases and other matrix-degrading enzymes2 and (4) excavation of a metastatic nodule3 which is the likely mechanism in our case given the prior presence of lung nodules on PET-CT scan.…”

Section: Descriptionmentioning

confidence: 99%