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Letter to the Editor
Cystic Lung Disease: The Importance of a Multidisciplinary Approach
Enfermedad quística del pulmón: la importancia de un enfoque multidisciplinar
Edson Marchiori
Corresponding author
edmarchiori@gmail.com

Corresponding author.
, Gláucia Zanetti, Miriam Menna Barreto
Departamento de Radiología, Federal University of Rio de Janeiro, Río de Janeiro, Brazil
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We read with great interest the well-argued letter to the editor from Herrero et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">1</span></a> who reported on the association between pulmonary cysts caused by lymphangioleiomyomatosis &#40;LAM&#41; and abdominal findings in two patients&#46; We would like to highlight some aspects related to these cases&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">The main differential diagnoses of cystic disease in LAM include pulmonary Langerhans&#8217; cell histiocytosis &#40;PLCH&#41;&#44; Birt&#8211;Hogg&#8211;Dub&#233; syndrome &#40;BHDS&#41;&#44; lymphoid interstitial pneumonia &#40;LIP&#41;&#44; and <span class="elsevierStyleItalic">Pneumocystis jiroveci</span> pneumonia&#46; The distribution of cysts&#44; along with ancillary findings from clinical and imaging studies&#44; may be helpful in distinguishing these entities&#46; PLCH presents with variably sized and often bizarrely shaped cysts&#46; Cysts predominate in the upper and middle lung zones&#44; sparing the costophrenic angles&#46; Nodules in the intervening lung are very suggestive of PLCH&#44; while in the setting of autoimmune disease&#44; particularly Sj&#246;gren syndrome&#44; cysts are suggestive of LIP&#46; LIP typically manifests with few thin-walled cysts&#44; predominantly in the lung bases&#44; and parenchymal changes&#44; such as reticulonodular and ground-glass opacities&#46; <span class="elsevierStyleItalic">P&#46; jiroveci</span> pneumonia is seen in patients with immunosuppression and typically presents on imaging studies with bilateral ground-glass opacities&#46; Cystic lesions&#44; or pneumatoceles&#44; seen in approximately 30&#37; of cases&#44; are typically transient and occur predominantly in the upper zones&#46; Spontaneous pneumothorax can also occur&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Among cystic lung diseases&#44; BHDS is the most difficult to differentiate from LAM&#44; especially when LAM is associated with tuberous sclerosis complex &#40;TSC&#41; with renal and cutaneous involvement&#46; Like BHDS&#44; TSC has a wide clinical spectrum&#46; It is an autosomal-dominant syndrome characterized by widespread hamartomatous lesions&#46; Any organ system can be involved&#44; but the prevalence of manifestations among specific organs differs between children and adults&#46; Patients with TSC usually present angiomyolipomas&#44; angiofibromas&#44; hypopigmented macules&#44; shagreen patch&#44; and&#47;or periungual fibromas&#46; Cysts in BHDS and LAM are thin-walled&#44; but those associated with LAM are smaller and more circular&#44; homogeneous&#44; and evenly distributed&#46; In BHDS&#44; thin-walled cysts&#44; often larger&#44; are distributed asymmetrically and predominate in the subpleural and paramediastinal regions of the lower lung zones&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">LAM is typically seen in adult women&#44; as in the two cases described by Herrero et al&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">1</span></a> In some cases&#44; pulmonary findings alone may not give an accurate diagnosis&#46; However&#44; TSC may be diagnosed without the aid of interventional procedures if associated findings in other organs or systems are carefully evaluated&#46; In view of the abdominal involvement reported by Herrero et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">1</span></a> these two patients probably presented TSC&#46; Careful histological analysis of the renal mass in the first case was extremely important for diagnosis&#46; In contrast to TSC&#44; in which tumors are usually benign&#44; BHDS carries an increased risk of renal carcinoma&#46; In conclusion&#44; a multidisciplinary approach is required for the diagnosis and assessment of TSC due to the possible involvement of various organs&#46; Assessment and monitoring of patients&#8217; relatives is also important&#44; as TSC is a genetic disease&#46;</p></span>"
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Article information
ISSN: 15792129
Original language: English
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2017 June 43 13 56
2017 May 29 7 36
2017 April 31 12 43
2017 March 23 39 62
2017 February 20 8 28
2017 January 17 8 25
2016 December 24 10 34
2016 November 43 28 71
2016 October 62 28 90
2016 September 39 6 45
2016 August 2 0 2
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Archivos de Bronconeumología

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