{"id":502,"date":"2018-01-03T12:45:33","date_gmt":"2018-01-03T17:45:33","guid":{"rendered":"http:\/\/uchealth.com\/pulmonary-insights\/?p=502"},"modified":"2018-01-15T10:32:15","modified_gmt":"2018-01-15T15:32:15","slug":"hrct-is-cost-effective-in-screening-for-diffuse-cystic-lung-diseases-in-patients-presenting-with-a-spontaneous-pneumothorax","status":"publish","type":"post","link":"https:\/\/www.uchealth.com\/pulmonary-insights\/hrct-is-cost-effective-in-screening-for-diffuse-cystic-lung-diseases-in-patients-presenting-with-a-spontaneous-pneumothorax\/","title":{"rendered":"HRCT is Cost-Effective in Screening for Diffuse Cystic Lung Diseases in Patients Presenting with a Spontaneous Pneumothorax"},"content":{"rendered":"<p>\u201cA strategy of performing high-resolution computed tomography [HRCT] scanning followed by pleurodesis is cost-effective in screening for the presence of diffuse cystic lung diseases (DCLDs) in patients who present with an apparent primary spontaneous pneumothorax (PSP),\u201d said<a href=\"https:\/\/www.uchealth.com\/physician\/nishant-gupta\/\"> Nishant Gupta, MD<\/a>, assistant professor of medicine and director of the Interstitial Lung Diseases Center, <a href=\"https:\/\/www.uchealth.com\/university-of-cincinnati-medical-center\/\">University of Cincinnati (UC) Medical Center<\/a>.<\/p>\n<div id=\"attachment_508\" style=\"width: 327px\" class=\"wp-caption alignright\"><a href=\"\/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image-1.png\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-508\" class=\"wp-image-508\" src=\"\/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image-1.png\" alt=\"\" width=\"317\" height=\"201\" srcset=\"\/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image-1.png 1279w, \/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image-1-300x191.png 300w, \/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image-1-768x488.png 768w, \/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image-1-1024x651.png 1024w\" sizes=\"auto, (max-width: 317px) 100vw, 317px\" \/><\/a><p id=\"caption-attachment-508\" class=\"wp-caption-text\"><strong>Figure 1<\/strong>: HRCT scan in a patient with LAM showing the characteristic thin-walled, round, uniform cysts present bilaterally in a diffuse distribution.<\/p><\/div>\n<p>Dr. Gupta and his UC Medical Center co-authors reached this conclusion in a 2017 study that assessed the cost-effectiveness of HRCT\u00a0chest imaging in the early diagnosis of Birt-Hogg-Dub\u00e9 syndrome (BHD), lymphangioleiomyomatosis (LAM), and pulmonary Langerhans cell histiocytosis (PLCH)<sup>1<\/sup>.<\/p>\n<p>According to the 2001 American College of Chest Physicians guidelines<sup>2<\/sup>, routine CT scans to evaluate for the presence of an underlying lung disease are not recommended in patients presenting with an apparent PSP. However, spontaneous pneumothorax is a common mode of presentation among patients with DCLDs, and \u201cDCLDs such as BHD, LAM, and PLCH can be the underlying etiology of the pneumothorax in approximately 8-10% of the patients presenting with an apparent PSP,\u201d Dr. Gupta explained<sup>1<\/sup>.<\/p>\n<div id=\"attachment_509\" style=\"width: 335px\" class=\"wp-caption alignright\"><a href=\"\/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image.png\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-509\" class=\"wp-image-509\" src=\"\/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image.png\" alt=\"\" width=\"325\" height=\"513\" srcset=\"\/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image.png 671w, \/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image-190x300.png 190w, \/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image-649x1024.png 649w\" sizes=\"auto, (max-width: 325px) 100vw, 325px\" \/><\/a><p id=\"caption-attachment-509\" class=\"wp-caption-text\"><strong>Figure 2<\/strong>: HRCT scans in two patients with BHD showing the characteristic oval\/lentiform cysts present in a lower lobe distribution. Notice the predilection for pulmonary vasculature (2A, arrow), and the pleural surface (2B, arrow).<\/p><\/div>\n<p>HRCT has significantly improved the ability of clinicians to diagnose these three diseases.3 A critical review of HRCT can reveal cyst characteristics that are unique to BHD, LAM and PLCH. For example, the cysts in LAM are thin-walled, round, uniform, and present diffusely in both lungs (Figure 1). In contrast, the cysts in PLCH tend to be more irregular in shape, can have thin as well thick walls, may have associated nodules, and are present in an upper-lobe predominant distribution (Figure 2). In patients with BHD, the pulmonary cysts are typically less numerous than LAM, tend to be more oval\/lentiform in shape, are present mainly in the lower lobes, and have a strong predilection for the subpleural areas and the pulmonary vasculature (Figure 3)<sup>4,5<\/sup>.<\/p>\n<div id=\"attachment_505\" style=\"width: 339px\" class=\"wp-caption alignright\"><a href=\"\/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image-3.png\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-505\" class=\"wp-image-505\" src=\"\/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image-3.png\" alt=\"\" width=\"329\" height=\"247\" srcset=\"\/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image-3.png 1224w, \/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image-3-300x225.png 300w, \/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image-3-768x577.png 768w, \/wp-content\/uploads\/sites\/39\/2018\/01\/HRCT-image-3-1024x770.png 1024w\" sizes=\"auto, (max-width: 329px) 100vw, 329px\" \/><\/a><p id=\"caption-attachment-505\" class=\"wp-caption-text\"><strong>Figure 3<\/strong>: HRCT scan in a patient with PLCH showing the characteristic irregular\/bizarre shaped cysts.<\/p><\/div>\n<p>Early detection followed by timely pleurodesis (following the first episode of pneumothorax as opposed to waiting for a recurrent pneumothorax), said Dr. Gupta, would result in fewer pneumothorax recurrences. In patients with BHD, LAM and PLCH, the risk of recurrence after a first pneumothorax is 58%, 73% and 75%, respectively, as compared to a recurrence rate of approximately 30% in patients with PSP<sup>6-9<\/sup>. Dr. Gupta noted other disease-specific advantages to early detection. Because an effective, FDA approved treatment exists for patients with LAM<sup>10<\/sup>, early diagnosis could lead to drug intervention and prevent continued loss of lung function. BHD patients have a greater risk of renal cancer; earlier diagnosis would enable patients to enroll in surveillance programs for kidney tumors, as well as enable testing of asymptomatic family members for the presence of BHD<sup>5<\/sup>. In PLCH, which has a strong causal association with cigarette smoking, early diagnostic determination would facilitate counseling about smoking cessation, as well as provide an opportunity to screen for the presence of underlying genetic mutations.<sup>11<\/sup><\/p>\n<p>Dr. Gupta and his co-authors determined that using HRCT screening for LAM, BHD and PLCH followed by pleurodesis in patients presenting with an apparent PSP was both more costly and more effective. The marginal cost-effectiveness ratio of HRCT screening was $1,427 per quality-adjusted life-year (QALY) gained. Traditionally, strategies and interventions costing less than $50,000\/QALY are characterized as extremely cost-effective. Based on these findings, the authors recommend \u201cadult patients of all ages with an apparent PSP should undergo a screening chest HRCT scan to evaluate for the presence of underlying BHD, LAM, and PLCH.\u201d <sup>1<\/sup><\/p>\n<p><strong>References<\/strong>:<br \/>\n1. Gupta N, Langenderfer D, McCormack FX, Schauer DP, Eckman MH. <a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/27737563\">Chest Computed Tomographic Image Screening for Cystic Lung Diseases in Patients with Spontaneous Pneumothorax Is Cost Effective<\/a>. <em>Ann Am Thorac Soc<\/em> 2017;14:17-25.<br \/>\n2. Baumann MH, Strange C, Heffner JE, et al. <a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/11171742\">Management of spontaneous pneumothorax: an American College of Chest Physicians Delphi consensus statement<\/a>. <em>Chest<\/em> 2001;119:590-602.<br \/>\n3. Gupta N, Meraj R, Tanase D, et al. <a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/26160866\">Accuracy of chest high-resolution computed tomography in diagnosing diffuse cystic lung diseases<\/a>. Eur Respir J 2015;46:1196-9.<br \/>\n4. Gupta N, Vassallo R, Wikenheiser-Brokamp KA, McCormack FX. <a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/25906089\">Diffuse Cystic Lung Disease. Part I<\/a>. <em>Am J Respir Crit Care Med<\/em> 2015;191:1354-66.<br \/>\n5. Gupta N, Sunwoo BY, Kotloff RM. <a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/27514594\">Birt-Hogg-Dube Syndrome<\/a>. Clin Chest Med 2016;37:475-86.<br \/>\n6. Almoosa KF, Ryu JH, Mendez J, et al. <a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Management+of+pneumothorax+in+lymphangioleiomyomatosis%3A+effects+on+recurrence+and+lung+transplantation+complications\">Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications<\/a>. <em>Chest<\/em> 2006;129:1274-81.<br \/>\n7. Mendez JL, Nadrous HF, Vassallo R, Decker PA, Ryu JH. <a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/15006964\">Pneumothorax in pulmonary Langerhans cell histiocytosis<\/a>. <em>Chest<\/em> 2004;125:1028-32.<br \/>\n8. Toro JR, Pautler SE, Stewart L, et al. <a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/?term=Toro+JR%2C+Pautler+SE%2C+Stewart+L%2C+et+al.+Lung+cysts%2C+spontaneous+pneumothorax%2C+and+genetic+associations+in+89+families+with+Birt-Hogg-Dube+syndrome.+Am+J+Respir+Crit+Care+Med+2007%3B175%3A1044-53\">Lung cysts, spontaneous pneumothorax, and genetic associations in 89 families with Birt-Hogg-Dube syndrome<\/a>. <em>Am J Respir Crit Care Med<\/em> 2007;175:1044-53.<br \/>\n9. O&#8217;Rourke JP, Yee ES. <a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/2582835\">Civilian spontaneous pneumothorax. Treatment options and long-term results<\/a>. <em>Chest<\/em> 1989;96:1302-6.<br \/>\n10. McCormack FX, Inoue Y, Moss J, et al. <a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/21410393\">Efficacy and safety of sirolimus in lymphangioleiomyomatosis<\/a>. <em>N Engl J Med<\/em> 2011;364:1595-606.<br \/>\n11. Mourah S, How-Kit A, Meignin V, et al. <a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pubmed\/27076591\">Recurrent NRAS mutations in pulmonary Langerhans cell histiocytosis<\/a>. <em>Eur Respir J<\/em> 2016;47:1785-96.<\/p>\n<p><strong><a href=\"\/wp-content\/uploads\/sites\/39\/2018\/01\/Nishant-Gupta-MD.jpg\"><img loading=\"lazy\" decoding=\"async\" class=\" wp-image-511 alignleft\" src=\"\/wp-content\/uploads\/sites\/39\/2018\/01\/Nishant-Gupta-MD.jpg\" alt=\"\" width=\"138\" height=\"193\" srcset=\"\/wp-content\/uploads\/sites\/39\/2018\/01\/Nishant-Gupta-MD.jpg 1500w, \/wp-content\/uploads\/sites\/39\/2018\/01\/Nishant-Gupta-MD-214x300.jpg 214w, \/wp-content\/uploads\/sites\/39\/2018\/01\/Nishant-Gupta-MD-768x1075.jpg 768w, \/wp-content\/uploads\/sites\/39\/2018\/01\/Nishant-Gupta-MD-731x1024.jpg 731w\" sizes=\"auto, (max-width: 138px) 100vw, 138px\" \/><\/a>Nishant Gupta, MD, MS<\/strong><br \/>\nAssistant Professor of Medicine<br \/>\nDirector &#8211; Interstitial Lung Diseases Center,<br \/>\nDivision of Pulmonary Critical Care and Sleep Medicine<br \/>\nUniversity of Cincinnati Medical Center<br \/>\n<strong>PHONE<\/strong>: 513-558-4831<br \/>\n<strong>EMAIL<\/strong>: <a href=\"mailto:guptans@ucmail.uc.edu\">guptans@ucmail.uc.edu<\/a><\/p>\n<p><a href=\"https:\/\/www.doximity.com\/pub\/nishant-gupta-md\"><strong>Connect with Dr. Gupta on Doximity<\/strong><\/a><\/p>\n","protected":false},"excerpt":{"rendered":"<p>\u201cA strategy of performing high-resolution computed tomography [HRCT] scanning followed by pleurodesis is cost-effective in screening for the presence of diffuse cystic lung diseases (DCLDs) in patients who present with an apparent primary spontaneous pneumothorax (PSP),\u201d said Nishant Gupta, MD, assistant professor of medicine and director of the Interstitial Lung Diseases Center, University of Cincinnati<\/p>\n","protected":false},"author":78,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[1],"tags":[109,47,108,8,103,106,107],"class_list":["post-502","post","type-post","status-publish","format-standard","hentry","category-uncategorized","tag-dcld","tag-diffuse-cystic-lung-diseases","tag-hrct","tag-lam","tag-nishant-gupta","tag-primary-spontaneous-pneumothorax","tag-psp"],"aioseo_notices":[],"_links":{"self":[{"href":"https:\/\/www.uchealth.com\/pulmonary-insights\/wp-json\/wp\/v2\/posts\/502","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.uchealth.com\/pulmonary-insights\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.uchealth.com\/pulmonary-insights\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.uchealth.com\/pulmonary-insights\/wp-json\/wp\/v2\/users\/78"}],"replies":[{"embeddable":true,"href":"https:\/\/www.uchealth.com\/pulmonary-insights\/wp-json\/wp\/v2\/comments?post=502"}],"version-history":[{"count":8,"href":"https:\/\/www.uchealth.com\/pulmonary-insights\/wp-json\/wp\/v2\/posts\/502\/revisions"}],"predecessor-version":[{"id":528,"href":"https:\/\/www.uchealth.com\/pulmonary-insights\/wp-json\/wp\/v2\/posts\/502\/revisions\/528"}],"wp:attachment":[{"href":"https:\/\/www.uchealth.com\/pulmonary-insights\/wp-json\/wp\/v2\/media?parent=502"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.uchealth.com\/pulmonary-insights\/wp-json\/wp\/v2\/categories?post=502"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.uchealth.com\/pulmonary-insights\/wp-json\/wp\/v2\/tags?post=502"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}