Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan

Objectives Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexis...
Ausführliche Beschreibung

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Autor*in:	Castoldi, M. C. [verfasserIn] Verrioli, A. De Juli, E. Vanzulli, A.

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2014

Schlagwörter:	Histiocytosis Interstitial lung disease Cysts Nodule Lung

Anmerkung:	© The Author(s) 2014

Übergeordnetes Werk:	Enthalten in: Insights into imaging - Berlin : Springer, 2010, 5(2014), 4 vom: 05. Juli, Seite 483-492
Übergeordnetes Werk:	volume:5 ; year:2014 ; number:4 ; day:05 ; month:07 ; pages:483-492

Links:	Volltext

DOI / URN:	10.1007/s13244-014-0338-0

Katalog-ID:	SPR031003222

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520			\|a Objectives Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexist and predominate in the upper and middle lungs. Methods This article includes a pictorial essay of typical and atypical presentations of PLCH at initial chest CT. Various appearances of PLCH are illustrated and possible differential diagnosis is discussed. Results PLCH can present with some aspecific features that may cause diagnosis of the initial disease to be overlooked or other pulmonary diseases to be suspected. In cases of nodule presentation alone, the main differential diagnosis should include lung metastasis, tuberculosis and other infections, sarcoidosis, silicosis and Wegener’s disease. In cases of cysts alone, the most common diseases to be differentiated are centrilobular emphysema and lymphangiomyomatosis. Clinical symptoms are usually non-specific, although a history of cigarette smoking, coupled with the presence of typical or suggestive findings at imaging, is key to suspecting the disease. Atypical presentations require surgical biopsy for diagnosis. Conclusions The radiologist should be familiar with PLCH imaging features to correctly diagnose the disease or need for further investigation. Teaching Points • PLCH is a rare interstitial smoking-related disease that usually affects young adults. • The typical first CT shows a mix of nodules, cavitary nodules and cysts in the upper-middle lungs. • Atypical appearance, either cysts or nodules alone, mandates that other diagnoses be considered. • Lung cystic involvement correlates with lung function abnormalities and predicts functional decline. • Integration of the clinical history and imaging results is key to diagnosis.
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allfields	10.1007/s13244-014-0338-0 doi (DE-627)SPR031003222 (SPR)s13244-014-0338-0-e DE-627 ger DE-627 rakwb eng Castoldi, M. C. verfasserin aut Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2014 Objectives Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexist and predominate in the upper and middle lungs. Methods This article includes a pictorial essay of typical and atypical presentations of PLCH at initial chest CT. Various appearances of PLCH are illustrated and possible differential diagnosis is discussed. Results PLCH can present with some aspecific features that may cause diagnosis of the initial disease to be overlooked or other pulmonary diseases to be suspected. In cases of nodule presentation alone, the main differential diagnosis should include lung metastasis, tuberculosis and other infections, sarcoidosis, silicosis and Wegener’s disease. In cases of cysts alone, the most common diseases to be differentiated are centrilobular emphysema and lymphangiomyomatosis. Clinical symptoms are usually non-specific, although a history of cigarette smoking, coupled with the presence of typical or suggestive findings at imaging, is key to suspecting the disease. Atypical presentations require surgical biopsy for diagnosis. Conclusions The radiologist should be familiar with PLCH imaging features to correctly diagnose the disease or need for further investigation. Teaching Points • PLCH is a rare interstitial smoking-related disease that usually affects young adults. • The typical first CT shows a mix of nodules, cavitary nodules and cysts in the upper-middle lungs. • Atypical appearance, either cysts or nodules alone, mandates that other diagnoses be considered. • Lung cystic involvement correlates with lung function abnormalities and predicts functional decline. • Integration of the clinical history and imaging results is key to diagnosis. Histiocytosis (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Cysts (dpeaa)DE-He213 Nodule (dpeaa)DE-He213 Lung (dpeaa)DE-He213 Verrioli, A. aut De Juli, E. aut Vanzulli, A. aut Enthalten in Insights into imaging Berlin : Springer, 2010 5(2014), 4 vom: 05. Juli, Seite 483-492 (DE-627)621547425 (DE-600)2543323-4 1869-4101 nnns volume:5 year:2014 number:4 day:05 month:07 pages:483-492 https://dx.doi.org/10.1007/s13244-014-0338-0 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4277 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 5 2014 4 05 07 483-492
spelling	10.1007/s13244-014-0338-0 doi (DE-627)SPR031003222 (SPR)s13244-014-0338-0-e DE-627 ger DE-627 rakwb eng Castoldi, M. C. verfasserin aut Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2014 Objectives Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexist and predominate in the upper and middle lungs. Methods This article includes a pictorial essay of typical and atypical presentations of PLCH at initial chest CT. Various appearances of PLCH are illustrated and possible differential diagnosis is discussed. Results PLCH can present with some aspecific features that may cause diagnosis of the initial disease to be overlooked or other pulmonary diseases to be suspected. In cases of nodule presentation alone, the main differential diagnosis should include lung metastasis, tuberculosis and other infections, sarcoidosis, silicosis and Wegener’s disease. In cases of cysts alone, the most common diseases to be differentiated are centrilobular emphysema and lymphangiomyomatosis. Clinical symptoms are usually non-specific, although a history of cigarette smoking, coupled with the presence of typical or suggestive findings at imaging, is key to suspecting the disease. Atypical presentations require surgical biopsy for diagnosis. Conclusions The radiologist should be familiar with PLCH imaging features to correctly diagnose the disease or need for further investigation. Teaching Points • PLCH is a rare interstitial smoking-related disease that usually affects young adults. • The typical first CT shows a mix of nodules, cavitary nodules and cysts in the upper-middle lungs. • Atypical appearance, either cysts or nodules alone, mandates that other diagnoses be considered. • Lung cystic involvement correlates with lung function abnormalities and predicts functional decline. • Integration of the clinical history and imaging results is key to diagnosis. Histiocytosis (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Cysts (dpeaa)DE-He213 Nodule (dpeaa)DE-He213 Lung (dpeaa)DE-He213 Verrioli, A. aut De Juli, E. aut Vanzulli, A. aut Enthalten in Insights into imaging Berlin : Springer, 2010 5(2014), 4 vom: 05. Juli, Seite 483-492 (DE-627)621547425 (DE-600)2543323-4 1869-4101 nnns volume:5 year:2014 number:4 day:05 month:07 pages:483-492 https://dx.doi.org/10.1007/s13244-014-0338-0 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4277 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 5 2014 4 05 07 483-492
allfields_unstemmed	10.1007/s13244-014-0338-0 doi (DE-627)SPR031003222 (SPR)s13244-014-0338-0-e DE-627 ger DE-627 rakwb eng Castoldi, M. C. verfasserin aut Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2014 Objectives Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexist and predominate in the upper and middle lungs. Methods This article includes a pictorial essay of typical and atypical presentations of PLCH at initial chest CT. Various appearances of PLCH are illustrated and possible differential diagnosis is discussed. Results PLCH can present with some aspecific features that may cause diagnosis of the initial disease to be overlooked or other pulmonary diseases to be suspected. In cases of nodule presentation alone, the main differential diagnosis should include lung metastasis, tuberculosis and other infections, sarcoidosis, silicosis and Wegener’s disease. In cases of cysts alone, the most common diseases to be differentiated are centrilobular emphysema and lymphangiomyomatosis. Clinical symptoms are usually non-specific, although a history of cigarette smoking, coupled with the presence of typical or suggestive findings at imaging, is key to suspecting the disease. Atypical presentations require surgical biopsy for diagnosis. Conclusions The radiologist should be familiar with PLCH imaging features to correctly diagnose the disease or need for further investigation. Teaching Points • PLCH is a rare interstitial smoking-related disease that usually affects young adults. • The typical first CT shows a mix of nodules, cavitary nodules and cysts in the upper-middle lungs. • Atypical appearance, either cysts or nodules alone, mandates that other diagnoses be considered. • Lung cystic involvement correlates with lung function abnormalities and predicts functional decline. • Integration of the clinical history and imaging results is key to diagnosis. Histiocytosis (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Cysts (dpeaa)DE-He213 Nodule (dpeaa)DE-He213 Lung (dpeaa)DE-He213 Verrioli, A. aut De Juli, E. aut Vanzulli, A. aut Enthalten in Insights into imaging Berlin : Springer, 2010 5(2014), 4 vom: 05. Juli, Seite 483-492 (DE-627)621547425 (DE-600)2543323-4 1869-4101 nnns volume:5 year:2014 number:4 day:05 month:07 pages:483-492 https://dx.doi.org/10.1007/s13244-014-0338-0 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4277 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 5 2014 4 05 07 483-492
allfieldsGer	10.1007/s13244-014-0338-0 doi (DE-627)SPR031003222 (SPR)s13244-014-0338-0-e DE-627 ger DE-627 rakwb eng Castoldi, M. C. verfasserin aut Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2014 Objectives Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexist and predominate in the upper and middle lungs. Methods This article includes a pictorial essay of typical and atypical presentations of PLCH at initial chest CT. Various appearances of PLCH are illustrated and possible differential diagnosis is discussed. Results PLCH can present with some aspecific features that may cause diagnosis of the initial disease to be overlooked or other pulmonary diseases to be suspected. In cases of nodule presentation alone, the main differential diagnosis should include lung metastasis, tuberculosis and other infections, sarcoidosis, silicosis and Wegener’s disease. In cases of cysts alone, the most common diseases to be differentiated are centrilobular emphysema and lymphangiomyomatosis. Clinical symptoms are usually non-specific, although a history of cigarette smoking, coupled with the presence of typical or suggestive findings at imaging, is key to suspecting the disease. Atypical presentations require surgical biopsy for diagnosis. Conclusions The radiologist should be familiar with PLCH imaging features to correctly diagnose the disease or need for further investigation. Teaching Points • PLCH is a rare interstitial smoking-related disease that usually affects young adults. • The typical first CT shows a mix of nodules, cavitary nodules and cysts in the upper-middle lungs. • Atypical appearance, either cysts or nodules alone, mandates that other diagnoses be considered. • Lung cystic involvement correlates with lung function abnormalities and predicts functional decline. • Integration of the clinical history and imaging results is key to diagnosis. Histiocytosis (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Cysts (dpeaa)DE-He213 Nodule (dpeaa)DE-He213 Lung (dpeaa)DE-He213 Verrioli, A. aut De Juli, E. aut Vanzulli, A. aut Enthalten in Insights into imaging Berlin : Springer, 2010 5(2014), 4 vom: 05. Juli, Seite 483-492 (DE-627)621547425 (DE-600)2543323-4 1869-4101 nnns volume:5 year:2014 number:4 day:05 month:07 pages:483-492 https://dx.doi.org/10.1007/s13244-014-0338-0 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4277 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 5 2014 4 05 07 483-492
allfieldsSound	10.1007/s13244-014-0338-0 doi (DE-627)SPR031003222 (SPR)s13244-014-0338-0-e DE-627 ger DE-627 rakwb eng Castoldi, M. C. verfasserin aut Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2014 Objectives Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexist and predominate in the upper and middle lungs. Methods This article includes a pictorial essay of typical and atypical presentations of PLCH at initial chest CT. Various appearances of PLCH are illustrated and possible differential diagnosis is discussed. Results PLCH can present with some aspecific features that may cause diagnosis of the initial disease to be overlooked or other pulmonary diseases to be suspected. In cases of nodule presentation alone, the main differential diagnosis should include lung metastasis, tuberculosis and other infections, sarcoidosis, silicosis and Wegener’s disease. In cases of cysts alone, the most common diseases to be differentiated are centrilobular emphysema and lymphangiomyomatosis. Clinical symptoms are usually non-specific, although a history of cigarette smoking, coupled with the presence of typical or suggestive findings at imaging, is key to suspecting the disease. Atypical presentations require surgical biopsy for diagnosis. Conclusions The radiologist should be familiar with PLCH imaging features to correctly diagnose the disease or need for further investigation. Teaching Points • PLCH is a rare interstitial smoking-related disease that usually affects young adults. • The typical first CT shows a mix of nodules, cavitary nodules and cysts in the upper-middle lungs. • Atypical appearance, either cysts or nodules alone, mandates that other diagnoses be considered. • Lung cystic involvement correlates with lung function abnormalities and predicts functional decline. • Integration of the clinical history and imaging results is key to diagnosis. Histiocytosis (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Cysts (dpeaa)DE-He213 Nodule (dpeaa)DE-He213 Lung (dpeaa)DE-He213 Verrioli, A. aut De Juli, E. aut Vanzulli, A. aut Enthalten in Insights into imaging Berlin : Springer, 2010 5(2014), 4 vom: 05. Juli, Seite 483-492 (DE-627)621547425 (DE-600)2543323-4 1869-4101 nnns volume:5 year:2014 number:4 day:05 month:07 pages:483-492 https://dx.doi.org/10.1007/s13244-014-0338-0 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4277 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 5 2014 4 05 07 483-492
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Clinical symptoms are usually non-specific, although a history of cigarette smoking, coupled with the presence of typical or suggestive findings at imaging, is key to suspecting the disease. Atypical presentations require surgical biopsy for diagnosis. Conclusions The radiologist should be familiar with PLCH imaging features to correctly diagnose the disease or need for further investigation. 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author	Castoldi, M. C.
spellingShingle	Castoldi, M. C. misc Histiocytosis misc Interstitial lung disease misc Cysts misc Nodule misc Lung Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan
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topic_title	Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan Histiocytosis (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Cysts (dpeaa)DE-He213 Nodule (dpeaa)DE-He213 Lung (dpeaa)DE-He213
topic	misc Histiocytosis misc Interstitial lung disease misc Cysts misc Nodule misc Lung
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title	Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan
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title_full	Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan
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author_browse	Castoldi, M. C. Verrioli, A. De Juli, E. Vanzulli, A.
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title_sort	pulmonary langerhans cell histiocytosis: the many faces of presentation at initial ct scan
title_auth	Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan
abstract	Objectives Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexist and predominate in the upper and middle lungs. Methods This article includes a pictorial essay of typical and atypical presentations of PLCH at initial chest CT. Various appearances of PLCH are illustrated and possible differential diagnosis is discussed. Results PLCH can present with some aspecific features that may cause diagnosis of the initial disease to be overlooked or other pulmonary diseases to be suspected. In cases of nodule presentation alone, the main differential diagnosis should include lung metastasis, tuberculosis and other infections, sarcoidosis, silicosis and Wegener’s disease. In cases of cysts alone, the most common diseases to be differentiated are centrilobular emphysema and lymphangiomyomatosis. Clinical symptoms are usually non-specific, although a history of cigarette smoking, coupled with the presence of typical or suggestive findings at imaging, is key to suspecting the disease. Atypical presentations require surgical biopsy for diagnosis. Conclusions The radiologist should be familiar with PLCH imaging features to correctly diagnose the disease or need for further investigation. Teaching Points • PLCH is a rare interstitial smoking-related disease that usually affects young adults. • The typical first CT shows a mix of nodules, cavitary nodules and cysts in the upper-middle lungs. • Atypical appearance, either cysts or nodules alone, mandates that other diagnoses be considered. • Lung cystic involvement correlates with lung function abnormalities and predicts functional decline. • Integration of the clinical history and imaging results is key to diagnosis. © The Author(s) 2014
abstractGer	Objectives Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexist and predominate in the upper and middle lungs. Methods This article includes a pictorial essay of typical and atypical presentations of PLCH at initial chest CT. Various appearances of PLCH are illustrated and possible differential diagnosis is discussed. Results PLCH can present with some aspecific features that may cause diagnosis of the initial disease to be overlooked or other pulmonary diseases to be suspected. In cases of nodule presentation alone, the main differential diagnosis should include lung metastasis, tuberculosis and other infections, sarcoidosis, silicosis and Wegener’s disease. In cases of cysts alone, the most common diseases to be differentiated are centrilobular emphysema and lymphangiomyomatosis. Clinical symptoms are usually non-specific, although a history of cigarette smoking, coupled with the presence of typical or suggestive findings at imaging, is key to suspecting the disease. Atypical presentations require surgical biopsy for diagnosis. Conclusions The radiologist should be familiar with PLCH imaging features to correctly diagnose the disease or need for further investigation. Teaching Points • PLCH is a rare interstitial smoking-related disease that usually affects young adults. • The typical first CT shows a mix of nodules, cavitary nodules and cysts in the upper-middle lungs. • Atypical appearance, either cysts or nodules alone, mandates that other diagnoses be considered. • Lung cystic involvement correlates with lung function abnormalities and predicts functional decline. • Integration of the clinical history and imaging results is key to diagnosis. © The Author(s) 2014
abstract_unstemmed	Objectives Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexist and predominate in the upper and middle lungs. Methods This article includes a pictorial essay of typical and atypical presentations of PLCH at initial chest CT. Various appearances of PLCH are illustrated and possible differential diagnosis is discussed. Results PLCH can present with some aspecific features that may cause diagnosis of the initial disease to be overlooked or other pulmonary diseases to be suspected. In cases of nodule presentation alone, the main differential diagnosis should include lung metastasis, tuberculosis and other infections, sarcoidosis, silicosis and Wegener’s disease. In cases of cysts alone, the most common diseases to be differentiated are centrilobular emphysema and lymphangiomyomatosis. Clinical symptoms are usually non-specific, although a history of cigarette smoking, coupled with the presence of typical or suggestive findings at imaging, is key to suspecting the disease. Atypical presentations require surgical biopsy for diagnosis. Conclusions The radiologist should be familiar with PLCH imaging features to correctly diagnose the disease or need for further investigation. Teaching Points • PLCH is a rare interstitial smoking-related disease that usually affects young adults. • The typical first CT shows a mix of nodules, cavitary nodules and cysts in the upper-middle lungs. • Atypical appearance, either cysts or nodules alone, mandates that other diagnoses be considered. • Lung cystic involvement correlates with lung function abnormalities and predicts functional decline. • Integration of the clinical history and imaging results is key to diagnosis. © The Author(s) 2014
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title_short	Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan
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Teaching Points • PLCH is a rare interstitial smoking-related disease that usually affects young adults. • The typical first CT shows a mix of nodules, cavitary nodules and cysts in the upper-middle lungs. • Atypical appearance, either cysts or nodules alone, mandates that other diagnoses be considered. • Lung cystic involvement correlates with lung function abnormalities and predicts functional decline. • Integration of the clinical history and imaging results is key to diagnosis.</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Histiocytosis</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Interstitial lung disease</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Cysts</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Nodule</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Lung</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Verrioli, A.</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">De Juli, E.</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Vanzulli, A.</subfield><subfield code="4">aut</subfield></datafield><datafield tag="773" ind1="0" ind2="8"><subfield code="i">Enthalten in</subfield><subfield code="t">Insights into imaging</subfield><subfield code="d">Berlin : Springer, 2010</subfield><subfield code="g">5(2014), 4 vom: 05. 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Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan

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