Rare diseases bullet 9: Adult pulmonary Langerhans' cell histiocytosis

Tazi, Abdellatif; Stolzmann, Paul; Hance, Allan J.

doi:10.1136/thorax.55.5.405

Cited by 144 publications

(122 citation statements)

References 105 publications

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“…Isolated pulmonary lesions, however, are distinctly rare under the age of 15 years [3, 4]. Instead, the majority of patients are young adult males with heavy smoking habits [5, 6]. Hence, isolated lung involvement suggests a role for inhaled antigens from cigarette smoke [7, 8].…”

Section: Introductionmentioning

confidence: 99%

Natural Course of Isolated Pulmonary Langerhans’ Cell Histiocytosis in a Toddler

et al. 2005

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Isolated pulmonary Langerhans’ cell histiocytosis (LCH) is distinctly rare under the age of 15 years, since the majority of patients are young adult males with heavy smoking habits. Isolated pulmonary involvement suggests that antigens inhaled from cigarette smoke are involved. Here we present a case of LCH restricted to the lungs in a toddler whose parents were heavy smokers. Since LCH was not medically treated for 3 years due to parental refusal, the disease can be regarded as having followed its natural course. During the 3-year follow-up, the disease progressed to severe pulmonary fibrosis resulting in honeycomb lungs. Based on the comparative immunohistochemical analyses of the cells obtained from bronchoalveolar lavages during the disease course, it appears that the evolution of fibrosis is rather a result from the accumulating alveolar macrophages than from the persistence of the Langerhans’ cells. Passive cigarette smoking may be considered a significant risk factor in both the pathogenesis and development of pulmonary LCH in a small child.

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Section: Introductionmentioning

confidence: 99%

Natural Course of Isolated Pulmonary Langerhans’ Cell Histiocytosis in a Toddler

et al. 2005

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“…PLCH is defined by the presence of inflammatory granulomas containing Langerhans cells, located around the distal bronchioles [1, 2]. …”

Section: Introductionmentioning

confidence: 99%

Correlation between High-Resolution Computed Tomography Findings and Lung Function in Pulmonary Langerhans Cell Histiocytosis

et al. 2007

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Background: Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon interstitial lung disease which can lead to serious respiratory failure. The correlation between high-resolution computed tomography (HRCT) findings and lung function have not been studied in depth. Objectives: To assess the relationship between HRCT findings and lung function in PLCH. Methods: Since HRCT abnormalities in PLCH consist mainly of nodular opacities and cystic abnormalities, we determined semiquantitative scores of nodular profusion and cystic extent. We therefore assessed the relationship between HRCT abnormalities on one hand and lung function and gas exchange parameters on the other, in patients with PLCH. Results: In our series of 26 consecutive patients, we found no significant correlation between the score of nodular profusion and lung function or gas exchange parameters. The score of cystic extent showed a quite strong and significant correlation with FEV₁/FVC (r = –0.62; p = 0.01), but also with PaO₂ (r = –0.69; p = 0.001) and carbon monoxide diffusing capacity (r = –0.60; p < 0.01). Furthermore, the patients with a predominant cystic pattern (n = 7) had the highest grade of dyspnea on exertion (p = 0.004), the lowest FEV₁/FVC ratio (p = 0.02) and the lowest PaO₂ (p = 0.02) compared to patients with a predominant nodular (n = 12) or a mixed pattern (n = 7). Conclusions: We conclude that in PLCH, the cystic extent on HRCT, but not the nodular profusion, correlates significantly with lung function abnormalities and impairment of gas exchange.

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“…Weniger die Gesamtanzahl der "Packungsjahre", als vielmehr die konsumierte Dosis an inhalativem Zigarettenrauch scheint die wesentliche Rolle als Risikofaktor zu spielen [1,2].…”

Section: Pulmonale Langerhans-zell-histiozytoseunclassified

“…Bei Frauen mit überwiegend zystischen Veränderungen kann die Differenzierung zur Lymphangioleiomyomatose schwierig sein. Andere mit dem inhalativen Rauchen assoziierte interstitielle Erkrankungen sind die desquamative interstitielle Pneumonie (DIP), die "respiratory bronchiolitis-interstitial lung disease" (RBILD) und die idiopathische Lungenfibrose (IPF) [1][2][3].…”

Section: Pulmonale Langerhans-zell-histiozytoseunclassified

“…Zusammengefasst ist festzuhalten, dass bei entsprechendem klinischem Zusammenhang und typischen Befunden im HRCT eine weitere invasive Diagnostik oftmals nicht notwendig erscheint. Bei zweifelhaften Fällen kann der histologische Befund (mittels bronchoskopischer oder operativer Lungenbiopsie) oder der Nachweis von > 5% CD1a-Antikörpern in der BAL zur Diagnose führen [1,2].…”

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