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.

Keywords:
Pulmonary Langerhans cell histiocytosis, Lung nodules, Lung function, Lung cysts, Computed tomography
1.
Tazi A, Soler P, Hance AJ: Adult pulmonary Langerhans’ cell histiocytosis. Thorax 2000;55:405–416.
2.
Tazi A, Soler P, Hance AJ: Pulmonary Langerhans’ cell histiocytosis. Eur Respir Mon 2000;14:181–193.
3.
Soler P, Bergeron A, Kambouchner M, Groussard O, Brauner M, Grenier P, Crestani B, Mal H, Tazi A, Battesti JP, Loiseau P, Valeyre D: Is high-resolution computed tomography a reliable tool to predict the histopathological activity of pulmonary Langerhans cell histiocytosis? Am J Respir Crit Care Med 2000;162:264–270.
4.
Basset F, Corrin B, Spencer H, Lacronique J, Roth C, Soler P, Battesti JP, Georges R, Chretien J: Pulmonary histiocytosis X. Am Rev Respir Dis 1978;118:811–820.
5.
Schonfeld N, Frank W, Wenig S, Uhrmeister P, Allica E, Preussler H, Grassot A, Loddenkemper R: Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X. Respiration 1993;60:38–44.
6.
Vassallo R, Ryu JH, Schroeder DR, Decker PA, Limper AH: Clinical outcomes of pulmonary Langerhans’-cell histiocytosis in adults. N Engl J Med 2002;346:484–490.
7.
Diette GB, Scatarige JC, Haponik EF, Merriman B, Fishman EK: Do high-resolution CT findings of usual interstitial pneumonitis obviate lung biopsy? Views of pulmonologists. Respiration 2005;72:134–141.
8.
Kleinerman J, Green F, Harley RA, Lapp L, Laqueur W, Naeye RL, Pratt P, Taylor G, Wiott J, Wyatt J: Pathology standards for coal worker’s pneumoconiosis. Report of the Pneumoconiosis Committee of the College of American Pathologists to the National Institute for Occupational Safety and Health. Arch Pathol Lab Med 1979;103:375–432.
9.
Park KJ, Bergin CJ, Clausen JL: Quantitation of emphysema with three-dimensional CT densitometry: comparison with two-dimensional analysis, visual emphysema scores, and pulmonary function test results. Radiology 1999;211:541–547.
10.
Clausen JL, Coates AL, Quanjer PH: Measurement of lung volumes in humans: review and recommendations from an ATS/ERS workshop. Eur Respir J 1997;10:1205–1206.
11.
Kambouchner M, Basset F, Marchal J, Uhl JF, Hance AJ, Soler P: Three-dimensional characterization of pathologic lesions in pulmonary Langerhans cell histiocytosis. Am J Respir Crit Care Med 2002;166:1483–1490.
12.
Moore AD, Godwin JD, Muller NL, Naidich DP, Hammar SP, Buschman DL, Takasugi JE, de Carvalho CR: Pulmonary histiocytosis X: comparison of radiographic and CT findings. Radiology 1989;172:249–254.
13.
Bonelli FS, Hartman TE, Swensen SJ, Sherrick A: Accuracy of high-resolution CT in diagnosing lung diseases. Am J Roentgenol 1998;170:1507–1512.
14.
Brauner MW, Grenier P, Mouelhi MM, Mompoint D, Lenoir S: Pulmonary histiocytosis X: evaluation with high-resolution CT. Radiology 1989;172:255–258.
15.
Brauner MW, Grenier P, Tijani K, Battesti JP, Valeyre D: Pulmonary Langerhans cell histiocytosis: evolution of lesions on CT scans. Radiology 1997;204:497–502.
16.
Crausman RS, Jennings CA, Tuder RM, Ackerson LM, Irvin CG, King TE Jr: Pulmonary histiocytosis X: pulmonary function and exercise pathophysiology. Am J Respir Crit Care Med 1996;153:426–435.
17.
Delobbe A, Durieu J, Duhamel A, Wallaert B: Determinants of survival in pulmonary Langerhans’ cell granulomatosis (histiocytosis X). Groupe d’Etude en Pathologie Interstitielle de la Société de Pathologie Thoracique du Nord. Eur Respir J 1996;9:2002– 2006.
18.
Divertie MB, Cassan SM, Brown AL Jr: Application of ultrastructural morphometry to lung biopsy specimens in pulmonary histiocytosis X. Thorax 1975;30:326–332.
19.
Kelkel E, Pison C, Brambilla E, Ferreti G, Coulomb M, Brambilla C: Value of high resolution tomodensitometry in pulmonary histiocytosis X: radiological, clinical and functional correlations. Rev Mal Respir 1992;9:307–311.
20.
Paciocco G, Uslenghi E, Bianchi A, Mazzarella G, Roviaro GC, Vecchi G, Harari S: Diffuse cystic lung diseases: correlation between radiologic and functional status. Chest 2004;125:135–142.
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2007
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