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Am. J. Respir. Crit. Care Med., Volume 162, Number 1, July 2000, 264-270

Is High-Resolution Computed Tomography a Reliable Tool to Predict the Histopathological Activity of Pulmonary Langerhans Cell Histiocytosis?

PAUL SOLER, ANNE BERGERON, MARIANNE KAMBOUCHNER, ODILE GROUSSARD, MICHEL BRAUNER, PHILIPPE GRENIER, BRUNO CRESTANI, HERVÉ MAL, ABDELLATIF TAZI, JEAN PAUL BATTESTI, PAULE LOISEAU, and DOMINIQUE VALEYRE

INSERM U 408, UFR Xavier Bichat, Paris, France; Fédération des Maladies Respiratoires et Thoraciques, Hôpital Avicenne, AP-HP, Bobigny, France; Service d'Anatomopathologie, Hôpital Beaujon, AP-HP, Clichy, France; Service de Radiologie, Hôpital La Pitié, AP-HP, Paris, France; Service de Pneumologie, Hôpital Bichat, AP-HP, Paris, France; Service de Pneumologie, Hôpital Beaujon, AP-HP, Clichy, France; and INSERM U 82, UFR Xavier Bichat, Paris, France

High-resolution computed tomography (HRCT) has proved to be very useful in the diagnosis and follow-up of pulmonary Langerhans cell histiocytosis (PLCH), but the precise relationships between nodules and thin-wall cysts observed by HRCT, and granulomatous or cystic lesions present in lung tissue, remain to be established. The aim of this study was to compare quantitative data obtained by HRCT and those obtained by histopathological examination of corresponding lung tissue specimens in patients with biopsy-proven PLCH. The results demonstrated that the extent of nodular abnormalities was strongly correlated with the density of florid granulomatous lesions in lung tissue. A strong correlation was also found between the extent of cystic abnormalities and the density of cavitary lesions, but the latter included both still inflammatory cavitary granulomas and cicatricial fibrous cysts. Interestingly, small isolated florid granulomas were found in lung tissue from most patients with a predominant cystic CT scan pattern. Taken together, these results demonstrate that HRCT has to be considered with caution to evaluate the histopathological activity of PLCH. Patients presenting with predominant HRCT cystic abnormalities should benefit from a long-term follow-up. Because these patients are susceptible to developing severe respiratory insufficiency, they should also be considered for treatment as soon as an effective therapy for LCH is available.




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