Background: Pulmonary involvement with Langerhans cell histiocytosis (LCH, formerly known as histiocytosis-X) presents as an interstitial process in children and adults either with or without symptoms. In contrast to other manifestations of LCH, most patients with pulmonary disease are adults.
Procedures: We reviewed the literature on pulmonary LCH to determine what were the clinical presentations, prognostic variables, and treatment options for this disease.
Results: Although there are spontaneous remissions, a large number of patients have progressive pulmonary deficiency and experience significant morbidity if not mortality from the disease. The efficacy of steroid versus chemotherapy in halting the process remains controversial, even if smoking is taken into consideration.
Conclusions: A multicenter study of therapy for pulmonary LCH is the obvious answer to this dilemma. We propose that interested centers organize via the Histiocyte Society to plan and execute such a trial.
Copyright 2002 Wiley-Liss, Inc.