Serial computed tomography and lung function testing in pulmonary Langerhans’ cell histiocytosis

Abstract

Little is known about longitudinal lung function variation in patients with pulmonary Langerhans' cell histiocytosis (LCH). The contribution of serial lung computed tomography (CT) to managing these patients has not been evaluated.This long-term retrospective study included 49 patients who were serially evaluated by lung CT and pulmonary function tests. The lung function variation was categorised as improvement or deterioration. The extent of the CT lesions was correlated with lung function.Lung function deteriorated in ,60% of the patients. Forced expiratory volume in 1 s (FEV1) and diffusing capacity of the lung for carbon monoxide (DL,CO) were the parameters that most frequently deteriorated. A subgroup of patients experienced a dramatic decline in FEV1 within 2 yrs of diagnosis. Airway obstruction was the major functional pattern observed. In a multivariate analysis, % predicted FEV1 at diagnosis was the only factor associated with the incidence of airway obstruction. The increase in cystic lesions on the lung CTs was associated with impaired lung function but did not anticipate the decline in FEV1 or DL,CO.Serial lung function tests are essential for following patients with pulmonary LCH, who frequently develop airway obstruction. A lung CT at diagnosis is informative, but routine sequential CTs seem less useful. A prospective study is needed to characterise those patients with early progressive disease.

Pulmonary Langerhans cell histiocytosis: analysis of 14 patients and literature review

“…Also, previous study found that a lung CT at diagnosis of PLCH was informative (16) and confident (31,41). In the present study, not all the patients underwent tissue biopsy by open lung biopsy or thoracoscope, and this is a limitation of our study.…”

“…Serial lung function assessment is necessary for the follow-up of PLCH patients to identify their disease progression (28). However, in the current study, PFTs data during follow-up are not achieved, so a larger follow-up study is needed to prove the findings in the previous study (16).…”

“…Three patients showed DLCO% >80%, which are at the early stages of PLCH confirmed by CT scan; the decrease of DLCO is not evident, possibly because the sample size is small. Previous study found that lung function deteriorated in 60% of the patients and improved in 20% (16). Serial lung function assessment is necessary for the follow-up of PLCH patients to identify their disease progression (28).…”

“…All the coherent patients were hospitalized at the Shanghai Pulmonary Hospital, Shanghai, China, between December 2008 and June 2012. The diagnosis of PLCH should fulfill one of the following criteria: (I) disease proven by lung biopsy; (II) a positive biopsy of an extra thoracic localization of the disease or the presence of diabetes insipidus associated with characteristic lung HRCT findings; or (III) the combination of an appropriate clinical setting, a typical lung HRCT pattern (showing both nodules and cysts) and the exclusion of alternative diagnoses (16).…”

Pulmonary Langerhans Cell Histiocytosis-associated Pulmonary Hypertension Showing a Drastic Improvement Following Smoking Cessation

“…Recent studies imply that cigarette smoking is an etiology of PLCH (2). Although smoking cessation's effect on the outcome of PLCH remains inconclusive, it is sometimes a reasonable option in some cases (2)(3)(4)(5). We herein discuss a case of PLCH that was complicated by severe pulmonary hypertension (PH) and respiratory impairment that dramatically improved after the patient stopped smoking.…”