Determinants of survival in pulmonary Langerhans' cell granulomatosis (histiocytosis X). Groupe d'Etude en Pathologie Interstitielle de la Societe de Pathologie Thoracique du Nord

Delobbe, Alain; Durieu, J; Duhamel, Alain; Wallaert, B.

doi:10.1183/09031936.96.09102002

Cited by 123 publications

(65 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Finally, as systemic treatments were prescribed according to the physicians' discretion, we could not evaluate their effects on the pulmonary LCH outcomes. In a previous study, corticosteroid therapy was associated with poorer outcomes, probably as a result of selection bias towards more severe forms of the disease [6].…”

Section: Discussionmentioning

confidence: 85%

“…Although adequate statistical methods for time-to-event data were used, we cannot exclude a bias toward an over-representation of patients with unfavourable outcomes. It is also possible that the patients selected for this study may not have been fully representative of the general pulmonary LCH population, although the lung function at diagnosis of our patients was similar to the lung function reported in previous studies [5][6][7][8]. Finally, as systemic treatments were prescribed according to the physicians' discretion, we could not evaluate their effects on the pulmonary LCH outcomes.…”

Section: Discussionmentioning

confidence: 94%

“…The natural history of pulmonary LCH is widely variable and difficult to predict in an individual patient [5][6][7][8]. It has been suggested that older age, evidence of obstruction, air trapping (an increased residual volume (RV)/total lung capacity (TLC) ratio) and a reduced diffusing capacity for carbon monoxide (DL,CO) at diagnosis are associated with the development of respiratory failure and increased mortality [5,6,9]. However, scant information is available concerning the longitudinal variations in lung function over time, particularly over a long period.…”

mentioning

confidence: 99%

See 2 more Smart Citations

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

Tazi¹,

Marc²,

Dominique³

et al. 2012

Eur Respir J

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 85%

Section: Discussionmentioning

confidence: 94%

mentioning

confidence: 99%

See 1 more Smart Citation

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

Tazi¹,

Marc²,

Dominique³

et al. 2012

Eur Respir J

View full text Add to dashboard Cite

show abstract

“…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

View full text Add to dashboard Cite

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.

show abstract

“…[5] One study demonstrated corticosteroid use of 0.5-1 mg/kg/day of prednisone to be associated with clinical and radiological improvement but no change in overall lung function, [2] while a separate retrospective study demonstrated a deterioration in the patients treated with corticosteroids compared to untreated patients. [6] In isolated pulmonary pathology in patients with severe respiratory deficiency, lung transplant with smoking cessation is often the only curative method available.…”

Section: Discussionmentioning

confidence: 99%

A diagnosis more than skin deep: A case of late-stage pulmonary langerhans’ cell histiocytosis with skin lesions

Verson

Egusquiza

Nguyen

2017

CRIM

View full text Add to dashboard Cite

Introduction: Langerhans' cell histiocytosis (LCH) is a group of rare diseases with a wide variety of presentations across age groups. We describe a case of pulmonary LCH diagnosed at a late stage despite having additional skin findings for several years. Case description: A 32-year-old male presented with acute exacerbation of chronic dyspnea resulting in markedly decreased functional capacity as well as cutaneous lesions. He had smoked cigarettes for 10 years with no occupational exposures. The patient also complained of a pruritic rash in his axillary and groin areas. His vitals were within normal limits. His exam was notable for diminished right lung sounds and expiratory wheezes bilaterally. A chest x-ray showed marked emphysema and bilateral upper lobe reticular opacities suggestive of scarring. Computerized tomography of the chest showed loculated right pneumothorax and hyperinflated lungs with destructive changes secondary to numerous irregular, thick walled cysts and scattered irregular pulmonary nodules. Diagnosis was ultimately confirmed with biopsy of concomitant cutaneous lesions and characteristic radiographic findings on Chest CT. The patient started inhaled corticosteroids, inhaled anticholinergic, and inhaled short-acting beta agonists. He was sent home with oxygen and strongly encouraged to abstain from smoking. Discussion: LCH is characterized by end-organ infiltration of proliferating monoclonal Langerhans' cells, a histiocyte involved in antigen presentation. The highest incidence of LCH is between ages 5-10 years; the adult incidence is estimated to be less than half as frequent than the pediatric, about one patient in 500,000. LCH most commonly involves the skin, bones, and lungs. Pulmonary LCH (PLCH) is interesting in that it has a peak incidence in ages 20-40 years and occurs almost exclusively in smokers (> 90% of cases). Conclusion: Smoking cessation is the cornerstone of management of PLCH. It has been hypothesized that tobacco smoke can incite the production of cytokines by alveolar macrophages, leading to increased recruitment of histiocytes to the lung parenchyma. In isolated pulmonary pathology, lung transplant with smoking cessation is often curative.

show abstract

Determinants of survival in pulmonary Langerhans' cell granulomatosis (histiocytosis X). Groupe d'Etude en Pathologie Interstitielle de la Societe de Pathologie Thoracique du Nord

Cited by 123 publications

References 29 publications

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

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

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

A diagnosis more than skin deep: A case of late-stage pulmonary langerhans’ cell histiocytosis with skin lesions

Contact Info

Product

Resources

About