Lung Transplantation for Pulmonary Langerhans' Cell Histiocytosis: A Multicenter Analysis

Dauriat, Gaëlle; Mal, Hervé; Thabut, Gabriel; Mornex, Jean‐François; Bertocchi, M.; Tronc, François; Leroy-Ladurie, François; Dartevelle, Philippe; Reynaud‐Gaubert, Martine; Thomas, Philippe; Pison, Christophe; Blin, Dominique; Stern, M.; Bonnette, P.; Dromer, C.; Velly, Jean-François; Brugière, Olivier; Lesèche, Guy; Fournier, Michel

doi:10.1097/01.tp.0000200304.64613.af

Cited by 166 publications

(128 citation statements)

References 17 publications

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“…PH is frequent in patients with advanced PLCH [70], and is related to an intrinsic pulmonary vascular disease in which the pulmonary circulation is involved independent of small airway and lung parenchyma injury [71]. In the study of DAURIAT et al [72], PH as diagnosed by RHC, was present in 92% of the 39 patients with PLCH referred for lung transplantation and was moderate-to-severe (mean PAP o35 mmHg) in 72.5% of cases. However, PH may not occur only in end-stage PLCH patients [73].…”

Section: Other Ilds Associated With Phmentioning

confidence: 99%

Pulmonary hypertension in chronic interstitial lung diseases

Caminati¹,

Cassandro²,

Harari³

2013

Eur Respir Rev

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Pulmonary hypertension (PH) is a common complication of interstitial lung diseases (ILDs), particularly in idiopathic pulmonary fibrosis and ILD associated with connective tissue disease. However, other lung diseases, such as combined pulmonary fibrosis and emphysema syndrome, pulmonary Langerhans cell histiocytosis, and lymphangioleiomyomatosis, may also include PH in their clinical manifestations. In all of these diseases, PH is associated with reduced exercise capacity and poor prognosis. The degree of PH in ILDs is typically mild-to-moderate. However, some of these patients may develop a disproportionate increase in PH that cannot be justified solely by hypoxia and parenchymal injury: this condition has been termed “out-of-proportion” PH. The pathogenesis of PH in these diseases is various, incompletely understood and may be multifactorial. The clinical suspicion (i.e. increased dyspnoea, low diffusion capacity) and echocardiographic assessment are the first steps towards proper diagnosis of PH; however, right heart catheterisation remains the current gold standard for diagnosis of PH. At present, no specific therapies have been approved for the treatment of PH in patients with ILDs

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Section: Other Ilds Associated With Phmentioning

confidence: 99%

Pulmonary hypertension in chronic interstitial lung diseases

Caminati¹,

Cassandro²,

Harari³

2013

Eur Respir Rev

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“…The long-term outcome is marked by a slow progression to respiratory insufficiency and may be an indication for lung transplantation [4]. Recently, cladribine (2-chlorodeoxyadenosine), a synthetic chlorinated deoxyadenosine analogue, was found to be effective in adult patients with chronic cystic PCLH, with improvement in both lung function and computed tomography (CT) findings [5,6].…”

Section: Cladribine Improves Lung Cysts and Pulmonary Function In A Cmentioning

confidence: 99%

Cladribine improves lung cysts and pulmonary function in a child with histiocytosis

Epaud¹,

Pointe

Fasola

et al. 2014

Eur Respir J

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“…In PLCH, PH was very common in late stages of the disease (92−100%) [15,16], nevertheless there is not much data concerning its early stage.…”

Section: Epidemiology Of Ph In the Course Of Dpldmentioning

confidence: 99%

“…On such circumstance, the clinical picture may be more similar to that found in PAH, with severe PH and mild lung disease only. Moreover, fibrous remodeling of the pulmonary veins mimicking PVOD has been frequently found in systemic sclerosis (SSc) and PLCH [12,16] and occasionally in IPF and sarcoidosis [13,14].…”

Section: Pathobiology Of Ph In the Course Of Dpldmentioning

confidence: 99%

Pulmonary Hypertension in the Course of Diffuse Parenchymal Lung Diseases—State of Art and Future Considerations

Szturmowicz¹,

Kacprzak²,

Błasińska‐Przerwa³

et al. 2015

Advances in Respiratory Medicine

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Lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The development of PH influences the course of lung disease, worsening the clinical symptoms and prognosis. According to the most recent publications, PH in the course of lung diseases develops as a result of both "parenchymal" and vascular pathology, in the patients with genetic predisposition. Prolonged infection (especially viral one) may be an additional promoting factor. Right heart catheterization (RHC), which is an invasive procedure, is the only objective method of diagnosing PH. According to the latest recommendations, the management algorithm of PH and coexisting interstitial lung disease is based on RHC and the results of pulmonary function tests. Majority of the patients develop mild PH in the course of advanced lung disease. Best treatment of underlying lung pathology combined with long term oxygen treatment is recommended in this group. In case of severe PH (mean resting pulmonary artery pressure (mPAP) ≥ 35 mm Hg) the alternate cause of PH has to be sought. PAH-specific drugs use should be limited to patients with severe PH participating in clinical trials. In this review, the value of various non-invasive methods (echocardiography, radiological examination, exercise capacity and brain natriuretic peptides assessment) in the process of screening for PH is presented, and the results of recent randomized clinical trials with PAH-specific drugs in patients with diffuse parenchymal lung diseases are discussed.

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Lung Transplantation for Pulmonary Langerhans' Cell Histiocytosis: A Multicenter Analysis

Abstract: Severe pulmonary hypertension is a common feature in patients with end-stage PLCH. Given the good postransplant survival rate and despite a recurrence rate of the disease of approximately 20% after LT, we conclude that LT is a therapeutic option in this setting.

Cited by 166 publications

References 17 publications

Pulmonary hypertension in chronic interstitial lung diseases

Pulmonary hypertension in chronic interstitial lung diseases

Cladribine improves lung cysts and pulmonary function in a child with histiocytosis

Pulmonary Hypertension in the Course of Diffuse Parenchymal Lung Diseases—State of Art and Future Considerations

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