Characterising patients with pulmonary Langerhans’ cell histiocytosis and pulmonary hypertension

Résumé fourni par la source

The mechanisms for pulmonary Langerhans’ cell histiocytosis associated pulmonary hypertension (PLCH-PH) are incompletely understood and classified as group 5 PH. We aimed to describe characteristics and outcomes of patients with PLCH-PH. Retrospective cohort study from five expert centres. Adult patients with diagnostic right heart catheterisation (RHC) were included. Data for anthropometry, pulmonary function tests (PFTs), RHC haemodynamics and treatment was collected. Outcome data included long term oxygen therapy (LTOT), lung transplantation and death. 31 patients were identified (M:18) with a mean age of 40 years at the time of PLCH diagnosis. All had a smoking history. Median (IQR) PFTs at PLCH diagnosis: FEV1 53% (38-72), FVC 79% (65-90), TLCO 30% (24-39), KCO 38% (29-45) and RV 93% (74-155). PFTs at PH diagnosis: FEV1 51% (34-68), FVC 70% (52-87), TLCO 22% (19-29), KCO 32% (22-35) and RV 152% (92-193). There was a statistically significant change in all PFT parameters between PLCH and PH diagnoses (p < 0.05). Baseline RHC: mRAP 7 (5-10) mmHg, mPAP 41 (29-49) mmHg, PCWP 10.5 (8-13) mmHg, CO 4.6 (4-5) L/min and PVR 6.4 (4-11) WU. 17/31 commenced pulmonary vasodilators: PDE5i (n = 12), ERA (n=5) and dual therapy (n=5). Nine patients had a repeat RHC: mPAP decreased from 39 (36-51) to 37 (32-45) mmHg and PVR from 7.9 (4.4-11.3) to 6 (4.3-9.7) WU. 20/31 commenced LTOT. 12/31 underwent lung transplantation.12/31 were deceased with a mean age of 53 years. PLCH can be associated with severe pre-capillary PH and significant mortality. Pulmonary vasodilators appear to be safe. Future directions include identifying predictive factors for the development of PLCH-PH and response to treatment.

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