Source: International Congress 2014 – ILDs 6
Disease area: Interstitial lung diseases

Abstract

Introduction: Over the last years the clinical evaluation and management of diffuse parenchymal lung disease (DPLD) progressed quickly. Elucidation of pathobiological mechanisms led to new therapeutic approaches with promising clinical results. Pirfenidone is an orally administered pyridine for the treatment of mild to moderate idiopathic pulmonary fibrosis (IPF). The mechanism of action is not fully understood yet and it might also be a useful therapeutic approach in other lung diseases.Case Presentation: We report on an 18 year old female patient receiving bone marrow transplantation twice by the age of 5 for the suspected diagnosis of myelodysplastic syndrome. The underlying condition in fact was dyskeratosis congenita. After transplantation she developed a mild graft versus host disease of the lung. Six years later lung function declined quickly and didn't respond to steroid pulses (VC_max 1.37l, 55%-pred., FEV1 0.98l, 47%-pred.). Diffusion capacity of the lung for CO (DLCO) dropped to 16% and the patient became oxygen dependent. Lung biopsies revealed bronchiolitis, alveolitis and subpleural fibrosis. After introduction of pirfenidone to therapy lung function (VC_max 1.55l, 64 %-pred., FEV1 1.0l, 50%-pred.) and DLCO (34%) increased.Conclusion: For DPLD few treatment options are available yet, especially in steroid unresponsive cases. Due to the broad mechanism of action pirfenidone might be a useful therapeutic approach in other lung diseases leading to fibrosis like.

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Citations should be made in the following way:
M. Rosewich, O. Eickmeier, J. Eckrich, R. Schubert, S. Zieen (Frankfurt/M, Germany). Pirfenidone in diffuse parenchymal lung disease after stem cell transplantation of a patient with dyskeratosis congenita. Eur Respir J 2014; 44: Suppl. 58, 3791

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