Arch Bronconeumol. 2013;49(5):213–215 w w w.archbronconeumol.org Case Report Is Treatment With Hydroxychloroquine Effective in Surfactant Protein C Deficiency?  Ingrid Rabach, a,* Furio Poli, a Floriana Zennaro, b Claudio Germani, b Alessandro Ventura, a Egidio Barbi b a Institute for Maternal and Child Health - IRCCS «Burlo Garofolo», University of Trieste, Trieste, Italy b Institute for Maternal and Child Health - IRCCS «Burlo Garofolo», Trieste, Italy a r t i c l e i n f o Article history: Received 18 July 2012 Accepted 28 August 2012 Available online 6 April 2013 Keywords: Surfactant protein C deficiency Juvenile interstitial lung disease Respiratory distress syndrome Hydroxychloroquine a b s t r a c t We present the case of two twin brothers with surfactant protein C deficiency who were treated with hydroxychloroquine for three years, with apparent success. The exact physiopathology of this disease is not known and there is no specific treatment for it. There is merely news from a few previous descriptions in the literature about the use of hydroxychloroquine for surfactant protein C deficiency with satisfac- tory results. Two years after the treatment was withdrawn, the twins were evaluated once again: they presented no new infections, growth and general state were normal and chest CT showed a notable addi- tional reduction in the interstitial pneumopathy. These data seem to cast some doubt on the efficacy of hydroxychloroquine, and they suggest that the clinical improvement was simply the natural evolution of the disease. © 2012 SEPAR. Published by Elsevier España, S.L. All rights reserved. ¿Es eficaz el tratamiento con hidroxicloroquina en el déficit de proteína C surfactante? Palabras clave: Déficit de proteína C surfactante Neumopatía intersticial infantil Síndrome de dificultad respiratoria Hidroxicloroquina r e s u m e n Presentamos el caso de dos hermanos gemelos con déficit de proteína C surfactante que fueron tratados mediante el empleo de hidroxicloroquina durante tres a˜ nos, con aparente éxito. La fisiopatología exacta de esta enfermedad no se conoce, y no disponemos de ningún tratamiento específico para ella; tan solo tenemos noticia de unas pocas descripciones previas en la literatura sobre el uso de hidroxicloroquina para el déficit de proteína C surfactante con resultados satisfactorios. Dos a˜ nos después de la retirada del tratamiento se volvió a evaluar a los gemelos: no presentaron nuevas infecciones, el crecimiento y el estado general fueron normales, y la TC de tórax mostró una notable reducción adicional de la neumopatía intersticial. Estos datos parecen poner en duda la eficacia de la hidroxicloroquina, y sugieren que la mejoría clínica fue simplemente la evolución natural de la enfermedad. © 2012 SEPAR. Publicado por Elsevier España, S.L. Todos los derechos reservados. Introduction Surfactant protein C deficiency is characterised by deficiency of the protein and its intracellular accumulation, with subsequent local inflammation and interstitial lung disease. There is no spe- cific treatment, but hydroxychloroquine has been reported to be effective.  Please cite this article as: Rabach I, et al. ¿Es eficaz el tratamiento con hidrox- icloroquina en el déficit de proteína C surfactante? Arch Bronconeumol. 2013; 49:213-5. * Corresponding author. E-mail address: ingridrabach@gmail.com (I. Rabach). We describe the case of twin brothers with surfactant protein C deficiency who presented recurrent episodes of respiratory dis- tress. They were treated with hydroxychloroquine for three years, after which the treatment was withdrawn without any further recurrences. Case Presentation Monozygotic twin brothers were born at 31 weeks gestation, with birth weights of 1490 and 1710 g. Their mother received pro- phylaxis with corticoids for hyaline membrane disease. The first few months were characterised by failure to thrive and, starting from 7 months of age, by recurrent episodes of respi- ratory distress, with cough, fever, wheezing, tachydyspnoea and 1579-2129/$ – see front matter © 2012 SEPAR. Published by Elsevier España, S.L. All rights reserved.