Do tertiary paediatric hospitals deal with the same spectrum of paediatric pulmonary hypertension as multicentre registries?

In July 2009, the patient’s condition worsened with PFT results comparable to those at first presentation (fig. 1). Treatment with azathioprine (150 mg?day) was then started as hydroxychloroquine was withdrawn. This treatment allowed a significant gradual clinical, radiological and functional improvement (fig. 2), with almost normalised PFTs by December 2011 (fig 1). SACE level also normalised. Only stable fibrotic chest CT lesions and DL,CO abnormalities persisted. Treatment efficacy and tolerance were still excellent after 17 months and a reduction of azathioprine (100 mg?day) was initiated.