RT Journal Article
SR Electronic
T1 Interstitial lung disease in systemic sclerosis: where do we stand?
JF European Respiratory Review
JO EUROPEAN RESPIRATORY REVIEW
FD European Respiratory Society
SP 411
OP 419
DO 10.1183/16000617.00002915
VO 24
IS 137
A1 Susanna Cappelli
A1 Silvia Bellando Randone
A1 Gianna Camiciottoli
A1 Amato De Paulis
A1 Serena Guiducci
A1 Marco Matucci-Cerinic
YR 2015
UL http://err.ersjournals.com/content/24/137/411.abstract
AB Interstitial lung disease (ILD) is common in systemic sclerosis (SSc) patients and despite recent advances in the treatment is, at present, the major cause of death. Today, an early diagnosis of ILD is possible, and is mandatory to improve the prognosis of the disease.Pulmonary function tests and high-resolution computed tomography remain the mainstay for the diagnosis of SSc-ILD, but there is a growing interest in lung ultrasound. Recently, the correlation between severity of fibrosis and some peripheral blood biomarkers has been described.Nonselective immunosuppressors are still the main treatment for ILD, with cyclophosphamide (CYC) most widely used to obtain remission. Novel therapies towards specific molecular and cellular targets have been suggested; in particular, rituximab (RTX) has shown promising results, but further research is needed. It is of paramount importance to define the severity of the disease and the risk of progression in order to define the need for treatment and the treatment intensity. We propose the division of the treatment strategies at our disposal to induce remission into three categories: high intensity (haematopoietic stem cell transplantation), medium intensity (CYC and RTX) and low intensity (azathioprine (AZA) and mycophenolate mofetil (MMF)). After obtaining remission, maintenance treatment with AZA or MMF should be started.In this review we explore new advances in the pathogenesis, diagnosis and treatment of SSc-ILD.Early diagnosis of ILD is possible, and is mandatory to improve the prognosis of the disease http://ow.ly/P28JH