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Revista americana de medicina respiratoria

On-line version ISSN 1852-236X


QUADRELLI, Silvia; DUBINSKY, Diana  and  ALVAREZ, Sabrina. ¿Hay un rol para los agentes biológicos en el tratamiento de la patología pulmonar intersticial asociada a enfermedades del tejido conectivo?. Rev. am. med. respir. [online]. 2016, vol.16, n.1, pp.56-63. ISSN 1852-236X.

Except for SSc, there are no controlled clinical trial data available to guide decision making in CVD-ILD. To date, only two powered, randomized controlled trials of treatment have been conducted in scleroderma patients; with both of these assessing the benefit of cyclophosphamide compared with placebo for the treatment of scleroderma-related interstitial lung. Currently accepted initial treatments in CVD-ILD include corticosteroids, azathioprine and mycophenolate mofetil for mild disease, while cyclophosphamide has been used in severe or rapidly progressive disease. However, an optimal general approach or specific criteria for the selection of each different treatment modality, is not completely defined. During the last decade, rituximab (RTX), has been used in the treatment of a interstitial lung disease associated to connective tissue diseases (ILD-CTD). A recent study including 50 cases of severe and refractory ILD showed that RTX therapy resulted in an an improvement of 8.9% of the FVC. Several small retrospective studies have shown at least partial benefit of RTX in refractory PM/DM, including antisynthetase syndrome (ASS). RTX may have a role in specific subsets of CVD-ILD. Further studies are needed, but there is enough evidence to consider RTX as a suitable and safe option for the treatment of severe, relapsing or refractory patients. Several other biologic agents are now being studied in CVD, including tocilizumab that has shown a possible therapeutic benefit in patients with CVD-ILD.

Keywords : Interstitial lung disease; Autoimmune disease; Immunosuppression; Biological agents; Rituximab.

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