There are few studies that offer guidance in the treatment of CTD-ILD, and perhaps the most valid ones come from those examining treatment strategies for patients within the scleroderma spectrum of disease. In that paradigm, patients with “alveolitis,” as demonstrated by ground-glass opacities on HRCT or either neutrophilia or eosinophilia on BAL, are treated with CYC because of the high likelihood that the lung parenchymal disease will progress. Unpublished results from the Scleroderma Lung Study, presented at the 2005 international conference of the American Thoracic Society, suggest that CYC improves lung function (and skin scores) in patients with scleroderma-related alveolitis (ie, ILD). Full results from that study are eagerly awaited. Such definitive studies have not been conducted for other CTD-ILD. further
Recognized limitations in this study include the variability in therapeutic regimens and the lack of systematic data collection. Although MMF appeared to be well tolerated, in the absence of prospective evaluations, the rates of adverse effects of MMF observed in this study may be underestimated. Most patients in our study received prednisone along with MMF; therefore, it is difficult to determine the beneficial effects of either medication individually. However, by tabulating total and daily prednisone intake for each patient, we found that daily prednisone doses on average were not increased while disease control was maintained with MMF. In fact, five patients were able to discontinue their longterm, daily prednisone altogether after reaching their goal MMF doses. Our small sample size prohibited us from performing a more rigorous statistical analysis and from formulating more inferences regarding the apparent beneficial effects of MMF for CTD-ILD.
MMF appears to be safe and well tolerated in subjects with CTD-ILD. Given this and the evidence of disease control, we suggest that further longitudinal studies of MMF for CTD-ILD are warranted.