6. Since pulmonary disease independent of skin score (NEJM, 2006, 345:25 2655-2709) carries a poor prognosis, patient may be enroled for only lung involvement defined as active alveolitis on BAL or ground-glass opacity on CT, a DLCO < 80% predicted or decrease in lung function (TLC), DLCO, FVC) of 10% or more in last 12 months.