A 38-year-old, house keeper woman was admitted in Alzahra hospital because of chills, fever, and generalized malaise. She also complained about exertional dyspnea and nonproductive cough of 2 years duration and had a history of Raynaud's phenomenon that started 8 months prior to admission. She was a nonsmoker with no documented lung disease and denied any other symptom. Physical examination revealed Velcro-like rales at the bases of both the lungs, but no sign of skin nail involvement or heart failure. Tuberculosis and other infections were ruled out. Laboratory tests showed a high positive titer of anti-nuclear antibody, erythrocyte sedimentation rate of 73, and C-reactive protein of 3 + and a normocytic normochromic anemia, whereas test for anti-Scl70 antibody and anti-centromere antibody was negative [Table 1].
Computed tomography scan showed bilateral ground-glass pattern suggestive of ILD [Figure 1]. Spirometry was done, which showed a moderate restrictive pattern.
She was treated with prednisolone (1 mg/kg) and pulse of cyclophosphamide (1 g/month) for 6 months, and then put on Cellcept for another 6 months, but ILD did not improve. Thus, the patient was treated with rituximab 2 g (1 gram every 14 days) and showed gradual improvement, then she was subsequently treated with 100 mg of azathioprine daily, prednisolone 5 mg bid, and calcium plus vitamin-D. She has been followed up for 2 years and has not shown any other manifestation.
High-resolution CT scan showing lung fibrosis.