SIR, Interferon alfa (IFN-o) therapy is highly effective in patients with chronic myelogenous leukaemia and other malignant diseases. 1 Most side-effects, such as chills, malaise, myalgia and fatigue, are transient and tend to disappear with ongoing therapy, while others, such as anorexia, neurological and mood disorders, are dose-related and need dose adjustment or withdrawal of IFN-o. 1 Induction of immunemediated disease, including thyroid disease, 2, 3 rheumatoid arthritis, 3 systemic lupus erythematosus, 2, 4 vasculitis, 2 thrombocytopenia, 5 autoimmune haemolytic anaemia, 6 and insulin-dependent diabetes mellitus, 7 has also been described. Moreover, other immunological entities, such as myasthenia gravis, 8 vitiligo, 9 lichen planus, 9 Henoch–Schönlein purpura9 and Sjögren syndrome, 9 have been reported after IFN-o therapy for chronic viral hepatitis or multiple sclerosis (MS). Here, we report on the first case of systemic sclerosis (SSc) developing after therapy with IFN-o for chronic myelogenous leukaemia.

In October 1998, a 52-year-old-woman without a personal or a familiar history of autoimmune disease, was diagnosed as having chronic myelogenous leukaemia, with a karyotype 46, XX, t (9; 22), bcr⁄ abl positive in all the metaphases. She was given hydroxyurea 20 mg kg) 1 day) 1 for 2 months and then IFN-o 2a to a maximum daily dose of 10· 106 U. In June 1999, cytogenic evaluation showed a normalization of the karyotype in up to 33% of metaphases. Meanwhile, treatment was reduced to 6· 106 U day) 1 for the appraisal of arthralgias, ataxia and depression. In June 2000 a further evaluation revealed the loss of efficacy of therapy (Ph chromosome positive in all the analysable metaphases). In the same period, the patient experienced fever, dyspnoea and oedema of the extremities. Laboratory findings were normal, except for an increased erythrocyte sedimentation rate (ESR, 50mm in the first hour). Chest X-ray and computed tomography (CT) of the lung demonstrated the presence of pulmonary vascular congestion. Cardiac function, evaluated by electrocardiogram and Doppler echocardiography, was normal. The possible diagnosis of congestive heart failure was discarded and the patient’s symptoms were ascribed to a leak capillary syndrome secondary to interferon therapy; interferon therapy was interrupted. Peripheral oedema and respiratory symptoms improved on treatment with diuretics. Nevertheless, 3 months later, in November 2000 she experienced dyspnoea, oedema of both the upper and lower limbs and progressive skin thickening of the hands and wrists. A further echocardiographic evaluation revealed a normal ejection fraction (70%) and minimal tricuspid regurgitation with increased pulmonary artery pressure (34 mmHg). CT of the chest showed a picture of diffuse parenchymal and interstitial fibrosis with multiple areas of ground-glass attenuation. Pulmonary function tests demonstrated the