We report the case of a 47 year old woman with a 19 year history of mixed connective tissue disease (MCTD) diagnosed according to Alarcón-Segovia classification criteria.¹ She had Raynaud’s phenomenon, arthritis, swollen hands, and anti-ribonucleoprotein antibodies (anti-RNP 1/1280). She received low doses of prednisone and her clinical course was uneventful, except for sporadic Raynaud’s phenomenon. Her drug treatment was stopped 10 years ago, but medical supervision continued without evidence of pulmonary, gastrointestinal or renal disease during her follow up.

In 2003, she sought medical attention complaining of malaise during the past 3 months. Physical examination disclosed paleness, facial telangiectases, and hypertension (160/100). Laboratory findings were high—erythrocyte sedimentation rate (102 mm/1st h), normocytic/normochromic anaemia (haemoglobin 90 g/l), severely decreased renal function (creatinine …