Pregnancy and childbirth after treatment with autologous hematopoietic stem cell transplantation for severe systemic sclerosis requiring parenteral nutrition. Ethical issues

We report a pregnancy in a patient who had undergone autologous hematopoietic stem cell transplantation (AHSCT) for diffuse cutaneous systemic sclerosis (SSc). SSc onset was at age 25, with Raynaud phenomenon and evolved to include diffuse cutaneous, arthritis, pulmonary fibrosis and extensive gastrointestinal involvement. AHSCT (cyclophosphamide/ATG for conditioning) was performed four years later with improvement of all features apart from the gastrointestinal symptoms requiring parenteral nutrition (PN). Forty months after AHSCT, she had a spontaneous miscarriage necessitating curettage. Despite advice to avoid pregnancy because of poor nutritional status and recurring catheter infections from her PN, she fell pregnant one year later. The pregnancy proceeded normally and she delivered at 34 weeks, under cesarean section. The baby girl, (1990g and 4 APGAR score) after initial respiratory distress, is now 4 years old with normal growth and development. Unfortunately, the patient died early in 2008 due to severe disease progression terminating with gastrointestinal obstruction and pericarditis. This first report of a successful pregnancy in a patient with diffuse SSc treated by AHSCT illustrates that despite the possibility for a normal pregnancy, the decision to do so includes aspects of maternal prognosis.