No abstract

Pregnancy has been reported infrequently in patients with systemic sclerosis. Consequently, the outcome and appropriate management of such patients is uncertain. We review the obstetric experience of 19 female patients with systemic sclerosis, and report 2 women in whom 3 pregnancies occurred during the course of disease. Maternal complications included hypertension during 2 of these pregnancies and congestive heart failure during 1. There were 2 premature deliveries, although all 3 infants survived. Our experience with these patients and a review of the available literature suggest that, in patients with systemic sclerosis, fertility may be reduced and complications of pregnancy may be more frequent.The occurrence of pregnancy in women who have systemic sclerosis (scleroderma) appears to be uncommon. The infrequency of reports of this association has led to somewhat divergent views concerning the outcome and management of such patients. A review in the obstetric literature recommends consideration of early therapeutic abortion and permanent sterilization because of high maternal morbidity ( I ) . No firm conclusions can be obtained from the nonobstetric literature. The 1966 Rheumatism Review recorded that no effect on pregnancy was noted in We have recently managed 2 scleroderma patients through the course of 3 pregnancies. Herein we describe our experience with these women and survey the reported series published in the English language literature. PATIENTS AND METHODSPatient 1. A 26-year-old black woman developed Raynaud's phenomenon and digital ulcerations in 1975. She subsequently developed sclerodactyly, telangiectasia, diffuse proximal and distal cutaneous scleroderma, myosjtis, gastrointestinal involvement (including esophageal dysmotility and wide-mouth colonic pseudodiverticula), and pulmonary involvement characterized by radiographic interstitial fibrosis, restrictive pulmonary function, and mild resting hypoxemia. She remained normotensive with normal renal function.Serologic evaluation revealed a positive antinuclear factor with a nucleolar pattern and negative test results for rheumatoid factor, anti-DNA, anti-RNP, and anti-Sm antibodies. Her obstetric history included 4 pregnancies, with I term and 3 premature deliveries resuljing in 2 neonatal deaths and 2 living children. All pregnancies had occurred prior to the onset of scleroderma.She became pregnant for the fifth time in 1979. During the pregnancy she was maintained on a regimen of prednisone 15 mg daily and methyldopa 500 mg 3 tiyes daily. These medications were given for treatment of myositis and Raynaud's phenomenon, respectively. She remained normotensive and noted moderate improvement in her digital ulcers.At 32 weeks' gestation she was hospitalized with a blood pressure of 154/108 and facial and lower extremity edema. Serum creatinine was 0.5 mg/dl and urinalysis showed 2+ proteinuria. Her hypertension proved refractory to management with bed rest, methyldopa, and intravenous