Systemic sclerosis (scleroderma) and pregnancy
- Bonnie L Bermas, MD
Bonnie L Bermas, MD
- Associate Professor of Medicine
- Harvard Medical School
- Section Editors
- John S Axford, DSc, MD, FRCP, FRCPCH
John S Axford, DSc, MD, FRCP, FRCPCH
- Section Editor — Scleroderma
- Emeritus Professor of Clinical Rheumatology
- St George's University of London
- Charles J Lockwood, MD, MHCM
Charles J Lockwood, MD, MHCM
- Section Editor — Obstetrics
- Senior Vice President, USF Health
- Dean, Morsani College of Medicine
- Professor, Obstetrics and Gynecology
- University of South Florida
Systemic sclerosis (SSc; scleroderma) is an uncommon illness with an annual incidence of 1 to 2 per 100,000 individuals in the United States [1,2]. The peak onset is between the ages of 30 and 50. (See "Overview of the clinical manifestations of systemic sclerosis (scleroderma) in adults".)
As of 1971, only 12 cases of pregnancy in women with SSc had been reported. Mortality among these patients was alarmingly high: four patients died (three from renal and pulmonary disease and one from a postpartum viral infection) . The incidence of pregnancy in women with SSc is increasing, since a greater number of women are choosing to have children later in life.
This topic will review issues of fertility and pregnancy outcome among patients with SSc, as well as the impact of pregnancy upon disease activity.
It is difficult to draw any clear conclusions concerning fertility in women with systemic sclerosis (SSc). In the 1950s, women with SSc were thought to have diminished fertility because of the low incidence of pregnancy in those with preexisting disease . However, subsequent studies showed mixed results with respect to pregnancy rates and to the frequency of pregnancy difficulties among women with SSc, both prior to and after the diagnosis.
The following findings were noted in different observational studies of women with SSc who were of childbearing age:
- Medsger TA Jr, Masi AT. Epidemiology of progressive systemic sclerosis. Clin Rheum Dis 1979; 5:15.
- Sánchez-Guerrero J, Colditz GA, Karlson EW, et al. Silicone breast implants and the risk of connective-tissue diseases and symptoms. N Engl J Med 1995; 332:1666.
- Knupp MZ, O'Leary JA. Pregnancy and scleroderma. Systemic sclerosis. J Fla Med Assoc 1971; 58:28.
- LEINWAND I, DURYEE AW, RICHTER MN. Scleroderma; based on a study of over 150 cases. Ann Intern Med 1954; 41:1003.
- Silman AJ, Black C. Increased incidence of spontaneous abortion and infertility in women with scleroderma before disease onset: a controlled study. Ann Rheum Dis 1988; 47:441.
- Englert H, Brennan P, McNeil D, et al. Reproductive function prior to disease onset in women with scleroderma. J Rheumatol 1992; 19:1575.
- Bernatsky S, Hudson M, Pope J, et al. Assessment of reproductive history in systemic sclerosis. Arthritis Rheum 2008; 59:1661.
- Steen VD, Medsger TA Jr. Fertility and pregnancy outcome in women with systemic sclerosis. Arthritis Rheum 1999; 42:763.
- Giordano M, Valentini G, Lupoli S, Giordano A. Pregnancy and systemic sclerosis. Arthritis Rheum 1985; 28:237.
- Steen VD, Conte C, Day N, et al. Pregnancy in women with systemic sclerosis. Arthritis Rheum 1989; 32:151.
- Bermas BL, Hill JA. Effects of immunosuppressive drugs during pregnancy. Arthritis Rheum 1995; 38:1722.
- Taraborelli M, Ramoni V, Brucato A, et al. Brief report: successful pregnancies but a higher risk of preterm births in patients with systemic sclerosis: an Italian multicenter study. Arthritis Rheum 2012; 64:1970.
- Steen VD. Scleroderma and pregnancy. Rheum Dis Clin North Am 1997; 23:133.
- Bellucci MJ, Coustan DR, Plotz RD. Cervical scleroderma: a case of soft tissue dystocia. Am J Obstet Gynecol 1984; 150:891.
- Stenchever MA, Ng AB. Scleroderma of the uterine cervix. Am J Obstet Gynecol 1970; 107:965.
- Ballou SP, Morley JJ, Kushner I. Pregnancy and systemic sclerosis. Arthritis Rheum 1984; 27:295.
- Traub YM, Shapiro AP, Rodnan GP, et al. Hypertension and renal failure (scleroderma renal crisis) in progressive systemic sclerosis. Review of a 25-year experience with 68 cases. Medicine (Baltimore) 1983; 62:335.
- Black CM, Stevens WM. Scleroderma. Rheum Dis Clin North Am 1989; 15:193.
- Baethge BA, Wolf RE. Successful pregnancy with scleroderma renal disease and pulmonary hypertension in a patient using angiotensin converting enzyme inhibitors. Ann Rheum Dis 1989; 48:776.
- Karlen JR, Cook WA. Renal scleroderma and pregnancy. Obstet Gynecol 1974; 44:349.