Interstitial restrictive lung disease is common and frequently becomes life threatening. Associated pulmonary hypertension is treated with bosentan or sildenail (Chap. 49, p. 960).
The prevalence of scleroderma in pregnancy approximates 1 in 22,000 pregnancies (Chakravarty, 2008). These women usually have stable disease during gestation if their baseline unction is good. As perhaps expected, are aggravated by pregnancy (Steen, 1999). Dysphagia results from loss of esophageal motility due to neuromuscular dysunction. A decrease in amplitude or disappearance of peristaltic waves in the lower two thirds of the esophagus is seen using manometry. Symptomatic treatment for reflux is described in Chapter 54 (p. 1046).
Women with renal insuiciency and malignant hypertension have a higher incidence of superimposed preeclampsia. With rapidly worsening renal or cardiac disease, pregnancy termination should be considered. As discussed, renal crisis is life threatening and is treated with ACE inhibitors, but it does not improve with delivery (Gayed, 2007). Pulmonary hypertension usually contraindicates pregnancy.
Vaginal delivery may be anticipated, unless the soft tissue thickening wrought by scleroderma produces dystocia requiring cesarean delivery. Tracheal intubation for general anesthesia has special concerns because of limited ability of these women to open their mouths widely (Sobanski, 2016). Because of esophageal dysfunction, aspiration is also more likely, and epidural analgesia is preferable. Warming the delivery room and intravenous fluids, extra blankets, and socks and gloves are recommended to improve impaired circulation from Raynaud phenomenon. If corticosteroids were used frequently, stress doses of hydrocortisone are recommended (Sobanski, 2016).
Maternal and fetal outcomes correlate with underlying dis ease severity. In a review of 214 gravidas with systemic sclerosis, 45 percent had difuse disease. Major complications included renal crisis in three and greater rates of preterm birth (Steen, 1989, 1999). Chung and coworkers (2006) also reported elevated rates of preterm delivery, fetal-growth restriction, and perinatal mortality. A multicenter study of 109 pregnan cies from 25 centers reported higher rates of preterm delivery, fetal-growth restriction, and very-low-birthweight newborns (Taraborelli, 2012). hese are likely related to placental abnor malities that include decidual vasculopathy, acute atherosis, and infarcts (Sobanski, 2016).
Scleroderma may be associated with subfertility (Bernatsky, 2008; Lambe, 2004). For women who do not choose preg nancy, several reversible contraceptive methods are acceptable.
However, hormonal agents, especially combination oral con traceptives, probably should not be used, especially in women with pulmonary, cardiac, or renal involvement. Due to the often unrelenting progression of systemic sclerosis, permanent sterilization is also considered.