Clinicopathology of maternal scleroderma

Women with scleroderma who become pregnant have an increased incidence of perinatal loss, premature birth, and growth restriction [1]. Placental abnormalities, including acute atherosis, infarcts, and other manifestations of reduced placental blood flow, are found in many cases of collagen vascular disease [2]. A 27-year-old woman (gravida 1) with scleroderma was admitted to hospital at 31 weeks of gestation with uterine contractions and intrauterine growth restriction (IUGR). Scleroderma was diagnosed 2 years previously and she was treated with penicillamine (250 mg per day). Antitopoisomerase I (anti-Scl-70) and anti-nuclear antibodies were positive. Doppler examination revealed IUGR and increased resistance index in the umbilical artery (pulsatility index (PI), 1.24). At 37 weeks of gestation she underwent a cesarean delivery; the 1860 g female infant developed necrotic enterocolitis after 4 days. Three days postpartum the patient developed elevated blood pressure (170/100 mm Hg), mild proteinuria (280 mg/ 24 h), with normal serum creatinine level and liver function tests, but reduced creatinine clearance (70 mL/min). Renal crisis episode and pre-eclampsia were included in the differential diagnosis. In order to control hypertension, an angiotensin-converting enzyme (ACE) inhibitor was used. On discharge from hospital the patient had normal blood pressure and normal renal function. At histopathology examination the placenta weighed 395 g and measured 16×9×4 cm. The umbilical cord presented eccentric insertion and was 38 cm in length and 1.2 cm in diameter. It contained 3 vessels. More than 5 placental infarcts were identified (measuring 1–3 cm). Histological examination of multiple sections (15) showed old placental infarcts and changes consistent with placental mesenchymal dysplasia (PMD, Fig. 1a), decidual vasculopathy (Fig. 1b), foci of decreased vascularity, foci of chorioangiosis, stromal fibrosis of villi, and extensive fibrinoid deposition on the villi. Focal