Management of Takayasu Arteritis During Pregnancy

Abstract

A 25-year-old woman who was at 24 weeks’ gestation presented to our institution with a 2-day history of worsening epigastric and midthoracic back pain. The patient denied chest pain, shortness of breath, palpitations, nausea, and vomiting. Her medical history was significant for a history of 2 spontaneous abortions, hypertension, and anemia. Her only medications were prenatal vitamins and iron supplements. On physical examination, blood pressure (BP) of 120/70 mm Hg was noted in the right arm and 152/90 mm Hg in the left arm. Neck examination revealed 1+ bilateral carotid pulsations with loud carotid and subclavicular bruits. There was no jugular venous distension or thyromegaly. Normal first and second heart sounds were normal intensity with II/VI diastolic murmur at the left upper sternal border. Abdominal examination revealed positive bowel sounds, tender epigastrium with no fundal tenderness, rebound, or guarding. Lower extremities revealed 2+ pitting edema. Right upper extremity (radial and brachial) pulses were absent with 1+ left brachial and radial pulses. Initial laboratory values included mild anemia with hemoglobin of 11 g/dL. White blood cell and platelet counts, chemistries, liver function tests, urine toxicology, and pancreatic enzymes were within normal limits. Urinalysis revealing a 4+ proteinuria. Erythrocyte sedimentation rate was 41 mm/hr and C-reactive protein was 4.4, respectively. Electrocardiography results showed normal sinus rhythm at 84 beats per minute with no ST/T-wave changes. Chest x-ray showed a mildly ectatic thoracic aorta. Fetal heart tracing at baseline was 140 to 150 seconds with minimal heart rate variability. The patient was admitted to the obstetrics ward for preeclampsia and fetal monitoring. Considering the vascular and cardiac examination findings, the cardiology service was consulted. Transthoracic echocardiography was performed and revealed normal left and right systolic function with mild to moderate aortic insufficiency. No valvular or congenital abnormalities were identified. A computed tomography (CT) scan of the chest without contrast revealed circumferential wall thickening of the descending aorta with no dissection. Limited magnetic resonance imaging (MRI) of the abdomen without gadolinium showed irregular wall thickening of the aorta and significant stenoses of the proximal celiac and superior mesenteric arteries. Based on American Society of Rheumatology classification criteria, the patient was diagnosed with Takayasu arteritis and started on intravenous steroids. She later underwent duplex ultrasonography of the neck, which showed absence of flow in the innominate, common carotid, and right vertebral artery. Flow was maintained in the internal carotid artery through retrograde flow in the external carotid artery. The right subclavian artery was occluded with minimal flow in the right axillary artery through collaterals, which was later confirmed by selective angiography after pregnancy. The following morning, the patient complained of recurrent epigastric abdominal pain and had elevated BP 196/108 mm Hg measured from the left arm BP cuff. The patient was started on labetolol via oral and intravenous routes as well as methyldopa. These measures only temporarily decreased her BP and she was transferred to the intensive care unit. After placement of an arterial line in her left femoral artery for BP monitoring, the patient was started on an esmolol drip titrated to keep systolic BPs around 140 mm Hg. Fetal ultrasonography revealed a growth-restricted infant with high cephalization index, which is a screening tool for brain maturity based on the ratio of head circumference to body weight. There was absent end-diastolic flow through the umbilical artery and elevated systolic to diastolic ratio indicating poor fetal perfusion. Considering these findings, the decision was made to emergently deliver the baby. The patient underwent classical cesarean section and a 1 pound 4 ounce male infant was born without complications. The patient did well postoperatively. She decided not to breastfeed and was discharged on oral prednisone and lisinopril. The patient had a follow-up appointment 1 month after delivery, at which time she was asymptomatic. Her BP and inflammatory markers were normal. She underwent a selective angiography that revealed chronic occlusions of right common carotid and right subclavian arteries (Figure 1). There was significant cervical collaterals, which reconstitute the contralateral axillary and subclavian arteries as well as bilateral carotid bifurcations. Abdominal aortography showed significant stenosis of left renal, celiac, and mesenteric arteries (Figure 2). With her extensive disease, the lesions were deemed unsuitable for angioplasty, and surgical revascularization would be considered if the patient became symptomatic. The patient expressed no desire to become pregnant again and a bilateral tubal ligation was performed. Cerebral angiogram 1 month after delivery showing chronic occlusions of right common carotid (white arrow) and right subclavian (black arrow) arteries. Abdominal angiogram 1 month after delivery revealing an ectatic abdominal aorta (white arrow) and high-grade stenosis at the origin of the left renal artery (black arrow). Takayasu arteritis is a chronic idiopathic vasculitis affecting the aorta and its primary branches. It was first recognized by a Japanese ophthalmologist, Dr Mikoto Takayasu, in 1908 who reported ocular changes in a 21-year-old Japanese woman. Takayasu arteritis commonly affects young women of Asian or oriental descent during childbearing age. Symptoms range from fever, fatigue, and weight loss to life-threatening hemoptysis and heart failure from aortic regurgitation.1,2 Physical examination may lead to the detection of reduced pulse rate, differences of BP between the arms, or bruits over the neck, supraclavicular areas, and abdomen. The diagnosis is usually confirmed by CT, MRI, or digital subtraction angiography revealing the characteristic tapered luminal narrowing or occlusion that is accompanied by thickening of the vessel wall. The American Society of Rheumatology has devised classification criteria for Takayasu arteritis3 that helps distinguish this disorder from other forms of vasculitides (Table). Takayasu arteritis is diagnosed if at least 3 of the 6 criteria are present in a patient. This classification yields a sensitivity and specificity of 90.5% and 97.8%, respectively. Our patient fulfilled all 6 classification criteria for diagnosis of Takayasu arteritis. Because the incidence of Takayasu arteritis during childbearing years is relatively high, the management of pregnancies with this disease is of great importance in clinical obstetrics. Pregnancy with Takayasu arteritis can be complicated by hypertension, as seen in our case, and worsening of cardiovascular hemodynamic status. Hypertension is a serious complication that can lead to intrauterine growth retardation, fetal hemorrhage, and maternal heart failure.4 The increased intravascular volume seen during pregnancy may impair circulation and exacerbate maternal hypertension, aortic regurgitation, and congestive heart failure.5 BP control is of paramount importance as increases can cause rupture of aneurysms and falls in BP can lead to cerebral ischemia in the mother. Peripheral BP monitoring may not be accurate, complicating the treatment of hypertension in these patients. Most patients benefit from invasive BP monitoring or by monitoring peripheral BPs in multiple extremities as upper extremity pulses may be absent.6 Antihypertensive agents that are safely used with proven success in pregnancy are labetolol, hydralazine, and alpha-methyldopa. Routine tests in pregnant patients with Takayasu arteritis include serial ultrasound growth assessment, umbilical artery Doppler studies, and biophysical profile assessment from 24 weeks of gestation.7 Most cases of Takayasu arteritis during pregnancy have been reported in patients with a known diagnosis prenatally. We consider our case to be unique and quite rare because the diagnosis was made during pregnancy with the presenting symptom of abdominal pain. In our case, the diagnostic options were limited secondary to her pregnancy. In order to limit the radiation exposure to the mother and the baby, we decided to do a CT scan of the chest without contrast and a limited MRI of the abdomen with good diagnostic yield. Gadolinium was not used because it has been shown to cross the placenta and because its long-term effects on the fetus are largely unknown.8 Previous reports suggest that Takayasu arteritis during pregnancy may lower the inflammatory activity and hemodynamic status as shown by lowering of C-reactive protein levels.9 Takayasu arteritis during pregnancy has been associated with favorable maternal and fetal outcomes for unknown reasons. Our case highlights the importance of meticulous BP control and a multidisciplinary management approach during pregnancy in order for successful delivery.