Takayasu’s arteritis (TA), also known as pulseless disease/aortoarteritis/“young female arteritis,” is a rare chronic inflammatory progressive large vessel vasculitis (LVV) of unknown etiology afflicting women of childbearing age [
Pregnancy as such has no effect on the evolution of the disease;however, its peak incidence is in second and third trimesters. Thus, such patients warrant special attention during the peripartum period owing to the likelihood of development of complications such as hypertension, multiple organ dysfunction, and stenosis hindering regional blood flow leading to restricted intrauterine fetal growth and low birth weight in babies [
A 25-year-old primigravida was admitted in the hospital as pregnancy with chronic hypertension (not on any antihypertensives), at 36 completed weeks in active labor. She was a known case of active TA on treatment (taking prednisolone 2 mg, aspirin 150 mg, and clopidogrel 75 mg OD.) She was booked and supervised throughout her pregnancy at the same hospital, in liaison with cardiologists. Her past and personal histories were thoroughly reviewed in outpatient department; she had been a bidi smoker since teenage, one-two per day. She had surgical correction of complete stenosis of right common carotid artery (CCA) and right vertebral and subclavian artery by percutaneous stenting of right brachiocephalic (size 7 × 39 mm) and right CCA (size 9 × 30 mm) in some peripheral hospital. She had no history of other comorbidities like IBD and sinusitis. She had an uneventful antenatal and intrapartum period and delivered a healthy neonate weighing 2.8 kg. During her postpartum period, all necessary investigations were done and consultations were taken, and she was discharged on drugs after one week in stable condition, with the advice for regular follow-up and abstinence from smoking. During her subsequent visits, her MRA scan showed markedly attenuated flow in left CCA, for which she was conservatively managed by cardiologist.
One year later, she had an accidental second conception but could not get herself booked for antenatal care anywhere till the third trimester, due to social and financial issues. At her first visit at 34 weeks in the institute, she was admitted for safe confinement for severe fetal growth restriction and neurological sequelae of aortoarteritis in the form of tonic-clonic convulsions (three episodes in last 24 hours before admission). She gave history of myalgias, arthralgias, and fever off and on throughout her pregnancy, despite continued glucorticoid treatment. On admission, her BP was 200/110 in ankle and pulse was 100/min. There was no albuminuria. Per-abdomen examination showed a fundal height corresponding to 26 weeks with faintly audible fetal heart sound. She was put on strict fetomaternal surveillance. After thorough history taking, cardiology, and CTVS references were taken, her anticoagulants were stopped in v/o anticipated termination of pregnancy, and she was started on labetalol 300 mg in divided doses and continuous BP monitoring (noninvasive). A neurology consultation was done, and levetiracetam 1000 mg was started to take care of seizures. All routine antenatal and specific blood investigations (INR, PT, and APTT) were normal. Though CRP was also normal, it was on a higher side of normal range (3 mg/dL). ESR was found to be raised being equal to 33 mm/1st hour. Echocardiography was performed which revealed mild concentric LVH, moderate AR (38 mm diameter), mildly thickened aortic valve leaflets, and ejection fraction 63% (Figure
Carotid artery Doppler of the patient.
Echocardiography of the patient.
Obstetric ultrasound of the patient.
Takayasu’s arteritis is a LVV with aortic inflammation leading to proximal occlusion and/or aneurysms of carotid, subclavian, pulmonary, iliac, and renal arteries [
Various types of TA have been acknowledged in the past: type I (disease embroiling aortic arch and its branches), type II (lesions constrained to descending thoracic aorta and abdominal aorta), type III (patients with characteristics of types I and II), type IV (involvement of pulmonary artery), and type V (combined features of types IIb and IV) [
Pregnancy does not interfere with disease progression [
Diagnosis is usually based on clinical manifestations, inflammatory markers (acute phase reactants), and arteriography demonstrating aortic stenosis and of its branches. Common features of active TA are fatigue, myalgia, arthralgia, and low-grade fever in initial stages and intermittent claudication, visual defects, and fainting attacks in later stages. Many may be diagnosed after clinical examination, when one or more peripheral pulses are not palpable or blood pressures vary in two limbs. However, computed tomography or magnetic resonance angiography can detect TA even before the development of severe vascular compromise as in our case [
Involvement of large vessels in our case excludes granulomatosis with polyangiitis and Behcet’s disease. Another cause of LVV is giant cell arteritis, which was one of the primary differential diagnoses in our case. But younger age, lower values of raised ESR, absence of any new onset headache, visual symptoms, and jaw claudication symptoms favored the diagnosis of TA [
Management of TA entails an interdisciplinary approach with involvement of obstetricans, anesthesiologists, cardiologists, rheumatologists, and neonatologist in a tertiary care center. The aims are control of inflammation, prevention and treatment of complications like hypertension and revascularization by percutaneous angioplasty, use of endoprosthesis, or surgical correction for occlusive and stenotic lesions.
Preconception counselling is essential regarding dosage adjustment or cessation of cytotoxic drugs, folic acid supplementation in the periconceptional period, and optimal timing of pregnancy. Pregnancy should be ideally planned in remission phase. There should be an early booking with regular antenatal supervision. Along with routine antenatal visits, serial monitoring of blood pressure, renal function, cardiac status, and preeclamptic screening are vital in such patients. Fetal surveillance including daily fetal kick count, gravidogram, serial fetal biometry, biophysical profile, and fetal Doppler is also imperative as per requirements [
Blood pressure monitoring can be challenging in patients with pulseless peripheral arteries. In most cases described in literature, and in the present case, it was possible to use the noninvasive technique [
Utilization of immunomodulatory agents like mycophenolate mofetil, infliximab, tocilizumab, leflunomide, and abatacept has gained momentum in recent times for treatment of TA, especially in refractory cases [
Vaginal delivery is the preferred mode, and epidural analgesia has been advocated for labor and delivery. In women with hypertension, delivery should be abbreviated by the use of outlet forceps. In women with stages IIb and III, LSCS is preferred to prevent cardiac decompensation due to increased blood volume and blood pressure observed during uterine contractions and increased cardiac output observed during labor. Our patient was hemodynamically stable and was also induced as she belonged to group IIa and she did not have additional supplementary investigation of the abdominal vessels.
Patients with metallic valvular prosthesis should be maintained anticoagulated during pregnancy. The choice of medication should take into account the probable due date and reversibility of the method. Heparin should be discontinued 4 to 6 hours before anesthesia, and it can be reversed with protamine if the gravida goes into labor or in case of bleeding. Patients on prophylactic doses of enoxaparin should receive their last dose 12 hours before anesthesia. In the case of therapeutic doses the drug should be discontinued 24 hours before anesthesia.
Table
Study | Parity | Clinical presentation | Maternal complications | Mode of delivery | Fetal outcome |
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Leal et al. (2011) [ | G4P3l3 | Precordial squeezing pain | Controlled HTN | Elective LSCS at 39 weeks | 2.7 kgs live baby |
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Rengaraj and Rani (2015) [ | PGR | Dyspnea | GTCS | Outlet forceps | 2.4 kgs live baby |
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Nalini and Santa (2015) [ | PGR | Oliguria | IUGR | Elective LSCS at 37 weeks | 2.4 kgs live baby |
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De Lucena et al. (2008) [ | G3P1L0 | Dyspnea | Uncontrolled | Elective LSCS at 37 weeks | 2.1 kgs |
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Soma-Pillay et al. (2015) [ | G2P1L1 | Claudication in LL | Compensated HTN | Elective LSCS | 2.3 kgs |
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Satia et al. (2016) [ | G3A2 | Exertional dyspnea | Dilated cardiomyopathy | MTP at 6 weeks | |
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Khandelwal and Gandhi (2016) [ | G7P1A5 | Blurring of vision | Grade IV hypertensive retinopathy | Elective LSCS at 37 weeks | 2.5 kgs live baby |
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Present report (2017) | G2P1L1 | Pain in right lower limb | Controlled HTN | NVD | 1.2 kgs live baby |
The overall five-year survival rate after diagnosis was 83.1%. Death typically is a consequence of congestive heart failure or cerebrovascular events. The survival is better in patients without a progressive course and in those below 35 years of age. Early diagnosis with proper medical or surgical management is essential for a good prognosis. A high index of clinical suspicion in patients presenting with pulseless peripheral vessels could be kept in mind to optimize the management following a multidisciplinary approach.
Pregnancy with TA presents as an onerous medical condition to manage for an obstetrician.
The authors declare that there is no conflict of interests regarding the publication of this paper.
The authors are grateful to their patient, who besides having experienced this rare condition allowed them to evaluate her in detail for a better comprehension of the clinical entity.