Author Information
Fernandes S*, Rao SV**, D’Almeida J*, Kumari P***
(* Assistant Professor, ** Professor, *** Senior Resident, Department of Obstetrics and Gynecology, Father Muller Medical College, Mangalore, India.)
Abstract
Takayasu arteritis is a vasculitis of unknown etiology affecting women of the reproductive age group and involving branches of the aortic arch. Pregnancy may have a favourable outcome, but complications are bound to happen particularly for those diagnosed for the first time in pregnancy. Although, those already diagnosed may enter pregnancy in remission and on medications. Here a case of pregnancy with Takayasu arteritis is presented.
Introduction
Takayasu arteritis (TA), also known as pulseless disease/ aortoarteritis, is a chronic inflammatory progressive large vessel vasculitis of unknown etiology affecting women of childbearing age.[1–3] It leads to narrowing, occlusion and aneurysms of systemic and pulmonary arteries, affecting primarily the aorta and its branches.[4] Patients warrant special attention during the peripartum period owing to stenosis hindering regional blood flow.[5,6] An interdisciplinary collaboration of obstetricians, cardiologists, rheumatologists, and neurologists is necessary for optimal maternal and fetal prognosis.
Case Report
26 year old gravida 3 para 1 living 1 abortion 1 presented to the outpatient department at 34 weeks for the first time. She was admitted for evaluation of elevated blood pressure. She had prior antenatal visits elsewhere and was on tablet labetalol 100 mg since third month of pregnancy. Upon detailed clinical examination, a difference of more than 20 mm Hg was noted between the two upper limbs. On detailed questioning, she revealed a history of takayasu arteritis. Previous medical records revealed that almost 10 years prior, she had presented with claudication in both lower limbs. Lower limb pulses were feeble bilaterally, with an abdominal bruit. ESR and CRP had been elevated with mild anemia and ANA negative. CT Angiogram had revealed an occluded infra renal abdominal aorta with focal stenotic lesions at origin of celiac axis and superior mesenteric artery and few circumferential aortic wall thickening involving descending thoracic aorta suggestive of aorto-arteritis. She had undergone angiography with stenting of infra renal abdominal aorta. She had been discharged on steroids, methotrexate and folinic acid. She was on regular follow up with rheumatologist. Doppler done two years later showed monophasic waveform of the left lower limb arterial system suggesting abdominal aorta stenosis and normal colour flow in right lower limb arterial system with the stent in situ. Following this, she got married and had a spontaneous abortion at 2 months for with suction evacuation was done.
Her second pregnancy was four years later. She presented at term in labour to our hospital. She was on tablet labetalol 200 mg once a day at the time of admission. She had at no point of time revealed history of TA. She delivered a female 3 kg baby uneventfully.
In the present pregnancy, she had no history of claudication. She was found to be anemic and was treated with parenteral iron. Tablet labetalol was stepped up to 200 mg thrice a day in view of persistently elevated BP. Rheumatologists opined that gadolinium enhanced MR Angiography was to be done post-delivery.
She was induced at 37 completed weeks with dinoprostone gel and delivered a male child of 2.575 kg baby. She was discharged on tablet nifedipine sustained release 20 mg twice a day.
At follow up postnatally, right arm blood pressure was 150/80 mm Hg and left arm blood pressure was 120/70 mm Hg. She was advised to follow up regularly and ensure that she declares her medical condition to any medical practitioner she visits for any reason.
Her second pregnancy was four years later. She presented at term in labour to our hospital. She was on tablet labetalol 200 mg once a day at the time of admission. She had at no point of time revealed history of TA. She delivered a female 3 kg baby uneventfully.
In the present pregnancy, she had no history of claudication. She was found to be anemic and was treated with parenteral iron. Tablet labetalol was stepped up to 200 mg thrice a day in view of persistently elevated BP. Rheumatologists opined that gadolinium enhanced MR Angiography was to be done post-delivery.
She was induced at 37 completed weeks with dinoprostone gel and delivered a male child of 2.575 kg baby. She was discharged on tablet nifedipine sustained release 20 mg twice a day.
At follow up postnatally, right arm blood pressure was 150/80 mm Hg and left arm blood pressure was 120/70 mm Hg. She was advised to follow up regularly and ensure that she declares her medical condition to any medical practitioner she visits for any reason.
Discussion
Takayasu’s arteritis is an inflammatory condition. It can lead to proximal occlusion or aneurysms of aortic branches like subclavian, carotid, renal or iliac arteries.[3,7] Mean age is reported between second and third decade of life.[8,9] Etiology remains predominantly idiopathic. Autoimmunity, sex hormones, and genetic factors have been hypothesized as plausible causes.[10]
Disruption of the elastic fibres of the vessel wall, inflammatory cell infiltration with occasional granulomatous reaction, fibrotic adventitial thickening and intima are histological features.
The disease has a triphasic course, with an initial active phase where patients may have malaise, weight loss, fever, myalgia, arthralgia, and is associated with an increase in acute phase reactants. The second stage presents with cerebral, extremity or visceral ischemic changes before resulting in a quiescent phase of fibrosis. Unfortunately, such a typical course seen in only 33% of patients and the severity and progression of vessel involvement are extremely variable.[11]
Pregnancy does not interfere with disease progression.[1–3] Hypertension may be associated with abortion, preterm labor, IUGR, abruption ,IUD.[2,9,12] BP in such patients should be measured in the lower extremity to identify discrepancies. Our patient had a measurable difference in the blood pressure of the extremities. Adverse pregnancy outcomes are associated with involvement of abdominal aorta. Arterial ultrasound Doppler quantifying uterine artery flow may have benefit in women with TA.
According to the Angiographic Classification(1994), our patient was of Type 3 TA.[13] The disease can be staged depending on the presence of major complications like hypertension, retinopathy, aneurysms, and aortic insufficiency[14,15] Our patient did not have any of these complications.
Diagnosis is usually based on clinical findings, raised inflammatory markers and angiographic demonstration of stenosis. However, vessel biopsy remains the gold standard.[16]
Management requires an interdisciplinary approach in a tertiary care center aiming to control inflammation, prevention and treatment of complications and revascularization by percutaneous angioplasty or surgical correction of lesions. Preconception counselling is essential. Dosage adjustment or cessation of cytotoxic drugs, periconceptional folic acid and optimal timing of pregnancy which is when the disease is in remission. However, despite having diagnosis in the prior pregnancy itself, our patient came out with the history of such disease only late in pregnancy. This is probably because the patient did not completely understand the possible severity and importance of the condition.
Along with routine antenatal visits, blood pressure monitoring, renal function, cardiac status, and preeclampsia screening are vital as is fetal surveillance.[17]
Antihypertensives and antiplatelets may be started. Some symptoms of TA may respond to steroid therapy.[18] Vaginal delivery is the preferred mode of delivery. Epidural analgesia can be advocated for labor and delivery. In women with advanced stages, LSCS may be preferred to prevent cardiac decompensation due to increased cardiac output observed during labor.
The overall five-year survival rate after diagnosis is around 80%. Death is a consequence of congestive heart failure or cerebrovascular events. Survival is better in patients without a progressive course and in those below 35 years of age.
Disruption of the elastic fibres of the vessel wall, inflammatory cell infiltration with occasional granulomatous reaction, fibrotic adventitial thickening and intima are histological features.
The disease has a triphasic course, with an initial active phase where patients may have malaise, weight loss, fever, myalgia, arthralgia, and is associated with an increase in acute phase reactants. The second stage presents with cerebral, extremity or visceral ischemic changes before resulting in a quiescent phase of fibrosis. Unfortunately, such a typical course seen in only 33% of patients and the severity and progression of vessel involvement are extremely variable.[11]
Pregnancy does not interfere with disease progression.[1–3] Hypertension may be associated with abortion, preterm labor, IUGR, abruption ,IUD.[2,9,12] BP in such patients should be measured in the lower extremity to identify discrepancies. Our patient had a measurable difference in the blood pressure of the extremities. Adverse pregnancy outcomes are associated with involvement of abdominal aorta. Arterial ultrasound Doppler quantifying uterine artery flow may have benefit in women with TA.
According to the Angiographic Classification(1994), our patient was of Type 3 TA.[13] The disease can be staged depending on the presence of major complications like hypertension, retinopathy, aneurysms, and aortic insufficiency[14,15] Our patient did not have any of these complications.
Diagnosis is usually based on clinical findings, raised inflammatory markers and angiographic demonstration of stenosis. However, vessel biopsy remains the gold standard.[16]
Management requires an interdisciplinary approach in a tertiary care center aiming to control inflammation, prevention and treatment of complications and revascularization by percutaneous angioplasty or surgical correction of lesions. Preconception counselling is essential. Dosage adjustment or cessation of cytotoxic drugs, periconceptional folic acid and optimal timing of pregnancy which is when the disease is in remission. However, despite having diagnosis in the prior pregnancy itself, our patient came out with the history of such disease only late in pregnancy. This is probably because the patient did not completely understand the possible severity and importance of the condition.
Along with routine antenatal visits, blood pressure monitoring, renal function, cardiac status, and preeclampsia screening are vital as is fetal surveillance.[17]
Antihypertensives and antiplatelets may be started. Some symptoms of TA may respond to steroid therapy.[18] Vaginal delivery is the preferred mode of delivery. Epidural analgesia can be advocated for labor and delivery. In women with advanced stages, LSCS may be preferred to prevent cardiac decompensation due to increased cardiac output observed during labor.
The overall five-year survival rate after diagnosis is around 80%. Death is a consequence of congestive heart failure or cerebrovascular events. Survival is better in patients without a progressive course and in those below 35 years of age.
Conclusion
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 should be kept in mind to optimize the management following a multidisciplinary approach.
References
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- Sise MJ, Counihan CM, Shackford SR, Rowley WR. The clinical spectrum of Takayasu's arteritis. Surgery. 1988 Nov;104(5):905-10.
- Hauenstein E, Frank H, Bauer JS, Schneider KT, Fischer T. Takayasu's arteritis in pregnancy: review of literature and discussion. J Perinat Med. 2010;38(1):55-62.
- Moriwaki R, Noda M, Yajima M, Sharma BK, Numano F. Clinical manifestations of Takayasu arteritis in India and Japan--new classification of angiographic findings. Angiology. 1997 May;48(5):369-79.
- Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990 Aug;33(8):1129-34.
- Wilke WS. Large vessel vasculitis (giant cell arteritis, Takayasu arteritis). Baillieres Clin Rheumatol. 1997 May;11(2):285-313.
- Sharma BK, Sagar S, Singh AP, Suri S. Takayasu's arteritis in India. Heart Vessels 1992; 7(Suppl):37-43.
- Papandony MC, Brady SR, Aw TJ. Vasculitis or fibromuscular dysplasia? Med J Aust. 2015 Feb 2;202(2):100-1.
- Ioscovich A, Gislason R, Fadeev A, Grisaru-Granovsky S, Halpern S. Peripartum anesthetic management of patients with Takayasu's arteritis: case series and review. Int J Obstet Anesth. 2008 Oct;17(4):358-64.
Citation
Fernandes S, Rao SV, D’Almeida J, Kumari P. Takayasu Arteritis In Pregnancy: A Case Report From A Tertiary Hospital In South India. JPGO 2018. Volume 5 No.5. Available from:http://www.jpgo.org/2018/04/takayasu-arteritis-in-pregnancy-case.html