Kumar M*, Mali K**, Warke HS***.
(* Junior Resident, ** Assistant Professor, *** Associate Professor, Department of Gynecology and Obstetrics, Seth GS Medical College and KEM Hospital, Mumbai, India.)
Takayasu Arteritis is a rare, chronic vasculitis of unknown etiology characterized by inflammation of the aorta or its branches. Females are more likely to be affected 8-9 times more than males. Features of the disease include pulseless upper extremities or with weak or absent pulse, leading to the name “pulseless disease”. There is not much effect of pregnancy on the evolution of the disease. However, the second and third trimesters of pregnancy warrant specific care. This case report describes one such pregnancy where prompt management led to good maternal and fetal outcome.
She was on tablets prednisolone, azathioprine, cyclosporine, aspirin, and nifedipine, and during pregnancy required addition of tablet labetalol. At 13 weeks of gestation, she had an episode of breathlessness, with wheezing following which she was given metred dose inhaler salbutamol. Her haemoglobin was 9.5gm %, white blood count of 10,000/ cu mm and platelet 1.46 lakh. Renal Doppler there was no evidence of renal artery stenosis All other investigations were within normal limits. Antenatal follow up was otherwise uneventful. Non-stress test done biweekly from 32 weeks of gestation was reactive. Obstetric Doppler showed symmetrical intrauterine growth restriction with oligohydramnios and normal Doppler flow studies. She was admitted in medicine department twice during antenatal period with complaints of breathlessness, giddiness and palpitations which was managed symptomatically, and discharged within 3 days. Induction of labor with dinoprostone gel was done at 38 weeks in view of IUGR with oligohydramnios. She underwent emergency LSCS in view of fetal distress in first stage of labour under general anesthesia, and delivered a healthy female child of 2.3 kg, not requiring NICU stay. Post operatively, she had accelerated hypertension with BP in right arm 190/100mmhg and left arm 140/90, and was shifted to ICU postoperatively and was aggressively monitored, requiring intravenous labetalol. At discharge she was sent on Nifedipine sustained release 20 mg given four times a day and tablet labetalol 100 mg thrice a day. Postoperative Echocardiography did not show any worsening. Recovery was otherwise uneventful and was discharged on day 15 postoperatively.
Pregnancy does not appear to interfere with the disease progression. In our patient also, the postoperative echocardiogram showed similar findings as that done during early pregnancy. However, it has implications on pregnancy like abortions, intrauterine growth restriction, intrauterine death and abruption. In our patient, though she did not have prior abortions, she did have intrauterine growth restriction. Prompt intervention prevented poor neonatal outcomes.
Presence of renal disease raises the chance of occurrence of IUGR. Renal Doppler and renal function tests were also normal. Retinopathy, secondary hypertension and aneurysmal complications can occur. Hence, patient has been asked to have meticulous follow up of the disease even after pregnancy, the need for which should be emphasized. Obstetric Doppler is beneficial in the evaluation of fetal well-being in women with Takayasu arteritis, as in our case also. Pre-conceptional counseling is essential regarding timing of pregnancy and modification of dose of cytotoxic drugs in the peri-conceptional period. Conception during remission phase is preferable. Along with routine antenatal visits, serial monitoring of blood pressure, renal function, cardiac status and fetal Doppler is also imperative as per requirement.
Treatment includes starting antihypertensive and antiplatelet as per need. Steroid therapy at a dose of 1-2mg/kg/body weight is suggested. Our patient was on prednisolone tablets throughout pregnancy. Use of immunomodulators like mycophenolate mofetil, infliximab, toclizumab and azathioprine is gaining popularity, however their safety in pregnancy has not been established. This case adds to the literature that use of azathioprine in pregnancy did not result in any specific adverse outcome. Vaginal delivery is the preferred mode of delivery epidural analgesia advocated for labor and delivery. In women with advanced disease, lower segment caesarean section is preferred to prevent sudden cardiac deterioration due to changes in blood volume and pressure. The overall 5-year survival rate after diagnosis is 83.1 %. Death is usually due to congestive heart failure or cerebrovascular events.
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