Table of Contents  
Year : 2021  |  Volume : 8  |  Issue : 2  |  Page : 119-121

Management of vasculitis in pregnancy

1 Department of Obstetrics and Gynaecology, AIIMS, Bibinagar, Telangana, India
2 Department of Vascular and Endovascular Surgery Apollo Hospital, Hyderabad, Telangana, India

Date of Submission31-May-2020
Date of Decision06-Jun-2020
Date of Acceptance13-Jun-2020
Date of Web Publication13-Apr-2021

Correspondence Address:
Nabnita Patnaik
Department of Obstetrics and Gynaecology, AIIMS, Bibinagar, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijves.ijves_74_20

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Vasculitis in pregnancy is a rare disease-modifying effect on autoimmune connective tissue diseases such as vasculitis and that connective tissue disease can have an adverse effect on pregnancy. It is essential that pregnant women with vasculitis are managed to the highest standards of care in order to prevent negative outcomes not only for the mother but also for the child. Vasculitis in pregnancy is probably underdiagnosed and under-reported; cases of vasculitis are rare and so disease flares may not always be recognized by a general clinician. Preconceptual counseling is critical, and assessment of disease activity, major organ involvement, and risk factors, such as hypertension and renal impairment, will help to guide both the woman and her clinicians as to the risks entailed in embarking on a pregnancy. Such patients are best catered for in multidisciplinary settings where there is access to specialized care from nephrologists, rheumatologists and respiratory medicine physicians, as well as obstetricians and pediatricians skilled in caring for complex pregnancies. This article aims to provide an approach to managing these patients with the best current evidence-based practice.

Keywords: Antiphospholipid antibody syndrome, pregnancy, systemic lupus erythematosus, vasculitis

How to cite this article:
Patnaik N, Pradhan NR. Management of vasculitis in pregnancy. Indian J Vasc Endovasc Surg 2021;8:119-21

How to cite this URL:
Patnaik N, Pradhan NR. Management of vasculitis in pregnancy. Indian J Vasc Endovasc Surg [serial online] 2021 [cited 2023 Jan 29];8:119-21. Available from:

  Introduction Top

The general principles guiding the treatment of vasculitis are to induce and maintain remission before commencing a pregnancy, to recognize and promptly treat any relapse both during pregnancy and postnatally, and to avoid drug toxicity. These principles remain true when the vasculitis affects a woman during pregnancy. It is paramount to induce and/or maintain remission, as active disease can be life threatening to both mother and child, but at the same time, it is extremely important to try to control the disease with a regimen that causes as little teratogenicity and harm to the fetus as possible for drugs used in vasculitis and their risks in pregnancy. Patients with vasculitis are at increased risk of thromboembolic events as pregnancy is a further thrombotic risk factor; aspirin and/or heparin may need to be considered.

  Overview of Vasculitis Top

Systemic vasculitis is a term encompassing a diverse group of diseases that are all characterized by inflammatory cell infiltration and necrosis of blood vessel walls. The primary systemic vasculitides include Wegener's granulomatosis (WG), Takayasu's arteritis (TA), polyarteritis nodosa (PAN), microscopic polyangiitis (MPA) and Churg–Strauss syndrome (CSS). Vasculitis[1],[2] can also be secondary to an established connective tissue disease, such as systemic lupus erythematosus or rheumatoid arthritis, or to infection or malignancy. This article will discuss the primary systemic vasculitis.

The management of vasculitis in pregnancy depends on; whether the vasculitis affects pregnancy outcome or the treatment of the vasculitis in pregnancy affects the pregnancy outcome.[3],[4]

  Takayasu's Arteritis Top

TA predominantly affects women of childbearing age and so the issue of pregnancy is an important one. To date, there have been three major case series of pregnant patients with TA with 76 pregnancies in total, and the guidelines below are drawn mainly from these series.[5],[6]

TA disease activity, as measured by serum C-reactive protein and erythrocyte sedimentation rate, suggests that inflammation may actually be reduced during pregnancy.[7] In any case, there does not appear to be an increase in disease activity.[8] Magnetic resonance imaging (MRI) is used as a noninvasive, nonradioactive means of assessing disease activity and response to treatment by providing high-resolution images of the vessels commonly involved in TA. Aortic wall uptake, by contrast, is suggestive of active arteritis. Positron emission tomography scanning should be avoided during pregnancy owing to the radiation dose involved, and MRI is best avoided in the first trimester of pregnancy. These tools are best used periconceptually to ensure that disease remission has been achieved before pregnancy. In general, it appears that TA is compatible with favorable maternal and fetal outcomes. Both maternal and fetal complications were more common in patients who had diffuse disease with abdominal aortic involvement and uncontrolled hypertension and in those who presented to antenatal services late in pregnancy. Fertility is not adversely affected by TA.

Treatment of Takayasu's arteritis in pregnancy

Corticosteroids are the first-line treatment, as in nonpregnant disease. In nonpregnant cohorts, approximately half of all patients treated for TA respond to corticosteroids and approximately a third of nonresponders achieve remission with subsequent cytotoxics. If first-line steroid therapy fails, then an appropriate cytotoxic agent thought to be least harmful in pregnancy should be used (e.g., azathioprine).

  Wegener's Granulomatosis Top

WG in pregnancy is unusual because WG is itself a rare disease that predominantly affects older men and women who are not of childbearing age [Figure 1]. It can be a very severe disease, which in itself may discourage patients from trying to conceive. In a series of published case reports,[9],[10] there are currently no agreed guidelines or standards for management of these patients.
Figure 1: (a) Cytoplasmic antineutrophil antibody; (b) Perinuclear staining of ethanol-fixed neutrophils

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Prematurity and cesarean sections are common in WG, whether the disease is controlled or not. Patients with WG who develop glomerulonephritis are at increased risk of preeclampsia, prematurity, intrauterine growth retardation, and late pregnancy loss. Patients with WG are at increased risk of venous thrombotic events over and above the risk posed by pregnancy itself. Subglottic stenosis, which is seen in pregnant women with WG, can cause significant problems, particularly at delivery, when a temporary tracheotomy may need to be considered.

Treatment of Wegener's granulomatosis in pregnancy[11]

Any relapse or new onset of the disease is potentially life threatening, as it is in the nonpregnant state, and therefore needs to be treated aggressively with the most appropriate medication. Where possible, drugs thought to be relatively safe in pregnancy (e.g., azathioprine and prednisolone) should be used. Some patients have remained in remission on prednisolone monotherapy during pregnancy, but it is generally felt that the addition of a cytotoxic agent results in more effective treatment. A recent randomized controlled trial suggests that, in nonpregnant patients, azathioprine is as effective as the gold-standard cyclophosphamide in the remission phase of WG.[12],[13]

  Churg–Strauss Syndrome Top

CSS is rare, and despite this being a disease that affects women of childbearing age, there have been very few cases of CSS in pregnancy published in the medical literature.[14],[15] The main symptoms of disease relapse include mononeuritis multiplex, rash, and worsened asthma control. A predictor of mortality in CSS is congestive cardiac failure from an eosinophilic myocarditis, and clearly, patients with this complication will be at increased risk of worsening cardiac function during pregnancy.

If the disease remains in remission, there does not appear to be a significant effect on pregnancy outcome; women with controlled CSS tend to deliver lower birth weight, but healthy, babies. If the disease is uncontrolled, or if a woman is diagnosed with CSS during pregnancy, higher rates of maternal and fetal death are observed.

Treatment options in Churg–Strauss syndrome

Corticosteroids are the drug of choice in treating relapses or new-onset disease in pregnancy. There is no consensus on which cytotoxic agent to add as second-line therapy, but the least teratogenic option (i.e., azathioprine) should probably be trialed first.

  Polyarteritis Nodosa Top

PAN is a rare disease that tends to present in older patients, and thus, there have been only a very few published reports of pregnancy in women with PAN,[16],[17] from which the following observations are drawn. Of the small number of patients reported, it appears that pregnancy does not have an adverse effect on PAN, provided that the disease is in remission at conception. However, patients diagnosed with PAN late in pregnancy appear to perform badly, with higher rates of maternal death from renal failure, gastrointestinal hemorrhage, respiratory failure, and coma.

Treatment of polyarteritis nodosa in pregnancy

Life-threatening disease calls for the addition of cytotoxic therapy to steroid monotherapy, although there is no consensus regarding which cytotoxic agent should be the first-line treatment. Owing to the generally poor outcomes of women who develop PAN during pregnancy, therapeutic abortion should be considered in early pregnancy, and aggressive, potentially teratogenic therapy should be considered in late pregnancy in order to protect the mother who has a real risk of death.[18]

  Microscopic Polyangiitis Top

Very little is known regarding MPA in pregnancy, and there have been only a handful of case reports published, with the potential for reporting bias skewing toward adverse outcomes.[19] This lack of publications may in part be owing to the fact that, before reclassification of the vasculitis in the 1990s, many patients with MPA would have been classed as having PAN, and more recently, MPA has been classed by many alongside WG in the antineutrophil cytoplasmic antibody-associated vasculitis class.

Treatment options in microscopic polyangiitis

Therapeutic options are much the same as for WG.

  Conclusion Top

A definition of a risk threshold, above which a pregnancy should be strongly discouraged, does not exist at the moment for vasculitis and kidney involvement in pregnancy: Evidence-based medicine (EBM) and ethics bear upon clinical choices. EBM is usually considered helpful in managing those common diseases for which well-coded pathways may exist. However, the EBM approach is of interest also in the case of rare diseases, to quantify experience worldwide and to provide the grounds for an informed decision, while also highlighting the gray areas of existing knowledge. Decisions on pregnancy call for both clinical judgment (definition of risks) and an ethical approach (definition of the limits of a patient's right of self-determination). A well-coordinated, strict clinical surveillance founded on the close cooperation of the different specialists involved is indispensable in the management of high-risk pregnancies. Systemic vasculitis is rare, and the published data on vasculitis in pregnancy are limited to case reports and case series. With this small and imperfect database, it is impossible to compile fully comprehensive guidelines on the management of these patients. A physician–patient interaction of therapeutic alliance based on knowledge and trust may help define the limits of reasonable choice and a course of action in each specific case.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Seo P. Pregnancy and vasculitis. Rheum Dis Clin N Am 2007;33:299-317.  Back to cited text no. 1
Doria A, Iaccarino L, Ghirardello A, Briani C, Zampieri S, Tarricone E, et al. Pregnancy in rare autoimmune rheumatic diseases: UCTD, MCTD, myositis, systemic vasculitis and Behcet disease. Lupus 2004;13:690-5.  Back to cited text no. 2
Langford CA, Kerr GS. Pregnancy in vasculitis. Curr Opin Rheumatol 2002;14:36-41.  Back to cited text no. 3
Sharma BK, Jain S, Vasishta K. Outcome of pregnancy in Takayasu arteritis. Int J Cardiol 2000;75:S159-62.  Back to cited text no. 4
Matsumura A, Moriwaki R, Numano F. Pregnancy in Takayasu arteritis from the view of internal medicine. Heart Vessels Suppl 1992;7:120-4.  Back to cited text no. 5
Aso T, Abe S, Yaguchi T. Clinical gynaecologic features of pregnancy in Takayasu arteritis. Heart Vessels Suppl 1992;7:125-32.  Back to cited text no. 6
Andrews J, Al-Nahhas A, Pennell DJ, Hossain MS, Davies KA, Haskard DO, et al. Non-invasive imaging in the diagnosis and management of Takayasu's arteritis. Ann Rheum Dis 2004;63:995-1000.  Back to cited text no. 7
Andrews J, Mason JC. Takayasu's arteritis Recent advances in imaging offer promise. Rheumatology 2007;46:6-15.  Back to cited text no. 8
Harber MA, Tso A, Taheri S, Tuck SM, Burns A. Wegener's granulomatosis in pregnancy – The therapeutic dilemma. Nephrol Dial Transplant 1999;14:1789-91.  Back to cited text no. 9
Sahni V, Agarwal SK, Singh NP, Sikdar S, Yadav A, Wadhwa A, et al. Successful pregnancy in untreated limited Wegener's granulomatosis. Med J Malaysia 2005;60:492-4.  Back to cited text no. 10
Woywodt A, de Groot K, Bahte S, Schwarz A, Haller H, Haubitz M. Severe relapse of Wegener's granulomatosis during the early postpartum period. Ann Rheum Dis 2006;65:137.  Back to cited text no. 11
Benenson E, Fries JW, Heilig B, Pollok M, Rubbert A. High-dose azathioprine pulse therapy as a new treatment option in patients with active Wegener's granulomatosis and lupus nephritis refractory or intolerant to cyclophosphamide. Clin Rheumatol 2005;24:251-7.  Back to cited text no. 12
Fields CL, Ossorio MA, Roy TM, Bunke CM. Wegener's granulomatosis complicated by pregnancy. A case report. J Reprod Med 1991;6:139-40.  Back to cited text no. 13
Hiyama J, Shiota Y, Marukawa M, Horita N, Kanehisa Y, Ono T, et al. Churg-Strauss syndrome associated with pregnancy. Intern Med 2000;39:985-90.  Back to cited text no. 14
Ito Y, Endo Y, Kojima A, Sato M, Horinouchi T, Kato M. A case of Churg-Strauss syndrome undergoing cesarean section. Masui 2004;53:420-1.  Back to cited text no. 15
Foster R, D'Cruz DP. Vasculitis in pregnancy. Expert Rev of Obstet Gynecol 2007;2:681-8.  Back to cited text no. 16
Gatto M, Iaccarino L, Canova M, Zen M, Nalotto L, Ramonda R, et al. Pregnancy and vasculitis: A systematic review of the literature. Autoimmun Rev 2012;11:A447-59.  Back to cited text no. 17
Howard T, Ahmad K, Swanson JA, Misra S. Polyarteritis nodosa. Tech Vasc Interv Radiol 2014;17:247-51.  Back to cited text no. 18
Milne KL, Stanley KP, Temple RC, Barker TH, Ross CN. Microscopic polyangiitis: First report of a case with onset during pregnancy. Nephrol Dial Transplant 2004;19:234-7.  Back to cited text no. 19


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