COMMENTARY

Pregnant With a Rare Vasculitis: How to Treat

Stephen Paget, MD

Disclosures

September 13, 2017

Editorial Collaboration

Medscape &

Clinical Profile

At her first visit to the Hospital for Special Surgery Inflammatory Arthritis Center, a 37-year-old African-American woman presented for evaluation and treatment of Takayasu arteritis (TAK).

TAK had been present since she was 12 years of age, when the patient developed fatigue, fever, weight loss, and poor appetite and was admitted to Methodist Hospital in Brooklyn for 6 weeks without a specific diagnosis. At 13 years of age, she was seen by a rheumatologist who diagnosed TAK and treated her with prednisone, initially at 50 mg/day and eventually at 20 mg/day throughout her 20s, with complete resolution of all of her symptoms. Most of the prednisone decisions were based on the erythrocyte sedimentation rate and not the reappearance of TAK-type symptoms. Her average dose over the years was 10 mg/day.

At 33 years of age, the patient was found to have an expanding aortic thoracic aneurysm, which was repaired with good results. She was never placed on anticoagulation.

Five years later, when the patient was down to 8 mg/day of prednisone, she developed a typical flare with fatigue, throbbing headache, and a markedly elevated erythrocyte sedimentation rate that responded to 70 mg of prednisone, with complete control of symptoms; the dosage was tapered down to 5 mg/day. Throughout the course of her illness, the patient was offered various immunosuppressive disease-modifying drugs, such as azathioprine and methotrexate, that could help to spare steroids, but she refused.

At 40 years of age, the patient returned, noting that she had recently been pregnant but that the pregnancy was terminated at 23 weeks because of profound intrauterine growth restriction; genetic studies on the fetus were negative. The placenta demonstrated ischemic changes. At the time, she had none of her characteristic signs or symptoms of active vasculitis. The patient could not obtain MRI or magnetic resonance angiography (MRA) while pregnant because her insurance company refused to support it, owing to fear of fetal damage.

At 6 weeks into a new pregnancy, the patient returned for guidance. She remained on prednisone 5 mg/day with no signs/symptoms of active vasculitis, including no joint pain, fatigue, weight loss, neurologic problems, hypertension, or vascular insufficiency. She had a normal complete blood cell count, erythrocyte sedimentation rate, and C-reactive protein level.

Physical examination revealed an anterior chest scar from prior surgery, and she had a 2/6 systolic ejection murmur at the second right intercostal space with radiation into the right neck area. The patient had bruits in the right and left side of the neck and in the supraclavicular areas bilaterally. Pulses were normal in the right arm and slightly decreased in the left arm, but there were no ischemic changes in the upper and lower extremities.

I spoke to the patient's high-risk obstetrician. We agreed that her pregnancy loss was probably related to vascular compromise due to TAK, we would probably get no further information about the vascular supply to the uterus using MRI/MRA or ultrasound, and she should be treated empirically with 40 mg/day of prednisone. The patient maintained this prednisone dosage throughout her recent pregnancy, and at 40 weeks gave birth to a healthy daughter.

Formulation

What did we learn from this case?

  1. Large-vessel vasculitides, such as TAK, can affect multiple vascular beds, including those surrounding the uterus. During the first pregnancy, although the patient had none of her characteristic symptoms of active TAK, her pregnancy loss was probably due to placental ischemia related to active visceral vasculitis. This is supported by her full-term pregnancy while treated with steroids.

  2. With regard to vascular imaging during pregnancy, gadolinium is not recommended, but it is safe to perform MRI. However, without the use of gadolinium, MRI would probably not add much clinically important information.

  3. Cooperation between the rheumatologist and the obstetrician was vitally important in this situation in order to bring about a full-term pregnancy and healthy baby.

  4. Needless to say, having the patient involved in all decisions was mandatory and allowed her to appreciate the costs and benefits related to long-term steroids.

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