Case 1: A pregnant woman with SLE and APS

JB is a Caucasian female (born in 1978), smoker; she consulted us in 2003 (25 years old). She complained frequent episodes of lipothymia and dizziness, reported arterial hypertension diagnosed 3 years before that was successfully treated. In the last year, she had been taking oral contraceptive pill. The physical examination was normal, but livedo reticularis was noted. Her blood tests showed: thrombocytopenia 75,500/mm³, prolongation of aPTT, hypergammaglobulinemia (25%); positive ANA, anti Ro/SS-A, low titer anti DNA; mild reduction C3 and C4. She tested positive for lupus anticoagulant, anticardiolipin and anti-beta2-GPI. Renal arteries ultrasound was normal; echocardiogram showed mild mitral insufficiency with thickened leaflets, and ENT consultation was without pathological findings. At brain MRI, multiple hyper-intense foci in white matter compatible with ischemic lesions were present in T2.

Her diagnosis was probable antiphospholipid syndrome (APS) with lupus-like disease. She was managed with sun protection 50+, she stopped smoking and oral contraceptive, and started low-dose aspirin (LDA), whilst continuing anti-hypertensive therapy.

She returned to clinic in 2005, 12 gestational weeks pregnant, she was already on LDA and folic acid. The treatment was adjusted with the addition of prednisone 5 mg/day and enoxaparin at prophylactic dose. Unfortunately, intrauterine death occurred at 14 gestational weeks; placenta histology showed multiple infarctions. The diagnosis of APS with lupus-like disease was made. She was discharged with hydroxychloroquine (HCQ) 200 mg/day, LDA and prednisone 5 mg/day.

One year later, the patient returned to the clinic 6 gestational weeks pregnant. Low molecular weight heparin at prophylactic dose was added to the treatment and prednisone was increased at 10 mg/day.

In December 2005 at 30 gestational weeks, urgent caesarean section was performed for pre-eclampsia (proteinuria 5310 mg/24 h) evolved in HELLP syndrome.

In the following years she was treated with HCQ, low dose prednisone (5 mg/day), LDA, anti-hypertensive therapy and supplementation with vitamin D, folic acid and iron. Proteinuria progressively decreased (<500 mg/24 hours) and GFR reduced to 55 ml/min. She was persistently anemic (Hb 10 g) due to metrorrhagia and remained thrombocytopenic (60,000/mm³). The baby was healthy and grew regularly.

In 2013 (aged 35-years-old) she was doing well, and she returned to the pregnancy clinic because she wanted another baby. After a joint consultation with the gynecologist and rheumatologist, she was discouraged to start a new pregnancy because of her history. In 2015 malar rash was observed together with evolution of her serology. The diagnosis of SLE with APS was made and she was started on belimumab.

Case 2: A 31-year-old woman

RI is a Caucasian female, that consulted us in 1995 at 31 years old.

She reported a pregnancy complicated by preeclampsia at 28 gestational weeks, 6 months before; a female baby of 850 g was born and, unfortunately, died after one day. Subsequent investigations revealed the presence of IgG anticardiolipin antibodies at high titre and lupus anticoagulant. At the time of our consultation, she reported frequent vision changes in the days before her monthly periods. She also reported photosensitivity since her 20s. Physical examination was normal with a normal blood pressure. Blood tests revealed low titre ANA and triple antiphospholipid antibody positivity. Treatment with low dose aspirin (LDA) was started.

In 1996 she came to see us at 6 weeks of gestation, she was already on LDA and folic acid. Prednisone 5 mg was added. The pregnancy was carried on without complications. At 38 gestational weeks. Caesarean section was performed, because of PROM, and a female baby was born with a birthweight of 3100 g.

The patient, living in another town, came for a follow-up visit in 1998. She reported some episodes of dizziness and diplopia. Physical examination and blood pressure were normal. The serology was repeated and resulted unchanged. Hydroxychloroquine and LDA were prescribed.

One year later (1999), the patient presented at the beginning of a second pregnancy. The clinical situation was substantially unchanged, we decided to continue HCQ and LDA with the addition of 5 mg prednisone and folic acid. At 17 gestational weeks, HCQ was withdrawn because of an itching diffuse rush. At 35.3 gestational weeks, blood pressure started to rise, and a Caesarean section was performed at 36 gestational weeks. A healthy female baby was born, with a birthweight of 2510 g.

Five days after delivery, the mother developed slurred speech and right hemiplegia suddenly occurred while she was taking unfractionated heparin 0.2 ml twice a day. Brain CT scan revealed and ischemic area in lentiform nucleus, caudate nucleus and left posterior limb of the internal capsule.

Learning Objectives

• Explain the importance of counselling in order to improve the pregnancy outcome

• Describe risk factors for recurrence in patient with obstetric APS, including triple antibody positivity as an important risk factor driving treatment adjustments

• Discuss why it could be necessary discourage a patient to start a new pregnancy

• Describe the possible effective treatments of obstetric APS

• Explain the importance of focusing on the post-partum period and to the possible occurrence of serious complications linked to the presence of antiphospholipid antibodies