Case 1: 23 year-old-female seeking contraception

Lisa Sammaritano

A 23-year-old woman with systemic lupus erythematosus (SLE) who recently relocated wishes to establish rheumatology care. She was diagnosed with SLE aged 14 years with idiopathic thrombocytopenic purpura; she was treated with high dose steroid with good response and tapered off steroid within 6 months. She was well until age 20 when she presented with malar rash, inflammatory arthritis of PIPs, MCPs and knees, fatigue, and petechia following a vacation in the Caribbean. She was diagnosed with SLE with positive ANA and double stranded DNA. Her hemoglobin was 6.0 gm/dl with positive Coombs and low haptoglobin, her platelet count was 12,000. C3 and C4 were low. She was treated with intravenous ‘pulse’ methylprednisolone followed by high dose prednisone, azathioprine and hydroxychloroquine with good response.

She currently feels well without complaints other than mild morning stiffness in her hands and occasional malar rash. Current medications are azathioprine 125 mg daily, hydroxychloroquine 300 mg daily, and prednisone 5 mg daily. Physical exam at the visit is unremarkable and routine labs including urinalysis are normal. She has been using barrier contraception (condoms) but asks about other options. She is very worried that any hormone therapy will cause her lupus to flare again. How do you assess her, and what do you recommend?

a. Serologies show negative anti-dsDNA, normal C3C4, and negative antiphospholipid antibodies (aPL) including lupus anticoagulant (LAC), anticardiolipin (aCL) and anti-beta 2 Glycoprotein I (ab2GPI).

b. Serologies show negative anti-dsDNA, normal C3C4, and positive LAC, positive aCL IgG 68 and positive ab2GPI IgG 45

Discussion Point

• The best way to assess patients with SLE for safe and effective contraception, in the setting of negative and positive aPL

Discussion Point Case 2: 33-year-old seeking assisted reproductive technology

A 33-year-old woman with SLE presents for routine follow-up. You have followed her since her diagnosis at age 27 years when she presented with oral ulcers, photosensitive rash, arthritis, pleuritis, positive ANA and positive dsDNA antibody. She started hydroxychloroquine with control of her symptoms after a brief steroid taper. At age 31 she developed nephritis, Class IV and V on renal biopsy. She was treated with intravenous ‘pulse’ methylprednisolone, rituximab (two doses of 1 gm each) and mycophenolate 2000 mg daily. She continues on the mycophenolate 2000 mg daily, enalapril 20 mg daily and prednisone 5 mg daily. Her serum creatinine has been 1.2 mg/dl, and her dsDNA antibody is low positive with mildly low C3 and normal C4 (markedly improved from her initial onset of nephritis). The urine protein/creatinine ratio is 1100 mg/gm, down from 3700 mg at onset of nephritis and stable over the last year. She is not in a relationship now but wants to preserve her ability to have biological children in the future and she is very concerned that she will develop recurrent nephritis. She has decided she would like to freeze her eggs and asks your opinion.

a. aPL (LAC, aCL, ab2GPI) are all negative

b. aPL are ‘triple positive’, with high levels of LAC, aCL and ab2GPI

Discussion Point

• The best way to assess and guide a patient with SLE who is planning oocyte cryopreservation, in the setting of negative and positive aPL

Learning Objectives

• Explain the importance of safe and effective contraception for women with SLE at risk for unintended pregnancy and be able to assess patients and recommend the best options for them

• Describe the risks of ovarian stimulation for patients with SLE, with and without aPL, and suggest and discuss specific management options with the reproductive endocrinologist

Learning Objectives Case 3: 36-year-old female seeking pregnancy counselling

Rebecca Fischer-Betz

Maggy is a 36-year-old female office assistant with 8-year history of systemic lupus erythematosus (SLE). Past manifestations of SLE included polyarthritis, pleuritis, positive ANA and dsDNA antibodies and low complement. She had been initially treated with corticosteroids and hydroxychloroquine. Two years ago she had a flare with polyarthritis and treatment with methotrexate was added. Currently she is feeling well. She asks about the possibility of a pregnancy. Her menstruation is regular and has had no previous pregnancies.

Laboratory tests revealed Hb 12,8 g/dl; Plt 233 K/µl; leucocytes 3.800/µl; Anti-dsDNA 122 (< 80 IU/ml), complement is normal. She is presently taking methotrexate 12.5 mg sc/week and hydroxychloroquine 200 mg/day.

Discussion Points

• Performing pregnancy counselling in women with SLE

• Treatment options available prior to conception and during pregnancy

Discussion Points Case 4: 29-year-old female with lupus nephritis

Rebecca Fischer-Betz

Sara is a 29-year old female from Sri Lanka living with her husband in Germany for five years. She was diagnosed with SLE about one year ago based on the presence of fever, fatigue, leukopenia, positive antinuclear antibodies, positive anti-dsDNA and low complement. In addition, she had proteinuria (>5 g/24 hours) and abnormalities of urinary sediment. A renal biopsy showed Class IV proliferative lupus nephritis. She was initially treated with prednisolone and cyclophosphamide (Euro Lupus protocol) followed by mycophenolate mofetil. She had an early abortion 3 years ago (unplanned pregnancy). Her menstruation cycle has been irregular since she stopped taking an estrogen-containing pill after diagnosis. She worries about infertility. She currently reports joint pain and fatigue, she sleeps a lot. Her physical examination is unremarkable, no arthritis, no edema, blood pressure 125/85 mmHg. Laboratory tests reveal Hb 10,8 (12–16 g/dl) g/dl, creatinine 1,1 (<0,9) mg/dl, urinalysis 40 RBC, Pr/Cr 2,1 g/g, anti-dsDNA 433 (<80 IU/ml), complement C3 66 (90–180 mg/dl), C4 <6 (10–40 mg/dl). Antiphospholipid-antibodies are negative. She is presently taking prednisone 10 mg, mycophenolate mofetil 2 g/day, ramipril 5 mg/day and furosemide 20 mg. Her older brother is also suffering from lupus nephritis. He has stopped immunosuppressive treatment several months ago and is doing well.

Discussion Point

• When and how to plan pregnancy in a patient with lupus nephritis

Learning Objectives

• Demonstrate knowledge of the influence of SLE on pregnancy and vice versa

• Describe main predictors of pregnancy complications in women with SLE

• Describe currently accepted management of SLE prior to conception and during pregnancy

• Explain the importance of pregnancy counselling in SLE patients