Pregnancy-related issues from the perspective of patients with inflammatory rheumatic diseases - Results from a survey of the members of the National Association for Inflammatory Rheumatic Diseases.

OBJECTIVES: This study aimed to clarify the issues related to pregnancy in patients with inflammatory rheumatic diseases (RDs) and to provide useful information for developing medical services from patients' perspectives. METHODS: A survey involving approximately 5,000 members of the Patients Association for Collagen Vascular Diseases Japan was conducted using a questionnaire that was sent and returned by mail. The questionnaire items included age at the time of the survey, types of RDs, association of RDs with pregnancy/childbirth outcomes, and pregnancy-related supports and hindrances. RESULTS: We received 491 completed questionnaires. The most common RD was systemic lupus erythematosus (n=309). Approximately 60% of participants had a history of childbirth. Approximately 60% of participants had previously experienced pregnancy-related challenges due to RDs. These included concerns about the influence of drugs on babies, genetic transmission, and active disease. Patients with active disease at the time of conception were more likely to experience disease exacerbation during pregnancy, but this did not correlate with whether the pregnancy was planned. CONCLUSION: This study revealed that many patients with RDs experienced pregnancy-related challenges and needed appropriate support based on appropriate information. The findings here should help rheumatologists, health care providers, and public agencies provide counseling and information.

A woman with systemic lupus erythematosus, multiple pregnancy complications, and cerebral infarction who was only positive for phosphatidylserine-dependent antiprothrombin antibodies.

A woman with systemic lupus erythematosus (SLE) had a history of two abortions before the 10th week, two foetal deaths with normal morphology, and one premature before the 34th week with early-onset hypertensive disorder of pregnancy (HDP) and placental dysfunction. Although she did not have any conventional antiphospholipid antibodies (aPLs), antiphospholipid syndrome (APS) was strongly suspected based on her obstetric history and renal biopsy findings consistent with aPL-associated nephropathy (APLN). Eventually, she was found to be positive for phosphatidylserine-dependent antiprothrombin antibodies (aPS/PTs). A healthy baby was born with anticoagulation and intravenous immunoglobulin (IVIG) therapy during pregnancy. aPS/PT titres gradually increased after delivery. Cerebral infarction occurred at 9 years after birth. If APS is clinically suspected but the antibodies included in the classification criteria for APS are all negative, we should consider an association with unconventional aPLs and manage according to APS.

A case of successful pregnancy following multidrug treatment including rituximab and intravenous immunoglobulin for primary antiphospholipid antibody syndrome refractory to conventional treatment.

Antiphospholipid antibody syndrome (APS) is defined by the presence of clinical symptoms caused by antiphospholipid antibodies. When APS occurs during pregnancy, it is conventionally treated with low-dose aspirin or heparin. In cases refractory to conventional treatment, intravenous immunoglobulin (IvIg) is sometimes added. We present the case of an APS patient with severe thrombocytopenia who experienced a successful pregnancy after treatment that included intravenous rituximab and IvIg. As far as we know, this is the first report demonstrating a positive pregnancy outcome in this context. Physicians may consider prescribing not only IvIg but also rituximab during the first trimester of pregnancy in APS patients with severe obstetrical complications and thrombocytopenia refractory to conventional treatment.

Clinical feature and anti-phospholipid antibody profiles of pregnancy failure in young women with antiphospholipid antibody syndrome treated with conventional therapy.

OBJECTIVE: To elucidate clinical feature and anti-phospholipid antibody (aPL) profiles, including lupus anticoagulant (LA), anti-cardiolipin (CL) antibodies and anti-phosphatidylserine/prothrombin (PS/PT) antibodies, of pregnancy failure in patients with antiphospholipid antibody syndrome (APS) already treated with conventional therapy. MATERIALS AND METHODS: Thirty-four women with a history of pregnancy who were diagnosed with APS between 2008 and 2016 were included in the study. We defined the successful pregnancy group as women who gave birth to a healthy baby over 1500 g after 34 weeks of pregnancy under conventional treatment (heparin and/or low-dose aspirin). The unsuccessful pregnancy group was defined as women whose pregnancy outcomes did not meet the aforementioned criteria despite the conventional therapy. The clinical features and aPL profiles were compared between the two groups. RESULTS: Fifteen women were classified into the unsuccessful pregnancy group; seven women were in the successful pregnancy group. Having history of both thrombosis and pregnancy morbidity and LA positivity were significantly more prevalent in the unsuccessful pregnancy group than in the successful pregnancy group (p <.05, respectively). In contrast, single positivity of anti-CL antibody was negatively associated with APS-associated pregnancy morbidity under the conventional treatment (p <.01). The proportion of anti-PS/PT IgG-positive patients was significantly higher in the unsuccessful pregnancy group (p = .02, OR 18.7, 95% CI 1.50, 232.29) with high concordance rate with LA (97% consistence). CONCLUSION: History of both thrombosis and pregnancy morbidity and the positivity of LA and/or anti-PS/PT-IgG, not but anti-CL-antibodies were correlated with APS-associated pregnancy morbidity refractory to conventional treatment. Clinical feature and aPL profiles might help us to make risk assessment for adverse pregnancy outcomes in patients with APS.