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Background/Purpose APS ACTION Registry was created to study the outcomes of patients with persistently positive antiphospholipid antibodies (aPL) with or without other systemic autoimmune disease (SAIDx). Given that immunosuppression (IS) is used for certain aPL manifestations, for example, thrombocytopenia (TP), our primary objective was to describe the indications for IS in aPL-positive patients without other SAIDx. Secondly, we report the type of IS used in patients with selected microvascular or non-thrombotic aPL manifestations. Methods An online database is used to collect clinical data. The inclusion criteria are positive aPL based on the laboratory section of the APS Classification Criteria, tested at least twice within one year prior to enrollment. Patients are followed every 12 ± 3 months. For this descriptive retrospective and prospective analysis, we included aPL-positive patients without other SAIDx and excluded those with new SAIDx classification during follow-up. For each patient, we retrieved clinical data at baseline and follow-up including selected aPL manifestations (diffuse alveolar hemorrhage [DAH], antiphospholipid-nephropathy [aPL-N], livedoid vasculopathy [LV]-related skin ulcers, TP, autoimmune hemolytic anemia [AIHA], cardiac valve disease [VD]), and IS medications. Results Of 899 patients enrolled, 537 were included in this analysis (mean age 45 ± 13 years, female 377 [70%], APS Classification in 438 [82%], and at least one selected microvascular or non-thrombotic aPL manifestation in 141 (26%)). Of 537 patients, 76 (14%) were reported to use IS (ever), and 41/76 (54%) received IS primarily for selected aPL manifestation. In six of 8 (75%) DAH patients, 6/19 (32%) aPL-N, 4/28 (14%) LV, 25/88 (28%) TP, 6/11 (55%) AIHA, and 1/43 (2%) VD, the IS (excluding corticosteroids/hydroxychloroquine) indication was specific for selected aPL manifestation. Conclusion In our international cohort, 14% of aPL-positive patients without other SAIDx were reported to receive IS; the indication was at least one of the selected microvascular and/or non-thrombotic aPL-related manifestations in half. Thrombocytopenia was the most frequent among those selected aPL-related manifestations; however, approximately one-third received IS specifically for that indication. Diffuse alveolar hemorrhage was frequently treated with IS followed by AIHA and aPL-N. Systematic controlled studies are urgently needed to better define the role of IS in APS.

ObjectiveTo map existing organizational care pathways in clinical centers of expertise that care for pregnant women affected by rare and complex connective tissue diseases (rcCTDs).MethodsAn international working group composed of experts in the field of pregnancy in rcCTDs co-designed a survey focused on organizational aspects related to the patient's pathway before, during, and after pregnancy. The survey was distributed to subject experts through referral sampling.ResultsAnswers were collected from 69 centers in 21 countries. Patients with systemic lupus erythematosus and/or antiphospholipid syndrome were followed by more than 90% of centers, whereas those with disorders such as IgG4-related diseases were rarely covered. In the majority of centers, a multidisciplinary team was involved, including an obstetrician/gynecologist in 91.3% of cases and other healthcare professionals less frequently. Respondents indicated that 96% of the centers provided routine pre-pregnancy care, whereas the number of patient visits during pregnancy varied across centers. A formalized care pathway was described in 49.2% of centers, and 20.3% of centers had a predefined protocol for the monitoring of pregnant patients. Access to therapies during pregnancy also was heterogeneous among different centers.ConclusionIn international referral centers, a high level of care is provided to patients with rcCTDs before, during, and after pregnancy. No significant discrepancies were found between European and non-European countries. However, this work highlights a potential benefit to streamlining the care approaches across countries to optimize pregnancy and perinatal outcomes among patients with rcCTDs.

To analyse maternal variables associated with occurrence of small for gestational age (SGA) newborns in pregnancies of women with systemic lupus erythematosus (SLE), considering clinical and laboratory characteristics prior to conception, during gestation and comorbidities.Retrospective cohort study with SLE pregnant patients and singleton deliveries after 22 weeks. SGA newborn was defined as birth weight below 10th percentile and SLE activity at conception and during gestation was measured using the SLE Pregnancy Disease Activity Index (SLEPDAI). Univariate analysis was employed to evaluate individual influence of demographic and clinical variables on the SGA newborn outcome, while variables with p0.20 were included in multivariate regression.Among 151 pregnancies, 28 (18.5%) had SGA newborns. History of proliferative nephritis (RR=3.84, CI 1.63-9.3) and positivity for anti-RNP and anti-Sm antibodies (RR=2.67, CI 1.11-6.43; 2.78, CI 1.44-5.32) were more frequent in the study group. Active proliferative nephritis at conception (RR=3.29, CI 1.75-6.18) and during gestation (RR=3.63, CI 1.97-6.71), as well as complement C3 consumption (RR=2.70, CI 1.09-6.67) and venous pulse therapy with methylprednisolone (RR=20.3, CI 2.18-190), were also associated with SGA newborns, the latter being independently associated in multivariate regression. Adverse perinatal outcomes, such as stillbirths (4.3 times) and neonatal intensive care unit admissions (3.2 times), were more frequent among SGA infants.Active proliferative lupus nephritis during pregnancy was associated with SGA newborns, while its treatment with venous pulse therapy with methylprednisolone may play a significant role in this context. Presence of previous proliferative nephritis, SLEPDAI ≥4, C3 consumption and presence of anti-RNP and anti-Sm antibodies were additional variables associated with SGA newborns in this population.