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Objectives: To determine whether disease remission or low disease activity state at the beginning of pregnancy in SLE patients is associated with better pregnancy outcome., Methods: Pregnancies in SLE patients prospectively monitored by pregnancy clinics at four rheumatology centres were enrolled. Patient demographics and clinical information were collected at baseline (pregnancy visit before 8 weeks of gestation) including whether patients were in remission according to the Definition of Remission in SLE (DORIS) criteria and and/or Lupus Low Disease Activity State (LLDAS). Univariate and multivariate analysis were performed to determine predictors of disease flare and adverse pregnancy outcomes (APOs) including preeclampsia, preterm delivery, small for gestational age infant, intrauterine growth restriction and intrauterine fetal death., Results: A total of 347 pregnancies were observed in 281 SLE patients. Excluding early pregnancy losses, 212 pregnancies (69.7%) occurred in patients who were in remission at baseline, 33 (10.9%) in patients in LLDAS, and the remainder in active patients. Seventy-three flares (24%) were observed during pregnancy or puerperium, and 105 (34.5%) APOs occurred. Multivariate analysis revealed that patients in disease remission or taking HCQ were less likely to have disease flare, while a history of LN increased the risk. The risk of APOs was increased in patients with shorter disease duration, while being on HCQ resulted a protective variable. An almost significant association between complete remission and a decreased risk of APOs was observed., Conclusions: Prenatal planning with a firm treat-to-target goal of disease remission is an important strategy to reduce the risk of disease flares and severe obstetric complications in SLE pregnancies., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objectives: To investigate the courses and outcomes of pregnancies involving JIA patients who were exposed to DMARDs., Methods: In the Juvenile arthritis MTX/Biologics long-term Observation study, pregnant patients or male patients with pregnant partners were identified. Standardized patient interviews were conducted, and the course and outcome of pregnancy were assessed. Prospectively collected physician- and patient-reported data were also considered in the analysis., Results: The study sample included 152 pregnancies in 98 women with JIA and 39 pregnancies involving 21 male patients as partners. The majority of patients had polyarticular-onset/-course JIA (61%). The average age of patients at first pregnancy was 24.1 (4.5) years, and their mean disease duration was 13.8 (5.9) years. Patients had been exposed to DMARDs for 9.5 (5.6) years, and 90% of these patients had received biologics before. Half of the pregnancies occurred during DMARD exposure, mostly with etanercept. Significant differences in pregnancy outcomes between DMARD-exposed and -unexposed pregnancies were not observed. Spontaneous abortion (13.1%) and congenital anomaly (3.6%) rates were not suggestive of increased risk compared with expected background rates. However, the rates of premature birth (12.3%) and caesarean section (37.7%) were slightly above those in the German birthing population. The disease activity of female patients remained relatively stable in pregnancy, with mean cJADAS-10 scores of 5.3, 7.1 and 5.6 in each trimester, respectively., Conclusion: Young adults with JIA often become pregnant or become fathers of children while still being treated with DMARDs. Data suggest no increased risk of major adverse pregnancy outcomes., (© The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objectives: Vaccinations are an important measure to prevent infections in immunocompromised patients. The knowledge of vaccination coverage and reasons for non-vaccination in patients with SLE is scarce. The aim of this study was to assess coverage rates of selected vaccinations in a representative sample of SLE patients and to identify predictors for non-vaccination., Methods: In 2013, information on selected vaccinations (coverage, application and reservations) and on demographics, clinical parameters and health beliefs was assessed by means of a self-reported questionnaire among a representative sample of SLE patients in Germany (LuLa cohort)., Results: Five hundred and seventy-nine patients participated. Vaccination status was primarily checked by their general practitioner (57.3%). Of all the patients, 24.9% did not get their vaccination status checked at all, 16.1% had generally been advised against the use of vaccinations by a physician, and 37.5% stated that they had rejected vaccinations themselves. Their main reasons were fears of developing a lupus flare (21.8%) or adverse events (13.5%). A greater belief by patients in the doctor controlling one's health and the general benefit of medication prevented the rejection of vaccines. Vaccination coverage was low for all recorded vaccinations (tetanus 65.8%, influenza 45.2%, pneumococcus 32.2% and meningococcus 6.1%). Older age was predictive of receiving influenza and pneumococcal vaccination. The same applies for CSs >7.5 mg for receiving influenza vaccination., Conclusion: Vaccination coverage in SLE patients is poor and reflects insufficient implementation of national and international recommendations. Rheumatologists need to recognize patients' reservations against vaccinations, to communicate their importance and safety and to give individual recommendations to patients and their health-care providers., Trial Registration: German Clinical Trials Register, www.germanctr.de, DRKS00011052.

Objective: Diagnosis of SLE relies on the detection of autoantibodies. We aimed to assess the diagnostic potential of histone H4 and H2A variant antibodies in SLE., Methods: IgG-autoantibodies to histones H4 (HIST1H4A), H2A type 2-A (HIST2H2AA3) and H2A type 2-C (HIST2H2AC) were measured along with a standard antibody (SA) set including SSA, SSB, Sm, U1-RNP and RPLP2 in a multiplex magnetic microsphere-based assay in 153 SLE patients [85% female, 41 (13.5) years] and 81 healthy controls [77% female, 43.3 (12.4) years]. Receiver operating characteristic analysis was performed to assess diagnostic performance of individual markers. Logistic regression analysis was performed on a random split of samples to determine the additional value of histone antibodies in comparison with SA by likelihood ratio test and determination of diagnostic accuracy in the remaining validation samples., Results: Microsphere-based assay showed good interclass correlation (mean 0.85, range 0.73-0.99) and diagnostic performance in receiver operating characteristic analysis (area under the curve (AUC) range 84.8-93.2) compared with routine assay for SA parameters. HIST1H4A-IgG was the marker with the best individual diagnostic performance for SLE vs healthy (AUC 0.97, sensitivity 95% at 90% specificity). HIST1H4A-IgG was an independent significant predictor for the diagnosis of SLE in multivariate modelling (P < 0.0001), and significantly improved prediction of SLE over SA parameters alone (residual deviance 45.9 vs 97.1, P = 4.3 × 10-11). Diagnostic accuracy in the training and validation samples was 89 and 86% for SA, and 95 and 89% with the addition of HIST1H4A-IgG., Conclusion: HIST1H4A-IgG antibodies improve diagnostic accuracy for SLE vs healthy., (© The Author(s) 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. [br]For Permissions, please email: journals.permissions@oup.com)

Objective: MMF is teratogenic and needs to be replaced before pregnancy. This change may lead to flares. Our aim was to determine the risk of renal flares in women with LN who switched treatment from MMF to AZA before conception and to evaluate the outcome of their pregnancies., Methods: Medical records of women with LN counselled for pregnancy wish were reviewed. Women receiving treatment with either MMF or AZA (control group), with inactive lupus (SLEDAI ≤ 4) and quiescent LN (serum creatinine <1.5 mg/dl, inactive sediment and proteinuria <1 g/24 h for the preceding 6 months) were eligible for this study., Results: We identified 54 women [23 treated with MMF (group 1) and 31 treated with AZA (group 2)]. MMF dosage was tapered and subsequently transferred to AZA, which was maintained throughout pregnancy. Three (13%) patients (group 1) vs none (group 2) developed a renal flare 3-6 months after transitioning from MMF to AZA (P = 0.14) before pregnancy ensued. The only parameter with a significant difference in those with flare compared with those without was younger age (median 27 vs 30 years; P = 0.03). Risk for adverse outcome within 48 pregnancies (pre-eclampsia 9%, preterm delivery 20.5%) increased with every milligramme of prednisone dosage [odds ratio (OR) 2.03] and every single unit of SLEDAI score (OR 3.92). Renal flares occurred post-partum in two women. No patient developed worsening of renal function., Conclusion: Replacing MMF with AZA in patients with quiescent LN for pregnancy planning rarely leads to renal flares. Pregnancy outcome was favourable.

Systemic connective tissue diseases (CTDs) are disease entities characterized by a systemic and heterogeneous spectrum of clinical symptoms. The treatment of CDTs has improved substantially, but with the developments of new and probably more expensive targeted therapies, there will be a need of rigorous evaluation in patient-oriented research. Proposed domains for outcome measurement of CTDs are activity, damage by disease and/or medications and quality of life. To evaluate the overall disease activity in CTDs, scores are developed that include typical signs and symptoms. For example, in systemic lupus erythematosus (SLE), various activity instruments have been developed and validated. Recently, a definition for significant reduction in activity based on these scores was proposed and has now to be validated in clinical trials. In addition, the American College of Rheumatology has framed recommendations for response criteria of major target organs. Besides, the rarity of CTDs other than SLE and the rare therapeutic options, especially the heterogeneous disease expression, has prevented development of instruments scoring the disease activity. In individual patients, there is need for a more organ-specific evaluation, and non-disease-specific and/or global health instruments may be used in evaluating the response of individual treatment regimen.