Male patients with inflammatory joint diseases are less likely than controls to be childless: results from a Norwegian population-based cohort study of 10 865 patients.

OBJECTIVES: To investigate the number of children per man and the proportion of childless men as a proxy of fertility in a national cohort of men with inflammatory joint diseases (IJDs), compared with matched controls from the general population. METHODS: This is a nationwide, population-based retrospective cohort study. Male patients with IJDs (n = 10 865) in the Norwegian Arthritis Registry were individually matched 1:5 on birth year and county of residence with men without IJDs obtained from the National Population Register (n = 54 325). Birth data were obtained from the Medical Birth Registry of Norway. We compared the mean number of children per man and the proportion of childless men and analysed the impact of age and year of diagnosis. RESULTS: The mean number of children per man in the patient group was 1.80 versus 1.69 in the comparison group (p <0.001), and 21% of the patients in the patient group were childless versus 27% in the comparison group (p <0.001). The finding of less childlessness and higher number of children per man remained consistent across age at diagnosis, except for those diagnosed at age 0-19 years. The difference in childlessness was most pronounced for men diagnosed after year 2000, especially when diagnosed at 30-39 years of age (22% vs 32%, p<0.001). CONCLUSION: In this large cohort study we found that patients with IJD have a higher number of children and are less likely to be childless compared with controls. Factors associated with developing or having an IJD might influence fertility and this requires further investigation.

Low serum lipocalin-2 in pregnant women with systemic lupus erythematosus.

OBJECTIVES: Systemic lupus erythematosus (SLE) pregnancies are considered high-risk due to risk of disease flare and pregnancy complications. A more in-depth understanding of the immunological alterations in SLE patients during pregnancy and identification of predictive biomarkers may help to achieve stable disease and to avoid pregnancy complications. Lipocalin-2 (LCN2) has been implicated as a potential biomarker for rheumatic diseases and preeclampsia, but remains unexplored in SLE pregnancies. METHODS: We measured LCN2 levels in serum samples from SLE pregnancies (n=25) at seven different time points. Samples were taken preconception, in each trimester, at 6 weeks, 6 months and 12 months postpartum. Serum LCN2 levels were compared to samples from rheumatoid arthritis (RA) (n=27) and healthy (n=18) pregnancies at each time point using t-test, and for all time points using a linear mixed effects model. In addition, we investigated the association between LCN2 levels and disease activity, CRP, kidney function, BMI, treatment regimen and adverse pregnancy outcome for SLE and RA patients. RESULTS: We found significantly lower serum LCN2 levels throughout pregnancy in SLE patients with quiescent disease compared to RA and healthy pregnancies. We did not find an association between serum LCN2 and disease activity or adverse pregnancy outcome in SLE pregnancies. CONCLUSIONS: In a population of SLE women with low disease activity we have not found evidence that serum LCN2 levels predict disease activity or adverse pregnancy outcomes. Further studies are needed to elucidate a possible biological role of low LCN2 levels in SLE pregnancies.

Pregnancy and neonatal outcomes in women with axial spondyloarthritis: pooled data analysis from the European Network of Pregnancy Registries in Rheumatology (EuNeP).

OBJECTIVE: To investigate outcome and course of pregnancies in women with axial spondyloarthritis (axSpA) in a pooled data analysis of pregnancy registries in rheumatology. METHODS: Prospectively followed women with axSpA, fulfilling ASAS classification criteria and for whom a pregnancy outcome was reported, were eligible for the analysis. Anonymised data of four registries was pooled. Rates of adverse pregnancy outcomes were calculated. Systemic inflammation, disease activity and treatment patterns with tumour necrosis factor inhibitor (TNFi) before, during and after pregnancy were analysed. RESULTS: In a total of 332 pregnancies from 304 axSpA women, 98.8% of the pregnancies resulted in live birth. Mean maternal age was 31 years and disease duration 5 years. Most of these patients received pre-conception counselling (78.4%). Before pregnancy, 53% received TNFi treatment, 27.5% in first and 21.4% in third trimester. Pregnancy and neonatal outcomes were favourable with rates of 2.2% for pre-eclampsia, 4.9% for preterm birth, 3.1% for low birth weight and 9.5% for small for gestational age. Neonates were delivered by caesarean section in 27.7% of pregnancies, of which 47.4% were emergencies. Pooled mean CRP was 4 mg/L before conception peaking in the second trimester at 9.4 mg/L. Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) was below 4 at all time-points. CONCLUSIONS: Pooled rates of most outcomes were better than what had been reported in the literature and within expected rates of those reported for the general population. Pre-conception counselling, planned pregnancies and a tight management in expert centres applying a tailored treatment approach may have contributed to the favourable pregnancy outcomes.

EULAR recommendations for a core data set for pregnancy registries in rheumatology.

BACKGROUND AND OBJECTIVE: There is an urgent need for robust data on the trajectories and outcomes of pregnancies in women with inflammatory rheumatic diseases (IRD). In particular when rare outcomes or rare diseases are to be investigated, collaborative approaches are required. However, joint data analyses are often limited by the heterogeneity of the different data sources.To facilitate future research collaboration, a European League Against Rheumatism (EULAR) Task Force defined a core data set with a minimum of items to be collected by pregnancy registries in rheumatology covering the period of pregnancy and the 28-day neonatal phase in women with any underlying IRD. METHODS: A stepwise process included a two-round Delphi survey and a face-to-face meeting to achieve consensus about relevant items. RESULTS: A total of 64 multidisciplinary stakeholders from 14 different countries participated in the two rounds of the Delphi process. During the following face-to-face meeting of the EULAR Task Force, consensus was reached on 51 main items covering 'maternal information', 'pregnancy' and 'treatment'. Generic instruments for assessment are recommended for every item. Furthermore, for the five most frequent IRDs rheumatoid arthritis, spondyloarthritis, juvenile idiopathic arthritis, systemic lupus erythematosus and other connective tissue diseases, disease-specific laboratory markers and disease activity measurements are proposed. CONCLUSION: This is the first consensus-based core data set for prospective pregnancy registries in rheumatology. Its purpose is to stimulate and facilitate multinational collaborations that aim to increase the knowledge about pregnancy course and safety of treatment in women with IRDs during pregnancy.

Pregnancy complications in women with systemic lupus erythematosus. / Svangerskapskomplikasjoner hos kvinner med systemisk lupus erythematosus.

Systemic lupus erythematosus is a rare inflammatory connective tissue disease that affects mainly women, often in their childbearing years. The disease entails an increased risk of fetal and maternal pregnancy complications. Inflammatory active disease and the occurrence of anticardiolipin antibodies are known risk factors. Planning before pregnancy and multidisciplinary structured follow-up reduce the risk of unwanted pregnancy outcomes. Only in exceptional cases should women with systemic lupus erythematosus be advised against pregnancy.

Multidimensional tracking of phenotypes and organ involvement in a complete nationwide systemic sclerosis cohort.

OBJECTIVE: SSc is a severe, heterogeneous multi-organ disease where population-based estimates on phenotypic spectrum, overall disease burden and societal impact are largely missing. Here the objective was to provide the first-ever complete national-level data on phenotype and major organ afflictions in SSc. METHODS: A stepwise strategy was applied to find and characterize every SSc patient resident in Norway from 2000 to 2012. First we identified every case in the country registered with an International Classification of Diseases, Tenth Revision code for SSc (M34). Next we manually reviewed all cases coded as M34 to determine whether they met the 1980 ACR and/or 2013 ACR/EULAR classification criteria for SSc and could be included in the Norwegian SSc cohort (Nor-SSc). Finally, all disease features from SSc onset to study end were reviewed. RESULTS: The Nor-SSc cohort included 815 SSc patients. The mean age at diagnosis was 53 years, with 84% females and 77% limited cutaneous SSc. The estimated incidence increased from 4 per million in 2000 to 13 per million in 2012. We identified high cumulative frequencies of internal organ involvement, coexistence of multiple organ afflictions across disease subsets and autoantibody status and stable frequencies of pulmonary arterial hypertension across haemodynamic definitions, but indications of referral-related differences in pulmonary hypertension detection rates across the study area. CONCLUSION: This nationwide cohort study provides new, unbiased evidence for a high disease burden in SSc patients of Caucasian descent and indicates the existence of hurdles preventing equality of assessment across the SSc population.

Tracking Impact of Interstitial Lung Disease in Systemic Sclerosis in a Complete Nationwide Cohort.

Rationale: Interstitial lung disease (ILD) represents a major challenge in systemic sclerosis (SSc), but there are no precise, population-based data on its overall impact, limiting opportunities for screening and management strategies.Objectives: Evaluate impact of ILD in a unique, nationwide, population-based SSc cohort.Methods: ILD was assessed prospectively in the Norwegian SSc (Nor-SSc) cohort, including all 815 patients with SSc resident in the country from 2000 to 2012. Lung high-resolution computed tomography (HRCT) scans were available for fibrosis quantification at baseline (n = 650, 80%) and follow-up. Pulmonary function tests were assessed at baseline (n = 703, 86%) and follow-up. Vital status and standardized mortality ratios (SMRs) were estimated at study end (2018) in the 630 incident Nor-SSc cases and 15 individually matched control subjects. Cumulative survival rates were computed.Measurements and Main Results: At baseline, 50% of the subjects with SSc (n = 324) had ILD by HRCT and 46% displayed pulmonary function declines consistent with ILD progression. Mortality correlated with extent of lung fibrosis as SMR increased from 2.2 with no fibrosis to 8.0 with greater than 25% fibrosis. SMR was inversely related to baseline FVC% and increased at all FVC levels below 100%. In patients with normal-range baseline FVC (80-100%), the 5- and 10-year survival rates correlated with presence or absence of lung fibrosis, being 83% and 80%, respectively, with no fibrosis and 69% and 56%, respectively, with lung fibrosis (P = 0.03).Conclusions: The mere presence of ILD at baseline appears to affect outcome in SSc, suggesting that all patients with SSc should undergo a baseline pulmonary function test and lung HRCT screening to diagnose ILD early and tailor further management.

Influence of disease activity and medications on offspring birth weight, pre-eclampsia and preterm birth in systemic lupus erythematosus: a population-based study.

OBJECTIVES: Exploring the associations between disease activity and medications with offspring birth weight, pre-eclampsia and preterm birth in systemic lupus erythematosus (SLE). METHODS: Data from the Medical Birth Registry of Norway (MBRN) were linked with data from RevNatus, a nationwide observational register recruiting women with inflammatory rheumatic diseases. Singleton births in women with SLE included in RevNatus 2006-2015 were cases (n=180). All other singleton births registered in MBRN during this time (n=498 849) served as population controls. Z-score for birth weight adjusted for gestational age and gender was calculated. Disease activity was assessed using Lupus Activity Index in Pregnancy. We compared z-scores for birth weight, pre-eclampsia and preterm birth in cases with inactive disease, cases with active disease and population controls. RESULTS: Z-scores for birth weight in offspring were lower in inactive (-0.64) and active (-0.53) diseases than population controls (-0.11). Inactive disease did not predict pre-eclampsia while active disease yielded OR 5.33 and OR 3.38 compared with population controls and inactive disease, respectively. Preterm birth occurred more often in inactive (OR 2.57) and active (OR 8.66) diseases compared with population controls, and in active compared with inactive disease (OR 3.36). CONCLUSIONS: SLE has an increased odds for low birth weight and preterm birth, amplified by active disease. The odds for pre-eclampsia is elevated in active, but not inactive disease. This calls for tight follow-up targeting inactive disease before and throughout pregnancy.

Disease activity during and after pregnancy in women with axial spondyloarthritis: a prospective multicentre study.

Objective: The aim was to study disease activity in women with axial spondyloarthritis (axSpA) during and after pregnancy. Methods: The study included 179 pregnancies in 166 women with axSpA from a Norwegian nationwide register. Disease activity was assessed at seven time points before, throughout and after pregnancy with the DAS BASDAI. Scores assessed at each time point were analysed in a linear mixed model. The same statistical method was used to study self-reported physical functioning, pain and mental health. Results: Altogether, disease activity was stable throughout the study period. We found the highest disease activity and worst self-reported pain in the second trimester, when 45% of the women had active disease. At this time point, disease activity was significantly higher than 6 weeks postpartum (mean BASDAI 3.97 vs 3.46, P = 0.005). Self-reported mental health was also stable, but significantly better 6 weeks postpartum than in the first trimester (mean RAND-36 mental health 79.3 vs 73.2, P < 0.001). Physical functioning was significantly worse in third trimester than postpartum (mean BASFI 3.6 vs 2.6, P < 0.001). Conclusion: Studying women with axSpA, we found that disease activity was highest in the second trimester, but altogether low and stable in the period from planning pregnancy to 1 year after delivery.

Women with systemic lupus erythematosus get pregnant more easily than women with rheumatoid arthritis.

Objectives: To examine possible differences in the ability to get pregnant and time to pregnancy (TTP) in women with SLE and RA, and to study possible influencing factors. Methods: Data from RevNatus, a Norwegian nationwide prospective observational register including women with inflammatory rheumatic diseases when planning pregnancy or after conception, was used. We compared rate of achieved pregnancy, the pregnancy outcomes live birth or pregnancy loss, and TTP between women with SLE (n = 53) and women with RA (n = 180). TTP was compared between the groups using Kaplan-Meier plots, and Cox proportional hazard regression was performed adjusting for maternal age, parity and medication use. RAND-36 was used to assess health-related quality of life (HRQoL) in women achieving and not achieving pregnancy. Results: Women with SLE had a pregnancy ratio of 1.91 (95% CI: 1.27, 2.88, P = 0.002) compared with women with RA, and a substantially shorter median TTP (3.0 vs 7.0 months, P = 0.001). Higher maternal age, medication use and low HRQoL in the physical domains may influence the ability to achieve pregnancy and prolong TTP in women with RA. Women with SLE not achieving pregnancy had lower HRQoL scores than SLE-women achieving pregnancy, while women with RA had generally low scores in physical domains whether or not achieving pregnancy, indicating poor HRQoL. Conclusions: In the studied cohort, women with SLE got pregnant more easily than women with RA.

Reproductive trends in females with inflammatory joint disease.

BACKGROUND: The study assessed birth trends per decade in offspring of females with inflammatory joint diseases (IJD) compared with women without IJD. METHODS: This retrospective cohort study is based on data from the Medical Birth Registry of Norway from 1967 to 2009. We investigated singleton births in females with IJD (n = 7502) and compared with births from the general population (n = 2 437 110). Four periods were examined: 1967-79, 1980-89, 1990-99 and 2000-09. In the logistic regression analysis adjustments were made for maternal age at delivery and birth order. Odds ratios were obtained for the associations between IJD and birth outcome for each period. RESULTS: Females with IJD had in average 65 deliveries / year (0.08 % of all births) in the 1970ies and 274 deliveries / year (0.5 % of all births) from 2000 to 2009. Adjusted Odds ratios (aOR) for newborns small for gestational age were 1.5 (95 % CI 1.2, 1.9) in the earliest and 1.1 (95 % CI 0.9, 1.2) in the last period. Correspondingly, for birth weight < 2500 grams aOR decreased from 1.4 (95 % CI 1.0, 1.9) to 1.1 (95 % CI 0.9, 1.4). For preterm birth aOR was 1.1 (95 % CI 0.8, 1.5) in the first and 1.3 (95 % CI (1.1, 1.5) in the last period. CONCLUSION: An increasing number of births among females with IJD were observed in the study period. Birth weights of newborns of IJD women approached to birth weights in the general population, but preterm birth remained a problem.

Secular trends of pregnancies in women with inflammatory connective tissue disease.

INTRODUCTION: This study examined secular trends in reproductive outcome in women with inflammatory connective tissue disease compared with reference deliveries from the general population. MATERIAL AND METHODS: Historical cohort study based on data registered in the Medical Birth Register of Norway from 1967 to 2009. The study included singleton births in women recorded with connective tissue disease (n = 851) and reference deliveries from the general population (n = 2 437 110). Births were stratified in four periods, 1967-1979, 1980-1989, 1990-1999 and 2000-2009. Associations between connective tissue disease and maternal and perinatal outcomes by decade were assessed in logistic regression analyses and adjusted for maternal age at delivery and parity. RESULTS: In the 1970s, around 2.7 deliveries/year were registered for women with connective tissue disease (0.004% of all deliveries). This increased to 42 deliveries/year (0.07% of all deliveries) after 2000. Adjusted odds ratios (aOR) for cesarean section were 5.0 (95% CI 2.1-11.9) in the first and 1.8 (95% CI 1.4-2.3) in the last period. For preterm delivery the aOR decreased from 4.9 (95% CI 2.1-11.4) to 3.1 (95% CI 2.3-4.2) and the aOR for birthweight <2500 g changed from 7.3 (95% CI 3.3-16.3) to 4.1 (95% CI 3.0-5.6). CONCLUSIONS: An increasing number of births were observed over time among women with connective tissue disease. Adverse pregnancy outcomes were more common among women with connective tissue disease but risks have decreased over time.

Rheumatoid arthritis and outcomes in first and subsequent births based on data from a national birth registry.

OBJECTIVE: To examine associations between rheumatoid arthritis (RA) and pregnancy outcomes in first and subsequent births. DESIGN: Cohort study. SETTING: Study based on data registered in the Medical Birth Registry of Norway from the period 1 December 1998 to 31 December 2009. POPULATION: Singleton births in women recorded with RA (n = 1496) and reference deliveries from the general population (n = 625,642). METHODS: Outcomes of first and subsequent births were analyzed separately. First birth was defined as the first delivery of nulliparous women. Associations between RA and maternal and perinatal outcomes were assessed in logistic regression analyses and adjusted for maternal age at delivery, gestational age, smoking habits and for previous cesarean section when relevant. MAIN OUTCOME MEASURES: Maternal and perinatal outcomes. RESULTS: Vaginal bleeding was observed more often among women with RA both in first pregnancy [adjusted odds ratio (aOR) 1.8, 95% CI 1.3-2.4] and in subsequent pregnancies (aOR 1.4, 95% CI 1.1-1.9). Elective cesarean section was more common among women with RA both in the first birth (aOR 2.0, 95% CI 1.4-2.8) and in subsequent births (aOR 1.5, 95% CI 1.2-2.0). Preterm delivery was more frequent among women with RA than the reference population in first pregnancy (aOR 1.5, 95% CI 1.1-2.0) and in subsequent pregnancies (aOR 1.5, 95% CI 1.1-1.9). CONCLUSION: Complications and poor pregnancy outcomes were more often observed in women with RA and the greatest differences were observed in the first pregnancy.

A population-based study of caesarean section in women with juvenile idiopathic arthritis.

Objectives: The literature on delivery methods in women with JIA is limited. Active inflammation is a risk factor for caesarean section (CS) in other arthritic diseases. A CS entails a higher risk for complications than vaginal delivery and restricted physical activity in the first weeks after birth. Our objective was to explore a possible association of inflammatory active disease and the proportion of CS in women with JIA. Methods: Data from the Norwegian nationwide observational register RevNatus were linked with data from the Medical Birth Registry of Norway (MBRN). Cases comprised singleton births in women with JIA (n = 196) included in RevNatus from 2010 to 2019. Singleton births registered in the MBRN during the same period of time, excluding births in mothers with rheumatic inflammatory diseases, served as population controls (n = 575 798). Results: CS was more frequent in women with JIA (20.4%) and in the subgroup of women with inflammatory active JIA (30.0%) than in population controls (15.6%). Women with active JIA had a risk for elective CS similar to population controls [risk difference 2.3% (95% CI -2.5, 12.9)] and a higher risk for emergency CS [risk difference 14.0% (95% CI 4.3, 27.4)] compared with population controls. Conclusion: Women with active JIA had a higher risk for emergency CS, but not elective CS, compared with population controls.

Breastfeeding in women with systemic lupus erythematosus: results from a Norwegian quality register.

BACKGROUND: Knowledge on breastfeeding among women with systemic lupus erythematosus (SLE) is sparse. We wanted to identify the frequency of breastfeeding in SLE, and to compare breastfeeding women with SLE to non-breastfeeding women to examine possible differences in disease characteristics and self-reported health data between the groups. METHODS: Prospective data on women with SLE from RevNatus, a consent-based Norwegian nationwide quality register was used for this study. Data were collected during January 2016 to September 2021. We used data registered at inclusion when planning pregnancy or in 1st trimester, and 6 weeks, 6 and 12 months after delivery. Breastfeeding and non-breastfeeding patients were compared according to demographic, serological and obstetric data as well as disease activity, medication, self-reported pain, and fatigue. RESULTS: A total of 114 pregnancies in 101 SLE women were included in the analysis. A majority of the women (78%) breastfed six weeks postpartum. Six and 12 months after delivery, breastfeeding rates were 54% and 30% respectively. Six weeks postpartum, non-breastfeeding women showed higher prevalence of emergency caesarean delivery (p = 0.038), preeclampsia (p = 0.056) and lower educational level (p = 0.046) compared to breastfeeding women. 12 months after delivery, we observed a higher frequency of multiparity among breastfeeding women (p = 0.017) compared to non-breastfeeding. Overall, we found low disease activity in both groups at all registrations in the follow-up, and disease activity did not differ between the groups. More than 70% of both breastfeeding and non-breastfeeding women used hydroxychloroquine (HCQ). CONCLUSIONS: Breastfeeding rate in women with SLE was high six weeks postpartum. Multiparous women breastfed longer than primiparas. Disease activity, use of HCQ, and self-reported health data were comparable between the groups. Our data indicate that health professionals should encourage women with SLE to breastfeed.

Caesarean section in women with axial spondyloarthritis and psoriatic arthritis: a population-based study.

BACKGROUND: There is sparse documentation on pregnancy outcomes in women with axial spondyloarthritis (axSpA) and psoriatic arthritis (PsA). Data on disease activity are often lacking, preventing the direct investigation of the effect of inflammation on pregnancy outcomes. A caesarean section (CS) implies a higher risk for complications than vaginal delivery. It delays mobilisation after birth necessary to counteract inflammatory pain and stiffness. OBJECTIVE: To explore a possible association of inflammatory active disease and CS rates in women with axSpA and PsA. METHODS: Data from the Medical Birth Registry of Norway (MBRN) were linked with data from RevNatus, a Norwegian nationwide observational register recruiting women with inflammatory rheumatic diseases. Singleton births in women with axSpA (n=312) and PsA (n=121) included in RevNatus 2010-2019 were cases. Singleton births, excluding mothers with rheumatic inflammatory diseases, registered in MBRN during the same period time (n=575 798) served as population controls. RESULTS: CS occurred more frequently in both axSpA (22.4%) and PsA (30.6%) groups compared with population controls (15.6%), with even higher frequencies in inflammatory active axSpA (23.7%) and PsA (33.3%) groups. Compared with population controls, women with axSpA had higher risk for elective CS (risk difference 4.4%, 95% CI 1.5% to 8.2%) but not emergency CS. Women with PsA had higher risk for emergency CS (risk difference 10.6%, 95% CI 4.4% to 18.7%) but not elective CS. CONCLUSION: Women with axSpA had higher risk for elective and women with PsA for emergency CS. Active disease amplified this risk.

The SPROUT study: A survey on current management practice of reproductive aspects in women of childbearing age with systemic autoimmune rheumatic diseases.

The SPROUT (Survey on reproduction in RheUmaTology) study explored current practice in women of childbearing age with systemic autoimmune rheumatic diseases, investigating the counselling on contraception, the prescription of low dose acetylsalicylic acid (LDASA) to pregnant patients and the management of disease activity in the post-partum period. The SPROUT questionnaire was designed ad hoc and promoted in the three months before the "11th International Conference on Reproduction, Pregnancy and Rheumatic Disease". Between June and August 2021, 121 physicians responded to the survey. Even though 66.8% of the participants declared themselves to be confident in counselling surrounding birth control, only 62.8% of physicians always discuss contraception and family planning with women of childbearing age. Approximately 20% of respondents do not prescribe LDASA to pregnant women with rheumatic diseases, and wide heterogeneity exists in the dose and timing of LDASA prescription. Most respondents (43.8%) restart treatment with biological agents soon after delivery to prevent disease flares, opting for a drug compatible with breastfeeding while 41.3% of physicians continue biologics throughout pregnancy and post-partum. The SPROUT study highlighted the necessity to further foster physicians' education and identified the management of disease activity after delivery as a matter for discussion between all the clinicians involved in the care of pregnant women with rheumatic conditions.

Differential sensitivity of the 2020 revised comprehensive diagnostic criteria and the 2019 ACR/EULAR classification criteria across IgG4-related disease phenotypes: results from a Norwegian cohort.

BACKGROUND: We investigated sensitivity of the 2020 Revised Comprehensive Diagnostic Criteria (RCD) and the 2019 ACR/EULAR classification criteria across the four identified IgG4-related disease (IgG4-RD) phenotypes: "Pancreato-Hepato-Biliary", "Retroperitoneum and Aorta", "Head and Neck-limited" and "Mikulicz' and Systemic" in a well-characterized patient cohort. METHODS: We included adult patients diagnosed with IgG4-RD after comprehensive clinical assessment at Oslo University Hospital in Norway. We assigned patients to IgG4-RD phenotypes based on pattern of organ involvement and assessed fulfillment of RCD and 2019 ACR/EULAR classification criteria. Differences between phenotype groups were analyzed using one-way ANOVA for continuous variables, and contingency tables for categorical variables. RESULTS: The study cohort included 79 IgG4-RD patients assigned to the "Pancreato-Hepato-Biliary" (22.8%), Retroperitoneum and Aorta" (22.8%) "Head and Neck-limited" (29.1%), and "Mikulicz' and Systemic" (25.3%) phenotype groups, respectively. While 72/79 (91.1%) patients in total fulfilled the RCD, proportion differed across phenotype groups and was lowest in the "Retroperitoneum and Aorta" group (66.7%, p < 0.001). Among the 57 (72.2%) patients meeting the 2019 ACR/EULAR classification criteria, proportion was again lowest in the "Retroperitoneum and Aorta" group (27.8%, p < 0.001). CONCLUSION: The results from this study indicate that IgG4-RD patients having the "Retroperitoneum and Aorta" phenotype less often fulfill diagnostic criteria and classification criteria than patients with other IgG4-RD phenotypes. Accordingly, this phenotype is at risk of being systematically selected against in observational studies and randomized clinical trials, with potential implications for patients, caregivers and future definitions of IgG4-RD.

Immunology of pregnancy and reproductive health in autoimmune rheumatic diseases. Update from the 11th International Conference on Reproduction, Pregnancy and Rheumatic Diseases.

Autoimmune rheumatic diseases (ARD) can affect women and men during fertile age, therefore reproductive health is a priority issue in rheumatology. Many topics need to be considered during preconception counselling: fertility, the impact of disease-related factors on pregnancy outcomes, the influence of pregnancy on disease activity, the compatibility of medications with pregnancy and breastfeeding. Risk stratification and individualized treatment approach elaborated by a multidisciplinary team minimize the risk of adverse pregnancy outcomes (APO). Research has been focused on identifying biomarkers that can be predictive of APO. Specifically, preeclampsia and hypertensive disorders of pregnancy tend to develop more frequently in women with ARD. Placental insufficiency can lead to intrauterine growth restriction and small-for-gestational age newborns. Such APO have been shown to be associated with maternal disease activity in different ARD. Therefore, a key message to be addressed to the woman wishing for a pregnancy and to her family is that treatment with compatible drugs is the best way to ensure maternal and fetal wellbeing. An increasing number of medications have entered the management of ARD, but data about their use in pregnancy and lactation are scarce. More information is needed for most biologic drugs and their biosimilars, and for the so-called small molecules, while there is sufficient evidence to recommend the use of TNF inhibitors if needed for keeping maternal disease under control. Other issues related to the reproductive journey have emerged as "unmet needs", such as sexual dysfunction, contraception, medically assisted reproduction techniques, long-term outcome of children, and they will be addressed in this review paper. Collaborative research has been instrumental to reach current knowledge and the future will bring novel insights thanks to pregnancy registries and prospective studies that have been established in several Countries and to their joint efforts in merging data.

Pregnancy and delivery in women with chronic inflammatory arthritides with a specific focus on first birth.

OBJECTIVE: To examine possible associations between chronic inflammatory arthritides and pregnancy outcomes with separate analyses of first and subsequent births before and after diagnosis. METHODS: Linkage of data from a registry of patients with chronic inflammatory arthritides and the Medical Birth Registry of Norway enabled a comparison of pregnancy outcomes in women with chronic inflammatory arthritides and pregnancy outcomes in reference subjects. Outcomes of first birth and subsequent births before and after diagnosis were analyzed separately. Associations between chronic inflammatory arthritides and the women's health during pregnancy and delivery as well as perinatal outcomes were assessed in logistic regression analyses with adjustments for maternal age at delivery and gestational age. RESULTS: We analyzed 128 first births and 151 subsequent births after diagnosis and 286 first births and 262 subsequent births before diagnosis in patients and compared them with first and subsequent births in reference subjects. Firstborn children of women diagnosed as having chronic inflammatory arthritides were more often preterm (odds ratio [OR] 1.85 [95% confidence interval (95% CI) 1.09-3.13]) and small for gestational age (OR 1.60 [95% CI 1.00-2.56]). They also had lower mean birth weight (P=0.01) and higher perinatal mortality (OR 3.26 [95% CI 1.04-10.24]). Birth by caesarean section (all classifications) was more frequent in patients than in reference subjects, and elective caesarean section was 2-fold more frequent in patients, both in first birth (OR 2.60 [95% CI 1.43-4.75]) and in subsequent births (OR 2.18 [95% CI 1.33-3.58]). No excess risks of clinical importance were observed prior to diagnosis of chronic inflammatory arthritides. CONCLUSION: Excess risks were related to first birth in women diagnosed as having chronic inflammatory arthritides, including a higher rate of perinatal mortality. A higher caesarean section rate was related to all patient deliveries. Mainly, pregnancy outcomes before diagnosis did not differ from those in reference subjects.