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Kathryn H Dao, Bonnie L Bermas Division of Rheumatic Diseases, UTSouthwestern Medical Center, Dallas, TX, USACorrespondence: Kathryn H Dao, Email Kathryn.Dao@UTSouthwestren.eduAbstract: Systemic lupus erythematosus (SLE) affects reproductive aged women. Issues regarding family planning are an important part of SLE patient care. Women with SLE can flare during pregnancy, in particular those who have active disease at conception or prior history of renal disease. These flares can lead to increased adverse pregnancy outcomes including fetal loss, pre-eclampsia, preterm birth and small for gestational aged infants. In addition, women with antiphospholipid antibodies can have thrombosis during pregnancy or higher rates of fetal loss. Women who have anti-Ro/SSA and anti-La/SSB antibodies need special monitoring as their offspring are at risk for congenital complete heart block and neonatal lupus. Ideally, SLE patients should have their disease under good control on medications compatible with pregnancy prior to conception. All patients with SLE should remain on hydroxychloroquine unless contraindicated. We recommend the addition of 81mg/d of aspirin at the end of the first trimester to reduce the risk of pre-eclampsia. The immunosuppressive azathioprine, tacrolimus and cyclosporine are compatible with pregnancy and lactation, mycophenolate mofetil (MMF)/mycophenolic acid are not. Providers should use glucocorticoids at the lowest possible dose. Methotrexate, leflunomide and cyclophosphamide are contraindicated in pregnancy and lactation. SLE patients on the biologics rituximab, belimumab and abatacept can continue these medications until conception and resume during lactation.Keywords: systemic lupus erythematosus, fertility, pregnancy, medications

The article presents a study on designing an intervention for with systemic lupus erythematosus (SLE). The study employs henomenologic analysis and single counting methods. Results suggests that community-based intervention is needed to improve lupus education, facilitate better medical care coordination, and decrease isolation.

Immune checkpoint inhibitors (ICIs) have revolutionized oncology treatment. However, their success is mitigated by the recognition that ICI-induced immune-related adverse events (irAEs) pose considerable challenges to patients and clinicians. These autoimmune toxicities are heterogeneous, unpredictable, and reflect a disease state resulting from a change in the immune system of patients. This contrasts with the typical acute nature of toxicities from chemotherapy and molecularly targeted oncology therapies. Management is further complicated by the extended bioavailability of these agents in patients as well as the persistence of autoimmune pathology. Currently, irAE treatment remains suboptimal in many areas, as many expert guidelines remain vague on the optimal selection, dosing, and duration of steroids and the use of other immunosuppressive agents. This coupled with delays in diagnosis and difficulties for patients accessing effective irAE treatment results in barriers to effective irAE care. The latter is complicated by the lack of US Food and Drug Administration-approved irAE treatments that lead to insurance denials, as well as the high cost of biological immunosuppressant therapies. Fortunately, rheumatologists and other subspecialists with expertize in the management of chronic autoimmune conditions have become more involved in irAE diagnosis and management and may help navigate treatment. In this commentary, we discuss these issues and propose potential solutions to advance the field., Competing Interests: Competing interests: DEG reports research funding from AstraZeneca, BerGenBio, Karyopharm, and Novocure; stock ownership in Gilead; consultant/advisory fees from AbbVie, AstraZeneca, Catalyst Pharmaceuticals, Daiichi-Sankyo, Elevation Oncology, Janssen Scientific Affairs, LLC, Jazz Pharmaceuticals, Regeneron Pharmaceuticals, and Sanofi; and serving as co-founder and chief scientific officer of OncoSeer Diagnostics, Inc. AM reports consultant/advisory fees with AbbVie, AstraZeneca, Sanofi, and Amgen., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose of Review: This article will review the current understanding of the immunologic changes that occur during pregnancy. It will discuss the impact of pregnancy on the disease activity of autoimmune or inflammatory rheumatic diseases (AIRD). Lastly, it will highlight the most recent data on pre-conception and pregnancy management practices that can improve pregnancy outcomes in autoimmune patients., Recent Findings: Pregnancy is an immunologically complex and dynamic state that may affect the activity of AIRDs, with more patients having active disease during pregnancy than previously thought. Uncontrolled inflammatory diseases are associated with poor pregnancy outcomes such as preeclampsia, small for gestational age infants, and prematurity. Pre-conception counseling and early pregnancy planning discussions can help ensure optimal disease control and medication management prior to attempting conception. Adequate control of AIRDs on pregnancy-compatible medications during the pre-conception, pregnancy, and postpartum periods is required for optimal pregnancy outcomes., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Purpose of Review: To discuss the current understanding regarding the use of biologic therapeutics in pregnancy., Recent Findings: Our understanding of the mechanisms underlying the potential fetal and infant exposure to biologics as well as a growing body of empirical evidence from real world use of biologics in pregnancy have demonstrated that biologics are generally compatible preconception and during pregnancy. Long-term effects of exposure to biologic agents in utero are not known, but will be uncovered in time. Biosimilars, which are becoming more popular, may not always share the same safety profiles as their originators., Summary: Biologics have revolutionized the management of rheumatologic disease and ushered in a new era of clinical remission among patients. These agents, developed and introduced into clinical use at the beginning of the new millennium, are very potent, yet their efficacy in treating disease often in reproductive aged women, raises questions regarding their safety during pregnancy. These therapeutics can cause immunosuppression and can inhibit immunologic circuits that are not only involved in disease pathophysiology but hypothetically could impact the development of the fetal immune system. Reassuringly, biologics, typically antibodies or antibody-based proteins, are introduced to the fetus via the typical route of transplacental antibody transfer, and thus only begin to be transferred in appreciable amounts in the second trimester (after organogenesis). From theoretic and empirical standpoints, biologic use during pregnancy appears well tolerated for fetal development and to not substantially affect infant immune development., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Systemic lupus erythematosus (SLE), a multiorgan systemic inflammatory disorder, predominantly affects women during their reproductive years. In this review, we summarize the state of knowledge about preconception planning and management of SLE during pregnancy. Achieving remission or low disease activity for several months on medications compatible with pregnancy prior to conception is essential to decreasing the risk of disease flare and improving pregnancy outcomes, including pre-eclampsia, preterm birth, and intrauterine growth restriction. With close management and well-controlled disease before and during pregnancy, <10% of patients flare. All patients with SLE should remain on hydroxychloroquine unless contraindicated. Expectant mothers with a history of antiphospholipid syndrome should be treated with anticoagulant therapy during pregnancy. Women with anti-Ro/SSA or anti-La/SSB antibodies require additional monitoring because their offspring are at increased risk for congenital heart block. Patients with SLE should be offered low-dose aspirin starting at the end of the first trimester to reduce the risk of pre-eclampsia. Flares of SLE during pregnancy require escalation of therapy. The immunosuppressives azathioprine, tacrolimus, and cyclosporine are compatible with pregnancy, and biologic agents can also be considered. Glucocorticoid use in pregnancy should be limited to the lowest effective dose. Mycophenolate mofetil/mycophenolic acid, methotrexate, leflunomide, and cyclophosphamide are known to be teratogenic and are contraindicated in pregnancy. Distinguishing a flare of lupus nephritis during pregnancy from pre-eclampsia can be particularly challenging. Overall, outcomes in pregnancy for women with lupus are improving, but gaps in knowledge about optimal management strategies persist., (© 2023 American College of Rheumatology.)

The antiphospholipid syndrome (APS) is an autoimmune disease characterized by the persistent presence of antibodies directed against phospholipids and phospholipid-binding proteins that are associated with thrombosis and pregnancy-related morbidity. The latter includes fetal deaths, premature birth and maternal complications. In the early 1990s, a distinct set of autoantibodies, termed collectively antiphospholipid antibodies (aPL), were identified as the causative agents of this disorder. Subsequently histological analyses of the placenta from APS pregnancies revealed various abnormalities, including inflammation at maternal-fetal interface and poor placentation manifested by reduced trophoblast invasion and limited uterine spiral artery remodeling. Further preclinical investigations identified the molecular targets of aPL and the downstream intracellular pathways of key placental cell types. While these discoveries suggest potential therapeutics for this disorder, definitive clinical trials have not been completed. This concise review focuses on the recent developments in the field of basic and translational research pursuing novel mechanisms underlying obstetric APS., Competing Interests: Declaration of Competing Interest The authors declare no competing interest., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Competing Interests: The idea for this collaborative global response was born at the inaugural meeting of the EULAR study group for Reproductive Health and Family Planning in Milan in June, 2023. We would like to thank Kirsten Frøhlich (Danish Centre for Expertise in Rheumatology (CeViG), Danish Hospital for Rheumatic Diseases, Sonderborg, Denmark) for her immense logistic support throughout the process of this work. CN-P reports consultancy fees from Sanofi Aventis and UCB; speakers fees from UCB, Sanofi and Otsuka; is a medical advisor (medical advisory board) for the pregnancy sickness support charity; and a patron of the Lauren Page Trust. RJEMD reports grants from Galapagos and UCB; speakers fees from Galapagos, Eli Lilly, Novartis, and UCB; support for attending meetings from UCB; and participation on advisory boards for Galapagos and UCB. MM reports consulting fees from GSK, Eli Lilly, and AstraZeneca; speaker fees from UCB, Eli Lilly, AstraZeneca, GSK, Janssen, and AbbVie; participation on an advisory board for Idorsia; and has received Anifrolumab from AstraZeneca for compassionate use. RF-B reports consulting fees from AbbVie, Otsuka, and UCB; and speakers fees from Biogen, Bristol Myers Squibb, GSK, MSD, Novartis, Sanofi, and UCB. AM reports consulting fees from AbbVie, Biogen, Bristol Myers Squibb, MSD, Novartis, Janssen, Eli Lilly, Pfizer, Amgen, UCB, Gilead, Galapagos; speakers fees from AbbVie, Biogen, Bristol Myers Squibb, MSD, Novartis, Janssen, Eli Lilly, Pfizer, Amgen, UCB, Gilead, Galapagos; and support for attending meetings from AbbVie, Novartis, Galapagos, UCB, Janssen. AT reports speakers fees from GSK and UCB, and participation on advisory boards for Galapagos and UCB. BLB reports royalties from Up To Date and is a member of the data safety monitoring committee for the Stop Bloq study. MEBC reports grants from GSK and UCB and consulting fees from UCB. CC reports research support form Amgen, AstraZeneca, GSK, Janssen, Pfizer, Regeneron, Hoffman La-Roche-Genentech, Genzyme Sanofi-Aventis, Takeda Pharmaceutical Company, Sanofi, UCB, Leo Pharma, Sun Pharma Global FZE, Gilead, Novartis, and the Gerber Foundation; and Speaker honorarium from the American Academy of Allergy, Asthma & Immunology aunual meeting. JB reports consultancy fees from Related Sciences, GSK, and Bristol Myers Squibb. DA reports speakers fees from Bristol Myers Squibb. LA reports consulting fees from Sanofi. GB reports grants from AstraZeneca and Pfizer; consulting fees from AstraZeneca and Eli Lilly; and speakers fees from Aenorasis, AstraZeneca, GSK, Eli Lilly, Novartis, and SOBI. WB reports grants from UCB; is an advisory board member for UCB; payment for expert testimony; and is a member of data safety monitoring boards for the Lutein and Zeaxanthin in Pregnancy study and the Stop Bloq study. AB reports research grants from SOBI and participation on an advisory board for SOBI. IB reports consulting fees from AstraZeneca; speakers fees from Amgen, AstraZeneca, GSK, Eli Lilly, MSD, Roche, Sanofi Genzyme, and UCB; support for attending meetings from UCB; and is an advisory group member for AstraZeneca. RC reports speakers fees from GSK, AstraZeneca, Celgene, Janssen, Eli Lilly, Pfizer, UCB, Rubió, and Werfen. SAE reports support for attending meetings from Pfizer. GE reports consulting fees from GSK and Otsuka; and speakers fees from GSK, Otsuka, and Boehringer Ingelheim. JF reports speakers fees and support for attending meetings from UCB. RF-S reports consultancy fees, speakers fees, and support for attending meetings from AstraZeneca. BG reports grants from Bristol Myers Squibb, Pfizer, and Sandoz. IG reports speakers’ fees from Otsuka and participation on an advisory board for Novartis. MH reports research grants from AbbVie, Biogen, Bristol Myers Squibb, Celltrion, Eli Lilly, Janssen Biologics BV, Lundbeck Foundation, MSD, Pfizer, Roche, Samsung Biopies, Sandoz, Novartis, and Nordforsk; speakers fees from Pfizer, Medac, and Sandoz; and participation on an advisory board for AbbVie. MH has chaired the steering committee of the Danish Rheumatology Quality Registry, which receives public funding from the hospital owners and funding from pharmaceutical companies. MH cochairs EuroSpA, which generates real-world evidence of treatment of psoriatic arthritis and axial spondylorthritis on the basis of secondary data and is partly funded by Novartis. MK is an employee of GSK. RL is an employee of GSK. LFP-G reports consulting fees from Galapagos. MR is a member of the data safety monitoring committee for the Comparison of Step-up and step-down therapeutic strategies in childhood ArthritiS, which is partly supported by Pfizer. AS reports speakers fees from AbbVie, Amgen, Bristol Myers Squibb, Celltrion, MSD, Eli Lilly, Pfizer, Roche, and UCB; and is principle investigator of the German Biologics Register RABBIT, which is supported by grants from AbbVie, Amgen, Bristol Myers Squibb, Celltrion, Fresenius Kabi, Galapagos, Hexal, Eli Lilly, MSD, Pfizer, Samsung Bioepis, Sanofi Aventis, Viatris Sante, UCB, and previously Roche. JV reports speakers’ fees from Eli Lilly, Novartis, Sandoz, and AbbVie. All other authors declare no competing interests.

Family planning in women with vasculitis requires an interdisciplinary approach. This article summarizes recommendations and guidance for each phase of family planning in persons with vasculitis including preconception counseling, birth control, pregnancy, and breastfeeding. Pregnancy complications are presented by category of vasculitis with accompanying diagnostic and therapeutic recommendations. Birth control and assisted reproductive technology options are reviewed with special considerations for women who are high risk or have a history of blood clots. This article can be used as a clinical reference for reproductive discussions in all patients with vasculitis., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose of Review: Immune check point inhibitors (ICIs) are a unique class of cancer treatments that harness the body's innate antitumor response. Although these medications have transformed oncology care, they also lead to generalized immune activation that can result in toxicities across a spectrum of organ systems called immune-related adverse events. This article reviews the most common rheumatologic immune-related adverse events and their management., Recent Findings: Inflammatory arthritis, polymyalgia rheumatic, sicca symptoms, systemic sclerosis, myositis, and vasculitis have all been reported as ICI adverse events. Treatment includes nonsteroidal anti-inflammatory drugs, glucocorticoids, traditional DMARDs, and biologics., Summary: Rheumatologists have an important role in the management of patients with rheumatologic immune-related adverse events. Working with our oncology colleagues, we can help manage rheumatologic immune-related adverse events while optimally preserving ICI's antitumor effects., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Little is known about pregnancy in women with morphea. We aimed to describe the clinical presentation, pregnancy outcomes, and medical management of morphea during pregnancy. We conducted a case series of female patients of reproductive age (18-49 years) who were part of the longitudinal MAC (Morphea in Adults and Children) cohort seen in the outpatient dermatology clinic at the University of Texas Southwestern from July 2007 to February 2022. Women who were pregnant during research visits and had at least 6 months of follow-up in the MAC cohort were included. Data collected included demographics, morphea characteristics, pregnancy outcomes, and medication history. Median clinical disease activity and damage scores using the Localized Scleroderma Cutaneous Assessment Tool were recorded. Ten patients were pregnant during the study period. Five patients had pediatric-onset morphea and five had adult-onset morphea. Eight patients had linear and two had plaque morphea. Six patients had at least one morphea lesion on their abdomen. Median age at first pregnancy was 31 years (interquartile range [IQR], 26.0-35.8 years) and median duration of morphea was 13.5 years (IQR, 4.8-19.0 years). In seven patients, damage scores were stable with no increased morphea activity. Three patients (30%) experienced reactivation of morphea activity during pregnancy with a median Localized Scleroderma Activity Index of 4 (IQR, 2.5-10). Only one patient required immunosuppressive therapy during pregnancy for her morphea diagnosis. Seven of 10 patients had cesarean deliveries and one adverse fetal outcome was reported. In this small series, most patients maintained stability of their morphea and there were no adverse pregnancy outcomes directly related to morphea., (© 2022 Japanese Dermatological Association.)

Objective: To identify perceived health literacy (HL) and patient activation (PA) needs during the transition from pediatric to adult rheumatology among patients with childhood-onset systemic lupus erythematosus (cSLE)., Methods: Semistructured interviews of patients and health care professionals were conducted from November 2019 through May 2020, until thematic saturation was achieved. Interviews were audio-recorded, transcribed, coded, and analyzed using thematic analysis., Results: Thirteen post-transition adult female participants with cSLE were recruited from a public safety-net hospital system or from private practice. Thirteen health care team members were recruited from two pediatric and four adult rheumatology clinical sites serving patients in the same metropolitan area. Patients and health care team members acknowledged numerous HL components as important to transition, including language fluency, education, SLE-specific knowledge, self-efficacy, and accurate knowledge of personal medical history. Our interviews found PA to be an important component of the transition process, driven by internalization of the implications of cSLE diagnosis, self-education, autonomy, introspection, and trustworthy doctor-patient relationships. Patients valued access to their online electronic medical record, recommended multimodal SLE-specific education materials, and desired increased access to social workers. Health care team members stressed the importance of early preparation for transition and use of mobile medical applications and endorsed interventions such as lupus camp and increased partnership with psychologists and social workers., Conclusion: HL and PA are perceived by patients and health care team members as substantially influencing transition success. Further research is needed to evaluate whether interventions to improve HL and PA positively influence cSLE transition outcomes., (© 2022 The Authors. ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)

Objective: Systemic rheumatic conditions affect reproductive-aged patients and often require potentially teratogenic medications. We assessed the feasibility and impact of a standardized pregnancy intention screening question (One Key Question [OKQ]) in a large academic rheumatology practice., Methods: This 6-month pilot quality improvement initiative prompted rheumatologists to ask female patients aged 18 to 49 years about their pregnancy intentions using OKQ. We administered surveys to assess rheumatologists' barriers to and comfort with reproductive health issues. We performed chart reviews to assess uptake and impact on documentation, comparing charts with OKQ documented with 100 randomly selected charts eligible for pregnancy intention screening but without OKQ documented., Results: When we compared 32 of 43 preimplementation responses with 29 of 41 postimplementation responses, the proportion of rheumatologists who reported they were very comfortable with assessing their patients' reproductive goals increased (31%-38%) and the proportion reporting obstetrics and gynecology (OB/GYN) referral challenges as barriers to discussing reproductive goals decreased (41%-21%). During the implementation period, 83 of 957 (9%) eligible patients had OKQ documented in their chart. Female providers were more likely to screen than male providers (odds ratio 2.42, 95% confidence interval 1.21-4.85). Screened patients were more likely to have their contraceptive method documented (P < 0.001) and more likely to have been referred to OB/GYN for follow-up (P = 0.003) compared with patients who were not screened with OKQ., Conclusion: Although uptake was low, this tool improved provider comfort with assessing reproductive goals, the quality of documentation, and the likelihood of OB/GYN referral. Future studies should examine whether automated medical record alerts to prompt screening increase uptake., (© 2022 The Authors. ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)

Background: The psychosocial burden of having a chronic disease can be substantial for adolescents with childhood-onset systemic lupus erythematosus (cSLE). Current literature is scarce on interventions that can improve psychosocial outcomes for this population. Therapeutic recreation camps have been proposed as a beneficial experience for chronically ill pediatric populations. However, their effective components have not been well characterized in patients with cSLE. In this study, we sought to understand the various components of the camp experience for adolescents with cSLE from both the patient and parent perspective., Methods: We recruited patients with cSLE who had participated in one or more annual, weekend-long recreational lupus camp(s) near Dallas, Texas. Semi-structured in-depth telephone interviews were conducted from March-June 2020 with both the patients and parents. Questions focused on overall patient experience, psychosocial impact of camp participation, coping skills gained, and opportunities to prepare for the transition from pediatric to adult care. Interviews were coded and analyzed using inductive thematic analysis., Results: We interviewed 9 current and former campers (ages 16-24), including a current camp counselor, and 3 of their parents separately. Reported benefits included a positive impact on social support through peer bonding, opportunities to develop coping mechanisms through structured activities and peer/medical staff interactions, opportunities for education about the cSLE disease experience, improved adherence through peer modeling, overall increase in self-efficacy, and better parental insight into the patient experience. Participants also provided suggestions for expansion and improvement in program development to optimize educational opportunities for both campers and parents. In addition, they advocated for longitudinal social support and community building., Conclusions: In this qualitative study, in which cSLE patients and their parents reflected on their experiences with therapeutic recreation camps, we found several perceived benefits impacting the patient and parent experience. Participants expressed a desire for more educational opportunities that could contribute to their successful transition from pediatric to adult care. Further studies are needed to demonstrate the effects of therapeutic recreation camps on the psychosocial health of this population., (© 2022. The Author(s).)

Management of women with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and obstetric antiphospholipid syndrome (APS) during pregnancy presents unique clinical challenges. Women with both RA and SLE can have disease flares during pregnancy, leading to pregnancy complications, such as preeclampsia, small-for-gestational-age infants, and preterm delivery. Disease should be under control prior to conception. Women with obstetric APS need to be anticoagulated during pregnancy. Many but not all antirheumatic medications can be used during pregnancy and lactation., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: To describe coronavirus disease 2019 (COVID-19) and pregnancy outcomes in patients with rheumatic disease who were pregnant at the time of infection., Methods: Since March 2020, the COVID-19 Global Rheumatology Alliance has collected cases of patients with rheumatic disease with COVID-19. We report details of pregnant women at the time of COVID-19 infection, including obstetric details separately ascertained from providers., Results: We report on 39 patients, including 22 with obstetric detail available. The mean and median age was 33 years, range 24-45 years. Rheumatic disease diagnoses included rheumatoid arthritis (n = 9), systemic lupus erythematosus (n = 9), psoriatic arthritis/other inflammatory arthritides (n = 8), and antiphospholipid syndrome (n = 6). Most had a term birth (16/22), with 3 preterm births, 1 termination, and 1 miscarriage; 1 woman had yet to deliver at the time of report. One-quarter (n = 10/39) of pregnant women were hospitalized following COVID-19 diagnosis. Two of 39 (5%) required supplemental oxygen (both hospitalized); no patients died. The majority did not receive specific medication treatment for their COVID-19 (n = 32/39, 82%), and 7 patients received some combination of antimalarials, colchicine, anti-interleukin 1β, azithromycin, glucocorticoids, and lopinavir/ritonavir., Conclusion: Women with rheumatic diseases who were pregnant at the time of COVID-19 had favorable outcomes. These data have limitations due to the small size and methodology; however, they provide cautious optimism for pregnancy outcomes for women with rheumatic disease particularly in comparison to the increased risk of poor outcomes that have been reported in other series of pregnant women with COVID-19., (© 2022 The Journal of Rheumatology.)

Immune-related adverse events (irAE) may affect almost any organ system and occur at any point during treatment with immune checkpoint inhibitors (ICI). We present a patient with advanced lung cancer receiving antiprogrammed death 1 checkpoint inhibitor who developed a delayed-onset visual irAE treated with corticosteroids. Through assessment of longitudinal biospecimens, we analyzed serial autoantibodies, cytokines, and cellular populations. Months after ICI initiation and preceding clinical toxicity, the patient developed broad increases in cytokines (most notably interleukin-6 (IL-6), interferon-γ (IFNγ), C-X-C motif chemokine ligand 2 (CXCL2), and C-C motif chemokine ligand 17 (CCL17)), autoantibodies (including anti-angiotensin receptor, α-actin, and amyloid), CD8 T cells, and plasmablasts. Such changes were not observed in healthy controls and ICI-treated patients without irAE. Administration of corticosteroids resulted in immediate and profound decreases in cytokines, autoantibodies, and inflammatory cells. This case highlights the potential for late-onset changes in humoral and cellular immunity in patients receiving ICI. It also demonstrates the biologic effects of corticosteroids on these parameters. Application of humoral and cellular immune biomarkers across ICI populations may inform toxicity monitoring and management., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: The transfer from pediatric to adult care for young adults is a vulnerable period. Our objectives were to quantify the time between the final pediatric and the first adult visit and to evaluate unscheduled utilization in care and progression to end-stage renal disease (ESRD) or death., Methods: We conducted a retrospective analysis of pediatric patients transferring to a large adult rheumatology clinic. Outcomes included time to first completed adult visit, unscheduled health care utilization (hospitalizations and emergency department [ED] visits), and progression to ESRD or death. Multivariable regression models assessed variables predictive of outcomes of interest., Results: A total of 141 pediatric patients who transferred care were identified: 77% female, 65% Hispanic, and 60% with connective tissue diseases (CTDs). The mean time between final pediatric and first completed adult rheumatology visit was 221 days (range 0-1,207 days). In regression modeling, we found that continued insurance coverage, younger age at referral, and referral from a pediatric rheumatologist were predictive of shorter time to completed adult visit (P < 0.005). Factors associated with hospitalizations and ED visits included CTD diagnosis and Black race (odds ratio [OR] 8.54 [95% confidence interval (95% CI) 1.84-39.58] and 3.04 [95% CI 1.02-9.12] for hospitalizations and OR 3.6 [95% CI 1.59-8.14] and 6.0 [95% CI 1.60-22.69] for ED visits, respectively). ESRD or death occurred among 15% of patients with a CTD., Conclusion: In pediatric patients transferring to an adult rheumatology clinic, continued insurance coverage and referral from a pediatric rheumatologist decreased delays in attending an adult visit; CTD and Black race were associated with high rates of unscheduled health care utilization., (© 2020, American College of Rheumatology.)

Objective: African American and Hispanic women with systemic lupus erythematosus (SLE) have the highest rates of potentially avoidable pregnancy complications, yet racial disparities in family planning among reproductive-age women with SLE have not been well-studied. Our objective was to examine whether there are racial differences in contraception encounters and dispensing among US Medicaid-insured women with SLE., Methods: Using Medicaid claims data from 2000-2010, we identified women ages 18-50 years with SLE. We examined contraception encounters and uptake over 24 months. We used multivariable logistic regression to estimate the odds ratio and 95% confidence interval by race/ethnicity of contraception encounters, any contraception dispensing, and highly effective contraception (HEC) use, adjusted for age, region, year, SLE severity, and contraindication to estrogen. We also compared contraception encounters and dispensing among women with SLE to the general population and women with diabetes mellitus., Results: We identified 24,693 reproductive-age women with SLE; 43% were African American, 35% White, 15% Hispanic, 4% Asian, 2% other race, and 1% American Indian/Alaska Native. Nine percent had a contraceptive visit, 10% received any contraception, and 2% received HEC. Compared to White women, African American and Asian women had lower odds of contraception dispensing, and African American women had lower odds of HEC use. Women with SLE were more likely to receive HEC than the general population and women with diabetes mellitus., Conclusion: In this study of reproductive-age women with SLE, African American and Asian women had lower odds of contraception dispensing and African American women had lower odds of HEC use. Further study is needed to understand the factors driving these racial disparities among this population., (© 2020, American College of Rheumatology.)

Background: The transition from pediatric to adult care is a vulnerable period for individuals with chronic diseases. We sought to identify risk factors associated with poor outcomes in patients with childhood-onset systemic lupus erythematosus (cSLE) who have transitioned to adult care., Methods: A retrospective analysis of cSLE patients was performed. Outcomes of interest were development of end-stage renal disease (ESRD) or death and time to first hospitalization following final pediatric rheumatology visit. Multivariable logistic and Cox regression models were used., Results: Of 190 patients with cSLE, 21 (11%) developed ESRD and 9 (5%) died following the final pediatric rheumatology visit. In logistic regression, public insurance, history of Child Protective Services involvement, and an unscheduled hospitalization during the final year in pediatric care were predictive of ESRD or death (odds ratio (95% confidence intervals (CI)) 6.7 (1.5-30.7), 6.6 (2.3-19.1), and 3.2 (1.3-8.3), respectively). Among 114 patients with healthcare utilization data, 53% had a hospitalization in adult care. In Cox regression analysis, a pediatric outpatient opioid prescription was associated with shorter time to adult hospitalization and White or Asian race was associated with longer time to adult hospitalization (hazard ratio (CI) 3.5 (1.7-7.0) and 0.1 (0.03-0.4), respectively)., Conclusions: Risks factors associated with poor outcomes in adult care amongst patients with cSLE include public insurance, history of Child Protective Services involvement, unscheduled care utilization in pediatric care, pediatric outpatient opioid prescription, Black race and Hispanic ethnicity. Efforts to improve long-term outcomes among patients with cSLE should focus on these populations., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest relevant to this article to disclose., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Management of women with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and obstetric antiphospholipid syndrome (APS) during pregnancy presents unique clinical challenges. Women with both RA and SLE can have disease flares during pregnancy, leading to pregnancy complications, such as preeclampsia, small-for-gestational-age infants, and preterm delivery. Disease should be under control prior to conception. Women with obstetric APS need to be anticoagulated during pregnancy. Many but not all antirheumatic medications can be used during pregnancy and lactation., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: To provide guidance to rheumatology providers on the management of adult rheumatic disease in the context of the coronavirus disease 2019 (COVID-19) pandemic., Methods: A task force, including 10 rheumatologists and 4 infectious disease specialists from North America, was convened. Clinical questions were collated, and an evidence report was rapidly generated and disseminated. Questions and drafted statements were reviewed and assessed using a modified Delphi process. This included asynchronous anonymous voting by email and webinars with the entire panel. Task force members voted on agreement with draft statements using a 1-9-point numerical scoring system, and consensus was determined to be low, moderate, or high based on the dispersion of votes. For approval, median votes were required to meet predefined levels of agreement (median values of 7-9, 4-6, and 1-3 defined as agreement, uncertainty, or disagreement, respectively) with either moderate or high levels of consensus., Results: Draft guidance statements approved by the task force have been combined to form final guidance., Conclusion: These guidance statements are provided to promote optimal care during the current pandemic. However, given the low level of available evidence and the rapidly evolving literature, this guidance is presented as a "living document," and future updates are anticipated., (© 2020, American College of Rheumatology.)

Objective: We sought to evaluate and improve knowledge of lactation compatibility of medications commonly used to treat rheumatic diseases among rheumatology, obstetric, and newborn providers practicing at an academic safety net hospital., Methods: Baseline knowledge of rheumatic disease medication compatibility with lactation among 49 providers was obtained via a multiple-choice questionnaire. Following initial evaluation, providers were given a rheumatic diseases and lactation information card. The questionnaire was readministered at the time of card distribution and 5 months later., Results: At baseline, more rheumatology providers correctly identified a higher number of lactation-compatible and noncompatible medications than nonrheumatology providers (78% and 65% vs 31% and 46%, respectively; P < 0.0001). After the intervention, rheumatology providers correctly identified lactation-compatible and noncompatible medications 98% and 100% of the time, compared with 78% and 65% of the time before the intervention (P < 0.0001 and P < 0.0001). This improvement was durable because rheumatology providers correctly identified lactation-compatible and noncompatible medications 96% and 98% of the time 5 months following the initial intervention (P = 0.0021 and P < 0.0001). Nonrheumatology providers correctly identified lactation-compatible and noncompatible medications 31% and 46% of the time before the intervention and 95% and 100% of the time after the intervention (P < 0.0001 and P < 0.0001)., Conclusion: Rheumatology providers had better baseline knowledge than obstetric and newborn providers of the breastfeeding compatibility of medications used to treat rheumatic diseases. However, all providers had knowledge gaps. After a simple educational intervention, the knowledge gap was significantly narrowed in all provider groups. This improvement was durable because repeat testing of the rheumatology provider subset 5 months post intervention continued to show significant improvement., (© 2021 The Authors. ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)

Objective: The transition from pediatric to adult care for youth with childhood-onset systemic lupus erythematosus (SLE) is a vulnerable period. Adverse outcomes during this transition include gaps in care, unscheduled health care utilization, loss of insurance, and high disease activity. The objective of this study was to examine the clinical care teams' perspective on the psychosocial factors associated with transition outcomes, which are poorly understood in this population., Methods: We conducted in-depth interviews with clinical care team members who interact with childhood-onset SLE patients during transfer from pediatric to adult rheumatology. A semistructured interview guide was used to prompt participants' perspectives about the psychosocial factors associated with the transition process for patients with childhood-onset SLE. Audio recordings were transcribed and analyzed using the constant comparative method. We stopped conducting interviews once thematic saturation was achieved., Results: Thirteen in-depth interviews were conducted. Participants included pediatric rheumatologists (n = 4), adult rheumatologists from both academic and private practice settings (n = 4), nurses (n = 2), a nurse practitioner, a social worker, and a psychologist. We identified several themes deemed by clinical care teams as important during the transition, including the impact of the family, patient resilience and coping mechanisms, the role of mental health and emotional support, and the need for education, peer support, and social connectedness., Conclusion: We identified several psychosocial themes that clinical team members believe impact the transition of patients with childhood-onset SLE into adult care. The role of parental modeling, youth resilience, mental health and emotional care, improved childhood-onset SLE education, and structured peer support and social connectedness are highlighted, which may be amenable to interventions., (© 2020, American College of Rheumatology.)

Objective: To provide guidance to rheumatology providers on the management of adult rheumatic disease in the context of the coronavirus disease 2019 (COVID-19) pandemic., Methods: A task force, including 10 rheumatologists and 4 infectious disease specialists from North America, was convened. Clinical questions were collated, and an evidence report was rapidly generated and disseminated. Questions and drafted statements were reviewed and assessed using a modified Delphi process. This included asynchronous anonymous voting by e-mail and webinars with the entire panel. Task force members voted on agreement with draft statements using a 1-9-point numerical scoring system, and consensus was determined to be low, moderate, or high based on the dispersion of votes. For approval, median votes were required to meet predefined levels of agreement (median values of 7-9, 4-6, and 1-3 defined as agreement, uncertainty, or disagreement, respectively) with either moderate or high levels of consensus., Results: To date, the task force has approved 80 guidance statements: 36 with moderate and 44 with high consensus. These were combined, resulting in 27 final guidance statements., Conclusion: These guidance statements are provided to promote optimal care during the current pandemic. However, given the low level of available evidence and the rapidly evolving literature, this guidance is presented as a "living document," and future updates are anticipated., (© 2020, American College of Rheumatology.)

Objective: To provide guidance to rheumatology providers on the management of adult rheumatic disease in the context of the coronavirus disease 2019 (COVID-19) pandemic., Methods: A task force, including 10 rheumatologists and 4 infectious disease specialists from North America, was convened. Clinical questions were collated, and an evidence report was rapidly generated and disseminated. Questions and drafted statements were reviewed and assessed using a modified Delphi process. This included 2 rounds of asynchronous anonymous voting by e-mail and 3 webinars with the entire panel. Task force members voted on agreement with draft statements using a 1-9-point numerical scoring system, and consensus was determined to be low, moderate, or high based on the dispersion of votes. For approval, median votes were required to meet predefined levels of agreement (median values of 7-9, 4-6, and 1-3 defined as agreement, uncertainty, or disagreement, respectively) with either moderate or high levels of consensus., Results: The task force approved 77 initial guidance statements: 36 with moderate and 41 with high consensus. These were combined, resulting in 25 final guidance statements., Conclusion: These guidance statements are provided to promote optimal care during the current pandemic. However, given the low level of available evidence and the rapidly evolving literature, this guidance is presented as a "living document," and future updates are anticipated., (© 2020, American College of Rheumatology.)

Immune-related adverse events induced by immune checkpoint inhibitor (ICI) therapy may affect diverse organ systems, including skeletal and cardiac muscle. ICI-associated myositis may result in substantial morbidity and occasional mortality. We present a case of a patient with advanced non-small cell lung cancer who developed grade 4 myositis with concurrent myocarditis early after initiation of anti-programmed death ligand 1 therapy (durvalumab). Autoantibody analysis revealed marked increases in anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibody levels that preceded clinical toxicity, and further increased during toxicity. Notably, the patient had a history of intolerable statin myopathy, which had resolved clinically after statin discontinuation and prior to ICI initiation. This case demonstrates a potential association between statin exposure, autoantibodies, and ICI-associated myositis., (© AlphaMed Press 2020.)

Background: The coexistence of inflammatory myositis in systemic lupus erythematosus (SLE) has not been extensively studied. In this study, we describe the incidence, distinct types of inflammatory myositis, and risk factors for this finding in a cohort of pediatric and adult SLE patients., Methods: We identified SLE patients with coexisting myositis followed between 2010 and 2019 at two pediatric hospitals and one adult hospital. Demographic, clinical, laboratory, and pathological features of myositis were collected, and descriptive statistics were applied., Results: A total of 1718 individuals were identified as having SLE (451 pediatric and 1267 adult patients). Of these, 108 were also diagnosed with inflammatory myositis (6.3%). People of black race had a significantly higher prevalence of inflammatory myositis, as did those with childhood-onset SLE compared to adult-onset disease. In the majority of patients (68%), SLE and inflammatory myositis presented concurrently. Overlapping features of systemic sclerosis occurred in 48%, while dermatomyositis-specific rashes were present in a third. Arthralgias and inflammatory arthritis were seen in >90%. Thrombotic events and significant pregnancy-related morbidity were present in more than a third of patients. Lymphopenia, hypocomplementemia, and a positive RNP were the most common laboratory features noted. Myositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) were present in >40% of patients. A review of 28 muscle biopsy reports revealed a wide array of pathological features, including nonspecific changes, dermatomyositis, polymyositis, and necrotizing auto-immune myopathy., Conclusion: In our SLE patient population, 6.3% presented with concurrent inflammatory myositis. Dermatomyositis-specific rashes, clinical features of systemic sclerosis, arthralgias and arthritis, and cytopenias were common coexisting clinical manifestations. A high frequency of RNP, MSA, and MAA were found. People of black race and with childhood-onset disease had a higher prevalence of myositis. Our findings suggest that SLE patients of black race, with childhood-onset SLE, and who possess MSA or MAA should be routinely screened for myositis.

Immune checkpoint inhibitor (ICI)-induced immune-related adverse events (irAEs) may affect almost any organ system and occur at any point during therapy. Autoantibody analysis may provide insight into the mechanism, nature, and timing of these events. We report a case of ICI-induced late-onset Raynaud's-like phenomenon in a patient receiving combination immunotherapy. A 53-year-old woman with advanced non-small lung cancer received combination anti-cytotoxic T-lymphocyte antigen 4 and anti-programmed death 1 ICI therapy. She developed early (hypophysitis at 4 months) and late (Raynaud's at >20 months) irAEs. Longitudinal assessment of 124 autoantibodies was correlated with toxicity. Although autoantibody levels were generally stable for the first 18 months of therapy, shortly before the development of Raynaud's, a marked increase in multiple autoantibodies was observed. This case highlights the potential for delayed autoimmune toxicities and provides potential biologic insights into the dynamic nature of these events. KEY POINTS: A patient treated with dual anti-PD1 and anti-CTLA4 therapy developed Raynaud's-like signs and symptoms more than 18 months after starting therapy. In this case, autoantibody changes became apparent shortly before onset of clinical toxicity. This case highlights the potential for late-onset immune-related adverse events checkpoint inhibitors, requiring continuous clinical vigilance. The optimal duration of checkpoint inhibitor therapy in patients with profound and prolonged responses remains unclear., (© 2020 The Authors. The Oncologist published by Wiley Periodicals, Inc. on behalf of AlphaMed Press.)

Objective: To develop an evidence-based guideline on contraception, assisted reproductive technologies (ART), fertility preservation with gonadotoxic therapy, use of menopausal hormone replacement therapy (HRT), pregnancy assessment and management, and medication use in patients with rheumatic and musculoskeletal disease (RMD)., Methods: We conducted a systematic review of evidence relating to contraception, ART, fertility preservation, HRT, pregnancy and lactation, and medication use in RMD populations, using Grading of Recommendations Assessment, Development and Evaluation methodology to rate the quality of evidence and a group consensus process to determine final recommendations and grade their strength (conditional or strong). Good practice statements were agreed upon when indirect evidence was sufficiently compelling that a formal vote was unnecessary., Results: This American College of Rheumatology guideline provides 12 ungraded good practice statements and 131 graded recommendations for reproductive health care in RMD patients. These recommendations are intended to guide care for all patients with RMD, except where indicated as being specific for patients with systemic lupus erythematosus, those positive for antiphospholipid antibody, and/or those positive for anti-Ro/SSA and/or anti-La/SSB antibodies. Recommendations and good practice statements support several guiding principles: use of safe and effective contraception to prevent unplanned pregnancy, pre-pregnancy counseling to encourage conception during periods of disease quiescence and while receiving pregnancy-compatible medications, and ongoing physician-patient discussion with obstetrics/gynecology collaboration for all reproductive health issues, given the overall low level of available evidence that relates specifically to RMD., Conclusion: This guideline provides evidence-based recommendations developed and reviewed by panels of experts and RMD patients. Many recommendations are conditional, reflecting a lack of data or low-level data. We intend that this guideline be used to inform a shared decision-making process between patients and their physicians on issues related to reproductive health that incorporates patients' values, preferences, and comorbidities., (© 2020, American College of Rheumatology.)

There is lack of research on the safety of medications used to treat rheumatic diseases in men wishing to conceive. When evaluating medication safety for potential fathers, two major reproductive issues are to be considered: first, whether a drug induces infertility, and second, whether a drug can cause adverse pregnancy outcomes. Cyclophosphamide is the only medication used in rheumatic disease management that causes irreversible infertility. All men prescribed cyclophosphamide should be counseled on fertility preservation including sperm banking. Sulfasalazine can cause reversible azoospermia; when conception is delayed, this medication should be held for three months and semen analysis should be performed. There are limited data on the teratogenic risk of paternal exposure to medications. Men wanting to conceive should avoid cyclophosphamide and thalidomide. Methotrexate; NSAIDs; glucocorticoids; sulfasalazine; the immunosuppressive agents azathioprine, 6-mercaptopurine, cyclosporine, tacrolimus, and mycophenolate mofetil; colchicine; TNF-alpha blockers; hydroxychloroquine; IVIG; rituximab; abatacept; and anakinra are compatible with paternal exposure. There are insufficient data to conclude the safety of other biologics and small molecules in men seeking to father a child., (Copyright © 2019. Published by Elsevier Ltd.)

Management of pregnancy in patients with rheumatic and musculoskeletal disorders is both challenging and rewarding. Most patients with rheumatic disease can have a successful pregnancy. Pregnancy outcome is improved when maternal autoimmune disease is stable or quiescent. Prepregnancy evaluation and counseling is important to identify risk factors for adverse maternal and pregnancy outcomes and to guide therapy and monitoring. Severe disease-related damage, disease activity, and the presence of antiphospholipid and anti-Ro/SS-A and La/SS-B antibodies may all impact prognosis. Knowledge of the safety of certain medications during pregnancy and lactation is still incomplete, but an increasing number of effective rheumatic disease medications can be used during these periods with low risk to maintain stable disease and to improve outcomes for mother and child., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Pediatric patients often have poor pregnancy outcomes. Systemic lupus erythematosus predominantly impacts women in their second to fourth decade of life, with childhood-onset disease being particularly aggressive. Reproductive issues are an important clinical consideration for pediatric patients with systemic lupus erythematosus (SLE), as maintaining good disease control and planning a pregnancy are important for maternal and fetal outcomes. In this clinical review, we will consider the safety of medications in managing childhood-onset SLE during conception, pregnancy, and breastfeeding. The developing fetus is at highest risk for teratogenicity from maternal medications during the period of critical organogenesis, which occurs between the first 3-8 weeks following conception. Medications known to be teratogenic, leading to a specific pattern of malformations, include mycophenolic acid, methotrexate, and cyclophosphamide. These should be discontinued prior to a planned pregnancy or as soon as pregnancy is suspected. Hydroxychloroquine is safe and should be continued throughout pregnancy and breastfeeding in those without contraindications to it. Azathioprine and calcineurin inhibitors are felt to be compatible with pregnancy in usual doses and may be used prior to and throughout pregnancy and lactation. Non-fluorinated corticosteroids including methylprednisolone and prednisone are inactivated by the placenta and can be used if needed for maternal indication during gestation. Addition of aspirin may be considered around the 12th week of gestation for prevention of pre-eclampsia. Illustrative cases are presented that demonstrate management of adolescents with childhood-onset SLE through conception, pregnancy, and breastfeeding.

After several decades of deliberation, the US Food and Drug Administration updated the Pregnancy and Lactation Labeling Rule in 2015, eliminating the prior A, B, C, D, X grading system for medication use in pregnancy. Although physicians and patients liked the relative ease of use of this system, it was often misconstrued and not updated to include new data suggesting greater compatibility of medications with pregnancy. The new label is designed to include more clinically relevant data, including data from human studies and registries, and fewer animal data. A key goal of the new label is to assist physicians and patients as they weigh the risks and benefits of medications vs the risks of pregnancy in a woman with a chronic, untreated illness. As such, each label now includes a section outlining the pregnancy risks of the diseases that the medication treats. This review includes a historical perspective on the label change and a guide to the interpretation of the new label. It also includes an assessment of the baseline risk of pregnancy in women with SLE and RA, to help balance the consideration of medication risks and benefits in pregnancy.

Objective: To determine the effect of disclosure of rheumatoid arthritis (RA) risk personalized with genetics, biomarkers, and lifestyle factors on health behavior intentions., Methods: We performed a randomized controlled trial among first-degree relatives without RA. Subjects assigned to the Personalized Risk Estimator for Rheumatoid Arthritis (PRE-RA) group received the web-based PRE-RA tool for RA risk factor education and disclosure of personalized RA risk estimates, including genotype/autoantibody results and behaviors (n = 158). Subjects assigned to the comparison arm received standard RA education (n = 80). The primary outcome was readiness for change based on the trans-theoretical model, using validated contemplation ladder scales. Increased motivation to improve RA risk-related behaviors (smoking, diet, exercise, or dental hygiene) was defined as an increase in any ladder score compared to baseline, assessed immediately, 6 weeks, and 6 months post-intervention. Subjects reported behavior change at each visit. We performed intent-to-treat analyses using generalized estimating equations for the binary outcome., Results: Subjects randomized to PRE-RA were more likely to increase ladder scores over post-intervention assessments (relative risk 1.23, 95% confidence interval [95% CI] 1.01, 1.51) than those randomized to nonpersonalized education. At 6 months, 63.9% of PRE-RA subjects and 50.0% of comparison subjects increased motivation to improve behaviors (age-adjusted difference 15.8%; 95% CI 2.8%, 28.8%). Compared to nonpersonalized education, more PRE-RA subjects increased fish intake (45.0% versus 22.1%; P = 0.005), brushed more frequently (40.7% versus 22.9%; P = 0.01), flossed more frequently (55.7% versus 34.8%; P = 0.004), and quit smoking (62.5% versus 0.0% among 11 smokers; P = 0.18)., Conclusion: Disclosure of RA risk personalized with genotype/biomarker results and behaviors increased motivation to improve RA risk-related behaviors. Personalized medicine approaches may motivate health behavior improvements for those at risk for RA and provide rationale for larger studies evaluating effects of behavior changes on clinical outcomes, such as RA-related autoantibody production or RA development., (© 2017, American College of Rheumatology.)

Objective: Immune checkpoint inhibitors are effective cancer therapies that have been associated with immune-related adverse events (irAEs). Recent reports of irAEs describe symptoms resembling classic rheumatologic syndromes, most notably associated with cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) inhibitor blockade. Though cases have been described, there are fewer reports of rheumatologic disease associated with programmed cell death protein-1 (PD-1) inhibitors. Here, we describe a series of four patients presenting to the Brigham and Women's Hospital (BWH) Arthritis Center with de novo polymyalgia rheumatica (PMR)-type conditions and/or peripheral synovitis after treatment with PD-1/PD-Ligand 1 (PD-L1) pathway inhibitors., Methods: Patients with metastatic renal cell carcinoma (RCC) who were treated with PD-1/PD-L1 pathway inhibitors and subsequently developed complaints of new joint pain were referred to the BWH Arthritis Center as part of routine care and identified retrospectively. The electronic medical record was reviewed for cancer history and treatment, rheumatologic symptoms, physical exam, laboratory testing, and clinical course., Results: All four patients developed irAEs consistent with a PMR-type syndrome and/or peripheral synovitis. Symptoms persisted despite discontinuation of the PD-1/PD-L1 pathway inhibitors; however, three of the patients responded well to oral glucocorticoids alone while one patient required the addition of oral methotrexate. All patients had an eventual decline in inflammatory markers., Conclusion: These cases highlight the need for both oncologists and rheumatologists to recognize the development of rheumatologic disease during treatment with immune checkpoint blockade. Further investigation is needed to optimize the management of irAEs, particularly considering the increasing use of checkpoint inhibitors to treat malignancies., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Evidence suggests that continuing hydroxychloroquine (HCQ) during pregnancy in women with systemic lupus erythematosus (SLE) improves outcomes. We sought to describe time trends in the continuation, initiation, and duration of HCQ in a large population-based cohort of pregnant SLE women., Methods: A cohort of pregnant women with SLE enrolled continuously in public (Medicaid, 2001-2010) or private (Optum Clinformatics, 2003-2015) health insurance between three months prior to conception and one month after delivery was identified. We assessed the proportion of women initiating or continuing HCQ and the duration of therapy during each calendar year in the study., Results: A total of 5300 women with SLE were included. Of these, 852 (16.1%) were on HCQ treatment in the three-month period prior to their pregnancy. During pregnancy, the overall proportion of women with SLE taking HCQ increased from 12.4% in 2001 to 37.7% in 2015. Initiation of HCQ therapy during pregnancy increased from 2.7% in 2001 to 7.5% in 2010 (  p  = 0.0002) (Medicaid) and from 4.9% in 2003 to 13.6% in 2015 (  p  = 0.0001) (Clinformatics). Continuation of HCQ during pregnancy did not change significantly over time in either data set. The average cumulative day-supply of HCQ prescriptions during pregnancy increased from 37 days in 2001 to 77 days in 2010 (  p  = 0.05) among HCQ initiators and from 79 days in 2001 to 125 days in 2010 (  p  = 0.0009) among HCQ continuers in Medicaid. Among privately insured women, the average cumulative day-supply of HCQ prescriptions among HCQ continuers increased from 84 in 2004 to 163 in 2015 (  p  = 0.0006) but did not change significantly among HCQ initiators., Conclusion: The proportion of women initiating HCQ during pregnancy and the average cumulative day-supply of HCQ increased from 2001 to 2015. While these findings are encouraging, overall HCQ use during pregnancy remains low.