A review of neonatal lupus syndrome.

This review article discusses neonatal lupus syndrome (NLS), an immune-mediated disease caused by maternal antibodies. Maternal antibodies in the fetal circulation are mostly but not always protective. NLS is a disease caused by pathogenic maternal autoantibodies in the fetal circulation. The passive immunization of the fetus by NLS-causing maternal antibodies may occur in the absence of a previously known maternal systemic autoimmune rheumatic disease (SARD). Screening for NLS-related antibodies in patients with related SARD or those in whom there is a risk of NLS including first-degree relatives should occur before pregnancy. This screening is best performed as part of a collaborative relationship between obstetrics and rheumatology. Pregnancy preparations in those with SARD include transitioning to pregnancy-safe medications. The symptoms of NLS range from minor skin rashes to fetal demise from heart block. Fetal screening allows for maternal therapeutic interventions that may be beneficial, as well as the use of fetal pacemakers in the more severe cases that include cardiac NLS.

Glucocorticoid-Induced Osteoporosis: Increased Awareness as a Management Strategy for Prevention of this Complication in Patients with Systemic Autoimmune Rheumatic Disease.

BACKGROUND: It has been estimated that about 1% of the US population is treated with long-term glucocorticoids. High doses of glucocorticoids particularly those used by rheumatologists and oth- ers for systemic autoimmune rheumatic disease result in bone loss, causing glucocorticoid-induced osteoporosis and an increase in the risk of fractures. The increased risk is related to both the daily dose and the cumulative dose of the glucocorticoids. Despite the availability of effective preventative and treatment options, glucocorticoid-induced osteoporosis is often not mitigated with the use of these preventive therapies. The risk of glucocorticoid-induced osteoporosis often also goes under- recognized, because it occurs in a different group of patients compared to age-related osteoporosis. As a result, glucocorticoid-induced osteoporosis is not always treated until after fractures may have occurred. Our objective is to determine if a structured health-care provider's educational interven- tion with intermittent educational updates would lead to improvement in the identification, evalu- ation, and prevention of glucocorticoid-induced osteoporosisin those patients at the highest risk of glucocorticoid-induced osteoporosis. METHODS: In this single-center, prospective study, patients over 40 years of age, receiving a total cumu- lative dose of glucocorticoids of >5 g or a single dose of >30 mg of prednisone or its equivalent was enrolled. All providers attended an academic Journal Club, where the current American College of Rheumatology guidelines regarding glucocorticoid-induced osteoporosiswas reviewed. All providers received monthly reminders during academic meetings within the department. RESULTS: There was a statistically significant improvement between pre- and post-educational data, with increasing use of glucocorticoid-induced osteoporosis preventive measures, which was sus- tained over the 12-month duration of the study. CONCLUSION: This research shows the importance of provider education as a means of disseminating information and improving the quality of patient care.

Impact of SARS-CoV-2/COVID-19 on Provision of Medical Care to Patients With Systemic Autoimmune Rheumatic Disease and the Practice of Rheumatology.

The SARS-CoV-2 pandemic has had a significant impact on the healthcare field that resulted in changes to the way safe and effective medical care is delivered. The effects range from service disruption including ambulatory clinic closure due to both patient and provider concerns, to lack of capacity in hospital services. In rheumatology, there were other effects including viral infection-related autoantibody production, concerns about the use of systemic immunosuppression in the presence of an infectious pandemic and even concerns for viral infection-induced flares of rheumatic disease. Coronavirus disease 2019 (COVID-19) led to the rapid adoption of innovative technologies that permitted the introduction and increased use of telemedicine via a number of platforms. Rapid discoveries and innovations led to the development of diagnostic and therapeutic agents in the management of COVID-19. Scientific advancement and discoveries around COVID-19 infection, symptoms, autoantibody production, chronic sequela and the repurposing of rheumatic immunosuppressive agents led to improved survival and an expanded role for the rheumatologist. Rheumatologists may sometimes be involved in the diagnosis and management of the hospitalized COVID-19 patient. In the ambulatory clinic, a rheumatologist also helps to differentiate between symptoms of long COVID and those of systemic autoimmune rheumatic disease (SARD). Rheumatologists must also grapple with the concerns related to immunosuppressive therapy and the risk of COVID-19 infections. In addition, there are concerns around vaccine effectiveness in people with SARD and those on immunosuppressive medications. Although the SARS-CoV-2 pandemic and the effects on healthcare resulted in difficulties, both patients and providers have risen to the challenge. The long-term outcome of COVID-19 for the medical system and rheumatologists in particular is not yet fully understood and will need further study. This review concentrates on the changing role of the rheumatologists, improved understanding of rheumatic disease and immunosuppressive therapies in the wake of the pandemic and how this has led to an improvement in the care of patients with COVID-19.

A Therapeutic Approach to Primary Hyperparathyroidism: A Collaborative Evaluation Between Endocrinology and Surgery.

Introduction Primary hyperparathyroidism (PHPT) is a disorder in which one or more parathyroid glands overproduce parathyroid hormone, leading to hypercalcemia. It may present with symptoms like constipation, abdominal pain, psychiatric complaints as well as signs of nephrolithiasis, and osteoporosis that may need surgical treatment. PHPT is often underdiagnosed and undertreated. Our study aimed to review hypercalcemia in a single center to evaluate for undiagnosed PHPT. Methods A group of 546 patients from Southwest Virginia was selected using the EMR Epic (Epic Systems, Verona, USA) who had a diagnosis of hypercalcemia in the previous six months. Charts were manually reviewed, and patients were excluded based on the lack of hypercalcemia as well as previous testing of parathyroid hormone (PTH) levels. One hundred and fifty patients were excluded because of a lack of documented hypercalcemia. Letters were sent to the patients advising the patients to discuss whether a PTH would be indicated with their primary care provider (PCP). These patients' charts were re-examined six months later and a determination of whether a PTH level was performed as well as any referrals explicitly for hypercalcemia or PHPT was done. Results A total of 20 (5.1%) patients had a new PTH test during the time assessed. Of these patients, five received referrals to surgical treatment and six received referrals to endocrinology for treatment (no patients received referrals to both). Of those who obtained a PTH level, 50% of them had significantly elevated PTH levels consistent with the diagnosis of primary hyperparathyroidism. An additional 45% had levels of parathyroid hormone that were in the normal range but or most likely inappropriately normal for the concurrent level of calcium. Only one patient (5%) had a suppressed PTH level. Conclusion The impact of an intervention on clinician evaluation and treatment of patients with hypercalcemia has been previously tested and shown to be beneficial. In this study, the method of directly sending a letter to patients yielded clinically significant results, with 20 patients out of 396 (5.1%) having a PTH level tested. A majority had an overt or suspected parathyroid disease, and out of them, 11 underwent referral for treatment.

Kikuchi-Fujimoto Disease: A Differential for When It is Not Systemic Lupus Erythematosus.

Kikuchi-Fujimoto disease (KFD) is a rare and benign disease process that is characterized by fever and lymphadenopathy that was first described in young Japanese women in the early 1970s. Knowledge of KFD is important as it can often mimic other causes of lymphadenopathy including systemic lupus erythematosus (SLE) or malignancies, and this can lead to invasive diagnostic testing and even treatments that can be avoided. The etiology and exact mechanism by which KFD develops is not fully understood at this time, but is thought to be an immune response of T cells and histiocytes to viral or bacterial infections. We present a 35-year-old African-American woman who was referred to the rheumatology clinic by our colleagues in the breast clinic with new onset right axillary lymphadenopathy and abnormal serologic testing with the suspicion of SLE after a malignancy had been ruled out.

Statin-Related Necrotizing Autoimmune Myositis: More Than Myalgia.

Statins are widely prescribed for cardiovascular disease, and, in general, are well tolerated by most people. Most side effects related to statins are mild, with some side effects also considered a nocebo effect. Occasionally, statins can be associated with severe side effects. One of the more severe adverse events is immune-mediated necrotizing myositis, which is both difficult to diagnose and treat. The symptoms can be debilitating, and aggressive immunosuppressive therapy is the best-recognized method of treatment of this complication. In this case report, we discuss the clinical features, diagnosis, and treatment of this disease entity with an emphasis on the need for rapid diagnosis and aggressive treatment to help reduce morbidity.

The Shifting Landscape of Lupus Nephritis Management: A Review.

Systemic lupus erythematosus (SLE) is commonly the first autoimmune disease that comes to mind for most people when rheumatology is mentioned. It remains an enigma that many of us, including patients and healthcare providers, do not fully understand. Although an ancient disease, it still remains difficult to both diagnose and treat. Historically, there has always been a paucity of therapeutic interventions for SLE as a whole. One of the most distressing manifestations for the patient and diagnostic and therapeutically challenging aspects of SLE is lupus nephritis (LN). There has historically been some difficultly in the development of LN drugs that provide significant therapeutic benefits while having an acceptable side-effect profile. This difficulty led to decades in which no drugs were approved for LN. With a better understanding of the pathogenesis of SLE and LN and improvement in trial design, great therapeutic strides have recently been made. The immunosuppressive landscape of LN has changed recently with the approval of two newer agents as well as a number of promising trials in LN. With the increased number of therapeutic agents (both immunosuppressive and non-immunosuppressive), the clinical question is how and when to use these medications, and, more importantly, which agents to use first. With the increased number of agents, the answers to these questions are becoming more difficult to answer. The purpose of the paper is to review updates in LN diagnosis and management.

Determination of the Risk Factors Contributing to the Development of Neuropsychiatric Lupus in a Systemic Lupus Erythematosus Cohort.

Background Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a complex, varied clinical presentation that is both more common and has poor outcomes in women of color. SLE outcomes also seem to be influenced by socioeconomic factors. Neuropsychiatric lupus (NPL) is a common manifestation of SLE that is difficult to diagnose and treat and has poor clinical outcomes. There is no clear relationship between NPL and SLE-related autoantibodies, and this contributes to the difficulty in diagnosing NPL. As a result, NPL is a significant contributor to morbidity and mortality in patients with SLE. Objective The purpose of this study was to examine the relationship between serological and socioeconomic factors in the development of NPL in our patient cohort and determine the risk factors for the development of NPL. Methods This was an SLE single-center, retrospective chart review study that was performed at a university-based tertiary referral center. Patients aged 18 and older who meet the American College of Rheumatology (ACR) 1997 criteria and were seen between June 1st, 2015, and June 1st, 2019, were included in this study. Overall, 629 patients with SLE were identified, and 263 patients were included. Demographic and serological data were collected. Supplemental socioeconomic information for each zip code in Southwest Virginia was obtained from the United States Government Census website. Continuous variables were analyzed using the T-test or Mann-Whitney U test. Categorical variables were analyzed using chi-square tests or Fisher's exact tests. Statistical analysis was performed using SAS9.4, and p-value < 0.05 was considered statistically significant. Results We reviewed a number of risk factors including age, sex, race, and median household income (MHI), noting no statistical relationship between these factors and the diagnosis of NPL. We did find that the presence of antiphospholipid antibodies (aPL) was significantly associated with a diagnosis of NPL and that complement 4 (C4) levels trended toward statistical significance. Conclusion In our cohort of patients, there was no relationship between age, sex, race, and median household income, and the diagnosis of NPL. There was a statistically significant relationship between aPL and the diagnosis of NPL. Other SLE-related antibodies showed no statistical relationship with the diagnosis of NPL. Although not statistically significant, there was a trend toward significance between complement 4 (C4) levels and the diagnosis of NPL.

The Best of Times and Yet the Worst of Times.

Young adults represent a vulnerable population for multiple reasons. This time in an individual's life coincides with many personal and professional changes and challenges. Some individuals may embrace the change, but for those with a chronic illness, this transition may be difficult. As one is navigating decisions that will ultimately impact their future, they must also navigate changes related to their healthcare. Here, we discuss a case involving a transition from a pediatric to an adult rheumatology clinic and the impact on the patient. This case will highlight some of the challenges patients face and will explore how this process could be improved for our patients. For many young individuals, this is the best of times as they are transitioning to an adult but also the worst of times as they must now make adult decisions with adult consequences.

Myocarditis Secondary to Mesalamine-Induced Cardiotoxicity in a Patient with Ulcerative Colitis.

Development of cardiac manifestations in patients diagnosed with inflammatory bowel disease undergoing treatment with mesalamine is a rare. When this occurs, it can be difficult to tease out the primary etiology, as both IBD and mesalamine can cause cardiac manifestations independently of each other. The exact mechanism of mesalamine-induced cardiotoxicity is yet to be determined although several mechanisms have been described. We present the case of a gentleman with nonexertional chest pain in the setting of ulcerative colitis exacerbation believed to have occurred secondary to mesalamine.

Gut Microbiota in Human Systemic Lupus Erythematosus and a Mouse Model of Lupus.

Gut microbiota dysbiosis has been observed in a number of autoimmune diseases. However, the role of the gut microbiota in systemic lupus erythematosus (SLE), a prototypical autoimmune disease characterized by persistent inflammation in multiple organs of the body, remains elusive. Here we report the dynamics of the gut microbiota in a murine lupus model, NZB/W F1, as well as intestinal dysbiosis in a small group of SLE patients with active disease. The composition of the gut microbiota changed markedly before and after the onset of lupus disease in NZB/W F1 mice, with greater diversity and increased representation of several bacterial species as lupus progressed from the predisease stage to the diseased stage. However, we did not control for age and the cage effect. Using dexamethasone as an intervention to treat SLE-like signs, we also found that a greater abundance of a group of lactobacilli (for which a species assignment could not be made) in the gut microbiota might be correlated with more severe disease in NZB/W F1 mice. Results of the human study suggest that, compared to control subjects without immune-mediated diseases, SLE patients with active lupus disease possessed an altered gut microbiota that differed in several particular bacterial species (within the genera Odoribacter and Blautia and an unnamed genus in the family Rikenellaceae) and was less diverse, with increased representation of Gram-negative bacteria. The Firmicutes/Bacteroidetes ratios did not differ between the SLE microbiota and the non-SLE microbiota in our human cohort.IMPORTANCE SLE is a complex autoimmune disease with no known cure. Dysbiosis of the gut microbiota has been reported for both mice and humans with SLE. In this emerging field, however, more studies are required to delineate the roles of the gut microbiota in different lupus-prone mouse models and people with diverse manifestations of SLE. Here, we report changes in the gut microbiota in NZB/W F1 lupus-prone mice and a group of SLE patients with active disease.