Nationwide Analysis of Adult-Onset Still Disease With and Without Hemophagocytic Lymphohistiocytosis.

INTRODUCTION: Adult-onset Still disease (AOSD) is a rare inflammatory condition with a monophasic, intermittent, or chronic clinical course, and a subset may experience life-threatening complications such as hemophagocytic lymphohistiocytosis (HLH). This study aims to characterize concurrent AOSD and HLH and identify variables independently associated with in-hospital death. METHODS: We performed a medical records review of AOSD with and without HLH from the 2016-2019 National Inpatient Sample database. We performed a multivariable logistic regression analysis for in-hospital death. Results were reported as adjusted odds ratios (OR adj ). RESULTS: There were 5495 hospitalizations with AOSD, of which 340 (6.2%) had HLH. Thirty (9.0%) of the combined AOSD and HLH group died in the hospital compared with 75 (1.5%) of those without HLH. Multivariable analysis in AOSD inpatients showed that disseminated intravascular coagulation (OR adj 6.13), hepatic failure (OR adj 7.16), infection (OR adj 3.72), respiratory failure (OR adj 6.89), and thrombotic microangiopathy (OR adj 14.05) were associated with higher odds of death. However, HLH itself was not an independent predictor of mortality in AOSD population. CONCLUSIONS: HLH occurred in a small minority of inpatients with AOSD. HLH itself was not an independent risk factor for in-hospital death. Disseminated intravascular coagulation, hepatic failure, infection, respiratory failure, and thrombotic microangiopathy were associated with higher odds of in-hospital death in AOSD. Better awareness of these life-threatening complications may improve hospital outcomes.

Management of immunosuppressants in the era of coronavirus disease-2019.

PURPOSE OF REVIEW: Patients on chronic immunosuppressive treatments at baseline are at increased risk of opportunistic infections. These patients are at especially increased risk of morbidity and mortality during the coronavirus-19 (COVID-19) pandemic. This review will focus on patients with diseases in which immunosuppression is a vital part of the treatment regimen, including those with solid organ transplants, rheumatologic disorders, sarcoidosis, and inflammatory bowel disease (IBD). We will summarize the current knowledge of immunosuppression in these diseases and the risk of contracting COVID-19. Furthermore, we will discuss if immunosuppression increases severity of COVID-19 presentation. RECENT FINDINGS: Since the start of the COVID-19 pandemic, a large number patients receiving chronic immunosuppression have been infected with SARS-CoV-2. Moreover, our understanding of the immunology of SARS-CoV-2 is advancing at a rapid pace. Currently, a number of clinical trials are underway to investigate the role of immunosuppressive treatments in the management of this disease. SUMMARY: Currently, there is no conclusive evidence to suggest that solid organ transplant recipients on chronic immunosuppression are at increased risk of contracting COVID-19. Solid organ transplant recipients may be at increased risk for worse COVID-19 outcomes but the data are not consistent. There is evidence to suggest that patients with rheumatologic disorders or IBDs are not at increased risk of contracting COVID-19 and do not necessarily experience worse clinical outcomes. Patients with sarcoidosis are not necessarily at increased risk of COVID-19, although there is limited data available to determine if immunosuppression worsens outcomes in this population.

COVID-19 infection in patients with sarcoidosis: susceptibility and clinical outcomes.

PURPOSE OF REVIEW: Patients with sarcoidosis may be at higher risk of coronavirus disease-19 (COVID-19) as over 90% of the patients have pulmonary involvement and many are treated with immunosuppressive agents. This review will summarize the current literature regarding sarcoidosis and COVID-19, with a particular focus on susceptibility, clinical outcomes, management, and approach to vaccination. RECENT FINDINGS: Data about COVID-19 and sarcoidosis include a number of case series and reports, cohort studies, and registries. Literature is not conclusive whether patients with sarcoidosis have increased susceptibility to COVID-19. Patients with moderate to severe impaired pulmonary function may be at increased risk of adverse outcomes and mortality. Whether immunosuppressive medication increases risk of COVID-19 severity or affects vaccination response is not yet clear. Novel approaches, such as telemedicine and home monitoring programs, are promising to ensure continuity of care for patients with sarcoidosis during the COVID-19 pandemic. SUMMARY: Current evidence about the risk and clinical outcomes of COVID-19 infection in patient with sarcoidosis, is mainly extrapolated from other immune-mediated diseases. Hence, further research that focuses on the sarcoidosis population is warranted.

Sarcoidosis and autoimmunity.

PURPOSE OF REVIEW: Sarcoidosis is a poorly understood multisystem granulomatous disease that frequently involves the lungs but can affect any organ system. In this review, we summarize recent developments in the understanding of the immune dysregulation seen in sarcoidosis and propose a new expanded definition of human autoimmunity in sarcoidosis, and the implications it would have on treating sarcoidosis with targeted immunotherapy regimens in the future. RECENT FINDINGS: Sarcoidosis has been linked to infectious organisms like Mycobacterium and Cutibacterium, and certain manifestations of sarcoidosis have been linked to specific HLA alleles, but the overall pathogenesis remains uncertain. Sarcoidosis patients have similar patterns of cellular immune dysregulation seen in other autoimmune diseases like rheumatoid arthritis, and recent large-scale population studies show that sarcoidosis frequently presents with other autoimmune diseases. SUMMARY: Advancements in the understanding of sarcoidosis support its consideration as an autoimmune disease. Sarcoidosis patients carry a higher risk of comorbid autoimmune conditions which offers an excellent opportunity to further understand autoimmunity and explore biologic therapies in sarcoidosis treatment, and furthermore will better targeted immunotherapy regimens for sarcoidosis patients in the future.

Treatment of Periorbital Edema in a Patient With Systemic Lupus Erythematosus During Pregnancy: A Case Report Written With the Assistance of ChatGPT.

Systemic lupus erythematosus (SLE) is an autoimmune disease that has a wide range of manifestations and can affect nearly every organ system. Skin manifestations are a common finding in SLE. They are often photosensitive and can be exacerbated by exposure to ultraviolet light. Here, we discuss the case of a 34-year-old African American woman who presented with periorbital edema while 12 weeks pregnant. This case highlights the importance of avoiding sun exposure in patients with SLE and the challenge of treating SLE during pregnancy.

Reasons for Hospitalization and All-Cause Mortality for Adults with Sarcoidosis.

Purpose: Sarcoidosis is a multisystem immune disease with a high rate of hospitalization. There is a paucity of large population-based studies on sarcoid inpatients. We aimed to examine the reasons for hospitalizations and mortality of adult sarcoid patients utilizing the National Inpatient Sample (NIS) database. Methods: Adult hospitalizations in 2016-2019 NIS database with sarcoidosis (ICD-10 code D86) were analyzed. The "reason for hospitalization" and "reason for in-hospital death" were divided into 19 organ system/disease categories based on their principal ICD-10 hospital billing diagnosis. Results: Among the 330,470 sarcoid hospitalizations, cardiovascular (20.4%) and respiratory (16.9%) diagnoses were the most common reasons for hospitalization. The most common individual diagnoses were sepsis and pneumonia. In-hospital death occurred in 2.4% of sarcoid hospitalizations. The most common reasons for death were infectious (30%), cardiovascular (20.7%), and respiratory (20.3%) diagnoses. The most common individual diagnoses in the deceased group were sepsis and respiratory failure. Finally, the sarcoid group had a higher frequency of complications including arrhythmias/heart blocks, heart failure, cranial neuropathies, hypercalcemia, iridocyclitis, myocarditis, and myositis. Sarcoid inpatients had longer length of stay (4 vs 3 days; p < .001) and higher median total hospital charges ($36,865 vs $31,742; p < .001). Conclusions: The most common reasons for sarcoid hospitalizations were cardiovascular and respiratory. Nearly 1 in 40 hospitalizations resulted in death, with most common complications being conduction abnormalities and heart failure. The most common causes of in-hospital death were sepsis and respiratory failure. Sarcoid hospitalizations had 16% higher total hospital charges compared to nonsarcoid inpatients.

Treatment of COVID-19 in Patients With Sarcoidosis.

Recent case reports and studies on treating COVID-19 in patients with chronic sarcoidosis describe different treatment modalities ranging from glucocorticoids to biologic medications. This review article summarizes seven case series and reports totaling 46 patients. While one case report suggested that sarcoidosis medications such as glucocorticoids may lengthen the COVID-19 disease course, another study with a larger registry suggests they do not. More studies are needed to elucidate an improvement in outcomes. It is possible that addition of TNF-alpha inhibitors at COVID-19 diagnosis decreases hospitalization rate. Overall, it is difficult to make firm conclusions regarding treatment given the heterogeneity of treatment modalities in the current literature. Our summarized findings are outlined with the opinions of sarcoidosis, pulmonary, and infectious disease experts in a flow chart that provides clinicians with our proposed management algorithm for sarcoidosis patients who develop COVID-19. We emphasize a need for exchange of information regarding management of COVID-19 in the setting of sarcoidosis to further improve treatment in this vulnerable population of patients.

COVID-19 and Sarcoidosis, Readiness for Vaccination: Challenges and Opportunities.

Sarcoidosis is an immune mediated chronic inflammatory disorder that is best characterized by non-caseating granulomas found in one or more affected organs. The COVID-19 pandemic poses a challenge for clinicians caring for sarcoidosis patients who may be at increased risk of infection compared to the general population. With the recent availability of COVID-19 vaccines, it is expected that clinicians raise questions regarding efficacy and safety in sarcoidosis. However, studies examining safety and efficacy of vaccines in sarcoidosis are lacking. In this review, we examine the current literature regarding vaccination in immunocompromised populations and apply them to sarcoidosis patients. The available literature suggests that vaccines are safe and effective in patients with autoimmune disorders and in those taking immunosuppressive medications. We strongly recommend the administration of COVID-19 vaccines in patients with sarcoidosis. We also present a clinical decision algorithm to provide guidance on vaccination of sarcoidosis patients against COVID-19.

Case Series: COVID-19 in African American Patients With Sarcoidosis.

Data on the clinical presentation and outcomes of sarcoidosis patients with coronavirus disease 19 (COVID-19) are scarce. In this case series, we identified 5 out of 238 sarcoidosis patients who are enrolled in an ongoing longitudinal observational study who developed COVID-19 during the study period and follow their clinical course. Four patients recovered completely, whereas one patient expired during hospital admission. Our preliminary experience suggests that African American patients with chronic sarcoidosis treated with disease-modifying anti-rheumatic drugs (DMARDs) or anti-tumor necrosis factor (TNF) therapy do not seem to be at increased risk of respiratory or life-threatening complications from severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) compared with the general population, although at the present time, we advocate for maintaining a high level of vigilance and strict follow-up in this patient population.

Effects of hand gestures on auditory learning of second-language vowel length contrasts.

PURPOSE: Research has shown that hand gestures affect comprehension and production of speech at semantic, syntactic, and pragmatic levels for both native language and second language (L2). This study investigated a relatively less explored question: Do hand gestures influence auditory learning of an L2 at the segmental phonology level? METHOD: To examine auditory learning of phonemic vowel length contrasts in Japanese, 88 native English-speaking participants took an auditory test before and after one of the following 4 types of training in which they (a) observed an instructor in a video speaking Japanese words while she made syllabic-rhythm hand gesture, (b) produced this gesture with the instructor, (c) observed the instructor speaking those words and her moraic-rhythm hand gesture, or (d) produced the moraic-rhythm gesture with the instructor. RESULTS: All of the training types yielded similar auditory improvement in identifying vowel length contrast. However, observing the syllabic-rhythm hand gesture yielded the most balanced improvement between word-initial and word-final vowels and between slow and fast speaking rates. CONCLUSIONS: The overall effect of hand gesture on learning of segmental phonology is limited. Implications for theories of hand gesture are discussed in terms of the role it plays at different linguistic levels.

Exploring the role of hand gestures in learning novel phoneme contrasts and vocabulary in a second language.

Co-speech hand gestures are a type of multimodal input that has received relatively little attention in the context of second language learning. The present study explored the role that observing and producing different types of gestures plays in learning novel speech sounds and word meanings in an L2. Naïve English-speakers were taught two components of Japanese-novel phonemic vowel length contrasts and vocabulary items comprised of those contrasts-in one of four different gesture conditions: Syllable Observe, Syllable Produce, Mora Observe, and Mora Produce. Half of the gestures conveyed intuitive information about syllable structure, and the other half, unintuitive information about Japanese mora structure. Within each Syllable and Mora condition, half of the participants only observed the gestures that accompanied speech during training, and the other half also produced the gestures that they observed along with the speech. The main finding was that participants across all four conditions had similar outcomes in two different types of auditory identification tasks and a vocabulary test. The results suggest that hand gestures may not be well suited for learning novel phonetic distinctions at the syllable level within a word, and thus, gesture-speech integration may break down at the lowest levels of language processing and learning.