The estimated prevalence of osteoporosis in Bahrain: a multi-centered-based study.

OBJECTIVES: the primary aim of this study was to examine the prevalence and risk factors of low bone mineral density in Bahrain. METHODS: this was a retrospective study, which targeted a cohort of 4822 Bahraini subjects (mean age 59.36 years: 93% females). Demographic data and results of lumbar and femur DEXA scan for the targeted sample, over the period 2016-2018, were retrieved from four hospitals. RESULTS: The prevalence of low BMD was 62.3% (46.4% had osteopenia and 15.9% had osteoporosis). The highest rate of osteopenia was detected at the age group younger than 44 years. However, with increasing age, the rate of osteopenia declined, whereas osteoporosis increased (P < 0.001). Females were found to be at higher risk of developing both osteopenia (45.8%) and osteoporosis (18.1%) compared to males (39% and 12.4%, respectively) (P < 0.001). Postmenopausal women exhibited higher rates of low BMD (42.4% osteopenia, 22.3% osteoporosis) compared to elderly men (30.9% osteopenia, 9% osteoporosis). CONCLUSIONS: We reported high prevalence of osteopenia and osteoporosis in Bahrain. Low BMD was more common in females, especially in postmenopausal women. Highest prevalence of osteopenia happened at young age. Therefore, we advocate screening at younger age than previously recommended.

Literature review of lupus nephritis From the Arabian Gulf region.

INTRODUCTION: The severity of lupus nephritis (LN) varies between different ethnicities. However, there are limited data regarding disease severity for LN in patients from the Arabian Gulf region; moreover, there are no treatment guidelines developed specifically for this population. The objective of this review was to characterise the incidence of LN, current treatment practices, the severity of LN, and the pathophysiology and biomarkers associated with LN in the Arabian Gulf region. METHODS: A literature search using EMBASE was conducted in October, 2021 to identify publications reporting on the incidence, treatment practices, severity, pathophysiology or biomarkers associated with LN, from countries in the Arabian Gulf region (including Bahrain, Kuwait, Oman, Qatar, Saudi Arabia and the United Arab Emirates). Additional relevant publications were provided by collaborators. A manual review of the publications was conducted to determine their relevance and data on the outcomes of interest were extracted. RESULTS: Of 3705 publications, 54 publications were identified as relevant. LN is one of the most commonly diagnosed renal diseases within the Arabian Gulf and approximately 10%-36% of all renal biopsies are for LN. Treatment patterns within the region appear to vary and generally follow treatment guidelines recommended by the Asia Pacific League of Associations for Rheumatology (APLAR), the European Alliance of Associations for Rheumatology (EULAR) and Kidney Disease Improving Global Outcomes (KDIGO). The majority of patients receive cyclophosphamide for induction therapy, whilst others receive mycophenolate mofetil. Most studies showed that the most frequently diagnosed class of LN within the Arabian Gulf region was Class IV (up to 63% of patients with LN). Sustained or increased levels of serum creatinine and proteinuria; and depressed levels of complement C3/C4 were commonly seen among patients with LN from the Arabian Gulf region. CONCLUSIONS: This review identified that LN may manifest more severely among patients from the Arabian Gulf region than in other populations, such as Caucasian populations. A greater understanding of LN and the treatment practices within the region, as well as the development of more specific treatment guidelines for this population may help improve outcomes for patients with LN in the Arabian Gulf region.

Diagnostic yield of whole-body 18F-FDG PET/CT in patients with suspected cardiac sarcoidosis.

BACKGROUND: Whole-body F-18 FDG PET has been included in the 2014 Heart Rhythm Society guidelines for cardiac sarcoidosis evaluation to identify alternate sites of biopsy prior to endomyocardial biopsy. The purpose of this study was to evaluate the diagnostic yield of whole-body F-18 FDG PET/CT. METHODS: All adult patients with suspected cardiac sarcoidosis undergoing same-day cardiac F-18 FDG PET/CT and whole-body F-18 FDG PET/CT between 10/1/2016 and 6/14/2021 to assess potential biopsy sites were retrospectively identified. Clinical indications, findings, recommendations, and outcomes were assessed. RESULTS: Eighty-eight patients were included. Extracardiac PET findings suggestive of sarcoidosis were present in 30 patients (34%), 27 of which had thoracic findings (90%). Sarcoidosis was diagnosed in 11% of patients. Only 1% (1/88) was diagnosed by extrathoracic biopsy of a whole-body PET finding. Incidental findings were common (31%), resulting in 11 additional tests or interventions. Recommendations from extrathoracic findings affected treatment in one case: a drainage catheter placement into an unsuspected pelvic abscess. CONCLUSION: Addition of whole-body F-18 FDG PET/CT to cardiac F-18 FDG PET/CT for the identification of extrathoracic sites of biopsy in patients with suspected cardiac sarcoidosis has marginal diagnostic yield but commonly results in incidental findings that rarely affect patient outcome.

The rheumatology workforce in the Arab countries: current status, challenges, opportunities, and future needs from an ArLAR cross-sectional survey.

The Arab League of Associations for Rheumatology (ArLAR) Research Group (ARCH) conducted this study to investigate the number of current practicing rheumatologists in the Arab countries, to estimate the projected number of rheumatologists in 10 years, and to evaluate the current workload, practice profile, consultation waiting time, and geographical mobilities of these rheumatologists. This cross-sectional survey study was conducted in 16 Arab countries in two parts. The first survey was addressed nominally to national societies to estimate the current and projected workforce. The second was an anonymous e-survey elaborated by the study steering committee on the Google Forms platform and distributed to Arab rheumatologists using social media, WhatsApp, and mass e-mails to evaluate their practice. The mean number of rheumatologists in Arab countries was 0.84 per 100,000 inhabitants (mean age 47.5 years, 55% females), ranging from 0.06 (Sudan) to 1.86 (Tunisia). The number of rheumatologists is expected to increase by 50% in 2032. Nevertheless, a 20% increase in population associated with an increase in demand is also expected. Data from 446 rheumatologists (mean age 43.9 years, 60.5% females) revealed that 72% worked full-time, and 53% were employed in the public sector only. The average waiting time for a rheumatology consultation was 19.9 days. Of 394 rheumatologists, 19% obtained their rheumatology diplomas from non-Arab countries, and 47% of Gulf rheumatologists were non-citizen physicians. Considering local demographic disparities, healthcare system differences, and geographical mobilities, national authorities are advised to implement effective intervention plans to optimize the rheumatology workforce.

Rising Events and Improved Outcomes of Gastrointestinal Bleed With Shock in USA: A 12-year National Analysis.

OBJECTIVE: Limited information is available based on single-center studies on trends of incidence and outcomes in gastrointestinal (GI) bleed with shock. METHODS: We analyzed data from 2002 to 2013 National Inpatient Sample. Using ICD-9 codes we identified 6.4 million hospital discharges of GI bleed from National Inpatient Sample database. Events were analyzed based on type of GI bleed, in-hospital mortality, hemodynamic status, and use of blood products. RESULTS: GI bleed with shock results in higher hospital mortality (20.77% with shock vs. 2.6% without shock). Between 2002 and 2013, there has been an increase in the percentage of upper and lower GI bleed with shock (1.35% to 4.92% and 1.49% to 3.06%) along with a reduction in mortality in both upper GI bleed with shock (26.9% to 13.8%) and lower GI bleed with shock (54.7% to 19.7%). Consistent with the rise in GI bleed with shock was an increase in blood product utilization. Packed red blood cell (pRBC) transfusion was associated with reduction in mortality in both nonvariceal upper GI bleed with shock (18.3% without pRBC vs. 13.9% receiving pRBC) and lower GI bleed with shock (36.05% without pRBC vs. 22.13% receiving pRBC), but did not affect mortality in variceal upper GI bleed with shock (31.79% vs. 32.22%). CONCLUSIONS: GI bleed with shock carries a higher mortality and have been steadily increasing from 2002 to 2013. pRBC transfusion was associated in improved mortality in GI bleed with shock except variceal bleed.

Disease burden and treatment challenges of psoriatic arthritis in Africa and the Middle East.

Psoriatic arthritis (PsA) is a chronic, inflammatory arthropathy occurring in up to 30% of patients with psoriasis, and is characterized by multiple manifestations including peripheral arthritis, enthesitis, dactylitis, spondylitis, and psoriatic skin and nail disease. This complex and heterogeneous disease is poorly understood and its diagnosis and treatment are suboptimal, particularly in Africa and the Middle East, where very few studies into the impact of PsA have been carried out. This article aims to highlight the disease burden of PsA in the region as well as to identify unmet clinical needs. A non-systematic review was carried out in the PubMed database and the most relevant publications were selected. Expert rheumatologists practicing in Africa and the Middle East provide an insight into the challenges of treating PsA in daily practice, along with recommendations for improvements.

A Novel Technique Utilizing a Vastus Lateralis Anchor in Treatment of Chronic Patellar Dislocation After Total Knee Arthroplasty.

Extensor mechanism complications involving the patella frequently result in total knee arthroplasty (TKA) failure. Postoperative patellar dislocation may be caused by soft tissue imbalance, improper sizing, and position of the prosthesis. This report describes a case of revision TKA requiring several rounds of soft tissue releases in an effort to treat the patient's chronic patellar dislocation. In the process, a novel surgical approach was developed for the surgical management of refractory chronic patellar dislocations. Postoperative follow-up at 1, 2, 6, 12, and 24 months indicated no complaints of patellar subluxation or dislocation. Plain films also demonstrated no effusion, fracture, or patellar dislocation on sunrise, anteroposterior, and lateral views. This novel anatomic repositioning of the vastus lateralis around the quadriceps tendon converted a pathologic lateralizing force into a medial stabilizer. On follow-up evaluations, successful patellar tracking without dislocation has been demonstrated. (Journal of Surgical Orthopaedic Advances.

Effect of Smoking on Joint Replacement Outcomes: Opportunities for Improvement Through Preoperative Smoking Cessation.

Because orthopaedic surgeons focus on identifying serious potential complications, such as heart attack, stroke, and deep vein thrombosis, during the preoperative assessment, correctable factors, such as smoking, may be overlooked. Chronic exposure to nicotine has been correlated with perioperative complications that lead to worse outcomes, including decreased patient satisfaction, longer hospitalization periods, and an increased rate of hospital readmission. It has been proven that smoking is a negative risk factor for decreased bone mineral density, which leads to increased fracture risk, heightened pain, postoperative wound and bone healing complications, decreased fusion rates, and postoperative tendon and ligament healing complications. Physician-led preoperative smoking cessation programs that include, but are not limited to, pharmacotherapy plans have been shown to improve primary surgical outcomes and smoking cessation rates. Smoking has detrimental effects on specialty-specific physiology; however, there are many effective options for intervention that can improve primary outcomes.

Genome wide analysis of novel copy number variations duplications/deletions of different epileptic patients in Saudi Arabia.

BACKGROUND: Epilepsy is genetically complex neurological disorder affecting millions of people of different age groups varying in its type and severity. Copy number variants (CNVs) are key players in the genetic etiology of numerous neurodevelopmental disorders and prior findings also revealed that chromosomal aberrations are more susceptible against the pathogenesis of epilepsy. Novel technologies, such as array comparative genomic hybridization (array-CGH), may help to uncover the pathogenic CNVs in patients with epilepsy. RESULTS: This study was carried out by high density whole genome array-CGH analysis with blood DNA samples from a cohort of 22 epilepsy patients to search for CNVs associated with epilepsy. Pathogenic rearrangements which include 6p12.1 microduplications in 5 patients covering a total region of 99.9kb and 7q32.3 microdeletions in 3 patients covering a total region of 63.9kb were detected. Two genes BMP5 and PODXL were located in the predicted duplicated and deleted regions respectively. Furthermore, these CNV findings were confirmed by qPCR. CONCLUSION: We have described, for the first time, several novel CNVs/genes implicated in epilepsy in the Saudi population. These findings enable us to better describe the genetic variations in epilepsy, and could provide a foundation for understanding the critical regions of the genome which might be involved in the development of epilepsy.

Diagnosis and Management of Extra-articular Causes of Pain After Total Knee Arthroplasty.

Postoperative pain, which has been attributed to poor outcomes after total knee arthroplasty (TKA), remains problematic for many patients. Although the source of TKA pain can often be delineated, establishing a precise diagnosis can be challenging. It is often classified as intra-articular or extra-articular pain, depending on etiology. After intra-articular causes, such as instability, aseptic loosening, infection, or osteolysis, have been ruled out, extra-articular sources of pain should be considered. Physical examination of the other joints may reveal sources of localized knee pain, including diseases of the spine, hip, foot, and ankle. Additional extra-articular pathologies that have potential to instigate pain after TKA include vascular pathologies, tendinitis, bursitis, and iliotibial band friction syndrome. Patients with medical comorbidities, such as metabolic bone disease and psychological illness, may also experience prolonged postoperative pain. By better understanding the diagnosis and treatment options for extra-articular causes of pain after TKA, orthopaedic surgeons may better treat patients with this potentially debilitating complication.

Prevalence and Predictors of Remission and Sustained Remission in Patients with Rheumatoid Arthritis from the United Arab Emirates: A Two-Year Prospective Study.

Aim: To estimate the prevalence of remission and sustained remission for more than 12 months in a cohort of patients with rheumatoid arthritis in the United Arab Emirates and explore predictors of remission and sustained remission in these patients. Methods: A two-year prospective study conducted in Dubai Hospital (January 1, 2018-December 31, 2019) included all consecutive patients with rheumatoid arthritis attending the rheumatology clinic. Patients with a Simplified Disease Activity Index ≤3.3 and/or Clinical Disease Activity Index ≤2.8 in December 2018 were considered in remission and followed until December 2019. Those who maintained remission through 2019 were considered in sustained remission. Results: In this study, a total of 444 patients were followed for a 12-months period. The percentage of remission achieved in RA patients was 30.4% according to the Clinical Disease Activity Index, 31.1% according to Simplified Disease Activity Index, and 50.9% according to the Value of Disease Activity Score 28 (DAS28) remission criteria. The 12-months sustained remission rates ranged from 38.3% for the ACR-EULAR to 69.3% for the DAS28. Male gender, shorter disease duration, better functioning as evaluated by the Health Assessment Questionnaire Disability Index (lower HAQ scores), and higher compliance rates are among sustained remission predictors. Conclusion: Establishing "real-world" data and understanding local predictors to sustained remission is principal for implementing timely and appropriate patient-tailored strategies. These strategies include early detection, close monitoring, and enhancing treatment adherence among UAE patients.

Assessing the Burden of Osteoarthritis in Africa and the Middle East: A Rapid Evidence Assessment.

Introduction/Objectives: This rapid evidence assessment (REA) was conducted to assess the burden of weight-bearing joint osteoarthritis in the developing countries of Africa and the Middle East. Methods: Our REA methodology used a standardized search strategy to identify observational studies, published between January 1, 2010, and April 23, 2020, reporting on outcomes pertaining to the epidemiology and humanistic or economic burden of weight-bearing osteoarthritis. Relevant data from the included studies were used for qualitative analysis. Results: Among the 20 publications reporting on knee osteoarthritis in 10 countries in Africa and the Middle East, 2 also reported on hip, and 1 on foot osteoarthritis. Prevalence of symptomatic/radiographic knee OA was 9-14% among rheumatology outpatients and 31-34% among those with mixed etiology osteoarthritis. Prevalence of knee OA diagnosed by magnetic resonance imaging was 70% among patients ≥40 years of age attending a hospital in Saudi Arabia. Quality-of-life outcomes were reported in 16 publications and suggested a substantial humanistic burden of osteoarthritis, including worse pain, function, and quality of life, and more depression; comparisons between studies were hampered by the variety of tools and scoring scales used, however. No studies reported on economic outcomes. Conclusion: This REA indicates a substantial burden of osteoarthritis in weight-bearing joints in Africa and the Middle East, consistent with publications from other regions of the world.

Comprehensive description of the prevalence, serological and clinical characteristics, and visceral involvement of systemic sclerosis (scleroderma) in a large cohort from the United Arab Emirates Systemic Sclerosis Registry.

Systemic sclerosis is an autoimmune condition characterized by a wide range of clinical presentations. Registries may serve to expand understanding about systemic sclerosis and aid in patient care and follow-up. The objective of this study was to analyze the prevalence of systemic sclerosis in a large cohort from the United Arab Emirates Systemic Sclerosis Registry and find the significant similarities and differences between the different subsets. All scleroderma patients in the United Arab Emirates were included in this multicenter national retrospective analysis. Data on demographics, comorbidities, serological characteristics, clinical aspects, and treatment were collected and analyzed, highlighting the most common traits identified. A total of 167 systemic scleroderma patients from diverse ethnic backgrounds were enrolled. Overall, 54.5% (91/167) of the patients were diagnosed with diffuse cutaneous systemic sclerosis, and 45.5% (76/167) with limited cutaneous systemic sclerosis. The prevalence of systemic sclerosis was 1.66 per 100,000 for the total registry and 7.78 per 100,000 for United Arab Emirates patients. Almost all patients in the diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis groups tested positive for the immunofluorescence antinuclear antibody. Antibodies against Scl-70 were significantly more associated with diffuse cutaneous systemic sclerosis, whereas anticentromere antibodies were significantly more associated with the limited cutaneous systemic sclerosis group (p < 0.001). Sclerodactyly, shortness of breath, and digital ulcers were more common in diffuse cutaneous systemic sclerosis patients compared with the limited cutaneous systemic sclerosis subtype in terms of clinical symptoms and organ involvement. Telangiectasia was much more common in the limited cutaneous systemic sclerosis group. Furthermore, diffuse cutaneous systemic sclerosis patients had more lung fibrosis (interstitial lung disease) than limited cutaneous systemic sclerosis patients (70.5% vs 45.7%), and pulmonary arterial hypertension was twice as common in limited cutaneous systemic sclerosis patients as it was in diffuse cutaneous systemic sclerosis patients. Local registries are paramount to understanding the clinical/serological characteristics of scleroderma. This study emphasizes the importance of raising disease awareness and distinguishing between the various systemic sclerosis subsets to implement patient-tailored strategies for early detection, better management, and higher quality of care.

Consensus statements for pharmacological management, monitoring of therapies, and comorbidity management of psoriatic arthritis in the United Arab Emirates.

OBJECTIVE: Psoriatic arthritis (PsA), a chronic inflammatory disease characterized by heterogeneous clinical manifestations, substantially impacts the quality of life of affected individuals. This article aims at developing consensus recommendations for the management of PsA and associated comorbidities and screening and monitoring requirements of PsA therapies in the United Arab Emirates (UAE) population. METHODS: An extensive review of present international and regional guidelines and publications on the pharmacological management, monitoring of therapies in the context of PsA was performed. Key findings from guidelines and literature were reviewed by a panel of experts from the UAE at several meetings to align with current clinical practices. Consensus statements were formulated based on collective agreement of the experts and members of Emirates Society for Rheumatology. RESULTS: The consensus recommendations were developed to aid practitioners in clinical decision-making with respect to dosage recommendations for pharmacological therapies for PsA, including conventional drugs, non-biologic, and biologic therapies. Consensus recommendations for therapeutic options for the treatment of PsA domains, including peripheral arthritis, axial disease, enthesitis, dactylitis, psoriasis, and nail disease, were developed. The panel emphasized the importance of monitoring PsA therapies and arrived at a consensus on monitoring requirements for PsA therapies. The expert panel proposed recommendations for the management of common comorbidities associated with PsA. CONCLUSION: These consensus recommendations can guide physicians and healthcare professionals in the UAE in making proper treatment decisions, as well as efficiently managing comorbidities and monitoring therapies in patients with PsA.

Consensus statements for evaluation and nonpharmacological Management of Psoriatic Arthritis in UAE.

OBJECTIVE: Psoriatic arthritis (PsA), a chronic inflammatory arthropathy, is often underdiagnosed in Middle Eastern countries, substantially impacting the treatment of affected individuals. This article aims to highlight current unmet clinical needs and provide consensus recommendations for region-specific evaluation methods and nonpharmacological therapies in the United Arab Emirates (UAE). METHOD: An extensive literature review was conducted, focusing especially on global and regional guidelines for the evaluation and treatment of PsA. These form the basis of the consensus statements formulated. Additionally, an expert panel of key opinion leaders from the UAE reviewed these guidelines and available literature at an advisory board meeting to identify unmet needs, bridge clinical gaps in the UAE, and develop consensus statements for the evaluation and treatment of PsA. RESULT: The consensus statements were developed based on overarching principles for the management of PsA, evaluation of patients with PsA, and nonpharmacological approaches for the management of PsA. The overarching principles included adopting a targeted, multidisciplinary approach, along with collaboration between rheumatologists and dermatologists in cases of clinically significant skin involvement. The panel also highlighted the value of composite disease severity measures for characterizing clinical manifestations of PsA. In terms of nonpharmacological management approaches, lifestyle modification (comprising dietary change, exercise, and cessation of smoking) and psychotherapy were recommended. CONCLUSION: The consensus statements will aid healthcare professionals in clinical decision-making in the context of PsA.

Predictors of not Achieving Remission or Low Disease Activity in Axial Spondyloarthritis Patients from Middle Eastern Countries: A Prospective, Multicenter, Real-world Study.

Objectives: We sought to identify the predictors of not achieving remission or low disease activity (LDA) among axial spondyloarthritis (SpA) patients in four Middle Eastern countries. Methods: In this multicenter prospective real-world study, adult patients with axial SpA diagnosed clinically during January-June 2019, and who met the Assessment of SpondyloArthritis International Society classification criteria for axial SpA, were enrolled from the participating centers of four countries-Lebanon, Oman, Qatar, and the UAE. Patient demographics, disease history, comorbidities, treatment, and compliance data were obtained at baseline. The primary outcome was to determine the percentage of patients who did not achieve the clinical target of remission or LDA as indicated by Ankylosing Spondylitis Disease Activity Score-C-reactive protein (ASDAS-CRP) < 2.1 after a three-month follow-up period. Secondary outcomes were assessing the demographic and clinical characteristics of 'achievers' and 'non-achievers' and to study the predictors of ASDAS-CRP ≥ 2.1 in different clinical subsets. Results: The participants were 309 patients of both sexes, with a median age of 43 years. Women had a slight majority (53.7%). At the end of the study, 72.2% of patients achieved the clinical target of ASDAS-CRP < 2.1. Non-achievers were significantly more likely to have enthesitis, positive human leukocyte antigen B 27 status, psoriasis, peripheral involvement, fibromyalgia, and a lower score on Compliance Questionnaire for Rheumatology (CQR). Multiple regression analysis showed that low CQR score, enthesitis, psoriasis, and family history of SpA were independent predictors of ASDAS-CRP ≥ 2.1. Conclusions: This real-world study suggests that low compliance, positive human leukocyte antigen B 27 status, peripheral involvement, and presence of enthesitis, psoriasis, and fibromyalgia are predictors of not achieving remission or LDA in axial SpA patients.

Burden, Treatment Patterns and Unmet Needs of Osteoarthritis in Dubai: a Retrospective Analysis of the Dubai Real-World Claims Database.

INTRODUCTION: Osteoarthritis (OA) is a degenerative joint disease that impacts 3.3-3.6% of population globally with significant health and societal impact. The current study assessed the disease burden, treatment patterns, and healthcare resource utilisation (HCRU) and costs in patients with OA and subgroups of hip and/or knee OA, in Dubai, United Arab Emirates (UAE). METHODOLOGY: This retrospective longitudinal case-control study collected OA-related data from January 1, 2014 to May 31, 2020 from the Dubai Real-World Claims Database (DRWD). Adults aged at least 18 years old with OA diagnosis and at least two claims and continuous enrolment during the study period were included in the study. The patients with OA were 1:1 matched with individuals without OA. The patients with OA were divided into four cohorts on the basis of an a priori algorithm: OA of the hip and/or knee (cohort 1) and (difficult-to-treat) subsets of patients with moderate-to-severe OA of the hip and/or knee (cohort 2), inadequate response or inability to tolerate at least three pain-related medications (cohort 3), and contraindications to nonsteroidal anti-inflammatory drugs (NSAIDs) (cohort 4). RESULTS: Disease burden of OA in Dubai and HCRU and treatment costs in patients with OA were evaluated from January 1, 2014 to May 31, 2021. Patients were compared with matched controls in 1:1 ratio. The overall cohort comprised 11,651 patients with a median age of 48 years and predominantly male population (61.6%). HCRU was calculated for each cohort and it was highest (United States dollar [USD] 11,354.39) in cohort 4 (patients with contraindication to NSAIDS); in cohort 3 (inability to respond to at least three pain-related medications), USD 495.30 and USD 765.14 were spent on medication and procedures, respectively. Highest cost burden was seen in cohort 4, USD 3120.49 on consumables and USD 228.18 on services. CONCLUSION: Osteoarthritis imposes a substantial healthcare and economic burden in the UAE. The study findings elucidate the unmet need among patients with difficult-to-treat OA and inform development of new therapeutics to alleviate their burden.

Clinical Characteristics, Disease Course, and Outcomes of Patients With Acute Generalized Exanthematous Pustulosis in the US.

Importance: Acute generalized exanthematous pustulosis (AGEP) is a rare, severe cutaneous adverse reaction associated with systemic complications. Currently available data are largely limited to small retrospective case series. Objective: To describe the clinical characteristics, disease course, and outcomes of a heterogeneous group of patients with AGEP across the US. Design, Setting, and Participants: A retrospective review of a case series of patients was conducted from January 1, 2000, through July 31, 2020. All 340 included cases throughout 10 academic health systems in the US were scored retrospectively using the EuroSCAR scoring system, and patients with a score corresponding to probable or definite AGEP and aged 18 years or older were included. Main Outcomes and Measures: Patient demographic characteristics, clinical course, suspected causative agent, treatment, and short- and long-term outcomes. Results: Most of the 340 included patients were women (214 [62.9%]), White (206 [60.6%]), and non-Hispanic (239 [70.3%]); mean (SD) age was 57.8 (17.4) years. A total of 154 of 310 patients (49.7%) had a temperature greater than or equal to 38.0 °C that lasted for a median of 2 (IQR, 1-4) days. Of 309 patients, 263 (85.1%) developed absolute neutrophilia and 161 patients (52.1%) developed either absolute or relative eosinophilia. Suspected causes of AGEP were medications (291 [85.6%]), intravenous contrast agents (7 [2.1%]), infection (3 [0.9%]), or unknown (39 [11.5%]). In 151 cases in which a single medication was identified, 63 (41.7%) were ß-lactam antimicrobials, 51 (33.8%) were non-ß-lactam antimicrobials, 9 (6.0%) were anticonvulsants, and 5 (3.3%) were calcium channel blockers. The median time from medication initiation to AGEP start date was 3 (IQR, 1-9) days. Twenty-five of 298 patients (8.4%) had an acute elevation of aspartate aminotransferase and alanine aminotransferase levels, with a peak at 6 (IQR, 3-9) days. Twenty-five of 319 patients (7.8%) experienced acute kidney insufficiency, with the median time to peak creatinine level being 4 (IQR, 2-5) days after the AGEP start date. Treatments included topical corticosteroids (277 [81.5%], either alone or in combination), systemic corticosteroids (109 [32.1%]), cyclosporine (10 [2.9%]), or supportive care only (36 [10.6%]). All-cause mortality within 30 days was 3.5% (n = 12), none of which was suspected to be due to AGEP. Conclusions and Relevance: This retrospective case series evaluation of 340 patients, the largest known study cohort to date, suggests that AGEP onset is acute, is usually triggered by recent exposure to an antimicrobial, may be associated with liver or kidney complications in a minority of patients, and that discontinuation of the triggering treatment may lead to improvement or resolution.

Evaluation of a Case Series of Patients With Generalized Pustular Psoriasis in the United States.

IMPORTANCE: Generalized pustular psoriasis (GPP) is a chronic, orphan disease with limited epidemiological data. OBJECTIVE: To describe the clinical characteristics, treatments, longitudinal disease course, and disease-specific health care utilization among patients with GPP across the United States. DESIGN, SETTING, AND PARTICIPANTS: A retrospective longitudinal case series involving 95 adults who met the European Rare and Severe Psoriasis Expert Network consensus definition for GPP and were treated at 20 US academic dermatology practices between January 1, 2007, and December 31, 2018. MAIN OUTCOMES AND MEASURES: The primary outcome is to describe the patient characteristics, associated medical comorbidities, treatment patterns complications, and GPP-specific health care utilization. RESULTS: Sixty-seven of 95 patients (70.5%) were women (mean age, 50.3 years [SD, 16.1 years]). In the initial encounter, 35 patients (36.8%) were hospitalized and 64 (67.4%) were treated with systemic therapies. In total, more than 20 different systemic therapies were tried. During the follow-up period, 19 patients (35.8%) reported hospitalizations at a median rate of 0.5 hospitalizations per year (IQR, 0.4-1.6). Women had a decreased risk of an emergency department or hospital encounter (odds ratio, 0.19; 95% CI, 0.04-0.83). CONCLUSIONS AND RELEVANCE: Generalized pustular psoriasis is a rare, chronic disease without standard treatment and is associated with continued health care utilization over time.