Medicine and Health Sciences Commons^™

Gender Dysphoria In Adolescents With Ehlers-Danlos Syndrome., Jordan T. Jones, William R. Black, Christine Moser, Eric T. Rush, Lindsey Malloy-Walton

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: Ehlers-Danlos Syndrome represents a family of heritable connective tissue disorders that include joint hypermobility, tissue fragility, and skin hyperextensibility. Ehlers-Danlos Syndrome presents with clinical sequela across multiple body systems that require multidisciplinary care. Little is known about adolescents with Ehlers-Danlos Syndrome who are transgender and gender diverse. To date, there have been no reports of transgender and gender diverse youth in pediatric patients with Ehlers-Danlos Syndrome. The objective of this study was to characterize transgender and gender diverse adolescents with Ehlers-Danlos Syndrome seen in a pediatric multidisciplinary specialty clinic.

METHODS: A retrospective chart review was performed and it was …

Treatment Of Severe Swallowing Dysfunction In Systemic Sclerosis With Ivig: Role Of Antimuscarinic Antibodies, Fabian A Mendoza, Anthony J. Dimarino, Sidney Cohen, Christopher Adkins, Shady Abdelbaki, Satish Rattan, Christopher Cao, Susie Denuna-Rivera, Sergio A. Jimenez

Division of Gastroenterology and Hepatology Faculty Papers

Oropharyngeal and esophageal dysmotility can cause serious clinical complications such as aspiration pneumonia, cachexia, and sarcopenia, with a resulting increase in mortality and disability. The current standard of care for the treatment of SSc-associated swallowing dysfunction is mainly supportive, although severe cases are usually refractory to conventional management. Recent studies have shown that the abnormal production of functional autoantibodies such as anti-cholinergic muscarinic receptor III antibodies may participate in the pathogenesis of SSc-associated gastrointestinal dysmotility and may provide a novel target for therapeutic intervention. We describe two patients with severe and rapid onset of SSc-associated severe swallowing dysfunction and esophageal …

False Positive Anti-Topoisomerase I (Scl-70) Antibody Results In Clinical Practice: A Case Series From A Scleroderma Referral Center, Brian H Lam, Shervin Assassi, Julio Charles, Rana Taherian, Marka A Lyons, Bochra Jandali, Maureen D Mayes, Brian Skaug

Journal Articles

PURPOSE: To determine if some patients who tested positive for anti-Scl-70 antibody in clinical practice, but did not have classifiable systemic sclerosis, were negative for anti-Scl-70 antibody by the more specific immunodiffusion method of testing.

METHODS: Patients evaluated by a rheumatologist at a Scleroderma referral center who had tested positive for anti-Scl-70 antibody prior to referral, but did not have classifiable SSc based on clinical criteria, were invited to undergo testing for anti-Scl-70 antibody by immunodiffusion. Patient demographics and clinical features were recorded at the time of their evaluation, and diagnostic testing results were reviewed using the medical records.

RESULTS: …

Minimal Impact Of The Covid-19 Pandemic On Disease Activity And Health-Related Quality Of Life In Patients With Ankylosing Spondylitis Receiving Bimekizumab: Exploratory Analyses From A Phase 2b Open-Label Extension Study., Philip C Robinson, Pedro M Machado, Nigil Haroon, Lianne S Gensler, John D Reveille, Vanessa Taieb, Thomas Vaux, Carmen Fleurinck, Marga Oortgiesen, Natasha De Peyrecave, Atul Deodhar

Journal Articles

OBJECTIVE: The impact of the COVID-19 pandemic on patients with inflammatory rheumatic diseases, such as ankylosing spondylitis (AS), has been variable. Here, we assess disease activity and health-related quality of life (HRQoL) through the pandemic in patients with AS.

METHODS: In the open-label extension (OLE) of the phase 2b BE AGILE study, patients with AS received 160 mg of subcutaneous bimekizumab every 4 weeks. We assessed Ankylosing Spondylitis Disease Activity Score with C-reactive protein (ASDAS-CRP), Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and Ankylosing Spondylitis Quality of Life (ASQoL) scores in the OLE immediately before and during the COVID-19 pandemic …

Biological Treatment Usage In Patients With Hiv And Rheumatic Disease, 2003-2021: Long-Term Safety And Follow-Up, Benjamin Sornrung Naovarat, Gloria Salazar, Mariko Ishimori, Francis M Williams, John D Reveille

Journal Articles

OBJECTIVE: This study examined the safety and efficacy of biological agents, especially tumour necrosis factor (TNF) inhibitors, for HIV-positive rheumatology patients refractory to standard therapy.

METHODS: This study is a retrospective case series including patients derived from a community HIV clinic as well as from two academic centres. Initial visit data collected included: sociodemographic characteristics, CD4 counts, HIV viral load and medication use. Patients with persistent disease activity despite standard conservative therapy were begun on biological agents.The main outcomes were patient and physician global assessment of treatment response and medication side effects in patients with rheumatological disorders treated with biological …

Role Of Lipid-Lowering Therapy In Peripheral Artery Disease, Agastya Deepak Belur, Aangi J. Shah, Salim S. Virani, Mounica Vorla, Dinesh K. Kalra

Office of the Provost

Atherosclerosis is a multifactorial, lipoprotein-driven condition that leads to plaque formation within the arterial tree, leading to subsequent arterial stenosis and thrombosis that accounts for a large burden of cardiovascular morbidity and mortality globally. Atherosclerosis of the lower extremities is called peripheral artery disease and is a major cause of loss in mobility, amputation, and critical limb ischemia. Peripheral artery disease is a common condition with a gamut of clinical manifestations that affects an estimated 10 million people in the United States of America and 200 million people worldwide. The role of apolipoprotein B-containing lipoproteins, such as LDL and remnant …

Urate-Lowering Therapy For Patients With Gout On Hemodialysis, Reem Alkilany, Douglas Einstadter, Maria Antonelli

Faculty Scholarship

Objective: Gout is the most common form of inflammatory arthritis and is caused by deposition of monosodium urate crystals resulting from a high burden of uric acid (UA). High UA burden also has been associated with increased morbidity and mortality in the general population and progression to chronic kidney disease. In persons with gout and end-stage renal disease (ESRD), prior studies suggest that UA levels decrease after initiation of hemodialysis (HD). We evaluated UA level and the use of urate-lowering therapies (ULTs) in patients with gout and ESRD on HD. Methods: We performed a retrospective review of patients with gout …

A Rare Case Of Segmental Arterial Mediolysis, Ashbina Pokharel, Lauren Reinhold, Ashbita Pokharel, Judith Bateman

Conference Presentation Abstracts

Segmental arterial mediolysis (SAM) is a rare vasculopathy of unknown etiology characterized by disruption of the arterial layer, which can result in vessel dissection, hemorrhage and ischemia. Literature review has demonstrated a varied presentation ranging from benign abdominal pain to life threatening hemorrhage. We report a case of 58 year old female with past medical history of deep vein thrombosis/pulmonary embolism secondary to Protein C and S deficiency on Coumadin who presented with right flank pain for 3 days. Complete blood count and complete metabolic panel were within normal limits. CT scan of abdomen and MRI showed ischemia of segment …

Perifollicular Hypopigmentation In Systemic Sclerosis: Associations With Clinical Features And Internal Organ Involvement, Melody P Chung, Christopher A Mecoli, Jamie Perin, Carrie Richardson, Zsuzsanna H Mcmahan

Journal Articles

OBJECTIVE: To determine whether perifollicular hypopigmentation in systemic sclerosis (SSc) is associated with demographics, distinct clinical features, and autoantibody profiles.

METHODS: Patients with SSc were prospectively enrolled, with a standardized data form used to collect anatomic distribution of perifollicular hypopigmentation. Associations between hypopigmentation and features of SSc were assessed.

RESULTS: Of 179 adult patients with SSc, 36 (20%) patients had perifollicular hypopigmentation. Of these 36 patients, 94% (n = 34) were female and 33% (n = 12) had limited cutaneous SSc. In univariable logistic regression, Black race (odds ratio [OR] 15.63, 95% CI 6.6-37.20,

CONCLUSION: Perifollicular hypopigmentation is observed in …

Anakinra Treatment Efficacy In Reduction Of Inflammatory Biomarkers In Covid-19 Patients: A Meta-Analysis, Zunaira Naveed, Musharraf Sarwar, Zahid Ali, Danish Saeed, Khadija Choudhry, Azza Sarfraz, Zouina Sarfraz, Miguel Felix, Ivan Cherrez-Ojeda

Department of Paediatrics and Child Health

Introduction: Anakinra is being empirically considered for the treatment of COVID-19 patients. The aim is to assess the efficacy of anakinra treatment on inflammatory marker reduction, including c-reactive protein (CRP) concentrations, serum ferritin, and serum d-dimer levels.
Methods: Adhering to PRISMA 2020 statement guidelines, a systematic search was conducted across the following databases from December 2019 until January 10, 2022: PubMed/MEDLINE, Cochrane Central, Web of Science, Scopus, and EMBASE. The following keywords were employed: Anakinra, COVID*, SARS-CoV-2, inflammatory, CRP, D-dimer, Ferritin, hematological, laboratory, clinical, trials. The findings were collated and presented in a tabulated manner, and statistically analyzed using Review …

Telemedicine For Rheumatology Patients In Covid-19 Pandemic: Perspectives Of Patients, Muhammad Mehmood Riaz, Saad Bin Zafar Mahmood, Noreen Nasir

Section of Internal Medicine

Telemedicine use for patients with autoimmune rheumatic conditions during the pandemic mandates better characterization. We conducted a telephonic survey on patients who consulted rheumatologists remotely to determine their attitudes toward telemedicine. The study was conducted at Aga Khan University Hospital, a tertiary care hospital in Pakistan which is a lower-middle-income country. Descriptive analyses were performed. Fifty patients visited tele-rheumatology clinic, 35 (70.0%) were female with a mean (SD) age of 47.6 (18.1) years. Majority (27, 54.0%) learned about telemedicine from hospital website and outpatient clinic desk or helpline. Lack of examination was the biggest concern with teleconsultation (18, 22.2%). Most …

The Need For Space To Plan The Future For Spondyloarthritis, Mark Hwang, Michael H Weisman

Journal Articles

No abstract provided.

Down Syndrome-Associated Arthritis (Da): Diagnostic And Management Challenges., Jordan T. Jones, Jacqueline Kitchen, Nasreen Talib

Manuscripts, Articles, Book Chapters and Other Papers

Down syndrome (DS) is one of the most common birth defects in the United States, the most common genomic disorder of intellectual disability, and results from trisomy 21. This chromosome disorder causes an extensive, heterogenous phenotype that results in a broad presentation of symptoms that includes atlantoaxial instability, congenital heart defects, muscle hypotonia, hypothyroidism, hematologic disorders, recurrent infections, and autoimmune diseases. The autoimmune diseases are caused by immune system dysregulation that results in increased pro-inflammatory cytokines, along with other innate and adaptive immune system dysregulation. This is the likely cause of the increased risk of inflammatory arthritis or Down syndrome-associated …

Dermatologic Manifestations Of Rheumatic Disease: Cutaneous Manifestations Of Sarcoidosis, Sarah Shapiro, Sindhuja Koppu, Mavra Masood, Beth Rubinstein, Huzaefah Syed

Open and Affordable Course Content at VCU

Cutaneous Manifestations of Sarcoidosis is a Powerpoint based learning module that depicts fictional patient scenarios detailing sarcoidosis and erythema nodosum. It explores other cutaneous and non-cutaneous manifestations, diagnostic criteria, and medical evaluation of sarcoidosis and the possible etiologies, workup, treatment, and prognosis of erythema nodosum. Health and racial disparities experienced by patients with sarcoidosis are also highlighted and discussed. It takes approximately 25 minutes to complete. This module was created for use in the VCU School of Medicine M1 Movement block, Rheumatology and Dermatology sections.

It is part of the Dermatologic Manifestations of Rheumatic Disease project, which provides overviews of …