Rheumatology Commons^™

Case Report And Literature Review Of An Atypical Polymyalgia Rheumatica And Its Management, Saad Bilal Ahmed, Saara Ahmad, Hanmei Pan

Department of Biological & Biomedical Sciences

Polymyalgia rheumatica (PMR) is a systemic inflammatory disease of the elderly population that increases in incidence as age advances. It is characterised by the sudden or sub-acute onset of symptoms affecting the shoulder and pelvic girdles, often accompanied by constitutional symptoms. Due to the lack of consensual diagnostic criteria and specific laboratory or radiological investigations for PMR, its diagnosis can be very challenging, particularly because it can be mimicked or masked by other geriatric syndromes. PMR responds well to glucocorticoid treatment, but if left untreated, can lead to morbidity and poor quality of life. We present the case of an …

Barriers To The Diagnosis, Care, And Management Of Pediatric Patients With Ehlers-Danlos Syndrome In The United States: A Qualitative Analysis., William R. Black, Lora L. Black, Jordan T. Jones

Manuscripts, Articles, Book Chapters and Other Papers

Objectives: Ehlers-Danlos Syndromes (EDS) are a family of heritable connective tissue diseases. Primary practitioners are capable of diagnosing and managing EDS; however, few are knowledgeable and comfortable enough to see patients with EDS, resulting in delays in diagnosis and care. This study explores the barriers physicians experience with diagnosing, managing, and caring for patients with EDS, and potential resolutions to those barriers. Methods: As part of a larger online study, providers (n = 107) in the United States were asked to specify "What information would improve (their) comfort" in diagnosing, caring for, and managing EDS via open-ended questions. Results …

Autoimmune Encephalitis Of Unknown Etiology, Elizabeth Cook, Giselle Ricoy

Research Colloquium

Encephalitis is the inflammation of the brain which can come about through various etiologies. Autoimmune encephalitis is a rare form that is most common among women and children. The clinical presentation can vary between patients with a constellation of symptoms including deficits in memory, cognition, seizures, abnormal movement, psychosis, and coma. Because of the variation in clinical presentation and the lack of specificity in imaging and laboratory findings, diagnosis and intervention are often delayed for months to years. These delays in diagnosis can have long term ramifications on patients especially pediatric patients whose neural pathways are still developing. In pediatric …

The Importance Of A Broad Differential Diagnosis: Hepatitis C Virus Associated Cryoglobulinemic Vasculitis, Shadi Jafari-Esfahani, Christine E. Loftis, Juan Naranjo, Emilia Dulgheru

Research Symposium

Introduction: Mixed cryoglobulinemia syndrome (MCS) is a systemic inflammatory syndrome affecting small-medium sized vessels due to the presence of type II or III cryoglobulins in the serum. MSC can manifest as systemic vasculitis with symptoms varying from weakness, arthralgia, palpable purpura, peripheral neuropathy, and renal involvement. The most common cause of MCS includes lymphoproliferative disorders, autoimmune diseases and viral infections, with hepatitis C virus (HCV) being the most common etiology.

Case Presentation: A 60-year-old lady was referred to our office for evaluation of rheumatoid arthritis. She reported history of bilateral thumb pain, bilateral knee pain associated with episodes of swelling, …

Generalized Lymphadenopathy As The Initial Presentation Of A Young Woman With Systemic Lupus Erythematous, Mery Bartl, Christine E. Loftis, Jose Gomez, Daniela Hernandez, Emilia C. Dulgheru

Research Symposium

Background: Generalized lymphadenopathy (LAP) refers to abnormal enlargement of more than two non-contiguous lymph node regions. There are various causes of LAP, including malignancy, infection, autoimmune disorders, medications, and iatrogenic causes. Obtaining a thorough history and physical examination is paramount in identifying the underlying etiology. Most of the time further investigation with laboratory and radiographic studies should be performed to identify the cause. Patients with high-risk features should undergo a biopsy for the diagnosis of malignancy.

Case presentation: A 36-year-old lady with a remote history of COVID-19 presented with complaints of orthopnea, cough, arthralgia, and left-sided abdominal pain for the …

A Whole Clotta Pain: A Case Of Ivc Thrombosis Presenting As Severe Abdominal Pain In A Patient With Anti-Phospholipid Syndrome, Mery Bartl, Christine E. Loftis, Josenny Rodriguez-Paez, Emilia C. Dulgheru

Research Symposium

Background: Anti-phospholipid syndrome (APS) is an immune-mediated condition characterized by the presence of antiphospholipid antibodies in the setting of venous and arterial thrombosis and or pregnancy loss [1]. APS can be a primary syndrome or can be associated with connective tissue diseases such as systemic lupus erythematosus. The goal of treatment in patients with APS is to decrease the risk of thromboembolic events and the standard of care is warfarin therapy. We describe a case of a 42-year-old gentleman with a history of APS on anticoagulation with apixaban who presented to the hospital with severe abdominal pain secondary to infrarenal …

That Which Keeps On Giving - A Case Of Class Iv Diffuse Proliferative Lupus Nephritis In A Hispanic Woman With Underlying Systemic Lupus Erythematosus, Josenny L. Rodriguez-Paez, Christine E. Loftis, Rosa White-Guedez

Research Symposium

Background: Glomerulonephritis is the primary cause of morbidity and mortality of systemic lupus erythematosus (SLE). Lupus nephritis is characterized by immune complex deposition in the mesangium leading to complement activation and hypocomplementemia. Studies show that up to 60% of adults with lupus develop renal involvement and it has been well established that Hispanic patients show poorer outcomes than Caucasians despite advances in treatment. Preserved kidney function with new-onset proteinuria should raise clinical suspicion for acute lupus nephritis. Further evaluation with a kidney biopsy is paramount in establishing a diagnosis, helping to define treatment strategy, and determining response to treatment.

Case …

Pericardial Effusions And Cardiac Tamponade In Hospitalized Systemic Sclerosis Patients: Analysis Of The National Inpatient Sample, Bikash Basyal, Waqas Ullah, Chris T. Derk

Abington Jefferson Health Papers

Introduction

Clinically significant pericardial effusions and cardiac tamponade in systemic sclerosis (SSc) patients is uncommon and the factors that contribute to progression of pericardial involvement in SSc patients have not been well established.

Methods

A review of the national inpatient sample database was performed looking SSc related hospitalizations between 2002 and 2019. Data was collected on patients with pericardial effusions and cardiac tamponade and analyzed to identify and describe patient characteristics and comorbidities.

Results

Out of a total of 523,410 SSc hospitalizations, with an overall inpatient mortality rate of 4.7% (24,764 patients), pericardial effusion was identified in 3.1% of all …

Biliary Dyskinesia In Stiff Person Syndrome: An Association Between Reduced Gaba Production And Gastroenteric Dysmotility, Scarlet Louis-Jean, Nirav Agrawal, Shire Chaudhry, Adrien Mazer

Journal of Community Hospital Internal Medicine Perspectives

Stiff person syndrome (SPS) and biliary dyskinesia are two rare but potentially debilitating conditions that can significantly impact quality of life. SPS is a rare neurological disorder characterized by muscle stiffness, rigidity, and muscle spasms that primarily affect the trunk and limbs and is associated with extra-axial manifestations involving the gastrointestinal tract. Biliary dyskinesia is a gastrointestinal disorder characterized by abnormal gallbladder emptying, leading to symptoms of intense abdominal pain, nausea, and vomiting. Despite their distinct clinical presentations, studies have suggested a possible connection between the two disorders. This link may be due to involvement of similar neurotransmitters and autoantibodies …

Treatment Of Knee Synovitis From Psoriatic Arthritis With Platelet Rich Plasma Injection, Nicolle Mazzei, Arielle Hall, Cody Clinton

Cooper Rowan Medical Journal

Psoriatic arthritis has been known to cause many inflammatory clinical features, including synovitis. In the past, synovitis has been primarily treated with NSAIDs, corticosteroid injections, and synovectomy when needed. This case report outlines a 49-year-old female with synovitis caused by psoriatic arthritis who did not respond to conventional treatment options and was apprehensive to surgery. She was offered a platelet rich plasma (PRP) injection as alternative therapy to a synovectomy. This case explores the use of PRP injections as a potential treatment modality for patients with synovitis caused by psoriatic arthritis. This patient was able to show improvement in her …

Hydroxychloroquine Induced Cardiomyopathy, Mohamed Salah Mohamed, Katrina Wojciechowski, Scott Feitell, Muhammad Osama, Anas Hashem, Jayesh Patel, Amir Mahmoud, Ali Abdelhay, Prakash Upreti, Soidjon Khodjaev

Advances in Clinical Medical Research and Healthcare Delivery

Hydroxychloroquine (HCQ) is one of the immunomodulatory medications used in treatment of autoimmune diseases. Rarely, HCQ can cause serious complications, such as cardiotoxicity. We present a rare case of HCQ-induced cardiomyopathy. 60-year-old female patient with a medical history of SLE on chronic HCQ therapy for 28 years, preexisting non-ischemic cardiomyopathy and heart failure with reduced ejection fraction for 7 years, and complete heart block status post pacemaker insertion presented with acute chest pain and severe weight loss. Patient underwent coronary angiogram that showed normal coronaries and right-sided heart catheterization that showed acute heart failure. Echocardiogram showed LVEF of 30% with …

Eosinophilic Granulomatosis With Polyangiitis (Churg Strauss Syndrome) – A Case Report, Rudrani Mukherjee, Bhanukumar M

Digital Journal of Clinical Medicine

Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg Strauss syndrome, is a rare small vessel vasculitis characterized by asthma, eosinophilia, and systemic involvement. This case report presents a 51-year-old man with a history of asthma, who presented with neurological symptoms including blurring of vision, bilateral ptosis, and altered sensorium. The patient had a previous history of cervical and lumbar spine surgeries for symptoms attributed to spondylosis. Further examination revealed hyperpigmented lesions on the limbs, wasting of small muscles, and foot drop.

Investigations showed eosinophilia and thrombocytosis on blood analysis, with bone marrow aspiration revealing eosinophilic and megakaryocytic hyperplasia. The …

Extrinsic Allergic Alveolitis: A Systematic Review Of Hla-Dr In Pigeon Breeder’S Disease, Dylan Thibaut, Ryan A. Witcher, Anitha Kunnath, James Toldi

Advances in Clinical Medical Research and Healthcare Delivery

Abstract

Introduction: Pigeon Breeder’s Pneumonitis (PBP) results due to a complex pathophysiology that includes exposure to avian antigens. Susceptibility has been linked to human leukocyte antigen (HLA) class II, though consensus has not been reached. The goal of this systematic review is to further elucidate the association between PBP and HLA-DR subtypes.

Methods: Databases utilized included PubMed, Google Scholar, ScienceDirect, and Cochrane Library. Inclusion required a minimum of three studies in English presenting HLA-DR alleles of PBP and control subgroups. Exclusion was due to insufficient data or non-feasible control groups. Forest plots were created for HLA-DR subtypes’ association …

Pustular Psoriasis And The Potential Therapeutic Usage Of An Il-36 Receptor Monoclonal Antibody, Jeannel T. Miclat, Shafik Habal

Research Day

Pustular psoriasis is an uncommon subtype of psoriasis that dramatically affects the quality of life of affected patients. Pustules can emerge anywhere along the trunk, limbs, soles, palms, and fingers, which debilitates the functionality of these appendages. Currently, there are no approved treatments for pustular psoriasis in the US; off-label usage of psoriasis vulgaris medications is usually prescribed. These treatments are insufficient for patients with difficult to manage or severe forms of pustular psoriasis. Psoriasis vulgaris biologic medications mainly target the IL-17 and IL-23 axis. However, novel clinical findings have demonstrated that pustular psoriasis’s central inflammatory axis depends on the …

Bone Marrow Transplant For Macrophage Activation Syndrome In Systemic Juvenile Idiopathic Arthritis, Shailly Gaur, Michael J. Holland, J Allyson Hays, Ibrahim A. Ahmed

Research Days

Background

The recognition of macrophage-activation-syndrome (MAS) in the setting of systemic-juvenileidiopathic-arthritis (sJIA) is complex, but a necessary distinction given emerging treatment options. With overlapping clinical manifestations including fever, end-organ involvement and lymphadenopathy, there is a focus on laboratory values to differentiate MAS from a sJIA flare. Once parameters are met, multiple treatment modalities can be considered, as monotherapy or in combination, including steroids, immunosuppression, chemotherapy, intravenous immunoglobulin, and cytokine-directed biologics. Given the long-term morbidity and mortality associated with progressive inflammation and immune dysregulation due to MAS with sJIA, it is imperative to regain control over this complication. However, these treatments …

Maackia Amurensis Seed Lectin (Masl) Increases Movement Velocity Of Mice With Tnfα Induced Rheumatoid Arthritis, Amanda A. Greenspan, Kelly L. Hamilton, Alan J. Shienbaum, Bradford Fischer, Andrea Bottaro, Gary S. Goldberg

Rowan-Virtua Research Day

Up to 70 million people around the world suffer from rheumatoid arthritis. Current treatment options have varied efficacy and can cause unwanted side effects. New approaches are needed to treat this condition. Sialic acid modifications on chondrocyte receptors have been associated with arthritic inflammation and joint destruction. The transmembrane mucin receptor protein podoplanin (PDPN) has been identified as a functionally relevant receptor that presents extracellular sialic acid motifs. PDPN signaling promotes inflammation and invasion associated with arthritis and, therefore, has emerged as a target that can be used to inhibit arthritic inflammation. Maackia amurensis seed lectin (MASL) can target PDPN …

A Case Of Double-Positive Anti-Gbm And Mpo-Anca Vasculitis With No Pulmonary Involvement, Deonnie W. Huang, Patrick May, Thanh Nguyen

Research Day

Introduction

Anti-glomerular basement membrane (anti-GBM) disease is a small vessel vasculitis caused by autoantibodies directed at the glomerular and alveolar basement membranes. Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis is pauci-immune with no immune deposits. Both vasculitides can lead to precipitation of rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage. We report a case of a 58 year-old male who presented with acute renal failure with double positive anti-GBM and myeloperoxidase-anti neutrophil cytoplasmic antibodies (MPO-ANCA) but no pulmonary involvement.

Case Presentation

Patient is a 58-year-old male with a past medical history of nicotine dependence who presented to the hospital with abnormal blood work. …

Adipocytes And Innate Immunity In Systemic Sclerosis, Nancy Wareing

Dissertations & Theses (Open Access)

Systemic sclerosis (SSc; scleroderma) is a chronic systemic autoimmune and connective tissue disorder characterized by vasculopathy, autoimmune phenomena, and widespread fibrosis. Skin thickening and tightening is the cardinal feature of SSc and is responsible, in part, for the considerable morbidity of this disease. There are currently no targeted treatments for skin manifestations in SSc, primarily due to our fragmented understanding of its pathophysiologic mechanisms. In PART I, we report a previously unappreciated link between aberrant expression of the developmental gene sine oculis homeobox homolog 1 (SIX1) in skin-associated adipocytes in SSc skin and the early loss of dermal white adipose …

Bone Marrow Transplant For Macrophage Activation Syndrome In Systemic Juvenile Idiopathic Arthritis​, Shailly Gaur, Michael J. Holland, J Allyson Hays, Ibrahim A. Ahmed

Posters

7yo w/ systemic juvenile idiopathic arthritis who developed macrophage activation syndrome and interstitial lung disease non-responsive to steroids, immunosuppression, and directed biologics with significant adverse reactions who required chemotherapy and eventual bone marrow transplant leading to complete resolution of symptoms.

Antibodies For Β2-Microglobulin And The Heavy Chains Of Hla-E, Hla-F, And Hla-G Reflect The Hla-Variants On Activated Immune Cells And Phases Of Disease Progression In Rheumatoid Arthritis Patients Under Treatment, Mepur H. Ravindranath, Narendranath M. Ravindranath, Carly J. Amato-Menker, Fatiha El Hilali, Senthamil R. Selvan, Edward J Filippone, Luis Eduardo Morales-Buenrostro

Department of Medicine Faculty Papers

Rheumatoid arthritis (RA) is a progressive, inflammatory, autoimmune, symmetrical polyarticular arthritis. It is characterized by synovial infiltration and activation of several types of immune cells, culminating in their apoptosis and antibody generation against "altered" autoantigens. β2-microglobulin (β2m)-associated heavy chains (HCs) of HLA antigens, also known as closed conformers (Face-1), undergo "alteration" during activation of immune cells, resulting in β2m-free structural variants, including monomeric open conformers (Face-2) that are capable of dimerizing as either homodimers (Face-3) or as heterodimers (Face-4). β2m-free HCs uncover the cryptic epitopes that can elicit antibodies (Abs). We report here the levels of IgM and IgG Abs …

Graves' Disease And Major Histocompatibility Complex Class Ii: A Meta-Analysis Of Hla-Dq And Hla-Drb1, Dylan Thibaut, Connor Sweeney, Shannon South, Mohamed Hussein

Advances in Clinical Medical Research and Healthcare Delivery

Background: Human leukocyte antigen (HLA) class II has shown potential in determining prognosis, understanding medication reactions, and predicting onset of Graves’ disease. The aim of this study is to further investigate the association between Graves’ disease and HLA class II, specifically HLA-DQ and HLA-DR, via meta-analysis to find HLAs that can be further examined for prognostic reasons.

Methods: Statistical analysis was performed to determine if variants of HLA-DQA1, HLA-DQB1, or HLA-DRB1 were associated with significantly altered odds of Graves’ disease. A minimum of three studies pertaining to a particular HLA was required for inclusion. Studies were excluded if …

Rheumatoid Vasculitis Presenting With Incidental Renal Infarcts: Case Report And Literature Review, Fatima Halilu, Howard Hauptman

Journal of Community Hospital Internal Medicine Perspectives

Rheumatoid vasculitis is a rare and late complication of rheumatoid arthritis, affecting small to medium sized vessels. Inflammation in the vessel wall produces an occlusive vasculopathy with downstream infarction. We present the case of a 70-year-old female with long standing stable RA status post recent COVID-19 vaccination admitted with community acquired Legionnaires disease found to have an incidental renal infarct on chest imaging without a clear etiology. She improved from a respiratory standpoint but then had a fever spike with repeat imaging showing a second renal infarct and CT angiography showing extensive beading with aneurysmal dilation in the bilateral hepatic …

It Is Not Pneumocystis Jiroveci (Pcp), It Is Cyclophosphamide-Induced Pneumonitis, Mery Bartl, Yilen Karen Ng-Wong, Blesset Alexander, Jose Gomez Casanovas, Josenny L. Rodriguez-Paez, Andres Suarez, Christine Loftis

School of Medicine Publications and Presentations

Cyclophosphamide (CYC) is an immunosuppressive medication used to treat life-threatening complications of various rheumatic diseases like vasculitis and systemic lupus erythematosus. A rare side effect of this medication is pneumonitis, which occurs in less than 1% of patients. We describe a case of an 83-year-old woman with a past medical history of microscopic polyangiitis, who presented with progressive dyspnea at rest, exacerbated on exertion, and associated with orthopnea that was attributed to CYC-induced pneumonitis. Three months before this presentation, the patient was diagnosed with antineutrophil cytoplasmic antibodies (ANCA)-positive pauci-immune crescentic and necrotizing glomerulonephritis and started on CYC. On admission, a …

Rapid Onset Hydralazine-Induced Antineutrophil Cytoplasmic Antibody (Anca) Associated Vasculitis Presenting With Hemoptysis And Kidney Failure: Case Report, Rachel E. Easter, Andinet Gizaw, Natalie Klein

Internal Medicine Publications

Hydralazine-induced vasculitis can be challenging to recognize and diagnose as presenting symptoms vary and can mimic other conditions or diseases, however, swift intervention and treatment is key in halting progression of the disease and providing patients with the best possible outcomes. A 71-year-old African American female presented to the emergency department with weakness, fatigue, anemia, blood-streaked sputum, fever, chills, and severe myalgias. Hydralazine was prescribed to treat resistant hypertension eight months prior to presentation. Hydralazine was discontinued on hospital day (HD) 4. Pertinent laboratory values showed elevated inflammatory markers, positive antinuclear antibody (ANA), negative rheumatoid factor (RF), normal complement levels, …

Dermatologic Manifestations Of Rheumatic Disease: Cutaneous Manifestations Of Vasculitides, Julianna S. Kang, Julia Nunley, Tiffany Ho, Mavra Masood, Fnu Nutan, Beth Rubinstein

Open and Affordable Course Content at VCU

Cutaneous Manifestations of Vasculitides is a Powerpoint based learning module that begins with the definition of terms and findings often found in vasculitis, followed by a review of several systemic vasculitides, highlighting how characteristic cutaneous lesions may be predictive of the vessel of involvement as well as the associated histologic changes. Moreover, significant clinical and laboratory findings utilized to make the correct diagnosis will be emphasized. This module is not meant to be an exhaustive review of vasculitis, but to serve as an introduction to understanding disease processes and how systemic vasculitis and skin findings interrelate.

It is part of …

Dermatologic Manifestations Of Rheumatic Disease: Malignant Cutaneous Tumors, Divya Shan, Julianna S. Kang, Allison Cinats, Julia R. Nunley, Beth Rubinstein

Open and Affordable Course Content at VCU

Malignant Cutaneous Tumors is a Powerpoint-based learning module that focuses on skin cancers. It begins with the definition of terms and findings, followed by a review of several clinical cases. The goal of the module is to provide the students with the ability to differentiate the most common types of skin cancer and precancerous lesions and gain an awareness of cutaneous lymphomas. This module will include images and clinical findings from diverse skin tones to prepare students to identify cutaneous findings in a heterogeneous patient population. This module is not a comprehensive review of skin cancers but an introduction to …

Why A High Titer Rheumatoid Factor And Joint Pain Should Not Automatically Translate Into A Diagnosis Of Rheumatoid Arthritis, Saba Ashraf, Julio Gonzalez-Paoli

West Florida Division Research Day 2023

No abstract provided.

An Iceberg I Can’T Handle: A Qualitative Inquiry On Perceptions Towards Paediatric Rheumatology Among Healthcare Workers In Kenya, Angela Migowa, Sasha Bernatsky, Anthony Ngugi, Helen E. Foster, Peter Muriuki, Adelaide Lusambili, Stanley Luchters

Paediatrics and Child Health, East Africa

Background: Delay in diagnosis and access to specialist care is a major problem for many children and young people with rheumatic disease in sub-Saharan Africa. Most children with symptoms of rheumatic disease present to nonspecialists for care. There is an urgent need to understand and scale-up paediatric rheumatology knowledge and skills amongst non-specialist healthcare workers to promote early diagnosis, prompt referral, and management.

Purpose: We evaluated the knowledge, attitudes and practices towards diagnosis and care of paediatric rheumatology patients among health care workers in Kenya.

Methods: We conducted 12 focus group discussions with clinical officers (third-tier community health …

Improving Recombinant Zoster Vaccination Rates In Patients Receiving Biologic Therapy, Kathlyn Camargo, Taylor Kann, Marilyn Mosquera, Ravi Shahu, Ariail Schmitz, Kathleen Mccabe, Ashwini Komarla

North Florida Division Research Day 2023

No abstract provided.

Metformin-Induced Giant Cell Arteritis: An Uncommon Case Of Drug-Induced Vasculitis, Christine Angeles, Wendy Lee

South Atlantic Division Research Day 2023

No abstract provided.