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Pediatric Rheumatology 2018 Annual Report

Division Introduction

Under the direction of Marilynn G. Punaro, M.D., Professor and Master of the Fashena College, the Division of Pediatric Rheumatology at UT Southwestern Medical Center serves as a major resource for pediatric rheumatology in North Texas. In the United States there are approximately 300 board-certified pediatric rheumatologists, and five of those are at UT Southwestern. The Division receives referrals from all other children’s hospitals in the Dallas – Fort Worth metroplex, as well as more distant areas.

The Division operates the largest and longest established multispecialty clinic for children with rheumatic diseases in the region. In the rheumatology clinics at Texas Scottish Rite Hospital for Children (TSRHC), Scottish Rite for Children Frisco Campus, and Children’s Medical Center Dallas, diseases managed include juvenile idiopathic arthritis, systemic lupus erythematosus, juvenile dermatomyositis, and scleroderma. Faculty also have an interest in the diagnosis and management of a variety of rarer rheumatic diseases, including systemic vasculitis and auto-inflammatory conditions.

The Division is actively involved in both translational and clinical research endeavors directed toward improving pathophysiologic understanding, treatment options, and outcomes in a variety of pediatric rheumatologic disorders.

Pediatric Rheumatology has a significant commitment to medical education, offering a rheumatology elective for pediatric residents and fourth-year medical students. The fellowship training program is one of the only 36 ACGME-accredited programs for pediatric rheumatology; it has continuously trained fellows from all over the world for more than 30 years.

Faculty

In addition to Dr. Punaro, the Division has two full-time and two part-time faculty members, and five fellows.

Julie Fuller, M.D. Assistant Professor

Lorien Nassi, M.D. Assistant Professor

Katie Stewart, M.D. Assistant Professor

Tracey Wright, M.D. Associate Professor

Marilynn G. Punaro, M.D. Professor, Division Chief

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Pediatric Rheumatology 2018 Annual Report

Honors / Awards Best Doctors/Pediatric Specialists in Dallas, D Magazine

Julie Fuller

Lorien Nassi

Marilynn Punaro

Katie Stewart

Tracey Wright

Julie Fuller

Texas Rising Stars 2018, Texas Monthly

Marilynn Punaro

Distinguished Clinician Scholar Award of the American College of Rheumatology

Texas Super Doctor, Texas Monthly

Best Doctors in America

Katie Stewart

Texas Rising Stars 2018, Texas Monthly

Tracey Wright

Promotion to Associate Professor

Invited Lectures

Tracey Wright

Banner University, Tucson, AZ, April 2018

o “Moving Towards Personalized Medicine in

Pediatric Systemic Lupus Erythematosus”

Lupus Foundation of America Symposium, Dallas,

TX, December 2018

o “Pediatric Lupus”

Education and Training

The Division of Pediatric Rheumatology has a significant commitment to medical education and is actively involved in the teaching of fourth-year medical students and pediatric residents, in addition to its fully-accredited Pediatric Rheumatology Fellowship Program. Trainees in other departments also spend time in the rheumatology clinics.

Medical Students

Third-year medical students rotate through the arthritis clinic during their pediatric rotation, and an elective is available for fourth-year students. Tracey Wright, M.D., preceptor for this elective, and the other pediatric rheumatology faculty teach students to:

Pediatric Rheumatology Faculty L-R: Katie Stewart, Tracey Wright, Julie Fuller, Lorien Nassi

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Pediatric Rheumatology 2018 Annual Report

Develop a working differential diagnosis of arthritis in childhood

Learn a complete joint examination and the physical evaluation of rheumatologic patients

Examine and discuss patients from all major collagen vascular disease categories: juvenile arthritis, dermatomyositis, scleroderma, and systemic lupus erythematosus (SLE)

Residents

The Division provides the major instruction to pediatric residents in the evaluation of the musculoskeletal system and the assessment of the rheumatic diseases. Division faculty provide direct teaching for pediatric residents in the regular departmental clinical conferences, as well as part of the inpatient consultation service. Didactic teaching for the pediatric residents includes, but is not limited to:

Differential diagnosis of arthritis in childhood

Juvenile idiopathic arthritis

Systemic lupus

Juvenile dermatomyositis

Evaluation of musculoskeletal pain in children

A rheumatology elective is available for pediatric residents. Dr. Wright is the Course Director for elective; the objectives of this elective are to:

Develop a working differential diagnosis of arthritis in childhood

Learn a complete joint examination and the physical evaluation of rheumatologic patients

Examine and discuss patients from all major collagen vascular disease categories: juvenile arthritis, dermatomyositis, scleroderma, and systemic lupus erythematosus (SLE)

The residents see outpatients in the clinics under the supervision of the division faculty. A notebook of selected articles about rheumatic disease, including original material, has been developed as curriculum for the elective. Residents are encouraged to attend clinical conferences which offer didactic teaching for the fellows.

Fellows

The Division of Pediatric Rheumatology at UT Southwestern has one of only 36 ACGME-accredited fellowship training programs in pediatric rheumatology in the United States.

Fellows develop competence in the diagnosis and management of rheumatic diseases in children and gain a background in laboratory techniques of basic immunology and molecular biology as well as experience and education in application of the scientific method to clinical and laboratory research.

The large and varied patient population available at Children’s Medical Center Dallas and Texas Scottish Rite Hospital for Children (TSRHC) is extensive. The Arthritis Clinic at TSRHC is one of the longest established pediatric rheumatology clinics in the country and is the major regional referral center for North Texas.

Pediatric Rheumatology Fellows

L-R: Mailan Nguyen, Elizabeth Sloan, Vy Do, Simrat Morris

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Pediatric Rheumatology 2018 Annual Report

As required by the American Board of Pediatrics (ABP), all trainees spend a portion of their time involved in research. The Division collaborates with basic and translational scientists at TSRH and UTSW. Lab activities include journal club, work in progress and seminars with invited speakers. Research projects are tailored to the interests and capabilities of the individual trainees. Ongoing clinical trials and the opportunity to do clinical research are also available.

The three-year Pediatric fellowship training program aims to provide individuals with sufficient background to pursue a career of independent research, teaching and managing patients with a wide variety of pediatric rheumatic diseases.

Tracey Wright, M.D., is the Program Director for the Pediatric Rheumatology Fellowship Training Program. All other pediatric rheumatology faculty are actively involved in training the pediatric rheumatology fellows.

Current Fellows

Simrat Morris, M.D.

4th Year Fellow

MaiLan Nguyen, M.D.

3rd Year Fellow

Nicole Bitencourt, M.D.

3rd Year Fellow Med/Peds

Vy Do, D.O., M.P.H.

2nd Year Fellow

Elizabeth Sloan, M.D.

1st Year Fellow

Research Activities

All division faculty are members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA), which is dedicated to collaborative multicenter research on the cause and treatment of childhood rheumatic disease. They have participated in multiple clinical research studies including:

Biologic therapies for treatment of resistant arthritis

Effects of nutrition on systemic lupus

Comparative effectiveness trials in localized scleroderma and systemic juvenile arthritis

A large and varied patient population has provided the ability to conduct studies in several areas, and faculty have been involved in clinical research projects involving clinical trials of new therapies, as well as multicenter studies in pathogenesis and epidemiology of childhood rheumatic diseases.

They also have been involved in translational research, helping to make a bridge between the basic science researchers in pediatric collagen vascular diseases and the patients. Tracey Wright M.D. is the Principal Investigator for the Clinical and Sample Core of the NIAMS 1P50AR070594-01 Center for Lupus Excellence.

2018 Graduating Fellow, Keiji Akamine, with Dr. Tracey Wright

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Pediatric Rheumatology 2018 Annual Report

Clinical Activities

The Division offers expert evaluation, counseling, and treatment of rheumatic diseases found in children, which include the many forms of child arthritis, such as juvenile idiopathic arthritis, formerly known as juvenile rheumatoid arthritis. Faculty also manage children with lupus, juvenile dermatomyositis, morphea/scleroderma, vas-culitis, and a number of other conditions.

Clinical services are offered at Children's Medical Center Dallas, Scottish Rite for Children Frisco Campus, and at Texas Scottish Rite Hospital for Children’s Arthritis Clinic, which is one of the longest-established pediatric rheumatology clinics in the country and the major referral center for North Texas.

Between the two hospitals, Division faculty see more than 3,750 outpatient visits per year in eighteen clinics per week. In 2018, faculty saw 136 inpatient consultations and conducted inpatient rounds daily. There are more than 830 new patients per year. Trainees see and manage multiple patients of each major connective tissue disease and are exposed to rarer disorders.

Services provided by UT Southwestern pediatric rheumatology physicians include:

Evaluation and treatment of children with suspected rheumatic diseases

Physical and occupational therapy evaluations and recommendations

Coordination of team care, including ophthalmology, dental, and dietary counseling

Education about specific rheumatic conditions

Psychosocial support

Patient Statistics

Texas Scottish Rite Hospital 2017 2018 Outpatient Visits 2,787 2,580

New Outpatient Visits 366 407

Infusions 469 501

Children’s Medical Center Dallas 2017 2018

Outpatient Visits 1,850 1,178

New Outpatient Visits 737 427

Inpatient Consults 541 136

Dr. Julie Fuller with patient Aathyleia Tapia

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Pediatric Rheumatology 2018 Annual Report

Grant Support

Tracey Wright

Grantor: National Institute of Arthritis and Musculoskeletal and Skin Diseases (1P50AR070594-01) Title of Project: Center for Lupus Research Role: Principal Investigator for the Clinical and Sample Core Dates: 09/2016 – 08/2021

Grantor: Swedish Orphan Biovitrum Title of Project: A Study to Evaluate Efficacy and Safety of Anakinra in the Treatment of Still’s Disease Role: Site Principal Investigator Dates: 09/2017 – Present

Peer-Reviewed Publications

1. Arthur VL, Shuldiner E, Remmers EF, Hinks A, Grom AA, Foell D, Martini A, Gattorno M, Özen S, Prahalad S, Zeft AS, Bohnsack JF, Ilowite NT, Mellins ED, Russo R, Len C, Oliveira S, Yeung RSM, Rosenberg AM, Wedderburn LR, Anton J, Haas JP, Rösen-Wolff A, Minden K, Szymanski AM; INCHARGE Consortium (Punaro MG), et al. IL1RN Variation Influences Both Disease Susceptibility and Response to Recombinant Human Interleukin-1 Receptor Antagonist Therapy in Systemic Juvenile Idiopathic Arthritis. Arthritis Rheumatol. 2018 Aug;70(8):1319-1330. PMID: 29609200

2. Cooper JC, Rouster-Stevens K, Wright TB, et al. Pilot study comparing the childhood arthritis and rheumatology

research alliance consensus treatment plans for induction therapy of juvenile proliferative lupus nephritis. Pediatr Rheumatol Online J. 2018 Oct 22;16(1):65. PMID: 30348175

3. Drobysheva A, Fuller J, Pfeifer CM, Rakheja D.Orbital Granulomatosis With Polyangiitis Mimicking IgG4-Related Disease

in a 12-Year-Old Male. Int J Surg Pathol. 2018 Aug;26(5):453-458. PMID: 29366363

4. Hinks A, Marion MC, Cobb J, Comeau ME, Sudman M, Ainsworth HC, Bowes J; Juvenile Idiopathic Arthritis Consortium for Immunochip, Becker ML, Bohnsack JF, Haas JP, Lovell DJ, Mellins ED, Nelson JL, Nordal E, Punaro M, et al. Brief Report: The Genetic Profile of Rheumatoid Factor-Positive Polyarticular Juvenile Idiopathic Arthritis Resembles That of Adult Rheumatoid Arthritis. Arthritis Rheumatol. 2018 Jun;70(6):957-962. PMID: 29426059

5. Li SC, Li X, Pope E, Stewart K, Higgins GC, Rabinovich CE, O'Neil KM, Haines KA, Laxer RM, Punaro M, et al. New

Features for Measuring Disease Activity in Pediatric Localized Scleroderma. J Rheumatol. 2018 Dec;45(12):1680-1688. PMID: 30219769

6. Mansour M, Liy Wong C, Zulian F, Li S, Morishita K, Yeh EA, Stewart K, Laxer RM, Pope E. Natural history and extracutaneous involvement of congenital morphea: Multicenter retrospective cohort study and literature review. Pediatr Dermatol. 2018 Nov;35(6):761-768. PMID: 30187959

7. Rubinstein TB, Mowrey WB, Ilowite NT, Wahezi DM; Childhood Arthritis and Rheumatology Research Alliance INVESTIGATORS (Punaro M). Delays to Care in Pediatric Lupus Patients: Data From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry. Arthritis Care Res (Hoboken). 2018 Mar;70(3):420-427. PMID: 28544820

8. Weiss PF, Xiao R, Brandon TG, Pagnini I, Wright TB, et al. Comparative Effectiveness of Tumor Necrosis Factor Agents

and Disease-modifying Antirheumatic Therapy in Children with Enthesitis-related Arthritis: The First Year after Diagnosis. J Rheumatol. 2018 Jan;45(1):107-114. PMID: 28916542