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          Labs @ NIAMS
          Juvenile Myositis Pathogenesis and Therapeutics Unit








Juvenile Myositis Pathogenesis and Therapeutics Unit


Hanna Kim, M.S., M.D. FacebookEmailPrintFacebookEmail
        Image
              Hero image for Juvenile Myositis Lab

      Overview

                                  Assistant Clinical Investigator
      
  


Hanna Kim, M.S., M.D.
      
  


            hanna.kim@nih.gov
      
                                                                        

  
   Research Areas (IRP Lab Groups)
          Autoimmunity,
          Clinical Research,
          Computational Biology,
          Immunology,
          Muscle Biology
                                
            Juvenile myositis (JM) is a rare, complex, multi-system condition in which inflammation of the muscles results in weakness as well as a characteristic skin rash in cases of juvenile dermatomyositis (JDM). The exact cause or pathogenesis of JM is not well understood but it is thought to result from a combination of multiple genetic and environmental risk factors. Treatment is generally empiric with prolonged and high-doses of immunosuppression medications including glucocorticoids, which carry significant adverse effects.Sixty to seventy percent of patients have a chronic disease course, and some are unable to achieve remission with currently available medications. In the long term, the disease often causes damage such as calcinosis and impaired function. Better markers to help monitor and predict the disease course and guide therapy are needed, as well as more targeted therapy options with better efficacy and fewer side effects.Our group studies dysregulated gene and protein expression from patients with JM to better understand dysregulated biology and the pathogenesis of the disease. An interferon response signature has been demonstrated in peripheral blood, muscle, and skin in dermatomyositis. To better understand the role of interferon in JDM, we have compared the peripheral interferon signature, or score, in JDM to autoinflammatory Mendelian interferonopathies (PMID 32252809, PMID 29638206). Janus kinase (JAK) inhibitors can target this interferon signaling pathway. Given the interferon signature, which is well described in JDM, and previous use of JAK inhibitors in Mendelian interferonopathies, we studied the use of baricitinib (a JAK inhibitor) in refractory JDM, demonstrating proof of concept in pharmacodynamic studies as well as clinical efficacy (PMID 32843325).These studies have highlighted how we have looked at particular pathways and utilized Mendelian disease to better understand pathogenesis and guide the selection of targeted therapy.Research FocusOur collaborative group studies are focused on better understanding the etiology and mechanisms of disease in juvenile myositis in order to optimize management and treatment. We are focused on:Elucidating the immunopathogenesis of the disease and potential predictors of treatment response by the discovery of novel biomarkers through transcriptomic, proteomic, and/or immunophenotyping analyses.Investigating novel treatments in small clinical trials to establish more targeted therapy with the goal of identifying safe treatments with greater efficacy and fewer side effects.
      

                                            Related NIAMS LabsPediatric Translational Research BranchMuscle Disease UnitClinical Research @ NIAMS
  Scientific Advances
      
  

      September 1, 2023Proteins Found in Urine Might Provide a Better Understanding of Juvenile Dermatomyositis

                                                      Core Research Facilities

Labs at the NIAMS are supported by the following state-of-the-art facilities and services:

Biodata Mining and Discovery
	Flow Cytometry
	Genomic Technology
	Laboratory Animal Care and Use
	Light Imaging
	Translational Immunology

                                    Staff
                            Image
              Members of Hanna Kim's Lab, 2021
                            
            **Hanna Kim, M.S., M.D.**Assistant Clinical Investigatorhanna.kim@nih.gov
    **Amy Kaneshiro, B.A.**Postbaccalaureate Fellowamy.kaneshiro@nih.gov
    **Ujana Zajmi, B.S.**Postbaccalaureate Fellowujana.zajmi@nih.gov
      

              
                            Former Lab Members
              Postbaccalaureate FellowsAdeline Chin (2020-2022)Pursuing M.D. at Cleveland Clinic Lerner College of Medicine, Case Western Reserve UniversityMegan Darrell (2020-2022)Pursing M.D./Ph.D. at Albert Einstein College of Medicine
            
                                                Image & Media Gallery
              
            Adeline Chin Presenting at DC Rheum Society Fellows Forum 2022
Adeline Chin Presenting at DC Rheum Society Fellows Forum 2022
        ×
      Adeline Chin pointing to a figure on her research poster.
        Adeline Chin pointing to a figure on her research poster.
    Megan Darrell Presenting at DC Rheum Society Fellows Forum 2022
Megan Darrell Presenting at DC Rheum Society Fellows Forum 2022
        ×
      Megan Darrell standing with her research poster.
        Megan Darrell standing with her research poster.
    JMPTU at DC Rheum Society Fellows Forum 2022
JMPTU at DC Rheum Society Fellows Forum 2022
        ×
      Adeline Chin, Hanna Kim and Megan Darrell standing in front of their research posters.
        Adeline Chin (left), Hanna Kim (center), and Megan Darrell (right) standing in front of their research posters.
    What Our Proteins Can Tell Us About Autoimmune Diseases
What Our Proteins Can Tell Us About Autoimmune Diseases
        ×
      What Our Proteins Can Tell Us About Autoimmune Diseases

The immune system is supposed to protect us from threats like bacteria and viruses, but in autoimmune diseases, it also attacks our own cells. Adeline Chin, a postbaccalaureate research fellow in the Intramural Research Program at the National Institutes of Health, is searching for proteins present at abnormally high or low levels in the blood of kids with a rare autoimmune illness. Her work could help reveal what causes their symptoms and lead to ways for researchers to more easily figure out if new treatments are effective.
    Dr. Hanna Kim pipetting samples in the lab.
Dr. Hanna Kim pipetting samples in the lab.
        ×
      Dr. Hanna Kim pipetting samples in the lab.
        Dr. Hanna Kim pipetting samples in the lab.
         Download High Res
    (7.65 MB)
    Dr. Hanna Kim performing a joint exam.
Dr. Hanna Kim performing a joint exam.
        ×
      Dr. Hanna Kim performing a joint exam.
        Dr. Hanna Kim performing a joint exam.
         Download High Res
    (5.98 MB)
            
                        
                                    Clinical Trials
              
              
            Active, not recruitingCompassionate Use Treatment Protocol I4V-MC-JAGA: Treatment of Conditions Expected to Benefit From JAK 1/2 Inhibition: CANDLE, CANDLE-Related Conditions, SAVI and Severe Juvenile DermatomyositisClinicalTrials.gov Identifier: NCT01724580The Requesting Physician/Investigator contacts Lilly when, based on their medical opinion, a patient meets the criteria for inclusion in the expanded access program.
    RecruitingStudies of Natural History, Pathogenesis, and Outcomes in Autoimmune and Inflammatory Diseases Including Juvenile DermatomyositisClinicalTrials.gov Identifier: NCT00059748Autoinflammatory multisystem diseases are a group of diseases that are characterized by recurrent episodes of systemic inflammation as well as organ specific inflammation that can involve the skin, eyes, joints, bones, serosal surfaces, inner ear, and brain. In this research protocol we seek to comprehensively evaluate affected patients clinically, genetically, immunologically, and endocrinologically. In addition we intend to evaluate longterm outcomes and biomarkers over the time of observations.
            
                                    Scientific Publications
              Selected Recent Publications
                  
      Efficacy of topical ruxolitinib for cutaneous dermatomyositis.

    Lanis A, Kim H, Lu S, Tsai WL, Kaneshiro A, Ehrlich A, Martin G, Schiffenbauer A, Shenoi S
        

                    JAAD Case Rep.
        
                    2024 Mar;
        
                    45().
        
                    doi: 10.1016/j.jdcr.2023.09.043
                            PMID: 38379881
              Updates on efficacy and safety janus kinase inhibitors in juvenile dermatomyositis.

    Kim H
        

                    Expert Rev Clin Immunol.
        
                    2024 Feb 1;
        
        
                    doi: 10.1080/1744666X.2024.2312819
                            PMID: 38299575
              Insights into juvenile myositis via engineered muscle.

    Kim H
        

                    Nat Rev Rheumatol.
        
                    2023 Sep;
        
                    19(9).
        
                    doi: 10.1038/s41584-023-01003-6
                            PMID: 37495869
              Improvement in Disease Activity in Refractory Juvenile Dermatomyositis Following Abatacept Therapy.

    Curiel RV, Nguyen W, Mamyrova G, Jones D, Ehrlich A, Brindle KA, Haji-Momenian S, Sheets R, Kim H, Jones OY, Rider LG, Abatacept in Dermatomyositis (AID) Trial Investigators
        

                    Arthritis Rheumatol.
        
                    2023 Jul;
        
                    75(7).
        
                    doi: 10.1002/art.42450
                            PMID: 36657109
              Performance of the 2016 ACR-EULAR Myositis Response Criteria in adult dermatomyositis/polymyositis therapeutic trials and consensus profiles.

    Saygin D, Kim H, Douglas C, Erman B, Wilkerson J, McGrath JA, Oddis CV, Lundberg IE, Amato AA, García-De La Torre I, Chinoy H, Fiorentino D, Chung L, Song YW, Miller FW, Ruperto N, Vencovsky J, Aggarwal R, Rider LG, International Myositis Assessment and Clinical Studies Group (IMACS)
        

                    Rheumatology (Oxford).
        
                    2023 Nov 2;
        
                    62(11).
        
                    doi: 10.1093/rheumatology/kead110
                            PMID: 36929923
              Performance of the 2016 ACR-EULAR myositis response criteria in juvenile dermatomyositis therapeutic trials and consensus profiles.

    Kim H, Saygin D, Douglas C, Wilkerson J, Erman B, Pistorio A, McGrath JA, Reed AM, Oddis CV, Bracaglia C, van Royen-Kerkhof A, Bica B, Dolezalova P, Ferriani VPL, Flato B, Bernard-Medina AG, Herlin T, Miller FW, Vencovsky J, Ruperto N, Aggarwal R, Rider LG, International Myositis Assessment and Clinical Studies Group (IMACS) and Paediatric Rheumatology INternational Trials Organization (PRINTO)
        

                    Rheumatology (Oxford).
        
                    2023 Nov 2;
        
                    62(11).
        
                    doi: 10.1093/rheumatology/kead111
                            PMID: 36929918
              Urine proteomics by mass spectrometry identifies proteins involved in key pathogenic pathways in patients with juvenile dermatomyositis.

    Morales M, Alayi TD, Tawalbeh SM, Sydenstricker AV, Spathis R, Kim H, Nagaraju K, Hathout Y, Rider LG
        

                    Rheumatology (Oxford).
        
                    2023 Sep 1;
        
                    62(9).
        
                    doi: 10.1093/rheumatology/kead033
                            PMID: 36661295
              Improvement in Disease Activity in Refractory Juvenile Dermatomyositis Following Abatacept Therapy.

    Curiel RV, Nguyen W, Mamyrova G, Jones D, Ehrlich A, Brindle KA, Haji-Momenian S, Sheets R, Kim H, Jones OY, Rider LG, Abatacept in Dermatomyositis (AID) Trial Investigators
        

                    Arthritis Rheumatol.
        
                    2023 Jul;
        
                    75(7).
        
                    doi: 10.1002/art.42450
                            PMID: 36657109
              Treatment escalation patterns to start biologics in refractory moderate juvenile dermatomyositis among members of the Childhood Arthritis and Rheumatology Research Alliance.

    Sherman MA, Kim H, Banschbach K, Brown A, Gewanter HL, Lang B, Perron M, Robinson AB, Spitznagle J, Stingl C, Syverson G, Tory HO, Spencer CH, Tarvin SE
        

                    Pediatr Rheumatol Online J.
        
                    2023 Jan 6;
        
                    21(1).
        
                    doi: 10.1186/s12969-022-00785-5
                            PMID: 36609397
              Updates on Juvenile Dermatomyositis from the Last Decade: Classification to Outcomes.

    Kim H, Huber AM, Kim S
        

                    Rheum Dis Clin North Am.
        
                    2021 Nov;
        
                    47(4).
        
                    doi: 10.1016/j.rdc.2021.07.003
                            PMID: 34635298
              Updates on interferon in juvenile dermatomyositis: pathogenesis and therapy.

    Kim H
        

                    Curr Opin Rheumatol.
        
                    2021 Sep 1;
        
                    33(5).
        
                    doi: 10.1097/BOR.0000000000000816
                            PMID: 34230439
              Janus kinase (JAK) inhibition with baricitinib in refractory juvenile dermatomyositis.

    Kim H, Dill S, O'Brien M, Vian L, Li X, Manukyan M, Jain M, Adeojo LW, George J, Perez M, Grom AA, Sutter M, Feldman BM, Yao L, Millwood M, Brundidge A, Pichard DC, Cowen EW, Shi Y, Lu S, Tsai WL, Gadina M, Rider LG, Colbert RA
        

                    Ann Rheum Dis.
        
                    2021 Mar;
        
                    80(3).
        
                    doi: 10.1136/annrheumdis-2020-218690
                            PMID: 32843325
              Expression of interferon-regulated genes in juvenile dermatomyositis versus Mendelian autoinflammatory interferonopathies.

    Kim H, Gunter-Rahman F, McGrath JA, Lee E, de Jesus AA, Targoff IN, Huang Y, O'Hanlon TP, Tsai WL, Gadina M, Miller FW, Goldbach-Mansky R, Rider LG
        

                    Arthritis Res Ther.
        
                    2020 Apr 6;
        
                    22(1).
        
                    doi: 10.1186/s13075-020-02160-9
                            PMID: 32252809
              Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases.

    de Jesus AA, Hou Y, Brooks S, Malle L, Biancotto A, Huang Y, Calvo KR, Marrero B, Moir S, Oler AJ, Deng Z, Montealegre Sanchez GA, Ahmed A, Allenspach E, Arabshahi B, Behrens E, Benseler S, Bezrodnik L, Bout-Tabaku S, Brescia AC, Brown D, Burnham JM, Caldirola MS, Carrasco R, Chan AY, Cimaz R, Dancey P, Dare J, DeGuzman M, Dimitriades V, Ferguson I, Ferguson P, Finn L, Gattorno M, Grom AA, Hanson EP, Hashkes PJ, Hedrich CM, Herzog R, Horneff G, Jerath R, Kessler E, Kim H, Kingsbury DJ, Laxer RM, Lee PY, Lee-Kirsch MA, Lewandowski L, Li S, Lilleby V, Mammadova V, Moorthy LN, Nasrullayeva G, O'Neil KM, Onel K, Ozen S, Pan N, Pillet P, Piotto DG, Punaro MG, Reiff A, Reinhardt A, Rider LG, Rivas-Chacon R, Ronis T, Rösen-Wolff A, Roth J, Ruth NM, Rygg M, Schmeling H, Schulert G, Scott C, Seminario G, Shulman A, Sivaraman V, Son MB, Stepanovskiy Y, Stringer E, Taber S, Terreri MT, Tifft C, Torgerson T, Tosi L, Van Royen-Kerkhof A, Wampler Muskardin T, Canna SW, Goldbach-Mansky R
        

                    J Clin Invest.
        
                    2020 Apr 1;
        
                    130(4).
        
                    doi: 10.1172/JCI129301
                            PMID: 31874111
              Treatment of anti-MDA5 autoantibody-positive juvenile dermatomyositis using tofacitinib.

    Sabbagh S, Almeida de Jesus A, Hwang S, Kuehn HS, Kim H, Jung L, Carrasco R, Rosenzweig S, Goldbach-Mansky R, Rider LG
        

                    Brain.
        
                    2019 Nov 1;
        
                    142(11).
        
                    doi: 10.1093/brain/awz293
                            PMID: 31603187
              Pharmacokinetics, Pharmacodynamics, and Proposed Dosing of the Oral JAK1 and JAK2 Inhibitor Baricitinib in Pediatric and Young Adult CANDLE and SAVI Patients.

    Kim H, Brooks KM, Tang CC, Wakim P, Blake M, Brooks SR, Montealegre Sanchez GA, de Jesus AA, Huang Y, Tsai WL, Gadina M, Prakash A, Janes JM, Zhang X, Macias WL, Kumar P, Goldbach-Mansky R
        

                    Clin Pharmacol Ther.
        
                    2018 Aug;
        
                    104(2).
        
                    doi: 10.1002/cpt.936
                            PMID: 29134648
              JAK1/2 inhibition with baricitinib in the treatment of autoinflammatory interferonopathies.

    Sanchez GAM, Reinhardt A, Ramsey S, Wittkowski H, Hashkes PJ, Berkun Y, Schalm S, Murias S, Dare JA, Brown D, Stone DL, Gao L, Klausmeier T, Foell D, de Jesus AA, Chapelle DC, Kim H, Dill S, Colbert RA, Failla L, Kost B, O'Brien M, Reynolds JC, Folio LR, Calvo KR, Paul SM, Weir N, Brofferio A, Soldatos A, Biancotto A, Cowen EW, Digiovanna JJ, Gadina M, Lipton AJ, Hadigan C, Holland SM, Fontana J, Alawad AS, Brown RJ, Rother KI, Heller T, Brooks KM, Kumar P, Brooks SR, Waldman M, Singh HK, Nickeleit V, Silk M, Prakash A, Janes JM, Ozen S, Wakim PG, Brogan PA, Macias WL, Goldbach-Mansky R
        

                    J Clin Invest.
        
                    2018 Jul 2;
        
                    128(7).
        
                    doi: 10.1172/JCI98814
                            PMID: 29649002
              Development of a Validated Interferon Score Using NanoString Technology.

    Kim H, de Jesus AA, Brooks SR, Liu Y, Huang Y, VanTries R, Montealegre Sanchez GA, Rotman Y, Gadina M, Goldbach-Mansky R
        

                    J Interferon Cytokine Res.
        
                    2018 Apr;
        
                    38(4).
        
                    doi: 10.1089/jir.2017.0127
                            PMID: 29638206
              Biologic therapies for refractory juvenile dermatomyositis: five years of experience of the Childhood Arthritis and Rheumatology Research Alliance in North America.

    Spencer CH, Rouster-Stevens K, Gewanter H, Syverson G, Modica R, Schmidt K, Emery H, Wallace C, Grevich S, Nanda K, Zhao YD, Shenoi S, Tarvin S, Hong S, Lindsley C, Weiss JE, Passo M, Ede K, Brown A, Ardalan K, Bernal W, Stoll ML, Lang B, Carrasco R, Agaiar C, Feller L, Bukulmez H, Vehe R, Kim H, Schmeling H, Gerstbacher D, Hoeltzel M, Eberhard B, Sundel R, Kim S, Huber AM, Patwardhan A, Pediatric Rheumatologist Collaborators
        

                    Pediatr Rheumatol Online J.
        
                    2017 Jun 13;
        
                    15(1).
        
                    doi: 10.1186/s12969-017-0174-0
                            PMID: 28610606
              Insights from Mendelian Interferonopathies: Comparison of CANDLE, SAVI with AGS, Monogenic Lupus.

    Kim H, Sanchez GA, Goldbach-Mansky R
        

                    J Mol Med (Berl).
        
                    2016 Oct;
        
                    94(10).
              Additive loss-of-function proteasome subunit mutations in CANDLE/PRAAS patients promote type I IFN production.

    Brehm A, Liu Y, Sheikh A, Marrero B, Omoyinmi E, Zhou Q, Montealegre G, Biancotto A, Reinhardt A, de Jesus AA, Pelletier M, Tsai WL, Remmers EF, Kardava L, Hill S, Kim H, Lachmann HJ, Megarbane A, Chae JJ, Brady J, Castillo RD, Brown D, Casano AV, Gao L, Chapelle D, Huang Y, Stone D, Chen Y, Sotzny F, Lee CC, Kastner DL, Torrelo A, Zlotogorski A, Moir S, Gadina M, McCoy P, Wesley R, Rother KI, Hildebrand PW, Brogan P, Krüger E, Aksentijevich I, Goldbach-Mansky R
        

                    J Clin Invest.
        
                    2016 Feb;
        
                    126(2).
        
                    doi: 10.1172/JCI86020
                            PMID: 26829627
              Activated STING in a vascular and pulmonary syndrome.

    Liu Y, Jesus AA, Marrero B, Yang D, Ramsey SE, Sanchez GAM, Tenbrock K, Wittkowski H, Jones OY, Kuehn HS, Lee CR, DiMattia MA, Cowen EW, Gonzalez B, Palmer I, DiGiovanna JJ, Biancotto A, Kim H, Tsai WL, Trier AM, Huang Y, Stone DL, Hill S, Kim HJ, St Hilaire C, Gurprasad S, Plass N, Chapelle D, Horkayne-Szakaly I, Foell D, Barysenka A, Candotti F, Holland SM, Hughes JD, Mehmet H, Issekutz AC, Raffeld M, McElwee J, Fontana JR, Minniti CP, Moir S, Kastner DL, Gadina M, Steven AC, Wingfield PT, Brooks SR, Rosenzweig SD, Fleisher TA, Deng Z, Boehm M, Paller AS, Goldbach-Mansky R
        

                    N Engl J Med.
        
                    2014 Aug 7;
        
                    371(6).
        
                    doi: 10.1056/NEJMoa1312625
                            PMID: 25029335 
                            View all Publications on PubMed
            
                                    News & Highlights
              
            NIAMS-Related Article | March 4, 2022
What Our Proteins Can Tell Us About Autoimmune Diseases
Adeline Chin, a postbaccalaureate research fellow, is searching for proteins present at abnormally high or low levels in the blood of kids with a rare autoimmune illness.
Read More



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        Last Updated: March 2024
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