Rheum-inations For the physical therapist All you wanted to know about rheumatology and more! Susan Shenoi MD, MS, RhMsUS Assistant Professor Clinical Director Seattle Children s Hospital 10-2017 Objectives Case based learning Recognize patterns of joint pain Recognize signs of arthritis Recognize Juvenile Idiopathic Arthritis Categories Medications used Complications Impact exercise on child Brief overview of Juvenile dermatomyositis (JDM) Identify resources for further help Disclosures Pediatric Rheumatology pearls No financial disclosures Will reference off label use of drugs Disclaimer I am not kind enough, not patient enough, not skilled enough..to ever do what you do! Thank you for all that you do to help our kids! Recognize Patterns of Joint Pain
ANA leg pain 3 months activity rest Exam normal Labs - not needed If done normal Or + ANA (1:80) Inflammatory AM stiffness/ Gelling Pain better with activity Fatigue +/- Swelling/ Warmth/ Decreased ROM present Examples: JIA, Inflammatory bowel disease related arthritis Mechanical Usually no/ minimal am stiffness Worsening pain with increased activity Lack systemic symptoms like fatigue Swelling/ Warmth/ Decreased ROM +/- Examples: hypermobility, tendonitis, osteoarthritis* ANA body pain 3 months activity rest Pain all day long Pain with light touch Exam normal except for allodynia Labs - normal Or + ANA (1:80) * Seen more commonly in adults ANA leg pain 3 months activity rest such as after sitting for long time Exam shows swollen warm joint(s) that are not moving well Labs ESR and CRP are high Or + ANA (1:640) Central Pain Sensitization Prolonged AM stiffness Pain all over, several days (months/ years) Increasing pain with activity Systemic symptoms, fatigue +, non-restorative sleep Tender points/ Allodynia/ Hyperalgesia Joint exam usually normal no swelling, warmth, normal ROM
Rheumatology Pearl #1 Pattern recognition 3 patterns to joint pain: Inflammatory Mechanical ANA leg pain 3 months Waking up at night with pain Fevers on and off Exam arthritis of knee Labs ESR 30 CRP 2.5 CBC platelet 90K WBC 3 K/mm3 Central Pain Sensitization Rheumatology Pearl #2 Arthritis Rheumatology Recognize signs of Arthritis Juvenile arthritis is a diagnosis of exclusion Must rule out infection, malignancies, metabolic, mechanical etc Arthritis = Juvenile Arthritis False arthritis can be secondary to many causes infection, cancers etc Arthritis Rheumatology Malignancy may mimic JIA 21-33% of ALL present with bone pain 62% have musculoskeletal symptoms Examples leukemia, lymphoma, neuroblastoma, localized bone tumors (Ewing's) AVOID STEROIDS TILL DIAGNOSIS CLEAR Jones OY, Pediatrics, 2006
When do you suspect it! pain at rest, at night, or out of proportion back pain, bone pain, bone tenderness atypical fevers night sweats weight loss Useful studies low counts (>2 cell lines down) lactate dehydrogenase (LDH), uric acid Imaging (CT chest, abdomen mass/ lymph nodes) BM/ LN biopsy Right hip arthritis/ synovitis Rheumatology referral ROM, pain right hip warmth swelling both ankles Enthesitis (inflammation. tendons, muscle insertion to bone) Plantar fasciitis Diagnosis JIA - Enthesitis related arthritis (ERA) Arthritis = inflammation of joints Recognize Juvenile Arthritis True red, warm, swollen, decreased range of motion Shenoi S. JIA Pediatr Rev. 2017 May;38(5):221-232. Vignette DR 7 yr boy limp for12 months Saw orthopedic 8/15 - X-ray foot and ankle normal - Told limp was behavioral Second opinion - orthopedic 11/15 Decreased range of motion (ROM) hip Inflammatory markers CRP normal (<0.8) ESR 28 (0-10) Blood counts - CBC normal Bone scan normal MRI ankle and pelvis ordered Arthritis is only for old people False kids get arthritis too, CDC 1 in 250 kids have arthritis
Rheumatoid Arthritis Adult Juvenile Idiopathic Arthritis < 16 years > 6 weeks duration No known cause Petty R et al. ILAR Classification Edmonton J. Rheum. 2001 Arthritis in Children JIA is due to vitamin deficiency False we don t know what causes it? Complex Genetic Trait Gene-Environmental Factors Juvenile Arthritis = JCA = JRA = JIA Pathogenesis True one disease many names Other JIA Environment Genes
ILAR Classification International League of Associations for Rheumatology 7 mutually exclusive categories: Systemic arthritis Oligoarthritis (persistent, extended) Polyarthritis (RF +)* Polyarthritis (RF -) Psoriatic arthritis Enthesitis-related arthritis Undifferentiated arthritis Petty R et al. ILAR Classification Edmonton J. Rheum. 2001 Prakken B et al. Lancet 2011 Chronic inflammation: imbalance between mediators Chronic inflammation: Imbalance between mediators TNF IL-1 IL-8 IL-12 IFN IL-1Ra TGF IL-4/IL-13 IL-10 Categories of JIA All juvenile arthritis is the same False 7 categories of JIA different presentations Oligoarticular 4 joints Polyarticular > 4 joints RF(-) Extended oligo > 4 joints RF(+) JIA Systemic-onset Psoriatic arthritis Enthesitis-related Undifferentiated! Image courtesy: Kristin Hayward
ANA 3 yr old girl Parents noticed her to be walking limp for 3 months Preschool teachers - she limps too ESR and CRP are normal Polly 2yr girl difficulty running, cranky in morning, swollen fingers, knees, ankles Labs - CRP 15.3 ESR 63 WBC 11.1 HCT 28 Platelet 472 ANA negative RF negative Oligoarticular JIA 40-60% JIA Females 3:1 1-3 years < 5 joints knee, ankle, elbow 70% ANA + 30% asymptomatic uveitis Labs often normal ESR CRP CBC Polyarticular JIA 30-40% JIA Females > 4 joints large and small joints C-Spine, TM joint RF (any age) RF + (10%) usually adolescence 30% ANA Oligoarticular JIA Criteria Exclusions 1-4 joints in first 6 months Persistent -same as above Extended - > 5 joints beyond 6 months Family historypsoriasis Family history-hla B27 disease Positive RF test HLA B27 male with onset >6yrs Systemic features Polyarticular JIA RF Criteria Exclusions 5 or more joints in first 6 months RF - Family historypsoriasis Family history- HLA B27 disease Positive RF test HLA B27 male with onset >6yrs Systemic features
Polyarticular JIA RF + Criteria Exclusions 5 or more joints in first 6 months and + RF test twice 3 months apart Family historypsoriasis Family history-hla B27 disease HLA B27 male with onset >6yrs Systemic features Spykar 6 yr boy fevers high spiking 103F X 2 wks Rash on/ off, Recently shortness of breath O/E big liver spleen arthritis Rheumatoid Nodules Micrognathia and Retrognathia Systemic JIA Arthritis with or preceded by fever 2 weeks and any 1 of : -rash -adenopathy -serositis -liver-spleen
Systemic JIA 10-20% JIA fevers - daily spike (often pm) to >103F 1/3 pericarditis, pleuritis Adenopathy Hepatosplenomegaly Arthritis variable Evanescent rash in about 80% Anemia, Elevated WBC and platelets Very high ESR, CRP, ferritin ANA, RF negative. Shenoi S, Wallace CA. Diagnosis & Rx of SJIA. J Pediatr. 2016 Oct;177:19-26. Psoriatic JIA Criteria Exclusions Arthritis and psoriasis or Arthritis and two of - Dactylitis - Nail signs -FH Family history- HLA B27 disease Positive RF test HLA B27 male with onset >6yrs Systemic features 9 yr girl swelling of DIP/ PIP hands X 3 mths Silvery scaly rash elbows and scalp BTW mom says always had funny nails Labs at PMD CBC, ESR/ CRP normal, ANA 1:40 Enthesitis Related Arthritis Criteria Exclusions Arthritis and Enthesitis or Arthritis or Enthesitis two of - - Sacroiliac pain - HLA B 27 + -FH - Anterior uveitis - Male > 6 yrs Family history of psoriasis Systemic features RF + Psoriatic JIA Asymmetric oligoarthritis, DIP joint involvement, dactylitis Uveitis ANA + 30-50% Enthesitis Related Arthritis Adolescent boys, familial 10-20% Lower extremity oligoarthritis; knees and ankles Spine - Sacroiliitis decreased mobility of the lumbar spine Enthesitis Labs - mildly elevated WBC and ESR RF, ANA - negative. HLA-B27 + > 90%
Enthesitis Recognize common medications used to treat JIA Enthesitis There is no cure for arthritis True current therapy controls disease Undifferentiated JIA Criteria Exclusions Not fulfilling criteria for any category Fulfills criteria for more than one category Meeting criteria for other category Rheumatology Pearl #3 JIA requires multidisciplinary management Rheumatology Pharmacologic management PT / OT Ophthalmology Lab/ Radiology Psychosocial support Nutrition and growth. monitoring Pediatrician Nursing support School support Family/ Social support
Arthritis can lead to blindness Slit Lamp Exam Chronic Anterior Uveitis True some children have uveitis if untreated can cause blindness ANA positive children more risk regular ophthalmologic screening Rheumatology Pearl #4 Look into the Eyes Besides the PT the ophthalmologist is the rheumatologists best friend! Late Uveitis -Posterior Synechiae Weblike attachment pupillary margin to anterior lens capsule JIA Anterior Uveitis - Asymptomatic Increased risk if ANA+ AAP Guidelines Pediatrics 2006
We use chemotherapy to Rx JIA True many medications are known chemotherapeutic meds (methotrexate, cyclosporine, cyclophosphamide) Medications Used - JIA C) Disease Modifying Antirheumatic drugs (DMARDs) Methotrexate (po/ iv/ subcutaneous) Others Sulfasalazine Azathioprine Cyclosporine Hydroxychloroquine Leflunomide Powerful medicines Knowing how and when to use them Medications Used - JIA D) Biologics Anti-TNF Etanercept (Enbrel ), Adalimumab (Humira ) Infliximab (Remicade ), Golimumab (Simponi ), Certolizumab (Cimzia ) Anti-IL1 Anakinra (Kineret ), Canakinumab (Ilaris ), Rilonacept (Arcalyst ) AntiCD20 - Rituximab (Rituxan ) Anti-IL6 - Tocilizumab (Actemra ) CTLA4 - Abatacept (Orencia ) E) Small molecule: JAK kinase inhibitor - Tofacitinib (Xeljanz ) Subcutaneous (SC) Infusion Infusion or sc Oral pill Medications Used - JIA A) Nonsteroidal anti-inflammatory (do not combine) Ibuprofen, Naproxen, Meloxicam, Piroxicam, Diclofenac B) Steroids Prednisolone, IV methylprednisolone, Intraarticular injections triamcinolone acetonide, topical (eyes) Children outgrow their arthritis False it may undergo remission but flares are frequent
Children DO NOT outgrow their arthritis 80% of children continue to have active disease Joint damage frequently occurs, and is greatest in the first 2 years of disease 44% achieve remission But by 2 yrs off of meds, most have flared <10% are successfully off meds >5 years Rheumatology Pearl #5 EARLY RECOGNITION IS KEY (you can help!) Once diagnosed early and aggressive therapy improves outcomes! Ringold & Wallace. Rheumatology 2009. Early treatment TREAT Inactive disease (ID) and clinical remission on medications are achievable Best chance of achieving Rapid and early initiation of therapy For each month earlier a subject was treated odds ID increased by 1.32; p = 0.011 Treatment JIA - CARRA Consensus Treatment Plans Childhood Arthritis and Rheumatology Research Alliance North American organization > 400 pediatric rheumatologists, researchers and research coordinators Wallace CA et al. TREAT trial A & R 2012 www.carragroup.org Early treatment ACUTE - JIA Compelling evidence for early aggressive therapy 3 groups: open label study Infliximab and methotrexate Combination DMARD therapy (methotrexate, hydroxychloroquine, sulfasalazine) Methotrexate Response seen in 100% infliximab and methotrexate 65% combination DMARD 50% methotrexate (p<0.0001) Tynjälä P et al. Ann. Rheum. Dis. 2011 CARRA - CTP Consensus treatment plans (CTP) SJIA Polyarticular JIA Dewitt E et all. AC & R 2012 Ringold S, Shenoi S et al. AC & R 2014 Kimura Y et al. AC & R 2014
Polyarticular JIA CTP Other complications of JIA Joint space narrowing Destruction of cartilage Erosions Chronic pain Joint damage Ringold S, Shenoi S et al. AC & R 2014 Recognize common complications JIA Impact Exercise in rheumatic diseases Complications Ana who had OligoJIA is doing well and in remission without active arthritis she still has a gait that is limping and off why? Affected leg grows longer Leg length discrepancy Shoe lift PT = great resource for children with joint pain 100% TRUE
How can JIA impact Child Physical pain, weakness, stiffness after sitting, swelling joints, difficulty writing, unable to participate PE, medication side effects (steroids chubby, methotrexate - nausea) Emotional need for chronic medications, long term injections/ infusions, sense of being different from peers, drain on family resources (doctors appts, bills, copay, hospital stays) Education missing school (doctor visits, OT/PT, infusions, flares), difficulty with vision (rarely), completing assignments/ exams (stiff hands) Exercise in JIA Exercise may improve ROM, active joint count, function, QOL & fitness Exercise DOES NOT worsen disease activity or cause flares Intervention trials small to no effect Takken T et al. Eur J Phys Rehab Med 2008;44(3):287 97. Cavallo S et al. 2016. Arch Phys Med Rehabil. 2016 Dec 6. Exercise can damage the joints False - exercise helps maintain good range of motion - modifications in case of flares Switching gears! Rheumatology Pearl #6 Exercise is the ideal drug Safe Inexpensive Widely available Dose it (pace, self-limit, modify) Houghton K. The Physician and Sports medicine. 2012 Sep 1;40(3):77 82. Hebestreit, H et al. Journal of Rheumatology, 1998. 25(8): p. 1626 33. Lelieveld, O.T. et al. Arthritis & Rheumatism, 2008. 59(10): p. 1379 Henderson, C.J. et al. Arthritis Care & Research, 1995. 8(2): p. 11 Houghton K et al. Journal of Rheumatology, 2013:40(6):979. What is this? 4 year old girl Dermatologist rash on her hands first noticed after she had been swimming did not respond to topical steroids preschool teacher - wasn t keeping up with her classmates parents were complaining she was clumsy
Visual diagnosis JDM evaluation Labs: General blood counts, inflammatory markers CPK, aldolase, LDH Antibodies Imaging swallowing study, muscle MRI Electromyogram Muscle biopsy Juvenile dermatomyositis Rash face (includes eyelids) extensor surfaces hands, knees, elbows Muscle weakness proximal more than distal palate, pharynx, proximal esophagus Vasculitis GI tract, skin Treatment of JDM Supportive airway, nutrition Physical Therapy splints, ranging, active strengthening Medications sunscreen steroids (pulse if severe, daily oral) - need prolonged treatment hydroxychloroquine methotrexate, IVIG Cytoxan MMF, cyclosporine Rituximab Identify resources for further help
Resources Pediatric Rheumatology Staff Seattle Children s 206-987-2193 (parents permission) Doctor on call 206-987-7777 susan.shenoi@seattlechildrens.org Other resources www.arthritis.org www.rheumatology.org This is a picture of Arthritis Camp in 2010 True Arthritis Foundation This is a picture of Arthritis Camp 1970 s THANK YOU True For the invitation And your attention
AND MY HOPE... Too late is the medicine prepared, when the disease has gained strength by long delay Questions