100319 Brian V Joachims MD Identify characteristics consistent with a diagnosis of Lupus Interpret ANA results Identify characteristics of Rheumatoid Arthritis Describe Spondyloarthropathy ID: 775440
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Slide1
Rheumatology in Primary Care
10/03/19
Brian V Joachims, M.D.
Slide2Identify characteristics consistent with a diagnosis of LupusInterpret ANA resultsIdentify characteristics of Rheumatoid ArthritisDescribe Spondyloarthropathy
Objectives
Slide329 year-old Caucasian female presents with complaint of fatigueLab results:ANA IFA 1:80, speckled patternHgb 11.9TSH 1.6UA WNLHCG neg
Case 1
Slide4Does our patient have lupus?A) yes, because she has a positive ANAB ) no, because the ANA isn’t high enoughC ) don’t know yet, need more informationD ) what’s lupus?
Lupus (?)
Slide5Systemic Lupus Erythematosus (SLE)Chronic autoimmune disease of unknown cause that can affect virtually any organ of the bodyClinical manifestations:Constitutional symptomsCutaneous Arthritis / arthralgias / myalgiasRenalGIPulmonaryCardiacCNS (Neurologic / Psychiatric)
Lupus (!)
Slide6FatigueMost common complaint (up to 100% at some point during the course of disease)FeverDistinguish from other causes (infection, drug reaction, malignancy)MyalgiasSevere muscle weakness or myositis uncommonWeight changeMay be related to disease or treatments
Lupus (Constitutional Symptoms)
Slide7Acute cutaneous lupus erythematosus (ACLE)Localized (malar / butterfly rash)GeneralizedSubacute cutaneous lupus erythematosus (SCLE)AnnularPapulosquamousDrug-inducedChronic cutaneous lupus erythematosus (CCLE)Discoid lupusLupus panniculitis
Lupus (Skin)
Slide8Acute Cutaneous Lupus Erythematosus
Slide9ANA (Anti-Nuclear Antibody)The detection of antinuclear antibodies (ANA) in serum facilitates the diagnosis of patients with systemic lupus erythematosus (SLE) and related autoimmune diseasesThe absence of ANA in the serum of a patient with suspected SLE also provides important information in that it makes the diagnosis much less likely
Lupus (?)
Slide10IFA – indirect immunofluorescence testHomogeneousSpeckledCentromereNucleolarSolid phase assays (ELISA) – ANA, directPanel of antigens
Antinuclear Antibody
Slide11Titer matters1:40Approx 30% of normal controls97% of patients with SLE1:160Approx 5% of normal controls95% of patients with SLEPrevalence of ANA-associated diseases in the general population ~1%
ANA (IFA)
Slide12ANA IFA
Slide13ANA IFA
Slide14Panel of antigens:Anti-dsDNA AbRNP AbSmith AbRheumatoid Arthritis factorSjogren’s Anti-SS-A (Ro Ab)Sjogren’s Anti-SS-B (La Ab)Antichromatin Ab
Lupus Panel (
Reichlin
)
Slide15CriteriaClassification (not diagnostic)ACR – American College of RheumatologySLICC – Systemic Lupus International Collaborating Clinics
Diagnosing Lupus
Slide16Clinical criteria (“in the absence of other causes…”)Acute cutaneous lupusChronic cutaneous lupusOral or nasal ulcersNon-scarring alopecia(not):Male patternHypothyroidismIron deficiencyArthritisSwelling or effusion – OR –Tenderness in 2 or more joints and at least 30 minutes of morning stiffness
SLICC Criteria
Slide17Clinical criteria (cont.)SerositisPleurisy, pleural effusions or pleural rubRenalUrine protein-to-creatinine ratio representing 500 mg pro/24 hours- OR – RBC castsNeurologic
SLICC Criteria
Slide18Hemolytic anemiaDAT / direct CoombsLeukopenia / LymphopeniaWBC <4000/mm3Lymph # <1000/mm3ThrombocytopeniaPlatelets <100,000/mm3
SLICC Criteria
Slide19Immunologic criteriaANAAnti-dsDNAAnti-SmAntiphospholipid antibodyLupus anticoagulantAnticardiolipin antibodyAnti-2-glycoproteinFalse positive RPRLow complement (C3, C4 or CH50)Direct Coombs’ testIn the absence of hemolytic anemia
SLICC Criteria
Slide20RheumaHelper
Slide21Goals of therapy:ensure long-term survivalachieve the lowest possible disease activityprevent organ damageminimize drug toxicityimprove quality of lifeeducate patients about their role in disease management
Treatment
Slide22SteroidsIMOral (prednisone)Disease-Modifying Anti-Rheumatic Drugs (DMARD’s) – “steroid-sparing” agentsHydroxychloroquineMethotrexateSulfasalazineAzathioprineLeflunomide
Medications
Slide23FDA-approved:MalariaChemoprophylaxis, acute attackOff-label:LupusRheumatoid arthritisSjogren’s syndromePorphyriaQ fever
Hydroxychloroquine
Slide24Marketed as Plaquenil in the U.S.$11.88 (per pill)Generic $1.83 - $4.36GoodRx: $38.67 for 60 tablets200-400 mg daily (200 mg tablets)“Typical” dose – 400 mg daily 5 mg/Kg (80 Kg person – 400 mg)Mechanism of action:Antimalarial, blocks toll-like receptorsImpairs complement-dependent antigen-antibody reactions
Hydroxychloroquine
Slide25Adverse reactions:Retinal toxicity / maculopathyPotentially irreversible retinopathyAssociated with daily doses >5 mg/KgDuration of >5 yearsOphthalmologic exam – baseline / within the first year, followed by annual screening beginning after 5 years of use
Hydroxychloroquine
Slide26“Bull’s-Eye” Maculopathy
Slide27Adverse reactions (cont.):Hematologic effectsAnemiaLeukopeniaThrombocytopeniaNo prescribed surveillance lab (“CBC at baseline and ‘periodically’”)Skin discolorationDyschromia (skin and mucosal; black-blue color)
Hydroxychloroquine
Slide28HCQ Dyschromia
Slide29HCQ Dyschromia
Slide30Slide31Any-year-old any-ethnicity male/female presents with complaints of increasing joint pain, particularly in the hands, shoulders and ankles. The patient’s hands are typically swollen in the mornings and he/she has stiffness in most joints for about an hour after waking.
Case 2
Slide32Lab results:Rheumatoid factor 65.4 (0.0-13.9)Anti-CCP 124 (0-19)ESR 44CRP 8.9 (0.0-3.2)
Case 2
Slide33Does our patient have rheumatoid arthritis?A) yes
Rheumatoid Arthritis (?)
Slide34Rheumatoid arthritis (RA) is a chronic, systemic, inflammatory disorder of unknown etiology that primarily involves synovial joints
Rheumatoid Arthritis
Slide35Synovial Joint
Slide36Synovitis
Slide37Synovitis (clinically):Soft tissue swellingWarmth over a jointJoint effusionStiffness – “slowness or difficulty moving the joints when getting out of bed or after staying in one position too long, which involves both sides of the body and gets better with movement”Morning – at least 30 minutesGelling – stiffness with / after rest during the day
Rheumatoid Arthritis
Slide38Morning stiffnessBetter with activitySwellingWarmthTendernessRednessSteroids helpful?
Rheumatoid Arthritis
Slide39Rheumatoid Arthritis – Joint Distribution
Slide40Rheumatoid factors are antibodies directed against the Fc portion of immunoglobulin G (IgG)Found in 75 to 80 percent of RA patients at some time during the course of their diseaseTiter matters:RF titer of 1:40 or greater was 28 percent sensitive and 87 percent specific for RAA titer of 1:640 or greater increased the specificity to 99 percent
Rheumatoid Factor (RF)
Slide41Anti-citrullinated peptide antibodies (ACPA, which include anticyclic citrullinated peptides)More specific for rheumatoid arthritis than RFPearl: if low-titer RF (< 3 times upper limit of normal) and negative CCP, check for Hep C
CCP Antibodies
Slide42Rheumatoid Arthritis
Slide43Swan Neck Deformity
Slide44Swan Neck
Slide45Boutonniere Deformity
Slide46Boutonniere
Slide47Non-articular manifestationsOsteopeniaMyositisVasculitisSkin diseaseEye involvementLung diseaseCardiac diseaseKidney disease
Not Just the Joints…
Slide48MethotrexateFDA-approved:ALLBreast cancerNon-Hodgkin’s lymphomaPsoriasisRheumatoid arthritisOff-label:Crohn’s diseaseDermatomyositis / PolymyositisSLE
Treatment
Slide49Inhibits dihydrofolate reductase (DHFR)Can tightly bind to DHFR and inhibit DNA synthesis and cell proliferation7.5-15 mg weekly to beginLiterature indicates titrating to 20-30 mg weeklyAdjust gradually to optimum responseToxicity increased at doses >20 mg per weekConcomitant folic acid1-5 mg dailyAt least 5 mg per week
Methotrexate
Slide50Adverse effects:AlopeciaStomatitisGI upsetIncreasing CrLiver enzyme abnormalitiesThrombocytopeniaAnemiaLeukopeniaPulmonary diseaseTeratogenic
Methotrexate
Slide51• Pregnancy: [US Boxed Warning]: Methotrexate has been reported to cause fetal death and/or congenital abnormalities. Methotrexate is not recommended for women of childbearing potential unless there is clear medical evidence that the benefits can be expected to outweigh the considered risks. Pregnant women with psoriasis or rheumatoid arthritis should not receive methotrexate. Some products are contraindicated in pregnant women.
Methotrexate
Slide52Methotrexate treatment should be discontinued for at least three months before attempting to become pregnant www.ACRPatientInfo.org © 2018 American College of Rheumatology
Methotrexate
Slide53Slide5439-year-old male with psoriasis presents with a 6-month history of progressive low back pain and stiffness which is most aggravating at rest. He indicates this is OK as long as he keeps moving.
Case 3
Slide55In patients with chronic back pain >3 months:4 out of 5:Onset at age <40 (or 45) yearsInsidious onsetImprovement with exerciseNo improvement with restPain at night (with improvement upon getting up)
Inflammatory Back Pain
Slide56Ankylosing spondylitis (AS)Nonradiographic axial SpA (nr-axSpA)Peripheral SpASpA associated with psoriasis or psoriatic arthritisSpA associated with Crohn disease and ulcerative colitisReactive arthritis (formerly called Reiter's syndrome)Juvenile-onset SpA
Spondyloarthritis
Slide57Peripheral arthritisEnthesitis - inflammation around the enthesis, which is the site of insertion of ligaments, tendons, joint capsule, or fascia to bone, and is relatively specific to SpADactylitis (sausage digits)
Spondyloarthritis
Slide58Enthesitis
Slide59Enthesitis
Slide60Enthesitis
Slide61Dactylitis
(“Sausage Digit”)
Slide62SacroiliitisHLA B27UveitisPsoriasisCrohn’s colitis (IBD)Good response to NSAID’sFam Hx of SpAElevated CRPPreceding infection (reactive arthritis)
Other Features
Slide63TNF-inhibitorsHumira (Adalimumab)Human monoclonal antibodyEnbrel (Etanercept)Soluble receptor fusion proteinRemicade (Infliximab)Chimeric (mouse / human) antibody
Biologic DMARD’s
Slide64Anti-IL-6Actemra (tocilizumab)Anti-IL-1Kineret (anakinra)JAK inhibitorsXeljanz (tofacitinib)
Biologic DMARD’s
Slide65Anti-IL-17ACosentyx (secukinumab)Taltz (ixekizumab)Selective T-Cell Costimulation BlockerOrencia (abatacept)Phosphodiesterase-4 Enzyme InhibitorOtezla (apremilast)
Biologic DMARD’s
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