rheumatology basics kalyani mccullough, md clinics and mop didactic
TRANSCRIPT
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Rheumatology BasicsRheumatology Basics
Kalyani McCullough, MDKalyani McCullough, MD
Clinics and MOP DidacticClinics and MOP Didactic
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Purpose of this talkPurpose of this talk
To provide a foundation for: To provide a foundation for: Seeing patients in Rheumatology clinicSeeing patients in Rheumatology clinic Getting started on the work-up of Getting started on the work-up of
suspected rheumatologic diseases in suspected rheumatologic diseases in your clinicyour clinic
Review most common rheumatologic Review most common rheumatologic diseasesdiseases Fast overviewFast overview Resource for laterResource for later
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Main diseases you’ll be Main diseases you’ll be seeingseeing
Inflammatory arthritisInflammatory arthritis Rheumatoid, spondyloarthropathiesRheumatoid, spondyloarthropathies
Crystal arthritisCrystal arthritis Gout, PseudogoutGout, Pseudogout
Autoimmune disordersAutoimmune disorders Lupus, Sclerodema, Autoimmune myositisLupus, Sclerodema, Autoimmune myositis
VasculitisVasculitis Large, medium, small vesselLarge, medium, small vessel
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What are some clues your patient What are some clues your patient may have a rheumatologic may have a rheumatologic
disease?disease? Constitutional symptomsConstitutional symptoms
Fevers, sweats, weight loss, fatigueFevers, sweats, weight loss, fatigue Subacute time courseSubacute time course
Started over weeks to monthsStarted over weeks to months Characteristic symptomsCharacteristic symptoms
Mono or polyarticular joint pain, multisystem Mono or polyarticular joint pain, multisystem complaints (i.e. skin, lung, GI)complaints (i.e. skin, lung, GI)
Are there signs of an inflammatory Are there signs of an inflammatory process?process? Synovitis, rash, serositis, elevated ESR/CRPSynovitis, rash, serositis, elevated ESR/CRP
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How do you get started on the How do you get started on the work-up?work-up?
Thorough physical examThorough physical exam Lab testsLab tests
CBC w diff, ESR, CRP, ANA, targeted CBC w diff, ESR, CRP, ANA, targeted autoantibodiesautoantibodies
RadiographsRadiographs Erosive arthritis, sacroillitisErosive arthritis, sacroillitis
Biopsy?Biopsy? Skin, muscle, kidneySkin, muscle, kidney
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Basics of treatmentBasics of treatment NSAIDsNSAIDs
Symptom relief, especially joint painSymptom relief, especially joint pain GlucocorticoidsGlucocorticoids
Acutely, try to use steroid sparing agent if possibleAcutely, try to use steroid sparing agent if possible Disease-modifying antirheumatic drugs Disease-modifying antirheumatic drugs
(DMARDs)(DMARDs) Synthetic DMARDsSynthetic DMARDs
Slow onset, 2-6 monthsSlow onset, 2-6 months Methotrexate, Hydroxychloroquine, Sulfasalazine, Azathioprine, Methotrexate, Hydroxychloroquine, Sulfasalazine, Azathioprine,
Mycophenolate mofetil, CyclophosphamideMycophenolate mofetil, Cyclophosphamide Biologic DMARDsBiologic DMARDs
Bioengineered protein drugs (antibodies or receptor-antibody Bioengineered protein drugs (antibodies or receptor-antibody chimeras )chimeras )
IV or SQ onlyIV or SQ only Rapid onsetRapid onset Screen for and treat LTBI, HBV firstScreen for and treat LTBI, HBV first Infliximab, Etancercept, RituximabInfliximab, Etancercept, Rituximab
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Steroid Side EffectsSteroid Side Effects Weight gainWeight gain DiabetesDiabetes CataractsCataracts InsomniaInsomnia Fluid retentionFluid retention HypertensionHypertension Proximal weaknessProximal weakness AlopeciaAlopecia SweatsSweats OsteoporosisOsteoporosis InfectionInfection Psychiatric disturbance (eg, depression, mania, psychosis)Psychiatric disturbance (eg, depression, mania, psychosis) Easy bruising of the skinEasy bruising of the skin StressStress TremorTremor Peptic ulcer diseasePeptic ulcer disease
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Long term considerations for Long term considerations for most autoimmune diseasesmost autoimmune diseases
Increased risk of CV death due to Increased risk of CV death due to inflammation and accelerated atherosclerosisinflammation and accelerated atherosclerosis Aggressive risk factor reduction: BP<120/80, Aggressive risk factor reduction: BP<120/80,
LDL<100LDL<100 Osteoporosis from glucocorticoidsOsteoporosis from glucocorticoids
Dexa screening, Calcium-Vit D, bisphosphonates Dexa screening, Calcium-Vit D, bisphosphonates prnprn
Infections Infections Increased risk of certain malignanciesIncreased risk of certain malignancies
Lymphoma, Lung/Cervical CA in LupusLymphoma, Lung/Cervical CA in Lupus
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Inflammatory ArthritisInflammatory Arthritis
Rheumatoid arthritisRheumatoid arthritis SpondyloarthropathiesSpondyloarthropathies
Axial skeletal involvement and Axial skeletal involvement and polyarthritis of peripheral jointspolyarthritis of peripheral joints
Ankylosing spondylitisAnkylosing spondylitis Reactive arthritisReactive arthritis Psoriatic arthritisPsoriatic arthritis IBD-associated arthritisIBD-associated arthritis
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Rheumatoid arthritisRheumatoid arthritis Clinical presentation:Clinical presentation:
Chronic, symmetric polyarthritisChronic, symmetric polyarthritis Often starts with the PIPs, MCPs, MTPsOften starts with the PIPs, MCPs, MTPs
Can affect most synovial joints (wrists, knees, elbows Can affect most synovial joints (wrists, knees, elbows etc.)etc.)
Doesn’t affect DIPsDoesn’t affect DIPs Morning stiffness, pain, swelling (vs. OA: worse with Morning stiffness, pain, swelling (vs. OA: worse with
activity)activity) Labs:Labs:
RF, anti-CCP Ab or both in 70% of patientsRF, anti-CCP Ab or both in 70% of patients RF not specific, anti-CCP 90-98% specificRF not specific, anti-CCP 90-98% specific ACD, ESR, CRPACD, ESR, CRP
Xrays:Xrays: articular erosions and joint space narrowingarticular erosions and joint space narrowing
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Joint distribution in RA vs. Joint distribution in RA vs. OAOA
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RA – Physical Exam FindingsRA – Physical Exam Findings• Ulnar deviation
• at mcps• Swan neck deformity
• hyperextension of PIPs, flexion of DIPs
• Boutonniere deformity• flexion of PIP, hyperextension of DIP
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OA – Physical Exam FindingsOA – Physical Exam Findings
Bony outgrowths/ calcific spurs on PIPs and DIPs
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RA – Extra-articular RA – Extra-articular ManifestationsManifestations
Many extra-articular Many extra-articular findings:findings: Rheumatoid nodulesRheumatoid nodules Other organ Other organ
involvement:involvement: Sjogren’s syndromeSjogren’s syndrome ScleritisScleritis Interstitial fibrosisInterstitial fibrosis Pericardial effusionsPericardial effusions Peripheral neuropathyPeripheral neuropathy AmyloidosisAmyloidosis
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RA - TreatmentRA - Treatment Early aggressive treatment leads to better Early aggressive treatment leads to better
longterm outcomeslongterm outcomes Glucocorticoids acutelyGlucocorticoids acutely MTX frequently usedMTX frequently used
Start 7.5mg weekly, increase to a max dose of Start 7.5mg weekly, increase to a max dose of 20-25mg weekly20-25mg weekly
Give with folate 1mg daily to reduce toxicityGive with folate 1mg daily to reduce toxicity Check CBC, AST/ALT, Cr q 3 moCheck CBC, AST/ALT, Cr q 3 mo Contraindications:Contraindications: liver disease, HBV/HCV, liver disease, HBV/HCV,
alcohol use, CrCl<30alcohol use, CrCl<30 Toxicities:Toxicities: oral ulcers, nausea, hepatotoxicity, oral ulcers, nausea, hepatotoxicity,
BMS, pneumonitisBMS, pneumonitis
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SponydloarthropathiesSponydloarthropathies
Inflammatory arthritis with Inflammatory arthritis with involvement of:involvement of: Sacroiliac (SI) jointSacroiliac (SI) joint Axial spineAxial spine Tendon fasciaTendon fascia Ligament insertion points (entheses)Ligament insertion points (entheses) Peripheral jointsPeripheral joints
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Ankylosing SpondylitisAnkylosing Spondylitis Psoriatic ArthritisPsoriatic Arthritis
DIPs often involved, assoc with nail DIPs often involved, assoc with nail pittingpitting
Reactive ArthritisReactive Arthritis Triggered by GI/GU infectionTriggered by GI/GU infection
IBD-associated arthritisIBD-associated arthritis Correlates with bowel activityCorrelates with bowel activity
SpondyloarthropathiesSpondyloarthropathies
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Ankylosing SpondylitisAnkylosing Spondylitis Clinical presentationClinical presentation
Inflammatory back pain in Inflammatory back pain in young adultyoung adult
Reduction in spinal Reduction in spinal mobility, particularly mobility, particularly lumbar flexionlumbar flexion
Association with anterior Association with anterior uveitisuveitis
LabsLabs Increased relative risk of Increased relative risk of
HLA-B27HLA-B27 •XrayXray- Sacroiliitis- Sacroiliitis
•TreatmentTreatment - PT, NSAIDs- PT, NSAIDs
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Crystal ArthritisCrystal ArthritisGout – Natural HistoryGout – Natural History
1) Asymptomatic hyperuricemia1) Asymptomatic hyperuricemia Over production or under excretion (or both)Over production or under excretion (or both)
2) Acute and intermittent gout2) Acute and intermittent gout Attacks triggered by fluctuations in UA level (up Attacks triggered by fluctuations in UA level (up
or down) and deposition of UA crystals in jointor down) and deposition of UA crystals in joint Acute monoarthritis, often 1Acute monoarthritis, often 1stst attack is at 1 attack is at 1stst mtp mtp After years see bony erosions on xrayAfter years see bony erosions on xray
3) Chronic tophaceous gout3) Chronic tophaceous gout
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Gout - TreatmentGout - Treatment Acute attackAcute attack – most efficacious if initiate – most efficacious if initiate
treatment within 48h of onsettreatment within 48h of onset NSAIDs – indomethacinNSAIDs – indomethacin
Avoid if h/o PUD or renal impairmentAvoid if h/o PUD or renal impairment Colchisine Colchisine
1.2mg PO x 1, then 0.6mg 1h later and q24h until 1.2mg PO x 1, then 0.6mg 1h later and q24h until symptoms improvesymptoms improve
Side effects: Diarrhea, abdominal pain, NSAIDs better Side effects: Diarrhea, abdominal pain, NSAIDs better toleratedtolerated
Reduce dose if Cr > 1.5, risk of neuromuscular toxicityReduce dose if Cr > 1.5, risk of neuromuscular toxicity GlucocorticoidsGlucocorticoids
Prednisone 20-40mg/d, taper 1-2 wks after symptoms Prednisone 20-40mg/d, taper 1-2 wks after symptoms resolveresolve
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Gout - ProphylaxisGout - Prophylaxis Goal UA<6Goal UA<6 Xanthine oxidase inhibitors (decrease production)Xanthine oxidase inhibitors (decrease production)
AllopurinolAllopurinol Start with 100mg daily, gradually increase doseStart with 100mg daily, gradually increase dose Max is 800mg/d, though 300-400mg usually effectiveMax is 800mg/d, though 300-400mg usually effective Reduce dose if diminished CrReduce dose if diminished Cr Hypersensitivity reaction can be fatal, more common in Hypersensitivity reaction can be fatal, more common in
older patients with impaired Cr on diureticsolder patients with impaired Cr on diuretics If develop rash, stopIf develop rash, stop
FebuxostatFebuxostat Metabolized by liver, so better with renal impairmentMetabolized by liver, so better with renal impairment Ok with mild-moderate hepatic insufficiencyOk with mild-moderate hepatic insufficiency
Uricosuric agents (increase excretion)Uricosuric agents (increase excretion) ProbenacidProbenacid
Age <65, good renal function, not on more than ASA 81 Age <65, good renal function, not on more than ASA 81 dailydaily
Side effects: rash and GI symptomsSide effects: rash and GI symptoms
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PseudogoutPseudogout
Clinical presentationClinical presentation Intermittent monoarthritis, often knee/wrist, Intermittent monoarthritis, often knee/wrist,
though can be 1though can be 1stst mtp mtp LabsLabs
rhomboid shaped, positively birefringent calcium rhomboid shaped, positively birefringent calcium pyrophosphate dihydrate (CPPD) crystalspyrophosphate dihydrate (CPPD) crystals
Can be associated with other diseases: Can be associated with other diseases: hemochromatosis, thyroid diseasehemochromatosis, thyroid disease
TreatmentTreatment NSAIDs, colchisineNSAIDs, colchisine
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Autoimmune disordersAutoimmune disorders
Systemic lupus erythematosis (SLE)Systemic lupus erythematosis (SLE) Antiphospholipid antibody syndromeAntiphospholipid antibody syndrome Raynaud’s phenomenonRaynaud’s phenomenon SclerodermaScleroderma Sjogren’s DiseaseSjogren’s Disease Dermatomyositis and PolymyositisDermatomyositis and Polymyositis Relapsing PolychondritisRelapsing Polychondritis
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LupusLupus Clinical presentationClinical presentation
Typically affects women of Typically affects women of childbearing agechildbearing age
Multisystem Multisystem relapsing/remitting diseaserelapsing/remitting disease
Affects skin, Affects skin, musculoskeletal, renal, CV, musculoskeletal, renal, CV, pulmonary, GI, CNS, psychpulmonary, GI, CNS, psych
- Hallmark symptoms: photosensitive rash, - Hallmark symptoms: photosensitive rash, polyarthritis, serositis, fatigue polyarthritis, serositis, fatigue - Renal and CNS involvement -> significant - Renal and CNS involvement -> significant morbidity morbidity
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Lupus Lupus LabsLabs
Presence of antinuclear Presence of antinuclear antibodies antibodies
Once ANA positive, no need Once ANA positive, no need to recheckto recheck
Anti-dsDNA and anti-Sm Anti-dsDNA and anti-Sm have great specificity, have great specificity, but lack sensitivity.but lack sensitivity.
Hypocomplementemia Hypocomplementemia may occur during flares. may occur during flares.
C3, C4, CH50C3, C4, CH50
TreatmentTreatment Glucocorticoids, DMARDsGlucocorticoids, DMARDs
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Lupus - MonitoringLupus - Monitoring Lupus nephritisLupus nephritis – approx 50% – approx 50%
Typically immune complex glomerulonephritisTypically immune complex glomerulonephritis Screen q3 mo:Screen q3 mo:
Ask about polyuria, nocturia, foamy urineAsk about polyuria, nocturia, foamy urine Look for HTN and edemaLook for HTN and edema Labs: UA – RBC casts, spot prot: Cr ratioLabs: UA – RBC casts, spot prot: Cr ratio
Diagnose with biopsyDiagnose with biopsy Increased risk of malignancyIncreased risk of malignancy: Hodkin’s and : Hodkin’s and
Non-Hodgkins lymphoma, Lung CA, Cervical CANon-Hodgkins lymphoma, Lung CA, Cervical CA Annual cervical CA screeningAnnual cervical CA screening
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SclerodermaScleroderma Clinical presentationClinical presentation
Early in disease:Early in disease: msk pain, fatigue, weight loss, GERDmsk pain, fatigue, weight loss, GERD Rare disorder, but symptoms common in primary careRare disorder, but symptoms common in primary care
Late in disease:Late in disease: SkinSkin: thickening, ischemic digital ulcers, pitting, : thickening, ischemic digital ulcers, pitting,
telangectasias on face and palmstelangectasias on face and palms LungsLungs: interstitial fibrosis, pulmonary htn: interstitial fibrosis, pulmonary htn GIGI: GERD, dysphagia: GERD, dysphagia RenalRenal: rare, but Scleroderma Renal Crisis (SRC) is life : rare, but Scleroderma Renal Crisis (SRC) is life
threatening emergencythreatening emergency CardiacCardiac: ischemic injury: ischemic injury MSKMSK: erosive arthritis, tendon friction rub: erosive arthritis, tendon friction rub
CREST (limited scleroderma): calcinosis, raynaud’s, CREST (limited scleroderma): calcinosis, raynaud’s, esophageal dysmotility, sclerodactyly, telangectasiasesophageal dysmotility, sclerodactyly, telangectasias
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SclerodermaScleroderma
Digital ulcers
Sclerodactyly Telangectasias
Thickened, tight skin
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Scleroderma Scleroderma LabsLabs
If negative ANA, diagnosis is very unlikelyIf negative ANA, diagnosis is very unlikely Indirect immunofluorescence better than ELISAIndirect immunofluorescence better than ELISA
Anti-centromere Ab in 20-40%Anti-centromere Ab in 20-40% Anti-Scl-70 (anti-topoisomerase) 20-40%Anti-Scl-70 (anti-topoisomerase) 20-40%
Correlates with worse prognosisCorrelates with worse prognosis TreatmentTreatment
No No provenproven disease-modifying medication disease-modifying medication Screening and early identification of internal Screening and early identification of internal
organ involvement importantorgan involvement important Organ specific treatmentOrgan specific treatment
Fibrosis – immunosuppresionFibrosis – immunosuppresion Renal crisis – ACEiRenal crisis – ACEi GERD – PPI and behavioral changeGERD – PPI and behavioral change
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Autoimmune myositisAutoimmune myositis
Polymyositis (PM)Polymyositis (PM) Dermatomyositis Dermatomyositis
Dermatomyositis sine myositis (just Dermatomyositis sine myositis (just skin)skin)
Dermatomyositis sine dermatitis (classic Dermatomyositis sine dermatitis (classic muscle biopsy findings)muscle biopsy findings)
Immune-mediated necrotizing Immune-mediated necrotizing myopathy (IMNM)myopathy (IMNM) Statin exposureStatin exposure
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Clinical presentationClinical presentation Symmetric, Symmetric, proximal proximal muscle weakness that muscle weakness that
develops over weeks to monthsdevelops over weeks to months Difficulty rising from a chair, walking up steps, Difficulty rising from a chair, walking up steps,
washing or brushing hairwashing or brushing hair If severe: dysphagia and respiratory distress from If severe: dysphagia and respiratory distress from
weakness of neck, pharyngeal and diapghragmatic weakness of neck, pharyngeal and diapghragmatic musclesmuscles
Distal weakness very uncommon, suspect another Distal weakness very uncommon, suspect another diagnosisdiagnosis
Autoimmune myositis Autoimmune myositis
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DermatomyositisDermatomyositis Clinical Presentation: SkinClinical Presentation: Skin
Gottron papulesGottron papules: raised : raised violaceous lesions at violaceous lesions at extensor surfaces of mcp, extensor surfaces of mcp, pip, dippip, dip
Gottron signGottron sign: : erythematous rash at above erythematous rash at above sitessites
Heliotrope rashHeliotrope rash: : red/purplish discoloration of red/purplish discoloration of eyelidseyelids
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DermatomyositisDermatomyositis
Shawl signShawl sign: erythematous rash : erythematous rash across posterior neck and across posterior neck and shouldersshoulders
V signV sign: erythematous rash on : erythematous rash on anterior neck and chestanterior neck and chest
Mechanic’s hands/feetMechanic’s hands/feet: : hyperkeratotic skin thickening, hyperkeratotic skin thickening, often with painful crackingoften with painful cracking
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Autoimmune myositis Autoimmune myositis LabsLabs
Elevated CK, aldolase, AST, ALT, LD from Elevated CK, aldolase, AST, ALT, LD from damaged muscle cellsdamaged muscle cells
Auto antibodies (anti-Jo-1, anti PL-7)Auto antibodies (anti-Jo-1, anti PL-7) ANA positive in >50%ANA positive in >50% Antisynthetase syndrome in dermatomysoitis: Antisynthetase syndrome in dermatomysoitis:
also with ILD, arthritis, fevers, raynaud’salso with ILD, arthritis, fevers, raynaud’s
MRI:MRI: muscle edema, fascial inflammation muscle edema, fascial inflammation EMG:EMG: irritable myopathy irritable myopathy Muscle biopsy:Muscle biopsy: generally nonspecific generally nonspecific
findings, but rules out non-autoimmune findings, but rules out non-autoimmune myopathiesmyopathies DM: perifascicular atrophy is specificDM: perifascicular atrophy is specific
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TreatmentTreatment Glucocorticoids to control acute diseaseGlucocorticoids to control acute disease
Taper to lowest effective doseTaper to lowest effective dose Steroid sparing agent: MTX, azathioprineSteroid sparing agent: MTX, azathioprine Exercise: especially isometric, avoid Exercise: especially isometric, avoid
heavy weight liftingheavy weight lifting
Autoimmune myositis Autoimmune myositis
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Autoimmune myositis vs. Autoimmune myositis vs. PMRPMR
Polymyalgia Rheumatica (PMR) Polymyalgia Rheumatica (PMR) aching and stiffness of neck, shoulders, aching and stiffness of neck, shoulders,
hips (vs. weakness)hips (vs. weakness) Elevated ESR >40Elevated ESR >40 Age >50Age >50 Rapid response to prednisone 20mg/d or Rapid response to prednisone 20mg/d or
lessless Association with GCA, but can occur Association with GCA, but can occur
independently tooindependently too
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Mixed Connective Tissue Mixed Connective Tissue DiseaseDisease
Overlap syndrome with features of Overlap syndrome with features of SLE, Scleroderma and PolymyositisSLE, Scleroderma and Polymyositis
Characteristic clinical symptoms: Characteristic clinical symptoms: Raynaud phenomenon, hand edema, Raynaud phenomenon, hand edema,
synovitis, inflammatory muscle disease, synovitis, inflammatory muscle disease, sclerodactyly sclerodactyly
High titers of anti-U1 RNP High titers of anti-U1 RNP autoantibodyautoantibody
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VasculitisVasculitis
Inflammatory destruction of blood Inflammatory destruction of blood vesselsvessels
Occlusion, rupture, thrombus can Occlusion, rupture, thrombus can lead to tissue ischemialead to tissue ischemia
Vary in severity and presentation Vary in severity and presentation based on vessels affectedbased on vessels affected
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Vasculitis Vasculitis Clinical presentationClinical presentation
Constitutional symptomsConstitutional symptoms Subacute Subacute Signs of inflammationSigns of inflammation Multisystem diseaseMultisystem disease
i.e. Mononeuritis multiplexi.e. Mononeuritis multiplex Distinct/named peripheral nerves infarcted one at a timeDistinct/named peripheral nerves infarcted one at a time Due to vasculitis of vasa nervorum leading to ischemiaDue to vasculitis of vasa nervorum leading to ischemia Hallmarks are asynchrony and asymmetryHallmarks are asynchrony and asymmetry
LabsLabs Diagnose with biopsyDiagnose with biopsy
TreatmentTreatment Disease specific, often immunosuppressive therapyDisease specific, often immunosuppressive therapy
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In SummaryIn Summary Suspect a rheumatologic process in patients with Suspect a rheumatologic process in patients with
subacute onset of constitutional symptoms, signs subacute onset of constitutional symptoms, signs of inflammation and multi system involvementof inflammation and multi system involvement
Get work-up started with thorough PE, targeted Get work-up started with thorough PE, targeted labs and xrayslabs and xrays
Consult with Rheum and consider getting Consult with Rheum and consider getting prednisone started to control acute disease, with prednisone started to control acute disease, with a goal of tapering to the lowest effective dose a goal of tapering to the lowest effective dose when possiblewhen possible
Monitor for the longterm complications of Monitor for the longterm complications of autoimmune diseaseautoimmune disease
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Questions?Questions?