Download - Rheumatic Diseases in Children
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Rheumatic Diseases in Rheumatic Diseases in ChildrenChildren
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ObjectivesObjectives
Review Rheumatic Diseases Discuss the medications utilized to treat
Rheumatic Conditions
By the end of this presentation you will:
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The Immune SystemThe Immune System 101101
AntibodiesLymphocytes
T-cellsB-cells
PhagocytoesNatural killer cells
Granulocytesmacrophils
Skin, mucus membranes, enzymesNatural microbial floraComplement proteins
B and T cells and their productsare the target for many of the
treatmentsfor
Autoimmune diseases seen in
Rheumatology
3rd line
2nd line
1st line
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The FactorsThe Factors of Autoimmune of Autoimmune DiseaseDisease
Genetic predisposition
Environment Timing
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Rheumatic Rheumatic ConditionsConditions Systemic Lupus Erythematosus
(SLE) Juvenile Arthritis
UveitisLinear Scleroderma Systemic Sclerosis
Juvenile Dermatomyositis Vasculities
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Juvenile ArthritisJuvenile Arthritis•85,0000-115,000 children in the United States have Juvenile Arthritis
•Most Common Rheumatic Disorder in Children
•Diagnostic Criteria •Age at onset <16•Arthritis in one or more joints•Duration of disease 3 months or longer (6weeks for ACR)
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Criteria Juvenile Criteria Juvenile ArthritisArthritis
• Types defined by characteristics of disease
• Pauciarticular (Oligoarthritis for JIA) <5 joints
• Polyarticular: >5 joints• Systemic:arthritis with characteristic
fever (rash) • Juvenile Psoriatic arthritis• Spondyloarthropathies
• Juvenile Anklylosing Spondyloarthritis • Enthesitis-related arthritis
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Clinical Clinical Manifestations Manifestations Juvenile ArthritisJuvenile ArthritisJoint specificMorning stiffnessPain on motionLoss of motionTenosynovitisJoint inflammation: Swelling, redness, heat, pain, loss of function
Extra-articular Abnormalities in growth and developmentOsteopenia
Organ-Specific Nodules*** Systemic or rare involvement=vasculitis, cardiac disease, pleuropulmonary disease, GI tract, Lympadenopathy and splenomegaly, hepatosplenomegaly, neurologic, renal
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Systemic JA – RashSystemic JA – Rash
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Juvenile ArthritisJuvenile Arthritisprior to the age of methotrexate and prior to the age of methotrexate and biologicsbiologics
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Treatment JATreatment JA NSAIDS Intra-articular injection: Ibuprofen Aristospan Naprosyn Diclofenac
Glucocorticosteroids DMARDS Prednisone Methotrexate Methylprednisolone Sulfasalazine Leflunomide
Biologic Response Modrifiers Etanercept(Enbrel);
Adalimumab (Humira)Infliximab (Remicade);
Anakinra (Kineret)/systemicAbatacept (Orencia)Rituximab(Rituxan)
Tocilizumab(in study)
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Laboratory StudiesLaboratory StudiesNo laboratory testing is diagnostic
for JA
Used for evidence of inflammation, determine pathogenesis, support diagnosis, and monitor treatment
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Laboratory StudiesLaboratory Studies • Antinuclear antibody (ANA)
– Pauciarticular disease (+) demonstrates increase risk of uveitis• Rheumatoid Factor
– More indicative erosive disease 3% (Cassidy, 2005) • Sedimentation rate (ESR)
– Non specific measure of inflammation • C reactive protein (CRP)
– more reliable monitor of inflammatory response• CBC • Chem 14
– monitoring potential side effects NSAIDS and methotrexate increased LFT’s
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UVEITISUVEITISInflammation of uveal tract
◦ Iris, ciliary body, and/or choroid Asymptomatic until very late stagesUveitis is often progressive & difficult to
control◦ Possible Symptoms: synichiae, reduced vision,
glaucoma, increased inflammation in the other eye, and blindness
Slit Lamp examination for diagnosis and follow-up
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Uveitis
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Treatment UveitisTreatment Uveitis Opthalmology: Topical steroid drops
Systemic Treatment
DMARD Methotrexate
Corticosteroids Prednisone
Methylprednisonlone
Biologic Response Modifiers
Infliximab (Remicade)
Etanercept(Enbrel); Adalimumab (Humira)
Diclizumab (Zenapak)
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Systemic Lupus Systemic Lupus Erythematosus (SLE)Erythematosus (SLE)• Incidence: 0.5 -0.6/100,000 children• Prevalence: 5-10,000 children in the USA• Onset: 15% in childhood• Female to Male ratio
– Higher female onset post pubescent– Equal pre pubescent
• Affects multiple systems • Characterized by inflammation of the
small blood vessels and connective tissue
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Diagnosis of SLEDiagnosis of SLE• 4 out of 11 criteria
◦ Malar rash◦ Discoid rash◦ Photosensitive rash◦ Mucosal ulcers◦ Serositis◦ Arthritis ◦ Renal disease/cellular casts◦ CNS: Seizure or psychosis◦ Hematology: Leukopenia <4000/cubic mm; lymphopenia <
1500/cubic mm; thrombocytopenia <100,000/mm3
◦ Immunoserology: anti double stranded DNA (anti ds DNA), anti Smith - specific marker for active SLE, false + (VDRL)
◦ Positive AntiNuclear Antibody test (ANA) (95%, typical pattern is homogeneous)
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SLE rashes
Upper Malar
Lower left Discoid
Lower rightMixed rashes
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Laboratory Studies: Laboratory Studies: DiagnosticDiagnostic Cytopenia
◦ Thrombocytopenia ◦ Anemia: hemolytic (Coombs +) ◦ Leucopenia
Positive Immunoserology◦ dsDNA: (+) presence of antibodies◦ Sm nuclear antigen: (+) presence of antibodies◦ Antiphospholipid antibodies: (+) risk of clotting◦ VDRL (syphilis) false (+)
Antinuclear antibody (ANA)◦ antibody most commonly found in SLE
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Laboratory Studies: Laboratory Studies: MonitoringMonitoring• CBC • Chem 14• Antinuclear Antibody• dsDNA:• Complement 3 and 4
– low in most active SLE disease, used for tracking not diagnostic
• Urinalysis with micro– initial indication of renal disease– usually shows lots of blood, protein and high specific
gravity!!!• Spot urine protein and creatinine
– monitoring of renal disease (UP/UC ratio)
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Treatment SLETreatment SLEPlaquenil Aspirin NSAIDS
Prednisone Methotrexate
Imuran Rituximab IVIG
Orencia
Cellcept Cytoxan IV
Daily Cytoxan
Plasmapheresis
Nitrogen MustardCampath
BMT
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Heliotrope RashGottran’s Papules
Features of Juvenile Dermatomyositis
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Juvenile Dermatomyositis: Radiographical features
Thigh of 12yr old maleInflammation is bright white
Calcinosis
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Laboratory JDMSLaboratory JDMSCBCChem 14: monitoring medication side effects Aldolase: elevated with muscle
inflammation: monitoring and confirmation not diagnostic
Neopterin: same as aldolaseCPK: same as aldolase and NeopterinESR: inflammation unspecified locationUrinalysis
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Treatment Treatment DermatomyositisDermatomyositis
Glucocorticosteroids
Hydroxychloroquine
IVIG
Biologics Infliximab Etanercept
AbataceptMethotrexate
Cyclosporin
Stem Cell Transplant
Cyclophosphamide
Campath
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Linear SclerodermaLinear Scleroderma11 yr old girl
Coupe de Sabre
10 year old girlDiagnosis age 4
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Systemic Sclerosis Systemic Sclerosis ChildrenChildren
0.2-0.9% of the Major Mixed Connective Tissue Disorders
•Prevalence: 0.8/100,000 children in the USA•Onset: 3 % in childhood•Female to Male ratio: 1:1= <8yrs old and 3:1 = >8yrs old•Average age onset in childhood: undefined •Affects multiple systems connective tissue disorder•Characterized by thickening and hardening of the skin in conjunction with fibrous and degeneration of multiple organsCassidy, 2005
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Systemic Sclerosis: Systemic Sclerosis: Clinical Clinical ManifestationsManifestations• Raynaud’s phenomenon
• Skin changes– Sclerosis, edema, atrophy, Telangiectases, calcinosis
• Sclerodactyly• Musculoskeletal Disease estimated 35%
– Morning stiffness, joint pains, contractures, tendon tightening• Gastrointestinal Disease 25%
– Ulcerations of the mouth• Digestive problems• Kidneys
– high blood pressure– kidney failure
• Heart and lung– arrhythmias, heat failure– scaring of the lung tissue
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Scleroderma: Scleroderma: Acrolysis and Acrolysis and calcinosiscalcinosis
Unaffected
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SclerodermaSclerodermaRenal arteriogram:
Left is normalRight is renal insufficiency
Pulmonary x-rayInterstitial Fibrosis
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Laboratory Laboratory SclerodermaSclerodermaCBCChem. 14UrinalysisSCL70 antibody (diagnostic for SSc <30% of children, >70% in adults)
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Treatment Treatment SclerodermaScleroderma
Glucocorticosteroids
Hydroxychloroquine
IVIGMethotrexate
Cyclosporin
Stem Cell Transplant
Cyclophosphamide
CampathMycophenolate Mofetil
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Classification of Classification of VasculitidesVasculitides• Small Vessel Vasculitis
– ANCA associated• microscopic polyangitis; Wegener’s granulomatosis; Churg-Strauss Syndrome; Drug induced
– Immune complex• Henoch-Schonlein purpura; (SLE,JIA, Sjogrens); Bechets; Drug associated; Infection associated
– Paraneoplastic• lymphoproliferative neoplams induced, myeloproliferative neoplasm induced, carcinoma induced
– Inflammatory Bowl Disease (IBD)
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Classification of Classification of VasculitidesVasculitides
Medium Vessel Vasculitides
◦ Polyarteritis nodosa◦ Kawasaki disease
Large Vessel Vasculitides
◦ Giant Cell arteritis◦ Takayasus’s arterititis
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Wegener’s Wegener’s GranulomatosisGranulomatosis
•Prevalence: 0.1/100,000 children•Onset: 3 % in childhood•Female to Male ratio: undefined•Average age onset in childhood: 15.4•Characterized by granulomatous vasculitis in the upper and lower respiratory tracks •Criteria for diagnosis: 2 of 4 must be present
• Nasal of Oral Inflammation• Abnormal appearing chest radiograph• Abnormal urinary sediment
• Granulomatous inflammation Cassidy, 2005
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Wegeners Wegeners GranulomatosisGranulomatosisSaddle Nose Saddle Nose
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Wegener Wegener Granulamotosis Granulamotosis granulomas and cavitations granulomas and cavitations
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Treatment Treatment Wegener’s Wegener’s GranulomatosisGranulomatosis
GlucocorticosteroidsIVIG
Methotrexate
Cyclophosphamide
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Takayasu’s ArteritisTakayasu’s Arteritis•Most common in young women of Japanese origin
•Classification criteria for diagnosis• Sub clavian or aortic bruit • Decreased brachial artery pulse• Blood pressure difference of >10mm between arms• Claudication of extremities• Arteriographic evidence of narrowing or occlusion of aorta, its primary branches
or large arteries in the proximal, upper, or lower extremities
Cassidy, 2005
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Takayasu: Takayasu: AngiogramsAngiograms
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Treatment Treatment Takayasu’s ArteritisTakayasu’s Arteritis
Glucocorticosteroids
Methotrexate
Infliximab
Cyclophosphamide