rheumatic diseases in children
DESCRIPTION
Rheumatic Diseases in Children. Objectives. Review Rheumatic Diseases Discuss the medications utilized to treat Rheumatic Conditions. By the end of this presentation you will:. Antibodies Lymphocytes T-cells B-cells. Phagocytoes Natural killer cells Granulocytes macrophils. - PowerPoint PPT PresentationTRANSCRIPT
Rheumatic Diseases in Rheumatic Diseases in ChildrenChildren
ObjectivesObjectives
Review Rheumatic Diseases Discuss the medications utilized to treat
Rheumatic Conditions
By the end of this presentation you will:
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
The FactorsThe Factors of Autoimmune of Autoimmune DiseaseDisease
Genetic predisposition
Environment Timing
Rheumatic Rheumatic ConditionsConditions Systemic Lupus Erythematosus
(SLE) Juvenile Arthritis
UveitisLinear Scleroderma Systemic Sclerosis
Juvenile Dermatomyositis Vasculities
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)
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
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
Systemic JA – RashSystemic JA – Rash
Juvenile ArthritisJuvenile Arthritisprior to the age of methotrexate and prior to the age of methotrexate and biologicsbiologics
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)
Laboratory StudiesLaboratory StudiesNo laboratory testing is diagnostic
for JA
Used for evidence of inflammation, determine pathogenesis, support diagnosis, and monitor treatment
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
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
Uveitis
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)
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
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)
SLE rashes
Upper Malar
Lower left Discoid
Lower rightMixed rashes
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
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)
Treatment SLETreatment SLEPlaquenil Aspirin NSAIDS
Prednisone Methotrexate
Imuran Rituximab IVIG
Orencia
Cellcept Cytoxan IV
Daily Cytoxan
Plasmapheresis
Nitrogen MustardCampath
BMT
Heliotrope RashGottran’s Papules
Features of Juvenile Dermatomyositis
Juvenile Dermatomyositis: Radiographical features
Thigh of 12yr old maleInflammation is bright white
Calcinosis
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
Treatment Treatment DermatomyositisDermatomyositis
Glucocorticosteroids
Hydroxychloroquine
IVIG
Biologics Infliximab Etanercept
AbataceptMethotrexate
Cyclosporin
Stem Cell Transplant
Cyclophosphamide
Campath
Linear SclerodermaLinear Scleroderma11 yr old girl
Coupe de Sabre
10 year old girlDiagnosis age 4
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
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
Scleroderma: Scleroderma: Acrolysis and Acrolysis and calcinosiscalcinosis
Unaffected
SclerodermaSclerodermaRenal arteriogram:
Left is normalRight is renal insufficiency
Pulmonary x-rayInterstitial Fibrosis
Laboratory Laboratory SclerodermaSclerodermaCBCChem. 14UrinalysisSCL70 antibody (diagnostic for SSc <30% of children, >70% in adults)
Treatment Treatment SclerodermaScleroderma
Glucocorticosteroids
Hydroxychloroquine
IVIGMethotrexate
Cyclosporin
Stem Cell Transplant
Cyclophosphamide
CampathMycophenolate Mofetil
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)
Classification of Classification of VasculitidesVasculitides
Medium Vessel Vasculitides
◦ Polyarteritis nodosa◦ Kawasaki disease
Large Vessel Vasculitides
◦ Giant Cell arteritis◦ Takayasus’s arterititis
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
Wegeners Wegeners GranulomatosisGranulomatosisSaddle Nose Saddle Nose
Wegener Wegener Granulamotosis Granulamotosis granulomas and cavitations granulomas and cavitations
Treatment Treatment Wegener’s Wegener’s GranulomatosisGranulomatosis
GlucocorticosteroidsIVIG
Methotrexate
Cyclophosphamide
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
Takayasu: Takayasu: AngiogramsAngiograms
Treatment Treatment Takayasu’s ArteritisTakayasu’s Arteritis
Glucocorticosteroids
Methotrexate
Infliximab
Cyclophosphamide