1 / 42

Rheumatic Diseases in Children

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.

casey
Download Presentation

Rheumatic Diseases in Children

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Rheumatic Diseases in Children

  2. Objectives Review Rheumatic Diseases Discuss the medications utilized to treat Rheumatic Conditions By the end of this presentation you will:

  3. Antibodies Lymphocytes T-cells B-cells Phagocytoes Natural killer cells Granulocytes macrophils Skin, mucus membranes, enzymes Natural microbial flora Complement proteins The Immune System101 B and T cells and their products are the target for many of the treatments for Autoimmune diseases seen in Rheumatology 3rd line 2nd line 1st line

  4. The Factors of Autoimmune Disease Genetic predisposition Environment Timing

  5. Rheumatic Conditions Systemic Lupus Erythematosus (SLE) Juvenile Arthritis Uveitis Linear Scleroderma Systemic Sclerosis Juvenile Dermatomyositis Vasculities

  6. Juvenile 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)

  7. Criteria Juvenile Arthritis 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

  8. Clinical Manifestations Juvenile Arthritis Joint specific Morning stiffness Pain on motion Loss of motion Tenosynovitis Joint inflammation: Swelling, redness, heat, pain, loss of function Extra-articular Abnormalities in growth and development Osteopenia Organ-Specific Nodules *** Systemic or rare involvement=vasculitis, cardiac disease, pleuropulmonary disease, GI tract, Lympadenopathy and splenomegaly, hepatosplenomegaly, neurologic, renal

  9. Systemic JA – Rash

  10. Juvenile Arthritisprior to the age of methotrexate and biologics

  11. Treatment JA NSAIDSIntra-articular injection: Ibuprofen Aristospan Naprosyn Diclofenac GlucocorticosteroidsDMARDS Prednisone Methotrexate Methylprednisolone Sulfasalazine Leflunomide Biologic Response Modrifiers Etanercept(Enbrel); Adalimumab (Humira) Infliximab (Remicade); Anakinra (Kineret)/systemic Abatacept (Orencia) Rituximab(Rituxan) Tocilizumab(in study)

  12. Laboratory Studies No laboratory testing is diagnostic for JA Used for evidence of inflammation, determine pathogenesis, support diagnosis, and monitor treatment

  13. Laboratory 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

  14. UVEITIS Inflammation of uveal tract Iris, ciliary body, and/or choroid Asymptomatic until very late stages Uveitis 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

  15. Uveitis

  16. Treatment Uveitis Opthalmology: Topical steroid drops Systemic Treatment DMARD Methotrexate Corticosteroids Prednisone Methylprednisonlone Biologic Response Modifiers Infliximab (Remicade) Etanercept(Enbrel); Adalimumab (Humira) Diclizumab (Zenapak)

  17. Systemic Lupus 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

  18. Diagnosis 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)

  19. SLE rashes Upper Malar Lower left Discoid Lower right Mixed rashes

  20. Laboratory Studies: Diagnostic 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

  21. Laboratory Studies: Monitoring 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)

  22. Treatment SLE Plaquenil Aspirin NSAIDS Prednisone Methotrexate Imuran Rituximab IVIG Orencia Cellcept Cytoxan IV Daily Cytoxan Plasmapheresis Nitrogen Mustard Campath BMT

  23. Features of Juvenile Dermatomyositis Gottran’s Papules Heliotrope Rash

  24. Juvenile Dermatomyositis: Radiographical features Calcinosis Thigh of 12yr old male Inflammation is bright white

  25. Laboratory JDMS CBC Chem 14: monitoring medication side effects Aldolase: elevated with muscle inflammation: monitoring and confirmation not diagnostic Neopterin: same as aldolase CPK: same as aldolase and Neopterin ESR: inflammation unspecified location Urinalysis

  26. Treatment Dermatomyositis Stem Cell Transplant Campath Biologics Infliximab Etanercept Abatacept Methotrexate Glucocorticosteroids Cyclosporin Hydroxychloroquine IVIG Cyclophosphamide

  27. Linear Scleroderma 11 yr old girl 10 year old girl Diagnosis age 4 Coupe de Sabre

  28. Systemic Sclerosis Children • 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 organs • Cassidy, 2005

  29. Systemic Sclerosis: Clinical Manifestations 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

  30. Scleroderma: Acrolysis and calcinosis Unaffected

  31. Scleroderma Renal arteriogram: Left is normal Right is renal insufficiency Pulmonary x-ray Interstitial Fibrosis

  32. Laboratory Scleroderma CBC Chem. 14 Urinalysis SCL70 antibody (diagnostic for SSc <30% of children, >70% in adults)

  33. Treatment Scleroderma Stem Cell Transplant Campath Mycophenolate Mofetil IVIG Methotrexate Cyclosporin Hydroxychloroquine Glucocorticosteroids Cyclophosphamide

  34. Classification of Vasculitides Small Vessel Vasculitis ANCA associated microscopic polyangitis; Wegener’s granulomatosis; Churg-Strauss Syndrome; Drug induced Immune complex Henoch-Schonleinpurpura; (SLE,JIA, Sjogrens); Bechets; Drug associated; Infection associated Paraneoplastic lymphoproliferativeneoplams induced, myeloproliferative neoplasm induced, carcinoma induced Inflammatory Bowl Disease (IBD)

  35. Classification of Vasculitides Medium Vessel Vasculitides Polyarteritis nodosa Kawasaki disease Large Vessel Vasculitides Giant Cell arteritis Takayasus’s arterititis

  36. Wegener’s Granulomatosis • 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

  37. WegenersGranulomatosisSaddle Nose

  38. Wegener Granulamotosisgranulomas and cavitations

  39. Treatment Wegener’s Granulomatosis Cyclophosphamide IVIG Glucocorticosteroids Methotrexate

  40. Takayasu’sArteritis • 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

  41. Takayasu: Angiograms

  42. Treatment Takayasu’sArteritis Cyclophosphamide Methotrexate Glucocorticosteroids Infliximab

More Related