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Understanding Connective Tissue Diseases in Children

Explore common rheumatic and connective tissue diseases affecting children with insights from Dr. Abdullah Al Mazyad. Learn about clinical presentations, symptoms, and exclusions of Juvenile Idiopathic Arthritis and its classifications. Discover management approaches for JIA.

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Understanding Connective Tissue Diseases in Children

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  1. COMMONRHEUMATIC DISEASES CONNECTIVE TISSUE DISEASES Dr. Abdullah Al Mazyad Consultant Pediatric Rheumatologist Department of Pediatrics King Saud University

  2. CONNECTIVE TISSUE DISEASESCTD . Diseases which affect tissue that supports,binds,and protect organs . . Two major proteins affected : - Collagen founds in tendons,ligaments, skin,cartilage,bone,blood vessles. - Elastin a stretchy protein like rubber, major component of ligaments and skin

  3. CTD • >100 Diseases. • Most body systems may be affected. • Most share common symptoms[joint inflammation,fever, rash,weakness,etc] • Etiology unknown [genatic,enviroment,autoimmunity] • All male and females affected • All countries.

  4. Clinical presentation Symptoms - APPROPRIATE RHEUMATOLOGIC HISTORY - Pain [joint,muscles,limbs] - Stiffness [morning] - Loss of function [limbing] - Systemic illnes [fever-rash-weakness-mucous membrane involement-etc] Signs - FULL CLINICAL EXAMINATION - Vital signs - Growth parmeter - Arthritis//Arthralgia - Rash [types] - Most related systems.

  5. Juvenile Idiopathic ArthritisJIA Abbreviations before: J.C.A. in Europe J.R.A. in U.S. Features: • Onset under 16 years • Persistent arthritis in one or more joints • Duration - three months or longer (Europe) - six weeks or longer (U.S.) 4. Exclude other defined causes of arthritis in childhood .

  6. Juvenile Idiopathic Arthritis: Common Exclusions .

  7. Juvenile Idiopathic Arthritis

  8. Classification of Juvenile Idiopathic Arthritis[ILAR] * ILAR: International League Against Rheumatism

  9. Systemic Arthritis (ILAR) • Arthritis in1 joint [for  6 weeks]. - with or preceded by • Fever  2 weeks, quotidian ( 390 returns to  370C),documented daily for 3days - with 3. At least one of the following:  Evanescent[not fixed] erythematous rash.  Generalized lymph node enlargement.  Hepatomegaly and/or splenomegaly.  Serositis.

  10. Oligoarthritis Arthritis in 1-4 joints in the first six months of oncet. Types • Persistent disease [1-4 joints throughout the disease] • Extended disease [5 joints after the first six months].

  11. Polyarthritis (RF negative) Arthritis affecting  5 joints in the first six months of disease; - RF negative.

  12. Polyarthritis (RF positive) Arthritis affecting 5 joints in the first six months of disease; -Positive RF in 2 separate occations 3months apart, during the first six months.

  13. Psoriatic Arthritis Arthritis and psoriasis OR Arthritis and at least two of: 1) Dactylitis, 2)Nail pitting or onycholysis, 3) Family history of psoriasis.

  14. Enthesitis Related Arthritis Arthritis and enthesitis, OR arthritis or enthesitis with  2 of : [1]Sacroiliac joint tendernes and/or inflammatory lumbosacral pain. [2] HLA-B27 [3]Family history of HLA-B27associated disease [4] Acute anterior uveitis [5] Oncet of oligoarthritis in a boy aged 8 years.

  15. Undifferentiated Arthritis Arthritis that does not fulfills any criteria. OR Arthritis that fulfills two OR more criteria.

  16. Pathology Serositis • Synovitis • Tendenitis • Bursae Serositis of pleura and pericardium Nodules Vasculitis

  17. Systemic onset JIA 20%

  18. Polyarticular JIA 30%

  19. Pauciarticular JIA (50% )

  20. Management of Juvenile idiopathic Arthritis

  21. Treatment for JIA

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