Autoimmune conditions and painful, swollen joints

1. Autoimmune conditions and painful, swollen joints Student lecture 7/2003

2. DDx painful swollen joints • Autoimmune conditions • Juvenile Idiopathic Arthritis • Systemic Lupus Erythematosis • Henoch Schönlein purpura • Kawasaki disease

3. Autoimmune • Collagen vascular • Connective tissue

4. Pain in the limbs • Where exactly is the pain / pathology ? • can be difficult to locate or localise • funny/limping gait referred pain ? Bones ? Joints ? Neurological (paraesthesia, weakness)

5. Differential Diagnosis • Arthritis related to infection • Septic arthritis (Staphylococcus, Streptococcus, Haemophilus influenzae, Tuberculosis, etc) • Reactive arthritis (Rubella, Hep B etc) • Malignancy • Leukemia • Neuroblastoma • Hemophilia

6. Differential Diagnosis • Trauma • Acute rheumatic fever • Inflammatory bowel disease • Henoch Schönlein purpura

7. Juvenile Idiopathic Arthritis Juvenile rheumatoid arthritis Juvenile chronic arthritis

8. Definition • presence of arthritis(swelling or effusion, or presence of 2 or more of the following signs: limitation of range of motion, tenderness or pain on motion & increased heat) in 1 or more joints • age on onset <16 yr • duration > 6 weeks • other causes of arthritis ( e.g. septic arthritis, malignancy) excluded

9. Criteria for the classification of juvenile rheumatoid arthritis • Age at onset < 16 years • Arthritis (swelling or effusion, or presence of two or more of the following signs: limitation of range of motion, tenderness or pain on motion, and increased heat) in one or more joints • Duration of disease 6 weeks or longer • Onset type defined by type of disease in first 6 months: • Polyarthritis: 5 or more inflamed joints • Oligoarthritis: <5 inflamed joints • Systemic: arthritis with characteristic fever • Exclusion of other forms of juvenile arthritis Modified from Cassidy JT, Levinson JE, Bass JC, et al.: A study of classification criteria for a diagnosis of juvenile rheumatoid arthritis. Arthritis Rheum 29:274, 1986.

10. Criteria for diagnosis of juvenile chronic arthritis • Age at onset < 16 yr • Arthrits in one or more joints • Duration of disease 3 months or longer • Type defined by characteristics at onset • Pauciarticular < five joints • Polyarticular > four joints, rheumatoid factor negative • Systemic Arthritis with characteristic fever • Juvenile rheumatoid arthritis > four joints, rheumatoid factor positive • Juvenile ankylosing spondylitis • Juvenile psoriatic arthritis European League Against Rheumatism (EULAR) Bulletin 4. No-menclature and Classification of Arthritis in Children. Basel, National Zeitung AG, 1977.

11. ILAR classification criteria for the idiopathic arthritis of childhood

12. ILAR Classification criteria for the idiopathic arthritis of childhood

13. Systemic arthritis

14. ILAR Classification criteria for the idiopathic arthritis of childhood

15. ILAR Classification criteria for the idiopathic arthritis of childhood

16. Enthesitis • Enthesis – site of attachment of tendon, ligament, fascia or capsule to bone • Achilles tendon & plantar fascia • discrete & tender

17. Enthesitis – related arthritis • HLA-B27 associated arthropathies • Diseases of synovium & enthesis • Lower extremities predominant • Spondylitis / sacroiliitis

18. Prospective follow for: • Sacroliitis • Uveitis • Loss of axial motion • Progressive arthropathy

19. Prognosis & outcome • active disease often persists into adult life • predictors of poor outcome: • extended oligoarthritis worse than persistent oligoarthritis • polyarthritis RF+ • systemic arthritis – polyarthritis within 1st 6 months, persistent systemic symptoms, need for steroid treatment, thrombocytosis

20. Chronic uveitis • ~ 20% oligoarthritis JIA • 5-10% polyarthritis JIA • slit lamp examination at diagnosis & ongoing surveillance

21. Management – multi-disciplinary approach • Paediatric rheumatologist • Nurse specialist • Social worker • Physiotherapist • Occupational therapist • Orthopaedic surgeon • Clinical psychologist

22. Aims of treatment • preserve cartilage • control pain • preserve range of motion, muscle strength & function • manage systemic complications • facilitate normal nutrition, growth, physical & psychological development

23. Medical treatment • Non-steroidal anti-inflammatory medication (NSAID) • first line • symptomatic control • not disease modifying • gastrointestinal side effects • COX 2 inhibitors

24. Disease modifying anti-rheumatic medications (DMARDs) • Methotrexate • Corticosteroid - systemic - intra-articular injection • Sulphasalazine • Hydroxycholorquine • Combination • Others

25. Etanercept Azathroprine Cyclophosphamide Infliximab COXII Sulfasalazine Hydroxychloroquine Auranofin Stem cell transplant Cyclosporin A IVIG Pulse IV methylpred

26. Systemic Lupus Erythematosis

27. Complex autoimmune multisystem • Lupus = wolf • Female predominate (> 1: 5) • Coloured race ( oriental & Afro-Caribbean)

28. Clinical features of SLE

29. Criteria for the classification of systemic lupus erythematosus* Malar (butterfly) rash Discoid-lupus rash Photosensitivity Oral or nasal mucocutaneous ulcerations Nonerosive arthritis Nephritis† Proteinuria > 0.5 g/d Cellular casts Encephalopathy † Seizures Psychosis Pleuritis or pericarditis Cytopenia Positive immunoserology † Antibodies to nDNA Antibodies to Sm nuclear antigen Positive LE-cell preparation Biologic false-positive test for syphilis Positive antinuclear antibody test * 4 of 11 criteria provide a sensitivity of 96% and a specificity of 96% †Any one item satisfies that criterion. (Adapted from Tam EM, Cohen AS, Fries JF, et al: The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271, 1982.)

30. Renal involvement • Determine prognosis • Haematuria, proteinuria, nephrotic syndrome, impaired renal function, hypertension • Renal biopsy • Activity index & chronicity index • Aggressive treatment

31. World Health Organization classification of Lupus Nephritis* *LM = light microscopy; IFM = immunofluorescence microscopy; EM = electron microscopy

32. Neonatal lupus erythematosis • Result of fetal damage as result of maternal transplacental autoantibodies • Cardiac • Congenital heart block • May require pacemaker • Skin • Photosensitive • Self-limiting

33. Management of children with SLE

34. Toxicity of Glucocorticoid Drugs • Features of Cushing’s syndrome • Growth suppression • Osteoporosis • Protein wasting, edema, hypokalemia • Gastric irritation • Hypertension • Susceptibility to infection • Impaired carbohydrate tolerance • Cataracts, glaucoma • Psychosis, depression, benign intracranial hypertension • Myopathy

36. The disease may not go away • It may be a permanent, chronic condition • Its course waxes & wanes

39. Henoch Schönlein purpura • Hallmark: palpable purpura

40. Musculoskeletal : knees, ankles > wrists, elbows (painful edema: articular, periarticular)

41. Gastrointestinal manifestation • Abdominal pain • GI bleed • Intussusception • Perforation/infarction • Protein losing enteropathy • Testicular/scrotal pain /swelling • Torsion testes

42. Renal manifestations • Haematuria +/- proteinuria • Nephrotic syndrome • Crescentic glomerulonephritis

43. Kawasaki Syndrome: Diagnostic Criteria of Center for Disease Control Fever of 5 or more days Presence of four of the following five conditions: • Bilateral conjunctival injection • Change(s)* in the mucous membranes of the upper respiratory tract, such as: Injected pharynx Injected lips Dry, fissured lips “Strawberry” tongue • Change(s)* of the peripheral extremities, such as: Peripheral edema Peripheral erythema Desquamation Periungual desquamation • Rash, primarily truncal; polymorphic but nonvesicular • Cervical lymphadenopathy Illness that cannot be explained by other known disease process * One is sufficient to establish criterion