Other Pediatric Rheumatic DIseases


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Other Pediatric Rheumatic DIseases

  1. 1. JA 2011 National ConferenceOther Pediatric Rheumatic DIseases<br />Natasha M. Ruth, MD, MS<br />Medical University of South Carolina<br />Division of Pediatric Rheumatology<br />Friday, July 8, 2011<br />
  2. 2. Objectives:<br />Review clinical features (symptoms) of:<br />Systemic Lupus Erythematosus (SLE)<br />Kawasaki’s Disease<br />HenochSchonleinPurpura<br />Wegener’s Granulomatosus<br />Briefly discuss treatment for diseases listed above.<br />
  3. 3. What is Lupus?<br />Autoimmune disease<br />Etiology is unknown<br />combination of genetics (heredity), environment, and hormones is likely involved <br />Autoantibodies<br />Causes the immune system to attack the body’s own tissue and organs including:<br />joints, kidneys, heart, lungs, brain, blood and skin<br />
  4. 4. How common is Lupus?<br />At least 1.5 million Americans have lupus. <br />The actual number may be higher; however, there have been no large-scale studies to show the actual number of people in the U.S. living with lupus. <br />It is believed that 5 million people throughout the world have a form of lupus. <br />Lupus strikes mostly women of childbearing age (15-44). However, men, children, and teenagers develop lupus, too. <br />Women of color are 2-3 times more likely to develop lupus. <br />People of all races and ethnic groups can develop lupus. <br />More than 16,000 new cases of lupus are reported annually across the country. <br />
  5. 5. Types of Lupus<br />Systemic Lupus Erythematosus (SLE)<br />Discoid Lupus<br />Drug-Induced Lupus<br />Neonatal Lupus<br />
  6. 6. Types of Lupus<br />Systemic Lupus Erythematosus (SLE)<br />
  7. 7.
  8. 8. How is SLE diagnosed?<br />Many symptoms of SLE mimic other diseases<br />Symptoms may come and go and are often vague<br />History, PE and laboratory evaluation<br />
  9. 9. Systemic Lupus Erythematosus<br />Malar Rash, photosensitive<br />
  10. 10.
  11. 11. Mouth ulcers in a patient with SLE<br />
  12. 12. Early vasculitic lesions <br />over the tips of the toes in a patient with active SLE<br />Gangrene of the toe in a patient with SLE and vasculitis<br />
  13. 13. Digital angiitis in a patient with SLE<br />
  14. 14. Raynaud’s Phenomenon<br />
  15. 15. Raynaud’s Phenomenon<br />Exaggerated vascular response to cold temperature or emotional stress<br />
  16. 16. ACR Slide Collection<br />
  17. 17. Laboratory Evaluation<br />Anti-nuclear antibody test (ANA)<br />Other antibodies<br />Double-stranded DNA antibodies<br />Smith antibodies<br />RNP antibodies<br />(SSA) Ro antibodies<br />(SSB) La antibodies<br />
  18. 18. Childhood Onset LupusA Unique Subgroup<br />Approximately one fourth of all systemic lupus cases start during childhood<br />Most cases occur after age 5; peak incidence during late childhood and adolescence<br />Cassidy and Petty,4th edition<br />
  19. 19. Childhood Onset LupusA Unique Subgroup<br />Females are affected more than males, particularly after puberty<br /> 0-9 yo: F:M is 4:3<br /> 10-14 yo: F:M is 4:1<br /> 15-19 yo: F:M is 5:1<br />Kidney disease and central nervous system dysfunction are more common in childhood onset SLE<br />Cassidy and Petty,4th edition<br />
  20. 20.
  21. 21. Lupus Maintenance<br />THE IDEAL LUPUS PILL<br />Anti-inflammatory<br />Immunosuppressive<br />Anti-thrombotic<br />Cholesterol Lowering<br />Steroid-sparing<br />Prevents Osteoporosis<br />Cheap<br />McCune et al, New Therapies in SLE,<br />Snowmass, 2005<br />
  22. 22. Kawasaki’s Disease<br />Fever>5 days<br />Limbic sparing conjuctival injection (can also get anterior uveitis)<br />Red, chapped, or cracked lips <br />Red mucous membranes in the mouth<br />Strawberry tongue, white coating on the tongue or prominent red bumps on the back of the tongue<br />Red palms of the hands and the soles of the feet<br />Peeling (desquamation) palms and soles (later in the illness); peeling may begin around the nails, groin.<br />Rash which may take many forms, non-specific, polymorphic, non-itchy<br />Swollen lymph nodes (frequently only one lymph node is swollen, and is usually on one side), particularly in the neck area >1.5cm).<br />Joint pain and swelling, frequently symmetrical<br />Irritability<br />
  23. 23. Most Concerning Complication<br />Coronary Artery Aneurysms<br />Noted on echocardiogram<br />
  24. 24. Phases<br />Acute (10 - 14 days)<br />Febrile phase<br />Subacute (2 - 4 weeks)<br />↑PLT, Coronary aneurysms <br />Convalescent (months - years)<br />Recovery phase<br />
  25. 25. Disease Course<br />
  26. 26.
  27. 27. Skin and mucous membrane<br />
  28. 28. Strawberry tongue<br />
  29. 29. Skin desquamation<br />
  30. 30. Skin desquamation, hand<br />
  31. 31. Edema feet<br />
  32. 32. Treatment<br />IVIG<br />Aspirin<br />Corticosteroids and chemotherapeutics for severe cases<br />
  33. 33. HenochSchonleinPurpura (HSP)<br />The most common vasculitis of childhood.<br />
  34. 34. HenochSchonleinPurpura (HSP)<br />Inflammation of small blood vessels<br />Deposition of immune complexes (IgA) in vessel walls<br />Affects arterioles, capillaries, and post <br /> capillary venules; especially those in the skin, gut, and kidney.<br />
  35. 35. Clinical Manifestations of HSPSkin Involvement<br />Classically described as “palpable purpura”; commonly also petechial.<br />Typically the rash doesn’t itch, but early in the course there can be an urticarial component.<br />Tends to occur on buttocks, lower extremities.<br />Described as gravity/pressure dependent.<br />
  36. 36.
  37. 37.
  38. 38. Clinical Manifestations of HSPJoint Involvement<br />Characterized by pain, swelling, and decreased range of motion of joints; tends to be peri-articular.<br />HSP most commonly affects knees/ankles, although wrist, elbow, finger, toe involvement also common.<br />May also see subcutaneous non-pitting edema, especially on dorsum of hands/feet, periorbitally.<br />
  39. 39. Clinical Manifestations of HSPGI Involvement<br />Diffuse abdominal pain/tenderness, can be severe and colicky.<br />Due to bowel wall edema/bleeding.<br />May also see hematemesis/melena.<br />
  40. 40. Clinical Manifestations of HSPRenal Involvement<br />Blood in urine<br />Protein in urine<br />High blood pressure<br />Kidney insufficiency<br />
  41. 41. Treatment<br />Time<br />Corticosteroids for gastrointestinal involvement<br />Chemotherapeutics for severe disease<br />
  42. 42. Wegener’s Granulomatosis<br />Inflammation of small blood vessels leading to necrosis and granuloma formation of upper, lower respiratory tracts and kidneys<br />
  43. 43. Clinical Presentation<br />Oto-rhino-laryngologic<br />Pulmonary<br />Renal<br />Ocular<br />Cutaneous<br />Musculoskeletal<br />GI<br />GU<br />Cardiac<br />Nervous System<br />
  44. 44. Wegener’s granulomatosis<br />Incidence ?<br />5 year incidence was 3.2 cases per 100,000<br />Disease onset before 20 yo accounted for 3.3%, giving an incidence in that age group of 0.1 cases per 100,000<br />Mean age of onset 15.4 years<br />No clear sex predominance in peds (M>F in adults)<br />Cotch MF and Hoffman GS et al, A & R, 39: 87-92, 1996<br />
  45. 45. Wegener’s granulomatosis<br />Sinus involvement with inflammation of nasal and tracheal cartilage<br />Lung disease<br />Cough, shortness of breath, coughing up blood<br />Nodular pulmonary infiltrates<br />Eye problems: blurred vision, eye pain, conjunctivitis, episcleritis, <br />Hearing problems: persistent otitis media, hearing loss<br />Joint and muscle pain<br />Kidney disease<br />May lead to kidney failure<br />Skin disease<br />May resemble HSP<br />Can be ulcerative, vesicular, purpular or nodular<br />
  46. 46. Wegener’s granulomatosis<br />
  47. 47. Chest radiograph findings<br />Pulmonary nodules, cavitation<br />Consolidation, necrosis<br />
  48. 48. Clinical manifestationsRenal involvement<br />Kidney Disease in 70-85%<br />Usually preceded by non-kidney related ssymptoms for months<br />Urine abnormal revealing blood and protein<br />Variable onset and course<br />Sometimes indolent (progression over months to years)<br />Sometimes rapidly progressive (ESRD within days to weeks)<br />
  49. 49. Wegener’s granulomatosis<br />Diagnosis<br />Requires typical changes on biopsy (lung, skin or kidney)<br />Characteristic clinical presentation with antibodies directed at neutrophilcytoplasmic antigens (cANCAs)<br />
  50. 50. Wegener’s granulomatosis<br />Treatment<br />Steroids<br />Other chemotherapeutic agents<br />
  51. 51. Juvenile Dermatomyositis<br />
  52. 52. Epidemiology<br />Bimodal peak 5-14 years, 45-64 years<br />Incidence <br />1-3.2/million, Caucasian<br />7.7/million, African-American<br />Pelkonen et al, J Rheum, 1994<br />Symmons et al, Br J Rhem, 1995<br />
  53. 53. Inflammatory Muscle Disease<br />Proximal muscle weakness<br />climbing stairs-“bus driver Dx”<br />combing hair<br />Gower’s sign<br />neck flexor weakness<br />Muscle tenderness<br />Symmetrical polyarthritis<br />
  54. 54. Rashes of JDM<br />
  55. 55. Periorbital erythema, edema, eyelid capillary telangiectasia<br />
  56. 56. Gottron’s papules/Gottron’s sign<br />
  57. 57.
  58. 58.
  59. 59. The heliotrope is a plant of the genus Heliotropium<br />
  60. 60.
  61. 61.
  62. 62. Nailfold capillary changes in JDM<br />
  63. 63. Gastrointestinal Involvement<br />Decreased esophageal motility<br />Esophageal reflux<br />Ulceration, perforation, hemorrhage<br />
  64. 64.
  65. 65.
  66. 66. Treatment<br />Prednisone<br />Methotrexate<br />IVIG<br />Chemotherapeutic agents for severe cases<br />
  67. 67. QUESTIONS?<br />