1. JA 2011 National ConferenceOther Pediatric Rheumatic DIseases Natasha M. Ruth, MD, MS Medical University of South Carolina Division of Pediatric Rheumatology Friday, July 8, 2011

2. Objectives: Review clinical features (symptoms) of: Systemic Lupus Erythematosus (SLE) Kawasaki’s Disease HenochSchonleinPurpura Wegener’s Granulomatosus Briefly discuss treatment for diseases listed above.

3. What is Lupus? Autoimmune disease Etiology is unknown combination of genetics (heredity), environment, and hormones is likely involved Autoantibodies Causes the immune system to attack the body’s own tissue and organs including: joints, kidneys, heart, lungs, brain, blood and skin

4. How common is Lupus? At least 1.5 million Americans have lupus. 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. It is believed that 5 million people throughout the world have a form of lupus. Lupus strikes mostly women of childbearing age (15-44). However, men, children, and teenagers develop lupus, too. Women of color are 2-3 times more likely to develop lupus. People of all races and ethnic groups can develop lupus. More than 16,000 new cases of lupus are reported annually across the country.

5. Types of Lupus Systemic Lupus Erythematosus (SLE) Discoid Lupus Drug-Induced Lupus Neonatal Lupus

6. Types of Lupus Systemic Lupus Erythematosus (SLE)

8. How is SLE diagnosed? Many symptoms of SLE mimic other diseases Symptoms may come and go and are often vague History, PE and laboratory evaluation

9. Systemic Lupus Erythematosus Malar Rash, photosensitive

11. Mouth ulcers in a patient with SLE

12. Early vasculitic lesions over the tips of the toes in a patient with active SLE Gangrene of the toe in a patient with SLE and vasculitis

13. Digital angiitis in a patient with SLE

14. Raynaud’s Phenomenon

15. Raynaud’s Phenomenon Exaggerated vascular response to cold temperature or emotional stress

16. ACR Slide Collection

17. Laboratory Evaluation Anti-nuclear antibody test (ANA) Other antibodies Double-stranded DNA antibodies Smith antibodies RNP antibodies (SSA) Ro antibodies (SSB) La antibodies

18. Childhood Onset LupusA Unique Subgroup Approximately one fourth of all systemic lupus cases start during childhood Most cases occur after age 5; peak incidence during late childhood and adolescence Cassidy and Petty,4th edition

19. Childhood Onset LupusA Unique Subgroup Females are affected more than males, particularly after puberty 0-9 yo: F:M is 4:3 10-14 yo: F:M is 4:1 15-19 yo: F:M is 5:1 Kidney disease and central nervous system dysfunction are more common in childhood onset SLE Cassidy and Petty,4th edition

21. Lupus Maintenance THE IDEAL LUPUS PILL Anti-inflammatory Immunosuppressive Anti-thrombotic Cholesterol Lowering Steroid-sparing Prevents Osteoporosis Cheap McCune et al, New Therapies in SLE, Snowmass, 2005

22. Kawasaki’s Disease Fever>5 days Limbic sparing conjuctival injection (can also get anterior uveitis) Red, chapped, or cracked lips Red mucous membranes in the mouth Strawberry tongue, white coating on the tongue or prominent red bumps on the back of the tongue Red palms of the hands and the soles of the feet Peeling (desquamation) palms and soles (later in the illness); peeling may begin around the nails, groin. Rash which may take many forms, non-specific, polymorphic, non-itchy Swollen lymph nodes (frequently only one lymph node is swollen, and is usually on one side), particularly in the neck area >1.5cm). Joint pain and swelling, frequently symmetrical Irritability

23. Most Concerning Complication Coronary Artery Aneurysms Noted on echocardiogram

24. Phases Acute (10 - 14 days) Febrile phase Subacute (2 - 4 weeks) ↑PLT, Coronary aneurysms Convalescent (months - years) Recovery phase

25. Disease Course

27. Skin and mucous membrane

28. Strawberry tongue

29. Skin desquamation

30. Skin desquamation, hand

31. Edema feet

32. Treatment IVIG Aspirin Corticosteroids and chemotherapeutics for severe cases

33. HenochSchonleinPurpura (HSP) The most common vasculitis of childhood.

34. HenochSchonleinPurpura (HSP) Inflammation of small blood vessels Deposition of immune complexes (IgA) in vessel walls Affects arterioles, capillaries, and post capillary venules; especially those in the skin, gut, and kidney.

35. Clinical Manifestations of HSPSkin Involvement Classically described as “palpable purpura”; commonly also petechial. Typically the rash doesn’t itch, but early in the course there can be an urticarial component. Tends to occur on buttocks, lower extremities. Described as gravity/pressure dependent.

38. Clinical Manifestations of HSPJoint Involvement Characterized by pain, swelling, and decreased range of motion of joints; tends to be peri-articular. HSP most commonly affects knees/ankles, although wrist, elbow, finger, toe involvement also common. May also see subcutaneous non-pitting edema, especially on dorsum of hands/feet, periorbitally.

39. Clinical Manifestations of HSPGI Involvement Diffuse abdominal pain/tenderness, can be severe and colicky. Due to bowel wall edema/bleeding. May also see hematemesis/melena.

40. Clinical Manifestations of HSPRenal Involvement Blood in urine Protein in urine High blood pressure Kidney insufficiency

41. Treatment Time Corticosteroids for gastrointestinal involvement Chemotherapeutics for severe disease

42. Wegener’s Granulomatosis Inflammation of small blood vessels leading to necrosis and granuloma formation of upper, lower respiratory tracts and kidneys

43. Clinical Presentation Oto-rhino-laryngologic Pulmonary Renal Ocular Cutaneous Musculoskeletal GI GU Cardiac Nervous System

44. Wegener’s granulomatosis Incidence ? 5 year incidence was 3.2 cases per 100,000 Disease onset before 20 yo accounted for 3.3%, giving an incidence in that age group of 0.1 cases per 100,000 Mean age of onset 15.4 years No clear sex predominance in peds (M>F in adults) Cotch MF and Hoffman GS et al, A & R, 39: 87-92, 1996

45. Wegener’s granulomatosis Sinus involvement with inflammation of nasal and tracheal cartilage Lung disease Cough, shortness of breath, coughing up blood Nodular pulmonary infiltrates Eye problems: blurred vision, eye pain, conjunctivitis, episcleritis, Hearing problems: persistent otitis media, hearing loss Joint and muscle pain Kidney disease May lead to kidney failure Skin disease May resemble HSP Can be ulcerative, vesicular, purpular or nodular

46. Wegener’s granulomatosis

47. Chest radiograph findings Pulmonary nodules, cavitation Consolidation, necrosis

48. Clinical manifestationsRenal involvement Kidney Disease in 70-85% Usually preceded by non-kidney related ssymptoms for months Urine abnormal revealing blood and protein Variable onset and course Sometimes indolent (progression over months to years) Sometimes rapidly progressive (ESRD within days to weeks)

49. Wegener’s granulomatosis Diagnosis Requires typical changes on biopsy (lung, skin or kidney) Characteristic clinical presentation with antibodies directed at neutrophilcytoplasmic antigens (cANCAs)

50. Wegener’s granulomatosis Treatment Steroids Other chemotherapeutic agents

51. Juvenile Dermatomyositis

52. Epidemiology Bimodal peak 5-14 years, 45-64 years Incidence 1-3.2/million, Caucasian 7.7/million, African-American Pelkonen et al, J Rheum, 1994 Symmons et al, Br J Rhem, 1995

53. Inflammatory Muscle Disease Proximal muscle weakness climbing stairs-“bus driver Dx” combing hair Gower’s sign neck flexor weakness Muscle tenderness Symmetrical polyarthritis

54. Rashes of JDM

55. Periorbital erythema, edema, eyelid capillary telangiectasia

56. Gottron’s papules/Gottron’s sign

59. The heliotrope is a plant of the genus Heliotropium

62. Nailfold capillary changes in JDM

63. Gastrointestinal Involvement Decreased esophageal motility Esophageal reflux Ulceration, perforation, hemorrhage

66. Treatment Prednisone Methotrexate IVIG Chemotherapeutic agents for severe cases

67. QUESTIONS?