other pediatric rheumatic diseases
TRANSCRIPT
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JA 2011 NATIONAL CONFERENCEOTHER PEDIATRIC RHEUMATIC DISEASES
Natasha M. Ruth, MD, MSMedical University of South CarolinaDivision of Pediatric RheumatologyFriday, July 8, 2011
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Objectives: Review clinical features (symptoms) of:
Systemic Lupus Erythematosus (SLE) Kawasaki’s Disease Henoch Schonlein Purpura Wegener’s Granulomatosus
Briefly discuss treatment for diseases listed above.
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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
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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.
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Types of Lupus Systemic Lupus Erythematosus (SLE) Discoid Lupus Drug-Induced Lupus Neonatal Lupus
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Types of Lupus
Systemic Lupus Erythematosus (SLE)
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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
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Systemic Lupus Erythematosus
Malar Rash, photosensitive
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Mouth ulcers in a patient with SLE
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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
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Digital angiitis in a patient with SLE
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Raynaud’s Phenomenon
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Raynaud’s Phenomenon Exaggerated vascular response to
cold temperature or emotional stress
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ACR Slide Collection
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Laboratory Evaluation Anti-nuclear antibody test (ANA) Other antibodies
Double-stranded DNA antibodies Smith antibodies RNP antibodies (SSA) Ro antibodies (SSB) La antibodies
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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
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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
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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
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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
Kawasaki’s Disease
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Most Concerning Complication Coronary Artery Aneurysms
Noted on echocardiogram
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Phases Acute (10 - 14 days)
Febrile phase Subacute (2 - 4 weeks)
↑PLT, Coronary aneurysms Convalescent (months - years)
Recovery phase
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Disease Course
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SKIN AND MUCOUS MEMBRANE
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STRAWBERRY TONGUE
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SKIN DESQUAMATION
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SKIN DESQUAMATION,
HAND
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EDEMA FEET
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Treatment IVIG Aspirin Corticosteroids and chemotherapeutics
for severe cases
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Henoch Schonlein Purpura (HSP) The most common vasculitis of
childhood.
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Henoch Schonlein Purpura (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.
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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.
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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.
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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.
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Clinical Manifestations of HSPRenal Involvement
Blood in urine Protein in urine High blood pressure Kidney insufficiency
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Treatment Time Corticosteroids for gastrointestinal
involvement Chemotherapeutics for severe disease
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Wegener’s Granulomatosis Inflammation of small blood vessels
leading to necrosis and granuloma formation of upper, lower respiratory tracts and kidneys
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Clinical Presentation Oto-rhino-laryngologic Pulmonary Renal Ocular Cutaneous Musculoskeletal GI GU Cardiac Nervous System
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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
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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
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Wegener’s granulomatosis
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Chest radiograph findings
Pulmonary nodules, cavitation
Consolidation, necrosis
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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)
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Wegener’s granulomatosis
Diagnosis Requires typical changes on biopsy (lung,
skin or kidney) Characteristic clinical presentation with
antibodies directed at neutrophil cytoplasmic antigens (cANCAs)
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Wegener’s granulomatosis Treatment
Steroids Other chemotherapeutic agents
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Juvenile Dermatomyositis
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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, 1994Symmons et al, Br J Rhem, 1995
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Inflammatory Muscle Disease
Proximal muscle weakness climbing stairs-“bus driver Dx” combing hair Gower’s sign neck flexor weakness
Muscle tenderness Symmetrical polyarthritis
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Rashes of JDM
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Periorbital erythema, edema, eyelid capillary telangiectasia
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Gottron’s papules/Gottron’s sign
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The heliotrope is a plant of the genus Heliotropium
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Nailfold capillary changes in JDM
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Gastrointestinal Involvement
Decreased esophageal motility Esophageal reflux Ulceration, perforation, hemorrhage
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Treatment Prednisone Methotrexate IVIG Chemotherapeutic agents for severe
cases
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QUESTIONS?