Collagen Vascular Disease in Children

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Dr. Maynard’s presentation on the types of collagen vascular disease in children (presented on 9/22/11).

Text of Collagen Vascular Disease in Children

Neuromuscular Diseases

Collagen Vascular Disease

in Children

Roy Maynard, M.D.

September 22, 2011

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Objectives

Define collagen vascular disease.

Identify 3 collagen vascular diseases that affect children.

Understand the role new biological modifying agents have on treating

collagen vascular disease.

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Definition

A diverse group of multi-system inflammatory disorders, affecting primarily collagen in the skin and joints. Autoantibody formation and other autoimmune dysfunction suggest a complex immune basis in the pathogenesis of the disease process which remains largely idiopathic

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Classification

Vasculitis

Arthritides

Connective tissue disease

Infectious diseases

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Collagen Vascular Disease

Systemic Lupus Erythematous

Dermatomyositis

Scleroderma

Juvenile Idiopathic Arthritis

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Epidemiology

150-200 children/million

0.015 to 0.02% of children

Female preponderance

Fairly uncommon disease in children

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Pathogenesis

Molecular mimicry: Infectious agents alter the surface markers of ones own cells leading to them being recognized as foreign by your immune system.

Autoimmunity: Idiopathic alterations in the basic immune system leading to the recognition of cells and tissues as foreign and subsequently attack as foreign.

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Treatment

Glucocorticsteroids

Immunosuppressives

Immunomodulators

Anti-inflammatory

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Systemic Lupus Erythematosus

Diagnosis

Malar rash

Discoid rash

Photosensitivity

Oral ulcers

Arthritis

Serositis (pleural or pericardial)

Renal (proteinuria)

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Systemic Lupus Erythematosus

Malar rash

http://www.emedicinehealth.com/script/main/art.asp?articlekey=107976 Accessed on 9/19/11

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Systemic Lupus Erythematosus

Epidemiology

0.36 to 0.4 children/100,000

Girls/boys (4:2 to 7:1)

Rare diagnosis before age 5

Peaks young adulthood

15-20% of adults started disease in childhood

Age 12 to 14 years when disease diagnosed

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Systemic Lupus Erythematosus

Diagnosis

Neurologic (seizures or psychosis)

Hematologic Hemolytic anemia

Leukopenia

Lymphopenia

Thrombocytopenia

Immunologic disorder Positive LE prep

Anti-dsDNA antibody

Anti-Smith antibody

False positive syphilis test

Antinuclear antibody, C3 and C4

Other organs Kidneys, lungs, cardiac

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Systemic Lupus Erythematosus

Prognosis

In 1950, only 30% survived 5 years

Now 5-year survival > 90%

Pediatric SLE has a worse prognosis than adult SLE

Risk factors for poor outcome include kidney disease, frequent flares, infections, neuropsychiatric manifestations

Lower physical and psychosocial than healthy children

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Systemic Lupus Erythematosus

Treatment

Hydroxychloroquine

Azathioprine

Glucocorticoids

NSAID

Chemotherapeutic agents Methotrexate, Cyclophosphamide

IVIG

Biologicals - rituximab

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Dermatomyositis

Autoimmune disease

Primarily affects skin and muscle

Incidence: under age 16, affects 3,000-5,000 children in U.S. 3 new cases per year/million people

Average age of onset is 7 (ages 5 10)

Found worldwide, more prevalent in North America

May be more common in African-Americans

Adult version presents age 50

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Dermatomyositis

Etiology idiopathic

Combination of genetics and infection

May be partially inherited (genetic predisposition to acquire the disease)

Not contagious

More common in females than males

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Dermatomyositis

Signs and symptoms:

Fever

Fatigue

Skin rash

Muscle weakness

Pain

Lung disease

Joint contractures

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Dermatomyositis

Heliotrope rash

http://www.pediatricsconsultantlive.com/pedsquiz/content/article/1803329/1691415 Accessed on 9/20/11

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Dermatomyositis

Diagnosis:

Clinical diagnosis

Increased CPK, aldolase, SGOT

MRI of involved muscles

Muscle biopsy

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Dermatomyositis

http://www.learningradiology.com/caseofweek/caseoftheweekpix2006/cow207lg.jpg Accessed on 9/20/11

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Dermatomyositis

http://www.nlm.nih.gov/medlineplus/ency/imagepages/1866.htm Accessed on 9/20/11

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Dermatomyositis

Treatment:

Prednisone

Methotrexate

Hydroxychloroquine

Cyclosporine

IVIG

Mycophenolate

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Dermatomyositis

Prognosis:

Most children go into remission within 2 years

Pulmonary involvement heralds poor prognosis

Increased risk for malignancy

Joint contractures are a long term complication

Some do not respond well to medications

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Scleroderma

Autoimmune disease

Normal tissues replaced with dense, thick scar tissue

Skin most common site

Kidneys, heart, lung, GI tract

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Scleroderma

5,000 7,000 children in U.S.

Only 1.5% develop disease before age 10

7% develop disease between age 10 to 19

2/3 are female

Immune system triggers other cells to produce excessive collagen

Idiopathic

Not contagious

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Pediatric Scleroderma

Two types:

Localized scleroderma Skin (linear and morphea)

Systemic scleroderma multiple organs

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Pediatric Scleroderma

Localized Scleroderma:

Most common form in children

Usually skin only, occasionally underlying muscle

Linear type appears as a band usually on extremities, may limit motion when crosses a joint

Morphea type - patches, waxy skin with an ivory or white color

Both appear white with purple borders

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Scleroderma

Signs and symptoms:

Loss of stretch in skin

Discoloration of skin

Thinning of the skin

Contractures of fingers

Joint inflammation

Raynauds phenomenon

Ulcers on fingertips

Dysphagia and other GI tract problems

Fatigue, muscle weakness

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Scleroderma

http://www.skinpatientalliance.ca/en/skin-conditions-diseases/connective-tissue-disorders Accessed on 9/20/11

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Scleroderma

Diagnosis:

Clinical diagnosis

Skin biopsy

Autoimmune blood testing

Swallow study

Lung testing

Cardiac echo

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Linear Scleroderma

http://bjo.bmj.com/content/91/10/1311/F1.large.jpg Accessed on 9/20/11

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Scleroderma

Treatment:

Prednisone

Methotrexate

NSAID

Environmental

Physical therapy

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Scleroderma

Prognosis:

Chronic and slowly progressive

Months to years

Localized vs. systemic

May stabilize without progression for years

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Juvenile Idiopathic Arthritis

Clinical manifestations: Fever

Rash

Serositis

Joint involvement pain, swelling, stiffness, limping

Growth delay/weight loss

Psoriasis

Uveitis, eye pain, vision disturbances

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Juvenile Idiopathic Arthritis

http://www.google.com/imgres?q=juvenile+idiopathic+arthritis&hl=en&sa=X&rlz=1R2ADFA_enUS410&tbm=isch&prmd=imvnsb&tbnid=27oK6oBuClFabM:&imgrefurl=http://www.isteroids.com/blog/canakinumab-effective-for-systemic-juvenile-

idiopathic-arthritis/&docid=L5cA1hDwO1EF4M&w=200&h=114&ei=gwN5TvKEJ8Watwey0cT2Dw&zoom=1&biw=1440&bih=730&ia

ct=rc&dur=225&page=1&tbnh=91&tbnw=160&start=0&ndsp=20&ved=1t:429,r:12,s:0&tx=89&ty=61)

Accessed on 9/19/11

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Juvenile Idiopathic Arthritis

Diagnosis: >6 weeks of arthritis Less than 16 years of age Unknown cause R/O Lymes, infection,

Kawasakis Disease, immunodeficiency

Epidemiology: Most common childhood chronic rheumatic

disease

Incidence: 2-20 cases/100,000 Prevalence: 16-150/100,000 0.074.01 per 1,000 children worldwide

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Juvenile Idiopathic Arthritis

(ROYCLARIFY WEBSITE REFERENCE FROM ORIGINAL SLIDE #38)

Accessed on 9/19/11

http://trialx.com/curetalk/wp-content/blogs.dir/7/files/2011/05/diseases/Juvenile_Idiopathic_Arthritis-1.jpg Accessed on 9/20/11

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Juvenile Idiopathic Arthritis

Drug Des Devel Ther. 2011; 5:61-70

Systemic 5-10

Oligoarthritis 40-50 Persistent 25-35 Extended 15-20

Polyarthritis