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39 Cards in this Set

  • Front
  • Back

Systemic IgA mediated vasculitis involving the skin, GI, kidneys

Henoch Schonlein Purpura

Henoch Schonlein Purpura
1. incidence/gender
2. presentation
3. stereotypical purpura occurence
4. Organs Affected (3)
5. Plt Count

4.

1. median 5 yoa, male
2. viral/URI prodrome
3. buttocks and Lower Extrem.
4. Skin/GI/Renal
5. NOT Thrombocytopenic

Most common cause of acquired heart disease in children in US

Kawaski Dz (unknwon origin)

Kawaski Dz
1. incidence/Ethnic/Gender
2. Treatment

1. 18-24mo, Asia, Male
2. IVIG, ASA (high acute/low subacute)

What are the Diagnostic Criteria for Kawaski's Dz

1. Fever (>102) for >5d
2. 4/5 bilateral conjunctivitis nonexudative, oropharnygeal changes, cercial adenopathy, variable rash, changes in distal extremities (acute edema+induration, later peeling around nail bed)
3. Cannot be explained otherwise

2 Risk Factors of Kawaski Dz

Coronary Artery Aneurysm around 7-14 days (20%), Hydrops of gallbladder (10%)

Most common pediatric rheumatic disease with arthritis

Juvenile RA

JRA
1. onset/gender
2. Types (3)

1. 1-3yoa, females except systemic (even) or late onset pauciarticular (males)
2. Pauciarticular, Polyarticular, Systemic Onset

JRA Pauciarticular
1. early onset dxn (3)
2. late onset dxn

(<4 joints involved)
1. early onset: female, +ANA, risk for chronic uveitis
2. late onset: male, HLA-B27 +, hips and SI joint ivolved

JRA Polyarticular
1. subtypes

(> 4 joints involved)
1. Serum RF (+ more severe)

JRA Systemic Onset (Still's Disease)

High spiking fevers with nonpruritic evanescent salmon colored rash and ANA negative

4 Common skin findings in SLE

malar rash, photosensitive, alopecia, Raynaud's phenomenon

T/F - SLE often causes joint deformity or erosion.

False

Hematolic Manifestations of SLE.

leukopenia, thrombocytopenia, AOCD

3 Rheumatologic markers of SLE

1. ANA, 2.anti-dsDNA, 3.anti-Smith

Diagnostic Criteria for SLE

SOAP BRAIN MD
Serositis, Oral ulcers, Arthritis, Photosens., Blood cytopenias, Renal Dz, ANA +, Imunoserology abn, neurologic symp., malar rash, discoid lupus

Dermatomyositis
1. incidence/gender
2. Cutaneous findings
3. Positive Clinical Sign?
4. Treatment

1. 5-14yoa/female
2. heliotrope periorbital rash and papules over knuckles
3. Gower's sign, climb up legs (proximal muscle weakness)
4. Corticosteroids are mainstay

T/F - There is no association with malignancy in pedaitric dermatomyositis.

True

T/F - The strains of streptococci that cause impetigo can not cause rheumatic fever

True

Endocarditis, most common finding of rheumatic fever, can cause insuffiency of the _.

Left sided valves

Describe the arthritis of rheumatic fever.

polyarthritis, migratory, asymmetric and exquistely painful but does not result in chronic joint dz

What skin Physical exam finding is associate with severe cardiac involvement in rheumatic disease?

subcutaneous nodules

Jones criteria for Rheumatic Fever

Evidence of recent strep infection and either 2 major or 1 major + 2 minor

Major
1) migratory polyarthritis
2)carditis
3) sydenham's chorea
4) erythema marginatum
5) Subcutaneous nodules

Minor
1) Fever
2) Arthralgia
3) previous rh. fever
4) leukocytosis
5) elevated ESR or CRP
6) prolonged PR on ECG

Rheumatic Fever Treatment

1) Benzathine Penicillin IM (once) (OR) Pen Oral for 10 days
2) NSAIDs only AFTER diagnosis (masking)
3) Often Steroids
4) if chorea, haloperidol
5) if CHF, diuretic, salt restriction, digoxin
6) long term abx prophylaxis

Organism of Lyme Dz, vector

Borrelia burgdorferi via Ixodes scapularis (deer tick)

How long must the tick be on for lyme Dz to transmit?

36-48 hours

Describe the classic skin manifestation of Lyme Dz.

Erythema migrans which is annular and targetlike with central clearing

The cardio finding of lyme dz when found (rare)

heart block

Hallmark of late disease lyme disease

arthritis

Laboratory test for lyme dz.

ELISA and Western blot

Treatment of Lyme Dz.

Arthritis only
1) doxycycline or amoxicillin

Carditis/Meningitis
1)Ceftriaxone or Penicillin

Triad of Arthritis, Urethritis, and Conjunctivitis? Often triggered by _?

Reiter's Disease via Chlamydia

HLA B27 Male arthritis of LE and axial skeleton

Ankylosing spondylitis

Asian female with aneurysmal dilation or thrombosis of the large vessels, vasculitis

Takayasu's arteritis

Small-medium vessel sized aneurysms and thrombosis vascultis with skin manifestations

Polyarteritis nodosa

Granulomatis vasculitis affecting kidneys and lungs with sinusitis, hemoptysis and GN

Wegener's granulomatosis

Triad of Wegner'sq

sinusitis, hemoptysis and GN

Triad of Sjogren's Syndrome

Sicca (dry eyes/mouth), high autoABs, and CT dz

Define syndrome related to less extensive scleroderma

CREST: calcinosis, Raynaud's phenomenon, esophageal involvement, sclerosis, telangiectasias