Connective Tissue Disorders

Question 1

Burn Def

Answer 1

Permanent destruction of tissue proteins by an external agent

Question 2

Etiology of Burns

Answer 2

Thermal: scald, direct flame, contact, explosion
Chemical
Electrical

Question 3

1st Degree Burn

Answer 3

Superficial epidermis
Redness
No blistering
Painful 1-3 days
No scarring

Question 4

2nd Degree Burn (Superficial dermis)

Answer 4

Superficial partial thickness
Painful red blisers
Heals 7-14 days
Possible permanent scarring

Question 5

2nd Degree Burn (Deep reticular dermis)

Answer 5

Deep partial thickness
Painful, red, blisters
Possible white eschar
Hperemia
Scarring likely 
3-4 weeks

Question 6

3rd Degree Burn

Answer 6

Full thickness
Muscle, tendon or bone
Non-painful, white, brown, black or red
Possible amputation
Severe scarring
Grafting required
Heals weeks to months

Question 7

% of total body surface area (TBSA)

Answer 7

Rule of 9’s
9% for each arm and head
18% for each leg, anterior trunk and posterior trunk

Question 8

Burns that warrant hospitalization

Answer 8

2nd deg >10% BSA
3rd deg >2% BSA
Significant burns of hands, feet, face, joints or perineum
Self inflicted
Circumferential may predispose to vascular compromise
Explosion, inhalation or chemical burns may have organ trauma

Question 9

Classic desired position for burn healing

Answer 9

Neck extended
Shoulders ABD to 90 deg
Elbows ext
Forearms supinated
Wrist 15-20 degvext
Palms up
Fingers ext
Hips ABD 10-15 deg
Knees ext
Ankles dorsiflexed

Question 10

Dermatomyositis

Answer 10

Inflammatory myopathy with characteristic skin rashes

Question 11

Dermatomyositis Clinical Features

Answer 11

Proximal symmetric muscle weakness
Heliotrope rash and gottrons papules
Shawl sign
Calcinosis

Question 12

Dermatomyositis labs

Answer 12

Inc CK, adolase or SGOT, LDH

Question 13

EMG findings in Dermatomyositis

Answer 13

Fibrillations
Positive sharp waves
Complex repetitive discharges
Short duration 
Low amp polyphasic motor unit potentials

Question 14

Muscle bx in Dermatomyositis

Answer 14

Perivasicular and interfasicular infiltrates with fiber degeneration and regeneration

Question 15

Tx of Dermatomyositis

Answer 15

Oral/IV steroids
MTX to shorten steroid course
Hydroxychloroquine for heliotrope rash
Diltiazem for Calcinosis

Question 16

Juvenile rheumatoid/idiopathic arthritis (JR/IA)

Answer 16

Group of dz with chronic joint inflammation

Question 17

Pauciarticular JRA

Answer 17

4 or

Question 18

Pathogensesis of JRA

Answer 18

Chronic synovial inflammation with B lymphocytes
Macrophage and T lymphocyte invasion and cytokines release with further synovial proliferation
Pannus l/t joint destruction

Question 19

CF of Pauciarticular JRA

Answer 19

Typically larger joints (knees, wrist, ankles)

Affected knee often with quad muscle wasting, limping and flexing contracture

Question 20

Natural history of Pauciarticular JRA

Answer 20

Persistent swelling l/t progessivs muscle wasting, weakness around joint and muscle contracture

Question 21

Presentation of Pauciarticular JRA

Answer 21

Morning stiffness presents with dec play activity in the toddler
Prominent limping with stiffness, weakness and contracture a
Joint fullness and warmth

Question 22

Polyarticular JRA

Answer 22

Joint inflammation in 5 or more joints within first 6 mo of dz onset

Question 23

CF of Polyarticular JRA

Answer 23

Symmetrical arthritis of small joints (hands, feet, jaw and cervical spine)

Question 24

Natural history of Polyarticular JRA

Answer 24

Rapid progression of weakness and contractures possible
Pain can be severe
Decline in activity
Loss of joint movement

Question 25

Systemic JRA

Answer 25

Joint pain +/- swelling with associated spiking fever and evanescent rash

Question 26

CF of Systemic JRA

Answer 26

``` Daily or twice daily fever Fleeting linear rash during fever on trunk/extremities Athralgia worse during fever Generalized myalgia Possible panserositis Hepatosplenomegaly Joint inflammation Wt loss, nausea, fatigue Sx present for at least 6 weeks ```

Question 27

Pathogenesis of Kawasaki Dz

Answer 27

Severe vasculitis of all BV, predominantly affecting medium sized arteries with coronary artery predilection

Question 28

CF of Kawasaki Dz

Answer 28

``` High fever up to 104 for 1-2 wk Conjunctivitis strawberry tongue dry cracked lips Rash Enlarged LN Edema of hands and feet ```

Question 29

Tx of Kawasaki Dz

Answer 29

IVIG w/in first 10 days of illness to dec risk of cardiac aneurysm High dose ASA Plasmapheresis if unresponsive to tx

Question 30

Cause of Lyme Dz

Answer 30

Spirochete, Borrelia burgdorferi, transmitted by tick Ixodes

Question 31

Cause of Babesiosis

Answer 31

Babesia microti | Can occur as confection with Lyme dz

Question 32

Early Localized Lyme Dz

Answer 32

``` Annular rash: erythema migrans 7-14 days after bite Uniform or target rash Fever, malaise, myalgia and HA Other: aseptic meningitis, carditis w/ heart block, papilledema, uveitis, focal neuro findings ```

Question 33

Late Lyme Dz

Answer 33

``` Weeks to months after infection Arthritis of large joints Tertiary neurobirreliosis Encephalitis Polyneuritis Memory problems ```

Question 34

Lyme dz labs

Answer 34

``` IfM Ab inc at 6-8 wk ELISA against Ab to B burgdorferi Western blot Inc WBC, ESR, CSF protein Cultures takes 4 wk ```

Question 35

Treatment of Lyme Dz

Answer 35

Doxycycline is DOC | Cefuroxime or ceftriaxone for children

Question 36

Rheumatic Fever

Answer 36

Multisystem inflammatory dz 2-3 wk after group A hemolytic strep infection

Question 37

Cause of Rheumatic Fever

Answer 37

Ab cross reactivity in the heart, skin, joints and brain | Ab formed against M proteinin group A strep

Question 38

Jones Major Criteria

Answer 38

``` Cardiac involvement (pancarditis, murmur, CHF) Migratory polyarthritis Erythema marginatum Subcutaneous nodules Sydenham's chorea ```

Question 39

Rheumatic Fever Labs

Answer 39

ASO titers DNase B (streptozyme) Inc CRP, ESR and WBC

Question 40

Rheumatic Fever Tx

Answer 40

Penicillin | Prednisone for carditis/CHF

Question 41

Septic Arthritis

Answer 41

infection of a joint d/t bactermia

Question 42

MC joints involved in Septic Arthritis

Answer 42

Hips>Knees>SI joint in Peds

Question 43

Systemic Lupus Erythematosus CF

Answer 43

``` Joint pain (fingers, hands, wrists, knees) Arthritis Pericarditis, myocarditis, endocarditis Fatigue and malaise Pulmonary dz Fever Skin rash (malar) Seizures ```

Question 44

SLE Labs

Answer 44

ANA Anti-ds DNA Antiphospholipid Ab Ani-Smith Ab