Kaplan Chapter 18 - Rheum

Front 1

Juvenile RA: Cause

Back 1

Immuogenic susceptibility + External triggers

*Otherwise thought to be AI

Front 2

Juvenile RA: General Findings

Back 2

Chronic, nonsuppurative inflammation of synovium

Front 3

Juvenile RA: 3 Key characteristics

Back 3

1. Joint effusions
2. Destruction of joint cartilage
3. Bone deformity, destruction, fusion

Front 4

Juvenile RA: Clinical Presentation:

Back 4

Key: Morning stiffness
- Good days, bad days
- Easy fatigability
- Warm joints, pain with ROM but NO erythema

Front 5

Juvenile RA: Dx

Back 5

CLINICAL

Front 6

Juvenile RA: Criterai

Back 6

- Onset < 16y
- Arrthritis OR ≥ 2 of: Limited ROM, tenderness pain on motion, increased heat in 1+ joints
- Duration over 6w
- Dx of exclusion (no other forms arthritis)

Front 7

Juvenile RA: What factor defines what 'type'?

Back 7

How it presents first 6 months

Front 8

Juvenile RA: DDx

Back 8

- SLE*
- Juvenile Dermatomyositis
- Sarcoid
- Scleroderma
- Rheu Fever*
- Vasculitis
- AI Hepatitis
- Late stage Lyme*
- Psoriatic arthritis
- Arthritis w/ IBD
- Lymphoproliferative disease*

Front 9

Juvenile RA: Types

Back 9

1. Pauciarticular
2. Polyarticular
3. Systemic onset

Front 10

Juvenile RA: Pauciarticular Defn

Back 10

< 5 joints
Lower extremity; NEVER hip
Larger joints

Front 11

Juvenile RA: Polyarticular Defn

Back 11

≥ 5 joints
Resembles nl adult arthritis
Rheumatoid nodules common
May involve cervical spine

Front 12

Juvenile RA: Systemic Onset Defn

Back 12

1. Arthritis and prominant visceral involvement
2. Daily temp spikes at least 39° for 2+ weeks
- E.g. present w/ FUO
3. Characteristic salmon-colored evanescent rash

Front 13

Juvenile RA: Systemic Onset visceral involvement?

Back 13

- Hepatosplenomegaly
- LAD
- Serositis

Front 14

Juvenile RA: Rash type?

Back 14

Fairly non-specific
Salmon colored
Comes and goes WITH fever (i.e. evanescent)

Front 15

Juvenile RA: Lab Findings

Back 15

- NO BEST TEST (Dx of exclusion
- High acute phase reactants
- Anemia of CD
- ANA in 40-85% --> More w/ poly/pauciarticular
- RF+ in some

Front 16

Juvenile RA: RF+ more likely in what cases?

Back 16

Older Kids
Polyarticular
Rheumatoid nodules

Front 17

Juvenile RA: Mgmt Goal

Back 17

Preserve Joint Function

Front 18

Juvenile RA: Mgmt Lines

Back 18

- NSAIDs first line
- Methotrexate
- Steroids --> Maybe but few indications
- PT/OT
- Optho follow-up

Front 19

Juvenile RA: When to use steroids?

Back 19

- Overwhelming inflammation
- Systemic illness
- Bridge therapy

Front 20

Juvenile RA: Optho problems?

Back 20

Characteristic iridocyclitis

Front 21

Juvenile RA: Which Form has excellent prognosis?

Back 21

Pauciarticular --> ANA+ --> Younger girls (except when eyes involved...)

Front 22

Juvenile RA: Which forms have Poor Prognosis?

Back 22

- Any RF+ (Poly or pauci)
- Erosions, nodules, unremitting course bad signs
- Polyarticular Involvement + Systemic also bad

Front 23

Juvenile RA: Other "Good" but not "Excellent" prognostic signs?

Back 23

- Seronegative (ANA and RF)
- HLA B27 (Typically older males, Pauciarticular)

Front 24

SLE: HLA Associations

Back 24

B8, DR2, DR3

Front 25

SLE: Exacerbating factor to know

Back 25

Sunlight
Infectious agents

Front 26

SLE: Drug Causes to know (Drug-Induced forms)

Back 26

- Anticonvulsants
- Sulfonamides
- Antiarrhythmics

Front 27

SLE: Age Range

Back 27

FEMALES
> 8y

Front 28

SLE: Most frequent presentation

Back 28

Fever + Fatigue + Arthralgia + Arthritis + Malar Rash

Front 29

SLE: Types of Rashes

Back 29

Malar
Discoid
Livedo reticularis

*All of these are photosensitive

Front 30

SLE: Renal problems

Back 30

- GN
- Nephrotic Syndrome
- HTN
- Renal Failure

Front 31

SLE: Endocarditis Type

Back 31

Libman Sacks

Front 32

SLE: Criteria (4/11) Pneumonic

Back 32

MD SOAP N HAIR:

Front 33

MD SOAP N HAIR

Back 33

Malar Rash, Discoid Rash, Serositis, Oral ulcers, ANA+, Photosensitive, Neurologic D/o, Hematologic d/o, Arthritis, Immune d/o (LE prep, Anti-DNA, Smith), Renal d/o

Front 34

SLE: Best Screen Test

Back 34

ANA

Front 35

SLE: Best Test Overall

Back 35

Anti ds-DNA

Front 36

SLE: Problem with ds-DNA

Back 36

- Highly reliable
- Only active disease

Front 37

SLE: Anti-Smith?

Back 37

- Good, specific, does not measure disease activity

Front 38

SLE: Other Tests

Back 38

1. Coombs (Hemolytic anemia)
2. APL (APL Syndrome)
3. Lupus anticoagulant
4. Antithyroid (hypothyroid)
5. Antiribosomal P Ab (Lupus cerebritis)
6. Complement studies

Front 39

SLE: Mgmt of Arthritis?

Back 39

- NSAIDS: For arthritis if NOT renal disease

Front 40

SLE: Mgmt for mild disease?

Back 40

- Hydroxychloroquine

Front 41

SLE: APL or Lupus anticoagulant Mgmt?

Back 41

- Anticoagulants: ASA, heparin

Front 42

SLE: When to use steroids?

Back 42

- Kidney disease
- Acute exacerbations

Front 43

SLE: When to cyclophosphamide

Back 43

Severe Disease: nephritis, vasculitits, pulmonary hemorrhage, CNS disease

Front 44

Neonatal Lupus: Cause

Back 44

- Transfer IgG (Anti-Rho) across placenta --> 12-16 weeks

Front 45

Neonatal Lupus: Findings

Back 45

- Cutaneous lesions
- Hepatitis
- Thrombocytopenia
- Neurologic Disease
- Heart blood

Front 46

Neonatal Lupus: Key to Findings

Back 46

- ALL ARE TEMPORARY EXCEPT HEART BLOCK

1. May require pacing

Front 47

Kawasaki Disease: Mech

Back 47

- Acute vasculitis of ALL VESSELS
- But especially coronaries

Front 48

Kawasaki Disease: Epi

Back 48

- Highest in Asians
- Leading cause acquired heart disease in U.S. and Japan

Front 49

Kawasaki Disease: Age Range

Back 49

80%+ < 5y

Very few adolescent and young adults

Front 50

Kawasaki Disease: Dx Based on

Back 50

Key: FEVER ≥ 5d Plus 4/5 criteria

Front 51

Kawasaki Disease: Criteria

Back 51

1. B/l nonpurlent conjunctival injection
2. Mucous membrane changes: Injected pharynx, dry, red crackled lips, strawberry tongue
3. Peripheral extremity changes: Edema/erythema/dequamation
4. Rash
5. Cervical LAD > 1.5cm

Front 52

Kawasaki Disease: Least constant feature

Back 52

LAD

Front 53

Kawasaki Disease: Desquamation pattern

Back 53

Starts at finger tips and goes back, can go as far as wrists

Front 54

Kawasaki Disease: General or occasional findings

Back 54

- Kids VERY irritable
- Aseptic meningitis
- Diarrhea
- Hepatitis
- Hydrops of gallbladder
- Urethritis w/ sterile pyuria
- Otitis media
- Arthritis

Front 55

Kawasaki Disease: Cardiac Findings Early

Back 55

*Half have myocarditis

1. Tachy and decreased ventricular function
2. Pericarditis
3. Aneurysm in 2-3rd weeks**

Front 56

Kawasaki Disease: Labs

Back 56

1. WBC normal to increase; Left shift
2. Increased ESR, CRP
3. Normocytic anemia
4. Plts nl-High week 1 --> Then HIGH weeks 2-3 (often 1 million+) Usually in covalescent stage
5. Sterile pyuria
6. Increased LFTs
7. CSF pleocytosis

Front 57

Kawasaki Disease: Eval --> W/u after Dx

Back 57

**Echo
EKG at Dx
Follow platelets

Front 58

Kawasaki Disease: When in the clear??

Back 58

No e/o long-term CV sequelae in those w/o coronary abnormalities w/in 2 mo of onset

Front 59

Kawasaki Disease: Acute Rx

Back 59

- IVIG
- High Dose ASA
- Steroids Limited to persistent fever

Front 60

Kawasaki Disease: Convalescnt Rx

Back 60

- Low dose ASA (3-5 mg/kg/day) --> b/c high platelets

Front 61

Kawasaki Disease: If acute coronary thrombosis during recovery

Back 61

- Fibrinolytics w/ tPA, streptokinase, or urokinase

Front 62

Henoch Schonlein Purpura: Etiology

Back 62

- IgA-mediated vasculitis of small vessels
- Usually follows URI

Front 63

Henoch Schonlein Purpura: Epi

Back 63

MCC: Nonthrombocytopenia purpura in children

More in Winter
Males > Females

Front 64

Henoch Schonlein Purpura: Age onset

Back 64

2-8y

Front 65

Henoch Schonlein Purpura: Presentation

Back 65

- Low-grade fever/fatigue
- Rash
- Arthritis
- GI pain/blood
- Renal (RN or NS)
- HSM, LAD

Front 66

Henoch Schonlein Purpura: Characteristics of Rash

Back 66

Pink, MP progresses to petechiae --> purpura crops over 3-10d

*Palpable purpura
*Posterior, dependent areas, below waist

Front 67

Henoch Schonlein Purpura: Type of arthritis

Back 67

Usually larger, weight bearing joints

Front 68

Henoch Schonlein Purpura: GI involvement

Back 68

- Intermittent abdominal pain
- Occult blood in stool
- Diarrhea
- Hematemesis
- Intussusception*

Front 69

Henoch Schonlein Purpura: Renal involvement (%)

Back 69

- Half with GN or NS

Front 70

Henoch Schonlein Purpura: Dx

Back 70

Clinical - Classic presentation

Front 71

Henoch Schonlein Purpura: Lab Findings

Back 71

- Increase Acute phase reactants (plts nl-High)
- Anemia
- Higher IgA, Igm
- May have anticardiolipin or APL Abs
- Urine RBCs, WBCs, Casts, albumin
- Heme positive stool

Front 72

Henoch Schonlein Purpura: Definitive Dx if wanted

Back 72

- Skin Bx (rarely done) --> Showing leukocytoclastic angiitis)

- Renal Bx --> Showing IgA mesangial deposition + occasional IgM, C3, Fibrin

Front 73

Henoch Schonlein Purpura: Tx and Course

Back 73

Supportive and Symptomatic

*Self-limited

Front 74

Henoch Schonlein Purpura: When to use steroids?

Back 74

- Intestinal involvement (oral or IV forms)
*Usually see dramatic improvement
- Renal Involvement

Front 75

Henoch Schonlein Purpura: What to do for APL Ab or Anticardiolipin Ab or Thrombotic events?

Back 75

ASA

Front 76

Henoch Schonlein Purpura: Complications

Back 76

- Renal failure/insufficiency
- Bowel perf
- Scrotal edema and testicular torsion