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76 Cards in this Set
- Front
- Back
Juvenile RA: Cause
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Immuogenic susceptibility + External triggers
*Otherwise thought to be AI |
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Juvenile RA: General Findings
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Chronic, nonsuppurative inflammation of synovium
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Juvenile RA: 3 Key characteristics
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1. Joint effusions
2. Destruction of joint cartilage 3. Bone deformity, destruction, fusion |
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Juvenile RA: Clinical Presentation:
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Key: Morning stiffness
- Good days, bad days - Easy fatigability - Warm joints, pain with ROM but NO erythema |
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Juvenile RA: Dx
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CLINICAL
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Juvenile RA: Criterai
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- 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) |
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Juvenile RA: What factor defines what 'type'?
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How it presents first 6 months
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Juvenile RA: DDx
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- SLE*
- Juvenile Dermatomyositis - Sarcoid - Scleroderma - Rheu Fever* - Vasculitis - AI Hepatitis - Late stage Lyme* - Psoriatic arthritis - Arthritis w/ IBD - Lymphoproliferative disease* |
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Juvenile RA: Types
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1. Pauciarticular
2. Polyarticular 3. Systemic onset |
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Juvenile RA: Pauciarticular Defn
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< 5 joints
Lower extremity; NEVER hip Larger joints |
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Juvenile RA: Polyarticular Defn
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≥ 5 joints
Resembles nl adult arthritis Rheumatoid nodules common May involve cervical spine |
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Juvenile RA: Systemic Onset Defn
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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 |
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Juvenile RA: Systemic Onset visceral involvement?
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- Hepatosplenomegaly
- LAD - Serositis |
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Juvenile RA: Rash type?
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Fairly non-specific
Salmon colored Comes and goes WITH fever (i.e. evanescent) |
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Juvenile RA: Lab Findings
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- NO BEST TEST (Dx of exclusion
- High acute phase reactants - Anemia of CD - ANA in 40-85% --> More w/ poly/pauciarticular - RF+ in some |
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Juvenile RA: RF+ more likely in what cases?
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Older Kids
Polyarticular Rheumatoid nodules |
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Juvenile RA: Mgmt Goal
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Preserve Joint Function
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Juvenile RA: Mgmt Lines
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- NSAIDs first line
- Methotrexate - Steroids --> Maybe but few indications - PT/OT - Optho follow-up |
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Juvenile RA: When to use steroids?
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- Overwhelming inflammation
- Systemic illness - Bridge therapy |
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Juvenile RA: Optho problems?
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Characteristic iridocyclitis
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Juvenile RA: Which Form has excellent prognosis?
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Pauciarticular --> ANA+ --> Younger girls (except when eyes involved...)
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Juvenile RA: Which forms have Poor Prognosis?
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- Any RF+ (Poly or pauci)
- Erosions, nodules, unremitting course bad signs - Polyarticular Involvement + Systemic also bad |
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Juvenile RA: Other "Good" but not "Excellent" prognostic signs?
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- Seronegative (ANA and RF)
- HLA B27 (Typically older males, Pauciarticular) |
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SLE: HLA Associations
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B8, DR2, DR3
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SLE: Exacerbating factor to know
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Sunlight
Infectious agents |
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SLE: Drug Causes to know (Drug-Induced forms)
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- Anticonvulsants
- Sulfonamides - Antiarrhythmics |
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SLE: Age Range
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FEMALES
> 8y |
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SLE: Most frequent presentation
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Fever + Fatigue + Arthralgia + Arthritis + Malar Rash
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SLE: Types of Rashes
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Malar
Discoid Livedo reticularis *All of these are photosensitive |
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SLE: Renal problems
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- GN
- Nephrotic Syndrome - HTN - Renal Failure |
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SLE: Endocarditis Type
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Libman Sacks
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SLE: Criteria (4/11) Pneumonic
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MD SOAP N HAIR:
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MD SOAP N HAIR
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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
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SLE: Best Screen Test
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ANA
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SLE: Best Test Overall
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Anti ds-DNA
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SLE: Problem with ds-DNA
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- Highly reliable
- Only active disease |
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SLE: Anti-Smith?
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- Good, specific, does not measure disease activity
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SLE: Other Tests
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1. Coombs (Hemolytic anemia)
2. APL (APL Syndrome) 3. Lupus anticoagulant 4. Antithyroid (hypothyroid) 5. Antiribosomal P Ab (Lupus cerebritis) 6. Complement studies |
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SLE: Mgmt of Arthritis?
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- NSAIDS: For arthritis if NOT renal disease
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SLE: Mgmt for mild disease?
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- Hydroxychloroquine
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SLE: APL or Lupus anticoagulant Mgmt?
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- Anticoagulants: ASA, heparin
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SLE: When to use steroids?
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- Kidney disease
- Acute exacerbations |
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SLE: When to cyclophosphamide
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Severe Disease: nephritis, vasculitits, pulmonary hemorrhage, CNS disease
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Neonatal Lupus: Cause
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- Transfer IgG (Anti-Rho) across placenta --> 12-16 weeks
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Neonatal Lupus: Findings
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- Cutaneous lesions
- Hepatitis - Thrombocytopenia - Neurologic Disease - Heart blood |
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Neonatal Lupus: Key to Findings
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- ALL ARE TEMPORARY EXCEPT HEART BLOCK
1. May require pacing |
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Kawasaki Disease: Mech
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- Acute vasculitis of ALL VESSELS
- But especially coronaries |
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Kawasaki Disease: Epi
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- Highest in Asians
- Leading cause acquired heart disease in U.S. and Japan |
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Kawasaki Disease: Age Range
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80%+ < 5y
Very few adolescent and young adults |
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Kawasaki Disease: Dx Based on
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Key: FEVER ≥ 5d Plus 4/5 criteria
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Kawasaki Disease: Criteria
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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 |
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Kawasaki Disease: Least constant feature
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LAD
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Kawasaki Disease: Desquamation pattern
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Starts at finger tips and goes back, can go as far as wrists
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Kawasaki Disease: General or occasional findings
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- Kids VERY irritable
- Aseptic meningitis - Diarrhea - Hepatitis - Hydrops of gallbladder - Urethritis w/ sterile pyuria - Otitis media - Arthritis |
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Kawasaki Disease: Cardiac Findings Early
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*Half have myocarditis
1. Tachy and decreased ventricular function 2. Pericarditis 3. Aneurysm in 2-3rd weeks** |
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Kawasaki Disease: Labs
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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 |
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Kawasaki Disease: Eval --> W/u after Dx
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**Echo
EKG at Dx Follow platelets |
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Kawasaki Disease: When in the clear??
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No e/o long-term CV sequelae in those w/o coronary abnormalities w/in 2 mo of onset
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Kawasaki Disease: Acute Rx
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- IVIG
- High Dose ASA - Steroids Limited to persistent fever |
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Kawasaki Disease: Convalescnt Rx
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- Low dose ASA (3-5 mg/kg/day) --> b/c high platelets
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Kawasaki Disease: If acute coronary thrombosis during recovery
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- Fibrinolytics w/ tPA, streptokinase, or urokinase
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Henoch Schonlein Purpura: Etiology
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- IgA-mediated vasculitis of small vessels
- Usually follows URI |
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Henoch Schonlein Purpura: Epi
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MCC: Nonthrombocytopenia purpura in children
More in Winter Males > Females |
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Henoch Schonlein Purpura: Age onset
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2-8y
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Henoch Schonlein Purpura: Presentation
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- Low-grade fever/fatigue
- Rash - Arthritis - GI pain/blood - Renal (RN or NS) - HSM, LAD |
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Henoch Schonlein Purpura: Characteristics of Rash
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Pink, MP progresses to petechiae --> purpura crops over 3-10d
*Palpable purpura *Posterior, dependent areas, below waist |
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Henoch Schonlein Purpura: Type of arthritis
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Usually larger, weight bearing joints
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Henoch Schonlein Purpura: GI involvement
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- Intermittent abdominal pain
- Occult blood in stool - Diarrhea - Hematemesis - Intussusception* |
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Henoch Schonlein Purpura: Renal involvement (%)
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- Half with GN or NS
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Henoch Schonlein Purpura: Dx
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Clinical - Classic presentation
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Henoch Schonlein Purpura: Lab Findings
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- 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 |
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Henoch Schonlein Purpura: Definitive Dx if wanted
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- Skin Bx (rarely done) --> Showing leukocytoclastic angiitis)
- Renal Bx --> Showing IgA mesangial deposition + occasional IgM, C3, Fibrin |
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Henoch Schonlein Purpura: Tx and Course
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Supportive and Symptomatic
*Self-limited |
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Henoch Schonlein Purpura: When to use steroids?
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- Intestinal involvement (oral or IV forms)
*Usually see dramatic improvement - Renal Involvement |
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Henoch Schonlein Purpura: What to do for APL Ab or Anticardiolipin Ab or Thrombotic events?
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ASA
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Henoch Schonlein Purpura: Complications
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- Renal failure/insufficiency
- Bowel perf - Scrotal edema and testicular torsion |