Rheumatology Flashcards
Most common cause of acquired heart disease in children? Age of presentation? Clinical Features?
Kawasaki’s disease; 18 to 24 months
CRASH and BURN
- Conjunctivitis – bilateral without exiting
- Rash – Mostly on trunk
- Adenopathy – unilateral cervical lymph node
- Strawberry or cracked, swollen lips
- Hands and feet – edema, peeling
- Fever >39p
HSP – Type of vasculitis? Age of onset? Systems affected? Prognosis?
IGA mediated vasculitis
5 years
- Skin – palpable purpura, edema
- Joints
- G.I. – abdominal pain, bleeding, intussusception
- Renal
Most patients recover within four weeks
Other clinical features (not CRASH and BURN) of Kawasaki’s?
- Coronary artery aneurysms, myocarditis, CHF, arrhythmia
- Ureteritis
- Aseptic meningitis
- Hydrops of the gallbladder
- Arthritis
Phases of Kawasaki’s? Corresponding laboratory findings and tx?
- Acute – elevated ESR/CRP. IVIg and High-dose ASA for anti-inflammatory effect.
- Subacute – elevated platelet count, decreasing ESR/CRP. Low-dose ASA for antiplatelet effect
- Convalescent – normalization of labs
Juvenile rheumatoid arthritis – mean age of onset? Three types of JRA? Laboratory findings? Treatment?
One-three years
Pauciarticular, Polyarticular, systemic
Microcytic anemia, elevated ESR/CRP, ANA
- Inflammation control – NSAIDs
- Surgery for contractures
Pauciarticular JRA: early onset versus late onset –
- Sex/age?
- Markers?
- Uveitis?
- Large Joints affected?
- Possible complications?
1. 1-5 Female versus 8+ male 2. ANA versus HLA-B27 3. Yes versus uncommon 4. No versus hips/sacroilial 5. Blindness (from uveitis) versus spondyloarthropathy
Polyarticular JRA – RF negative versus RF positive:
- Sex?
- Markers?
- Uveitis?
- Joints affected?
- Possible complications?
- Female versus female
- RF negative versus RF positive
- Rare
- Both large and small
- Deforming arthritis
Systemic JRA:
- Sex?
- Markers?
- Uveitis?
- Large Joints affected?
- Possible complications?
- Both equally
- None
- Rare
- Large and small
- 50% developed chronic destructive arthritis
Unique findings in stills disease? (Systemic JRA)
- High spiking fevers >39
- Evanescent salmon colored rash
- Hepatosplenomegaly
- Lymphadenopathy
- Anorexia
- Serositis, myositis, tenosynovitis
- CMS – meningitis/Encephalopathy
Management for children with SLE?
- Anti-inflammation – NSAIDs
- Immunosuppressives – steroids, cyclophosphamide
- If antiphospholipid antibody positive – LMWH or warfarin
Dermatomyositis – age of onset? Diagnosis? Management? Complications? Prognosis versus adults?
5-14
Biopsy, EMG, elevated muscle enzymes (CPK, AST, lactate dehydrogenase, aldolase)
Corticosteroids
- Aspiration pneumonia from diminished gag reflex
- Intestinal perforation from vasculitis
- Osteopenia due to steroids
Better prognosis in children
Dermatomyositis – clinical features?
- Cutaneous findings – heliotrope rash, grottron’s papules
- Proximal muscle weakness – growers sign (difficulty standing from sitting position)
- C’s - Calcinosis, Constipation, cardiomyopathy
- Nail bed telangiectasias
Rheumatic fever – bacterial precipitant? Major risk factor? No risk in strains that cause? Age of onset?
Endocarditis suggested with what finding? Myocarditis?
GABHS, pharyngitis; skin infection (Impetigo); 5-15
Valvular insufficiency; tachycardia out of proportion to fever
Jones criteria?
Major criteria: 1. Migratory arthritis 2. Carditis 3. Subcutaneous nodules 4. Sydenham's Chorea 5. Everything marginatum Minor criteria: 1. Fever 2. Arthralgia 3. Previous rheumatic fever 4. Leukocytosis 5. Elevated ESR/CRP 6. Prolonged PR interval
Treatment for rheumatic fever?
- Penicillin for GA DHS eradication
- NSAIDs for inflammation control
- Corticosteroids if severe cardiac involvement
- haloperidol for Sydenham’s chorea
