Pediatric Rheumatology Flashcards
1
Q
Characteristics of Rheumatologic Conditions
A
- inflammation: warmth, swelling, redness, pain
- primarily immune system disorder
- autoimmunity
2
Q
who needs assessed - signs of inflammation
A
- systemic: fever, fatigue, growth failure, wt. loss
- arthritis: the joint
- synovitis: joint synovium
- enthesitis: ligamentous insertion into bone
- sersitis: pleuritis, pericarditis, peritonitits
- myositis: muscle
- vasculitis: petechiae, purpura, edema
3
Q
Important Hx in Rheumatologic Disease
A
- prior illness- pattern of redness, swelling
- meds
- immunizations- can induce autoimmune state
- trauma
- insect bites - tick, spider
- family hx of autoimmune- Crohn’s
4
Q
Rheumatologic Conditions in DDx
A
- prolonged, persistent fevers
- chronic pain and fatigue
- chronic inflammation
- rashes
- swelling
5
Q
differentiating inflammation from mechanical pain
A
- inflammation
- warmth, redness, swelling
- morning stiffness, worse in a.m.
- improves w/ movement
- mechanical pain
- lacks persistent swelling
- worse at end of day
- worse w/ movement
6
Q
PE of suspected rheumatologic disease
A
- gait and movement
- nutrition status
- height and weight*
- skin and mucous membrane findings
- muscle strength
- joint inspection, palpation, ROM
*look closely at growth chart -
some growth failure w/ inflammatory process
7
Q
Laboratory Evaluation
A
- CBCd (elevated WBCs, platelet count, anemia)
- ESR
- CRP (c-reactive protein)
- ANA (autoantibodies)
- rheumatoid factor
can provide enough for rheumatology referral
8
Q
vasculitides
(smallest to largest vessels)
A
- Henoch Schonlein Pupura
- Kawasaki Disease
- Polyarteritis Nodosa
- Takayasu Arteritis
9
Q
Henoch Schonlein Purpura (HSP)
Characteristics, S/s, Dx, Tx
A
- characteristics
- small vessel vasculitis d/t IgA complexes
- S/s (can wax/wane for months)
- non-thrombocytopenic purpura
- median age 5 (3-15)
- common in winter
- classic rash on LE- palpable purpura
- abdominal pain
- complications
- arthritis of knees and ankles
- GI- abd cramps, bloody diarrhea, intussusception
- 1/3 develop renal glomerulonephritis
- Dx
- clinical presetation
- elevated CBC, ESR; normal platelet
- UA to screen for glomerulonephritis (hematuria)
- stool guaiac
- biopsy of blood vessels for confirmation (if needed)
- Tx
- supportive care primarily
- hosp. only w/ complications
- GI, renal, arthritis
- Prednisone PO for abdominal pain (inpatients only)
10
Q
Kawasaki Disease
Characteristics, S/s, Dx, Tx
A
- characteristics
- small-med artereries w/ subsequent aneurysm
- MC vasculitis of childhood
- ~12 days if untreated
- greatest risk: coronary artery aneurysm
- S/s
- fever minimally responsive to antipyretics
- strawberry tongue
- rash w/ desquamation of fingers/toes
- conjunctivitis (75%)
- lymphadenitis
- Dx
- no specific clinical test
- normal WBC w/ hi ESR/CRP (CPR declines quickly, ESR longer - determine stage of disease)
- Tx
- prompt IVIG to avoid coronary artery dilation
11
Q
3 Diseases triggered by Sensitivity
(immune hypersensitivity primarily affecting mucous membranes brought on by infection or drug)
A
- Erethema Multiforme
- milder, self limiting
- target lesions pathognomic- round deep red, well demarcated macules and papules w/ grey or bullous center
- Stevens Johnson Syndrome
- prodrome, fever, cough, HA, malaise
- Transient Epidermal Necrolysis
- multisystem, severe
12
Q
Juvenile Idiopathic Arthritis (JIA)
Characteristics, S/s, Dx, Tx
A
- characteristics
- MC arthritis of childhood - age 1-3, 8-12 (<16 for juvenile)
- girls > boys
- chronic synovitis overarching manifestation (>6 weeks for chronic)
- 7 subtypes - classifications
- S/s
- morning stiffness at least 15min, improvement as day progresses
- recurrent stiffness after immobility
- joint swelling, redness, warmth, decreased ROM, bony overgrowth
- uveitis - referred to opthalmologist
- complications
- eye: iridocyclitis, uveitis
- chronic pain, loss of mobility, failure to thrive
- Dx
- arthrocentesis neg. for infection (mostly lymphocytes)
- Tx
- suppress inflammation
- maintain mobility
- screen for eye problems
- ANA pos. in oligoarticular & polyartricular)
- xrays normal early
13
Q
JIA - Oligoarticular
A
- MC JIA
- < 5 joints affected: knee, ankle, wrist MC
- screening for uveitis
- q 3mo if +ANA
- q 6mo if -ANA
14
Q
JIA - Polyarticular
A
- >4 joints in 1st 6mo of disease
- RF +/-
- (-) peaks ages 1-3: hands, feet, ankles, knees
- (+) begins late childhood/young adult: subcutaneous nodules in elbow, heels, hands, knees, ears, scapula, sacrum
15
Q
JIA - Systemic
A
- spiking fever
- morbilliform salmon colored rash
- pleuritis, pericarditis, peritonitis
- hepatosplenomegaly
- arthritis
17
Q
Juvenile Dermatomyositis
Characteristics, S/s, Dx, Tx
A
- characteristics
- autoimmune myopathy (assoc. w/ SLE, T1 DM)
- peak 4-10, girls > boys
- S/s
- slow progressive disease
- hallmark: proximal muscle weakness (hips, shoulders, neck)
- classic rash
- heliotrope discoloration of eyelids
- Gottron papules on joints
- dilated nail fold capillaries
- calcinoids anywhere if severe
- Dx
- elevated muscle enzymes
- CBC normal
- +ANA but not specific
- clinical diagnosis
- Tx
- Methotrexate and intervals of corticosteroids for immunosuppression
18
Q
Juvenile Systemic Sclerosis
Characteristics, S/s
A
- characteristics
- chronic multisystem connective tissue disorder
- rare
- S/s
- symmetrical fibrous thickening, hardening of skin
- fibrous changes in esophagus, intestines, heart, lungs, kidneys
-
Morphea - localized scleroderma (skin and underlying muscle)
*
19
Q
Juvenile Sarcoidosis
Characteristics, S/s
A
- characteristics
- non-caseating granulomas affecting multiple soft tissue (skin MC)
- S/s
- erythema nodosum - panniculitis of LE
- Lofgren’s syndrome is acute presentation w/ hilar adenopathy, erythema nodosum, and polyarthralgia
- “cellulitis that doesn’t respond to abx”
20
Q
Periodic Fever Syndrome
Characteristics, S/s, Tx
A
- characteristics
- antigen independent ativation of immune system
- age 2-5, cessation by age 10
- S/s
- fevers characteristic
- no sign of infection or malignancy
- ESR/SED elevated during flare-up
- PFAPA- periodic fever w/ aphthous stomatitis, pharangitis, adenitis (fever, mucous membrane, ocular involvement)
- Tx
- antipyretics, pain relievers
- single dose Prednisolone or Betamethosaone
- typically resolves by age 10
