Ms: Rheumatology

Front 1

Juvenile Idiopathic Arthritis (JIA) - Definition

Back 1

- occurring before the age of 16
- with persistent synovitis in one or more joints for at least 6 weeks (many prefer 3months)
- all other diagnoses excluded

Front 2

JIA - 3 subtypes

Back 2

Subtype varies according to how it presents in the 1st 6 months:
1) Oligoarticular (4 or fewer joints)
2) Polyarticular (>4 joints)
3) Systemic (spiking fevers)

Front 3

Outcome in JIA is most closely related to....

Back 3

course after the first 6 months

Front 4

JIA - etiology

Back 4

- unclear
- thought to be autoimmune
- some HLA alleles are important
- some microbial Ag's may be important

Front 5

JIA - how is it initiated?

Back 5

--> initiated by T-cell activation: presentation of antigens to T lymphocytes by APCs (macs, b-lymphs, fibroblasts)
--> T cell activation causes T and B lymphocyte production.
--> cytokines are released including TNF-alpha, IL-1, and IL-6, which cause release of other mediators, such as prostaglandins, neutrophils, complement, proteases

Front 6

How is - SYSTEMIC JIA - activated?

Back 6

mediated by IL-6 (and likely IL-1), which causes further migration of inflammatory cells into the synovia, which then damage synovial tissue, cartilage, and bone.

Front 7

Characteristics of Synovium in JIA

Back 7

- The inflamed synovia has lymphocytic and plasma cell infiltration (just like adult RA)
- WBC counts in synovial fluid are between 2,000 - 3,000/mL but can be as high as 100,000/mL
- Panus formation occurs, which is growth of the synovium into the articular cartilage

Front 8

JIA Clues for Diagnosis:

Back 8

- Morning stiffness that improves with movement later in the morning
- changes in walking, running, climbing, or willingness to play, esp in the morning hours
- leg length discrepancies
- return of need for assistance with dressing, eating, bathing, and toileting
- enuresis may recur
- developmental milestones may be lost

Front 9

Radiologic studies in JIA

Back 9

non-specific early in the course, but...
- if fingers involved, look for widening of the mid-portion of the affected phalanges from periosteal new borne formation (takes months to years of active inflammation)

Front 10

OLIGOARTICULAR onset
(aka: pauciarticular)

Back 10

- 4 or fewer joints involved during the first 6 months of illness.
- 40-60% of JIA

Front 11

What is the cause of the Right Knee Joint swelling?

Back 11

Oligoarticular JIA

Front 12

OLIGOARTICULAR - demographic

Back 12

- presents b/w 1-7yo, avg age is 5yo
- F:M 3:1 (overall)
- F:M 6.5:1 (+uveitis)

Front 13

OLIGOARTICULAR - presenting symptoms

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- slow onset
- usually present without many symptoms
- ~25% are without any pain, showing up with incidental joint swelling

Front 14

OLIGOARTICULAR - joints most commonly involved

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Most frequent to least:
knee --> ankle --> elbows --> wrists --> small joints of hands (10-15% of time)

Front 15

OLIGOARTICULAR - other findings

Back 15

- rare to have findings other than arthritis in oligo (fever, rash, night pain are NOT seen)
- EXCEPT asymptomatic uveitis (occurs in ~30%)

Front 16

OLIGOARTICULAR - most important prognostic factor in developing uveitis

Back 16

ANA(+) - predicts higher risk of developing uveitis

Front 17

OLIGOARTICULAR - when does uveitis usually present?

Back 17

- within the first 5-6 years of initial presentation
- screen more frequently in the early years, but continue annually in later years dt continued increased risk

Front 18

OLIGOARTICULAR - ophtho screening if ANA(+)

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- within first 4-7 years and ANA(+) --> you need to perform ophthalmologic screening by SLIT-LAMP EXAM
- perform q3 months in the first 4 years of diagnosis
- perform q6 months b/w 4-7 years of diagnosis

Front 19

OLIGOARTICULAR - uveitis or iridocyclitis

Back 19

- inflmmation of the anterior uveal tract and the adjacent ciliary body
- most serious and most common complication
- occurs in 20-25%
- greatest risk: ANA+ and <6 yo
- Uveitis is often silent, by time child complains of eye pain permanent damage has occurred.
- COMPLETE slit lamp exam is necessary
- complications: cataracts, synechiae, glaucoma, band keratopathy, macular edema
- chronic uveitis results in permanent visual damage in >15% of affected pts despite Rx

Front 20

OLIGOARTICULAR - screening if ANA(-) and diagnosis is >7 years

Back 20

- screen every 12 months

Front 21

OLIGOARTICULAR - limb length discrepancy

Back 21

- inflammation brings increased blood flow and nutrients
- results in boney overgrowth in length and width
- knee and ankle joint injection with glucocorticoids early in the course may prevent leg length discrepancies

Front 22

OLIGOARTICULAR - labs

Back 22

- NONspecific
- ANA(+) at least in low titer (< or = 1:320) in ~70%
- RF and Hg are usually normal
- ESR, CRP may be normal!

Front 23

OLIGOARTICULAR - long term articular outcome

Back 23

- good, although some patients may develop limb-length discrepancy
- 25-30% will eventually develop a polyarticular course

Front 24

POLYARTICULAR - definition

Back 24

- 30-40% of JIA
- 5 or more joints during the first 6 months

Front 25

POLYARTICULAR - demographic

Back 25

- F:M ratio is 3:1
RF(+) mc older, usually adolescent
RF(-) usually younger, with a peak incidence in the toddler age group

Front 26

POLYARTICULAR - joints affected

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affects large and small joints and typically involves the cervical spine, hips, shoulders, and TMJ.
**symmetric joint involvement may be seen (similar to adult RA)
- spine

Front 27

POLYARTICULAR - late findings

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- cervical spine fusion
- micrognathia
(**also represent late findings in SYSTEMIC)

Front 28

POLYARTICULAR - presenting symptoms

Back 28

-fatigue (mc)
- also fever, weightloss, and rheumatoid nodules

Front 29

POLYARTICULAR - prognostic factors

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RF(+) Polyarticular JIA: occurs in 10%, usu signifies a disease more similar to adult RA. Poor prognostic factor, signifies need for aggressive mgt

(anti-CCP antibody relevance has not been found)

ANA(+) Polyarticular JIA: (occurs in 30%). Uveitis is less common, affecting only 10-15% with +ANA which is ~half of occurance in oligoarticular

ANA+, <7 years old and polyarticular JIA: have intermediate risk of uveitis and should be monitored closely

Front 30

SYSTEMIC JIA - definition

Back 30

requires occurrence of fever and other systemic findings
10-20% with JIA

Front 31

SYSTEMIC JIA - demographic

Back 31

F:M 1:1, with peak age of 5-10 years.

Front 32

SYSTEMIC JIA - key findings when febrile

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1 or 2 fevers spikes to 103 degrees on a DAILY basis, which will return to normal without any antipyretics (quotidian or diquotidian fever pattern).
- Usually the fever is in the evening and can be associated with SEVERE MYALGIA and ARTHRALGIA.

Front 33

SYSTEMIC JIA - when Afebrile

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when the fever is gone, the child appears better and may have no significant symptoms

Front 34

SYSTEMIC JIA - things to look for, RASH

Back 34

- usually associated with the fever
- migratory in appearance
- macular
- pink-to-salmon coloring
- discrete borders
- with or without central clearing
- occurs on the TRUNK, THIGHS, and AXILLAE
- KOEBNER phenomenon - mild irritation such as rubbing or scratching may cause the rash to appear
- may be very pruritic or not
- biopsy will show only nonspecific lymphocytic infiltration

Front 35

SYSTEMIC JIA - things to look for, Synovitis/Arthritis

Back 35

- may or may not appear initially, but no dx until joint involvement presents
- Arthritis may occur as oligoarticular (~25-30%) or polyarticular (70-75%) involvement.

Front 36

SYSTEMIC JIA - worst prognostic indicators

Back 36

- active disease 1 year after onset and diagnosis before 4 years of age
- also the amount of arthritis predicts long-term outcome
- high aldolase, high ferritin in severe systemic illness

Front 37

SYSTEMIC JIA - signs/symptoms

Back 37

- sick appearing with constitutional symptoms, FTT
- Rash
- Synovitis/Arthritis
- Severe myalgias (CPK usually normal, but ALDOLASE usually quite elevated in severe systemic illness)
- pericarditis and myocarditis
- pleuritis
- lymphadenitis
- hepatosplenomegaly (in 70%)
- abdominal pain
- weight loss and fatigue
- Uveitis is rare! (<5%)

Front 38

SYSTEMIC JIA - labs

Back 38

- occasional leukemoid reaction >40,000
- thrombocytosis (occasionally >1million)
- high CRP and ESR
- Anemia is common (microcytic anemia of chronic disease due to inability to utilize iron stores)
- low albumin
- Ferritin levels >4000 (nl <200) correspond to more severe systemic disease
- RF always negative!
- ANA is rarely positive

Front 39

JIA age of onset in various types

Back 39

OLIGO - pk (2-3 yo), rare >10 yo
POLY - pk (2-5yo), 2pk (10-24 yo)
SYST - no peak

Front 40

JIA and MAS

Back 40

severely affected kids with JIA develop MAS - cytokine dysfunction resulting in uncontrolled accumulation of activated T cells and Macs in many organs
- elevated transaminases
- coagulopathy with +DD, prolonged PTT
- drop in plts, ESR
- bone marrow with hemophagocytosis
- may be triggered by viral infxn: EBV, CMV, HSV, VZV, parvo B19
- may be triggered by drugs: Sulfa drugs and NSAIDs
- Monitor with labs 1-2x/wk and clnical (every 1-2 wks)
- prompt tx with CORTICOSTEROIDS and CYCLOSPORINE if needed to prevent life-threatening complications
- METHOTREXATE and SULFASALAZINE are contraindicated !!!

Front 41

DDx JIA

Back 41

pain WITHOUT joint swelling - think of ortho problems like AVN, SCFE, OS, growing pains, benign hypermobility, psychogenic pain syndrome

DDx Systemic JIA - malignancies, SLE, acute rheumatic fever, serum sickness, Kawasakis

Front 42

Treatment for JIA - NSAIDs

Back 42

- may be used initially, either with or without DMARDs
- duration of use should NOT be longer than a couple months if the pt has not reached complete remission
- may be used in conjunction with other meds for symptom control

Front 43

Treatment for JIA - Intraarticular injection of Triamcinolone

Back 43

- used if only a few joints involved (ie: oligo)
- shown to decrease occurrence of leg-length discrepancies (affected leg is longer due to extra bone deposition)

Front 44

Treatment for JIA - DMARDs (FDA-approved)

Back 44

- FDA-approved: Methotrexate, sulfasalazine, leflunomide, TNF-inhibitors (eternercept and adalimumab), and T-cell modulators (abatacept)

Front 45

Treatment for JIA - DMARDs (NOT FDA-approved)

Back 45

- FDA - NON-approved = hydroxychloroquine, azathioprine, cyclophosphamide, infliximab (TNFinhibitor), anakinra (an IL-1 blocker).

Front 46

Treatment for JIA - DMARDs (FDA-approved, examples)

Back 46

- Methotrexate: usually the first DMARD to be used, given 1x/wk as pills or injected subQ
- Sulfasalazine and hydroxychloroquine: used less often bc there's less data
- Cyclosporine: also used occasionally in polyarticular JIA along with MTX
- Azathioprine, cyclophosphamide, and leflunomide: occasionally given for severe, resistent cases
- Eternercept and adalimumab: appear to work well for polyarticular JIA but LESS well for systemic onset.
- Abatacept, a soluble fusion protein that inhibits costimulation of T cells is also approved for polyarticular.
- Anakinra: helpful for systemic

Front 47

Treatment for JIA - Intraarticular injection of Triamcinolone

Back 47

- used if only a few joints involved (ie: oligo)
- shown to decrease occurrence of leg-length discrepancies (affected leg is longer due to extra bone deposition)

Front 48

Treatment for JIA - corticosteroids?

Back 48

- not used unless periods of VERY severe disease, flares, or systemic manifestations
- try to use the lowest doses possible to minimize side effects (<0.25mg/kg/day or <10 mg/day)

Front 49

Treatment for JIA - DMARDs (FDA-approved)

Back 49

- FDA-approved: Methotrexate, sulfasalazine, leflunomide, TNF-inhibitors (eternercept and adalimumab), and T-cell modulators (abatacept)

Front 50

Outcomes of JIA

Back 50

- follow q1-3months, but may be years before remission occurs
- 25-50% have functional limitations, but improved capacity over last 40yrs with better meds and joint replacement
- 30-40% may have active synovitis as adults
- mortality is rare <0.5% with systemic JIA died from amyloidosis in the past, now rarely seen due to better meds.
- major causes of death: infections dt immunosuppression and MAS

Front 51

Treatment for JIA - DMARDs (NOT FDA-approved)

Back 51

- FDA - NON-approved = hydroxychloroquine, azathioprine, cyclophosphamide, infliximab (TNFinhibitor), anakinra (an IL-1 blocker).

Front 52

Treatment for JIA - DMARDs (FDA-approved, examples)

Back 52

- Methotrexate: usually the first DMARD to be used, given 1x/wk as pills or injected subQ
- Sulfasalazine and hydroxychloroquine: used less often bc there's less data
- Cyclosporine: also used occasionally in polyarticular JIA along with MTX
- Azathioprine, cyclophosphamide, and leflunomide: occasionally given for severe, resistent cases
- Eternercept and adalimumab: appear to work well for polyarticular JIA but LESS well for systemic onset.
- Abatacept, a soluble fusion protein that inhibits costimulation of T cells is also approved for polyarticular.
- Anakinra: helpful for systemic

Front 53

Treatment for JIA - corticosteroids?

Back 53

- not used unless periods of VERY severe disease, flares, or systemic manifestations
- try to use the lowest doses possible to minimize side effects (<0.25mg/kg/day or <10 mg/day)

Front 54

Outcomes of JIA

Back 54

- follow q1-3months, but may be years before remission occurs
- 25-50% have functional limitations, but improved capacity over last 40yrs with better meds and joint replacement
- 30-40% may have active synovitis as adults
- mortality is rare <0.5% with systemic JIA died from amyloidosis in the past, now rarely seen due to better meds.
- major causes of death: infections dt immunosuppression and MAS

Front 55

Juvenile Spondyloarthropathy includes...

Back 55

1) juvenile ankylosing spondylitis
2) post-infectious reactive arthritis (Reiter's)
3) inflammatory bowel disease (and its associated arthropathy)
4) juvenile psoriatic arthritis

Front 56

Juvenile Spondyloarthropathy - Enthesitis

Back 56

means inflammation of the enthesis (which is where tendons, ligaments, or fasia attach to bone)

Front 57

Enthesitis-related Arthropathies (as part of new classification schema for JIA)

Back 57

1) juvenile spondyloarthropathy
2) SEA (syndrome of seronegativity, enthesopathy, and arthropathy)
3) HLA-B27-associated arthropathy and enthesopathy syndrome
- oligoarticular onset JIA "type II"
4) juvenile ankylosing spondylitis

Front 58

Enthesitis-Related Arthropathies (ERA) - incidence

Back 58

half as common as JIA, occuring in ~20/100,000

Front 59

How are ERAs different compared to other childhood inflammatory arthritides?

Back 59

- HLA-B27 association is variable (can range from 50% in psoriatic arthritis to 90% in ankylosing spondylitis)
- older children are affected
- it is familial 10-20% of the time
- arthritis is usually peripheral, with lower limb involvement in an asymmetric manner (except in psoriatic arthritis)

Front 60

You make the diagnosis of ERA in a child with chronic arthritis by these criteria:

Back 60

1) The child has both ARTHRITIS + ENTHESITIS or...
2) ARTHRITIS or ENTHESITIS + at least 2 of:
- Sacroiliac joint tenderness and/or inflammatory spinal pain
- HLA-B27(+)
- FHx of 1st or 2nd degree relative with HLA-B27(+) dz
- Anterior Uveitis
- Onset of arthritis in a boy after age 8 yo

Front 61

How do ERAs present clinically?

Back 61

- usually boys
- morning pain and stiffness
- pain relieved by playing or other activity
- pain predominantly in the joints of the LOWER extremities (often in low back/buttocks)
- pain at the entheses of the heels, feet, knees
- the oligoarthritis is usually asymmetrical
- the entheses that are affected may be exquisitely painful to palpation
- can elicit sacroiliac pain to direct palpation or pelvic manipulation

Front 62

How common are constitutional symptoms in ERAs?

Back 62

- less common; fever and weight loss occurring in fever than 10% of children with ERA.
- if growth delay is present, SUSPECT IBD !!
- acute symptomatic iritis (ie: acutely painful, red eye) in ~ 5-10%

Front 63

Labs in ERAs?

Back 63

ESR is normal in 50%
HLA-B27(+) in 50-90%
IgA may be elevated

Front 64

ERAs - first line treatment

Back 64

Begin treatment with NSAIDs; use for at least 1 month to see effect
- also orthotics made to redistribute weight away from the painful enthesis

Front 65

ERAs - second line treatment

Back 65

- if NSAIDs not effective: 2nd line is Joint injection with triamcinolone, or sulfasalazine and methotrexate, especially for peripheral joint disease.
- these are not as effective for axial disease

Front 66

ERAs - best treatment for psoriatic arthritis or ankylosing spondylitis

Back 66

- Etanercept
- Infliximab, adalimumab, and other anti-TNF therapies (also for IBD-associated arthropathy)

Front 67

ERAs - outcomes

Back 67

- some have disease for only 3-6 months before it resolves
- those with chronic course are more likely to have complications

Front 68

ERAs - outcomes of chronic course

Back 68

- sacroiliitis with spondylitis
- progress into adulthood with ankylosing disease of the back and sacroiliac joints
- 80% of adult AS patients do fairly well

Front 69

Arthritis with IBD: incidence

Back 69

occurs in ~25% of pts with IBD

Front 70

Arthritis with IBD: characteristics of peripheral arthritis dz

Back 70

Peripheral joints more commonly affected
- F=M
- NOT associated with HLA-B27
- Arthritis flares with gut flares

Front 71

Arthritis with IBD: characteristics of axial arthritis dz

Back 71

If axial arthritis occurs:
- Incidence in M>>F
- ASSOCIATED with HLA-B27
- NOT dependent on gut flares

Front 72

Arthritis with IBD: symptoms and signs

Back 72

constitutional symptoms: fatigue, weight loss, growth delay, fever, oral ulcers, abdominal pain/tenderness, diarrhea, erythema nodosum, pyoderma gangrenosum, clubbing

Front 73

Arthritis with IBD: treatment Peripheral Arthritis

Back 73

Peripheral Arthritis with a gut flare - will usually respond to appropriate therapy for the gut disease. So try corticosteroids and/or sulfasalazine.

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Arthritis with IBD: treatment Axial Arthritis

Back 74

Spine Disease - may need tx when gut inactive. Try Sulfasalazine, MTX, etanercept, Infliximab, Adalimumab.
**anti-TNF >> sulfasalazine and MTX for spine dz

Front 75

Juvenile Psoriatic Arthritis - diagnostic criteria

Back 75

Clinical:
1) ARTHRITIS + PSORIASIS or...
2) ARTHRITIS + at least 2 of:
- dactylitis
- nail findings (pitting, oil spots, onycholysis)
- FHx of psoriasis in 1st degree

Front 76

Juvenile Psoriatic Arthritis - distribution and clinical course of arthritis

Back 76

- arthritis can precede the psoriasis by many years
- initially, the arthritis is an ASYMMETRIC, OLIGOARTHRITIS of SMALL and LARGE joints with DACTYLITIS.
- DIP joint arthritis is common
- eventually, the arthritis may become POLYARTHRITIS
- some have chronic OLIGOARTHRITIS or DIP arthritis and NEVER progress to polyarthritis.

Front 77

Juvenile Psoriatic Arthritis - incidence of arthritis in cutaneous vs. nail psoriasis

Back 77

- arthritis develops in ~7% patients with CUTANEOUS psoriasis
- arthritis develops in ~30% with psoriatic NAIL involvement

Front 78

Juvenile Psoriatic Arthritis - other complications

Back 78

UVEITIS - is common

Front 79

Juvenile Psoriatic Arthritis - labs

Back 79

ANA positivity is common (30-50% of pts)

Front 80

Juvenile Psoriatic Arthritis - gender distribution depends on age at presentation

Back 80

- younger pts usu girls
- adolescents usu boys

Front 81

Reactive Arthritis - timing of onset

Back 81

1-4 weeks after a GI infection

Front 82

Reactive Arthritis - name infectious organisms responsible for triggering

Back 82

- GI infection: Yersinia, Shigella, Salmonella, or Campylobacter, C. diff, Giardia
- GU infection: Chlamydia or Mycoplasma

Front 83

Reiter's Syndrome - diagnostic critera

Back 83

Triad: Reactive Arthritis, conjunctivitis, Urethritis. (Urethritis occurs even if the infectious trigger was GI in origin)

Front 84

Reactive Arthritis - Mucocutaneous Features

Back 84

oral ulcers, genital ulcers, papular skin lesions

Front 85

Reactive Arthritis - features of arthritis

Back 85

- (enthesitis and dactylitis) that affects the LARGE, weight-bearing joints.
- arthritis may last 3-6 weeks, but can last for a few months

Front 86

Reactive Arthritis vs. Septic Arthritis

Back 86

both p/w fever, systemic symptoms; may need to aspirate joint fluid and do gut, urethra, or conjunctiva cultures for study to prove otherwise

Front 87

Reactive Arthritis - Treatment

Back 87

Treat with NSAIDs.
- more severe cases may require
- Abx therapy, such as doxycycline in children over age 7 yo. Abx is used as a remittive agent, although PCR can identify bacterial peptides in synovial tissue long after routine culture techniques are negative.
- Resistant cases may require sulfasalazine, MTX, and/or anti-TNF agents

Front 88

Vasculitides - 3 classes (depending on size of the vessel)

Back 88

Small-vessel: caused by immune complexes, p/w purpura and includes (drug rxns, Wegener's Granulomatosis, Serum Sickness, HSP)

Medium-vessel: causes organ system damage and incudes (polyarteritis nodosa (PAN) and Kawasakis)

Large-vessel: may cause claudication symptoms; includes (Takayasu's arteritis)

Front 89

Henoch-Schonlein Purpura (aka Anaphylactoid Purpura) - incidence/demographic

Back 89

the mc vasculitide in childhood
- mean age at diagnosis is 4 yo
- >75% are under age 7
- age range 3-15, but can be seen in older adolescents and adults
- M:F 2:1

Front 90

HSP - seasonality

Back 90

most cases in winter and spring

Front 91

HSP - mechanism

Back 91

IgA-mediated; it's a leukocytoclastic vasculitis with neutrophil infiltration in the vessel walls of arterioles, capillaries, and postcapillary venules. IgA and small amts of IgG and C3 are deposited

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HSP - underlying cause

Back 92

- in ~50% cases a URI infection precedes the disease
Triggers:
**Bacterial (GAS pyogenes, Legionella, Mycoplasma, Yersinia);
**Viruses (EBV, Varicella, CMV, parvovirus, hepatitis B); **Drugs (penicillin, cephalosporins, thiazide diuretics), vaccines (measles, yellow fever)
**Food additives
**Insect bites

Front 93

HSP - mc manifestation

Back 93

RASH!
seen in ALL patients, and is the presenting finding in ~50% patients

Front 94

HSP - description of skin lesions

Back 94

- begins as small wheels or red maculopapules --> progress to petechial and purpuric lesions
- found on LE and in dependent pressure-bearing areas (BUTTOCKS).
- also, face, ears in younger children
- rash lasts 4 days to 4 weeks
**ANGIOEDEMA may precede the rash
**ORCHITIS may be a hallmark of HSP !!

Front 95

HSP - arthritis outcome

Back 95

NO associated chronic arthritis

Front 96

HSP - second mc manifestation?

Back 96

- Joint Involvement in ~25% pts
- arthritis/arthralgia can be the initial HSP symp
(complicates dx until rash appears)
- Usu. arthritis of LARGE joints (knee, ankles).
- No joint effusions
- PERIARTHRITIS with edema around joints and inflammation of tendon sheaths is the mc joint manifestation!!

Front 97

HSP - 3rd mc manifestation?

Back 97

GI symptoms, occur in 65% pts: colicky abdominal pain, +/- vomiting. Pain can precede rash and other classic symptoms (dx more difficult until rash appears, may be confused with appendicitis)

Front 98

HSP and GI bleeding

Back 98

occult bleeding accompanies abdominal pain; 33% with MELENA; hematemesis rare; only 5% have major GI bleeding episode;

Front 99

HSP - next step if abd pain is severe or persistent?

Back 99

perform ULTRASOUND to r/o intussusception, which is NOT rare - especially ileoileal intussusception (2-14% of pts)

Front 100

HSP and Abdominal US findings

Back 100

AUS shows increased echogenicity and THICKENING of the wall of the 2nd portion of the DUODENUM and GB HYDROPS; occur ONLY if pt has GI symptoms

Front 101

HSP - Renal Manifestations - Incidence

Back 101

10-50% of those affected, usually mild and transient.

Front 102

HSP - Renal Manifestations

Back 102

Look for ISOLATED microscopic hematuria, or hematuria and proteinuria; mechanism: IgA deposition just like Berger Dz.

Front 103

HSP - MC Renal Outcome

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<1-2% have residual renal disease, and even fewer progress to end-stage renal disease.

Front 104

HSP - RFs for permanent renal damage?

Back 104

- > than 7 years old
- purpura lasting > 1 month
- severe, persistent GI symp
- decreased factor 13

Front 105

HSP - other features

Back 105

- orchitis
- pulmonary hemorrhage

Front 106

HSP - labs

Back 106

clinical dx, no specific labs needed to confirm. Nonspecific findings include high WBC counts, elevated ESR (50% of pts), **elevated IgA**, NORMAL platelet and coag studies

Front 107

HSP - studies

Back 107

you can use ultrasound to look for inc. echogenicity and thickening of the wall in the 2nd portion of the duodenum and gallbladder, as well as for ileoileal intussusception

Front 108

HSP - long term monitoring

Back 108

Monitor closely for at least 3 months after dx for development of renal involvement

Front 109

HSP - therapy

Back 109

- no specific Rx;
- supportive outpt care sufficient.
- no Rx for skin lesions unless severe and ulcerate, then use corticosteroids
- non-steroidals for pain control, avoid if renal or GI dz
- corticosteroids if severe abdominal pain or severe scrotal swelling/edema

Front 110

HSP nephritis - therapy?

Back 110

- no therapy has shown benefit for HSP nephritis
- Most improve w/o specific therapy, although have been trials of IV methylprednisolone, cyclophosphamide, azathioprine

Front 111

HSP - outcome

Back 111

- usu self-limited, lasting ~4wks in 65% children
- recurrences in up to 40% from 6 weeks to 2 years after initial presentation
- prognosis is excellent with most problems stemming from acute GI bleeds early in illness or long term renal involvment

Front 112

MC Vasculitis of Childhood?
2nd MC Vasculitis?

Back 112

#1 - HSP
#2 - Kawasaki

Front 113

Leading cause of acquired heart disease in children in the US?

Back 113

Kawasalki

Front 114

Kawasaki - demographic and incidence

Back 114

occurs in children under 5 years of age, affects boys more than girls (1.5:1), incidence highest in Asian children; Japan: incidence is 90/100,000 in kids <5 yo

Front 115

Kawasaki - incidence of recurrence

Back 115

nearly 1-3% have a recurrence, which is mc in boys > 6 yo or < 6 months

Front 116

Kawasaki Disease - etiology

Back 116

unknown, KD simulates an infectious disease but no organism can be identified. One theory - staphylococcal and streptococcal superantigen stimulation of the immune system

Front 117

Kawasaki Disease - diagnostic criteria

Back 117

FEVER for at least 5 days PLUS min. 4 of 5 of:
1) B/L conjunctival injection w/o exudate
2) Rash
3) MM changes (lips + oral cavity) - red pharynx, dry fissured lips, or injected, strawberry tongue
4) changes in peripheral extremities - edema or redness of the hands/feet --> later desquamation of fingers/toes
5) Cervical LAD - usu non fluctuant with at least 1 node of 1.5cm diameter
(these 5 things occur with 80-90% frequency except for cervical LAD, which is 60-70%.
**You can dx with <4 criteria if +coronary artery dz on echo

Front 118

Kawasaki Disease - describe the characteristic rash

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usually macular, polymorphous with no vesicles, scaling, or crusting on the trunk AND frequently more prominent in the perineal area later in the course followed by desquamation of this area

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3 stages of Kawasaki - Stage I

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Stage 1 KD:
Acute phase: fever 1-2 weeks, 104 or >er, 4 of 5 major criteria. IRRITABILITY. Also: aseptic meningitis, acute uveitis, diarrhea, mild obstructive jaundice with elevated LFTs, GB hydrops, sterile pyuria. Polyarthritis or polyarthralgia (knees, ankles, hands) in 33%. Asprirated fluid with PMNs, Cx (-).

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3 stages of Kawasaki - Stage I cardiac manifestations

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nearly 33% will have pericrdial effusions, and muocarditis. Coronary artery abnormalities can occur as early as day 3 of illness, buar are mc seen from 10 days to 4 weeeks after onset. Even with tx they are found in 5-9%. Increased risks in kids <1 yo, make, fever >16 days, cardiomegaly, arrhythmias other than Type I AV block, fever recurrence after 48 hrs of being afebrile.