Pediatric Rheumatology Flashcards

Question

What are the laboratory findings of KD?

Answer 1

increased ESR and CRP elevated white blood cell count anemia platelets rise by 2nd week ECG and echocardiogram - carditis, coronary aneurisms

Answer 2

goal - control acute inflammation and symptoms prevent long-term sequelae and aneurismss high dose **aspirin and IVIG** within the first 10 days greatly reduces risk of coronary involvement steroids, biologics if **refractory** to IVIG

Answer 3

IVIG reduces incidence of aneurisms by 78% 5% of kids treated with IVIG develop aneurisms almost always monophasic - very rare to recur risk of cardiac death due to aneurisms

Answer 4

purpuric rash in the skin vasculitis of the kidney - proteinuria abdominal pain with bloody diarrhea arthritis

Answer 5

most common childhood vasculitis most frequent between 3-15 years mean age of onset is approximately 7 years old female-male ratio is 1:1.5 more cases in winter months

Answer 6

could be triggered by Group A strep 30-50% preceded by URI IgA-mediated dysregulated immune response to antigen alternative complement pathway is triggered familial clustering - HLA predisposition

Answer 7

palpable purpura - dependent distribution that can be preceded by macules and urticaria GI manifestations within a week of rash due to vasculitis **currant jelly stools** colicky abdominal pain - risk of **_intussusception_** **kidney involvement** within 4-6 weeks of rash - microscopic hematuria, proteinuria, and renal failure **arthralgia, arthritis** in 50-80% predominantly lower extremities **subcutaneous edema** often periarticular scrotal pain and swelling

Answer 8

**NO thrombocytopenia!** basic labs often unremarkable elevated IgA with deposition in mesangial and GI vasculature check urinalysis (not dip) with spot protein/creatinine and blood pressure

Answer 9

symptomatic - hydration, bland diet, analgesia, anti-inflammatories if severe abdominal pain, bloody stools, evaluate for intussusception and consider steroids corticosteroids do not prevent renal disease but may be helpful when renal involvement is established

Answer 10

in 2/3 of cases, HSP runs entire course within 4 weeks (monophasic) generally prognosis is excellent \<5% progress to renal failure late kidney involvement rare but possible - monitor urinalysis

Answer 11

inflammatory myopathy systemic vasculopathy cutaneous findings proximal symmetric myositis progressive proximal muscle weakness not associated with cancer in children

Answer 12

incidence of 3.2/million children/year 73% of cases in the US are caucasian female:male ratio is 2.3:1 average age at onset is 6.7 years but 25% are below 4

Answer 13

definite diagnosis reqires rash plus 3 or 4 of the following: symmetrical proximal muscle weakness elevation of skeletal muscle enzymes in serum electromyographic findings typical of myositis muscle biopsy findings typical of JDM

Answer 14

**UV exposure** antibodies - myositis specific and associated genetic susceptibility - 80% have DQQA1\*0501 cytokine polymorphisms: TNF-alpha

Answer 15

putative antigen and/or immune complexes activate endothelial cells endothelium is damaged by complement activation - release of von Willebrand factor antigen occlusion of capillaries and arterioles results in infarction of local tissues cutaneous manifestations caused by capillary and arteriolar vasculopathy and occlusion dermis - vascular inflammation and edema thinned epidermis

Answer 16

disease onset is usually insidious nonspecific symptoms - fatigue, low grade fever, weight loss, irritability, arthralgia, and abdominal pain can be acute with high fever and profound clinical symptoms

Answer 17

rash + weakness in 50% of cases rash occurs first in 25% of cases **rash is prominent on sun-exposed surfaces** - torso (shawl or V distribution) - extensor surfaces of extremities - scalp with hair loss due to chronic inflammation

Answer 18

a finding of juvenile dermatomyositis hypertrophic, reddish pink areas of skin with are papular and scaly occur over the MCP and interphalangeal joints as well as elbows and knees heal as atrophic colorless bands

Answer 19

a finding in juvenile dermatomyositis periorbital violaceous erythema may cross nasal bridge

Answer 20

nail bed telangiectasia infarction of oral epithelium and skin-folds digital, skin and gastrointestinal ulceration

Answer 21

proximal muscle weakness often insidiuous cmon signs include difficulty climbing stairs, getting on school bus, combing hair, arising from bed or chair **Gower's sign** - difficulty rising from floor inability to raise head off bed (neck flexors) inability to perform a sit-up (abdominals)

Answer 22

hoarse voice, nasal speech dysphagia - poor prognostic sign requiring aggressive mdial management shortness of breath - interstitial lung diseae constipation, hematochezia from weakness, gut vasculopathy

Answer 23

muscle and fat atrophy calcinosis cutis - in subcutaneous tissue and fascia - 20% of patients - consequenece of delayed/insufficient suppression of inlammation - potential source of ulceration, infection

Answer 24

elevated muscle enzymes reflecting leaky muscle membranes - can be subtle **- CK** **- aldolase** **- AST, ALT** **- lactate dehydrogenase** **elevated von Willebrand factor** antigen reflecting disrupted endothelium **CRP/ESR usually normal** anemia is possible **RF is negative** kidney function tests are normal myositis specific antibodies predict findings, prognosis

Answer 25

decreased bone density - increased fractures with increase disease duration calcifications - associatated with TNF-alpha-308A, untreated long disease, major contributor to mortality and morbidity partial lipodystrophy - associated with insulin resistant diabetes

Answer 26

oral and IV corticosteroids methotrexate other steroids sparing immunosuppressants - cyclosporine, mycophenolate mofetil IVIG for skin disease hydroxycloroquine for skin disease calcium and vitamin D sunscreen at least SPF 45

Answer 27

current morality is 1% long term outcome secondary to systemic vasculopathy is unclear - cardiovascular morbidity likely long duration of untreated disease predicts poor outcome aggressive treatment strategies decreases time to remission