Adult onset Still's dx Flashcards

1
Q

most common features of adult onset Still’s dx in clinical presentation:

A

high fever
arthritis
transient rash

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2
Q

variable features on adult onset Still’s dx in clinical presentation:

A

LAD
sore throat
hepatomegaly, splenomegaly
pleuritis, pericarditis

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3
Q

Lab findings of adult onset Still’s dx?

A

leukocytosis
elevated inflammatory markers (ESR and C reactive protein)
markedly elevated ferritin
negative ANA and neg RF

can have normocytic anemia
can have thrombocytosis
can have elevated LFTs

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4
Q

lab that you should associate with acute adult onset still’s dx?

A

ferritin (will be in the thousands)

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5
Q

acute adult onset still’s dx rash is characterized by:

A

transient faint salmon colored macular rash that comes and goes with fever

not pruritic

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6
Q

acute adult onset still’s dx is a

A

systemic onset juvenile idiopathic arthritis and inflammatory disorder characterized by high daily recurring fevers and polyarthritis and neutrophilic leukocytosis

see a daily transient, non pruritic salmon colored rash that appears only with fever

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7
Q

what causes acute adult onset still’s dx

A

high levels of infalmmatory cytokines IL-1 and IL-6 and TNF alpha inhibitors
see elevated ESR and CRP

see high ferritin which is in 1000’s.

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8
Q

classic triade:

A

fever
arthalgia
rash

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9
Q

major Yamaguchi criteria:

A

four major:
fever at least 102.2 for at least one week
arthralgias lasting at least 2 weeks
non pruritic macular or maculopapular salmon color rash over the trunk and extremiteis that coincides with fever

leukcoytosis >10K and at least 80% granulocytes

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10
Q

minor Yamaguchi criteria

A
sore throat
hepatomegaly/splenomegaly
lymphadenopathy
abnormal liver function tests
negative ANA and RF
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11
Q

diagnosis of Acute onset still’s dx

A

need to have 5 criteria met and 2 must be major

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12
Q

treatment acute onset still’s disease is based on

A

symptom severity

ranges from NSAIDS for mild symptoms to high dose steroids and biologics for moderate to severe dx

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13
Q

follow up for acute onset still’s dx

A

3 month intervals with blood chemistreis

follow up CBC CMP and ferritin

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14
Q

acute febrile neutrophilic dermatosis

A

acute febrile neutrophilic dermatosis AKA Sweet’s syndrome

has sudden eruption of erythematous papules, plaques, or nodules that are painful and edematous.

See fever and leukocytosis with Sweet’s but skin biopsy will tell the difference between the two.

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15
Q

differential diagnosis for Still’s

A

acute febrile neutrophilic dermatosis AKA Sweet’s syndrome

non hodgkin lympohma - see night sweats, weight loss >10% body weight

polyarteritis nodosa (PAN) systemic necrotizing vasculitis and diagnosis is confirmed with skin biopsy.

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16
Q

Adult Still’s dx is also known as

A

juvenile idiopathic arthritis

presents in 15-25 years and 35-45 years are similar.

can have cardiopulmonary symptoms such as pericarditis, pleural effusions, pleuritic chest pain