Children of Men
Signs
Pride and Prejudice
The Big Sick
Lorenzo's Oil
100
Typical age of AOSD
What is Young adult (<35 yo) acceptable w/o #
100
Timing of Quotidian Fever in terms of time of day + frequency
What is late afternoon/ early evening (anything PM accepted) and daily
100
Gold standard classification scheme
What is Yamaguchi criteria- bc most sensitive
100
Typical serologies/antibodies
What is None- seronegative
100
First line tx of mild
What is NSAIDS
200
Prognosis of AOSD survival rate in 5 yrs
What is good-90-95% acceptable w/o #
200
Triad of Still's
What are fever, rash, arthritis (esp poly but optional)
200
Most specific criteria for AOSD
What is Fautrel- 99%
200
Typical ferritin level
What is 1000+
200
First line DMARD in Still's
What is MTX
300
Life-threatening Complication of Still's that you should watch for
What is MAS/ sHLH
300
Typical fever temperature
What are 39 degrees C or 102 degrees F- very high
300
at least 3 of the 4 major criteria of Yamaguchi
fever 39 degrees 7+ days
arthritis/arthralgias 2+ weeks
rash
WBC >10, 80% PMNS (will accept if just say w/o details)
300
Ferritin level you should be concerned about for MAS
What is 10,000 (can start as early as 4000)
300
First line biologics (3)
What are TNF alpha IL1 IL6
Biologics: anti TNF, IL1 (anakinra canakinumab rilonacept), IL6 (tocilizumab)- induces remission in 70-80% of AOSD
Joint- TNF inhibitor (infliximab), MTX
Resistant- RTX, abatacept, IVIG, stem cell transplant
400
3 Patterns of Still's disease course
What are Monocyclic- self limiting
Intermittent Polycyclic
Chronic
400
Rash typical color, timing, and triggers (at least one)
What are salmon-colored, evanescent/ transient, heat/ mechanical irritation/ trauma (Koebner)
-other features:
Macular/ maculopapular, nonpruritic
14% Atypical urticaria
Purpura w/ low plt suggests MAS
400
Scoring needed to be Still's by Yamaguchi
What are 5 with at least 2 major
400
Typical CBC in MAS
What are cytopenias (at least 2 of 3 cell lines)
d/t interferon gamma elevation and/or phagocytosis of hematopoietic cells by macrophages in bone marrow and reticuloendothelial system
Dysregulated CD8+ T lymphocytes and excessive production of cytokines-> abnormal macrophage activation-> consumptive coagulopathy (DIC with elevated PT/PTT, low fibrinogen, inappropriately low ESR)-> multiorgan dysfxn
400
Consider this in refractory Stills or if genetic mutation for sHLH
What is etoposide/ stem cell/ bone marrow transplant either one
500
Arthritis complication that can happen and is very characteristic per Dr Cush
What is ankylosis of carpal bone
500
Reason for sore throat
What is perichondritis of cricothyroid cartilage
500
Distinctions between Yamaguchi and Fautrel Criteria (at least 2)
What are: Fautrel needs 4 major or 3 major + 2 minor; Yamaguchi 5 with at least 2 major
What are: Fautrel accounts for exceptions that Yamaguchi doesn't
-major glycosylated ferritin <20, pharyngitis (instead of being minor)
-minor maculopapular rash; leukocytosis (instead of being major)
500
Receptor elevated in MAS that you could consider
What is sIL2 R
500
First line treatment in MAS
What is high dose steroids (1 mg/kg/d)