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MOS > SJIA, HLH, MAS > Flashcards

SJIA, HLH, MAS Flashcards

1
Q

Most common type of chronic rheumatic disease of childhood; onset <16 years, one more more joints (swelling, effusion, limited ROM, etc.) and exclude any other form; due to genetic predisposition, environmental triggers and disordered immune response

A

Juvenile Idiopathic Arthritis

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2
Q

Diagnostic Criteria for Juvenile Idiopathic Arthritis

A

Onset <16 yo
Arthritis one or more joints >6 weeks (Swelling, effusion, limited ROM, etc.)
Exclude other arthritis or disorders

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3
Q

Lab values for Juvenile Idiopathic Arthritis

A

NO SPECIFIC LAB VALUES

Inc. WBCs
Mild normocytic anemia
Inc. ESR/CRP

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4
Q

Subtypes of Juvenile Idiopathic Arthritis (6 total)

A 
Oligoarthritis (<4 joints)
Polyarthritis (RF-)
Polyarhtritis (RF+)
Enthesitis-related Arthritis
Psoriatic Arthritis
Systemic Arthritis
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5
Q

Subtype of Juvenile Idiopathic Arthritis; <4 joints; MAJORITY of JIA; peaks between 1 and 2 years old; FEMALES; affects lower extremities; GOOD prognosis

A

Oligoarticular

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6
Q

Oligoarticular arthritis involves (males/females) and the (upper/lower) extremities with a (good/poor) prognosis

A

Females; Lower; Good

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7
Q

Most common subtype of Juvenile Idiopathic Arthritis

A

Oligoarthritis (<4 joints)

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8
Q

Eye finding that is commonly found with HLA-B27 arthritis (Oligoarthritis, Psoriatic, etc.)

A

Uveitis

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9
Q

RF (positive/negative) Polyarthritis is most common

A

RF-negative

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10
Q

Antibody associated with many types of arthritis; IgM antibody against the Fc portion of IgG

A

Rheumatoid Factor

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11
Q

Antibody that is more SPECIFIC for Rheumatoid Arthritis; against cyclic citrullinated proteins; predictor of more PROGRESSIVE disease

A

Citrullinated Protein Antibody

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12
Q

(RF/Anti-CCP) is more specific for Rheumatoid Arthritis

A

Anti-CCP (a few different arthritis have RF)

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13
Q

Subtype of Juvenile Idiopathic Arthritis; seen more commonly in MALES; inflammation of tendons/ligaments, resulting in Sacroiliac tenderness, uveitis and HLV-B27

A

Enthesitis-Related Arthritis

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14
Q

Subtype of Juvenile Idiopathic Arthritis is more common in males

A

Enthesitis-Related Arthritis

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15
Q

Subtype of Juvenile Idiopathic Arthritis; associated with dermatologic findings, such as dactylitis, nail pitting, onycholysis and Psoriasis

A

Juvenile Psoriatic Arthritis

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16
Q

Subtype of Juvenile Idiopathic Arthritis; characterized by FEVER, arthritis of any joint, rash, lymphadenopathy, hepato/splenomegaly and serositis

A

Systemic Juvenile Idiopathic Arthritis

17
Q

Diagnostic Criteria for Systemic Juvenile Idiopathic Arthritis

A

FEVER
Arthritis of any joint >6 weeks
Any of the following: rash, lymphadenopathy, hepato/splenomegaly or serositis

18
Q

Almost 1/3rd of patients with JIA will develop…..

A

Macrophage Activation Syndrome

19
Q

Seen in 1/3rd of patients with JIA; overwhelming systemic inflammatory response with HIGH FATALITY rates; characterized by Fever, Hepatosplenomegaly, Lymphadenopathy, Encephalopathy, Rash, Bruising and Multi-Organ failure

A

Macrophage Activation Syndrome

20
Q

Signs/Symptoms of Macrophage Activation Syndrome

A 
Fever
Hepatosplenomegaly
Lymphadenopathy
Encephalopathy
Rash
Bruising/Purpura
Multi-Organ Failure
21
Q

IL-1 inhibitors (3 total)

A

Anakinra
Canakinumab
Rilanocept

22
Q

IL-6 inhibitors

A

Tocilizumab

23
Q

Rare but potentially fatal disease of normal but overactive histiocytes and CD8 T cells that commonly appears in infancy; characterized by fever, hepatosplenomegaly, pancytopenia, lymphadenopathy, rash, low NK cell function and histiocytes with ENGLUFED RBCs

A

Hemophagocytic Lymphohistiocytosis (HLH)

24
Q

Lab findings for Hemophagocytic Lymphohistiocytosis

A 
INC. FERRITIN
Dec. fibrinogen
Low/absent NK cell
Cytopenias
Fever
Splenomegaly
Hemophagocytosis (below)
25
Q

What is the distinction between HLH and MAS?

A

HLH has inflammation due to impaired CD8 T cells

26
Q

HLH is associated with what kinds of malignancy

A

T/NK Lymphomas and Leukemias

ALL and AML

27
Q

Most frequent infections that lead to HLH

A

EBV*
CMV
HIV

28
Q

Most common drugs that cause HLH

A 
Etanercept (TNFa)
Rituximamb (anti CD20)
Vancomycin
TMP-SMX
G-CSF
Anti-epileptics (Lamotrigine, Phenytoin, etc.)
29
Q

Treatment for HLH

A

Dexamethasone
Etoposide
IVIg

*chronic may need stem cell transplant

MOS (7 decks)

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