SJIA, HLH, MAS Flashcards
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
Juvenile Idiopathic Arthritis
Diagnostic Criteria for Juvenile Idiopathic Arthritis
Onset <16 yo
Arthritis one or more joints >6 weeks (Swelling, effusion, limited ROM, etc.)
Exclude other arthritis or disorders
Lab values for Juvenile Idiopathic Arthritis
NO SPECIFIC LAB VALUES
Inc. WBCs
Mild normocytic anemia
Inc. ESR/CRP
Subtypes of Juvenile Idiopathic Arthritis (6 total)
Oligoarthritis (<4 joints) Polyarthritis (RF-) Polyarhtritis (RF+) Enthesitis-related Arthritis Psoriatic Arthritis Systemic Arthritis
Subtype of Juvenile Idiopathic Arthritis; <4 joints; MAJORITY of JIA; peaks between 1 and 2 years old; FEMALES; affects lower extremities; GOOD prognosis
Oligoarticular
Oligoarticular arthritis involves (males/females) and the (upper/lower) extremities with a (good/poor) prognosis
Females; Lower; Good
Most common subtype of Juvenile Idiopathic Arthritis
Oligoarthritis (<4 joints)
Eye finding that is commonly found with HLA-B27 arthritis (Oligoarthritis, Psoriatic, etc.)
Uveitis
RF (positive/negative) Polyarthritis is most common
RF-negative
Antibody associated with many types of arthritis; IgM antibody against the Fc portion of IgG
Rheumatoid Factor
Antibody that is more SPECIFIC for Rheumatoid Arthritis; against cyclic citrullinated proteins; predictor of more PROGRESSIVE disease
Citrullinated Protein Antibody
(RF/Anti-CCP) is more specific for Rheumatoid Arthritis
Anti-CCP (a few different arthritis have RF)
Subtype of Juvenile Idiopathic Arthritis; seen more commonly in MALES; inflammation of tendons/ligaments, resulting in Sacroiliac tenderness, uveitis and HLV-B27
Enthesitis-Related Arthritis
Subtype of Juvenile Idiopathic Arthritis is more common in males
Enthesitis-Related Arthritis
Subtype of Juvenile Idiopathic Arthritis; associated with dermatologic findings, such as dactylitis, nail pitting, onycholysis and Psoriasis
Juvenile Psoriatic Arthritis
Subtype of Juvenile Idiopathic Arthritis; characterized by FEVER, arthritis of any joint, rash, lymphadenopathy, hepato/splenomegaly and serositis
Systemic Juvenile Idiopathic Arthritis
Diagnostic Criteria for Systemic Juvenile Idiopathic Arthritis
FEVER
Arthritis of any joint >6 weeks
Any of the following: rash, lymphadenopathy, hepato/splenomegaly or serositis
Almost 1/3rd of patients with JIA will develop…..
Macrophage Activation Syndrome
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
Macrophage Activation Syndrome
Signs/Symptoms of Macrophage Activation Syndrome
Fever Hepatosplenomegaly Lymphadenopathy Encephalopathy Rash Bruising/Purpura Multi-Organ Failure
IL-1 inhibitors (3 total)
Anakinra
Canakinumab
Rilanocept
IL-6 inhibitors
Tocilizumab
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
Hemophagocytic Lymphohistiocytosis (HLH)
Lab findings for Hemophagocytic Lymphohistiocytosis
INC. FERRITIN Dec. fibrinogen Low/absent NK cell Cytopenias Fever Splenomegaly Hemophagocytosis (below)
What is the distinction between HLH and MAS?
HLH has inflammation due to impaired CD8 T cells
HLH is associated with what kinds of malignancy
T/NK Lymphomas and Leukemias
ALL and AML
Most frequent infections that lead to HLH
EBV*
CMV
HIV
Most common drugs that cause HLH
Etanercept (TNFa) Rituximamb (anti CD20) Vancomycin TMP-SMX G-CSF Anti-epileptics (Lamotrigine, Phenytoin, etc.)
Treatment for HLH
Dexamethasone
Etoposide
IVIg
*chronic may need stem cell transplant
