SYSTEMIC ONSET JUVENILE IDIOPATHIC ARTHRITIS

Systemic Onset Juvenile Idiopathic Arthritis: Cause: Disordered Immune Response; Of all kids with JIA, how much is this type? What is the name of the disease if diagnosed in adulthood? What is the pathogenesis of systemic JIA? How does that differ from the oligo/Poly subtypes?

! Accounts for 5 to 15% of all children with JIA ² Incidence of 0.3 to 0.8 cases/100,000 children/year ² Accounts for higher percentage of JIA in Asia ! Broad peak of onset from 1 to 5 years ! Also occurs in adolescence and adulthood ² Called adult-onset Still's disease with later age of onset ! Equal gender distribution

JIA subtypes: What is the definition? What are the 6 subtypes?

! Group of related, but distinct, types of arthritis is the most common type of arthritis in children under the age of 17. Juvenile rheumatoid arthritis causes persistent joint pain, swelling and stiffness.

Serositis: What is the definition (what 2 places)? Is parenchymal pulmonary disease common? Is spleen or liver more commonly involved? What may you see?

! No specific laboratory test for diagnosis ! Supports the clinical diagnosis and indicative of inflammation ² Leukocytosis with WBC 20,000 to 30,000 cells/mm3 and predominance of neutrophils ² Anemia ² Marked elevation in platelet count ² Elevated inflammatory markers, ferritin, fibrinogen ! Autoantibodies are uncommon

Differential Diagnosis: What are 5 things on the DDx?

! Other rheumatic diseases ² Systemic lupus erythematosus ² Dermatomyositis ² Polyarteritis nodosa ² Kawasaki disease ² Serum sickness ² Sarcoidosis ! Inflammatory bowel disease ! Autoinflammatory syndromes ² Familial Mediterranean fever (FMF) ² Hyperimmunoglobulin D syndrome (HIDS) ² TNF receptor-associated periodic syndrome (TRAPS) ² Muckle-Wells syndrome (MWS) ² Familial cold autoinflammatory syndrome (FCAS) ² Chronic infantile neurological cutaneous and articular syndrome (CINCA) ² Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA)

Criteria for Diagnosis: What does the age of onset have to be? What 4 things have to happen to the joints (these all define arthritis)? For how long? What has to be excluded (2 things)?

! The most common chronic rheumatic disease of childhood ² Incidence of 1-20/100,000 children ² Prevalence of 10-20/100,000 children ! One of the more common chronic diseases of childhood ² 1 child in 1,000 will develop juvenile arthritis ² >300,000 children in US have juvenile arthritis

IL-1 Inhibitors: What are the 3 drugs? What does each do?

3rd: pieces of the receptor floating around IL1-A and IL1-B...Theses are pro-inflammatory...when they bind, you increase activation of transcription that increases expression of pro-inflammatory molecules...ACTIVATES the INNATE IMMUNE SYSTEM LEADING TO inflammation, synovial pannus formation

Secondary Amyloidosis: What is this a complication of? When does it occur? What is it assc with (elevations)? What is the major cause of death? What type of diagnosis is this?

ANOTHER COMPLICATION OF JIA Diagnostic Challenge ! Diagnosis of exclusion ! Arthritis may not be evident early in the course ! Systemic features may not be present in their typical forms initially ! There are no diagnostic tests for the disorder ! Affected children are quite ill, but with nonspecific signs and symptoms such as fever, rash, anemia, and/or leukocytosis

Indications for IL-1 Inhibitors: What are 3 indications? (3 of the 3rd)? What are the 3 main side-effects?

IL-1 Inhibitors Side Effects ! Increased risk of infections ² Bacterial and fungal infections ² Tuberculosis ² No live viral vaccines ² No concomitant administration of TNF inhibitors ! Cytopenias, including neutropenia ! Injection site reactions

Indications for tocilizumab: What are the 2 indications (3 of the second)? What are the 4 side effects?

Tocilizumab Adverse Effects ! Increased risk of infections ² Bacterial and fungal infections ² Tuberculosis ² No live viral vaccines ! Changes in blood cell counts ! Liver toxicity ! Infusion reactions

Systemic JIA: Diagnostic Criteria: What are the 4 diagnostic criteria?

Other Clinical Manifestations ! Fatigue ! Malaise ! Weight loss ! Myalgias ! Myositis

Disease Course and Outcomes: What are the 3 disease course patterns? Death common? What are some poor prognostic indicators?

Poor Prognostic Indicators ! Persistent fever ! Steroid dependency ! Marked thrombocytosis ! Polyarticular arthritis ! Hip involvement ! Early joint damage ! Delay in and lack of response to treatment

Enthesitis-Related Arthritis: B or G? Are the ok with function and diffuse bodily pain? Symetric and Asymetric? What are 2 axial skeletal disease they can get? What other disease is there overlap with?

THE ONLY ONE THAT IS MORE COMMON IN BOYS!!! -may be precursor to JAS

Citrullinated Protein Antibody: What is this antibody against? Concordance with RF positivity? More or less specific for RA? What are 2 predictors of disease course?

That portion of the protein is changed to "foreign"

MAS: Laboratory Evaluation: What kind of cytopenia will they have? What will be increased in the liver? Therefore, what will be prolonged? Why is ferritin elevated? Triglycerides? Fibrinogenemia? What will there be a sharp drop in ? OVERALL, WHAT IS THE HALLMARK?

The hallmark: In the liver, spleen , lymph nodes...you get macros that engulf other hematopeotic cells

IL-6 Inhibitors: How does this work?

binds to both soluble and insoluble IL-6 receptors pro-inflammatory...Hot, T-bone Steak...K=6=akut phase reactancts

Traditional Therapies for sJIA: What are 7 traditional therapies for sJIA? What is a biologic?

biologic: molecules that require some type of living organisms for production

Juvenile Psoriatic Arthritis: What are the 2 definitions of this? (3 of the second)

dactylitis: sausage digit is inflammation of an entire digit (a finger or toe), and can be painful. The word dactyl comes from the Greek word "daktylos" meaning "finger"

Enthesitis-Related Arthritis: What are the 2 ways this can be diagnosed? (5 of the second)

enthesitis: is the connective tissue between tendon or ligament and bone

Uveitis: Why are we talking about this? Symptoms? Bilateral? Independent of the arthritis? What 3 subtypes more commonly have this complications?

inflammation that can occur in any chamber...this is a common complication of arthritis

RF Positive Polyarthritis: How many joints? How do definitely say it is RF +? What other antibody? Symmetrical? Which joints? Remission rate? Joint deformity?

most like adult RA