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Flashcards in Musculoskeletal / Rheumatology Overview Deck (56)

What is the most common chronic rheumatic disease of childhood? Is it more common in men or women?

juvenile idiopathic arthritis (JIA) in women (especially in oligoarticular JIA)


What is JIA?

JIA includes eight heterogeneous subgroups of arthritis with no apparent cause, lasting more than 6 weeks, with disease onset prior to age 16


T or F. It is unusual for children to develop JIA before 6 months of age.



What is systemic onset JIA?

Arthritis with or preceded by at least 2 weeks of daily fever, with at least 3 days of documented daily (“quotidian”) fever

Plus one of more of the following:
1. Evanescent, non-fixed erythematous rash
2. Generalized lymphadenopathy
3. Hepatomegaly and/or splenomegaly
4. Serositis


What is oligoarthritic onset JIA?

Arthritis affecting 1-4 joints during the first 6 months of disease (swollen but NOT tender)


What are the subtypes of oligoarthritic onset JIA?

-persistent oligoarthritis
-extended olgioarthritis


What is persistent oligoarthritis JIA?

Arthritis of 4 or fewer joints throughout disease course of oligoarthritis JIA


What is extended oligoarthritis JIA?

Arthritis of 5 or more joints after initial 6 months of
oligoarticular disease


What are the types of polyarthritis onset JIA?

Rheumatoid Factor negative or positive


Describe RF neg polyarthritis onset JIA.

Arthritis of 5 or more joints during initial 6 months of disease AND Rheumatoid factor negative


Describe RF positive polyarthritis onset JIA.

Arthritis of 5 or more joints during initial 6 months of disease AND Rheumatoid factor positive on two or more occasions, at least 3 months apart


What is psoriatic arthritis?

Arthritis and psoriasis

Arthritis and at least two of the following:
1. Dactylitis
2. Nail pitting or onycholysis
3. Psoriasis in a first-degree relative


What is enthesitic related arthritis?

Arthritis and enthesitis

Arthritis OR enthesitis with at least two of the following
1. Sacroiliac joint tenderness and/or inflammatory
lumbosacral pain
2. HLA B27 positive
3. Arthritis in a male over 6 years of age
4. Acute anterior uveitis
5. History of ankylosing spondylitis, enthesitis-related arthritis, sacroiliitis with inflammatory bowel disease, reactive arthritis (Reiter’s syndrome), or acute anterior uveitis in a first-degree relative


T or F. One should avoid prolonged casting or immobilization in JIA

T. It is imperative that the child try to resume 'normal' functioning and activity


What is the most common type of JIA?

oligoarticular JIA accounts for almsot 60% of patients with 80% of those being girls


When does oligoarticular JIA onset occur?

most commonly from 1-3 yoa and most commonly indolently, making diagnosis more challenging


Why are disuse atrophy and joint contracture a common part of oligoarticular JIA?

It is common for children with oligo-JIA to avoid stressful positions that
aggravate their arthritis, which may result in disuse atrophy or joint contracture.


Is pain common in oligo-JIA?

not very, neither is erythema or hip involvement but they can occur


A positive ___ is present in up to 85% of patients with oligo-JIA.

ANA. NOTE: Rheumatoid factor is not seen in this subset.


What are some predictors of oligo patients evolving into extended-oligo patients?

-ankle, wrist or hand
-symmetric arthritis, -arthritis in 2-4 joints, and
-the presence of an elevated ANA titer or ESR.


Up to 50% of oligoarthritis patients with a positive ANA may develop ____.



What other things increase the risk of developing uveitis in oligo-JIA patients?

female gender, under 6 years of age, and less
than 4 years of disease duration

Such patients require slit lamp examinations every 3 months.


How is uveitis treated in oligo-JIA patients?

Most children respond to topical steroids, yet some patients may be refractory or develop complications, including glaucoma, cataracts, synechiae,
band keratopathy, macular edema, and blindness.


What is the cornerstone therapy for oligo-JIA?



Other treatment options for oligo-JIA?

Intra-articular (IA) glucocorticoid injections are often the treatment of choice for persistent arthritis of one or two joints after a trial of NSAIDs.


Polyarticular JIA acocunts for what percentage of JIA?



What are some common features of Polyarticular JIA?

Constitutional features
may be present, including fatigue, anorexia, weight loss, anemia, and low grade fever. Anterior uveitis is uncommon.


Describe RF positive Polyarticular JIA

Rheumatoid factor positive poly-JIA accounts for less than 10% of JIA and is essentially childhood onset of (adult)
rheumatoid arthritis


What patient population commonly gets RF positive Polyarticular JIA?

Seropositive disease onset is typically seen in children older than 8, with a 90% female


Which has a worse prognosis, RF + or RF- Polyarticular JIA?

RF + (although RF- is much more common)


What patient population commonly gets RF negative Polyarticular JIA?

Ninety percent of patients are girls, with peak age of onset between 1 to 3 years, although it may occur at any time.


All children with polyarthritis ultimately require what?

disease modifying anti-rheumatic drug (DMARD) therapy or a biologic agent.

Low-dose corticosteroids are used sparingly as a bridging drug for their immediate anti-inflammatory properties, as many DMARDs may require several weeks
to reach full therapeutic effect.


What is the gold standard for DMARDs?

low-dose weekly Methotrexate is effective
in over 75% of patients with JIA.


Other drugs for DMARDs?



AEs of Sulfasalazine?

Adverse events are not uncommon, including anorexia, abdominal
pain, and rash


T or F. It is advisable to update all vaccinations prior to the initiation of therapy and avoid live vaccinations during
DMARD or biologic therapy.

T, As risk of infection and response to vaccination is
of concern.


Do methotrexate of anti-TNF drugs increase the likelihood of malignancy in JIA patients?

No, even though JIA patients have a higher risk of malignancy


Biologic drugs for Poly-JIA?

-Infliximab (off-label)

combo therapy may be beneficial


AEs of Adalimumab?

-viral infections,
-pharyngitis, and -pneumonia.


How common is systemic-onset JIA?

Systemic-onset JIA (SJIA) comprises only 10% of JIA, though it accounts for a significant percentage of the morbidity and mortality in JIA.


How does systemic JIA present?

It is characterized by daily high spiking fever for at least 2 weeks and the classic salmon colored evanescent rash, found most commonly on the
trunk, axilla, and inguinal areas, with exacerbation by fever.


T or F. Arthritis may be absent at the time of diagnosis in systemic JIA

T. The extra-articular features, such as serositis, fever, anemia, general LAD, or hepato splenomegaly, often predominate.


Patient population for systemic JIA?

Unlike other subsets of JIA, there is no gender disparity, and SJIA may occur at any age.


T or F. Uveitis, ANA positivity, and
positive rheumatoid factor is very rare in SJIA.



Labs for SJIA?

-hepatitis, and -hyperferritinemia.


What are the predictors of poor prognosis in SJIA?

Onset at less than 6 years of age, disease duration for greater than 5 years, or persistent systemic features at 6 months of disease including fever, the need for corticosteroids, and thrombocytosis.


What is an uncommon but potentially life-threatening complication of SJIA?

Macrophage activation syndrome (excessive activation of T-lymph and macrophages that exhibit hemophagocytic activity)


Treatment for SJIA?

-Anakinra (anti-IL1)
-Tocilizumab (anti-IL6)


What is needed to call a JIA RF+ poslyarticular JIA?

5+ joints at onset and at least 2 occasions at least three months apart of positive IgM RF and may see joint space narrowing and joint erosion


T or F. RF Negative polyarticular JIA tends to have biphasic age peaks

T. 1-3 yo and again in late childhood /adolescence


What is a classic presentation of RF- JIA?

insidious onset of morning stiffness and gelling after inactivity that can last for hours


What joints are commonly affected by RF-neg JIA?

knees, wrists, and ankles most commonly but the small joints of the hands can be affected, as well as the TMJ (producing underdevelopment of the lower jaw) and can see fusion of spinal processes


How is systemic onset JIA defined?

arthritis with quotidian fever of at least 2+ weeks plus 1+ of the following:
1) evanescent, non-fixed rash with fever spike
2) Hepatosplenomegaly
3) Generalized LAD
4) Serositis


What are some of the triggers of MAS?

-viral infections
-Methotrexate, Etanercept


How does MAS present differently from SJIA?

you may see unremitting fever with continued hepatosplenomegaly and LAD but LESS arthritis and fibrinogen and severe cytopenias of all cell counts (WBC, RBC, and platelets) and increased PT, PTT, and D-Dimer plus encephalopathy


T or F. SCD25 and CD163 will be upregulated in MAS vs. SJIA