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STEP Pathology > deck_5362190 > Flashcards

deck_5362190 Flashcards

1
Q

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

A

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

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2
Q

What is JIA?

A

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

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3
Q

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

A

T.

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4
Q

What is systemic onset JIA?

A

Arthritis with or preceded by at least 2 weeks of daily fever, with at least 3 days of documented daily (“quotidian”) feverPlus one of more of the following:1. Evanescent, non-fixed erythematous rash2. Generalized lymphadenopathy3. Hepatomegaly and/or splenomegaly4. Serositis

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5
Q

What is oligoarthritic onset JIA?

A

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

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6
Q

What are the subtypes of oligoarthritic onset JIA?

A

-persistent oligoarthritis-extended olgioarthritis

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7
Q

What is persistent oligoarthritis JIA?

A

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

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8
Q

What is extended oligoarthritis JIA?

A

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

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9
Q

What are the types of polyarthritis onset JIA?

A

Rheumatoid Factor negative or positive

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10
Q

Describe RF neg polyarthritis onset JIA.

A

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

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11
Q

Describe RF positive polyarthritis onset JIA.

A

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

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12
Q

What is psoriatic arthritis?

A

Arthritis and psoriasisorArthritis and at least two of the following:1. Dactylitis2. Nail pitting or onycholysis3. Psoriasis in a first-degree relative

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13
Q

What is enthesitic related arthritis?

A

Arthritis and enthesitisorArthritis OR enthesitis with at least two of the following1. Sacroiliac joint tenderness and/or inflammatorylumbosacral pain2. HLA B27 positive3. Arthritis in a male over 6 years of age4. Acute anterior uveitis5. 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

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14
Q

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

A

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

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15
Q

What is the most common type of JIA?

A

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

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16
Q

When does oligoarticular JIA onset occur?

A

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

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17
Q

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

A

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

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18
Q

Is pain common in oligo-JIA?

A

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

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19
Q

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

A

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

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20
Q

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

A

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

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21
Q

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

A

uveitis

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22
Q

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

A

female gender, under 6 years of age, and lessthan 4 years of disease durationSuch patients require slit lamp examinations every 3 months.

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23
Q

How is uveitis treated in oligo-JIA patients?

A

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.

24
Q

What is the cornerstone therapy for oligo-JIA?

A

NSAIDs

25
Q

Other treatment options for oligo-JIA?

A

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

26
Q

Polyarticular JIA acocunts for what percentage of JIA?

A

25-40%

27
Q

What are some common features of Polyarticular JIA?

A

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

28
Q

Describe RF positive Polyarticular JIA

A

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

29
Q

What patient population commonly gets RF positive Polyarticular JIA?

A

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

30
Q

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

A

RF + (although RF- is much more common)

31
Q

What patient population commonly gets RF negative Polyarticular JIA?

A

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

32
Q

All children with polyarthritis ultimately require what?

A

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 weeksto reach full therapeutic effect.

33
Q

What is the gold standard for DMARDs?

A

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

34
Q

Other drugs for DMARDs?

A

-Sulfasalazine -Leflunomide

35
Q

AEs of Sulfasalazine?

A

Adverse events are not uncommon, including anorexia, abdominalpain, and rash

36
Q

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

A

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

37
Q

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

A

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

38
Q

Biologic drugs for Poly-JIA?

A

-Etanercept-Adalimumab-Infliximab (off-label)-Abataceptcombo therapy may be beneficial

39
Q

AEs of Adalimumab?

A

-viral infections,-pharyngitis, and -pneumonia.

40
Q

How common is systemic-onset JIA?

A

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

41
Q

How does systemic JIA present?

A

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

42
Q

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

A

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

43
Q

Patient population for systemic JIA?

A

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

44
Q

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

A

T.

45
Q

Labs for SJIA?

A

-Leukocytosis,-thrombocytosis, -anemia, -hepatitis, and -hyperferritinemia.

46
Q

What are the predictors of poor prognosis in SJIA?

A

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.

47
Q

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

A

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

48
Q

Treatment for SJIA?

A

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

49
Q

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

A

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

50
Q

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

A

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

51
Q

What is a classic presentation of RF- JIA?

A

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

52
Q

What joints are commonly affected by RF-neg JIA?

A

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

53
Q

How is systemic onset JIA defined?

A

arthritis with quotidian fever of at least 2+ weeks plus 1+ of the following:1) evanescent, non-fixed rash with fever spike2) Hepatosplenomegaly3) Generalized LAD4) Serositis

54
Q

What are some of the triggers of MAS?

A

-viral infections-NSAIDS-Methotrexate, Etanercept-Sulphasalazine

55
Q

How does MAS present differently from SJIA?

A

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

56
Q

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

A

T.

STEP Pathology (80 decks)

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