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PBR: Rheumatology > Juvenile Idiopathic Arthritis > Flashcards

Juvenile Idiopathic Arthritis Flashcards

1
Q

What is the most common rheumatic disease diagnosed in children?

A

Juvenile idiopathis arthritis

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2
Q

How is Juvenile Idiopathic Arthritis defined?

A

Occurring before the age of 16 years, persistent synovitis in ≥1 joint(s), synovitis for at least 6 weeks.

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3
Q

What are the 7 categories of JIA?

A

Systemic (sJIA), Oligoarthritis (oJIA), Polyarthritis rheumatoid factor negative (poJIA RF-), Polyarthritis rheumatoid factor positive (poJIA RF+), Psoriatic arthritis (psJIA), Enthesitis-related arthropathies (ERAs), and Undifferentiated (uJIA).

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4
Q

What findings are required to make a diagnosis of systemic JIA?

A

Arthritis + fever + a systemic finding (rash, hepatomegaly, splenomegaly, lymphadenopathy, or serositis).

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5
Q

What findings are required to make a diagnosis of oligoarthritis JIA?

A

No more than 4 joints affected during the first 6 months of the disease.

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6
Q

What are the two subcategories of oligoarthritis JIA?

A

Persistent oligoarthritis (affecting ≤4 joints for the duration of the disease) and Extended arthritis (affecting ≥5 joints after the first 6 months of disease).

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7
Q

What findings are required to make a diagnosis of polyarthritis RF- JIA?

A

Affects ≥5 joints during the first 6 months of disease and RF -

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8
Q

What findings are required to make a diagnosis of polyarthritis RF+ JIA?

A

Affects ≥5 joints during the first 6 months of disease and RF +

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9
Q

Which cytokines are believed to mediate JIA?

A

TNF-α, IL-1, and IL-6, which cause the release of other mediators such as prostaglandins, complements, proteases, and others.

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10
Q

What would you expect to see in the synovial fluid of a child with JIA?

A

Synovial WBC, comprised mostly of lymphocytes, are usually between 2,000 and 30,000 cells/mL but can be as high as 100,000 cells/mL.

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11
Q

What is the typical pattern of joint stiffness associated with JIA?

A

Morning stiffness that improves with movement as the day goes on.

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12
Q

In patients with JIA, what might be seen on x-ray of involved fingers?

A

If JIA involves the fingers, it is characteristic to see widening of the midportion of the affected phalanges from periosteal new bone formation.

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13
Q

Name and describe the fever seen in systemic JIA.

A

Characteristically, these patients have 1-2 fever spikes on a daily basis, and the fevers resolve without antipyretics. Usually, the fever is in the evening and can be associated with severe myalgia and arthralgia. This is called a quotidian fever.

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14
Q

What is the peak age for diagnosis of systemic JIA?

A

2 years of age, with incidence levels decreasing with increasing age.

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15
Q

What is the only type of JIA to affect males and females equally?

A

Systemic JIA

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16
Q

What is the only type of JIA to affect Males > Females?

A

Enthesitis-related arthropathies

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17
Q

List the 4 subtypes of JIA which have Female > Male incidence.

A

Oligoarticular, Polyarticular RF+/-, and psoriatic.

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18
Q

Name two poor prognostic indicators for systemic JIA.

A

Persistent, active disease 1 year after onset and diagnosis before 4 years of age.

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19
Q

Describe the rash of systemic JIA.

A

The rash is migratory with macular, pink-to-salmon coloring and discrete borders with or without central clearing, usually coinciding with the fever. The rash is usually present on the trunk, thighs, and axillae.

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20
Q

What are the expected lab findings in a patient with systemic JIA (WBC, Plt, CRP, ESR, Ferritin, RF, and ANA)?

A

Lab findings in systemic JIA can be very abnormal: leukemoid reaction (>40,000 WBC/µL), thrombocytosis (occasionally >1 million cells/µL), high CRP and ESR, Ferritin levels are often elevated (however, if >5,000-100,000 ng/mL, think MAS), RF is negative, and ANA is only rarely positive.

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21
Q

What is macrophage activation syndrome (MAS)?

A

A life-threatening condition found most commonly in patients with uncontrolled systemic JIA (though it can occur due to some types of infection and drugs as well). It is characterized by: persistent fever, hepatosplenomegaly, extremely elevated ferritin, cytopenias (affecting at least 2 of the 3 cell lines), liver dysfunction (elevated LFTs, coagulopathy, low fibrinogen, and/or elevated triglycerides), and neurologic dysfunction.

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22
Q

Which cell type engulfs the other cell types (hemophagocytosis) in MAS?

A

Hemophagocytosis by macrophages/histiocytes is evident on bone marrow biopsy, although not always present.

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23
Q

How is macrophage activation syndrome treated?

A

High-dose corticosteroids, IL-1 or IL-6 inhibitors, and/or cyclosporine.

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24
Q

What is the average age of onset for oligoarticular JIA?

A

1-3 years of age

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25
Q

Which joint is frequently involved in oJIA but is often silent, requiring diagnosis by MRI?

A

The temporomandibular joint (TMJ)

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26
Q

Which joints are most commonly involved in oJIA (in descending order of frequency)?

A

Knee > Ankle > Fingers/toes > Elbows > Wrists > Hips (rare)

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27
Q

Which of the two subtypes of oJIA has a worse prognosis?

A

Extended oligoarthritis (disease in ≥5 joints after the first 6 months)

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28
Q

In which eye chamber is uveitis due to JIA found?

A

Aymptomatic anterior uveitis can occur in about 30% of patients with oJIA.

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29
Q

In oligoarticular JIA, the presence of a positive ANA increases the risk of developing what eye finding?

A

Uveitis

30
Q

When is uveitis most likely to occur in patients with oJIA?

A

Within the first 5-7 years of initial JIA presentation.

31
Q

Which patients with oJIA require slit-lamp exams every 3 months?

A

≤6 years of age at time of diagnosis, ANA positive, and recent onset of disease (within 4 years)

32
Q

Which patients with oJIA require slit-lamp exams every 6 months?

A

≤6 years of age at time of diagnosis, ANA negative, and child is 4-7 years beyond the time of diagnosis

33
Q

Which patients with oJIA require slit-lamp exams every 12 months?

A

> 6 years of age at time of diagnosis, ANA negative, and >7 years beyond diagnosis

34
Q

What is the relationship between ANA positivity and JIA?

A

ANA should not be used as a diagnostic tool for JIA, but is instead used as a prognostic indicator for risk of uveitis in children with JIA.

35
Q

What are the typical laboratory findings in patients with oJIA?

A

Labs are typically normal, including inflammatory markers. Thrombocytopenia is nonspecific and can be seen in any patient with JIA and inflammation.

36
Q

At what ages is polyarticular JIA RF- usually diagnosed?

A

It has two age peaks: 1-3 years of age and 9-14 years of age.

37
Q

Which joints are most commonly involved in RF- poJIA?

A

poJIA affects both large and small joints: it typically involves the cervical spine, hips, shoulders, TMJs, MCPs, and PIPs.

38
Q

What age group is more commonly affected by RF+ poJIA?

A

RF+ disease more frequently occurs in older adolescents

39
Q

In polyarticular JIA, what does the presence of RF and anti-CCP antibodies indicate?

A

RF positivity is a poor prognostic finding and mandates aggressive management of the patient. Anti-CCP antibodies are associated with erosive arthritis. Most, but not all, RF+ poJIA patients are CCP+ and vice versa.

40
Q

What criteria are required to make the diagnosis of psoriatic JIA?

A

Arthritis and psoriasis or Arthritis and at least two of the following: dactylitis, nail findings (pitting, oil spots, or onycholysis), family history of psoriasis in at least one 1st degree relative.

41
Q

In psoriatic JIA, what is the temporal relationship between the development of arthritis and the development of psoriatic skin changes?

A

Arthritis can precede the psoriasis by many years.

42
Q

Arthritis of which joints is unique to psoriatic JIA?

A

Distal interphalangeal joints (DIP)

43
Q

What is enthesitis-related arthropathies (ERA)?

A

ERA is enthesitis and arthritis, or either enthesitis or arthritis with ≥2 of the following: history or presence of sacroiliac joint tenderness and/or inflammatory lumbosacral pain; presence of HLA-B27 antigen; onset of arthritis in a male >6 years of age; acute symptomatic uveitis; a 1st degree relative with ankylosing spondylitis, ERA, sacroiliitis with IBD, or reactive arthritis.

44
Q

Describe the typical pattern of joint involvement in patients with enthesitis-related arthropathy.

A

Arthritis is typically peripheral, with lower limb involvement in an asymmetric manner.

45
Q

Define enthesitis.

A

Enthesitis is inflammation and tenderness of the enthesis, which is where a tendon or ligament inserts into bone.

46
Q

What ocular abnormality occurs in 5-10% of patients with enthesitis-related arthropathy?

A

Acute symptomatic iritis (this finding requires referral to ophthalmology).

47
Q

What (9) systemic illnesses should be on the differential for a patient who presents with fever, rash, lymphadenopathy, joint symptoms, and lab abnormalities?

A

sJIA, malignancy, bone/joint infection, SLE, acute rheumatic fever, serum sickness, Kawasaki disease, sarcoidosis, and Sjogren syndrome.

48
Q

What is the typical treatment for JIA?

A

NSAIDs + DMARDs (disease-modifying anti-rheumatic drugs). Intraarticular steroid injections can be useful in patients with only a few affected joints.

49
Q

What complication of oJIA can be decreased with use of intraarticular joint injections?

A

Patients with oJIA can have leg length discrepancies if the knees are involved. Joint injections decrease this occurrence.

50
Q

Which DMARD is most commonly used in the treatment of JIA?

A

Methotrexate (1x/week either as pills or sQ injections.

51
Q

What supplement should be given alongside methotrexate to decrease side effects in patients being treated for JIA?

A

Patients receiving methotrexate should be on folic acid supplementation to decrease side effects (liver abnormalities, oral ulcers, and cytopenias).

52
Q

What DMARD is useful in the treatment of enthesitis-related arthropathies?

A

Sulfasalazine

53
Q

Which biologics can be used for JIA?

A

TNF inhibitors (etanercept, adalinumab, infliximab), T-cell modulators (abatacept), IL-6 receptor blockers (tocilizumab), and IL-1 blockers (anakinra, canakinumab, rilonacept).

54
Q

Name 3 TNF inhibitors.

A

Etanercept, adalimumab, and infliximab.

55
Q

What is the mechanism of action of etanercept (Enbrel)?

A

Tumor necrosis factor inhibitor (TNFi)

56
Q

What is the mechanism of action of adalimumab (Humira)?

A

Tumor necrosis factor inhibitor (TNFi)

57
Q

What is the mechanism of action of infliximab (Remicade)?

A

Tumor necrosis factor inhibitor (TNFi)

58
Q

Name a T-cell modulating biologic.

A

Abatacept

59
Q

What is the mechanism of action of abatacept (Orencia)?

A

T-cell modulator

60
Q

Name an IL-6 receptor blocker.

A

Tocilizumab

61
Q

What is the mechanism of action of Tocilizumab?

A

IL-6 receptor blocker

62
Q

Name 3 IL-1 blockers.

A

Anakinra, Canakinumab, and Rilonacept

63
Q

What is the mechanism of action of anakinra?

A

IL-1 blocker

64
Q

What is the mechanism of action of canakinumab (Ilaris)?

A

IL-1 blocker

65
Q

What is the mechanism of action of rilonacept?

A

IL-1 blocker

66
Q

Which category of JIA responds best to TNF inhibitors?

A

Polyarticular JIA

67
Q

Which category of JIA responds best to IL-6 and IL-1 inhibitors?

A

Systemic JIA

68
Q

What biologic agent is useful in the treatment of refractory cases of poJIA?

A

Abatacept (Orencia)

69
Q

What diseases must you screen for prior to starting a patient on biologic therapy?

A

Latent TB, Hepatitis B, and Hepatitis C.

70
Q

What vaccine restrictions apply to patients on biologic therapy?

A

Patients on biologic therapy should not receive live attenuated vaccines.

71
Q

In what situations should systemic corticosteroids be used in the treatment of JIA?

A

Generally, do not use corticosteroids except for very severe disease, flares of the disease, or systemic manifestations.

72
Q

What are the (10) side effects to watch for in patients receiving long-term systemic corticosteroid therapy?

A

Diabetes, stunted growth, osteoporosis, infection, cataracts, glaucoma, HTN, hyperlipidemia, mood changes, and adrenal insufficiency.

PBR: Rheumatology (13 decks)

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