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Pediatric Rheumatology Board Exam > Juvenile Arthritis 18% > Flashcards

Juvenile Arthritis 18% Flashcards

1
Q

What is JRA?

A

ACR criteria for arthritis from 1970s-95 when replaced with JIA. Age of onset <16 years; Arthritis in 1 or more joints for >6 weeks; Onset type defined by type of disease in first 6 months: Polyarthritis (>/=5 joints), Oligoarthritis, Systemic onset. Exclusion of other forms of juvenile arthritis.

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2
Q

What is JCA (juvenile chronic arthritis)?

A

EULAR classification in 1970s-95 when replaced with JIA. Differed from ACR criteria in 3 ways: 1. arthritis must be present for at least 3 months. 2. juvenile ankylosing spondylitis, psoriatic arthropathy and arthropathies associated with IBD are separate categories. 3. Term JRA was applied only to children with arthritis and RF positivity.

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3
Q

What is JIA?

A

ILAR’s classification criteria for idiopathic arthritides of childhood developed in 1995. Developed with aim of achieving homogeneity within disease categories to better facilitate clinical and basic research and eliminate inconsistencies from ACR and EULAR classifications. Used for children <16 and based on expression of disease in first 6 months. Includes 6 subtypes + undifferentiated for those that do not meet criteria for other category or meet criteria for more than 1 category.

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4
Q

What are the exclusion criteria for the oligo JIA ILAR criteria?

A

Psoriasis, history of psoriasis in patient/1st degree relative Arthritis in HLA B27+ male > 6 yo AS, ERA, sacroiliitis with IBD, Reiter syndrome, acute anterior uveitis or a history of one of these in a 1st degree relative RF IgM positive x 2, 3 months apart Systemic JIA Exclusions are part of ILAR criteria but NOT EULAR or ACR criteria

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5
Q

What is the DDx for acute monoarthritis? (10 items)

A

Oligo JIA ERA PsA Septic arthritis Reactive arthritis Leukemia Neuroblastoma Hemophilia Trauma FMF

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6
Q

What is the DDx for chronic mono arthritis? (9 items)

A

Oligo JIA ERA PsA Villonodular synovitis Sarcoidosis, Blau syndrome Tuberculosis Hemophilia Pseudoarthritis (e.g. hemangioma, synovial chondromatosis, lipoma arborescens) Some autoinflammatory syndromes (e.g. MVK deficiency, CINCAS, NOMID)

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7
Q

What is the frequency of asymptomatic uveitis in oligoarticular JIA? What if the ANA is positive?

A

Frequency of asymptomatic uveitis: 20% ANA positive: 30%

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8
Q

What is the most common age of onset for oligoarticular JIA?

A

1-3 years old

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9
Q

What are the predictors of poor prognosis in oligoarticular JIA?

A

Arthritis of hip, cervical spine, wrist, or ankle Marked or prolonged elevation of ESR or CRP Radiographic joint damage

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10
Q

What are the predictors of disease extension in oligoarticular JIA?

A

Ankle or wrist disease Symmetrical joint involvement Elevated ESR

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11
Q

When would a child need a shoe lift when they have a leg length discrepancy?

A

Leg length inequalities: need shoe lift if >2 cm

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12
Q

What is the sex ratio in oligoarticular JIA? What if the patient also has uveitis?

A

Sex ratio: 3 female : 1 male If oligoarticular JIA + uveitis: 5-6.6 female : 1 male

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13
Q

Serum prolactin levels are ___ in kids with JIA and associated with ___ ANA. Prolactin concentration correlated with levels of ___ and a chronic course of disease.

A

Serum prolactin levels are INCREASED in kids with JIA and associated with POSITIVE ANA. Prolactin concentration correlated with levels of IL-6 and a chronic course of disease.

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14
Q

Younger patients with psoriatic arthritis may look like a patient with oligoarticular JIA with 3 key differences:

A

Dactylitis Wrist and small joints of hands and feet involvement Progression to poly disease in absence of effective therapy

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15
Q

Nail pitting, dactylitis involvement, asymmetric joint involvement (small and large) are more likely in what type of JIA?

A

Psoriatic arthritis

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16
Q

“Pencil in cup” deformity on XR is a classic finding for ___.

A

Dactylitis of psoriatic arthritis

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17
Q

In psoriatic arthritis, ___ can be seen on XR in the affected digit with dactylitis.

A

Periosteal reaction

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18
Q

Differential diagnosis for dactylitis includes the following 3 conditions:

A

TB osteomyelitis Sarcoidosis Sickle cell disease

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19
Q

Nail changes occurs in ___% of patients with juvenile psoriatic arthritis.

A

50-80% Uniformly seen in patients with DIP involvement. Nail pits, onycholysis, horizontal ridging, discoloration

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20
Q

Psoriasis can be exacerbated by the following 2 drug classes:

A

Anti-malarial drugs (hydroxychlorquine) Paradoxical effect of anti-TNF drugs

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21
Q

What is the age of onset for juvenile psoriatic arthritis?

A

Bimodal 1. Preschool age (mainly female) 2. Middle to late childhood Uncommon before 12 months of age

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22
Q

Patients with psoriasis are at increased risk of having ___, which may lead to fatty liver disease. Methotrexate may exacerbate a transaminitis in these patients.

A

Metabolic syndrome

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23
Q

What MHC I allele is associated with adult psoriatic arthritis and possibly juvenile psoriatic arthritis?

A

HLA-Cw6

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24
Q

SNPs (single nucleotide polymorphisms) near ___ are seen in juvenile psoriatic arthritis.

A

IL-23R IL-23 is involved in differentiation of Th17 cells, which is increased in frequency in patients with PsA. IL-23 responsive cells are also found in theses and the aortic root. IL-23 over expression in mice can induce SpA with inflammation at both entheses and aortic root.

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25
Q

What is the most common type of uveitis associated with JIA?

A

Bilateral anterior uveitis, usually asymptomatic and insidious in onset

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26
Q

What percent of patients present with uveitis before onset of arthritis?

A

10%

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27
Q

If patients with oligoarticular JIA develop uveitis, 90% of patients will develop uveitis within the first ___ years.

A

4 years

28
Q

What is the differential diagnosis for uveitis in rheumatology patients?

A

JIA ERA - acute symptomatic uveitis Blau syndrome - chronic panuveitis, can lead to vision loss Sarcoidosis Behcet syndrome - panuveitis, can lead to vision loss Kawasaki disease - uveitis with conjunctivitis with limbic sparing Takayasu arteritis - vasculitis ANCA-associated vasculitis - vasculitis or episcleritis HSP, rheumatic fever (very rare) Crohn disease Reactive arthritis Tubular interstitial nephritis and uveitis (TINU) JDM - rapidly progressive vasculitis SLE - retinitis, vasculitis

29
Q

How frequently should one perform uveitis screening in a 4-year-old patient with ANA positive oligoarticular JIA?

A

Every 3 months from diagnosis through the next 4 years. Every 6 months if no uveitis and > 5 years

30
Q

What are the 2 most common complications of uveitis?

A

Synechiae Band keratopathy - deposition of Ca in the corneal epithelium

31
Q

How would you approach treatment of chronic uveitis?

A
  1. Topical steroid +/- mydriatics (to prevent synechiae) prescribed by ophthalmology 2. If active uveitis at 3 months, add methotrexate 3. If active uveitis despite adequate trial of methotrexate, add adalimumab/infliximab 4. Consider mycophenolate mofetil for refractory chronic uveitis
32
Q

What is a typical work-up for a child with uveitis?

A

ANA Urinalysis Consider Lyme, Bartonella, Toxocara, toxoplasmosis serologies If sudden onset anterior uveitis: Urine beta-2 micro globulin, CBC, ESR, BUN, Cr If acute symptomatic uveitis: HLA-B27 If granulomatous uveitis: TB screening CXR ACE, lysozyme, serum Ca/Cr urine Ca/Cr If posterior uveitis: Toxoplasmosis serologies CMV serologies and PCR

33
Q

Uveitis disease activity parallels arthritis disease activity in JIA. True or False?

A

False Uveitis disease activity can be independent from JIA disease activity. A flare can occur despite JIA disease being quiescent for years.

34
Q

JIA-associated uveitis accounts for ___% of all uveitis cases.

A

20-40%

35
Q

What is the frequency of uveitis in oligoarticular JIA?

A

15-20%

36
Q

What is the frequency of uveitis in extended oligoarticular JIA?

A

30%

37
Q

What are the frequencies of ANA and rheumatoid factor in patients with JIA-associated uveitis?

A

ANA: 80% Rheumatoid factor < 1%

38
Q

What is Brown syndrome?

A

Superior oblique tenosynovitis Presents with pain with upward gaze with or without diplopia

39
Q

What gene is anterior uveitis associated with?

A

MHC I HLA-B27

40
Q

What are the 2 patterns of joint disease in IBD? Which is more common?

A
  1. Peripheral polyarthritis - more common, most frequently wrists, knees ankles 2. Sacroiliitis and anxial skeleton arthritis
41
Q

IBD with sacroiliitis has a high frequency of ___ association. IBD with peripheral poly arthritis does not have the same association.

A

IBD with sacroiliitis has a high frequency of HLA-B27 association. IBD with peripheral poly arthritis does not have the same association.

42
Q

NOD2 is associated with: 1. 2. 3:

A
  1. Blau syndrome
  2. Yao syndrome
  3. Crohn disease (certain NOD2/Card15 variants)
43
Q

What are 3 rheumatologic conditions are associated with IBD?

A

Sacroiliitis HLA-B27+ spondyloarthritis CRMO

44
Q

What is the typical presentation of IBD-associated uveitis?

A

Bilateral chronic posterior uveitis with insidious onset Also increased risk of episcleritis, scleritis, and glaucoma

45
Q

What is the DDx for IBD-associated arthropathies?

A

CRMO Infantile IBD Other autoinflammatory diseases ERA/JAS, other JIA w/o IBD Behcet Reactive arthritis Infectious colitis with associated reactive arthritis Arthritis with NSAID enteritis PAPA syndrome (orofacial granulomatosis) or cheilitis granulomatosa IBS Abdominal migraines and amplified pain SLE Kawasaki disease

46
Q

What is the risk of a HLA-B27 heterozygous parent with AS to have a male child with AS?

What if the child is HLA-B27 positive?

What if the child is HLA-B27 negative?

A

The general risk for a B27-heterozygous parent with AS to have a male child with disease is 5-10%, if the child is also B27+ it is 20%, if the child is B27 negative it is close to 0.

47
Q

What does serum neopterin used for?

A

Marker of interferon-activated monocytes and macrophages (for example, in MAS, SLE, JDM but not always)

48
Q

What would be considered appropriate first-line treatment in a patient with inflammatory bowel disease and knee arthritis?

A

Sulfasalazine

Mesalamine

Prednisone

49
Q

A 4-year-old male presents with significantly swollen, boggy knee with swelling that extends halfway up the thigh. Indolent onset. Associated with poor weight gain for the last several months. XR reveals erosive arthritis. What infectious etiology is on the differential?

A

Arthritis due to Mycobacteria tuberculosis

Indolent, boggy monoarthritis of knee or wrist with progression to erosive arthritis in a patient with risk factors for TB exposure

50
Q

How likely is a child to develop axial arthritis?

In PsA?

In IBD?

In reactive arthritis?

In juvenile AS?

A

In JPSa, axial arthritis develops in about 10-30%. IBD and ReA are both about 25-50%, and JAS greater than 75%.

51
Q

What is the most common extraintestinal manifestation of IBD?

A

Arthritis

  • peripheral polyarthritis
  • sacroiliitis and axial arthritis is less common
52
Q

___ is at least 30x more common in patients with IBD compared to the general population.

A

Sacroiliitis

53
Q

___ % of adult patients with ankylosing spondylitis also have chronic IBD.

A

5-10%

54
Q

What genetic mutations are associated with infantile-onset IBD, which is considered an autoinflammatory syndrome?

A

Genetic mutation in IL-10 or IL-10R

55
Q

What is the differential diagnosis of erythema nodosum?

A
  1. Idiopathic ~50%
  2. Infection: Strep, TB
  3. Drug-induced
  4. IBD
  5. Sarcoidosis
  6. Reactive arthritis
  7. Cutaneous PAN - usually periarticular and larger in diameter
56
Q

How does IBD-associated uveitis typically present?

A

Commonly insidious onset of chronic, bilateral posterior uveitis

Most patients with IBD-associated uveitis have Crohn disease and are female

57
Q

What (auto)antibodies may be positive in IBD-associated arthropathy?

A
  1. Positive p-ANCA in 73% of ulcerative colitis patients
  2. Positive anti-Saccharomyces cervisiae Ab
58
Q

What types of JIA are associated with increased risk of developing IBD?

A
  1. ERA
  2. PsA
  3. Extended oligoarticular JIA
59
Q

What manifestation of IBD-associated arthropathy is more likely to persist and progress without remission, to be independent of gut activity, and be unaffected by colectomy?

A

HLA-B27 positive spondyloarthropathy

Sulfasalazine is the initial drug of choice

60
Q

In a teen boy with inflammatory back pain, morning stiffness that improvemes with activity and bilateral sacroiliac tenderness on examination

Of the following, the MOST useful next step to help with diagnostic evaluation of this patient includes

A. imaging (radiography or MRI) of lumbosacral spine

B. imaging (radiography or MRI) of the pelvis to include sacroiliac joints

C. measuring ESR and CRP

D. obtaining HLA B27 test

A

B. imaging (radiography or MRI) of the pelvis to include sacroiliac joints

Hence, the next best step to make this diagnosis would include imaging of the pelvic region with inclusion of the sacroiliac joints by radiography (30° cephalad pelvic radiographs are best) or MRI.

The erythrocyte sedimentation rate and C-reactive protein level are likely elevated in this patient and will not assist in the diagnosis of sacroiliitis. Similarly, HLA-B27 testing is likely to have a positive result in this patient, and its presence or absence will not assist with the diagnosis of sacroiliitis. Radiography or MRI of the back will miss the pelvic region, which is the most crucial region to examine in this adolescent.

61
Q

What is the most common cause of posterior uveitis?

A

Infectious causes

In posterior uveitis, toxoplasmosis and other infectious etiologies (tuberculosis, syphilis, herpes simplex virus, varicella-zoster virus, cytomegalovirus) are far more common than idiopathic or other associated systemic diseases.

62
Q

What are the most common causes of panuveitis?

A
  1. Most common cause: idiopathic

followed by Vogt-Koyanagi-Harada disease, Behçet disease, and sarcoidosis.

63
Q

To test for Anti-cyclic citrullinated peptide antibodies (anti-CCP), which of the following should you order?

anti-CCP IgM

OR

anti-CCP IgG

A

anti-CCP IgG

the appropriate anti-CCP antibody is IgG

64
Q

To test for rheumatoid factor, what should you choose?

IgA RF

or

IgM RF

or

IgG RF

A

IgM RF

the IgM RF is the appropriate RF to be tested and is found in high titer in RF-positive polyarticular JIA.

65
Q

What HLA is associated with an increased risk of rheumatoid factor–positive polyarticular juvenile idiopathic arthritis and adult rheumatoid arthritis?

A

HLA-DRB1

The shared epitope is a specific sequence on the HLA-DRB1 antigen that is associated with an increased risk of rheumatoid factor–positive polyarticular juvenile idiopathic arthritis and adult rheumatoid arthritis.

66
Q

What is the incidence of uveitis in patients with psoriatic arthritis?

A

10% to 15%

The incidence of uveitis in juvenile psoriatic arthritis is 10% to 15% and monitoring should be the same schedule as other forms of juvenile idiopathic arthritis.

Both symptomatic and asymptomatic uveitis can occur. Acute (symptomatic) uveitis is more typically seen with the axial pattern of disease and is associated with HLA-B27 positivity. Antinuclear antibody positivity confers a higher risk of chronic (asymptomatic) uveitis and patients should be screened with ophthalmologic examinations at the same frequency of JIA class (oligo or polyarticular presentation).

67
Q

What cytokines/pathways are implicated in erosions of JIA?

A

Inflammatory cytokines, such as tumor necrosis factor α (TNF-α), IL-1, IL-6, and IL-17, can induce overexpression of receptor activator of nuclear factor kB ligand (RANKL) and decreased levels of osteoprotegerin. This can lead to an imbalance in the RANKL/osteoprotegerin ratio leading to increased osteoclastogenesis (ie, osteoclast differentiation).

Pediatric Rheumatology Board Exam (21 decks)

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