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Pédiatrie > 24 - Uveitis > Flashcards

24 - Uveitis Flashcards

1
Q

(Pediatric uveitis)

Prévalence des pediatric uveitis

A

The prevalence of pediatric uveitis varies among studies, with pediatric cases accounting for 2%–14% of all uveitis cases.

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2
Q

(Pediatric uveitis)

Ratio H : F des pediatric uveitis?

A

The distribution between the sexes is similar to that of uveitis in adults, showing a slight female preponderance.

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3
Q

(Pediatric uveitis)

Mean age at diagnosis des pediatric uveitis?

A

The mean age at diagnosis is 8–9 years.

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4
Q

(Pediatric uveitis)

% des pediatric uveitis avec une atteinte bilatérale?

A

75% of patients have bilateral disease.

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5
Q

(Pediatric uveitis)

% des pediatric uveitis qui sont dites « White »

A

In the United States, approximately 62% of pediatric patients with uveitis are White.

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6
Q

(Pediatric uveitis)

2 major etiologies of uveitis in children

A

The 2 major etiologies of uveitis in children are
* Idiopathic (25%–54% of cases)
* Juvenile idiopathic arthritis (15%–47% of cases)

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7
Q

(Pediatric uveitis)

La majorité des uvéites pédiatriques sont-elles héritées ou non héritées?

A

Most types of uveitis are not inherited

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8
Q

(Pediatric uveitis)

% des uvéites pédiatriques qui sont antérieures

A

Results of a recent claims-based study of uveitis in the United States showed that anterior uveitis accounted for 75% of pediatric uveitis cases, with posterior uveitis and panuveitis accounting for the remaining 25%.

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9
Q

(Pediatric uveitis)

% des uvéites pédiatriques qui sont postérieures et/ou panuvéites

A

Results of a recent claims-based study of uveitis in the United States showed that anterior uveitis accounted for 75% of pediatric uveitis cases, with posterior uveitis and panuveitis accounting for the remaining 25%.

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10
Q

(Pediatric uveitis)

Ddx des uvéites pédiatriques

A
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11
Q

(Anterior uveitis)

Que signifie uvéite antérieure?

A
  • Anterior uveitis primarily involves inflammation of the iris and ciliary body.
  • When the anterior chamber is the primary site where inflammation is observed, the term iritis may be used for this inflammation.
  • When inflammation is also observed in the anterior vitreous, the term iridocyclitis may be used.
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12
Q

(Juvenile Idiopathic Arthritis)

V ou F : Juvenile idiopathic arthritis (JIA) is the most common identifiable etiology of childhood anterior uveitis

A

Vrai.
Juvenile idiopathic arthritis (JIA) (formerly, juvenile rheumatoid arthritis) is the most common identifiable etiology of childhood anterior uveitis. (Après idiopathique)

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13
Q

(Juvenile Idiopathic Arthritis)

Définir JIA

A

JIA is defined as
* Arthritis of at least 6 weeks’ duration
* Without an identifiable cause
* In children younger than 16 years

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14
Q

(Juvenile Idiopathic Arthritis)

Sous-types de JIA

A
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15
Q

(Juvenile Idiopathic Arthritis)

Prévalence des uvéites en générale dans le JIA

A

Overall, the prevalence of uveitis in JIA varies from 2% to 34%.

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16
Q

(Juvenile Idiopathic Arthritis)

Subtypes of JIA that are particularly associated with uveitis

A

The subtypes of JIA that are particularly associated with uveitis are
* Oligoarthritis (formerly pauciarticular arthritis)
* Polyarticular arthritis
* Psoriatic arthritis
* Enthesitis related arthritis

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17
Q

Concernant les uvéites dans le JIA :
* FR positif ou négatif?
* Atteinte systémique?

A

Uveitis almost never occurs in children with systemic arthritis and is very rare in those with rheumatoid factor–positive polyarticular subtype.

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18
Q

Présentation clinique de l’uvéite associated with oligoarthritic JIA and polyarticular JIA?

A
  • In contrast to most forms of anterior uveitis, the uveitis associated with oligoarthritic JIA and polyarticular JIA is initially asymptomatic.
  • It has been described as “white iritis” because of the absence of a red eye.
  • Screening for uveitis among children with JIA is therefore of great importance.
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19
Q

Most frequent type of JIA in children in North America and Europe

A

Oligoarthritis is the most frequent type of JIA in children in North America and Europe.

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20
Q

Définition Oligoarthritic JIA

A

Oligoarthritic JIA affects 4 or fewer joints during the first 6 months of the disease.

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21
Q

(Oligoarthritic JIA)

Prévalence H versus F pour l’Oligoarthritic JIA

A

It occurs predominantly in young girls.

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22
Q

(Oligoarthritic JIA)

% de patient avec Oligoarthritic JIA développant une uvéite?

A

Anterior uveitis is most likely to occur with this type of arthritis, developing in 10%–30% of patients.

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23
Q

(Oligoarthritic JIA)

Concernant les marqueurs de laboratoire suivants, positif(s) ou négatif(s) dans l’Oligoarthritic JIA?
* Antinuclear antibodies (ANA)
* Rheumatoid factor

A
  • Laboratory markers include a high prevalence of antinuclear antibodies (ANA).
  • Rheumatoid factor is almost always absent.
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24
Q

(Polyarticular JIA)

Définition de Polyarticular JIA

A

Children with polyarticular JIA show involvement of more than 4 inflamed joints during the first 6 months of the disease.

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25
Q

(Polyarticular JIA)

Mean age at onset of Polyarticular JIA is higher or lower than oligo ?

A

This disease is more common in girls, and the mean age at onset is higher compared with oligoarthritic JIA.

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26
Q

(Polyarticular JIA)

Prévalence H versus F pour la Polyarticular JIA

A

This disease is more common in girls, and the mean age at onset is higher compared with oligoarthritic JIA.

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27
Q

(Polyarticular JIA)

% de patient avec Polyarticular JIA développant une uvéite?

A

Uveitis occurs in approximately 10% of these children.

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28
Q

(Polyarticular JIA)

Concernant les marqueurs de laboratoire suivants, positif(s) ou négatif(s) dans le Polyarticular JIA?
* Antinuclear antibodies (ANA)
* Rheumatoid factor

A

Affected patients may have a positive ANA test result, which is associated with an increased risk of uveitis.

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29
Q

(Juvenile Idiopathic Arthritis)

Association HLA et JIA?

A

Human leukocyte antigen (HLA) associations have not been consistently documented.

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30
Q

(Juvenile Idiopathic Arthritis)

Pathogénèse de l’uvéite associé à JIA

A

The pathogenesis of the anterior uveitis associated with JIA is unknown, but it is likely to have an immunologic basis.

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31
Q

(Juvenile Idiopathic Arthritis)

Quand/à quel moment le risque de développer une uvéite est-il plus élevé?

A
  • The risk for development of uveitis is highest during the first 4 years after diagnosis of JIA.
  • Among patients with JIA, 90% of uveitis cases develop within 4 years of onset of arthritis.
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32
Q

(Juvenile Idiopathic Arthritis)

Dans quelles situations le pronostic d’uvéite avec JIA est-il moins bon?

A
  • Occasionally, uveitis is diagnosed before or at the onset of joint symptoms; in these cases, unfortunately, the prognosis is often poorer because the initially asymptomatic nature of the ocular inflammation often delays diagnosis and thus treatment.
  • A shorter interval between the onset of arthritis and uveitis is also associated with a more aggressive course.
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33
Q

(Juvenile Idiopathic Arthritis)

Décrire les ¢ dans la CA et les KPS dans la JIA-associated uveitis.

A
  • JIA-associated uveitis is usually bilateral
  • Nongranulomatous with fine to medium sized keratic precipitates
  • A minority of children, especially African Americans, may have granulomatous precipitates.
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34
Q

(Juvenile Idiopathic Arthritis)

Uvéite unilatérale ou bilatérale dans la JIA-associated uveitis

A

Bilatérale

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35
Q

(Juvenile Idiopathic Arthritis)

Complications d’une inflammation chronique avec une JIA-associated uveitis.

A

Chronic inflammation may produce
* Band keratopathy (Fig 24-1)
* Posterior synechiae
* Ciliary membrane formation
* Hypotony
* Cataract
* Glaucoma
* Phthisis

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36
Q

(Juvenile Idiopathic Arthritis)

Vitréite ou oedème maculaire associés à la JIA?

A

Vitritis and macular edema occur infrequently.

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37
Q

(Juvenile Idiopathic Arthritis)

Concernant le JIA-associated uveitis :
% de patient avec visual impairment?
% de patients avec blindness?

A

However, visual impairment has been reported in up to 40% of children with JIA-associated uveitis, and blindness may occur in as many as 10% of affected eyes.

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38
Q

(Juvenile Idiopathic Arthritis)

4 factors that are associated with an increased risk of uveitis

A

Screening guidelines continue to undergo revision but are based on 4 factors that are associated with an increased risk of uveitis:
* Category of arthritis
* Age at onset of arthritis
* Presence of ANA positivity
* Duration of the disease

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39
Q

(Juvenile Idiopathic Arthritis)

Frequency of Eye Examination in Patients with Polyarticular and Oligoarthritis? (Bref le tableau et le truc à Dr Kirouac)

A
  • The eye examination schedule for oligoarthritis and polyarticular JIA, as recommended by the American Academy of Pediatrics.
  • After 4 years, the eye examinations become less frequent.
  • Although female sex is associated with a higher incidence of uveitis, this factor is not incorporated in the guidelines.
  • Initial ophthalmologic examination should occur within 6 weeks of diagnosis.

Truc Dr Kirouac :

Every point means higher disease activity
ANA + = 1 pt
Age at onset < 6 yo = 1 pt
Duration of disease < 4 y = 2 pts, 4-7 y = 1 pt

4 pts = q3 months
3 pts = q6 months
1-2 pts = qyear

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40
Q

(Juvenile Spondyloarthropathies)

% de patients ayant une uvéite dans le Juvenile spondyloarthropathies

A

Juvenile spondyloarthropathies are a group of HLAB27– associated disorders and are associated with uveitis in 25% of affected individuals.

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41
Q

(Juvenile spondyloarthropathies)

Assocition HLA et Juvenile spondyloarthropathies?

A

Juvenile spondyloarthropathies are a group of HLAB27– associated disorders

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42
Q

(Juvenile spondyloarthropathies)

Prévalence H versus F? Onset?

A

Boys are more commonly affected than girls, and the disease onset is usually in early adolescence.

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43
Q

(Juvenile spondyloarthropathies)

Il existe des formes différenciées et non différenciées de la Juvenile spondyloarthropathies. Nommez des exemples de formes différenciées.

A

Differentiated types include
* enthesitis-related arthritis
* juvenile ankylosing spondylitis
* juvenile psoriatic arthritis
* reactive arthritis
* arthritis associated with inflammatory bowel disease (enteropathic)

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44
Q

(Juvenile spondyloarthropathies)

Additional Systemic Manifestations of Enthesitis-related arthritis

A

None

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45
Q

(Juvenile spondyloarthropathies)

Additional Systemic Manifestations of Juvenile Psoriatic arthritis

A
  • Nail changes : Pitting or onycholysis
  • Dactylitis
  • Psoriasis
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46
Q

(Juvenile spondyloarthropathies)

Additional Systemic Manifestations of Juvenile ankylosing spondylitis

A
  • Involves the spine
  • Sacroiliitis (which may be subclinical)
  • Cardiac disease rare in children
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47
Q

(Juvenile spondyloarthropathies)

Additional Systemic Manifestations of Enteropathic arthritis

A
  • Crohn disease
  • Ulcerative colitis
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48
Q

(Juvenile spondyloarthropathies)

Additional Systemic Manifestations of Reactive arthritis

A
  • Follow an infection not involving the joints
  • Urethritis
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49
Q

(Juvenile spondyloarthropathies)

Uveitis Prevalence and Charasteristics of Enthesitis-related arthritis

A
  • Occurs in 20% of affected children
  • Acute, symptomatic
  • Unilateral, but both eyes may be affected at different times
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50
Q

(Juvenile spondyloarthropathies)

Uveitis Prevalence and Charasteristics of Juvenile Psoriasic arthritis

A
  • Occurs in 10% of affected children
  • Insidious and Chronic anterior uveitis
  • Usually bilateral
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51
Q

(Juvenile spondyloarthropathies)

Uveitis Prevalence and Charasteristics of Juvenile ankylosing spondylitis

A
  • Occurs in 20-30% of those affected
  • Acute, symptomatic
  • Unilateral, but both eyes may be affected at different times
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52
Q

(Juvenile spondyloarthropathies)

Uveitis Prevalence of Enteropathic arthritis

A

Occurs in 5% of those affected

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53
Q

(Juvenile spondyloarthropathies)

Uveitis Prevalence and Charasteristics of Reactive arthritis

A
  • Occurs in 12% of those affected
  • Acute, symptomatic
  • Unilateral and recurrent anterior uveitis
  • Conjunctivitis may also be present
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54
Q

(Juvenile spondyloarthropathies)

Unifying feature of the differentiated forms of Juvenile spondyloarthropathies

A

A unifying feature of the differentiated forms is enthesitis, an inflammation of the sites where the ligaments, tendons, and joint capsules attach to bone

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55
Q

(Juvenile spondyloarthropathies)

Enthesitis les plus fréquemment atteintes?

A

Enthesitis most commonly affects the insertions of the
* Patellar ligament at the inferior patella
* Plantar fascia at the calcaneus
* The Achilles tendon.

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56
Q

(Juvenile spondyloarthropathies)

V ou F : Asymmetric lower extremity oligoarthritis with involvement of the hips and midfoot is highly suggestive of the disease.

A

Vrai. Asymmetric lower extremity oligoarthritis with involvement of the hips and midfoot is highly suggestive of the disease.

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57
Q

(Juvenile spondyloarthropathies)

Présentation clinique d’un uvéite à Juvenile spondyloarthropathies

A

The anterior uveitis associated with juvenile spondyloarthropathies usually has an acute onset with photophobia, pain, and a red eye.

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58
Q

(Tubulointerstitial Nephritis and Uveitis Syndrome)

Définir Tubulointerstitial Nephritis and Uveitis Syndrome

A

Tubulointerstitial nephritis and uveitis (TINU) syndrome is kidney disease associated with chronic or recurrent anterior uveitis in adolescents.

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59
Q

(Tubulointerstitial Nephritis and Uveitis Syndrome)

Median age at onset of Tubulointerstitial Nephritis and Uveitis Syndrome

A

The median age at onset is 15 years.

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60
Q

(Tubulointerstitial Nephritis and Uveitis Syndrome)

Présentation clinique du renal disease dans Tubulointerstitial Nephritis and Uveitis Syndrome (TINU)

A

The renal disease is characterized by low-grade fever, fatigue, pallor, and weight loss.

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61
Q

(Tubulointerstitial Nephritis and Uveitis Syndrome)

Que faut-il doser dans les urines?

A

Elevated levels of β2microglobulin are usually present in the urine.

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62
Q

(Tubulointerstitial Nephritis and Uveitis Syndrome)

Concernant l’uvéite dans le Tubulointerstitial Nephritis and Uveitis Syndrome :
* Unilatéral ou bilatéral?
* Survient avant, pendant ou après the renal disease?

A
  • The uveitis is usually bilateral
  • May occur before, simultaneously with, or after the renal disease
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63
Q

(Tubulointerstitial Nephritis and Uveitis Syndrome)

Pronostic du Tubulointerstitial Nephritis and Uveitis Syndrome

A

The prognosis is generally good, but long-term follow-up is required because the inflammation may recur

64
Q

(Kawasaki disease)

Définir le Kawasaki disease

A

Kawasaki disease, also known as mucocutaneous lymph node syndrome, is a primary vasculitis mediated by immunoglobulin (Ig) A affecting children younger than 5 years.

65
Q

(Kawasaki disease)

Présentation clinique du Kawasaki disease

A

Abnormalities include
* Fever
* Conjunctival injection
* Mucous membrane changes
* Extremity changes involving the skin
* Rash
* Cervical lymphadenopathy

66
Q

(Kawasaki disease)

Most significant complication of Kawasaki disease? Et % de patients atteints si non traitée?

A

The most significant complication of Kawasaki disease is coronary artery aneurysm, which occurs in 15%–25% of untreated children

67
Q

(Kawasaki disease)

Tx du Kawasaki disease

A

Treatment with aspirin and intravenous IgG reduces the incidence of coronary artery aneurysm formation

68
Q

(Kawasaki disease)

2 présentations oculaires associées au Kawasaki Disease

A

After conjunctivitis, a generally self-limited anterior uveitis during the acute phase of the illness is the second most common ocular finding, occurring in approximately 10% of cases.

69
Q

(Kawasaki disease)

% de patients avec Kawasaki disease ayant une uvéite

A

After conjunctivitis, a generally self-limited anterior uveitis during the acute phase of the illness is the second most common ocular finding, occurring in approximately 10% of cases.

70
Q

Ddx d’une uvéite antérieure

A
  • Juvenile Idiopathic Arthritis
  • Juvenile Spondyloarthropathies
  • Tubulointerstitial Nephritis and Uveitis Syndrome
  • Kawasaki Disease
  • Many cases of anterior uveitis are idiopathic or are caused by trauma.
  • Other causes include a variety of infectious and noninfectious diseases
71
Q

(Intermediate Uveitis)

Que signifie uvéite intermédiaire?

A
  • The term intermediate uveitis is an anatomically based description of the primary site of the ocular inflammation.
  • The inflammation is localized to the vitreous base overlying the ciliary body, pars plana, and peripheral retina, as well as the anterior vitreous
72
Q

(Intermediate Uveitis)

% des uvéites pédiatriques qui sont intermédiaires

A

Intermediate uveitis accounts for 12%–28% of uveitis cases in the pediatric age group.

73
Q

(Intermediate Uveitis)

Ddx d’une uvéite intermédiaire

A
  • In children, it may occur with various conditions, including sarcoidosis, syphilis, Lyme disease, multiple sclerosis, and tuberculosis.
  • Idiopathic disease, known as pars planitis, accounts for 85%–90% of cases.
74
Q

(Intermediate Uveitis)

% des uvéites intermédiaires qui sont idiopathiques

A

Idiopathic disease, known as pars planitis, accounts for 85%–90% of cases.

75
Q

(Intermediate Uveitis)

Comment appelle-t-on des uvéites intermédiaires qui sont idiopathiques?

A

Idiopathic disease, known as pars planitis, accounts for 85%–90% of cases.

76
Q

(Posterior Uveitis)

Que signifie uvéite postérieure?

A

Posterior uveitis is defined as intraocular inflammation primarily involving the choroid; often, there is also retinal involvement.

77
Q

(Posterior Uveitis)

Ddx de Posterior Uveitis

A

Toxoplasmosis
Toxocariasis

78
Q

(Posterior Uveitis)

Most common cause of posterior uveitis in children

A

Toxoplasmosis is the most common cause of posterior uveitis in children.

79
Q

(Toxocariasis Uveitis)

Quels sont les germes du Ocular toxocariasis?

A

Ocular toxocariasis is caused by the nematode larvae of a common intestinal parasite of
* Dogs (Toxocara canis)
* Cats (Toxocara cati)

80
Q

(Toxocariasis Uveitis)

Comment se contracte le Toxocariasis?

A

This disease, contracted through ingestion of ascarid ova in soil contaminated by dog or cat feces, primarily affects children.

81
Q

(Toxocariasis Uveitis)

Comment s’appelle l’infection systémique à Toxocariasis?

A

Larva migrans viscérale.
Visceral larva migrans (VLM) is an acute systemic infection produced by these organisms; it commonly occurs at approximately age 2 years.

82
Q

(Toxocariasis Uveitis)

At what age does Viscereal larva migrans commonly occurs?

A

Larva migrans viscérale.
Visceral larva migrans (VLM) is an acute systemic infection produced by these organisms; it commonly occurs at approximately age 2 years.

83
Q

(Toxocariasis Uveitis)

Présentation clinique du Larva migrans viscérale/Visceral larva migrans (VLM)?

A

If symptomatic, it is associated with fever, cough, rashes, malaise, and anorexia.

84
Q

(Toxocariasis Uveitis)

Abnormal laboratory testing in the visceral larva migrans (VLM)?

A

Laboratory testing reveals eosinophilia.

85
Q

(Toxocariasis Uveitis)

Est-ce que la visceral larva migrans (VLM) et l’ocular toxocariasis surviennent chez un même patient?

A

VLM and ocular toxocariasis, for unknown reasons, seldom occur in the same patient.
(Les deux surviennent rarement chez le même patient.)

86
Q

(Toxocariasis Uveitis)

Concernant l’uvéite à Toxocarose :
* Unilatérale ou bilatérale?
* Sx systémiques?
* Éosinophiles?

A

Ocular toxocariasis is usually unilateral and is not associated with systemic illness or an elevated eosinophil count.

87
Q

(Toxocariasis Uveitis)

Average age at onset of Toxocariasis Uveitis?

A

The average age at onset is 11.5 years.

88
Q

(Toxocariasis Uveitis)

3 major retinal forms of Toxocariasis Uveitis

A

The 3 major retinal forms of the disease include
* Posterior pole granuloma
* Peripheral granuloma with macular traction
* Endophthalmitis

89
Q

(Toxocariasis Uveitis)

External evidence of inflammation in Toxocariasis Uveitis

A
  • There is often little external evidence of inflammation.
  • Patients may present with leukocoria, strabismus, or decreased vision.
  • These are also common presenting signs and symptoms of retinoblastoma, which must be differentiated from ocular toxocariasis.
90
Q

(Toxocariasis Uveitis)

V ou F : un elevated Toxocara titers permet de r/o le rétinoblastome en Ddx de la Toxocariasis Uveitis

A

Faux.
Because elevated Toxocara titers may be found in a significant percentage of children, a positive result on tests such as the enzyme-linked immunosorbent assay does not rule out other possibilities, including retinoblastoma.

91
Q

(Toxocariasis Uveitis)

Tx de la Toxocariasis Uveitis

A

Treatment includes
* Observation of peripheral lesions, periocular
* Periocular or systemic steroids for posterior lesions and endophthalmitis
* Surgical intervention to address retinal traction, cataract, glaucoma, or cyclitic membranes
* Systemic anthelmintics are not useful in treating ocular toxocariasis because the organism may already be dead or its death can produce significant inflammation.

92
Q

(Sarcoidosis)

Mutation génétique dans la Sarcoïdose

A

In both diseases (Blau syndrome and Sarcoidosis), there are mutations in the nucleotide binding oligomerization domain containing 2 gene (NOD2) on chromosome 16; however, in Blau syndrome, other family members are affected.

93
Q

(Sarcoidosis)

2 distinct forms of Sarcoidosis in children

A
  1. Lung disease
  2. Triad of uveitis, granulomatous arthritis, and rash
94
Q

(Sarcoidosis)

Forme la plus fréquente de Sarcoïdose chez les jeunes enfants

A

Triad of uveitis, granulomatous arthritis, and rash

95
Q

(Sarcoidosis)

Tranche d’âge où la forme uveitis, granulomatous arthritis, and rash est la plus fréquente

A

In young patients (<5 years)

96
Q

(Sarcoidosis)

V ou F : Early-onset sarcoidosis is considered a pediatric granulomatous arthritis

A

Vrai.
Early-onset sarcoidosis is considered a pediatric granulomatous arthritis and is phenotypically and genetically similar to familial juvenile systemic granulomatosis.

97
Q

(Sarcoidosis)

Est-ce que le Early-onset sarcoidosis est phenotypically and genetically similar to familial juvenile systemic granulomatosis.

A

Oui.
Early-onset sarcoidosis is considered a pediatric granulomatous arthritis and is phenotypically and genetically similar to familial juvenile systemic granulomatosis.

98
Q

(Sarcoidosis)

Forme la plus fréquente de Sarcoïdose chez les older enfants

A

Older children (8–15 years) with sarcoidosis have the pulmonary abnormalities and lymph node findings more commonly associated with the adult form of the disease and are also at risk for uveitis.

99
Q

(Sarcoidosis)

Tranche d’âge où la forme pulmonary abnormalities and lymph node findings est la plus fréquente

A

Older children (8–15 years) with sarcoidosis have the pulmonary abnormalities and lymph node findings more commonly associated with the adult form of the disease and are also at risk for uveitis.

100
Q

(Sarcoidosis)

Forme la plus fréquente d’uvéite à Sarcoïdose
* Antérieure?
* Intermédiaire?
* Postérieure?
* Panuvéite?

A

Although anterior granulomatous uveitis is the most common manifestation of ocular sarcoidosis in children, this disease can produce a panuveitis.

101
Q

(Sarcoidosis)

Est-ce qu’une valeur élevée d’ACE chez un enfant avec uvéite confirme le dx de sarcoïdose ?

A

Nop
Serum angiotensin-converting enzyme levels, which may be abnormally elevated in patients with sarcoidosis, are normally higher in healthy children than in adults and thus can be misleading in diagnosis of this disease.

102
Q

(Familial Juvenile Systemic Granulomatosis)

Autre nom/Synonyme de Familial juvenile systemic granulomatosis

A

Familial juvenile systemic granulomatosis (Blau syndrome) is an autosomal dominant disease that may be identical to early-onset sarcoidosis; both are classified as pediatric granulomatous arthritides.

103
Q

(Familial Juvenile Systemic Granulomatosis)

Définir le Familial juvenile systemic granulomatosis

A

Familial juvenile systemic granulomatosis (Blau syndrome) is an autosomal dominant disease that may be identical to early-onset sarcoidosis; both are classified as pediatric granulomatous arthritis

104
Q

(Familial Juvenile Systemic Granulomatosis)

Mutation génétique dans le Familial Juvenile Systemic Granulomatosis

A

In both diseases (Blau syndrome and Sarcoidosis), there are mutations in the nucleotide binding oligomerization domain containing 2 gene (NOD2) on chromosome 16; however, in Blau syndrome, other family members are affected.

105
Q

(Familial Juvenile Systemic Granulomatosis)

Différence a/n de la mutation entre le Familial Juvenile Systemic Granulomatosis et la Sarcoïdose?

A

In both diseases, there are mutations in the nucleotide binding oligomerization domain containing 2 gene (NOD2) on chromosome 16; however, in Blau syndrome, other family members are affected.

106
Q

(Familial Juvenile Systemic Granulomatosis)

Présentation clinique du Familial Juvenile Systemic Granulomatosis

A

Both diseases (Sarcoidosis and Familial Juvenile Systemic Granulomatosis) present with granulomatous arthritis, uveitis, and rash during childhood, but pulmonary involvement and lymphadenopathy are absent in Blau syndrome.

107
Q

(Familial Juvenile Systemic Granulomatosis)

Forme la plus fréquente de l’uvéite à Familial Juvenile Systemic Granulomatosis
* Aiguë ou chronique?
* Antérieure, Intermédiaire, Postérieur ou Panuvéite?

A
  • Chronic panuveitis associated with multifocal choroiditis is the most common ocular presentation;
  • In some cases, the uveitis may be limited to the anterior segment and the disease misdiagnosed as JIA.
108
Q

(Familial Juvenile Systemic Granulomatosis)

Ocular complications of Familial Juvenile Systemic Granulomatosis

A

Ocular complications, including cataract, glaucoma, band keratopathy, and vision loss, are common.

109
Q

(Vogt- Koyanagi- Harada Syndrome)

Définir Vogt- Koyanagi- Harada Syndrome
* Unilatéral ou Bilatéral?
* Aigu ou chronique?
* Autres sx oculaires associés?
* Sx systémiques associés?

A

Vogt Koyanagi Harada syndrome is
* A chronic, progressive bilateral panuveitis
* Associated with exudative retinal detachments
* May be accompanied by meningeal irritation, auditory disturbances, and skin changes.

110
Q

(Vogt- Koyanagi- Harada Syndrome)

Pronostic visuel du Vogt- Koyanagi- Harada Syndrome chez les enfants

A

It is rare in children, but in those affected, the rate of ocular complications such as cataract and glaucoma is higher and the visual prognosis poorer compared with that for adults.

111
Q

(Masquerade Syndromes)

Définir Masquerade Syndromes

A

Various conditions can simulate pediatric uveitis.

112
Q

(Masquerade Syndromes)

Ddx des Masquerade Syndromes

A
113
Q

V ou F : Il est possible de ne pas investiguer un premier épisode isolé d’uvéite antérieure chez un enfant?

A

Some ophthalmologists defer the workup of isolated anterior uveitis unless it is recurrent or unresponsive to initial therapy.

114
Q

Laboratory Tests and Imaging for Anterior Uveitis

A

ANA
ACE
RXP
FTA-ABS
Test à la tuberculine
Sérologie Lyme
FSC
HLA-B27
RX sacro-iliaque
Colonoscopie PRN
Anticorps antineutrophile cytoplasmique
Créat
B2-microglobuline urinaire

115
Q

Laboratory Tests and Imaging for Intermediate Uveitis

A
116
Q

Laboratory Tests and Imaging for Posterior Uveitis and Panuveitis

A
117
Q

(Treatment of Pediatric Uveitis)

Goals of treatment of uveitis in children

A

The goals of treatment of uveitis in children are
* to eliminate the inflammation of the eye before ocular complications occur
* to monitor for ocular and systemic adverse effects of the treatment

118
Q

(Treatment of Pediatric Uveitis)

Qu’est-ce qui indique la persistance d’inflammation active dans les yeux : persistance ¢ dans CA ou flare?

A

It is important to note that although the presence of cells in the anterior chamber indicates active inflammation, flare (protein) may persist long after the inflammation has been successfully treated.

119
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Tx uvéite antérieure

A

Anterior segment inflammation is initially treated with topical corticosteroid and mydriatic/cycloplegic agents.

120
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Dans quelles situations les injections subTenon de corticos sont-elles indiquées?

A

Because topical corticosteroids do not penetrate well into the vitreous or posterior segment, subTenon injections of a corticosteroid may be useful in the treatment of intermediate or posterior uveitis .

121
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Utilisation de corticos PO à long terme?

A

Short courses of oral corticosteroids may be used, but long-term use is usually avoided because of the potential for significant ocular and systemic adverse effects.

122
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Indication pour utilisation de corticos en implant vitréal

A

Corticosteroid intravitreal implants containing either fluocinolone acetonide or dexamethasone have been used successfully to treat posterior uveitis in children.

123
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Most serious ocular complications of any corticosteroid therapy

A

Glaucoma and cataract formation are the most serious ocular complications of any corticosteroid therapy.

124
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

V ou F : les corticos plus puissants sont plus à risque d’augmenter la PIO.

A

Vrai.
In general, more potent topical corticosteroids are more likely to increase intraocular pressure.

Cataracts and glaucoma are reported adverse effects of intravitreal steroid implants.

125
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Jusqu’à combien de temps après une injection intraoculaire de corticos est-ce que la PIO peut demeurer augmentée?

A

Periocular injections of corticosteroids can elevate intraocular pressure for weeks to months after injection.

126
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

ES systémique des CS systémique long-term

A

Risks of long-term systemic corticosteroid use in children include
* growth retardation
* osteoporosis and bone fractures
* cushingoid appearance
* diabetes mellitus
* peptic ulcers
* myopathy
* hypertension
* altered mental status
* idiopathic intracranial hypertension
* increased risk of infection

Patients may also require increased doses of corticosteroids during times of stress to avoid an addisonian crisis.

127
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Does systemic immunosuppressive therapy is beneficial for treating both uveitis and arthritis?

A

Oui. Systemic immunosuppressive therapy is beneficial in treating both uveitis and arthritis.
* It can sometimes reduce or eliminate the need for steroids.
* The therapy should be undertaken in cooperation with a pediatric specialist familiar with the use of immunosuppressive and immunomodulatory medications.

128
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

V ou F : In patients with JIA, immunosuppressive drugs increase the risk of vision loss from uveitis.

A

Faux. In patients with JIA, immunosuppressive drugs reduce the risk of vision loss from uveitis.

129
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Immunosupresseur le plus souvent utilisé pour traiter l’uvéite pédiatrique.

A

Methotrexate is the most common antimetabolite used to treat arthritis and uveitis in children.

130
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Autres immunosupresseurs de la classe des anti-métabolites utilisés (autre que MTX) pour Tx de l’uvéite pédiatrique

A

Less commonly used antimetabolites include azathioprine and mycophenolate mofetil.

131
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Mécanisme d’action MTX, azathioprine et mycophenolate mofetil

A

These agents inhibit nucleic acid synthesis by a variety of mechanisms.

132
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

ES du MTX

A

Gastrointestinal disturbance is the most common adverse effect of oral methotrexate and can be alleviated by switching to subcutaneous injections.

Rare but serious adverse effects of MTX use :
* Hepatic toxicity
* Interstitial pneumonitis
* Cytopenia

133
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Supplémentation recommandée avec le MTX

A

Oral folic acid supplementation is often recommended for patients using methotrexate.

134
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Most common biologic agents used to treat pediatric uveitis

A

Tumor necrosis factor-alpha (TNFa) inhibitors
* Adalimumab
* Infliximab

135
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Autres agents biologiques pour Tx de l’uvéite for patients who experience treatment failures or have contraindications to adalimumab and infliximab

A

For patients who experience treatment failures or have contraindications to adalimumab and infliximab, other biologic agents such as tocilizumab (antibody to interleukin6), abatacept (CTLA4 fusion protein), rituximab (antibody to CD20), or golimumab (TNFa inhibitor) may be considered.

136
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Etanercept peut-il être utilisé comme première ligne de Tx pour patients with JIA-associated uveitis?

A

Etanercept should not be used as the primary immunomodulatory agent for patients with JIA-associated uveitis because of lower rates of treatment success compared to other TNFa inhibitors.

137
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Quel traitement faut-il cessé en période d’infx active?

A

TNFa inhibitors should not be used during periods of active infection.

138
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Parmi l’Infliximab et l’Adalimumab, lequel s’administre s/c et lequel s’administre via infusion?

A
  • Infliximab is given by infusion
  • Adalimumab is given subcutaneously
139
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Abatacept, golimumab, and tocilizumab s’administrent-ils par voie s/c ou par infusion?

A

Abatacept, golimumab, and tocilizumab may be given by either infusion or subcutaneous injection.

140
Q

(Treatment of Pediatric Uveitis : Management of Inflammation)

Association entre utilisation de TNFa blockers et l’augmentation du risque de malignité chez les enfants et adolescents?

A

There is concern that children and adolescents treated with TNFa blockers may be at increased risk for malignancies; however, a recent study showed that children with JIA are at increased risk for malignancies unrelated to the use of biologic drugs.

141
Q

(Tx of Pediatric Uveitis : Surgical Tx of Complications of Uveitis)

Complications de l’uvéite

A

Complications of uveitis include
* Band keratopathy
* Cataract
* Glaucoma

142
Q

(Tx of Pediatric Uveitis : Surgical Tx of Complications of Uveitis)

Tx de la kératopathie en bande

A
  • Band keratopathy can be treated by removal of corneal epithelium, followed by calcium chelation with ethylenediaminetetraacetic acid (EDTA)
  • Treatment may have to be repeated
  • Phototherapeutic keratectomy has also been used to treat band keratopathy
143
Q

(Tx of Pediatric Uveitis : Surgical Tx of Complications of Uveitis)

Complications de la cataract surgery for patients with uveitis

A

Cataract surgery for patients with uveitis can be complicated by hypotony, glaucoma, synechiae formation, cystoid macular edema, and retinal detachment.

In patients with JIA, combined lensectomy and vitrectomy seems to produce better results than cataract extraction alone.

Uveitis must be aggressively treated so that it is under control both before and after surgery.

Intraocular lens implantation is debated.

144
Q

(Tx of Pediatric Uveitis : Surgical Tx of Complications of Uveitis)

Techniques chirurgicales pour le Tx du glaucome chez enfant avec uvéite?

A
  • Glaucoma surgery may become necessary in children with uveitis.
  • Many techniques have been used, and long-term success rates vary.
  • Standard trabeculectomy is associated with a high rate of failure due to scarring.
  • Goniotomy or trabeculotomy is effective in many children and can be the initial surgery if the anterior chamber angle is visible.
  • Tube shunts can be used when goniotomy fails or if the angle is closed
145
Q

Composition de l’uvée

A
  • Iris
  • Corps ciliaire
  • Choroïde
146
Q

Nouvelle terminologie pour arthrite pauciarticulaire?

A

Oligoarthritis (formerly pauciarticular arthritis)

147
Q

Qu’est-ce que le Toxoplasmose gondii?

A

Parasite protozoaire intracellulaire

148
Q

Parasite impliqué dans l’infx à toxoplasmose?

A

Toxoplasmose gondii

149
Q

Incidence de la toxoplasmose congénitale?

A

The incidence of congenital toxoplasmosis ranges from 1 to 10 per 10,000 live births.

150
Q

???

Facteurs influençant le % de antibody titer– positive persons aux USA?

A

The percentage of antibody titer– positive persons in the United States depends upon geographic location and increases with age.

151
Q

Qui sont des hôtes définitifs de la Toxoplasmose?

A

Les félins
(Felines are the definitive host)

152
Q

Mode d’acquisition de la Toxoplasmose congénitale?

A

Toxoplasmosis can be acquired congenitally via transplacental transmission from an infected mother to the fetus.

153
Q

Manifestations extraoculaires de la toxoplasmose congénitale?

A

Congenital infection can result in
* Retinitis
* Hepatosplenomegaly
* Intracranial calcifications
* Microcephaly
* Developmental delay

154
Q

Manifestations oculaires de la toxoplasmose congénitale?

A

Ocular manifestations include
* Retinitis
* Choroiditis
* Iritis
* Anterior uveitis

The area of active retinal inflammation is usually thickened and cream colored with an overlying vitritis, frequently in the macula.

155
Q

À quoi ressemble l’area d’une active retinal inflammation dans la toxoplasmose?

A

The area of active retinal inflammation is usually thickened and cream colored with an overlying vitritis, frequently in the macula.

This area may be at the edge of an old, flat, atrophic scar (a so- called satellite lesion).

156
Q

V ou F : le Dx de la toxoplasmose est principalement clinique.

A

Vrai. Diagnosis is primarily clinical.

157
Q

Indications et Tx de la toxoplasmose.

A

Indication :
* Ocular toxoplasmosis does not require treatment unless it threatens vision.

Tx :
* Systemic treatment involves the use of one or more antimicrobial drugs +/- oral corticosteroids.
* Commonly used antimicrobial agents are pyrimethamine and sulfadiazine.
* Intravitreal injection of clindamycin and dexamethasone has been reported as a possible alternative treatment.

Pédiatrie (17 decks)

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