24 - Uveitis Flashcards

Question

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

Answer 1

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

Answer 2

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

Answer 3

Uveitis occurs in approximately 10% of these children.

Answer 4

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

Answer 5

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

Answer 6

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

Answer 7

* 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.

Answer 8

* 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.

Answer 9

* 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.

Answer 10

Bilatérale

Answer 11

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

Answer 12

Vitritis and macular edema occur infrequently.

Answer 13

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.

Answer 14

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

Answer 15

* 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

Answer 16

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

Answer 17

Juvenile spondyloarthropathies are a group of **HLAB27**– associated disorders

Answer 18

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

Answer 19

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

Answer 20

* Nail changes : Pitting or onycholysis * Dactylitis * Psoriasis

Answer 21

* Involves the spine * Sacroiliitis (which may be subclinical) * Cardiac disease rare in children

Answer 22

* Crohn disease * Ulcerative colitis

Answer 23

* Follow an infection not involving the joints * Urethritis

Answer 24

* Occurs in 20% of affected children * Acute, symptomatic * Unilateral, but both eyes may be affected at different times

Answer 25

* Occurs in 10% of affected children * Insidious and Chronic anterior uveitis * Usually bilateral

Answer 26

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

Answer 27

Occurs in 5% of those affected

Answer 28

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

Answer 29

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

Answer 30

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

Answer 31

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

Answer 32

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

Answer 33

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

Answer 34

The median age at onset is **15 years**.

Answer 35

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

Answer 36

Elevated levels of **β2microglobulin** are usually present in the urine.

Answer 37

* The uveitis is usually bilateral * May occur before, simultaneously with, or after the renal disease

Answer 38

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

Answer 39

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

Answer 40

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

Answer 41

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

Answer 42

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

Answer 43

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.

Answer 44

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.

Answer 45

* 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

Answer 46

* 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**

Answer 47

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

Answer 48

* 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.

Answer 49

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

Answer 50

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

Answer 51

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

Answer 52

Toxoplasmosis Toxocariasis

Answer 53

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

Answer 54

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

Answer 55

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

Answer 56

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

Answer 57

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

Answer 58

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

Answer 59

Laboratory testing reveals eosinophilia.

Answer 60

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

Answer 61

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

Answer 62

The average age at onset is **11.5 years**.

Answer 63

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

Answer 64

* 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.

Answer 65

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**.

Answer 66

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.

Answer 67

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.

Answer 68

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

Answer 69

Triad of uveitis, granulomatous arthritis, and rash

Answer 70

In young patients (<5 years)

Answer 71

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

Answer 72

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

Answer 73

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.

Answer 74

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.

Answer 75

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

Answer 76

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.

Answer 77

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.

Answer 78

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

Answer 79

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.

Answer 80

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**.

Answer 81

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.

Answer 82

* **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.

Answer 83

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

Answer 84

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.

Answer 85

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.

Answer 86

Various conditions can simulate pediatric uveitis.

Answer 87

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

Answer 88

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

Answer 89

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

Answer 90

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.

Answer 91

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

Answer 92

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** .

Answer 93

**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.

Answer 94

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

Answer 95

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

Answer 96

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.

Answer 97

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

Answer 98

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.

Answer 99

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.

Answer 100

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

Answer 101

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

Answer 102

Less commonly used antimetabolites include **azathioprine** and **mycophenolate mofetil**.

Answer 103

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

Answer 104

**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

Answer 105

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

Answer 106

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

Answer 107

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.

Answer 108

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.

Answer 109

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

Answer 110

* Infliximab is given by infusion * Adalimumab is given subcutaneously

Answer 111

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

Answer 112

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.

Answer 113

Complications of uveitis include * Band keratopathy * Cataract * Glaucoma

Answer 114

* 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

Answer 115

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.

Answer 116

* 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**

Answer 117

* Iris * Corps ciliaire * Choroïde

Answer 118

Oligoarthritis (formerly pauciarticular arthritis)

Answer 119

Parasite protozoaire intracellulaire

Answer 120

Toxoplasmose gondii

Answer 121

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

Answer 122

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

Answer 123

Les félins (Felines are the definitive host)

Answer 124

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

Answer 125

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

Answer 126

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.

Answer 127

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).

Answer 128

Vrai. Diagnosis is primarily clinical.

Answer 129

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.

24 - Uveitis Flashcards

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