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CT.MS > JRA and pediatric rheumatology > Flashcards

JRA and pediatric rheumatology Flashcards

1
Q

What abnormalities might cue you in to a child with MSK problems?

A

Growth abnormalities, secondary sex characteristics delayed

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2
Q

How do you diagnose benign juvenile hypermobility syndrome?

A

beighton score positive @ >5/9

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3
Q

Definition of juvenile idiopathic arthritis

A

chronic arthritis in joint for > 6 weeks in pt under age 16

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4
Q

What are 3 types of JIA

A

pauciarticular polyarticular systemic

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5
Q

Describe clinical features of JIA subgroups: pauci, oligoarticular, and diffuse by different ages of onset

A
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6
Q

What is the most common type of JIA?

A

Oligoarthritis

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7
Q

Describe the pathophysiology of synovitis

A

TNF-Alpha is the pirmary mediator/

  1. T cells activate macrophages
  2. B cells are activated
  3. TNF is the key cytokine
  4. Thickening of synovial membrane, WBCs recruited and hypervascular
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8
Q

What is unique about systemic JIA?

A

Rash, ascites, enlarged axillary lymph nodes. Systemic Sx like fever and abnormal CBC

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9
Q

What is the Koebner phenomenon?

A

Scratching makes the rash worse

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10
Q

What are clinical features of JIA?

A

Growth plates closed (epiphyseal overgrowth) resulting in shortened bones

Fusion of C-spine joints

Microganthia

Uveitis

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11
Q

What does pauci articular arthritis in the 11-16 yo look like?

A

Ankylosing spondylitis

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12
Q

What does polyarticular disease in the 11-16 yo look like?

A

Rheumatoid arthritis. Will progress to this eventually

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13
Q

What lab values would you see in JRA?

A

WBC: Normal/High (systemic)

Hb: can have anemia of chronic dz

plts: normal or HIGH with acute phase rxn (rules out leukemia)

ESR: normal/high

igA: 10% are deficient

May be ANA or RF positive too. Or ACPA

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14
Q

Who is at highest risk for uveitis? What is so difficult with treating uveitis?

A

pauci and polyarticular, ANA+ children under 7. All the pts with systemic uveitis are at LOW risk. Difficult to diagnose, asymptomatic except for detection by slit lamp, and will lead to blindness. Tx with steroids

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15
Q

How do you treat JRA?

A

NSAIDS for oligoarthritis initially, then hydrochloroquinone or sulfazalazine/steroids. If not responsive, consider methotrexate

Methotrexate,leflunomide for polyarthritis/systemic cases

Biologics for systemic.

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16
Q

What are some biologic targets in JIA?

A
  1. Anti-CTLA-4
  2. Anti-IL1 and IL6
  3. Anti-TNF Humira
17
Q

What is the cornerstone tx for polyarthritis?

A

Methotrexate. Note that corticosteroids, if given intra-articularly, can be disease modifying

18
Q

What percent of kids will reach remission in JIA?

A

65% by adulthood

19
Q

What is the difference between primary and secondary Raynaud’s?

A

Primary: excessive vasospastic response. Not more likely to get frostbite

Secondary: Usually caused by some other connective tissue disease

20
Q

What are some causes of secondary Raynaud’s?

A

Scleroderma

SLE

Sjogren’s

Arteriolar disease

Drugs/toxins like beta blockers

Hyperproteinemia

21
Q

How can you tell whether Raynauds is primary or secondary?

A

Secondary will show pathologic nailfold capillaries

22
Q

What is chillblains? What do you need to diagnose it?

A

Perniosis: Venule vasodilatation, blood pools in the veins and doesn’t move. Happens in the toes. Can be primary or secondary to lupus

To tell chillblains from SLE, you need to biopsy. Will show lymphocytic infiltrate into the venules

23
Q

Acrocyanosis

A

Shunting of blood from the periphery to the core organs. It is a normal physiologic response after birth. Hands and feet of babies turn blue

24
Q

What does morphea look like? what is it?

A

Areas of hypopigmentation. caused by scleroderma limited to fibrosis in the dermis, subdermis, and superficial muscle. No systemic features. Only treat leasions on the face or large lesions because tight skin can cause a flexion contracture.

25
Q

Name for morphea on the face. How do you treat?

A

“coup de sabre” Treat aggressively with methotrexate

26
Q

Neonatal lupus: Cause? treatment?

A

Caused by moms with Ro and La antibodies/Sjogren’s. Usually, it’s self limited, only need to treat the heart block.

27
Q

What’s another name for Henoch-Schonlein Purpura?

A

Leukocytoclastic vasculitis

28
Q

How do you treat HSP? What is diagnostic?

A

Treat with hydration and time. maybe NSAIDs, if bad use steroids. Usually a clinical diagnosis, but can stain for igA immune complexes

29
Q

Diagnostic criteria for kawasaki’s

A

Fever for at least 5 days***

palmar erythema/edema

Lymphadenopathy

Conjunctival injection

erythematous rash/desquamation

oropharyngeal symptoms: “strawberry tongue”

30
Q

What are you most worried about with kawasakis

A

Coronary aneurysms: MI, AI, MR Get an echo! Then treat with aspirin and IVIg

CT.MS (13 decks)

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