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Pediatrics > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

strongest risk factor for SLE

A

being a female

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2
Q

this hormone promotes B-cell autoreactivity and thus play a role in SLE

A

Estrogen

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3
Q

hallmark of SLE

A

generation of autoantibodies directed against self-antigens

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4
Q

most common presenting complaints of children with SLE

A

-fever
-fatigue
-hematologic abnormalities
-arthralgia
-arthritis

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5
Q

most sensitivite test for SLE

A

ANA

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6
Q

Most specific test for SLE

A

anti dsDNA and anti-smith

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7
Q

SLICC criteria has higher sensitivity but lower specificity compared to ACR criteria. True or False?

A

True

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8
Q

this test correlates with disease activity in SLE

A

anti-dsDNA

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9
Q

Treatment of choice in SLE

A

Hydroxychloroquine

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10
Q

this medication is reserved for most severe, potentially life threatening SLE manifestations

A

Cyclophosphamide

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11
Q

most common rheumatic disease in children

A

Juvenile idiopathic arthritis

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12
Q

most common subtype of JIA

A

oligoarthritis

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13
Q

most common inflammatory myositis in children

A

Juvenile Dermatomyositis

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14
Q

How to diagnose Juvenile Dermatomyositis?

A

Classic rash (heliotrope rash of the eyelids/Gottron papules) +
3 of the following:
-Weakness (Symmetric/Proximal)
-Muscle enzyme elevation (>=1, CK, AST, LDH, Aldolase)
-Electromyographic changes (Short, small polyphasic motor unit potentials/Fibrillations/Positive sharp waves/Insertional irritability)
-Muscle biopsy (Necrosis/Inflammation)

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15
Q

describe what is heliotrope rash in JDM

A

blue-violet discoloration of the eyelids

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16
Q

describe what are Gottron papules

A

bright-pink or pale, shiny, thickened or atrophic plaques over the proximal interphalangeal joints and distal interphalangeal joints and occasionally on the knees, elbows, small joints of the toes, and ankle malleoli

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17
Q

this test is present in >80% of children with JDM

A

ANA

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18
Q

Mainstay of treatment in Juvenile Dermatomyositis

A

Corticosteroids

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19
Q

Key process in the pathogenesis of both localized and systemic scleroderma

A

Autoimmunity

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20
Q

most common visceral manifestation of systemic scleroderma

A

Pulmonary disease

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21
Q

most frequent initial symptom in pediatric systemic sclerosis

A

Raynaud Phenomenon (RP)
- present in 70% of affected children

22
Q

What is Raynaud Phenomenon?

A

classic triphasic sequence of blanching, cyanosis and erythema of the digits induced by cold exposure and/or emotional stress

23
Q

major criterion in the diagnosis of Juvenile Systemic Sclerosis (JSSc)

A

Proximal skin sclerosis/induration of the skin proximal to metacarpophalangeal or metatarsophalangeal joints

24
Q

Classic characteristic feature of advance Ankylosing spondylitis in xray

A

Bamboo spine
— squaring of the corners of the vertebral bodies and syndesmophyte formation

25
Q

differentiate reactive arthritis from postinfectious arthritis

A

reactive arthritis refers to arthritis that occurs following enteropathic/urogenital arthritis while postinfectious arthritis occurs after infectious illnesses such as Group A streptococcus or viruses

26
Q

when do symptoms of reactive athritis usually appear following infection?

A

3 days to 6 weeks

27
Q

diffentiate Poststreptococcal arthritis from Rheumatic fever

A

RF arthritis manifests as painful migratory polyarthritis of brief duration while Poststreptococcal arthritis is typically oligoarticular

28
Q

systemic inflammatory disorder manifesting as vasculitis with a predilection for the coronary arteries

A

Kawasaki Disease (KD)

29
Q

How many of untreated children with Kawasaki Disease develop Coronary Artery abnormalities (CAA)

A

20-25%

30
Q

predictors of poor outcome in Kawasaki Disease

A
  • Young age
  • Male gender
  • Persistent fever
  • Poor response to IVIG
  • Laboratory abnormalities: neutrophilia, thrombocytopenia, transaminitis, hyponatremia, hypoalbuminemia, elevated levels of NT pro BNP and CRP
31
Q

what type of arteries is affected in Kawasaki Disease?

A

Medium-size arteries (particularly Coronary arteries)

32
Q

How to diagnose Kawasaki Disease?

A

Present of High grade fever of at least 4 days duration (temp >38.3) that is remitting and unresponsive to antipyretics +
4 of 5 principal criteria as follows:
1. bilateral nonexudative conjunctival injection with limbal sparing
2. erythema of the oral and pharyngeal mucosa with strawberry tongue and red, cracked liups
3. edema (induration) and erythema of the hands and feet
4. rash of various forms (maculopapular, erythema multiforme, scarlatiniform, psoriatic-like, urticarial or micropustular)
5. nonsuppurative cervical lymphadenopathy, usually unilateral, with node size >1.5 cm

33
Q

describe the 3 clinical phases of Kawasaki Disease

A
  1. ACUTE Phase - fever and signs of illness, lasts 1-2 weeks
  2. SUBACUTE Phase - assoc with desquamation, thrombocytosis, development of CAA, highest risk of sudden death who develop aneurysms, lasts 3 weeks
  3. CONVALESCENT Phase - all clinical signs disappeared, continues until ESR becomes normal, 6-8 weeks after the onset of illness
34
Q

how often is 2D echo be performed in Kawasaki Disease?

A

at diagnosis
after 1-2 weeks of illness
if normal, repat 6-8 weeks after onset of illness

35
Q

diagnosis of KD should be made within ___ days, ideally within __ days of fever onset to improve coronary artery outcomes

A

10 days, ideally 7 days

36
Q

treatment of Kawasaki Disease

A

IVIG - 2 g/kg as single infusion over 10-12 hour within 10 days of disease onset ASAP ++
Moderate (30-50 mkday q6) - high dose (80-100 mkday q6) Aspirin until afebrile

37
Q

Antithrombotic dose of Aspirin used in KD after 48 hours afebrile

A

3-5 mkday single dose for 6-8 weeks

38
Q

KD patients undergoing long-term aspirin therapy should receive what kind of vaccine and what is the purpose of it?

A

Annual Influenza vaccination to reduce risk of Reye Syndrome

39
Q

most common vasculitis of childhood

A

Henoch-schonlein purpura

40
Q

90% of HSP cases occur in what age group in children

A

3 and 10 year

41
Q

hallmark of HSP

A

RASH — palpable purpura
» starting as pink macules or wheals and develops into petechiae, raised purpura or larger ecchymoses

42
Q

Criteria for HSP

A

Palpable purpura (without coagulopathy/thrombocytopenia) + 1 or more of the ff:
- abdominal pain (acute, diffuse, colicky)
- arthritis or arthralgia
- biopsy of affected tissue demonstrating predominant IgA deposition
- Renal involvement (proteinuria >3 g/24 hr), hematuria or red cell casts

43
Q

major long-term complication in HSP which occur in 1-2% of children

A

Renal disease

44
Q

recommended serial monitoring of children with HSP

A

BP and Urinalysis for at least 6 months after diagnosis
— to monitor development of nephritis

45
Q

known as pulseless disease

A

Takayasu Arteritis

46
Q

What kind of occlusive complication is predominantly seen in Southest asia and Africa in Takayasu Arteritis?

A

Aneurysm

47
Q

Most commonly involved aortic branches in Takayasu Arteritis

A
  • Renal artery
  • Subclavian artery
  • Carotid artery
48
Q

Crtieria for Pediatric-Onset Takayasu Arteritis

A
  • Angiographic abnormalities of the aorta or its main branches and at least 1 of the ff:
    > Decreased peripheral artery pulse and/or claudication of extremities
    > BP difference between arms or legs of >10 mmHg
    > Bruits over the aorta and/or its major branches
    > Hypertension
    > Elevatd ESR or CRP
49
Q

Mainstay therapy of Takayasu Arteritis

A

Glucocorticoids

50
Q

Systemic necrotizing vasculitis affecting small and medium0size arteries

A

Polyarteritis nodosa

51
Q

these vessles are usually spared in Polyarteritis Nodusa

A

Pulmonary Vasculature

52
Q

gold standard diagnostic imaging study for PAN

A

Conventional arteriography
— “beads on a string” appearance

Pediatrics (17 decks)

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