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CKPeds > Rheuma > Flashcards

Rheuma Flashcards

1
Q

idiopathic synovitis of peripheral joints associated with soft-tissue
swelling and joint effusion

A

JUVENILE IDIOPATHIC ARTHRITIS (JIA)

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2
Q

Pathophysio of JRA

A

Vascular endothelial hyperplasia and progressive erosion of articular cartilage
and contiguous bone

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3
Q

Association of JRA

A

− DR8 and DR5

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4
Q

SSx of JRA

A

− Morning stiffness; easy fatigability
− Joint pain later in the day, joint swelling, joints warm with decreased motion, and
pain on motion, but no redness

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5
Q

Dx of JRA

A

− Age of onset: <16 years
− Arthritis in one or more joints
− Duration: ≥6 weeks
− Onset type by disease presentation in first 6 months

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6
Q

Exclude other associations with JRA

A

Exclusion of other forms of arthritis, other connective tissue diseases and vasculitides,
Lyme disease, psoriatic arthritis, inflammatory bowel disease, lymphoproliferative
disease

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7
Q

Clinical PP factors for JRA

A

− Young age at onset
− Rheumatoid nodules
− Large number of affected joints
− Involvement of hip, hands and wrists

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8
Q

Lab PP factors for JRA

A

− RF+

− Persistence of anti-cyclic citrullinated peptide (CCP) antibodies

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9
Q

WHy is systemic JRA difficult to treat?

A

Systemic onset JRA is the most difficult to control in terms of both articular
inflammation and systemic manifestations (poorer with polyarthritis, fever >3
months and increased inflammatory markers for >6 months)

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10
Q

Categories of JRA

A

Pauciarticular (oligoarthritis)
Polyarticular, RF negative
Polyarticular RF positive
Systemic Onset

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11
Q

What type of JRA

° Pattern: 1-4 joints affected in first 6 months; primarily knees (++) and ankles
(+), less so the fingers; never presents with hip involvement
° Peak age <6 years
° F:M = 4:1
° % of all: 50-60%

A

Pauciarticular (oligoarthritis)

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12
Q

Extraarticular SSx of Pauciarticular (oligoarthritis) JRA

A

Extra-articular: 30% with anterior uveitis

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13
Q

Pauciarticular (oligoarthritis) JRA Labs

A

ANA+ in 60%; other tests normal; may have mildly increased ESR, CRP

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14
Q

auciarticular (oligoarthritis) JRA Tx

A

NSAIDs + intraarticular steroids as needed; methotrexate occasionally
needed

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15
Q

What type of JRA?

° Pattern: 5 joints in first 6 months; both UE and LE small and large joints;
may have C-spine and TMJ involvement
° Peak age: 6-7 years
° F:M: 3:1
° % of all: 30%
A

Polyarticular, RF negative

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16
Q

Association of Polyarticular, RF negative

A

Extra-articular: 10% with anterior uveitis

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17
Q

Labs of Extra-articular: 10% with anterior uveitis

A

ANA+ in 40%; RF negative; ESR increased (may be significantly), but
CRP increased slightly or normal; mild anemia

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18
Q

Tx of Polyarticular, RF negative

A

Treatment: NSAIDs + methotrexate; if not responsive, anti-TNF or other biologicals
(as FDA-approved for children)

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19
Q

What type of JRA

° Pattern: ≥5 joints as above but will be aggressive symmetric polyarthritis
° Peak age: 9-12 years
° F:M: 9:1
° % of all: <10%

A

Polyarticular RF positive

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20
Q

Extra-articular findings of JRA:

A

rheumatoid nodules in 10% (more aggressive)

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21
Q

Labs of Polyarticular RF positive JRA

A

RF positive; ESR greatly, CRP increased top normal; mild anemia; if anti-CCP antibodies are positive, then significantly worse disease

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22
Q

Tx of JRA

A

Treatment: long-term remission unlikely; early aggressive treatment is warranted

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23
Q

What type of JRA?

Pattern: arthritis may affect any number of joints, but course is usually polyarticular,
destructive and ultimately affecting hips, C-spine and TMJ
° Peak age: 2-4 years
° F:M: 1:1
° % of all: <10%

A

Systemic Onset

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24
Q

Fever pattern of systemic JRA

A

For initial diagnosis, in addition to arthritis in ≥1 joint, must have with or be preceded by fever ≥2 weeks documented to be quotidian (daily, rises to 39˚ then back to 37˚) for at least 3 days of the ≥2-week period

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25
Q

Systemic findings of JRA

A

 Evanescent (nonfixed, migratory; lasts about 1 hour) erythematous, salmoncolored
rash (linear or circular), most over the trunk and proximal extremities

 Generalized lymph node involvement

 Hepatomegaly, splenomegaly or both

 Serositis (pleuritis, pericarditis, peritonitis)

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26
Q

Tx of JRA

A

less responsive to standard treatment with methotrexate and anti-

TNF agents; consider IL-1 receptor antagonists in resistant cases.

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27
Q

Most with pauciarticular disease respond to __________

A

nonsteroidal antiinflammatory

drugs (NSAIDs) alone

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28
Q

Additional Tx to JRA

A

Additional treatmentmethotrexate (safest and most efficacious of secondline
agents); azathioprine or cyclophosphamide and biologicals

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29
Q

What is the prognosis?

Polyarticular disease

RF+

Older girls; hand and wrist;
erosions, nodules, unremitting

A

PP

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30
Q

What is the prognosis?

Polyarticular disease

ANA+ Younger girls

A

GP

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31
Q

What is the prognosis?

Pauci

ANA+

Younger girls; chronic iridocyclitis

A

Excellent,

except eyes

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32
Q

What is the prognosis?

Pauci

RF+ Polyarthritis, erosions, unremitting

A

PP

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33
Q

What is the prognosis?

Pauci

HLA B27/Seronegative

Older males

A

GP

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34
Q

What is the prognosis?

Systemic  Pauciarticular

A

GP

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35
Q

What is the prognosis?

Systemic Polyarticular

A

PP

36
Q

What is the etiolgy of SLE

A

Autoantibodies, especially against nucleic acids including DNA and other nuclear
antigens and ribosomes; blood cells and many tissue-specific antigens; immune
complex deposition

37
Q

Immune complex deposition in the dermal/epidermal junction is specific for
SLE (called the___________)

A

lupus band test)

38
Q

In SLE

________significantly increases risk for
severe renal morbidity (pathology varies from minimal mesangial changes to
advanced sclerosing nephritis

A

Diffuse proliferative glomerulonephritis

39
Q

Difference in adult and child SLE

A

Compared with adults, children have more severe disease and more widespread
organ involvement

40
Q

Usual presentation of SLE

A

Rare age <5 years and only up to 20% present age <16 years, so usual presentation
is mid-to-late adolescence

41
Q

Diagnosis of SLE—

“M.D. Soap ’n Hair

A 
• Malar rash
• Discoid rash
• Serositis
• Oral ulcers
• ANA-positive
• Photosensitivity
• Neurologic disorders
• Hematologic disorders
• Arthritis
• Immune disorders (LE
[lupus erythematosus]
prep test, anti-DNA,
Smith)
• Renal disorders
42
Q

MC Sx of SLE in children

A

Most common is a female with fever, fatigue, rash, hematological abnormalities
(anemia of chronic disease or hemolytic; thrombocytopenia, leukopenia)
and arthralgia/arthritis

43
Q

Non-specific tests for SLE

A

elevated ESR, CRP, platelets, anemia, elevated WBC or leukopenia/
lymphopenia; decreased CH50, C3, C4 (typically decreased in active disease and
increases with treatment

44
Q

______present in 95-99% of SLE patients but has poor specificity; does not
reflect disease activity; first screening test

A

+ANA:

45
Q

_______: more specific (but not 100%) and may correlate with disease
activity, especially nephritis

A

+anti-DS-DNA

46
Q

______ 100% specific but no disease activity correlation

A

+anti-Smith antibody (anti-Sm):

47
Q

_________increased with Raynaud’s phenomenon (blanching of fingers) and pulmonary hypertension; high titer may be diagnostic of mixed CT disorder

A

Antiribonucleoprotein antibodies:

48
Q

Antiribonucleoprotein antibodies: marker of?

A

antiribosomal-P-antibody is a marker for lupus cerebritis

49
Q

_______ IgG maternal antibodies crossing the placenta and produce transient neonatal lupus; may suggest Sjögren syndrome

A

Anti-Ro antibody (anti-SSA):

50
Q

________ also increased risk of neonatal lupus; may be associated with
cutaneous and pulmonary manifestations of SLE or isolated discoid lupus; also
seen in Sjögren syndrome

A

Anti-La (anti-SSB):

51
Q

What Ab can be present?

° Increased risk of arterial and venous thrombosis
° Livedo reticularis
° Raynaud’s phenomenon
° Recurrent fetal loss

A

Antiphospholipid antibodies (APL; including anticardiolipin):

52
Q

_______may give a false-positive serological test for syphilis; also seen in patients with neurological complications

A

Positive lupus anticoagulant

53
Q

Association of diff lab tests for SLE

– Coombs positive: ______
– Antiplatelet antibodies: ______
– Antithyroid antibodies: _____

A

hemolytic anemia

thrombocytopenia

autoimmune thyroiditis

54
Q

_______: may be found with drug-induced lupus; may act as a trigger in those prone to lupus or cause a reversible syndrome hepatitis is common

A

Antihistone antibodies

55
Q

General principles of Tx for SLE

A

– Sunscreen and direct sun avoidance
– Hydroxychloroquine for all, if tolerated
– NSAIDs for joints
– Corticosteroids for more severe disease, especially renal
– Steroid-sparing immunosuppressives for severe disease

56
Q

• Passive transfer of IgG across placenta; most is maternal anti-Ro and anti-La

A

NEONATAL LUPUS

57
Q

SSx of NEONATAL LUPUS

A

Mostly presents at age 6 weeks with annular or macular rash affecting the face, especially
periorbital area, trunk and scalp after exposure to any UV light; generally lasts
3-4 months

58
Q

Neonatal lupus:

May manifest with any SLE finding, but all resolve unless there is _________is permanent; if it is third degree,
pacing is usually required

A

congenital heart

block (

59
Q

Genetic susceptibility of KD: highest in ______irrespective of location and in children
and sibs of those with KD

A

Asians

60
Q

Age of occurence of KD

A

80% present at age <5 years (median is 2.5 years) but may occur in adolescence

61
Q

PP factors of KD

A

Poor outcome predictors with respect to coronary artery disease: very young age,
male, neutrophilia, decreased platelets, increased liver enzymes, decreased albumin,
hyponatremia, increased CRP, prolonged fever

62
Q

Pathology of thrombosis in KD

A

Loss of structural integrity weakens the vessel wall and results in ectasia or saccular
or fusiform aneurysms; thrombi may decrease flow with time and can become
progressively fibrotic, leading to stenosis

63
Q

Absolute reqt for dx of KD

A

Absolute requirement: fever ≥5 days (≥101˚ F), unremitting and unresponsive;
would last 1−2 weeks without treatment

64
Q

Rash pattern of KD

A

polymorphic exanthema (maculopapular, erythema multiforme or scarlatiniform
with accentuation in the groin); perineal desquamation common ion
acute phase

65
Q

Coronary Aneursym asstd with KD

A

up to 25% without treatment in week 2-3; approximately 2−4% with early diagnosis and treatment; giant aneurysms (>8 mm) pose greatest threat for rupture, thrombosis, stenosis and MI; best detected by 2D echocardiogram

66
Q

Myocarditis asstd with KD

A

in most in the acute phase; tachycardia out of proportion to the fever and decreased LV systolic function; occasional cardiogenic shock; pericarditis with small effusions.

About 25% with mitral regurgitation, mild and improves over time; best detected by 2D echocardiogram plus EKG

67
Q

Most important tests at admission of acute KD are (1-2 weeks)

A

platelet count, ESR, EKG, and baseline 2D-echocardiogram

68
Q

Tests for Subacute: next 2 weeks

A

acute symptoms resolving or resolved; extremity desquamation, significant increase in platelet count beyond upper limits of normal (rapid increase in weeks 2-3, often greater and a million); coronary aneurysm,
if present, this is the time of highest risk of sudden death

69
Q

Most important tests at admission of subacute KD are

A

Follow platelets, ESR and obtain 2nd echocardiogram.

70
Q

What KD stage

________: next 2-4 weeks; when all clinical signs of disease have disappeared
and continues until ESR normalizes

A

Convalescent

71
Q

What labs to do during Convalescent stage

A

follow platelet, ESR and if no evidence of

aneurysm, obtain 3rd echocardiogram; repeat echo and lipids at 1 year

72
Q

Tx of KD

A

follow platelet, ESR and if no evidence of

aneurysm, obtain 3rd echocardiogram; repeat echo and lipids at 1 year

73
Q

When to give 2nd infusion of IVIg

A

If continued fever after

36 hours, then increased risk of aneurysm; give 2nd infusion

74
Q

KD Preventive Tx if with aneurysms

Small solitary aneurysms: ______ indefinitely;

giant or numerous aneurysms need individualized therapy, including____

A

continue ASA

thrombolytic

75
Q

Long-term follow-up with aneurysms from KD:

A

periodic echo and stress test and perhaps
angiography; if giant, catheter intervention and percutaneous transluminal coronary
artery ablation, direct atherectomy and stent placement (and even bypass surgery)

76
Q

Prognosis of aneurysms

A

Overall- 50% of aneurysms regress over 1-2 years but continue to have vessel
wall anomalies; giant aneurysms are unlikely to resolve

77
Q

Acute KD recurs in ______

A

1-3%

78
Q

Most common vasculitis among children in United States; l

A

HSP

79
Q

Pathology of HSP

A

leukocytoclastic vasculitis (vascular damage from nuclear debris of infiltrating neutrophils) + IgA deposition in small vessels (arterioles and venules) of skin, joints, GI tract and kidney

80
Q

HSP

Skin biopsy shows _____

A

vasculitis of dermal capillaries and postcapillary venules with
infiltrates of neutrophils and monocytes; in all tissues, immunofluorescence shows
IgA deposition in walls of small vessels and smaller amounts of C3, fibrin and IgM

81
Q

Arthralgia in HSP

A

oligoarticular, self-limited and in lower extremities; resolves in about 2 weeks, but may recur

82
Q

GI Sx in HSP

A

pain, vomiting, diarrhea, ileus, melena, intussusception, mesenteric ischemia or perforation (purpura in GI tract)

83
Q

Renal Rx in HSP

A

Renal: up to 50%: hematuria, proteinuria, hypertension, nephritis, nephrosis,
acute or chronic renal failure

84
Q 
American College of Rheumatology for HSP diagnosis: need 2 of the following:
1
2
3
4
A

(a) palpable purpura
(b) age of onset <10 years
(c) bowel angina = postprandial pain, bloody diarrhea
(d) biopsy showing intramural granulocytes in small arterioles and venules

85
Q

Tx of HSP

A

Treatment: supportive and corticosteroids, but only with significant GI involvement
or life-threatening complications (but steroids do not alter course alter
overall prognosis nor prevent renal disease (c)

86
Q

Chronic HSP Tx

A

for chronic renal disease – azathioprine,

cyclophosphamide, mycophenolate mofetil.

CKPeds (20 decks)

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