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MSK & Rheum > Mohammad-Rheum-Core > Flashcards

Mohammad-Rheum-Core Flashcards

1
Q

What’s the most common vasculitis disorder in childhood?

Pathophysiology?

A

HSP

Leukocytoclastic vasculitis and IgA deposition in small vessel in the skin, joints, GI and Kidneys

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2
Q

Which age does HSP affect?

A

3-10yrs
More males than females
More in Asian and white ethnicity
Many follow URTI

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3
Q

What’s the hallmark of HSP manifestation?

A
  • palpable purpura (petechiae, or larger ecchymoses.)
  • Symmetric in pattern, in Gravity dependent areas (legs, buttocks). Usually lasting 3-10 days up to 4 months

Can also have subcutneous edema in dorsum of the hand, feet, periorbital area, lips, scrotum, or scalp.

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4
Q

What are the manifestations of HSP? (4)

A
  • Palpable purpra/patechia
  • MSK (arthritis/arthrlagia in 75%)
  • Abdo pain (80%)
  • Renal (50%)
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5
Q

What’s the pattern of arthritis in HSP?

A

Oligoarticular

Self limiting
Mostly lower extremities
2 wks to go away (may recur)

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6
Q

What are the GI manifestations of HSP (5)

A 
N/V
Diarrhea
Pain
Paralytic ileus 
Intussusception, possibly malena
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7
Q

Renal manifestations of HSP? (4)

A

Microscopic hematuria
HTN
Nephrotic syndrome
Proteinuria

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8
Q

Criteria for HSP diagnosis?

A
  • *American criteria: 2/4
  • Palpable Purpra
  • Age <20
  • Bowel angina
  • Biopsy showing intraluminal granulocyte

**Eurpean criteria:
-Palpable purpra in the abscence of coagulopathy or thrombocytopenia
1/4:
-Abdominal pain
-Arthritis
-Biopsy of an affected tissue showing IgA
-Renal involvement (protinuria >3g/d, hematuria, red cell cast)

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9
Q

How to differentiate Acute Hemorrhagic Edema from HSP ? (7)

A

AHE:

  • Usually <2 yrs**
  • Purpra usually larger**
  • Trunk clear
  • Fever
  • Tender edema**
  • Well appearing
  • No other organ involvement (normal UA and N plt)**
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10
Q

What causes Papular-purpuric gloves-and-socks syndrome (PPGSS)?

A

Parvo B19

Pupuric lesions end at the ankles and wrists
Adolescents more commonly affected

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11
Q

Common lab findings in HSP? (6)

A

Nothing is diagnostic:

  • Mild anemia
  • Leukocytosis
  • Thrombocytosis or NORMAL Plt
  • Elevated ESR, CRP
  • Occult blood
  • May have low Albumin
  • Elevated IgA**
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12
Q

Tx of HSP? (2)

A
  • Supportive

- May consider Steroids for significant GI involvement or lifethreatnening (1/Kg for 1-2 wks)

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13
Q

Utility of Prednison in HSP? (3)

A

Reduces abdominal and joint pain
Does not alter disease course
Does not prevent renal involvement

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14
Q

Complications of HSP? (3)

A

Renal disease (1-2%)
Intestinal perforation
Recurrence of HSP

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15
Q

What rheum conditions may be associated with oral ulcers? (4)

A
  • SLE
  • Bachet
  • HSV
  • PFAPA
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16
Q

Which conditions causes Malar Rash? (4)

A
  • SLE (spraing nasolabial folds)
  • JDM
  • KD
  • Parvo B19 (fifth disease)
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17
Q

Which autoantibodies are positive in SLE? (6)

A 
ANA
dsDNA**
Smith**
Anti-Ro
Anti-La
Anti phospholipid ab: (eg. False positive RPR)

**Very specific

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18
Q

MOE of NSAIDs?

A

Inhibiting COX= prostaglandin inhibitor

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19
Q

How long does it NSAIDs take before they can work for JIA?

A

Require 4-6 wks of regular use before they show effects.

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20
Q

Most common rheumatic disease of childhood?

A

JIA

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21
Q

Criteria for JIA? (3)

A
  • Age at onset <16
  • Arthritis in >=1 joint (Swelling or effusion OR the presence of >2 of the following: Limited ROM, pain on ROM, increased heat)
  • Duration of disease >=6 wks
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22
Q

Criteria for Systemic JIA? (5)

A

-Arthritis in >=1 joint OOOORRR fever of at least 2 wks duration that is quotidian for at least 3 days. (arthritis not necessary)

PLUS at least one of the following:

  • ‘Evanescent’ (Non-fixed) erythematous rash
  • Generalized lymph node enlargement
  • Hepatomegaly or splenomegaly or both
  • Serositis
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23
Q

What’s Quotidian fever?

A

Fever that rises to at least 39 once a day and goes back to normal between peaks.

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24
Q

What is serositis? (3)

A

pericarditis, pleuritis, or peritonitis, or some combination of the 3.

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25
Q

What’s Oligoarthritis?
Persistent oligo?
Extended Oligo?

A

-Arthritis affecting 1-4 joints for the first 6 months of the disease (i.e. cannot diagnose it before 6 months)

Persistent: =< 4 joints throught out the disease course
Extended: >4 AFTER the first 6 months of the disease

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26
Q

Criteria for Polyarthritis RF negative?

A

> =5 joints in 1st 6 months

RF negative

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27
Q

Criteria for polyarthritis RF +ve disease?

A

> =5 joints in 1st 6 months
= 2 RF tests positive 3 months apart
RF positive during 1st 6 months

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28
Q

What’s Enthesis?

A

Tenderness at the tendon insertion site, or fascia to bone, joint capsule.

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29
Q

Peak ages for JIA?

A

2-4 yrs for oligo
1-5 for sJIA
Bimodal for polyJIA: 2-4 and 10-14

More females in Oligo and Poly

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30
Q

T or F

All JIA have inflammatory synovitis

A

T

Characterized by lymphocyte predominance

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31
Q

What is arthritis ? (4)

A

Swelling or effusion OR the presence of >2 of the following: Limited ROM, pain on ROM, No erythema

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32
Q

Why may children w JIA have discrepant limb length?

A

Arthritis accelerates bone growth initially and then stimulated rapid and premature closure of growth plates.

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33
Q

Which joint tend to be most affected in Oligo JIA?

Prevalence of uveitis? Prevalance of ANA +ve?

A

Knee
Uveitis: 30%
ANA +ve in 60%

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34
Q

Main treatment for Oligo JIA? (2)

A

NSIADs and intrarticular steroids

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35
Q

How does RF status affect prognosis in Poly?

A

RF +ve is more aggressive

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36
Q

which type of JIA has cervical spine involvement? Risks w cervical spine involvement?

A

Usually in Poly JIA

Risk atlantoaxial subluxation and neurologic sequelae

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37
Q

Features of sJIA rash? (4)

A

salmon-colored, macular, and nonpruritic.
Evanescent: Migratory lasting <1hr
Can be heat provoked
Koebner phenomenon (brough on by superficial traum)

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38
Q

Rash brought on by superficial trauma. What to call?

A

Koebner phenomenon

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39
Q

What’s a rare lethal complication of sJIA?

A

MAS

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40
Q

Manifestation of MAS?

A
  • acute onset of high spiking fevers, nonremitting
  • lymphadenopathy
  • hepatosplenomegaly,
  • CNS dysfunction (headache, disorientation, lethargy, seizure, coma)

-Macrophages hemophagocytosis

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41
Q

Lab findings in MAS? (10)

A 
1-Thrombocytopenia
2-Leukopenia
3-Elevated LDH
4-Elevated Ferritin
5-Elevated Trig
6-Elevated Liver enzymes
7-Low ESR
8-Hyponatremia
9-Elevated CD25 and CD163
10-Hypoalbuminemia
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42
Q

What can help distinguish MAS from flares of other systemic diseases? (1)

A

In MAS, ESR falls because of hypofibrigenemia and hepatic dysfunction. This does not happen in flares of other systemic illnesses

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43
Q

Tx of MAS? (3)

A

EMERGENCY:
High dose Methylpred
Cyclosporin
Anakinra (anti IL1)

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44
Q

What are some of the complications associated w JIA? (4)

A
  • Osteopenia (the longer the disease the worse it gets)
  • Flexure contracture
  • Leg length discrepancies
  • Chronic uveitis
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45
Q

What organisms tend to cause reactive arthritis? (7)

A

Bacteria: Shigella, Salmonella, Yersinia, Chlamydia, or meningococcus, Campylobacter.
Ecoli sometimes

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46
Q

What’s pattern of arthritis in Acute Rheumatic fever?

A

Migratory polyarthritis

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47
Q

What’s the pattern of arthritis in lyme disease?

A

Oligoarthritis

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48
Q

What suggests septic arthritis? (4)

A

Fever
Swelling
Erythema
Hot joint

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49
Q

Characteristics of growing pain? (3)

A

Child 4-12 yr
Leg pain in the evenings
Normal investigations
No morning symptoms.

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50
Q

What’s ANA like in sJIA?

A

Rarely elevated

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51
Q

Why must RF be repeated twice in poly JIA?

A

Can be false +ve from viral infection

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52
Q

What’s the dose of NSAIDs for JIA? Who usually responds?

A

Ibuprofen:
40mg/Kg/Day divided TID

Naproxen:
15mg/Kg/Day divided BID

Oligo usually responds
Poly and sJIA don’t

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53
Q

What are potential SE of NSAIDs? (4)

A

Renal toxicity
Hepato toxicity
Risk of pseudoporphyria
Gastritis

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54
Q

SE of Methotrexate? (4)

A

N/V
oral ulcerations,
hepatic toxicity
Immunosuppresion

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55
Q

Indications for intraarticular joint injection in JIA? (4)

A

No response to steroids after 4-6wks
Joint contracture
leg length discrepancies
Functional limitation

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56
Q

What should pt with JIA routinely be screened for?

A

Asymptomatic uveitis

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57
Q

Who is at greater risk for developing chronic uveitis? (4)

A

Oligo
Girls
Disease <6 yr
ANA +ve

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58
Q

Predictors of severe JIA? (6)

A
  • RF +ve
  • Rheumatoid nodules
  • Anti CCP
  • Younger age at onset
  • Large number of affected joints
  • Disease affecting hand or hip
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59
Q

Characteristics of inflammatory bone pain? (5)

A
  • Pain at night w morning stiffness
  • No improvement w rest
  • Improvement w exercise
  • Insidious onset
  • Good response to NSAIDs
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60
Q

What’s the difference between reactive arthritis and post-infectious arthritis?

A
  • *Reactive arthritis:
  • occurs post enteropathic or urogenital infections
  • Can remit or progress to ankylosing spond
  • HLA B27 +ve**
  • Oligo arthritis in lower extremities
  • *Post infectious arthritis:
  • post infectious illnesses not classically considered in the reactive arthritis group, such as GAS or viruses
  • Transient pain and joint swelling lasting < 6wks
  • HLA -ve**
  • Pattern of arthritis depends on organism
  • Diagnosis of exclusion
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61
Q

What’s the difference between septic arthritis and post infectious/Reactive?

A

Post infectious and reactive are STERILE inflammatory reactions.

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62
Q

What organisms can cause post-infectious arthritis? (9)

A 
HSV
VZV
EBV
HIV
CMV
HepB
Rubella
Adeno
Parvo B19
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63
Q

When does reactive arthritis occur?

A

2-4 wks post infection

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64
Q

How to differentiates post streptococcal arthritis from rheumatic fever arthritis, knowing both cased by GAS?

A
  • Poststreptococcal arthritis is oligoarticular, affecting lower extremities, symptoms persist for months
  • Rheumatic fever arthritis is polyarticular and migratory of brief duration.
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65
Q

What’s transient senovitis?

A
  • form of post infectious arthritis affecting the hip
  • Usually post URTI
  • Usually affects boy 3-10
  • Acute onset of pain in the hip w radiation to the groin
  • Normal Inflammatory markers and normal WBC
  • US shows effusion
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66
Q

4 yr boy w acute hip pain radiating to the groin 5 days post URTI. Labs supporting transient senovitis. US shows some fluid in the hip. What to do?

A

Still need to aspirate joint fluid to make sure no septic hip

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67
Q

Pt w confirmed post infectious GAS arthritis. What to do?

A

Need penicillin prophylaxis for at least 1 yr

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68
Q

What’s the strongest RF for SLE? Most common ages?

A
  • being female

- During puberty 11-12 yr. Rare before 5

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69
Q

What are some dermatological rashes seen in SLE? (6)

A
  • Malar rash
  • Discoid rash: hyperkeratosis, follicular plugging, and infiltration of mononuclear cells into the dermal-epidermal junction.
  • UV photosensitivity
  • livedo reticularis
  • Oral ulcers
  • Alopecia
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70
Q

What’s the hallmark pathogenesis of SLE?

A

Generation of autoAbs against self: against nucliec acid

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71
Q

What are some of the manifestations of SLE?

A
  • Renal: HTN, Nephrotic syndrome, hematuria, protinuria, renal failure
  • Derm: Discoid, Malar, UV sensitivities, Alopacia, Oral ulcers
  • Cardiac: Pericarditis, myocarditis, conductive abnormalities
  • Constitutional: fatigue, fever, wt loss, anorexia
  • Neuro: seizure, coma, stroke, cerebritis, cognitive impairment
  • Pulm: PE, interstitial lung, pulm hemorrhage
  • Ocular. NO UVEITIS. Scleritis, episcleritis, dry eyes, optic neuritis
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72
Q

Criteria for SLE? (11)

A

4/11

  • Malar Rash
  • Discoid Rash
  • Photosensitivity
  • Oral ulcers
  • Arthritis: >=2joints
  • Serositis
  • Renal manifestations: on biopsy or persistent proteinuria, or renal cast
  • Seizure of psychosis
  • Hematologic manifestations (Leukopenia, lymphopenia, thrombocytopenia, hemolytic anemia)
  • Immunologic abnormalities
  • ANA
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73
Q

What are the immunologic criteria for SLE? (6)

A 
\+ve ANA
\+ve Anti dsDNA
\+ve Anti Smith
\+ve Anti phospholipid (lupus anticoagulant- higher risk of clotting, false positive RPR, high anti cardio lipin, positive anti glycoprotein B ab)
Low complements (c3, c4 or CH50)
\+ve Direct Coombs
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74
Q

Utility of ANA in SLE?

A

Very high sensitivity

Poor specificity 50%

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75
Q

Which Ab testings are very specific for SLE but not sensitive? (2)

A

Anti-Smith (does not correlate w disease activity)

Anti-dsDNA (correlated w disease activity)**

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76
Q

Which lupus antibodies increase the risk of neonatal lupus? (2)

A

Anti Ro

Anti La

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77
Q

What could differentiate Drug induced lupus from SLE? (4)

A

-Antihistone ab are only positive in drug induced
May be present in SLE
-Hepatitis more common in drug induced
-Drug induced less likely to have anti dsDNA
-Symptoms resolve with withdrawal of medication (but symptoms take another year to resolve)

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78
Q

Tx of SLE ? (2)

A

Steroids

Hydroxychloroquine (prevents flares) (safe in preg)

79
Q

Pt w SLE on Hydroxychloroquine. what potential SE and what should she be screened for? (2)

A

Retinal pigmental and color changes

Need ophtho exam q6-12months

80
Q

SE of steroids? (9)

A 
growth disturbance, 
weight gain
striae
acne
hyperglycemia
hypertension
cataracts
avascular necrosis
osteoporosis
81
Q

What occular complications are pt w SLE at risk for? (4)

A

Glaucoma
Cataracts
Retinal detachment
blindness

82
Q

When does neonatal SLE rash appear?

A

within the first 6 wks of life
Stays 3-4 months
comes on post UV light exposure.

83
Q

what’s the mode of inheritance of Familial Mediterranean fever ? What’s the gene?

A

Aut Res

MEFV gene

84
Q

What characterizes FMF? (4)

A
  • 1-3 days of recurrent fever
  • Serositis (commonly pleuritic chest pain or severe abdominal pain)
  • Arthritis (tend to be Oligo, can be poly)
  • Errysipelis rash

90% <20 yr
Other features include scrotal pain in males from tunica vaginals inflammation.
May have elevation of WBC and inflammatory markers

85
Q

What’s the feature of serositis in FMF? (2)

Feature of arthritis? (2)

A
  • Chest pain tends to be unilateral
  • Abdo pain: generalized or can occur in one quadrant. Can be peritonitic in nature.

Arthritis:
neutrophil rich
nondestructive or erosive.

86
Q

What’s the hallmark cutaneous feature of FMF? (1)

Where can it be seen? (2)

A

erysipeloid erythematous rash that overlies the ankle or dorsum of the foot

87
Q

Tx for FMF? How to confirm Diagnosis?

A

Daily colchicine: decreases frequency, intensity, and duration

Safe in preg and BF
Diagnosis Confirmed by rectal or renal biopsy

88
Q

One possible complication of FMF? Presentation of this complication?

A

Systemic AA amyloidosis

Protinuria

89
Q

SE of Cholchicine? (4)

A
  • Diarrhea
  • GI complaints
  • Lactose intolerance
  • Transaminitis
90
Q

Possible triggers for FMF? (3)

A
  • Stress
  • Menses
  • Exercise
91
Q

RF for development of AA Amyloidosis in FMF? (4)

A

Male
Non-compliance w colchicine
FmHx
Country of origin (middle east)

92
Q

SE of infliximab? (8)

A 
Hepatotoxicity
Infusion reaction
Malignancy
Reactivation of TB
Drug induced lupus
Demylinating disease
Injection site reaction
93
Q

How long does the fever last in PFAPA? How long are the intervals btw fever?

A

3-7 days, longer than in FMF, regardless of antipyretic or Abx
intervals btw: 3-6 wks…very regular

94
Q

Tx of PFAPA? (3)

A
  • *Steroids have dramatic effect; one dose of prednisone 0.6-2.0 mg/kg once
  • Does not prevent recurrance
  • Shortens the interval btw episodes

**Cimetidine prevents recurrence in 30%

**May have complete resolution w tonsilectomy in some pt

**Only 30% respond to colchicine

95
Q

What’s the most common recurrent fever syndrome in children?

A

Periodic Fever Aphthous stomatitis pharyngitis and adenitis

The pharyngitis is negative

96
Q

Which age group does PFAPA affect?

A

2-5 yrs

Frequency and intensity of episodes decrease w time

97
Q

Is the pharyngitis exudative or transidative?

A

exudative appearing

98
Q

lab findings in PFAPA
WBC
Inflammatory markers

A

Mild elevation of WBC

Elevated inflammatory markers

99
Q

Is the presence of Arthritis necessary in sJIA?

A

No, may occur weeks to months later.

100
Q

Which vessels does Kawasaki disease tend to preferentially affect?

A

Coronary artery

KD is the leading cause of acquired heart disease in developed countries

101
Q

Who develops coronary aneurysm in KD?

A

25% of untreated KD

<5% of IVIG treated KD

102
Q

Which age group does KD affect?

A

more boys affected
75% <5yr
Mean age of 3
More pt of asian descent

Ages <6 months and >5 yrs are highest risk for coronary artery disease

103
Q

Predictors of poor outcome in KD with development of coronary artery disease ? (5)

A

These don’t correlate w non-Japanese pop.

  • Young age (<6 months or > 5 yrs)
  • Male
  • Persistent fever
  • Poor response to IVIG
  • Labs (neutrophilia, thrombocytopenia, transaminitis, hyponatremia, hypoalbuminemia, high CRP…)
104
Q

Phases of arteritis in KD? (3)

A
  1. Early in first 2 wks: Neutrophilic necrotizing
  2. Chronic: lymphocytic and eosiniphilc infiltrates; takes a while
  3. Myofibroblast in smooth muscle cell creating stenosis
105
Q

Diagnostic criteria for KD?

A

**Fever for >=5 days (may be 4)
**4/5 of the following
-Nonexudative conjunctivitis bilateral
-Cervical lumphodenopathy >1.5 cm, usually unilateral
-Changed in lips and oral cavity: cracked lips, strawberry tongue, injection of oral mucosa
-Polymorphous rash (i.e. can be anything: (maculopapular,
erythema multiforme, or scarlatiniform)
-Extremities: edema and erythema of the hands, feet

106
Q

Diagnosis of SLE?

A

SOAP BRAIN MD

  • Serositis
  • Oral ulcers
  • Arthritis
  • Photosensitivity
  • Blood : all low
  • Renal : protein
  • ANA
  • Immunologic
  • Neuro: psychosis, seizure..
  • Malar rash
  • Discoid rash
107
Q

What are the lab findings associated with KD? (10)

A
  • Anemia
  • Thrombocytosis
  • Neutrophilia
  • Sterile pyuria
  • Elevated transaminitis
  • Elevated GGT
  • Hyponatremia
  • Hypoalbuminemia
  • Pleocytosis in CSF
  • Elevated CRP/ESR
108
Q

When would desquamation of the perineum be seen in KD? When would it be seen in hand/feet?

A

Perineum: in the acute phase

Hands/feet: after 2-3 wks

109
Q

T or F

Myocarditis occurs in most pts with KD

A

True

Manifests as tachy disproportionate to the fever

110
Q

Cardiac manifestations of KD? (6)

A 
Coronary artery aneurism
Myocarditis
Pericarditis
Valve regurgitation
CHF
Raynaud phenomenon
111
Q

Phases of KD? (3)

A
  1. Acute: 1-2 wks
  2. Subacute: 3 wks: Thrombocytosis occur. Highest risk of death in pt who developed coronary artery disease
  3. convalescent: 6-8 wks post
112
Q

When should Echo be done in pts with KD?

A
  • At diagnosis
  • 1-2 wks post
  • If normal, repeat again at 4-6 wks
  • If abnormal need closer f/u: at least x2/wk, until stability and no progression of the size of coronary abnormalities
113
Q

Treatment of KD (4)

A
  1. IVIG high dose 2g/kg, ASAP wi 10 days of illness

**Can still give if post 10 days if missed KD, if child still has fever, significant elevation of inflammatory markers, or if abnormal echo

  1. High dose ASA 80-100mg/kg/day q6 until pt is afebrile for 48 hrs
    * *No evidence it reduces CAA
  2. low dose ASA 3-5mg/Kg/Day if no evidence of CAA until 4-6 wks post illness

Need anticoagulation if has CAA
Stay away from NSAIDs

  1. Need Cardio assessment at 1 yr (can include stress test, lipid profile and Echo)
114
Q

What’s the utility of ESR in KD?

A

It’s an inflammatory markers, HOWEVER, post IVIG it is no longer reliable as it goes up.

115
Q

When can IVIG be repeated in KD?

A

If fever persists 36hrs post last IVIG dose.

116
Q

Tx of IVIG resistent KD? (2)

A
  • Can consider second dose of IVIG

- Can consider high dose pulse steroids IV for 3 days (with or without taper)

117
Q

When can live vaccine be given post IVIG?

A

3-11 months post

Non live vaccines are ok to be given

118
Q

Pt w hx of KD. Has some dilatation of the coronaries only. Need for ASA?

A

Reasonable to continue low dose post 6-8 wks

119
Q

Contraindications to IVIG?

A

Selective IgA def.

120
Q

Who is at risk for immune hemolysis from IVIG? (2)

A

Females w blood group A or B

Previous immune hemolysis

121
Q

Patient with concerns of IVIG allergy . What to do? (2)

A

Slow rate

Pre medicate w anti histamine

122
Q

Reactions associated w IVIG (4)

A
  • Similar to many blood prodcuts
  • Aseptic meningitis
  • Hemolytic anemia
  • Headache
123
Q

What’s the characteristic muscle weakness in JDM?

A

Proximal muscle weakeness

124
Q

Peak age for JDM?

A

4-10 yr

There may me FmHx of autoimmune.

125
Q

Clinical manifestations of JDM? (9)

A
  • Rash (erythematous of facial malar area, Heliotrope rash of the eyelid )***
  • Insidious onset of weakeness
  • Dysphagia/Dysphonia
  • Fever
  • Arthritis/Arthralgia
  • Gottron papules
  • Muscle pain, tenderness, and atrophy
  • Calcinosis of muscles (40%)
  • Periungal capillary abnormalities***
126
Q

Diagnostic criteria for JDM? (5)

A

-Classic rash (Gottron, heliotrope rash of the eyelid)
Plus 3/4
-Weakness (symmetric and proximal)
-Muscle enzyme elevation (>= CK, AST, LD, Aldolase)
-EMG Changes
-Muscle biopsy (necrosis, and inflammation)

127
Q

What are the EMG findings in JDM? (2)

A

Early JDM might not show problems

  • Myopathies (Fibrilation and sharp waves)
  • Muscle necrosis (Decreased action potential amplitude and duration)
128
Q

What’s a heliotrope rash?

A

blue-violet discoloration of the eyelids that may be associated with periorbital edema.

129
Q 
Lab findings in JDM?
RF
dsDNA
ALT
ESR
ANA
A 
RF typically -ve, dsDNA -ve
CK earlier on is normal, and raises later
ALT is elevated
ESR normal
ANA +ve in >80%
130
Q

Treatment of JDM? (3)

A
  • Steroids are the mainstay
  • Methotrexate are adjuncts
  • IVIG as adjunct for severe disease
131
Q

Medications commonly cause drug induced lupus? (4)

A

Definite:

  • Minocycline
  • Hydralazine
  • Isoniazid
  • Infliximab

Probable:

  • Phenytoin
  • Carbamazapine
  • Sulfasalizine
  • Quinidine
  • Rifampin
  • Nitrofrantoin
  • Beta blocker
  • Penicillin
  • Valporate

minocycline, many anticonvulsants, sulfonamides, antiarrhythmic agents,

132
Q

Why is ANA a poor screening test for SLE?

A

20% of Healthy pt have +ve ANA
99% of pt w SLE have +ve ANA

Sensitivity: odd of the test being +ve when the the disease is present

**ANA has high sensitivity

Specificity: odds of the test being negative when the disease is absent

**ANA has poor specificity

133
Q

Which immunologic marker is associated w increased risk of Uveitis in JIA?

A

ANA

Uveitis tend to be asymptomatic

134
Q

What are the most complications of uveitis from Oligo JIA? (3)

A
  1. Cataracts
  2. synechiae
  3. Glaucoma

Uveitis tend to be asymptomatic otherwise w no symptoms of pain or photophobia

135
Q

Which GAS serotypes tend to be more associated with Rheumatic fever?

A

GAS M types (1, 3, 5, 6, 18, 29)

136
Q

What’s the most common cause of acquired heart disease in developing countries?

A

Rheumatic heart disease

137
Q

What’s the period between GAS pharyngitis and symptoms of rheumatic heart disease

A

~2-4 wks

138
Q

How to diagnose Rheumatic Fever?

A

Jones Criteria
Must have evidence of GAS and:
-2 Major OR
-1 major and 2 minor

139
Q

What are the major criteria for Rheumatic fever?

A 
JONES
Joint: Migratory Polyarthritis (75%)
O: Carditis
N: Nodules, subcutaneous  (Rare; usually along the extensor surfaces)
E: Erythema marginatum (Rare 1%)
S: Synenham chorea
140
Q

What are the minor criteria for Rheumatic fever?

A

CAFE PAL

  • C: CPR increased
  • A: Arthralgia
  • F: Fever
  • E: ESR elevated
  • P: prolonged PR
  • A:
  • L: leukocytosis
141
Q

How can the diagnosis of recurrent Rheumatic fever be made?

A

3 minor

Plus history of previous GAS infection

142
Q

Which particular physical exam finding ALONE would allow for diagnosis of Acute rheumatic fever?

A

Sydenham chorea

143
Q

What’s arthritis like in rheumatic fever?

A

Poly and migratory
Affects larger joints
Hot, red, swollen, and very tender (clothes bother)

**Pain is disproportionate to objective finding
Migratory pain
-Dramatic response to ASA
high neutrofils in the synovial fluid

144
Q

Patient is suspected of having sydenham chorea. How to ilicit? (4)

A
  • Milkmaid grip (irregular contractions and relaxations of the muscles of the fingers while squeezing the examiner’s fingers
  • spooning and pronation of the hands when the patient’s arms are extended
  • wormian darting movements of the tongue
  • Examination of handwriting to evaluate fine motor.
145
Q

What are the characteristics of erythema marginatum?

A
  • Erythematous macular rashes with centres that are not pruritic
  • Not on the face.
146
Q

Tx of rheumatic fever?

A
  • 10 days PO penicillin (or one dose IM)
  • If allergic, 10 days erythro or 5 days azithro, or clinda
  • Long term prophylaxis:

No carditis: for 5 years post or 21 yr of age, whichever is later.
Carditis, no valvular/residual disease: 10 yr or 21, whichever is later
Valvular: 10 yr or until 40, whichever is later, or lifelong.

147
Q

Patient w Rheumatic heart disease. Should they receive endocarditis prophylaxis?

A

Not per se, unless meeting other criteria for endocarditis prophylaxis. If so, and already on penicillin prophylaxis then need another type for endocarditis prophylaxis.

148
Q

What is complex regional pain syndrome (CRPS)? Age of onset?

A

Ongoing burning limb pain secondary to an injury , immobilization or another noxious event affecting the extremity.

CRPS1 aka reflex sympathetic dystrophy has NO nerve symptoms

  • Age of onset: 8-16
  • More girls
  • More lower extremities
149
Q

Features of Complex regional pain syndrome? (4)

A

Allodynia
Hyperalgesia
Swelling of distal extremities
Autonomic dysfunction (cyanosis, coolness, mottling)

150
Q

Consequences of untreated Complex regional pain syndrome? (3)

A
  • Bone demineralization
  • Muscle wasting
  • Contractures
151
Q

Treatment of Complex regional pain dystrophy? (3)

A
  • Aggressive physio 3-4/wk.
  • CBT
  • Can consider pain services including nerve block to control autonomic symptoms
152
Q 
Compare JIA and JuvAnkSpon w regards:
Gender predominance
Arthritis
Back symptoms
FmHx
ANA
HLA 27
Eye disease
A

Female predominance in JIA
Male predominance in JAS

Arthritis in JIA, not much in JAS

Back symptoms: JAS not JIA

FmHx present in JAS, not JIA

ANA positive in JIA, not JAS

HLA +ve in JAS, not JIA

Anterior Uveitis in JIA, not JAS

153
Q

Why may patients with ankylosing spondylitis have heel pain?

A

Due to enthesitis.

154
Q

Common features of psoriatic arthritis? (3)

A

Nail pitting
Dactylitis
Onycholysis

155
Q

Criteria for psoriatic arthritis? ()2

A
  1. arthritis and psoriasis
  2. Arthritis and at least 2 of the following: (1) dactylitis, (2) nail pitting or onycholysis, or (3) psoriasis in a 1st-degree relative.
156
Q

Arthritis pattern in JAS?

A

Oligoarticular
usually lower extrem and involves the hip
usually with enthesitis

157
Q

Who is at highest risk of uveitis in JIA? (5) How often should they be screened?

A
  • Age <6
  • Female
  • Oligo
  • ANA+ve
  • Duration of disease < 4yr
  • Screening q3m for above
  • If age =<6 but disease duration >4 yrs, then q6m
  • disease duration >7 yr then q12m
  • sJIA has low risk; screen q12 m

**highest risk within first 4 yrs of disease. Risk goes down if duration longer w no issues

158
Q 
oligo/Poly JIA
ANA +ve
Age onset =< 6yr
Disease duration >7 yr
How often to screen?
A

q12

159
Q 
oligo/Poly JIA
ANA +ve
Age onset =< 6yr
Disease duration 5 yr
How often to screen?
A

q6m

160
Q 
oligo/Poly JIA
ANA +ve
Age onset =< 6yr
Disease duration <4 yr
How often to screen?
A

q3m

161
Q 
oligo/Poly JIA
ANA +ve
Age onset >6yr
Disease duration =<4 yr
How often to screen?
A

q6m

162
Q 
oligo/Poly JIA
ANA +ve
Age onset >6yr
Disease duration >4 yr
How often to screen?
A

q12m

163
Q 
oligo/Poly JIA
ANA -ve
Age onset =<6yr
Disease duration =<4 yr
How often to screen?
A

q6m

164
Q 
oligo/Poly JIA
ANA -ve
Age onset =<6yr
Disease duration >=4 yr
How often to screen?
A

q12m

165
Q

Non infectious causes of Macrophage activating syndrome? (5)

A 
SLE
KD
sJIA
IBD
Malignancy
166
Q

Which renal manifestations are most observed in SLE? (4)

A

Proteinuria
Hematuria
Low extremity edema
HTN

167
Q

What’s ricket?

A

Disease of mineral deficiency of the bone occurring at the growth plate leading to retarded bone growth and bone age

Can only be found in growing children before fusion of the epiphysis

168
Q

Relationship between osteomalacia and ricket?

What’s osteoporosis?

A

All patients wtih rickets have osteomalacia
Not all patients with osteomalacia have rickets
These are issues with mineralization

Osteoparosis: problem with both mineralization and bone volume.

169
Q

The primary difference in labs between calcium deficient rickets and PO4 deficient ricket is what?

A

Usually in PO4 deficient ricket, Ca is normal.
It may be low in Ca deficient ricket.

There is usually secondary hyperparathyroidism in Ca associated rickets.

170
Q

What are the bone changes associated with ricket? (5)

A
  • Thickening of the growth plate.
  • Cupping (becomes concave) and fraying (losing its sharp -boarder) of the metaphysis
  • Widening of the physis
  • General softening of the bone allowing it to bend more easily

Classically: widening of the wrists and ankles

171
Q

Causes of Rickets? (4)

A

Vit D deficiency

  • Nutritional def
  • Malabsorption
  • Chronic kidney disease

Ca deficiency

  • Low intake
  • Diet
  • Prematurity (rickets of prematurity)
  • Malabsorption

PO4 deficiency

  • Prematurity
  • Aluminum containing antacids

Renal losses:

  • Mccune albright
  • Neurofibromatosis
  • Distal renal tubular acidosis
  • Fanconi syndrome
172
Q

What are the clinical features of rickets? (6)

A

Head:

  • Frontal bossing**
  • Craniotabes**
  • Delayed frontal closure
  • Craniosynostosis
  • Delayed dentition

Extremities:

  • Widening of wrist and ankle
  • Valgus and varus deformities
  • Anterior bowing of tibia and femur
  • Leg pain

Hypocalcemia:
Tetany
Seizure
Stridor due to laryngeal spasm

General:
FTT
Muscle weakness
Irritability

Rachitic rosary**
Harrison groove**
Scoliosis/kyphosis

173
Q

What’s Harrison groove? What causes it? and implications?

A
  • Pulling of the ribs inwards by the diaphragm during inspiration. All from the softening of the ribs
  • Rickets can
  • Leads to inefficient air movement: atalectasis and infections
174
Q

Which type of ricket may have low PTH?

A

hypo PO4 ricket

175
Q

Causes of Toe walking? (6)

A
  • Tethered cord
  • CP
  • Leg length discrepancies
  • Tight achilis tendon
  • Autism spectrum disorder.
  • Muscular dystrophies
176
Q

Foot Abnormality in Charcot marie tooth?

A

significant cavus foot.

177
Q

Psychosocial complications of JIA? (2)

A

Problems w school attendance

Problems w socialization

178
Q

Orthopedic complications of JIA? (3)

A

Leg length discrepancies
Flexion contractures (particularly of wrist and knee and hips)
Popliteal cysts

179
Q

What’s Reiter’s syndrome? (3)

A

Old term for triad of:
Postinfectious arthritis
Urethritis
Conjunctivitis

180
Q

What are two disease associated with complete Heart block in newborn? (2)

A

Sjogren

Neonatal SLE

181
Q

What’s Sjogren?

A

Chronic autoimmune inflammatory disorder
Lymphocytic infiltrate of exocrine glands (mainly lacrimal and salivary)
Rare in children
Affects middle aged women

Main manifestations: dry eyes and dry mouth

182
Q

What are the manifestations of Sjogren in children? (4)

A
  • Parotid gland enlargement
  • Parotitis
  • Sicca syndrome (dry mouth, painful mucosa, sensitivity to spicy foods, halitosis, widespread dental caries)
  • Polyarthritis

ANA +ve
Anti Ro
Anti La

183
Q

Labs in Sjogren? Tx

A

High amylase
Leukopenia
High ESR

Tx: Artifical tears, massage of parotids, Steroids, NSAIDs

184
Q

How do children w Ehler Danlos appear at birth?

A

Normal

185
Q

Manifestations of Ehler Danlos? (6)

A
  • Skin hyperelasticity
  • Joint hypermobility
  • Fragility of skin and blood vessels.
  • Delayed wound healing
  • Autonomic dysfunction (GI disturbance, orthostatic, chronic MSK pain)
  • Pulmonary complication (Pneumothorax, hemoptysis, tracheomegaly)
186
Q

Defect in Ehler Danlos?

A

Fibrillar Collagen

187
Q

What CNS abnormalities do children w Ehler Danlos have? (1)

A

increased incidence of Chiari malformation

188
Q

Signs of joint hypermobility? (5)

A
  • Dorsiflex 5th metacarophalyngeal joint >90
  • Oppose thumb to volar surface of ipsilateral forarm
  • Hyperextend elbow >10
  • Hyperextend knee >10
  • Hands flat on floor wo flexing knee
189
Q

Presentation of JPM? (6)

A 
Wight loss
Falling episodes
Raynaud
Abnormal PFT
Dyspnea
Cardiac abnormalities
190
Q

Which conditions has + HLA B 27?

A

Enthesitis related arthritis, majority.

191
Q 
oligo/Poly JIA
ANA -ve
Age onset >6yr
Disease duration anything
How often to screen?
A

q12m

192
Q

Features of Sydenham chorea? (7)

A

**Chorea, hypotonia and emotional liability

-jerky movements of face / extremities / trunk

  • absence during sleep
  • “wormian” tongue that darts about, halting / explosive speech [irregular tone],
  • pronation during arm raise,
  • milkmaids grip [constant contractions during sustained grip],
  • irregular / poor drawing and writing,
  • difficulties with fine motor tasks
  • emotional lability
193
Q

What’s PANDAS? criteria? (5)

A

Pediatric autoimmune neuropsychiatric disorder associated with group A streptococci.

  1. OCD and/or tic disorder (Tourette disorder, chronic motor or vocal tic disorder)
  2. Pediatric onset (between three years and onset of puberty)
  3. Abrupt onset and episodic course of symptoms
  4. Temporal relation between GAS infection and onset and/or exacerbation
  5. Neurologic abnormalities, such as motoric hyperactivity, choreiform movements, or tics during exacerbations

MSK & Rheum (2 decks)

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