Mohammad-Rheum-Core Flashcards
(193 cards)
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
2
Q
Which age does HSP affect?
A
3-10yrs
More males than females
More in Asian and white ethnicity
Many follow URTI
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.
4
Q
What are the manifestations of HSP? (4)
A
- Palpable purpra/patechia
- MSK (arthritis/arthrlagia in 75%)
- Abdo pain (80%)
- Renal (50%)
5
Q
What’s the pattern of arthritis in HSP?
A
Oligoarticular
Self limiting
Mostly lower extremities
2 wks to go away (may recur)
6
Q
What are the GI manifestations of HSP (5)
A
N/V Diarrhea Pain Paralytic ileus Intussusception, possibly malena
7
Q
Renal manifestations of HSP? (4)
A
Microscopic hematuria
HTN
Nephrotic syndrome
Proteinuria
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)
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)**
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
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**
12
Q
Tx of HSP? (2)
A
- Supportive
- May consider Steroids for significant GI involvement or lifethreatnening (1/Kg for 1-2 wks)
13
Q
Utility of Prednison in HSP? (3)
A
Reduces abdominal and joint pain
Does not alter disease course
Does not prevent renal involvement
14
Q
Complications of HSP? (3)
A
Renal disease (1-2%)
Intestinal perforation
Recurrence of HSP
15
Q
What rheum conditions may be associated with oral ulcers? (4)
A
- SLE
- Bachet
- HSV
- PFAPA
16
Q
Which conditions causes Malar Rash? (4)
A
- SLE (spraing nasolabial folds)
- JDM
- KD
- Parvo B19 (fifth disease)
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
18
Q
MOE of NSAIDs?
A
Inhibiting COX= prostaglandin inhibitor
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.
20
Q
Most common rheumatic disease of childhood?
A
JIA
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
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
23
Q
What’s Quotidian fever?
A
Fever that rises to at least 39 once a day and goes back to normal between peaks.
24
Q
What is serositis? (3)
A
pericarditis, pleuritis, or peritonitis, or some combination of the 3.
25
What's Oligoarthritis?
Persistent oligo?
Extended Oligo?
-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
26
Criteria for Polyarthritis RF negative?
>=5 joints in 1st 6 months
| RF negative
27
Criteria for polyarthritis RF +ve disease?
>=5 joints in 1st 6 months
>= 2 RF tests positive 3 months apart
RF positive during 1st 6 months
28
What's Enthesis?
Tenderness at the tendon insertion site, or fascia to bone, joint capsule.
29
Peak ages for JIA?
2-4 yrs for oligo
1-5 for sJIA
Bimodal for polyJIA: 2-4 and 10-14
More females in Oligo and Poly
30
T or F
| All JIA have inflammatory synovitis
T
Characterized by lymphocyte predominance
31
What is arthritis ? (4)
Swelling or effusion OR the presence of >2 of the following: Limited ROM, pain on ROM, No erythema
32
Why may children w JIA have discrepant limb length?
Arthritis accelerates bone growth initially and then stimulated rapid and premature closure of growth plates.
33
Which joint tend to be most affected in Oligo JIA?
| Prevalence of uveitis? Prevalance of ANA +ve?
Knee
Uveitis: 30%
ANA +ve in 60%
34
Main treatment for Oligo JIA? (2)
NSIADs and intrarticular steroids
35
How does RF status affect prognosis in Poly?
RF +ve is more aggressive
36
which type of JIA has cervical spine involvement? Risks w cervical spine involvement?
Usually in Poly JIA
| Risk atlantoaxial subluxation and neurologic sequelae
37
Features of sJIA rash? (4)
salmon-colored, macular, and nonpruritic.
Evanescent: Migratory lasting <1hr
Can be heat provoked
Koebner phenomenon (brough on by superficial traum)
38
Rash brought on by superficial trauma. What to call?
Koebner phenomenon
39
What's a rare lethal complication of sJIA?
MAS
40
Manifestation of MAS?
- acute onset of high spiking fevers, nonremitting
- lymphadenopathy
- hepatosplenomegaly,
- CNS dysfunction (headache, disorientation, lethargy, seizure, coma)
-Macrophages hemophagocytosis
41
Lab findings in MAS? (10)
```
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
```
42
What can help distinguish MAS from flares of other systemic diseases? (1)
In MAS, ESR falls because of hypofibrigenemia and hepatic dysfunction. This does not happen in flares of other systemic illnesses
43
Tx of MAS? (3)
EMERGENCY:
High dose Methylpred
Cyclosporin
Anakinra (anti IL1)
44
What are some of the complications associated w JIA? (4)
- Osteopenia (the longer the disease the worse it gets)
- Flexure contracture
- Leg length discrepancies
- Chronic uveitis
45
What organisms tend to cause reactive arthritis? (7)
Bacteria: Shigella, Salmonella, Yersinia, Chlamydia, or meningococcus, Campylobacter.
Ecoli sometimes
46
What's pattern of arthritis in Acute Rheumatic fever?
Migratory polyarthritis
47
What's the pattern of arthritis in lyme disease?
Oligoarthritis
48
What suggests septic arthritis? (4)
Fever
Swelling
Erythema
Hot joint
49
Characteristics of growing pain? (3)
Child 4-12 yr
Leg pain in the evenings
Normal investigations
No morning symptoms.
50
What's ANA like in sJIA?
Rarely elevated
51
Why must RF be repeated twice in poly JIA?
Can be false +ve from viral infection
52
What's the dose of NSAIDs for JIA? Who usually responds?
Ibuprofen:
40mg/Kg/Day divided TID
Naproxen:
15mg/Kg/Day divided BID
Oligo usually responds
Poly and sJIA don't
53
What are potential SE of NSAIDs? (4)
Renal toxicity
Hepato toxicity
Risk of pseudoporphyria
Gastritis
54
SE of Methotrexate? (4)
N/V
oral ulcerations,
hepatic toxicity
Immunosuppresion
55
Indications for intraarticular joint injection in JIA? (4)
No response to steroids after 4-6wks
Joint contracture
leg length discrepancies
Functional limitation
56
What should pt with JIA routinely be screened for?
Asymptomatic uveitis
57
Who is at greater risk for developing chronic uveitis? (4)
Oligo
Girls
Disease <6 yr
ANA +ve
58
Predictors of severe JIA? (6)
- RF +ve
- Rheumatoid nodules
- Anti CCP
- Younger age at onset
- Large number of affected joints
- Disease affecting hand or hip
59
Characteristics of inflammatory bone pain? (5)
- Pain at night w morning stiffness
- No improvement w rest
- Improvement w exercise
- Insidious onset
- Good response to NSAIDs
60
What's the difference between reactive arthritis and post-infectious arthritis?
* *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
61
What's the difference between septic arthritis and post infectious/Reactive?
Post infectious and reactive are STERILE inflammatory reactions.
62
What organisms can cause post-infectious arthritis? (9)
```
HSV
VZV
EBV
HIV
CMV
HepB
Rubella
Adeno
Parvo B19
```
63
When does reactive arthritis occur?
2-4 wks post infection
64
How to differentiates post streptococcal arthritis from rheumatic fever arthritis, knowing both cased by GAS?
- Poststreptococcal arthritis is oligoarticular, affecting lower extremities, symptoms persist for months
- Rheumatic fever arthritis is polyarticular and migratory of brief duration.
65
What's transient senovitis?
- 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
66
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?
Still need to aspirate joint fluid to make sure no septic hip
67
Pt w confirmed post infectious GAS arthritis. What to do?
Need penicillin prophylaxis for at least 1 yr
68
What's the strongest RF for SLE? Most common ages?
- being female
| - During puberty 11-12 yr. Rare before 5
69
What are some dermatological rashes seen in SLE? (6)
- Malar rash
- Discoid rash: hyperkeratosis, follicular plugging, and infiltration of mononuclear cells into the dermal-epidermal junction.
- UV photosensitivity
- livedo reticularis
- Oral ulcers
- Alopecia
70
What's the hallmark pathogenesis of SLE?
Generation of autoAbs against self: against nucliec acid
71
What are some of the manifestations of SLE?
- 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
72
Criteria for SLE? (11)
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
73
What are the immunologic criteria for SLE? (6)
```
+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
```
74
Utility of ANA in SLE?
Very high sensitivity
| Poor specificity 50%
75
Which Ab testings are very specific for SLE but not sensitive? (2)
Anti-Smith (does not correlate w disease activity)
| Anti-dsDNA (correlated w disease activity)**
76
Which lupus antibodies increase the risk of neonatal lupus? (2)
Anti Ro
| Anti La
77
What could differentiate Drug induced lupus from SLE? (4)
-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)
78
Tx of SLE ? (2)
Steroids
| Hydroxychloroquine (prevents flares) (safe in preg)
79
Pt w SLE on Hydroxychloroquine. what potential SE and what should she be screened for? (2)
Retinal pigmental and color changes
| Need ophtho exam q6-12months
80
SE of steroids? (9)
```
growth disturbance,
weight gain
striae
acne
hyperglycemia
hypertension
cataracts
avascular necrosis
osteoporosis
```
81
What occular complications are pt w SLE at risk for? (4)
Glaucoma
Cataracts
Retinal detachment
blindness
82
When does neonatal SLE rash appear?
within the first 6 wks of life
Stays 3-4 months
comes on post UV light exposure.
83
what's the mode of inheritance of Familial Mediterranean fever ? What's the gene?
Aut Res
| MEFV gene
84
What characterizes FMF? (4)
- 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
What's the feature of serositis in FMF? (2)
| Feature of arthritis? (2)
- 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
What's the hallmark cutaneous feature of FMF? (1)
| Where can it be seen? (2)
erysipeloid erythematous rash that overlies the ankle or dorsum of the foot
87
Tx for FMF? How to confirm Diagnosis?
Daily colchicine: decreases frequency, intensity, and duration
Safe in preg and BF
Diagnosis Confirmed by rectal or renal biopsy
88
One possible complication of FMF? Presentation of this complication?
Systemic AA amyloidosis
Protinuria
89
SE of Cholchicine? (4)
- Diarrhea
- GI complaints
- Lactose intolerance
- Transaminitis
90
Possible triggers for FMF? (3)
- Stress
- Menses
- Exercise
91
RF for development of AA Amyloidosis in FMF? (4)
Male
Non-compliance w colchicine
FmHx
Country of origin (middle east)
92
SE of infliximab? (8)
```
Hepatotoxicity
Infusion reaction
Malignancy
Reactivation of TB
Drug induced lupus
Demylinating disease
Injection site reaction
```
93
How long does the fever last in PFAPA? How long are the intervals btw fever?
3-7 days, longer than in FMF, regardless of antipyretic or Abx
intervals btw: 3-6 wks...very regular
94
Tx of PFAPA? (3)
* *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
What's the most common recurrent fever syndrome in children?
Periodic Fever Aphthous stomatitis pharyngitis and adenitis
The pharyngitis is negative
96
Which age group does PFAPA affect?
2-5 yrs
Frequency and intensity of episodes decrease w time
97
Is the pharyngitis exudative or transidative?
exudative appearing
98
lab findings in PFAPA
WBC
Inflammatory markers
Mild elevation of WBC
| Elevated inflammatory markers
99
Is the presence of Arthritis necessary in sJIA?
No, may occur weeks to months later.
100
Which vessels does Kawasaki disease tend to preferentially affect?
Coronary artery
KD is the leading cause of acquired heart disease in developed countries
101
Who develops coronary aneurysm in KD?
25% of untreated KD
| <5% of IVIG treated KD
102
Which age group does KD affect?
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
Predictors of poor outcome in KD with development of coronary artery disease ? (5)
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
Phases of arteritis in KD? (3)
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
Diagnostic criteria for KD?
**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
Diagnosis of SLE?
SOAP BRAIN MD
- Serositis
- Oral ulcers
- Arthritis
- Photosensitivity
- Blood : all low
- Renal : protein
- ANA
- Immunologic
- Neuro: psychosis, seizure..
- Malar rash
- Discoid rash
107
What are the lab findings associated with KD? (10)
- Anemia
- Thrombocytosis
- Neutrophilia
- Sterile pyuria
- Elevated transaminitis
- Elevated GGT
- Hyponatremia
- Hypoalbuminemia
- Pleocytosis in CSF
- Elevated CRP/ESR
108
When would desquamation of the perineum be seen in KD? When would it be seen in hand/feet?
Perineum: in the acute phase
| Hands/feet: after 2-3 wks
109
T or F
| Myocarditis occurs in most pts with KD
True
| Manifests as tachy disproportionate to the fever
110
Cardiac manifestations of KD? (6)
```
Coronary artery aneurism
Myocarditis
Pericarditis
Valve regurgitation
CHF
Raynaud phenomenon
```
111
Phases of KD? (3)
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
When should Echo be done in pts with KD?
- 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
Treatment of KD (4)
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
2. High dose ASA 80-100mg/kg/day q6 until pt is afebrile for 48 hrs
* *No evidence it reduces CAA
3. 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
4. Need Cardio assessment at 1 yr (can include stress test, lipid profile and Echo)
114
What's the utility of ESR in KD?
It's an inflammatory markers, HOWEVER, post IVIG it is no longer reliable as it goes up.
115
When can IVIG be repeated in KD?
If fever persists 36hrs post last IVIG dose.
116
Tx of IVIG resistent KD? (2)
- Can consider second dose of IVIG
| - Can consider high dose pulse steroids IV for 3 days (with or without taper)
117
When can live vaccine be given post IVIG?
3-11 months post
Non live vaccines are ok to be given
118
Pt w hx of KD. Has some dilatation of the coronaries only. Need for ASA?
Reasonable to continue low dose post 6-8 wks
119
Contraindications to IVIG?
Selective IgA def.
120
Who is at risk for immune hemolysis from IVIG? (2)
Females w blood group A or B
| Previous immune hemolysis
121
Patient with concerns of IVIG allergy . What to do? (2)
Slow rate
| Pre medicate w anti histamine
122
Reactions associated w IVIG (4)
- Similar to many blood prodcuts
- Aseptic meningitis
- Hemolytic anemia
- Headache
123
What's the characteristic muscle weakness in JDM?
Proximal muscle weakeness
124
Peak age for JDM?
4-10 yr
| There may me FmHx of autoimmune.
125
Clinical manifestations of JDM? (9)
- 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
Diagnostic criteria for JDM? (5)
-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
What are the EMG findings in JDM? (2)
Early JDM might not show problems
- Myopathies (Fibrilation and sharp waves)
- Muscle necrosis (Decreased action potential amplitude and duration)
128
What's a heliotrope rash?
blue-violet discoloration of the eyelids that may be associated with periorbital edema.
129
```
Lab findings in JDM?
RF
dsDNA
ALT
ESR
ANA
```
```
RF typically -ve, dsDNA -ve
CK earlier on is normal, and raises later
ALT is elevated
ESR normal
ANA +ve in >80%
```
130
Treatment of JDM? (3)
- Steroids are the mainstay
- Methotrexate are adjuncts
- IVIG as adjunct for severe disease
131
Medications commonly cause drug induced lupus? (4)
Definite:
- Minocycline
- Hydralazine
- Isoniazid
- Infliximab
Probable:
- Phenytoin
- Carbamazapine
- Sulfasalizine
- Quinidine
- Rifampin
- Nitrofrantoin
- Beta blocker
- Penicillin
- Valporate
minocycline, many anticonvulsants, sulfonamides, antiarrhythmic agents,
132
Why is ANA a poor screening test for SLE?
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
Which immunologic marker is associated w increased risk of Uveitis in JIA?
ANA
Uveitis tend to be asymptomatic
134
What are the most complications of uveitis from Oligo JIA? (3)
1. Cataracts
2. synechiae
3. Glaucoma
Uveitis tend to be asymptomatic otherwise w no symptoms of pain or photophobia
135
Which GAS serotypes tend to be more associated with Rheumatic fever?
GAS M types (1, 3, 5, 6, 18, 29)
136
What's the most common cause of acquired heart disease in developing countries?
Rheumatic heart disease
137
What's the period between GAS pharyngitis and symptoms of rheumatic heart disease
~2-4 wks
138
How to diagnose Rheumatic Fever?
Jones Criteria
Must have evidence of GAS and:
-2 Major OR
-1 major and 2 minor
139
What are the major criteria for Rheumatic fever?
```
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
What are the minor criteria for Rheumatic fever?
CAFE PAL
- C: CPR increased
- A: Arthralgia
- F: Fever
- E: ESR elevated
- P: prolonged PR
- A:
- L: leukocytosis
141
How can the diagnosis of recurrent Rheumatic fever be made?
3 minor
| Plus history of previous GAS infection
142
Which particular physical exam finding ALONE would allow for diagnosis of Acute rheumatic fever?
Sydenham chorea
143
What's arthritis like in rheumatic fever?
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
Patient is suspected of having sydenham chorea. How to ilicit? (4)
- 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
What are the characteristics of erythema marginatum?
- Erythematous macular rashes with centres that are not pruritic
- Not on the face.
146
Tx of rheumatic fever?
- 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
Patient w Rheumatic heart disease. Should they receive endocarditis prophylaxis?
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
What is complex regional pain syndrome (CRPS)? Age of onset?
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
Features of Complex regional pain syndrome? (4)
Allodynia
Hyperalgesia
Swelling of distal extremities
Autonomic dysfunction (cyanosis, coolness, mottling)
150
Consequences of untreated Complex regional pain syndrome? (3)
- Bone demineralization
- Muscle wasting
- Contractures
151
Treatment of Complex regional pain dystrophy? (3)
- Aggressive physio 3-4/wk.
- CBT
- Can consider pain services including nerve block to control autonomic symptoms
152
```
Compare JIA and JuvAnkSpon w regards:
Gender predominance
Arthritis
Back symptoms
FmHx
ANA
HLA 27
Eye disease
```
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
Why may patients with ankylosing spondylitis have heel pain?
Due to enthesitis.
154
Common features of psoriatic arthritis? (3)
Nail pitting
Dactylitis
Onycholysis
155
Criteria for psoriatic arthritis? ()2
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
Arthritis pattern in JAS?
Oligoarticular
usually lower extrem and involves the hip
usually with enthesitis
157
Who is at highest risk of uveitis in JIA? (5) How often should they be screened?
- 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
```
oligo/Poly JIA
ANA +ve
Age onset =< 6yr
Disease duration >7 yr
How often to screen?
```
q12
159
```
oligo/Poly JIA
ANA +ve
Age onset =< 6yr
Disease duration 5 yr
How often to screen?
```
q6m
160
```
oligo/Poly JIA
ANA +ve
Age onset =< 6yr
Disease duration <4 yr
How often to screen?
```
q3m
161
```
oligo/Poly JIA
ANA +ve
Age onset >6yr
Disease duration =<4 yr
How often to screen?
```
q6m
162
```
oligo/Poly JIA
ANA +ve
Age onset >6yr
Disease duration >4 yr
How often to screen?
```
q12m
163
```
oligo/Poly JIA
ANA -ve
Age onset =<6yr
Disease duration =<4 yr
How often to screen?
```
q6m
164
```
oligo/Poly JIA
ANA -ve
Age onset =<6yr
Disease duration >=4 yr
How often to screen?
```
q12m
165
Non infectious causes of Macrophage activating syndrome? (5)
```
SLE
KD
sJIA
IBD
Malignancy
```
166
Which renal manifestations are most observed in SLE? (4)
Proteinuria
Hematuria
Low extremity edema
HTN
167
What's ricket?
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
Relationship between osteomalacia and ricket?
| What's osteoporosis?
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
The primary difference in labs between calcium deficient rickets and PO4 deficient ricket is what?
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
What are the bone changes associated with ricket? (5)
- 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
Causes of Rickets? (4)
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
What are the clinical features of rickets? (6)
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
What's Harrison groove? What causes it? and implications?
- 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
Which type of ricket may have low PTH?
hypo PO4 ricket
175
Causes of Toe walking? (6)
- Tethered cord
- CP
- Leg length discrepancies
- Tight achilis tendon
- Autism spectrum disorder.
- Muscular dystrophies
176
Foot Abnormality in Charcot marie tooth?
significant cavus foot.
177
Psychosocial complications of JIA? (2)
Problems w school attendance
| Problems w socialization
178
Orthopedic complications of JIA? (3)
Leg length discrepancies
Flexion contractures (particularly of wrist and knee and hips)
Popliteal cysts
179
What's Reiter's syndrome? (3)
Old term for triad of:
Postinfectious arthritis
Urethritis
Conjunctivitis
180
What are two disease associated with complete Heart block in newborn? (2)
Sjogren
| Neonatal SLE
181
What's Sjogren?
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
What are the manifestations of Sjogren in children? (4)
- 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
Labs in Sjogren? Tx
High amylase
Leukopenia
High ESR
Tx: Artifical tears, massage of parotids, Steroids, NSAIDs
184
How do children w Ehler Danlos appear at birth?
Normal
185
Manifestations of Ehler Danlos? (6)
- 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
Defect in Ehler Danlos?
Fibrillar Collagen
187
What CNS abnormalities do children w Ehler Danlos have? (1)
increased incidence of Chiari malformation
188
Signs of joint hypermobility? (5)
- 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
Presentation of JPM? (6)
```
Wight loss
Falling episodes
Raynaud
Abnormal PFT
Dyspnea
Cardiac abnormalities
```
190
Which conditions has + HLA B 27?
Enthesitis related arthritis, majority.
191
```
oligo/Poly JIA
ANA -ve
Age onset >6yr
Disease duration anything
How often to screen?
```
q12m
192
Features of Sydenham chorea? (7)
**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
What's PANDAS? criteria? (5)
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
