deck 2 Flashcards
(78 cards)
1
Q
Lab features of APS?
A
- low platelets
- hemolytic anemia
- antibodies to prothrombin, annexin, other phospholipid
- false positive VDRL
2
Q
Treatment of APS?
A
primary - treat as disorder of coagulation
secondary - treat underlying disorder, using steroids
anticoagulation - may need with heparin initially
consider ASA
sometimes rituximab to go against Ab
3
Q
Which of the following is not a large vessel vasculitis?
a) Takayasu
b) Giant cell arteritis
c) polyarteritis nodosa
A
c) polyarteritis nodosa is a medium vessel vasculitis
Takayasu and Gian cell are large vessel vasculitis
Kawasaki is the other medium vessel vasculitis
4
Q
Name 5 small vessel vasculitis
A
- Churg-Strauss
- microscopic polyangitis
- HSP
- Cryoglobulinemia
- urticarial vasculitis (hypocomplement)
5
Q
Other causes of vasculitis other than those above
A
Behcet, Cogan, primary CNS, secondary to Drugs, infection (hepB, parvo), cancer
6
Q
Name the 3 ANCA associated vasculitis
A
- Wegener granulomatosis (cANCA)
- Microscopic polyangiitis (p-ANCA) (rare in childhood)
- Churg-Strauss
7
Q
Clinical features of vasculitis (think broad) and diagnostic investigations
A
- end organ damage (eyes, skin, heart, lungs, kidneys, CNS)
- triggers/underlying disease
- immune serology - ANA, ANCA
- tissue biopsy
- angiography /MRI
8
Q
Large vessel vasculitis involving the aorta and its branches
A
Takayasu arteritis
treatment: steroids, plus second line
9
Q
Diagnostic criteria for Kawasaki
A
conjunctivities - bilateral non exudative
rash - polymorphous exanthem
adenopathy - cervical, frequently unilateral (>1.5 cm)
extremity changes (edema/erythema) or perineal area)
mucosal changes (lips, oral cavitiy)
(plus fever for 5 days) plus 4 or more of the criteria
10
Q
Diagnosis of atypical KD?
A
- Other ways to make diagnosis of KD:
a) In presence of fever and coronary artery involvement on echo, <4/5 criteria sufficient
b) Incomplete KD if ≥ 5 days of fever with 2 or 3 features (common in infants, who are at higher risk of coronary artery involvement)
c) Atypical KD if KD with unusual manifestation (e.g. renal failure)
11
Q
Age group for Kawasaki
A
3 months - 5 years
12
Q
What is the most common cause of acquired heart disease in developed countries in children?
A
kawasaki disease
13
Q
Other manifestations of Kawasaki disease
A
relatively common: aseptic meningitis, skin peeling in groin, arthritis, sterile pyuria, gastroenteritis, uveitis
uncommon: gallbladder hydrops, GI ischemia, jaundice
cardiac: myocarditis (treat with steroids), pericarditis, cardiac failure, valvular regurgitation
MAS (treat with steroids), DIC
periungal desquamation in weeks 2-3
14
Q
when is the highest chance of coronary artery aneurysms after Kawasaki disease
A
6-8 weeks after acute illness
15
Q
What are risk factors for coronary artery disease in KD?
A
males infants 9 years prolonged fever Asian or Hispanic ethnicity low platelets hyponatremia
16
Q
Lab features of Kawasaki disease
A
1.leukocytosis with left shift
2. normocytic anemia
3. elevated ESR/CRP
4. hypoalbuminemia
5. hyponatremia
6. may have transaminases elevated
thrombocytosis in second week of illness, nomad by 4-8 weeks
17
Q
Thrombocytosis in Kawasaki disease, when?
A
second week of illness, normal by 4-8 weeks
18
Q
When should treatment be started in Kawasaki disease
A
target 7-10 days after fever onset
IVIG 2g/kg
high dose ASA 80-100 mg/kg/day until afebrile (anti-inflammatory) then 3-5 mg/kg/day (anti-platelet
19
Q
What does IVIG help with in Kawasaki
A
reduces the incidence of coronary artery aneurysms
if still febrile 24-36 hours after IVIG give second dose
20
Q
If Kawasaki resistant to IVIG, what to do?
A
give pulse IV methylpred
this occurs in 10-15% of patients
21
Q
If patient with Kawasaki has a large coronary artery aneurysm, how to treat?
A
abciximab (glycoprotein IIb/IIIareceptor inhibitor)
22
Q
What is the in-hospital mortality of kawasaki disease
A
0.17% (all cardiac)
23
Q
risk of recurrent KD?
A
2%
24
Q
Without treatment what is the risk of Coronary aneurysms in KD?
A
25%
reduced to 4% with IVIG within 10 days
if coronary artery aneurysm, risk of outlet of thrombosis, obstruction and stenosis at inlet/outlet of aneurysm, ventricular dysfunction/arrhythmia, early atherosclerosis
25
Is polyarteritis nodosa common in childhood?
no, very rare in childhood
26
Features of Wegener granulomatosis?
```
mainly small vessel vasculitis, characterized by granulomatous inflammation
at least 3 of:
- histopathology - granuloma in artery/perivascular
- upper airway involvement
- laryngo/tracheo-bronchial involvement
- pulmonary involvement
- ANCA positive (90%) most are c ANCA
- renal involvement
```
27
Treatment of wegener
steroids, cyclophosphamide
significant morbidity
can get severe lung and/or renal disease
28
Criteria for HSP diagnosis?
purpura or petechiae - mainly lower limb PLUS 1 or more of the following:
1. abdo pain (including intussuecption and GI bleed)
2. skin biopsy showing vasculitis with predominant IgA deposits or kidney biopsy with GN with IGA deposits
3. acute arthritis or arthralgia
4. renal involvement - (most common microscopic hematuria) proteinuria/hematuria/casts or impaired renal function
29
Which is the most common vasculitis in children?
a) Kawasaki
b) HSP
b) HSP is the most common vasculitis in children
often follows a resp infection, most common group A strep
mainly small vessel vasculitis (IgA deposition and leukocytoclastic vasculitis)
30
Clinical features of HSP/
1. purport or petechiae- 100% of patients with palpable lesions
- abdo pain (may include intussusception and GI bleeding)
- skin biopsy /kidney biopsy showing IgA deposits
- arthritis/arthralgia of acute onset
- renal involvement - proteinuria >0.3 g in 24 hours, hematuria, RBC casts, impaired renal function
31
most common clinical feature of HSP?
a) cutaneous purpura
b) arthritis
c) GI involvement
d) renal involvement
e) orchitis
```
most common is purpua - 100% of patients
arthritis -75% of knees and ankles associated with painful edema
GI - (50-75%) includes abdo pain and intussuception
renal involvement (40-50%)
- microscopic hematuria
- proteinuria in 25%
- nephrotic syndrome in 5%
- orchitis - 10-20% of males
```
32
how long should you monitor HSP kids for urinary abnormalities?
may not present right away, monitor for 3 months after illness
33
lab abnormalities in HSP?
may have elevated WBC and ESR
| platelet and coats must be NORMAL
34
Serum IgA levels in HSP?
increased in 50% of patients
35
Treatment of HSP?
supportive
NSAIDS for joint pain
prednisone in select patients
36
Which of the following is likely helped by steroid treatment of HSP?
a) GI symptoms
b) renal disease
c) HSP recurrence
a) GI symptoms: may decrease the severity and duration of GI symptoms
unclear impact on renal disease (controversy), no definite benefit for prevention of HSP recurrence
37
How long does HSP usually take to resolve?
4 weeks, recurrent in 1/3 of patients
38
A mom wants to know what the chance of recurrence of HSP is?
recurrent in 1/3 of patients
39
What factors relate to the long-term prognosis of HSP?
depends on severity of nephritis
| worse with nephrotic syndrome or if >50% crescent formation on biopsy
40
How many patients with HSP can get end stage renal disease
1-3%, 20% of those with nephritis/nephrotic syndromes
41
What is childhood primary angitis of the CNS?
inflammatory disease of the vessels of the CNS
Calabrese criteria: new focal or diffuse neuro deficit, PLUS evidence of CNS vasculitis, plus absence of systemic condition to explain findings
42
What is Behcet disease?
systemic vasculitis
criteria for diagnosis:
1. recurrent oral ulcers
2. recurrent genital ulcers
3. eye lesions (anterior/posterior uveitis, cells in viteous on slit lamp exam/retinal vasculitis)
4. skin lesions (erythema nodosum, pseudo vasculitis, papulopustular lesions, acneiform nodules)
5. Pathergy - skin papule after needle insertion
43
What type of blood vessel is affected by Behcet disease
unique in that ALL sizes of vessels (small, medium, large) can be affected, arterial and venous
UNCOMMON in children
other findings: CNS, MSK, vascular treatment, include steroids, colchicine, thalidomide and anti-TNF
44
What are the diagnostic criteria for juvenile dermatomyositis?
1. symmetrical proximal muscle weakness
2. skin changes - Gottron papules on the dorsal surface of the knuckles and heliotrope rash over the eyelids
3. Elevated muscle enzymes, including CK, AST, LDH, aldolase
4. abnormal EMG demonstrating denervation and myopathy
5. abnormal muscle biopsy
45
A patient presents with trouble getting up from sitting/lying, trouble with walking up stairs and frequent falls. Having a pink skin rash around the eyes. What diagnosis to think about?
Juvenile dermatomyositis
Clinical history:
1. proximal muscle weakness - 95% of patients. may have gower sign
2. nasal voice, trouble swallowing/choking (18-44%) may indicate weak palate/cricopharyngeal muscles
3. Skin rashes:
- Gottron papules - 57-100%
- heliotrope rash - 66-100%
- malar rash 42-73% and photosensivite rash - can be confused with psoriasis
- capillary vasculopathy on nailbed capillaries
4. arthritis
5. GI tract
6. lungs - interstitial
7. heart - cardiomyopathy
46
What are some common medication related side effects of steroids for JDM?
```
infection
osteoporosis
growth delay
cataracts/glaucoma
type 2 diabetes
hypertension
```
47
Treatment of JDM?
steroids, methotrexate, may be role for IVIG, cyclosporine, rituximab in resistant or refractory disease
48
What are the features of limited systemic sclerosis (aka CREST syndrome)
```
Calcinosis
Raynaud's syndrome
esophageal dysmotility
sclerodactyly
telangiectasias
progresses more slowly
has a higher risk for later development of pulmonary hypertension
```
49
What are some precipitants of Raynaud phenomenon
precipitated by cold, physical or emotional stress, caffeine or smoking
can be primary or secondary
50
What are the phases of Raynaud phenomenon?
vascular spasm leading to triphasic colour sequence - >white->blue->red ; well demarcated areas, usually affects fingers and toes, may also involve other areas
51
Primary and secondary causes of Raynaud
primary - no underlying aetiology, positive family history, no ulcerations
secondary
- underlying autoimmune diseases: scleroderma, mixed connective tissue disease, SLE, JDM, overlap
- mechanical obstruction - thoracic outlet syndrome, cervical rib
- hyperviscositiy - polycythemia
- cryoglobulinemia
- drugs/toxins
52
A patient presents with Raynaud phenomenon. What are the two best predictive factors for development of future autoimmune disease
1. positive ANA
| 2. abnormal nail fold vasculature
53
Treatment of Raynaud
prevent, avoid triggers
| low dose of peripheral vasodilator - i.e. nifedipine may be considered in certain cases
54
What are the three broad categories of scleroderma and scleroderma-like disorders
1. morphea/localized scleroderma
- circuscribed morphea, linear scleroderma, generalized, pansclerotic, mixed
2. systemic sclerosis
- diffuse (more chance of internal organ inovlement/dysfunction), limited (CREST), overlap syndromes
3. scleroderma-like disorders
- GVHD, drug/toxin, diabetic cheiroarthropathy, PKU, premature aging syndromes
55
Most common form of localized scleroderma in children?
linear scleroderma - 1 or more linear streaks over upper/lower extremity
unilateral in >85% may extend over joints
can be associated with intracrnial lesions , seizures, uveitis and dental abnormalities
56
What is Parry -Romberg syndrome?En coup de sabre?
both types of linear scleroderma
en coup de sabre - linear lesion on face/scalp - usually forehead
Parry-Romberg - progressive hemi-facial atrophy - more disfiguring
57
most disabling form of localized scleroderma?
pansclerotic - most rare but most disabling, since it goes below the dermis
58
Treatment for localized scleroderma?
TopicaL : emolients, steroids, calcipotriene
Systemic: steroids, methotrexate, cellcept, cyclosporine
Other: psoralen with UvA radiation therapy
59
Features of systemic sclerosis
rare in chilren , mean age of onset
features include:
- raynaud, sclerosis, calcinosis, vascular changes (telangiectasias) abnormal nail fold capilaries, MSK findings include polyarthritis with minimal joint effusion, GI findings - are a major cause of morbidity, severe GERD , can lead to malabsoprtion with diarrhea , Resp - include pulmonary HTN, interstitial lung disease, inflammatory alveoli tis, cardiac, renal (proteinuria then hypertension, glomerular disease is unusual), rare - i.e. trigeminal neuropathy
60
What are investigations you should consider for a child where you are suspecting systemic scleroderma?
1. bloodwork and serology
- ANA, RF, anti-Scle-70, Anticentromere, blood pressure and urinalysis for proteinuria, ECG and echo to look for cardiac pathology (especially pulmonary hypertension), CXR, upper GI
Treamtent targets all the systemic manifestations
61
What is macrophage activation syndrome?
multisystem inflammatory emergency
can happen in lots of diseases, systemic JIA, SLE, Kawasaki
a form of secondary hemophagocytic lymphohistiocytosis (vs primary which is an inherited condition with similar manifestations)
62
What are the clinical and lab features of MAS
1. fever
2. splenomegaly
3. cytopenias - anemia, thrombocytopenia, neutropenia or in JIA, can see decrease in previously elevated cell counts
4. elevated TG
5. decreased fibrinogen
6. elevated ferritin
7. hemophagocytosis on bone marrow, LN, liver or spleen biopsy
Other findings: DIC like picture - increased INR, PTT
hepatic dysfunction
elevated LDN
increased CRP but decreasing ESR
CNS dysfunction
resp distress
***CAN BE LIFE Threatening
Treatment: supportive, often use IVIG, use stepwise immunosuppression
63
What is the duration when to consider fever of unknown origin
> 2 weeks, usual investigations haven't given an explanation
64
Big categories for FUO?
1. infection
2. inflammation - JIA (systemic), SLE, vasculitis, IBD, autoinflammatory syndromes, sarcoidosis
3. malignancy - leukemia, lymphoma, neuroblastoma
4. drug fever
65
A Turkish girl comes in with fever episodes that last 1-3 days and occur every 4-8 weeks, she also complains of some pleuritic pain and has a rash on her shins and feet. What is the likely diagnosis?
familial meditarranean fever
most common hereditary auto inflammatory disease
AR inheritence
ethnic: arabic, turkish, armenian, meditarranean
usually in childhood 60% before age 10
cilinical features: fever 1-3 days q4-8 weeks, serositis (peritonitis, pleuritis, synovitis), skin: erysipelas-like rash on shins/dorsum of feet, MSK: mono arthritis, myalgia
morbidity: with amyloidosis - especially renal
66
treatment of familial meditarreanean fever
colchicine - 75%
| anakinra - effective if resistant to colchicine
67
List of auto inflammatory disease?
1. FMF
2. TNF-Receptor Associated Periodic Syndrome (TRAPS)
3. hyperimmunoglobulinemia D syndrome
4. cryopyrin associated period syndrome (CAPS) - continuum
5. periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA)
68
What is the most common recurrent fever syndrome in North american children?
PFAPA
no known genetic association or inheritance pattern
starts in early childhood before 5 years, self-limited (resolves within 5 years)
clinical features: fever every 4-6 weeks, lasts up to 5 days at a time, small non-scarring aphthous ulcers, non exudative pharyngitis, cervical adenines, may have associated nausea, vomiting, abdo pain, headache.
negative throat culture
69
Diagnostic criteria for Chronic Recurrent Multifocal osteromyelitis ?
diagnosis of exlucsion
bone pain associated with fever and malaise
clinical and radiographic findings may mimic septic osteomyelitis, no abscess formation, negative cultures, poor response to antibiotic therapy
70
CRMO more common in females or males?
more in females
imaging plays a major role in diagnosis:
- osteolytic bone lesions close to growth plate, sclerosis and periosteal reaction, bone scan, MRI
Treatment: first line - NSAIDS
71
Common organisms that cause reactive arthritis
GI: salmonella, shigella, yersinia, campylobacter
GU: chlamydia, ureaplasma
clinical manifestations: clinical infection before the arthritis/enthesitis by 1-4 weeks, active period of weeks to months, sustained remission or recurrent episodes which may evolve to ERA, especially in patients that are positive for HLAB27.
acute arthritis/enthesitis, patients may continue to have fever, weight loss, fatigue and muscle weakness, can get painless ulcers, urethritis and cervitis (rare) conjunctivitis in 2/3 of kids at onset
72
What characteristics of arthritis in acute rheumatic fever helps differentiate from other causes
migratory and additive
mono arthritis of large joints initially
shot duration - hours to days
dramatic response to ASA/NSAIDS
73
major manifestations of ARF (acute rheumatic fever) ?
```
polyarthritis
carditis
erythema marginatum
sc nodules
**need 2 major or 1 major plus 2 minor manifestations if also have evidence of preceding GAS infection
```
74
Minor manifestations
Clinical: fever, arthralgia
Lab: increased ESR, CRP, prolonged PR interval
75
What is considered to be adequate supporting evidence of antecedent GAS infection
elevated or rising strep antibody tigers
| positive throat culture
76
Which layer of the eye is the uvea
middle layer, uveitis can be symptomatic or asymptomatic
| list of conditions associated with uveitis
77
What are the features of growing pains
usual onset between 4-10 year old
deep aching cramping pain in thighs/calves, at night, can awaken from sleep
improves with massage, heat/analgesia with tylenol, symptoms better by morning, nomad physical exam
78
A child presents with hyper mobility, what syndromes should you exclude before diagnosing hyper mobile joint syndrome?
ehler's danlos, marfan, down, turner, osteogenesis imperfecta
