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deck 3 Flashcards

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1
Q

what characteristics of pain suggest more inflammatory pain?

A

worse in the morning, swelling, warmth, redness and improvement with movement

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2
Q

What pain is more mechanical?

A

worse at the end of the day, worse with activity, and lacking persistent swelling

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3
Q

What type of joints are involved in acute rheumatic fever?

A

migratory
affects both large and small joints
pain may be out of proportion of physical findings, respond to NSAIDS quickly

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4
Q

What is suggested by arthritis which resolves within a few weeks?

A

reactive arthritis

DDX includes: strep, EBV, parvo

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5
Q

How long does arthritis need to last to be considered chronic?

A

> 6 weeks

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6
Q

True or false - having a first degree relative with RA increases chance of developing JIA?

A

false, does not (Zitelli)

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7
Q

true or false - wrists are frequently associated in childhood arthritis

A

true

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8
Q

True or false - all patients with JIA will have pain

A

false - they will have objective arthritis but may not have pain

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9
Q

A child presents to you with dactilytis of one joint and arthritis of the nee. Mom has psoriasis. What is the diagnosis?

A

psoriatic arthritis
diagnosed when child has arthritis and psoriasis or when child with arthritis has 2/3 findings of the following:
dactilytis
nail pitting
family history of psoriasis in first degree relative

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10
Q

True or false - there is always arthritis early in the presentation of systemic JIA?

A

false
often just starts with spiking fevers and rash
arthritis may come after
Koebner phenomenon
(can be fever of unknown origin initially)
other systems which can be affected: serositis, pleuritis, pericarditis, hyperbilirubinemia, liver enzyme elevation, leukocytosis and anemia

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11
Q

What is macrophage activation syndrome?

A

patients with systemic JIA are at risk of it
acutely ill with bruising, purport, mucosal bleeding
HSAM
lymphadenopathy
decreased WBC, Hg, Plt
decreased ESR
increased ALT, AST, PTT, aPTT, fibrin, ferritin, triglyceride, decreased fibrinogen, clotting factors, tissue biopsy may demonstrate active phagocytosis by macrophages
bone marrow will show macrophages engulfing cells

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12
Q

Ddx of mono arthritis:

A
  1. infectius - bacterial - especially in mono arthritis, if intensely red and tender with systemic symptoms do tap early
    causes include staph, strep, Hib
  2. lyme arthritis - knee, elbow, wrist with exacerbations and remissions, can get malaise, fever, myalgia, lymphadenopathy, headache, meningismus and weakness, rash is erythema migraines
    neurological complications, cardiac heart block, bilateral bell’s/seventh nerve
  3. reactive arthritis - bacteria and virus
  4. post streptococcal - ARF/post streptococcal reactive arthritis
  5. malignancy - neuroblastoma/leukemia
  6. sickle cell
  7. hemophilia, TB, gonorrhea
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13
Q

Bugs which cause reactive arthritis:

A

bacteria: salmonella, shigella, yersinia, campylobacter
viruses: rubella, hepB, adeno, herpesvirus (EBV, CMV, VZV, HSV); parvo, mumps, entero

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14
Q

A patient with JIA who becomes ill with thrombocytopenia, profound anemia and increased LFTS probably has which complication?

A

Macrophage activation syndrome
(MAS)
- massive up regulation of T cell function, often triggered by T cell
most impotant contributor ro mortality in JIA (the others are GI bleeding and infection)
features: worse fever and rash, profound anemia, leukopenia, thrombocytopenia, DIC, liver, hypertriglyceridemia, hyponatremia , increased ferritin, CNS

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15
Q

Treatment of JIA - 1st line

A

NSAIDS

peak action at 4-6 weeks

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16
Q

1st line option for oligoarticular JIA (other than NSAIDS)

A

joint injection with steroids

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17
Q

2nd line agent for JIA?

A

methtrexate

others include gold, penicillamine, hydroxychloroquine, sulfasalazine but not as much evidence

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18
Q

Side effects of steroids

A 
Mnemonic (Cushinoid map)
Cataracts
Ulcers
Striae
Hypertension
Infectius
Necrosis (AVN)
Growth retardation
Osteoporosis
Increased ICP
DM
Myopathy
Adipose tissue
Pancreatitis
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19
Q

Name 4 signs of uveitis

A 
1st - flare in the anterior changer (haze)
speckled posterior cornea
irregular/poorly reactive pupil
band keratopathy
cataracts
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20
Q

Clinical features of juvenile spondyloarthropathies

A
  1. males >8 years
  2. enthesitis (tibial tubercle, peripheral patella, achilles insertion/plantar fascia)
  3. prodromal oligoarthritis
  4. sacroiliac joings
  5. HLA B27
  6. usually ANA and RA negative
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21
Q

Criteria for acute rheumatic fever

A

Criteria for acute rheumatic fever (Nelson):
Need either 2 major or 1 major and 2 minor criteria
3 circustances when can make the diagnosis without all the criteria: Sydenham chorea, recurrence, indolent carditis
5 major criteria: JONES (joints, heart, nodules, erythema marginatum, Sydenham chorea)
4 minor criteria: Clinical: arthralgia, fever Lab increased acute phase reactants (ESR, CRP) prolonged PR interval
ABSOLUTELY NEED evidence of recent GAS infection : positive throat culture, or rapid strep test; elevated or increasing strep Ab titer

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22
Q

What is the earliest symptom in acute rheumatic fever?

A

migratory polyarthritis

arthritis responds very well to ASA

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23
Q

which patients with ARF should get steroids?

A

controversial, but usually if severe carditis then should get prednisone

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24
Q

Treatment for acute rheumatic fever?

A

Nelson
Antibiotic: 10 days of penicillin orally or erythromycin orally, or 1x IV benzathine penicillin ; then start long term prophylaxis
Anti-inflammatory: if they have typically migratory polyarthritis or carditis without CHF should get anti-inflammatory treatment with salicylates
If have carditis with CHF then should get steroids
Prophylaxis: if no carditis initially then low risk of carditis with recurrences – should get prophylaxis until age 21 or until years after attack, treatment choice is IM benzathine penicillin monthly

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25
what labs are associated with lupus?
1. ANA positive (useful screening test if patient has symptoms of lupus, but otherwise is useless since 10% of normal kids can have it) - most sensitive 2. other autoantibodies are more specific (but less sensitive): dsDNA and antiSm 3. low complement 4. increased ESR 5. anemia, leukopenia, lymphopenia, and/or thrombocytopenia
26
how much of SLE presents in childhood?
15-20%
27
Which are the most common manifestations of SE in children?
1. arthritis 80-90% 2. rash or fever: 70% 3. renal disease - proteinuria or casts - 70% 4. serositis 50% 5. hypertension 50% 6. CNS disease - 20-40% 7. anemia/leukopenia/thrombocytopenia
28
When to renal biopsy kids with SLE?
1. SLE and nephrotic syndrome - distinguish membranous glomerulonephritis from diffuse proliferative glomerulonephritis (need more aggressive therapy) 2. failure of high-dose steroids to reverse renal function 3. pre-clinical trials
29
what is the association of antiphospholipid antibodies and lupus?
often seen in patients with lupus, but not always in kids | can cause stroke etc
30
Best way to monitor treatment response in lupus?
anti DsDNA, complement, ESR, CBC | NOT ANA*** (does not correlate with disease activity)
31
True or false most mothers of kids with neonatal lupus have lupus?
false - 70-80% are asymptomatic | mom's can have SLE or Sjogren (this is where it was first described)
32
features of neonatal lupus
caused by IgG autoantibodies 1. skin - usually develops in the first 2-3 months of life , sunlight, transient and non scarring 2. cardiac: complete congenital heart block - 90% due to neonatal lupus , most are after the nonfatal period, mortally is 15% 3. hepatic : 15% have involvement, may have increased LFTs, similar to idiopathic neonatal gian cell 4. heme: thrombocytopenia, hemolytic anemia, neutropenia
33
What are some drugs that can cause drug induced lupus in children?
``` most common: antiepileptic meds (ethosuximide, phenytoin, primidone) 20% will have positive ANA taking these drugs other: 2. minocycline 3. hydralazine 4. isoniazid 5. alpha methyldopa 6. chlorpormazine 7. antithyroid 8. beta blockers 9. tetracycline (with long term, features include polyarthritis, positive ANA, mild liver dysfunction) ```
34
What are common features of drug-induced lupus
fever arthralgias arthritis ANA and antihistone antibodies often positive less likely to have the other systems involved (i.e. renal, CNS< malar rash, alopecia, oral ulcers)
35
What are some infectious agents associated with vasculitis?
viral: HIV, hepB/C, CMV, EBV, varicella, rubella, parvo Rickettsia: rocky mountain spotted fever, typhus, ricketssia Bacterial: meningococcus, sepsis, bacterial endocarditis syphilis TB
36
Name 5 pulmonary renal syndromes
1. Wegener 2. goodpasture 3. Churg-Strauss 4. SLE
37
What is the clinical triad of Behcet disease
1. aphthous stomatitis 2. genital ulcers 3. uveitis unclear aetiology can also get some polyarthritis and inflammatory GI lesions aseptic meningitis, sinus vein thrombosis and other forms of DVT are also characteristic of disease
38
True or false - ESR and CRP should be elevated in HSP?
true - ESR and CRP commonly elevated, sometimes mild leukocytosis
39
True or false - platelets often low in HSP
false - platelets CANNOT be low in HSP
40
What is the most important prognostic factor in HSP?
renal involvement - if have nephritis or nephrotic syndrome initially have long term problems with hypertension/impaired renal function as adults microscopic hematuria on its own is okay usually
41
You see a baby that seems to have large purpuric lesions, no renal or GI involvement, you think it looks similar to HSP but haven't seen it in a child so young. What diagnosis should you think of?
acute hemorrhagic edema of infancy (simple version is that this is the infantile version of HSP)
42
systemic JIA
diagnostic criteria: 2) arthritis of 1 or more joints associated with or preceded by fever for 2 weeks accompanied by 1 or more of the following: - HSM - lymphadenopathy - evanescent rash - serositis
43
what percentage of systemic JIA will get MAS?
``` 7% life-threatening complication MAS can be a complication of 3 rheum issues: - systemic JIA and other JIA - lupus - Kawasaki MAS is HLH secondary to JIA or SLE ```
44
Criteria for HLH
``` #1:molecular diagnosis #2: non genetic type ``` Lab findings: high ferritin - above 50 000, high TG >265 cytopenia (2 or more lines) Hg <1 Clinical Findings: need 5 fever, splenomegaly, cytopenia, TG, hemophagocytosis (macrophages eating your bone marrow/spleen), low or absent NK cells, elevated ferritin, elevated CD25)
45
most common malignant bone lesion
``` osteosarcoma peak age is during growth spurt distal femur or proximal tibia pre-disposing factors to osteosarcoma: - radiation - retinoblastoma (hereditary) - any benign bone condition - fibrous dysplasia (like in McCune Albright) mets to lungs (1/5 kids) and bone X ray findings: sunburst appearance with periosteal reaction ```
46
periosteal reaction
never good - means either osteosarcoma, swings or bad osteomyelitis
47
poor prognosis factors for osteosarcoma
lung mets, large mets, bilateral mets <20% high recurrence risk of 30%
48
dermatomyositis best confirmatory tests
EMG dermatomyositis: rash is often the first presentation, can get raynaud's, proximal muscle weakness - 1st presentation in 1/4 of patients, ultimately in 95% of kids gradual, fatigue is associated with it, problems going up stairs/chair +ve Gower sign can also have difficulty swallowing cardiac - can get a dilated cardiomyopathy resp - interstitial lung disease arthritis nail changes eyes mood retinitis activation of T and B lymphocytes leads to vasculitis and immune complex deposition autoimmune links with prior URTI diagnosis: look at the blood vessels, muscle biopsy
49
Tests for diagnosis
EMG - will show denervation and myopathy then do muscle biopsy - will show perifascicular atrophy normal or high muscle enzymes (the sick kids book says elevated) some places say also give methotrexate
50
HSP urinalysis how long to monitor
weekly for at least 6 months | Bp should
51
lytic lesion on X ray
look for periosteal reaction
52
Ewing's sarcoma
tumour of neural crest cells found in long bones can also be in pelvis, spine and chest wall lots have mets at presentation investigations: X ray - onion skinning and periosteal reaction bloodwork: advanced disease might see some systemic disease genetics - 85% have balanced translocation
53
Prognostic factors in Ewing's
1. mets at diagnosis 2. poor response to treatment initially 3. increased LDH
54
enthesitis related arthritis what family history is most likely
arthritis or enthesitis or enthesitis or arthritis with at least 2 of the following: - presence or history of SI joint tenderness - back pain presence of HLAB27 antigen, onset of arthritis in a male over 6 years of age, acute uveitis, history of ank spond, enthesitis related arthritis or sacroilitis with IBD or acute uveitis in a first degree relative
55
SCFE risk factors
1. hypothyroid 2. obesity 3. hypogonadism 4. growth hormone treatment 5. hypopituitarism 6. renal osteodystrophy 7. rickets males more likely left side more often 50-60% bilteral
56
complications of SCFE
``` 1. osteonecrosis (only if unstable) can happen even after you fix it 2. leg length discrepancy 3. dislocation AP and frog leg X ray don't weight bear admit, pin it if weird picture then do endo work up can weight bear after 4-6 weeks ```
57
Scoliosis diagnosis
1. assess level of pelvis 2. adam's foreword bend test 3. measure leg length discrepancy
58
casting of nicu baby ex 28 week pain who is ventilated
need to cast within 6 weeks | ponsetti method
59
clubfoot diagnosis
1. extreme plantar flexion 2. medial angulation of the hind foot 3. adduction and supination of the forefoot (metatarsus adductus)
60
when to not W sit
for femoral ante version only (Michelle learned this in ortho) although CPS statement says for metatarsus adductus also
61
What percentage of kids with septic arthritis have fever
70%
62
What percentage of kids with septic arthritis have positive blood culture?
50%
63
What is the joint aspiration findings in septic arthritis?
50 x 109
64
neonates septic and osteo antibiotics
clox and gent
65
for kids with septic arthritis
cefazolin (good gram positive and staph coverage)
66
teenager with septic arthritis
ceftriaxone (since will also cover gonorrhoea)
67
reflex sympathetic dystrophy
burning sensation of skin usually hand and foot not correspond to anatomical distribution of the nerve no objective signs of arthritis cool and clammy treatment is sympathetic blockage and physiotherapy
68
valves involved in rheumatic fever
mitral and aortic acute - regurg chronic - stenosis timing of acute rheumatic fever happens 2-4 weeks after the GAS infection
69
what percentage with rheumatic fever will have positive throat culture at diagnosis of rheumatic fever
10% need to to ASOT , antibodies to strep (anti DNase B, anti hyalironidase antibody) only 80% of patients with rheumatic fever will have elevated ASOT at presentation - 95%-100% will have elevation of one of the antibodies
70
criteria with diagnosis of rheumatic fever without all the criteria
indolent carditis - cardiac manifestations chorea previous episode of rheumatic fever
71
sydenham chorea signs
emotional labile | milkmaid grip
72
PTH in rickets
high
73
treatment of proximal humerus fracture
if open then OR | otherwise sling
74
when to refer idiopathic scoliosis
1. abnormal neurological exam 2. pre-pubertal 3. Cobb angle >20 degree 4. if left thoracic curve in adolescence - associated with more neuromuscular issues , also need to look for spinal cord abnormalities- spinal cord dysraphism
75
3 physical exam maneuvers
adam forward bend - thoracic rib bump leg length discrepancy level of the pelvis when they are facing you
76
what on X ray will tell you if scoliosis can progress rapidly?
Risser sign - look at iliac crest depends on if has complete fusion if hasn't started puberty yet

rheumatology (3 decks)

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