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Rheumatology Flashcards

1
Q

What are the features of Growing Pains (6)

A
  • age 3 - 10
  • Bilateral Leg pain
  • Occurs at night
  • Normal physical exam
  • Normal Lab tests
  • Normal Imaging
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2
Q

What are the 2 different Pain Amplification Syndromes?

A

1) Generalized
- Fibromyalgia
- specific trigger points
- associated psychiatric symptoms

2) Localized
- Reflex sympathetic dystrophy
- Complex regional pain syndrome

Autonomic changes

  • acute: swelling
  • chronic: atrophy/cold extremities

Girls> boys

Txt: Interdisciplinary assessment and therapy

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3
Q

Most common cause of limp according to age range?

A

Most common cause is often in the Hip

2 - 6 yrs: Transient Synovitis

4 - 10 yrs: Legg-Calve-Perthe’s

10 - 14 yr: Slipped Capital Femoral Epiphysis

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4
Q

What are the cardinal features of an acute joint infection and what is its management?

A

Cardinal features:

  • fever
  • severe pain/tenderness
  • swelling
  • redness

Surgical emergency!

  • joint aspirate and culture
  • irrigate joint
  • Antibiotics
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5
Q

What is the DDX for Monoarthritis

A

Trauma/Mechanical (overuse)

Orthopedic

  • AVN
  • SCFE

Infection:

  • acute (septic arthritis, osteomyelitis)
  • chronic ( TB and Lyme)
  • reactive arthritis

Inflammatory:

  • JIA
  • non-bacterial osteomyelitis
  • FMF

Tumor (localized)

Hemarthrosis

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6
Q

DDX for Polyarthritis

A

Inflammatory:

  • JIA
  • SLE, other connective tissue diseases
  • Vasculitis - HSP

Infection:

  • Acute: viral (parvo), bacterial (gonococcal)
  • Post-infectious (reactive/RF)

Malignancy (systemic)

Mechanical
-Hypermobility, sketetal dysplasia

Pain syndrome
-fibromyalgia

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7
Q

What is the Major and Minor Criteria of ARF

A

Need 2/5 Major Criteria or 1/5 Major and 2/4 Minor Criteria pluse evidence of of GAS infection - temporal relationship

Except chorea - don’t need to meet whole criteria

Major: SPACE
S ubcutaneous nodules
P ancarditis
A rthritis, migratory, poly
C horea
E rythema 
Minor: FLAP 
F ever
L ab abnormalities (increased ESR, CRP)
A rthralgia
P rolonged PR interval
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8
Q

Treatment for ARF

A
  • penicillin for 10 days
  • ASA for arthritis
  • ASA/steriods for carditis
  • Prophylactic penicillin for at least 5 yrs or until 21 years, lifelong for carditis
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9
Q

What is the definition of JIA

A

Arthritis before 16 yrs
Duration x 6 weeks

Exclude other causes

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10
Q

What level of ANA should you consider you do specific antibodies

A

Specific antibodies (Anti-double stranded DNA) only if ANA + at higher levels (>1:160) and disease other than JIA suspected

Low titres of nonspecific ANA in JIA (eg ANA 1:160

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11
Q

What is Rheumatoid Factor and how is it used in JIA

A

IGM autoantibody reacts to Fc portion of IgG antibodies

  • 85% in adults with RA
  • only 5 - 10% of children with JIA (poly)
  • no diagnostic utility but classification/prognostic factor (suggest worse prognosis)
  • positive in other rheum disease: SLE
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12
Q

What is HLA B27

A

HLA B27 is a class I gene only present in 10% of ppl

HLA class I and II associated with Rheumatic disorders

  • 90% of Caucasians with spondylarthritis
  • 70-90% of enthesitis related JIA
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13
Q

What are the components of Oligoarthritis

A
  • Girls > Boys
  • max 4 joints in the first 6 months
  • Persistent - only ever 4 joints after the first 6 monts
  • Extended - more than 4 joints after the first 6 months
  • as
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14
Q

What are some of the long term effects from untreated Oligoarthritis

A
  • malalignment of joints
  • muscle atrophy
  • growth disturbances
  • delay of motor development
  • motor and intellectual development are closely related
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15
Q

Which type of JIA is uveitis most commonly associated with?

A
  • most common in oligoarticular JIA (10 - 20%)
  • associated with positive ANA
  • usually ASYMPTOMATIC except with enthesitis related arthritis
  • does not parallel arthritic course
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16
Q

What are the complications of uveitis?

A
  • synechaie - irregular pupil
  • glaucoma
  • cataract
  • visual loss
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17
Q

What are X-ray changes seen in JIA

A
  • acceleration of growth (knee)
  • acceleration of maturation (wrist)
  • growth inhibition (TMJ)
  • osteoporosis
  • erosions
  • loss of cartilage

MRI changes

  • synovitis
  • bone marrow edema
  • cartilage
  • erosions
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18
Q

Features of JIA?

A
  • Girls - 75%
  • typically: symmetric joint involvement, small and large joints
  • slow progression (except for RF/anti CCP pos)
  • Uveitis in 5-10%

Labs:
ANA + 30 -40%
RF/anti CCP- if present = more aggressive course

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19
Q

Features of Enthesitis associated Arthritis

A
  • Boys = 75%
  • typically: asymmetric joint involvement esp lower extr
Enthesitis
Tarsitis
Daktylitis
Sakrolitis
Uveitis (acute) - red and painful

Labs:
HLA- B27 70-90%
ANA and RF negative

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20
Q

What is the criteria for Psoriatic Arthritis

A

Arthritis AND Psoriasis:
-search for ‘hidden’ psoriasis (scalp, umbilicus)

OR

Arthritis plus 2 of the following:

  • nail pits
  • dactylitis
  • family hx of psoriasis
21
Q

What is the criteria for Systemic JIA

A
  • > 2 weeks spiking fever
  • arthritis

+ at least 1 of:

  • rash
  • generalized lymphadenopathy
  • HSM
  • serositis

Course sJIA

  • monocyclic
  • polycyclic
  • persistent
22
Q

What are the 2 categories of HLH

A

Primary HLH (familial)

Secondary HLH (acquired)

  • infection associated
  • malignancy associated
  • rheumatic disease associated: MAS
23
Q

What is the therapy for JIA?

A

Traditionally step up

  • NSAID and joint infection for oligo JIA
  • NSAID then MTX and/or SSZ for poly JIA then biologics
  • NSAID then systemic glucocorticoids and/or MTX for sJIA then biologics
  • Anti TNF does not work well in SJIA
  • sJIA often now treated initially with anti IL-1 or anti IL-6
24
Q

JIA therapy side effects

A
  • monitor CBC, transaminases and creatinine
  • increased risk for infections
  • no live vaccines with MTX and biologics
  • monitor for TB with biologics
  • Varicella can be severe
  • no Sulfa Abx with MTX
25
What are the features of FMF?
- fever, brief episodes (1 - 3 days) - painful polyserositis: peritonitis, pleuritis, arthritis (70%) dx is important because of untreated Amyloidosis in 60% > 40 yrs -also can get renal failure
26
What is the txt for FMF
prophylaxis with colchicine (needs to be constant prophylaxis) - up to 96% Efficiency - not helpful in acute phase
27
What are the features of PFAPA
Periodic Fever, Aphtous stomatitis, pharingitis, adenitis (PFAPA) -regularly recurring fevers with an early age of onset (< 5 yrs of age) - symptoms in the absence of URTI with at least one of the following: - aphtous stomatitis - cervical lymphadenopathy - pharyngitis - exclusion of cyclic neutropenia - completely asymptomatic btw episodes - normal growth and development
28
What are the autoantibodies for SLE
- ANA (97%) - dsDNA (70%) - Sm (32%) - RoSSA/LaSSb (20 - 30%) - Antiphospholipid - Ab (35%)
29
What is the most Sensitive test for SLE What is the most specific test for SLE
Most sensitive test: ANA Most specific test: anti double stranded DNA antibodies
30
What are the features of Dermatomyositis
- symmetric proximal muscle weakness - cutaneous changes - heliotrope or Gottron's papules - elevated muscle enzymes - CK, AST, LDH, aldolase - Abnormal EMG - muscle biopsy - Difficulty swallowing - Resp failure with severe weakness - Lung involvement - Arthritis - Joint contractures - cutaneous and mucosal ulcerations
31
What are the features of HSP (7)
- palpable purpura - arthritis - abdominal pain, bleed, intussusception - IgA nephropathy, renal failure < 5% - edema - orchitis - leukocytoclastic vasculitis IgA deposition
32
What is the treatment for HSP
- supportive - analgesia/NSAIDS - systemic steroids: treat early with steroids in (severe abdo pain, severe renal disease) -recurrences common in approx 30% usually w/n the first 1 - 2 months
33
What is the criteria for Classical Kawasaki Syndrome
Fever >/ 5 days and at lest 4 of the following: - Polymorphous rash - conjunctivitis - cervical lymphadenopathy > 1.5 cm - Oromucosal changes (lips; strawberry tongue, pharyngitis (nonexudative) - extremity changes (palmar pantarerythema (edema, scaling)
34
What are other helpful clues for diagnosis of KD not part of the criteria
- peeling in the perineal area (acute phase) - aseptic meningitis - anterior uveitis in up to 80% - hydrops of the gallbladder - sterile pyuria - lung involvement - diarrhea in approx 25% - Gamma GT elevation
35
What are findings supporting a diagnosis OTHER THAN KD
- exudative conjunctivitis - generalized lymph node swelling - intraoral ulceration or vesicles - exudative pharyngitis - normal ESR - Leucocytes < 10, 000/uL
36
What is the treatment for KD
-IVIG (2g/kg) -ASA (80-100 mg/kg) 10- 20% don't respond => IVIG (2 g/kg) Steroids? -low dose ASA (3 - 5 mg/kg/day) until follow up 2D echo is normal and platelets are normal -be careful of hemolysis
37
what are the ANCA-related vasculitis?
Microscopic polyangitis Granulomatosis with polyangitis (wegner) Eosinophillic granulotosis with polyangitis (churg strauus)
38
Criteria for Takayasu artheritis
``` Angiographic abN of aorta or main branch(PA etc) AND 1of 5 1) peripheral deficit or claudication 2) 4limb - BP > 10mmHg in any limb 3) bruits 4) HTN (>95%) 5) Acute phase reactant (ESR/CRP) ```
39
neonatal lupus | name 4 complications
1) Cardiac - complete heart block 2) Skin - annular erythema, erythematous papulomacular rash (usually head), telaniectasia (later) 3) Heme - thrombocytopenia 4) GI - cholestatic hepatitis 5) Neuro - macrocephaly, hydrocephalus
40
antiphospholipid antibodies - list
lupus anticoagulant anticardiolipin anti b2 - glycoprotein I
41
KD - when and how much does IVIG change risk for Coronary aneurysms?
IVIG given within first 1- days | 25% risk to 4%
42
Criteria for HSP
Purpura/purpura + 1 of 4 1) abdominal colicky pain 2) arthritis/arthralgia 3) renal (proteinuria, hematuria, etc) 4) biopsy - skin or renal
43
JDM exam and investigations (4)
1) proximal muscle weakness(+/-nasal voice, dysphagia) 2) Gottron papules (dorsal knuckles) 3) heliotrope rash (eyelids) 4) elevated muscle enzymes - CK, ALT, LDH, Aldolase 5) EMG - denervation & myopathy 6) biopsy - necrosis & inflammation 7) ANA+ (70%)
44
JDM complications
muscle weakness - joint contractures soft tissue calcinfication/calcinosis lipoatrophy +/- hyperinsulin, TG, acanthosis nigricans, T2DM
45
Systemic sclerosis 5 complications
1) Raynauds 2) sclerodactyly (thickening of skin around fingers) 3) calcium deposits (skin) 4) polyarthritis 5) Resp - PulHTN, ILD 6) GI - dysmotility, bacterial overgrowth, malabsorption 7) GU - HTN, proteinuria
46
Systemic sclerosis investigations
``` ANA, anti Scl70, anticentromere (rare RF) BP, U/A ECHO, ECG - PulHTN CT chest, PFT - ILD UGIS - dysmotility ```
47
Raynaud treatment
1) avoid triggers - cold, caffeine, smoking, stress 2) nifiedipine - peripheral vasodilator 3) if severe - IV prostaglandin 4) topical nitroglycerin if ulcers
48
Sjogren syndrome | 2 main manifestations
``` keratoconjuntivitis sicca (dry eyes) xerostomia (dry mouth) ```

General Pediatrics (19 decks)

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