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
Q

What are the features of FMF?

A
  • 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
Q

What is the txt for FMF

A

prophylaxis with colchicine (needs to be constant prophylaxis)

  • up to 96% Efficiency
  • not helpful in acute phase
27
Q

What are the features of PFAPA

A

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
Q

What are the autoantibodies for SLE

A
  • ANA (97%)
  • dsDNA (70%)
  • Sm (32%)
  • RoSSA/LaSSb (20 - 30%)
  • Antiphospholipid - Ab (35%)
29
Q

What is the most Sensitive test for SLE

What is the most specific test for SLE

A

Most sensitive test: ANA

Most specific test: anti double stranded DNA antibodies

30
Q

What are the features of Dermatomyositis

A
  • 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
Q

What are the features of HSP (7)

A
  • palpable purpura
  • arthritis
  • abdominal pain, bleed, intussusception
  • IgA nephropathy, renal failure < 5%
  • edema
  • orchitis
  • leukocytoclastic vasculitis IgA deposition
32
Q

What is the treatment for HSP

A
  • 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
Q

What is the criteria for Classical Kawasaki Syndrome

A

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
Q

What are other helpful clues for diagnosis of KD not part of the criteria

A
  • 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
Q

What are findings supporting a diagnosis OTHER THAN KD

A
  • exudative conjunctivitis
  • generalized lymph node swelling
  • intraoral ulceration or vesicles
  • exudative pharyngitis
  • normal ESR
  • Leucocytes < 10, 000/uL
36
Q

What is the treatment for KD

A

-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
Q

what are the ANCA-related vasculitis?

A

Microscopic polyangitis
Granulomatosis with polyangitis (wegner)
Eosinophillic granulotosis with polyangitis (churg strauus)

38
Q

Criteria for Takayasu artheritis

A
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
Q

neonatal lupus

name 4 complications

A

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
Q

antiphospholipid antibodies - list

A

lupus anticoagulant
anticardiolipin
anti b2 - glycoprotein I

41
Q

KD - when and how much does IVIG change risk for Coronary aneurysms?

A

IVIG given within first 1- days

25% risk to 4%

42
Q

Criteria for HSP

A

Purpura/purpura + 1 of 4

1) abdominal colicky pain
2) arthritis/arthralgia
3) renal (proteinuria, hematuria, etc)
4) biopsy - skin or renal

43
Q

JDM exam and investigations (4)

A

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
Q

JDM complications

A

muscle weakness - joint contractures
soft tissue calcinfication/calcinosis
lipoatrophy +/- hyperinsulin, TG, acanthosis nigricans, T2DM

45
Q

Systemic sclerosis 5 complications

A

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
Q

Systemic sclerosis investigations

A
ANA, anti Scl70, anticentromere (rare RF)
BP, U/A
ECHO, ECG - PulHTN
CT chest, PFT - ILD
UGIS - dysmotility
47
Q

Raynaud treatment

A

1) avoid triggers - cold, caffeine, smoking, stress
2) nifiedipine - peripheral vasodilator
3) if severe - IV prostaglandin
4) topical nitroglycerin if ulcers

48
Q

Sjogren syndrome

2 main manifestations

A
keratoconjuntivitis sicca (dry eyes)
xerostomia (dry mouth)