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PPS 2018 > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Positive ANA is seen in in which viral infection

A

EBV and Parvovirus B19

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2
Q

ANA is positive in up to __ of normal children

A

30

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3
Q

Skin reaction characterized by small hypopigmented depressed scars occuring in areas of minor skin trauma. May be caused by NSAIDs, especially naproxen

A

pseudoporphyria

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4
Q

Most significant potential adverse effect of hydroxychloroquine

A

Retinal toxicity leading to irreversible color blindness or loss of central vision
Recommended dosing is <6.5 mg/kg/d not to exceed 400 mg/day

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5
Q 
Leflunomide
indication
classification
dose
adverse reaction
A

Leflunomide
I: rheumatoid arthritis (pJIA)
C: DMARD
D: PO, OD; 10 to <20 kg 10 mg; 20-40 kg: 15 mg; >40 kg: 20 mg
AR: hepatitis, cytopenias, mucositis, teratogenesis, peripheral neuropathy

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6
Q 
Methotrexate
Indication
Classification
Dose
Adverse reaction
A

Methotrexate
I: JIA, Uveitis, SLE (arthritis, serositis, rash)
C: DMARD
D: 10-20 mg/m2/wk PO; 20-30 mg/m2/wk SC
AR: GI intolerance, hepatitis, myelosuppression, mucositis, teratogenesis, lymphoma, interstitial pneumonitis

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7
Q 
Hydroxychloroquine 
Indication
Classification
Dose
Adverse Reaction
A

Hydroxychloroquine
I: SLE, JDMS, APAS
C: DMARD
D: 5-6 mg/kg PO OD, not to exceed 6.5 mg/kg/d, max dose 400 mg daily
AR: retinal toxicity, Gi intolerance, rash, skin discoloration, anemia, cytopenias, myopathy, CNS stimulation, death

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8
Q 
Sulfasalazine
Indication
Classification
Dose
Adverse Reaction
A

Sulfasalazine
I: Spondyloarthropathy, JIA (for poly, contraindicated in active systemic JIA)
C: DMARD
D: 30-50 mg/kg/d BID, max dose 3 g/d
AR: GI intolerance, rash, hypersensitivity reactions, SJS, cytopenias, hepatitis, headache

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9
Q 
Mycophenolate mofetil
Indication
Classification
Dose
Adverse reaction
A

Mycophenolate mofetil
I: SLE, uveitis, autoimmune skin manifestations
C: Immunosuppressive
D: 600 mg/m2 BID
AR: GI intolerance, cytopenias, secondary malignancies

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10
Q 
Etanercept
Indication 
Classification
Dose
Adverse reaction
A

Etanercept
I: JIA
C: TNF-a antagonist
D: 0.8 mg/kg SC once weekly (max dose 50 mg) or 0.4 mg/kg SC twice weekly (max dose 25 mg)
AR: Injection site reaction, rash, demyelinating disorders, cytopenias, potential increased malignancy risk

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11
Q 
Adalimumab
Indication
Classification
Dose
Adverse reaction
A

Adalimumab
I: JIA, spondyloarthropathy, psoriatic arthritis, uveitis
C: TNF-a antagonist
D: SC once every other week: 15 to <30 kg: 20 mg, ≥30 kg: 40 mg
AR: injection site reaction, infection, rash, cytopenias, lupus-like syndrome, potential increased malignancy risk

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12
Q 
Infliximab
Indication
Classification
Dose
Adverse reaction
A

Infliximab
I: JIA, spondyloarthropathy, uveitis, sarcoidosis (off-label)
C: TNF-a antagonist
D: 5-10 mg/kg IV q4-8 wk
AR: Infusion reactions, hepatitis, potential increased malignancy risk

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13
Q 
Abatacept
Indication
Classification
Dose
Adverse reaction
A

Abatacept
I: JIA (poly)
C: Modulator of T-cell activation
D: IV ever 2 weeks x 3 doses then monthly for ≥6 yr of age: <75 kg: 10 mg/kg; 75-100 kg: 750 mg; >100 kg: 1000 mg
AR: Infection, headache, optential increased malignancy risk

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14
Q 
Rituximab
Indication
Classification
Dose
Adverse reaction
A

Rituximab
I: SLE, JIA (poly)
C: Anti-CD20 (B cell) antibody
D: 575 mg/m2, max 1000 mg IV on days 1 and 15
AR: Infusion reactions, lymphopenia, reactivation hepatitis B< rash, serum sickness, arthritis, progressive multifocal leukoencephalopathy

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15
Q 
Belilumab
Indication
Classification
Dose
Adverse reaction
A

Belimumab
I: Sle
C: Anti-BLyS antibody
D: 10 mg/kg IV every 2 wk x 3 doses then ever 4 wk
AR: Infusion reactions, infection, depression

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16
Q 
Anakinra
Indication
Classification
Dose
Adverse reaction
A 
Anakinra
I: systemic JIA, cryopyrin-associated periodic syndrome (CAPS)
C: IL-1 antagonist
D: 1-2 mg/kg/d, max dose 100 mg
AR: injetion site reactions, infection
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17
Q 
Canakinumab
Indication
Classification
Dose
Adverse reaction
A

Canakinumab
I: CAPS, systemic JIA
C: IL-1 antagonist
D: SC every 8 wk (CAPS) every 4 wk (JIA)
15-40 kg: 2 mg/kg (up to 3 mg/kg); >40 kg: 150 mg
AR: injection site reaction, infection, diarrhea, nausea, vertigo, headache

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18
Q 
Tocilizumab
Indication
Classification
Dose
Adverse reaction
A

Tocilizumab
I: Systemic JIA, pJIA
C: IL-6 antagonist
D: ≥2 yr and ≥30 kg, 8 mg/kg/dose every 2 wk; ≥2 yr and ≤30 kg, 12 mg/kg/dose every 2 wk
AR: infusion reactions, elevated LFTs, elevated lipids, thrombocytopenia, infections

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19
Q

Intravenous immunoglobulin
Indication
Dose
Adverse reaction

A

IVIG
I: Kawasaki disease, JDMS, SLE
D: 1-2 g/kg IV. For JDMS give monthly
AR: Infusion reaction, aseptic meningitis, renal failure

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20
Q 
Cyclophosphamide
Indication
Classification
Dose
Adverse reaction
A

Cyclophosphamide
I: SLE, vasculitis, JDMS, pulmonary hemorrhage
D: 0.5-1 g/m2 IV (max 1.5 g) monthly for 6 mo induction, then every 2-3 mo
Oral regimen: 1-2 mg/kg/daily, max dose 150 mg
AR: Nausea, vomiting, myelosuppression, mucositis, hyponatremia, alopecia, hemorrhagic cystitis, gonadal failure, teratogenesis, secondary malignancy

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21
Q

Most common rheumatic disease in children

A

juvenila idiopathic arthritis

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22
Q

Pathologic characteristic of inflammatory synovitis

A

villous hypertrophy and hyperplasia with hyperemia and edema of the synovial tissue

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23
Q

Arthritis definition

A

Intraarticular swelling and presence of 2 or more of the ff: limitation in range of motion, tenderness or pain on motion, warmth

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24
Q 
ILAR definition of Juvenila Idiopathic Arthritis
minimum duration
age at onset
≤4 joints in 1st 6 mo after presentation
>4 joints in 1st 6 mo after presentation
fever, rash, arthritis
A

JIA (ILAR)
Min duration: ≥6 wk
Age at onset: <16 yr
≤4 joints: oligoarthritis (persistent <4 for course of disease, extended >4 after 6 mo
>4 joints: polyarthritis (RF -/+)
Systemic if with fever, rash
Includes psoriatic arthritis, enthesitis-related arthritis

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25
Q

Definition of systemic JIA

A

Arthritis in ≥1 joint with or preceded by fever of at ≥2 wk that is documented daily (quotidian) for at least 3 days and accompanied by ≥1 of the ff:

  1. evanescent erythematous rash (salmon-colored lesions over trunk and proximal ext, lasting <1 hr)
  2. generalized lymph node enlargement
  3. hepatomegaly and/or splenomegaly
  4. serositis
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26
Q

Definition of oligoarthritis

A

Arthritis affecting 1-4 joints during the 1st 6 mo of disease. Two subcategories:

  1. Persistent - affecting ≤4 joints throughout disease course
  2. Exended - affecting >4 joints after 1st 6 mo

large joints of the lower extremities

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27
Q

Definition of polyarthritis

A

Arthritis affecting ≥5 joints during the 1st 6 mo of disease
May be RF negative or
RF positive: ≥2 tests for RF at least 3 mo apart during the 1st 6 mo of disease are positive

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28
Q

Psoriatic arthritis

A

Arthritis and psoriasis, or arthritis and at least 2 of the ff:

  1. Dactylitis
  2. Nail pitting and onycholysis
  3. Psoriasis in a 1st-degree relative
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29
Q

Enthesitis-related arthritis

A

Arthritis and enthesitis or arthritis or enthesitis with at least 2 of the ff:

  1. History of sacroiliac joint tenderness or inflammatory lumbosacral pain or both
  2. Presence of HLA-B27 antigen
  3. Onset of arthritis in a male >6 yo
  4. Acute (symptomatic) anterior uveitis
  5. History of anykylosing spondylitis, enthesitis-related arthritis, sacroiliitis with IBD, Reiter syndrome or acute anterior uveitis in a 1st-degree relative
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30
Q

Rare but potentially fatal complication of sJIA, also referred to as secondary hemophagocytic syndrome or hemophagocytic lymphohistiocytosis

A

Macrophage activation syndrome

  • falling platelet count
  • falling leukocyte count
  • extreme hyperferritinemia
  • macrophage hemophagocytosis in the BM
  • increased liver enzymes
  • persistent continuous fever ≥38C
  • falling ESR
  • hypofibrinogenemia
  • hypertriglyceridemia
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31
Q 
Naproxen 
Indication
Classification
Dose
Adverse reaction
A

Naproxen
I: JIA (poly, systemic, oligo), spondyloarthropathy, pain, serositis, cutaneous vasculitis, uveitis
C: NSAID
D: 15 mg/kg/d PO in 2 divded doses, max 1000 mg/day
AR: GI intolerance, gastritis, hepatitis, tinnitus, anemia, pseudoporphyria, aseptic meningitis, headache, renal disease

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32
Q 
Etodolac
Indication
Classification
Dose
Adverse reaction
A 
Etodolac
I: JIA, spondyloarthropathy, pain, serositis, cutaneous vasculitis, uveitis
C: NSAID
D: PO once-daily dose:
20-30 kg: 400 mg
31-45 kg: 600 mg
46-60 kg: 800 mg
>60 kg: 1000 mg
AR: GI intolerance, gastritis, hepatitis, tinnitus, anemia, pseudoporphyria, aseptic meningitis, headache, renal disease
33
Q 
Celecoxib
Indication
Classification
Dose
Adverse reaction
A

Celecoxib
I: JIA, spondyloarthropathy, pain, serositis, cutaneous vasculitis, uveitis
C: NSAID
D: 10-25 kg: 50 mg PO BID;
>25 kg 100 mg PO BID
AR: GI intolerance, gastritis, hepatitis, tinnitus, anemia, pseudoporphyria, aseptic meningitis, headache, renal disease

34
Q 
Ibuprofen
Indication
Classification
Dose
Adverse reaction
A

Ibuprofen
I: JIA, spondyloarthropathy, pain, serositis, cutaneous vasculitis, uveitis
C: NSAID
D: 40 mg/kg/d PO divided 3 times daily
max 2400 mg per day
AR: GI intolerance, gastritis, hepatitis, tinnitus, anemia, pseudoporphyria, aseptic meningitis, headache, renal disease

35
Q

Joint involvement highly suggestive of enthesitis-related arthritis

A

tarsitis

36
Q

Joint involvement highly suggestive of psoriatic arthritis

A

distal interphalangeal joints

37
Q

Modified NY criteria for diagnosing ankylosing spondylitis

A
  1. Radiographic evidence of sacroiliitis
    - sacroiliitis of grade2 or greater bilaterally or at least grade 3 unilaterally
  2. Meets at least 1:
    - limitation of motion in the lumbar spine
    - limitation of chest expansion
38
Q

Axial spondyloarthritis criteria (Assessment of Spondyloarthritis International Society)

A
  1. At least 3 months of back pain
  2. Sacroiliitis on imaging (MRI)
    plus 1 feature of spondyloarthritis
    - inflammatory back pain
    - arthritis
    - enthesitis (heel)
    - uveitis
    - dactylitis
    - psoriasis
    - IBD
    - good response to NSAID
    - family history
    OR HLA-B27 positive with at least 2 features
39
Q

Gold standard for early visualization of sacroiliitis

A

Evidence of bone marrow edema adjacent to the joint on MRI with short T1 inversion recover (STIR) sequences

40
Q

Arthritis that occurs following enteropathic or urogenital infections

A

Reactive arthritis

41
Q

Probable etiologic microorganisms of reactive arthritis

A 
Chlamydia
Shigella
Salmonella
Yersinia
Campylobacter
42
Q

Symptoms of reactive arthritis begin approximately __ following infection

A

2-4 weeks

43
Q

Nonsuppurative arthritis, usually in adolescent boys, in association with severe truncal acne.

A

Pyogenic (sterile) arthritis, pyoderma gangrenosum and acne (Cystic) syndrome

44
Q

__ of patients with reactive arthritis are HLA-B27-positive

A

75%

45
Q

Deposition of immune complexes within the derma-epidermal junction on immunofluorescence examination

A

lupus band test

46
Q

SLICC 2012 Criteria

A 
4 criteria, at least 1 clinical, 1 immunologic 
Serositis
Oral ulcers
Arthritis (synovitis)
P
Blood 
Renal
Alopecia
Immunologic criteria
Neurologic
47
Q

Hematologic values in SLE clinical criteria

A

Leukopenia <4,000 or lymphopenia <1,000

Thrombocytopenia <100,000

48
Q

Immunologic criteria

A 
ANA
Anti-dsDNA
Anti-Smith
Antiphospholipid antibody
Low complement
Direct Coomb's in the absence of hemolytic anemia
49
Q

Blue-violet discoloration of the eyelids that may be associated with periorbital edema

Diagnosis?

A

Heliotrope rash

Juvenile dermatomyositis

50
Q

Erythema seen over the chest and neck after UV light exposure

Diagnosis?

A

Shawl sign

Juvenile dermatomyositis

51
Q

Bright pink or pale, shiny, thickened or atrophic plaques over the proximal interphalangeal joints and distal interphalangeal joints and occasionally on the knees, elbows, small joints of the toes and ankle malleoli

A

Grotton papules

Juvenile dermatomyositis

52
Q

Thickened erythematous and scaly rash over the palms

A

mechanic’s hands
Juvenile dermatomyositis
Associated with anti-Jo-1 antibodies

53
Q

Diagnostic criteria for JDM

A

Classic rash: heliotrope rash of the eyelids, Grotton papules
Plus 3 of the ff:
1. Weakness - symmetric, proximal
2. Muscle enzyme elevation (≥1) - CK, AST, LDH, aldolase
3. EMG changes - short, small polyphasic motor unit potentials; fibrillations; positive sharp waves; insertional irritability; bizarre, high-frequency repetitive discharges
4. Muscle biopsy - necrosis, inflammation

54
Q

Most common subtype of localized scleroderma

A

linear scleroderma

55
Q

Triphasic sequence of Reynaud phenomenon

A
  1. Blanching (vasoconstriction)
  2. Cyanosis (venous stasis)
  3. Erythema (vasodilation)
56
Q

Most common pharmacologic intervention for Reynaud phenomenon

A

calcium channel blockers
nifedipine 30-60 mg sustained release daily
amlodipine 2.5-10 mg daily

57
Q

Most frequent initial symptom of Behcet disease

A

oral ulcer

58
Q

Diagnostic criteria for Behcet disease

A

Oral ulcers at least 3 times per year plus 2 other major features:

  1. recurrent genital ulcers
  2. eye lesions
  3. characteristic skin lesions
  4. pathergy
59
Q

Most frequent vasculitic feature of Behcet disease

A

deep venous thrombosis of the lower limbs

60
Q

Most common CNS manifestation of Behcet disease in children

A

dural sinus thrombosis

61
Q

Definition of giant coronary artery aneurysm

A

> 8 mm internal diameter

greatest risk for rupture, thrombosis, stenosis, MI

62
Q

Diagnostic criteria of Kawasaki

A 
Conjunctival injection, nonexudative
Rash, polymorphous
Adenopathy, cervical, >1.5 cm
Strawberry tongue, lip cracking
Hand and feet edema, erythema, desquamation (2-3 weeks
Burn (fever ≥38.3, at least 5 days)
63
Q

3 clinical phases of Kawasaki disease

A
  1. Acute febrile phase (1-2 wk)
  2. Subacute phase (3 wk) - desquamation, thrombocytosis, CAA, sudden death
  3. Convalescent phase (6-8 wk) - until ESR becomes normal
64
Q

Timing of echocardiography in Kawasaki disease

A

2Decho should be performed at diagnosis and again after 2-3 of illness. If results are normal, a repeat study should be performed 6-8 wk after onset of illness. If normal repeat after 1 year. If normal repeat every 5 years.

65
Q

Treatment of acute Kawasaki disease

A

IVIG 2g/kg. If IVIG resistant, (still febrile after 36 hr), give another dose
high dose aspirin 80-100 mg/kg/d divided q6 until afebrile for at least 48 hr
within 10 days of disease and ideally as soon as possible after diagnosis

66
Q

Treatment of convalescent stage Kawasaki disease

A

Aspirin 3-5 mg/kg/d once daily PO until 6-8 wk after illness onset if normal coronary findings throughout course

67
Q

Long-term therapy for patients with coronary abnormalities

A
  1. Aspirin 3-5 mg/kg/d

2. Clopidogrel 1 mg/kg/d (max 75 mg)

68
Q

Live vaccines should generally be deferred until __ after IVIG therapy

A

11 months

69
Q

Most common vasculitis of childhood

A

Henoch-Schonlein purpura

70
Q

Diagnostic criteria for HSP (American College of Rheumatology)

A

Two of the ff:

  1. palpable purpura
  2. age of onset ≤20 yr
  3. bowel angina (postprandial abdominal pain, bloody diarrhea)
  4. biopsy demonstrating intramural granulocytes in small arterioles and/or venules
71
Q

Diagnostic criteria for HSP (European)

A

Palpable purpura (in absence of coagulopathy or thrombocytopenia) and 1 or more of the ff:

  1. abdominal pain
  2. arthritis or arthralgia
  3. biopsy of affected tissue demonstrating predominant IgA deposition
  4. Renal involvement (proteinuria <3 g/24 hr), hematuria or red cell cast
72
Q

Diagnostic criteria for Takayasu arteritis

A

Angiographic abnormalities (angio) of the aorta or its main branches and at least one of the ff:

  1. decreased peripheral artery pulses and .or claudication
  2. blood pressure difference between arms or legs of >10 mmHg
  3. bruits over the aorta and/or its major branches
  4. hypertension
  5. elevated ESR/CRP
73
Q

Polyarteritis nodosa

Histopathology

A

necrotizing vasculitis in medium or small arteries

74
Q

Polyarteritis nodosa

Angiographic abnormalities

A

“beads on a string”

aneurysm, stenosis or occlusion of a medium or small size artery

75
Q

Polyarteritis nodosa

Cutaneous findings

A

livedo reticularis, tender subcutaneous nodules, superficial skin ulcers, deep skin ulcers, digital necrosis, nail bed infarctions or splinter hemorrhages

76
Q

Polyarteritis nodosa

Muscle involvement

A

Myalgia or muscle tenderness

77
Q

Granulomatosis with polyangiitis

A

Wegener granulomatosis
3/6 criteria
1. histopathology showing granulomatous inflammation
2. upper airway involvement
3. laryngeal, tracheal or bronchial involvement
4. ANCA positivity
5. renal involvement
6. Proteinuria, hematuria, red blood cell casts, necrotizing pauci-imune glomerulonephritis

78
Q

Management of ANCA vasculitides

A

corticosteroids 2 mg/kg/d oral or 30 mg/kg/d x 3 days IV
in conjunction with cyclophosphamide 2 mg/kg/d
transition to MTX, azathioprine, MM within 3-6 months once remission is achieved
PCP prophylaxis

PPS 2018 (17 decks)

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