Rheumatology - Hurley Flashcards

1
Q

what is rheumatoid arthritis?

A

chronic inflammatory d/o that MAINLY attacks joints producing INFLAMMATORY SYNOVITIS

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2
Q

what does rheumatoid arthritis produce?

A

inflammatory synovitis

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3
Q

what is rheumatoid arthritis highlighted by?

A

severe pain

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4
Q

name of rheumatoid arthritis comes from?

A

rheumatic fever

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5
Q

what organs (besides joints) can RA affect?

A

lungs, pericardium, pleura, sclera

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6
Q

what cells play a role in RA?

A

APC, B cells, MHC, CD4+ T cells

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7
Q

what does RA’s pain intensity and deterioration of joint structures lead to?

A

deformities and disabilities

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8
Q

what race is affected more with RA? smokers or non-smokers affected more? gender?

A

Native Americans

Smokers more

Women 3x more

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9
Q

what happens to synovial membrane in RA?

A

becomes hyperplastic

gets infiltrated with immune and inflammatory cells (macrophages, B and T cells, dendritic cells, plasma cells)

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10
Q

what cells play a role in the continuous inflammation of RA?

A

increased level of cytokines

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11
Q

pts with what genetic sequence have higher RA and anti-CCP positivity? what MGC class is it?

A

HLA-DRB: “shared epitope” -> MHC class 2

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12
Q

what is synovitis?

A

inflammation of the synovial membrane that lines the joints and tendon sheaths

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13
Q

what happens to joints with synovitis?

A

joints become swollen, tender, warm, and stiff -> limited movement

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14
Q

does RA affect multiple or single joints?

A

MULTIPLE

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15
Q

what joints is RA commonly seen in?

A

small joints of hands, feet, cervical spine

larger joints - shoulders and knees

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16
Q

where is RA NOT seen in the back?

A

lower back

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17
Q

joints in RA are affected in what pattern?

A

symmetrical fashion

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18
Q

what is characteristic of RA?

A

rheumatoid nodule that’s subcutaneous

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19
Q

what is the central of the rheumatoid nodule like?

A

central area is of fibroid necrosis (pattern looks like fibrin)

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20
Q

what surrounds the central necrosis in the rheumatoid nodule?

A

layer of palisading macrophages and fibroblasts

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21
Q

how big is the rheumatoid nodule and where is it found?

A

3mm-few cm in diameter

found over bony prominences

  • the olecranon
  • the calcanea tuberosity
  • the meta-carpophalangeal joints (hands)
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22
Q

sx’s of cervical spine affected by RA?

A

neck stiffness and general loss of motion

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23
Q

sx’s of shoulders affected by RA?

A

loss of motion

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24
Q

sx’s of elbow affected by RA?

A

evidence synovitis - palpate fullness and thickening in the radiohumeral joint

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25
Q

sx’s of hand and wrist affects by RA?

A

wrists ALWAYS affected

MCP and PIP joints involved

DIPS ARE SPARED

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26
Q

what joints in the hands are spared in RA?

A

DIPS

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27
Q

what joint in foot/ankle are affected by RA?

A

MTP, talonavicular and ankle joints

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28
Q

what CLASSIC hand deformities are seen in RA?

A

Swan neck deformity (flexion at DIP and hyperextension at PIP)

Boutonniere deformity (flexion at PIP and hyperextension of DIP)

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29
Q

what happens to the lungs in RA?

A

develop excess fibrous connective tissue

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30
Q

what happens to the eyes in RA?

A

keratoconjunctivitis sick, episcleritis, sleritis

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31
Q

what happens to the skin in RA?

A

rheumatoid nodules, dermal vasculitis lesions

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32
Q

what happens to the heart in RA?

A

pericardial effusion but no sx’s

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33
Q

what happens to the nervous system in RA?

A

instability of C1-C2, peripheral nerve entrapment and vasculitis -> results in mono neuritis multiplex

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34
Q

what happens to the blood in RA?

A

hypochromatic-microcytic anemia with low serum ferritin or normal iron-binding capacity

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35
Q

cardiac complications of RA?

A

MI, stroke, atherosclerosis, pericarditis, endocarditis, left ventricular HF, vasculitis

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36
Q

what is seen on PE of RA?

A

warm tender erythematous joints, hands, and wrists

ulnar deviation of digits

Boutonniere’s deformity

Rheumatoid nodules

Baker’s cyst (cyst in popliteal space)

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37
Q

criteria for classification of RA

A

at least 4 must be met:

  • morning stiffness >1 hr for most mornings for 6 weeks
  • arthritis and soft tissue swelling of more than 3 of 14 joints, present for at least 6 weeks
  • swelling of hand joints, present for at least 6 weeks
  • symmetric joint swelling for at least 6 weeks
  • subcutaneous nodules
  • Rheumatoid factor level above 95th percentile
  • joint erosion seen on radiology
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38
Q

what is the BEST initial dx step for RA?

other dx for RA?

A

radiographs - x-ray or CT

others:

  • Rheumatoid factor
  • anti-cyclic citrullinated peptide antibody (MOST SPECIFIC)
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39
Q

what is MOST SPECIFIC for Ra dx?

A

anti-cyclic citrullinated peptide antibody

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40
Q

if pt has only 1 inflamed joint, or asymmetric joint inflammation, what should you check to r/o RA?

A

check synovial fluid to r/o RA

-may be SEPTIC ARTHRITIS

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41
Q

what is the rheumatoid factor (RF)? if negative does it rule out RA?

A

RF is a specific antibody in the blood

if negative RF, does NOT r/o RA (common to be negative in 1st year of disease)

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42
Q

what is RF negative arthritis called? when is it common to be negative?

A

SERONEGATIVE

common to be seronegative in the 1st year of the disease

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43
Q

when does damage of RA occur in most pts? death?

A

damage occurs early

  • joint space narrowing in first 2 years
  • disabled at 10 years

death comes early - women lose 10 years of life, men 4

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44
Q

what is the MAINSTAY of tx for RA?

A

Methotrexate (MTX) with or without TNF inhibitors (infliximab, etanercept, adalimumab)

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45
Q

newly dx RA pts may need what meds to hold over until MTX works?

A

corticosteroids

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46
Q

what corticosteroid and when can corticosteroids be used in RA?

A

Prednisone can be used to achieve rapid response or when there are “flares”

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47
Q

what meds are FIRST LINE for pain in RA? intra-articular injection of?

A

NSAIDs - FIRST LINE FOR PAIN

intra-articular injection of Triamcinolone

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48
Q

when is hydroxychlorquine used with MTX for RA? what is it NOT considered?

A

used early in mild disease

NOT considered a true DMARD b/c doesn’t slow progression

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49
Q

what is the DMARD of choice for tx of RA?

A

Methotrexate

50
Q

MOA of methotrexate?

A

inhibits DNA synthesis thru inhibition of dihydrofolate reductase -> decreasing de novo production of purines and pyrimidines

induces adenosine release

51
Q

monitoring of DMARDS? what are sites of adrs? monitoring when?

A

frequent monitoring - 4-8 weeks intervals

blood, liver, lung, and kidneys are frequent sites of adrs

see pt 3-6x/year and if get ill, see more urgently

52
Q

MAIN complication of RA?

A

Felty’s syndrome

53
Q

what is Felty’s syndrome?

A

complication of RA

Triad of RA + Neutropenia + splenomegaly

54
Q

how do you dx Felty’s syndrome?

A

on PE and CBC

55
Q

when does Felty’s syndrome resolve?

A

when treat RA

56
Q

if pt with Felty’s syndrome and fever, how do you treat?

A

treat with neutropenic precautions and IV abx

57
Q

why is spleen enlarged in Felty’s syndrome?

A

d/t inflammation

58
Q

other complications of RA besides Felty’s syndrome?

A

Baker’s cysts, Carpal Tunnel syndrome

Atlanto-axial subluxation

59
Q

if pt has Atlanto-axial subluxation, what should they be cautious with? avoid who? what can they develop?

A

caution with cervical motion, avoid chiropractors, can develop hand and foot paresthesia and paraplegia

60
Q

mechanism of Tocilizumab?

A

blocks IL-6

61
Q

mechanism of Abatacept?

A

inhibits co-stimulation of T cells by blocking CD28-CD80 and signaling of APC cells

62
Q

mechanism of Rituximab?

A

targets B cells, deplete B cells, no inflammation

63
Q

mechanism of Tofacitinib?

A

JAK1 and 3 inhibitors

64
Q

what is polymyalgia rheumatica?

A

idiopathic, inflammatory d/o of pain associated with PROXIMAL muscles (shoulder, hip, neck)

65
Q

onset of polymyalgia rheumatica? self-limiting?

A

abrupt onset and self-limiting

66
Q

unlike polymyositis and dermatomyositis, key feature of polymyalgia rheumatica is what?

A

PAIN, not weakness

67
Q

when are sx’s of polymyalgia rheumatica worse?

A

AM

68
Q

high correlation b/w polymyalgia rheumatica and what?

A

temporal arteritis

69
Q

who gets polymyalgia rheumatica?

A

elderly (50+)

70
Q

what will pt with polymyalgia rheumatica describe about their sx’s?

A

general aches and pains, stiffness, especially upper arms, thighs, hip girdle, and shoulders

TROUBLE GETTING DRESSED

71
Q

does polymyalgia rheumatica cause swollen joints?

A

no!!!

72
Q

strength is ___ in polymyalgia rheumatica

A

intact

73
Q

dx of polymyalgia rheumatica

A

dx of exclusion - made clinically

74
Q

best test for polymyalgia rheumatica?

A

ESR (will be increased)

75
Q

what is the FIRST LINE tx of polymyalgia rheumatica?

A
oral corticosteroids (lowest effective dose)
-Prenisone
76
Q

since polymyalgia rheumatica has high correlation with temporal arteritis, what should all pts have?

A

a temporal artery bx or consult with ophthalmologist

77
Q

what is reactive arthritis?

A

autoimmune response that can occur post-infection (typically GU or GI infections)

78
Q

what GU/GI infections are associated with reactive arthritis?

A

Chlamydia, campylobacter, salmonella, shigella, yesinia

79
Q

what protein is found on surface of wbcs and pre-disposes you to autoimmune diseases like Reactive Arthritis?

A

HLA-B27 - positive in Reactive Arthritis test

80
Q

what is the classic triad of reactive arthritis?

A

non-infectious urethritis, arthritis, conjunctivitis

CAN’T SEE, CAN’T PEE, CAN’T CLIMB A TREE

81
Q

dx of reactive arthritis

A

no dx test to confirm

dx based on hx and clinical findings
-CBC and CRP increased, UA, blood, urine, stool and wound cultures r/o infection, HIV testing, echo for aortic regurg

HLA-B27 + in 75%

82
Q

tx for reactive arthritis

A

Abx (if infection), NSAIDs, corticosteroids

DMARDs (when NSAIDs and steroids don’t work) - use Sulfasalazine or MTX

83
Q

what DMARDs are used in reactive arthritis when NSAIDs and steroids don’t work?

A

Sulfasalazine or MTX

84
Q

what is one of the leading causes of preventable visual loss in developed countries?

A

anterior uveitis (seen in reactive arthritis)

85
Q

sx’s of anterior uveitis?

A

red eye, pain worsens when reading, progressive, blurred vision, photophobia, excess tear production, abnormally shaped pupils

86
Q

what is juvenile idiopathic arthritis? primary pathology?

A

arthritis in pts <16 y/o (commonly 1-6 y/o)

primary pathology is inflammation of the connective tissues (autoimmune, non-infectious, inflammatory condition)

87
Q

what is the FIRST sx of uvenile idiopathic arthritis?

A

limping

88
Q

how many joints does juvenile idiopathic arthritis involve?

A

1 or many joints

89
Q

what is often seen with juvenile idiopathic arthritis?

A

fever and rash

90
Q

duration of juvenile idiopathic arthritis? when does it resolve?

A

> 6 weeks

-resolves by puberty

91
Q

juvenile idiopathic arthritis is characterized by what sx’s?

A

major changes in joints including inflammation, contractures and joint damage which affect mobility, strength, and endurance

lethargy, reduced activity, poor appetite

92
Q

all children with juvenile idiopathic arthritis experience what?

A

experience periods when sx’s reduce in severity or disappear, but may go from sx free to extreme pain quickly

93
Q

what are extra-articular sx’s of juvenile idiopathic arthritis?

A

iridocyclitis (inflammation of iris and ciliary body), growth disturbances, leg length discrepancies

94
Q

when all children go sx free with juvenile idiopathic arthritis, what should they be encouraged to participate in?

A

developmental activities

95
Q

4 types of juvenile idiopathic arthritis?

A

systemic onset arthritis

polyarticular arthritis

oligoarticular arthritis

enthesis-related arthritis

96
Q

sx’s of systemic onset arthritis?

A

repeated fevers to 103F w/fluctuating salmon colored rash

inflammation of the internal organs and joints, anemia, and leukocytosis

97
Q

sx’s of polyarticular arthritis?

A

arthritis in 5 or more joints with major sx’s of pain in the knees, ankles, wrists, fingers, elbows, and shoulders

CONSTANT PAIN

98
Q

sx’s of oligoarticular arthritis?

A

arthritis in 4 or fewer joints w/in the first 6 months of onset

females > males

those dx before age 7, best chance of disease subsiding

large joints (knees, ankles, elbow, wrists) affected

99
Q

what is anthesis-related arthritis? aka?

A

form of juvenile idiopathic arthritis that involves the ligaments as well as spine

aka spondyloarthritis

100
Q

work-up for juvenile idiopathic arthritis?

A
  • CBC (normal)
  • ESR (normal)
  • ANA (positive)
  • RF (negative)
  • Synovial fluid (Class 2 - inflammatory)

-X-rays (soft tissue swelling, periarticular osteoporosis, growth disturbances, loss of joint space)

101
Q

tx of juvenile idiopathic arthritis

A

refer to rheumatology

sx control, prevent joint damage and maintain fxn

intra-articular steroid injections if only a few joints involved

PO prednisone (lots of adrs)

DMARDs +/- biologics

102
Q

what are 3 inflammatory myopathies?

A

polymyositis, dermatomyositis, and inclusion body myositis

103
Q

what is polymyositis?

A

immune mediated muscle disease that involves inflammation of the muscles and associated tissues in response to cell damage

104
Q

polymyositis associated with? what race?

A

Raynaud’s phenomenon, RA, SLE, Sjogren’s, CV disease, cancer

drugs like D-penicillamine, statins, AZT

black race

105
Q

sx’s of polymyositis

A

PROGRESSIVE SYMMETRICAL PROXIMAL MUSCLE WEAKNESS BILATERALLY

difficult raising arms, lifting objects, combing hair

trouble climbing stairs, getting up from sitting

trouble lifting objects over head

106
Q

systemic sx’s of polymyositis?

A

GI (dysphagia, bloating, constipation)

Cardiac (arrhythmias, conduction defects)

Pulmonary (aspiration pneumonia, interstitial lung disease - anti-jo1, bronchiolitis obliterates)

Renal (ATN)

107
Q

dx work-up for polymyositis

A

CBC - leukocytosis

elevated CK, LDH (muscle enzymes)

elevated liver enzymes

Electromyography (EMG)

muscle bx (looks for inflammation, tissue damage, enzyme deficiencies)

108
Q

FIRST line tx for polymyositis? monitor what?

A

Corticosteroids (Prednisolone) - monitor CK levels, muscle strength

109
Q

SECOND line tx for polymyositis if Corticosteroids fail in 4 weeks?

A

Immunosuppressive agents

  • if no response to steroids in 4 weeks
  • if extra-skeletal manifestations

Aza, cyclophosphamide, chlorambucil, cyclosporine

110
Q

what is dermatomyositis?

A

rash accompanying muscle weakness

111
Q

pathognomonic rashes for dermatomyositis?

A

HELIOTROPE RASH
-purple discoloration on upper eyelids, flat red rash on cheeks and upper trunk

GROTTON’S PAPULES
-raised violaceous scaly rash on knuckles

112
Q

dx of dermatomysotitis?

A

muscle bx reveals perivascular and perimysial inflammation

113
Q

tx for dermatomysoitis?

A

Corticosteroids (Prednisolone)

114
Q

what is inclusion body myositis?

A

> 50 y/o, most common inflammatory myopathy in older pts

115
Q

what makes inclusion body myositis different from polymyositis and dermatomyositis?

A

can have asymmetrical weakness in inclusion body myositis

116
Q

what muscle weakness/atrophy in inclusion body myositis causes falling? what other muscle weakness is common?

A

quadriceps weakness and atrophy -> causes falling

facial muscle weakness (but not ocular)

117
Q

what is VERY COMMON sx of inclusion body myositis?

A

weakness and atrophy of distal muscles (foot extensors and deep finger flexors)

118
Q

pts with inclusion body myositis can’t do what?

A

tie knots or hold golf club

119
Q

what is seen in 60% of cases of inclusion body myositis?

A

dysphagia and choking

120
Q

how long does it take inclusion body myositis to progress? what other disease does it look like?

A

takes years to progress

-looks like MD

121
Q

inclusion body myositis resistant to what therapy?

A

immunotherapy

122
Q

what meds are tried for a few months for inclusion body myositis tx?

A

Aza and Prednisone (low dose)