Rheumatology Flashcards

1
Q

Chronic multi-organ autoimmune disorder

A

SLE

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2
Q

SLE age

A

young (reproductive years) adult females; african american > caucasion

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3
Q

SLE

A

effects skin, joint, kidneys, lungs, nervous system, CV; unpredictable flares and remission; mediated by anitbody formation and creation of immune complexes (Ab/antigen complexes); complexes deposit in and damage tissue

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4
Q

ANA antibodies

A

SLE; deposit in and damage tissues

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5
Q

SLE Etiology factors

A

Genetic, hormonal, immunologic, environmental

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6
Q

Clinical presentation of SLE

A

General: fever, FATIGUE, LAD, weight loss
Skin: malar rash, discoid ride
Mucocutaneous: PAINLESS oral and nasal ulcers
Alopecia
Raynaud phenomenon

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7
Q

Raynaud phenomenon

A

vasospasm of digital arteries with exposure to cold temps or stress

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8
Q

Erythematous ptches with keratotic scaling in sun exposed areas

A

discoid (SLE)

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9
Q

fixed erythema over nasal bridge, sparing nasal bridge

A

Malar (Butterfly rash) (SLE)

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10
Q

Raynaud Sx

A
worsens with cold and stress; fingertips
Bicentennial Disease (red, white, blue):
1. White (pallor)
2. Blue (cyanosis)
3. Red (erythema following rewarming)
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11
Q

Tx for Raynaud

A

Calcium channel blocker

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12
Q

Major organ system of SLE

A

nephritis with proteinuria (U/A)

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13
Q

SLE effects almost every organ

A

Heart, lungs (pleuritis, HTN), GI, Kidney (nephritis), MSK (joint pain, migratory, polyarticular, symmetrical), Neuro (seizures, depression)

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14
Q

Joint pain in SLE

A

migratory, polyarticular, symmetrical

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15
Q

Dx of SLE

A

clinical judgement; pt meets criteria

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16
Q

ACR criteria for lupus

A

malar, discoid, photosensitivity, mucosal ulcer, renal disorder, neuro disorder, heme disorder, +ANA, +anti-DNA, +anti-sm +anitphospholipid Ab (have at least 4

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17
Q

other clinical manifestations of SLE

A
coronary artery disease- at risk for MI, accelerated artheroslerosis;
opthalmologic involvment (One main Tx of RLE causes retinal toxicity)
Antiphosphlipid syndrom (recurrent fetal loss, arterial, venous thromboemobolic events)
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18
Q

Antiphospholipid syndrome

A

recurrent fetal loss, thromboembolic events

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19
Q

At risk of MI

A

SLE

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20
Q

Recurrent fetal loss

A

SLE

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21
Q

Labs for SLE

A

+ ANA (cardinal feature, not specific)

ANA suptypes

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22
Q

ANA parts in SLE

A

Titer (how much) 1:40
Staining pattern: loosely assocaited with underlying autoimmune disease. Not specific, Requires additional testing (homogenous, speckled, nucleolar, centromere)

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23
Q

ANA Subtypes

A

Anti-dsDNA
Anti-Sm
Antiphospholipid (anticardiolipid Ab, Beta 2 glycoprotien Ab, Lupus anticoagulant)

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24
Q

More Labs for SLE

A

CBC: anemia, leuipenia and/or thrombocytopenia
Serum creatinine: elevated w/ renal dysfunciton
LFTs
ESR/CRP: elevated w/ inflammation
C3 and C4- low complement levels indicate active lupus
ANA (+96%)

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25
Q

Imaging for SLE

A
x-ray of involved joints
renal U/S
CXR
EKG
echo
CT/MRI
biopsies of involved organ system
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26
Q

Nonpharm tx for SLE

A
SMOKING CESSATION
sun protection
diet/nutrition
exercise
immunizations
tx of comorbid conditions
pregnancy and contraception
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27
Q

Tx of SLE

A

Antimalarials (1st line)- Hydroxychloroquine- REQUIRES OPTHAMOLOGIC FOLLOW UP

Add based on severity:
NSAIDs, systemic steroid, immunosuppresives (Methotrexate, azathioprine, Monoclonal Ab: Belimumab or Rituximab (reserved for resistant disease)

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28
Q

Retinal ototoxicity

A

Hydroxychloroquine

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29
Q

Resistant SLE tx

A

Belimumab and Rituximab

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30
Q

Drug induced SLE

A

similar sx to SLE

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31
Q

Drugs that may lead to Drug Induced SLE

A

Procainamide, isoniazid, hydralazaine, chlorpromaxine, methyldopa, minocycline, and anti-TNF agents

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32
Q

Labs for drug-induced lupus

A

Positive antihistone antibody (negative anti-dsDNA, anti-Sm ab)

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33
Q

Positive antihistone antibody

A

Drug-induced SLE

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34
Q

Tx for drug induced SLE

A

Stop offending drug!

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35
Q

Poor dx of SLE

A

renal disease, HTN, male, anitphosholipid Ab, high overall disease activity, low SES

36
Q

Death due to SLE

A

systemic disease activity (renal, CNS)

infections

37
Q

When to refer SLE

A

Dx and management requires active participation of rheumatologist
severity of organ involvement dictates referral to other subspecialists

38
Q

When to admit for SLE

A

severe organ-threatening manifestations

severe infections, especially in the setting of immunosuppressant therapy

39
Q

Polymyositis

A

idiopathic progressive, inflammatory condition causing SYMMETRIC PROXIMAL MUSCLE WEAKNESS (deltoid and hip flexors)

40
Q

Common site of weakness in polymyositis

A

Deltoid (difficulty reaching high shelf) and hip flexors (difficult arising from chair)

41
Q

Polymyositis

A

F>M, 40-50 YO, Blacks > whites, gradual onset over weeks to months

42
Q

Polymyositis Sx

A

progressive weakness of proximal extremities; usually painless;
Lung: Cough/SOB (interstitial lung disease - scarring of lung tissue)
Raynaud phenomenon

43
Q

Raynaud phenomenon

A

SLE, Polymyositis, Sjogren’s, Systemic Sclerosis

44
Q

Interstitial lung disease is associated with

A

Polymyositis

45
Q

Complications of Polymyositis

A

Dysphagia, myocarditis, aspiration pneumonia

46
Q

Dermatomyositis

A

polymyositis + Cutaneous manifestations

47
Q

Sx of dermatomyositis

A

Polymyositis sx + cutaneous eruptions (heliotrope rash, Gottron’s papules, shawl sign); loo for OCCULT MALIGNANCY (cervix, lung, ovary, stomach)

48
Q

Occult malignancy

A

important to check in people with dermatomyositis (cervix, lung, ovary, stomach) - ask about last pap, etc.

49
Q

Heliotrope rash

A

violet color around eyes (dermatomyositis)

50
Q

Gottron’s papules

A

Papules on dorsal aspect of PIP, DIP or MCP (dermatomyositis)

51
Q

Shawl sign

A

Red rash across shoulders (dermatomyositis); worsen’s/darkens with exposure to sun

52
Q

Labs for polymyositis and dermatomyositis

A

Muscle enzymes: CK (creatine kinase) and aldolase elevated

ANA (+)- 80%

CXR, electromyography, MRI, muscle bx

53
Q

Tx for polymositis and dermatomyositis

A

Glucocorticoids (1st line)

Severe cases: Azathioprine or Methotrexate

PT/OT/Speech

54
Q

Immunosuppressants

A

Azathioprine or Methotrexate

55
Q

Sjogren’s Syndrome

A

chronic systemic autoimmune disorder, idiopathic, can be primary or secondary (SLE, RA< Systemic sclerosis), F>M, 40-60 YO

56
Q

What is Sjogren’s

A

chronic dysfunction of exocrine glands (lacrimal and salivary)

57
Q

Sicca complex

A

Combo of xerophthamlmia and xerostomia (Sjogrens)

58
Q

Clinical Presentation of Sjogren’s

A

fatigue; keratoconjunctivitis sicca (dry eyes), xerostomia (recurrent parotiditis), dryness elsewhere

59
Q

Extraglandula features of Sjogren’s

A
ARTHRALGIA/ARTHRITIS
raynaud
LAD
pulmonary disease
vasculitis
nephritis
lymphoma
60
Q

Dx of Sjogren’s

A
Shirmer test (tear production)
\+ ANA (+Anti-Ro, Anti-La)
\+RF
ESR, CBC
renal/liver/thyroid function tests, UA
salivary gland bx (lymphocytic infiltraton and destruction of glandular tissue)
61
Q

Anti-sjogren’s antibodies

A

Anti-Ro/SS-A, Anti-La/SS-B (primary)

62
Q

Tx of Sjogren’s

A

f/u with dentist and opthalmologist
Artificial tears, cyclosporin drops (Restasis)
-Xerostomia- saliva subsitute (Biotene OTC), frequent water, sugarless candy
-Extraglandular:
NSAIDs
steroids/immuosupressive for severe
Consider refer to rheumo

63
Q

Polyarteritis Nodosa (PAN)

A

Systemic necrotizing vasculitis; inflammation of MUSCULAR ARTERIES; doesn’t affect veins; results in thrombosis, ischemia or infart

64
Q

PAN commonly affects

A

kidneys, skin, joints, muscles, nerves, GI

65
Q

Prevalence of PAN

A

M>F, middle age to older (peak 6th decade)

66
Q

Cause of PAN

A

usually idiopathic

Can be triggered: HBV (most typical), HCV - do hep B panel

67
Q

Presentation of PAN

A

fever, weight loss, malaise
SENSORY & MOTOR POLYNEUROPATHY
arthralgia/myalgia
Cutaneous: nodules, ulcers, purpura (painful) (Bx reveals leukocytoclastic vasculitis)
Systemic:
Renal infarcations (proteinuria, hematuria, and renal insufficiency), HTN

68
Q

Bx of skin lesion in PAN

A

leuocytoclastic vasculitis

69
Q

Systemic manifestations in PAN

A

renal infarctions

HTN

70
Q

Labs for PAN

A

no dx labs
ESR, CRP elevated
Serum creatinine, CK, LFTs, Hepatitis serology, U/A
NEGATIVE ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES (ANCA) (+ANCA = other forms of vasculitis)
Bx of affected organ
Arteriography: microaneurysms

71
Q

Importants Dx for PAN

A

(-) ANCA

Microaneurysms on arteriograph

72
Q

Tx of PAN

A

Mild/cutaneous: Glucocorticoids
Moderate/Severe: Glucocorticoids + immunosuppressive

Associated with Hep B/C: primarily antiviral +/- glucocorticoid

HTN: ACE-I

Maintenance Regimens:
steroid taper
Immunosupressants

73
Q

Represents a spectrum of diseases

A

PAN

74
Q

Systemic Sclerosis (Scleroderma)

A

autoimmune disorder that causes diffuse fibrosis (tightening and thickening) of the skin and internal organs (narrowing of small blood vessels)

75
Q

Cause of Scleroderma

A

unknown; immune mechanism that leads to vascular endothelial damage and activation of fibroblasts (CT)

76
Q

More common in males

A

PAN

77
Q

Prevalence of Scleroderma

A

F

78
Q

2 Types of Scleroderma

A
  1. Limited cutaneous

2. Diffuse cutaneous

79
Q

Limited cutaneous

A

CREST syndrome;

Skin changes limited to hands, forearm, face, neck; better prognosis

80
Q

Diffuse cutaneous SS

A

sclerotic skin also on chest, abdomen, arms, shoulders; increased risk of internal organ involvement

81
Q

Sx of systemic sclerosis

A

(CREST)
Calcinosis
Raynaud’s
Esophageal dysfunction (reflux, decrease in motility)
Sclerodactyly - thickening and tightening of skin on fingers and hands (diffuse puffy, shiny hands)
Telangiectasia

82
Q

Manifestations of SS

A
cutaneous (universal)
arthralgias
pulmonary (fibrosis, HTN)
cardiac (pericarditis, cardiomyopahty)
Renal (HTN, renal failure)
83
Q

Pulmonary HTN

A

SLE, PAN, SS

84
Q

Labs for SS

A

(+) ANA
(+) ACA (anti-cenromere ab) (HIGHLY SPECIFIC FOR SSc)
Anti-SCL-79 (scleroderma ab, aka anti-topoisomerase I)
CBC: mild anemia
UA: proteinuria- renal involvement

85
Q

Specific lab results for SS

A

(+) anit-centromere ab (ACA)

(+) Anti-SCL-70

86
Q

Tx of SS

A

Symptomatic
Education- warm clothing, smoking cessation
Raynauds- Nifedipine (calcium channel blocker)
Esophageal disease: H2 blocker, proton pump inhibitors, small, more frequent meals
Renal and HTN: ACE-I
Immunosuppressant in severe cases

87
Q

Calcium channel blocker

A

Nifedipine