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
Imaging for SLE
``` x-ray of involved joints renal U/S CXR EKG echo CT/MRI biopsies of involved organ system ```
26
Nonpharm tx for SLE
``` SMOKING CESSATION sun protection diet/nutrition exercise immunizations tx of comorbid conditions pregnancy and contraception ```
27
Tx of SLE
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)
28
Retinal ototoxicity
Hydroxychloroquine
29
Resistant SLE tx
Belimumab and Rituximab
30
Drug induced SLE
similar sx to SLE
31
Drugs that may lead to Drug Induced SLE
Procainamide, isoniazid, hydralazaine, chlorpromaxine, methyldopa, minocycline, and anti-TNF agents
32
Labs for drug-induced lupus
Positive antihistone antibody (negative anti-dsDNA, anti-Sm ab)
33
Positive antihistone antibody
Drug-induced SLE
34
Tx for drug induced SLE
Stop offending drug!
35
Poor dx of SLE
renal disease, HTN, male, anitphosholipid Ab, high overall disease activity, low SES
36
Death due to SLE
systemic disease activity (renal, CNS) | infections
37
When to refer SLE
Dx and management requires active participation of rheumatologist severity of organ involvement dictates referral to other subspecialists
38
When to admit for SLE
severe organ-threatening manifestations | severe infections, especially in the setting of immunosuppressant therapy
39
Polymyositis
idiopathic progressive, inflammatory condition causing SYMMETRIC PROXIMAL MUSCLE WEAKNESS (deltoid and hip flexors)
40
Common site of weakness in polymyositis
Deltoid (difficulty reaching high shelf) and hip flexors (difficult arising from chair)
41
Polymyositis
F>M, 40-50 YO, Blacks > whites, gradual onset over weeks to months
42
Polymyositis Sx
progressive weakness of proximal extremities; usually painless; Lung: Cough/SOB (interstitial lung disease - scarring of lung tissue) Raynaud phenomenon
43
Raynaud phenomenon
SLE, Polymyositis, Sjogren's, Systemic Sclerosis
44
Interstitial lung disease is associated with
Polymyositis
45
Complications of Polymyositis
Dysphagia, myocarditis, aspiration pneumonia
46
Dermatomyositis
polymyositis + Cutaneous manifestations
47
Sx of dermatomyositis
Polymyositis sx + cutaneous eruptions (heliotrope rash, Gottron's papules, shawl sign); loo for OCCULT MALIGNANCY (cervix, lung, ovary, stomach)
48
Occult malignancy
important to check in people with dermatomyositis (cervix, lung, ovary, stomach) - ask about last pap, etc.
49
Heliotrope rash
violet color around eyes (dermatomyositis)
50
Gottron's papules
Papules on dorsal aspect of PIP, DIP or MCP (dermatomyositis)
51
Shawl sign
Red rash across shoulders (dermatomyositis); worsen's/darkens with exposure to sun
52
Labs for polymyositis and dermatomyositis
Muscle enzymes: CK (creatine kinase) and aldolase elevated ANA (+)- 80% CXR, electromyography, MRI, muscle bx
53
Tx for polymositis and dermatomyositis
Glucocorticoids (1st line) Severe cases: Azathioprine or Methotrexate PT/OT/Speech
54
Immunosuppressants
Azathioprine or Methotrexate
55
Sjogren's Syndrome
chronic systemic autoimmune disorder, idiopathic, can be primary or secondary (SLE, RA< Systemic sclerosis), F>M, 40-60 YO
56
What is Sjogren's
chronic dysfunction of exocrine glands (lacrimal and salivary)
57
Sicca complex
Combo of xerophthamlmia and xerostomia (Sjogrens)
58
Clinical Presentation of Sjogren's
fatigue; keratoconjunctivitis sicca (dry eyes), xerostomia (recurrent parotiditis), dryness elsewhere
59
Extraglandula features of Sjogren's
``` ARTHRALGIA/ARTHRITIS raynaud LAD pulmonary disease vasculitis nephritis lymphoma ```
60
Dx of Sjogren's
``` 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
Anti-sjogren's antibodies
Anti-Ro/SS-A, Anti-La/SS-B (primary)
62
Tx of Sjogren's
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
Polyarteritis Nodosa (PAN)
Systemic necrotizing vasculitis; inflammation of MUSCULAR ARTERIES; doesn't affect veins; results in thrombosis, ischemia or infart
64
PAN commonly affects
kidneys, skin, joints, muscles, nerves, GI
65
Prevalence of PAN
M>F, middle age to older (peak 6th decade)
66
Cause of PAN
usually idiopathic | Can be triggered: HBV (most typical), HCV - do hep B panel
67
Presentation of PAN
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
Bx of skin lesion in PAN
leuocytoclastic vasculitis
69
Systemic manifestations in PAN
renal infarctions | HTN
70
Labs for PAN
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
Importants Dx for PAN
(-) ANCA | Microaneurysms on arteriograph
72
Tx of PAN
Mild/cutaneous: Glucocorticoids Moderate/Severe: Glucocorticoids + immunosuppressive Associated with Hep B/C: primarily antiviral +/- glucocorticoid HTN: ACE-I Maintenance Regimens: steroid taper Immunosupressants
73
Represents a spectrum of diseases
PAN
74
Systemic Sclerosis (Scleroderma)
autoimmune disorder that causes diffuse fibrosis (tightening and thickening) of the skin and internal organs (narrowing of small blood vessels)
75
Cause of Scleroderma
unknown; immune mechanism that leads to vascular endothelial damage and activation of fibroblasts (CT)
76
More common in males
PAN
77
Prevalence of Scleroderma
F
78
2 Types of Scleroderma
1. Limited cutaneous | 2. Diffuse cutaneous
79
Limited cutaneous
CREST syndrome; | Skin changes limited to hands, forearm, face, neck; better prognosis
80
Diffuse cutaneous SS
sclerotic skin also on chest, abdomen, arms, shoulders; increased risk of internal organ involvement
81
Sx of systemic sclerosis
(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
Manifestations of SS
``` cutaneous (universal) arthralgias pulmonary (fibrosis, HTN) cardiac (pericarditis, cardiomyopahty) Renal (HTN, renal failure) ```
83
Pulmonary HTN
SLE, PAN, SS
84
Labs for SS
(+) 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
Specific lab results for SS
(+) anit-centromere ab (ACA) | (+) Anti-SCL-70
86
Tx of SS
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
Calcium channel blocker
Nifedipine