CT disorders and Vasculitides Flashcards

1
Q

anti-nuclear Ab test

A

NOT SPECIFIC, indirect immunofluorescence, not used to differentiate types of autoimmune diseases

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2
Q

Populations at risk for autoimmune dz

A

female prevalence, minority populations

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3
Q

What type of hypersensitivity reaction is SLE?

A

Type III, autoab to nuclear ag

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4
Q

Pericarditis presentation

A

substernal, constant, “crushing” chest pain worsens with inspiration and in supine position, pericardial friction rub, diffuse ST elevation

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5
Q

Malar rash pattern

A

erythematous eruption over cheeks and nasal bridge that spares nasolabial folds

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6
Q

SLE serology

A

+ANA, +anti-dsDNA, +anti-smith, low C3 and C4 due to increased consumption

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7
Q

Libman-Sacks Endocarditis general patho

A

Immune complexes deposit on heart valves

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8
Q

SLE treatment regimen

A

avoid sun exposure, NSAIDS, glucocorticoids, hydroxychloroquine

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9
Q

Cause of mortality in early years of SLE

A

infections with opportunistic organisms, kidney or CNS dz

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10
Q

Cause of mortality in later years of SLE

A

accelerated atherosclerosis, thromboembolic events and MI

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11
Q

SLE preventative measures and management

A

minimize risk factors for atherosclerosis, avoid smoking, influenza and pneumococcal vaccine, preventative CA screening, monitor for avascular necrosis and osteoporosis with corticosteroid use

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12
Q

Type I Antiphospholipid Ab

A

causes false-positive for syphilis

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13
Q

Type II Antiphospholipid Ab

A

lupus anticoagulant, risk factor for venous and arterial thrombosis and miscarriage, increases aPTT

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14
Q

Type III Antiphospholipid Ab

A

anti-cardiolipin antibodies, Beta2GPI

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15
Q

When should Abs for APS be measured to diagnose?

A

two occasions 12 weeks apart

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16
Q

Treatment for APS

A

indefinite coagulation

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17
Q

DDX for thrombosis

A

APS, protein S and protein C deficiency, anti-thrombin deficiency, FactorV Leiden deficiency, sepsis, DIC, TTP

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18
Q

SLE/APS retinopathy

A

cotton wool spots

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19
Q

DDX for cotton wool spots

A

hypertension, diabetes, ischemia, infection, trauma, idiopathic

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20
Q

Medications associated with lupus-like syndrome

A

hydralazine, isoniazid, minocycline, TNF inhibitor, methyldopa, quinidine, procainamide, Sulfa abx

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21
Q

DDx for DLE

A

discoid lupus, tinea infection (ring worm), psoriasis, morphea

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22
Q

Hallmarks of scleroderma

A

thickening and hardening of skin, fibrosis of organs, dry skin due to obliteration of eccrine sweat and sebaceous glands

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23
Q

Multisystem affects of SSc

A

lungs, GI, kidney, MSK, Raynaud phenomenon, hyper or hypopigmentation

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24
Q

Localized SSc is seen in what population?

A

children

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25
Localized SSc presentation
discreet areas of discolored skin induration, NOT systemic, no Raynaud's, morphea patches
26
Limited SSc presentation
CREST - Calcinosis cutitis, raynaud's, esophageal dismotitlity, sclerodactyly, telangiectasia; pulmonary HTN
27
Diffuse SSc presentation
systemic, early and progressive internal organ involvement resulting in interstitial lung disease and renal crises
28
Phases of Diffuse SSc
Inflammatory edematous phase then fibrotic; cutaneous manifestations will cause impaired sweating and loss of body hair, fibrosis of joints
29
Skin manifestations of SSc
hyper/hypopigmented, dry/itchy skin, masklike facies, microstomia, perioral furrowing, calcium deposits
30
Primary cause of M&M in SSc
pulmonology manifestations
31
Key pulmonary manifestations of SSc
aspiration pneumonia, interstitial lung disease, pulmonary artery hypertension
32
Common pulmonary manifestation of Diffuse SSc
Interstitial lung disease, chronic dry cough, dyspnea, rales
33
Common pulmonary manifestation of Limited SSc
pulmonary artery hypertension; right heart cath to measure pressure; sxs include dyspnea, syncope, angina, right heart failure
34
Common renal manifestations of SSc
CKD, Renal crises
35
Renal crisis sxs
malignant hypertension, hemolytic anemia, progressive renal insufficency
36
Cardiac manifestations of SSc
myocardial fibrosis, cardiomyopathy, pericarditis, myocarditis, pericardial effusion, arrhythmia
37
GI manifestations of SSc
malnutrition, xerostomia, esophagus (Barrett esophagus), gastroparesis, Gastric antral vascular ectasia, chronic diarrhea, primary biliary cirrhosis
38
MSK manifestations of SSc
carpal tunnel, tendon friction rubs, fibrosis and adhesion of tendon sheaths
39
Thyroid manifestations of SSc
hypothyroidism due to fibrosis
40
Diagnostic workup of SSc
H&P, especially bp; ESR, ANA, serology, UA, CXR, Barium swallow if indicated, hand x-rays, ECG/echo
41
SSc serology (diffuse vs limited)
+ANA Diffuse: Anti-Scl (Anti-topoisomerase I) and anti-RNA pol III Limited: anti-centromere
42
tx and management of SSc
no significant therapy; education, ACE inhibitors, calcium channel blockers, anti-reflux meds, glucocorticoids, cyclophosphamide, PDE5 inhibitor
43
CA associated with Sjogren syndrome
B cell NHL (MALT lymphoma)
44
Sjogren syndrome serology
+ANA, +RF, high ESR, polyclonal hypergammaglobulinemia, +AntiRo/LA, Low C4, anemia of chronic disease
45
Diagnostic biopsy essential for Sjogren diagnosis
lip biopsy revealing lymphoid foci in accessory salivary glands
46
Symptom management for Sjogren dx
artificial tears, ophthalmic lubricating ointments, sugarless candy and frequent sips of water, hydroxychloroquine, extraglandular manifestations
47
Drugs to avoid in Sjogren syndrome
atropinic drugs and decongestants
48
Sxs of inflammatory myopathies
symmetrical proximal muscle weakness, myalgias
49
Serology of inflammatory myopathies
normal ESR, CRP; elevated CK and aldolase
50
Characteristic skin lesions of dermatomyositis
Gottron's patches/papules, heliotrope rash, periungual erythema, v-neck erythema
51
Gottron's patches description
raised violaceous lesions overlying dorsa of DIP, PIP, MCP
52
Heliotrope rash
periorbital edema, purplish suffusion over eyelids
53
V-neck erythema
poikiloderma or "shawl sign;" erytherma over neck/shoulders, upper chest and back
54
Dx of DM
biopsy (perifascicular atrophy), elevated CK and aldolase, Anti-Jo1 ab
55
Malignancy associated with DM
ovarian, lung, pancreatic, stomach, colorectal, NHL, cervical, prostate, breast
56
Sxs of polymyositis
proximal muscle weakness without skin changes
57
Diagnostic biopsy of PM
endomysial inflammation with invasion of non-necrotic muscle fibers
58
Tx/management of DM and PM
glucocorticoids, methotrexate, azathioprine, cyclophosphamide
59
Inclusion body myositis sxs
weakness, especially finger flexion and quadriceps
60
IBM diagnostic biopsy
endomysial inflammation, rimmed vacuoles with invasion of non-necrotic muscle fibers
61
Serology and labs for IBM
mild elevation of CK, anti-cN1A autoabs
62
DDx of proximal muscle weakness
inflammatory myopathies, hypothyroidism, hyperthyroidism, cushing, neuro issues, vasculitides, drugs
63
Tetrad of IgA vasculitis
palpable purpura without thrombocytopenia, arthritis, abd pain, renal disease
64
Hallmarks of granulomatosis with polyangiitis
granulomatous inflammation, necrotizing vasculitis, segmental glomerulonephritis
65
Respiratory tract involvement of GPA
nasal involvement causing saddle nose, erosive sinus dz, CXR with cavitary lesions/infiltrates/nodules
66
ANCA
Anti-neutrophil cytoplasmic antibody
67
Hallmarks of Eosinophilic granulomatosis with polyangiitis
asthma and eosinophilia causing vasculitis with granulomas
68
Prodromal phase of EGPA
allergic disease (asthma/allergic rhinitis)
69
Eosinophilia-tissue infiltration phase of EGPA
high eosinophils in blood, tissue infiltration
70
Vasculitis phase of EGPA
systemic necrotizing heart/lungs/nerves/skin, palpable purpura
71
CBC with Diff EGPA
high eosinophil
72
Serology of EGPA
ANCA +
73
Lung biopsy of EGPA
granulomas with eosinophils in tissue
74
DDx of EGPA
HSP, GPA
75
Triad of small vessel Bechet Syndrome
mouth ulcers, genital ulcers, eye inflammation
76
DDx of pulmonary-renal syndromes
granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, SLE, IgA-mediated disorders, Bechet Syndrome, RA
77
Population affected by thromboangiitis obliterans
young males that are smokers
78
Diagnostic angiography of thromboangiitis obliterans
corkscrew appearance
79
Polyarteritis Nodosa system involvement
skin, nerves, GI, renal, cardiac
80
Polyarteritis nodosa biopsy
fibrinoid necrosis without granulomas
81
Polyarteritis nodosa angiogram
micro-aneurysm
82
Vessels typically involved in Takayasu arteritis
aorta, subclavian, innominate
83
Vessels typically involved in Giant cell arteritis
cranial arteries, aortic arch
84
Temporal artery biopsy in giant cell arteritis
segmental granulomatous vasculitis with multinucleated giant cells
85
Sxs of polymyalgia rheumatica
proximal stiffness, soreness and muscle pain; symmetrical, feelings of weakness due to pain
86
Primary Raynaud
benign, symmetric, exaggerated by emotion or cold
87
Secondary Raynaud
secondary to something --CTD, hematologic and endocrine conditions, cisplastin, bleomycin; unilateral
88
Episodic progression of Raynaud phenomenon
pallor -> cyanosis -> erythema
89
Management of Raynaud's
wearing gloves, keep warm, lotions, stop smoking, limit use of sympathomimetic drugs, surgery