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SBL > CT disorders and Vasculitides > Flashcards

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
Q

Localized SSc presentation

A

discreet areas of discolored skin induration, NOT systemic, no Raynaud’s, morphea patches

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26
Q

Limited SSc presentation

A

CREST - Calcinosis cutitis, raynaud’s, esophageal dismotitlity, sclerodactyly, telangiectasia; pulmonary HTN

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27
Q

Diffuse SSc presentation

A

systemic, early and progressive internal organ involvement resulting in interstitial lung disease and renal crises

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28
Q

Phases of Diffuse SSc

A

Inflammatory edematous phase then fibrotic; cutaneous manifestations will cause impaired sweating and loss of body hair, fibrosis of joints

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29
Q

Skin manifestations of SSc

A

hyper/hypopigmented, dry/itchy skin, masklike facies, microstomia, perioral furrowing, calcium deposits

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30
Q

Primary cause of M&M in SSc

A

pulmonology manifestations

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31
Q

Key pulmonary manifestations of SSc

A

aspiration pneumonia, interstitial lung disease, pulmonary artery hypertension

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32
Q

Common pulmonary manifestation of Diffuse SSc

A

Interstitial lung disease, chronic dry cough, dyspnea, rales

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33
Q

Common pulmonary manifestation of Limited SSc

A

pulmonary artery hypertension; right heart cath to measure pressure; sxs include dyspnea, syncope, angina, right heart failure

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34
Q

Common renal manifestations of SSc

A

CKD, Renal crises

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35
Q

Renal crisis sxs

A

malignant hypertension, hemolytic anemia, progressive renal insufficency

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36
Q

Cardiac manifestations of SSc

A

myocardial fibrosis, cardiomyopathy, pericarditis, myocarditis, pericardial effusion, arrhythmia

37
Q

GI manifestations of SSc

A

malnutrition, xerostomia, esophagus (Barrett esophagus), gastroparesis, Gastric antral vascular ectasia, chronic diarrhea, primary biliary cirrhosis

38
Q

MSK manifestations of SSc

A

carpal tunnel, tendon friction rubs, fibrosis and adhesion of tendon sheaths

39
Q

Thyroid manifestations of SSc

A

hypothyroidism due to fibrosis

40
Q

Diagnostic workup of SSc

A

H&P, especially bp; ESR, ANA, serology, UA, CXR, Barium swallow if indicated, hand x-rays, ECG/echo

41
Q

SSc serology (diffuse vs limited)

A

+ANA
Diffuse: Anti-Scl (Anti-topoisomerase I) and anti-RNA pol III
Limited: anti-centromere

42
Q

tx and management of SSc

A

no significant therapy; education, ACE inhibitors, calcium channel blockers, anti-reflux meds, glucocorticoids, cyclophosphamide, PDE5 inhibitor

43
Q

CA associated with Sjogren syndrome

A

B cell NHL (MALT lymphoma)

44
Q

Sjogren syndrome serology

A

+ANA, +RF, high ESR, polyclonal hypergammaglobulinemia, +AntiRo/LA, Low C4, anemia of chronic disease

45
Q

Diagnostic biopsy essential for Sjogren diagnosis

A

lip biopsy revealing lymphoid foci in accessory salivary glands

46
Q

Symptom management for Sjogren dx

A

artificial tears, ophthalmic lubricating ointments, sugarless candy and frequent sips of water, hydroxychloroquine, extraglandular manifestations

47
Q

Drugs to avoid in Sjogren syndrome

A

atropinic drugs and decongestants

48
Q

Sxs of inflammatory myopathies

A

symmetrical proximal muscle weakness, myalgias

49
Q

Serology of inflammatory myopathies

A

normal ESR, CRP; elevated CK and aldolase

50
Q

Characteristic skin lesions of dermatomyositis

A

Gottron’s patches/papules, heliotrope rash, periungual erythema, v-neck erythema

51
Q

Gottron’s patches description

A

raised violaceous lesions overlying dorsa of DIP, PIP, MCP

52
Q

Heliotrope rash

A

periorbital edema, purplish suffusion over eyelids

53
Q

V-neck erythema

A

poikiloderma or “shawl sign;” erytherma over neck/shoulders, upper chest and back

54
Q

Dx of DM

A

biopsy (perifascicular atrophy), elevated CK and aldolase, Anti-Jo1 ab

55
Q

Malignancy associated with DM

A

ovarian, lung, pancreatic, stomach, colorectal, NHL, cervical, prostate, breast

56
Q

Sxs of polymyositis

A

proximal muscle weakness without skin changes

57
Q

Diagnostic biopsy of PM

A

endomysial inflammation with invasion of non-necrotic muscle fibers

58
Q

Tx/management of DM and PM

A

glucocorticoids, methotrexate, azathioprine, cyclophosphamide

59
Q

Inclusion body myositis sxs

A

weakness, especially finger flexion and quadriceps

60
Q

IBM diagnostic biopsy

A

endomysial inflammation, rimmed vacuoles with invasion of non-necrotic muscle fibers

61
Q

Serology and labs for IBM

A

mild elevation of CK, anti-cN1A autoabs

62
Q

DDx of proximal muscle weakness

A

inflammatory myopathies, hypothyroidism, hyperthyroidism, cushing, neuro issues, vasculitides, drugs

63
Q

Tetrad of IgA vasculitis

A

palpable purpura without thrombocytopenia, arthritis, abd pain, renal disease

64
Q

Hallmarks of granulomatosis with polyangiitis

A

granulomatous inflammation, necrotizing vasculitis, segmental glomerulonephritis

65
Q

Respiratory tract involvement of GPA

A

nasal involvement causing saddle nose, erosive sinus dz, CXR with cavitary lesions/infiltrates/nodules

66
Q

ANCA

A

Anti-neutrophil cytoplasmic antibody

67
Q

Hallmarks of Eosinophilic granulomatosis with polyangiitis

A

asthma and eosinophilia causing vasculitis with granulomas

68
Q

Prodromal phase of EGPA

A

allergic disease (asthma/allergic rhinitis)

69
Q

Eosinophilia-tissue infiltration phase of EGPA

A

high eosinophils in blood, tissue infiltration

70
Q

Vasculitis phase of EGPA

A

systemic necrotizing heart/lungs/nerves/skin, palpable purpura

71
Q

CBC with Diff EGPA

A

high eosinophil

72
Q

Serology of EGPA

73
Q

Lung biopsy of EGPA

A

granulomas with eosinophils in tissue

74
Q

DDx of EGPA

75
Q

Triad of small vessel Bechet Syndrome

A

mouth ulcers, genital ulcers, eye inflammation

76
Q

DDx of pulmonary-renal syndromes

A

granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, SLE, IgA-mediated disorders, Bechet Syndrome, RA

77
Q

Population affected by thromboangiitis obliterans

A

young males that are smokers

78
Q

Diagnostic angiography of thromboangiitis obliterans

A

corkscrew appearance

79
Q

Polyarteritis Nodosa system involvement

A

skin, nerves, GI, renal, cardiac

80
Q

Polyarteritis nodosa biopsy

A

fibrinoid necrosis without granulomas

81
Q

Polyarteritis nodosa angiogram

A

micro-aneurysm

82
Q

Vessels typically involved in Takayasu arteritis

A

aorta, subclavian, innominate

83
Q

Vessels typically involved in Giant cell arteritis

A

cranial arteries, aortic arch

84
Q

Temporal artery biopsy in giant cell arteritis

A

segmental granulomatous vasculitis with multinucleated giant cells

85
Q

Sxs of polymyalgia rheumatica

A

proximal stiffness, soreness and muscle pain; symmetrical, feelings of weakness due to pain

86
Q

Primary Raynaud

A

benign, symmetric, exaggerated by emotion or cold

87
Q

Secondary Raynaud

A

secondary to something –CTD, hematologic and endocrine conditions, cisplastin, bleomycin; unilateral

88
Q

Episodic progression of Raynaud phenomenon

A

pallor -> cyanosis -> erythema

89
Q

Management of Raynaud’s

A

wearing gloves, keep warm, lotions, stop smoking, limit use of sympathomimetic drugs, surgery

SBL (21 decks)

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