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MSK Biweekly 3 > Al-Mehdi Pathogenetic treatment of CT disorders > Flashcards

Al-Mehdi Pathogenetic treatment of CT disorders Flashcards

1
Q

for most multisystem disorders, something in nucleus against which ______ are generated and cause disease

A

anti nuclear antibody (ANA)

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2
Q

neutrophil has something in cytoplasm to which Ab’s are developed against

A

antineutrophil cytoplasmic antibody (ANCA)

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3
Q

How to detect Ab’s

A

indirect immunofluorescence
ELISA (better specificity— higher predictive values)
Ab’s in dilution (titers)

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4
Q

general fluorescence imaging of ANA’s is the first step in the diagnosis of what

A

CT disorders

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5
Q

Female predominance (middle aged); autoimmune- autoantibodies against nucleic acids (nuclear antigens); deals w/ cell death of neutrophils by NET-osis

A

SLE (Lupus)

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6
Q

specific autoantigens of SLE

A

ssRNA, dsRNA, DNA

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7
Q

products of pro-inflammatory cytokines and type I interferons produce

A

inflammation

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8
Q

2 main things that occur due to the immune response of body with SLE patient

A

vasculitis and inflammation

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9
Q

vasculitis and inflammation can eventually lead to what that can lead to organs not functioning properly

A

tissue damage

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10
Q

malar rash that does not cross nasolabial fold characteristic of what

A

SLE

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11
Q

nephritis and pericarditis are also major features of what

A

SLE

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12
Q

specific antinuclear antibodies for SLE

A

anti-dsDNA or anti-Smith

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13
Q

in a female patient w/ lupus, what can cause spontaneous loss of pregnancy

A

antiphospholipids

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14
Q

diagnosis

A

SLE (lupus)

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15
Q

MoA for Tacrolimus and voclosporin to treat SLE

A

block IL-2

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16
Q

MoA of hydroxychloroquine (HCQ) for treatment of SLE

A

targets TLR9 (blocks DAMP recognition on macrophages)

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17
Q

2 main drugs that block PGE2 activity; and which one is better to treat SLE

A

celecoxib and aspirin(better)

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18
Q

MoA of prednisone and methylprednisolone to treat SLE

A

inhibit gene expression of all cytokines

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19
Q

MoA of belimumab and obinutuzumab and rituximab (the first two are best for SLE treatment)

A

block B cell activation

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20
Q

The effector mechanisms that lead to tissue damage in autoimmune diseases are similar to those elicited in response to environmental antigens that result in allergy. Which hypersensitivity reaction is correctly matched to an autoimmune condition that occurs through a similar mechanism?

A

SLE and serum sickness (type III)

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21
Q

multisystem noncaseating granulomatous disease

A

sarcoidosis

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22
Q

HLA DQB1 and DRB1

A

sarcoidosis

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23
Q

> 90% of patients w/ this disease exhibit mediastinal and hilar lymph node enlargement or lung disease

A

sarcoidosis

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24
Q

autoantigens for sarcoidosis

A

ssRNA, dsRNA, DNA

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25
Q

main organs involved in this disease include lungs, skin, and eyes

A

sarcoidosis

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26
Q

2 main outcomes of immune response in sarcoidosis

A

pulmonary fibrosis and vasculitis

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27
Q

50% of patients diagnosed with sarcoidosis by incidental what

A

CXR (chest xray)

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28
Q

lung involvement in >90% of patients with this disease; nonproductive cough

A

sarcoidosis

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29
Q

w/ sarcoidosis, what body part mainly experiences arthritis

A

ankles

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30
Q

Infiltrative skin lesions affecting the nose, cheeks, and ears in ___

A

chronic sarcoidosis

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31
Q
A

Lofgren syndrome in acute sarcoidosis

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32
Q

there is also increased_____ absorption in sarcoidosis

A

calcium

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33
Q
A

non-caseating granuloma in sarcoidosis

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34
Q

first line drugs for sarcoidosis

A

prednisone and ACTH

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35
Q

second line drugs for sarcoidosis

A

DMARDs

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36
Q

third line drugs for sarcoidosis

A

TNFa blockers (infliximab and adalimumab)

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37
Q

patient presents with painful lower extremity erythematous nodules and swelling and tenderness of ankles, what test should be ordered to support diagnosis

A

chest xray (for sarcoidosis

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38
Q

features of this include fibrosis and vasculopathy (thickening and fibrosis of skin)

A

systemic sclerosis

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39
Q

more prevalent in middle aged females and presents with thickened skin

A

systemic scleroderma

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40
Q

cause of death for patients w/ systemic scleroderma

A

pulmonary fibrosis and heart failure

41
Q

CREST (anti-centromere Ab’s) is diagnostic of what

A

systemic scleroderma

42
Q

capillary loss and vasculopathy along w/ fibrosis is distinguishing for what

A

systemic scleroderma

43
Q

the most characteristic and earliest vascular complication of systemic scleroderma

A

Raynaud’s phenomenon

44
Q

what do you not use to treat systemic scleroderma that could precipitate scleroderma renal crisis

A

glucocorticoids

45
Q

main treatment for systemic scleroderma

A

immunotherapy

46
Q

2 main types of drugs used to treat systemic scleroderma

A

antifibrotic and vascular drugs

47
Q

D-penicillamine, pirfenidone, and nintedanib are what kind of drugs used to treat systemic scleroderma

A

antifibrotic

48
Q

amlodipine and sildenafil are what type of drugs used to treat systemic scleroderma

A

vascular

49
Q

bosentan and sildenafil are what type of drugs used to treat systemic scleroderma

A

vascular + PAH

50
Q

Which of the following drugs may contribute to scleroderma renal crisis?

A

glucocorticoids

51
Q

systemic sclerosis + SLE + Raynaud’s

A

mixed connective tissue disease

52
Q

autoantibodies against U1-RNP

A

mixed CT disease

53
Q

one of the most common childhood disorders dealing with inflammatory arthritis in multiple joints

A

juvenile idiopathic arthritis

54
Q

female predominance and ANA + for this

A

oligoarticular juvenile idiopathic arthritis

55
Q

this has equal sex distribution and NO autoantibodies

A

systemic juvenile idiopathic arthritis

56
Q

Most common rheumatic disease in pediatrics

A

Juvenile idiopathic arthritis (Still disease)

57
Q

child presents with arthritis in multiple joints, fever w/ rash and hives and uveitis

A

juvenile idiopathic arthritis

58
Q

treatment for this is targeted against IL-1 and IL-6

A

juvenile idiopathic arthritis

59
Q

chronic autoimmune disorder; lymphocytic and plasma cell infiltration; destruction of salivary, lacrimal, and parotid glands

A

primary sjogren syndrome

60
Q

most common of the autoimmune disease

A

primary sjogren syndrome

61
Q

problem that presents with primary sjogren syndrome

A

risk of cancers

62
Q

HLA-DRB1
antibodies against Ro/SS-A and la/SS-B

A

primary sjogren syndrome

63
Q

due to T and B cell infiltration of exocrine glands

A

Sjogren syndrome

64
Q

xerostomia and keratoconjunctivitus sicca

A

dry mouth and eyes for Sjogren syndrome

65
Q

autoantibodies to SS-A and SS-B

A

Sjogren syndrome

66
Q

to diagnose Sjogren

A

auto-Ab’s against SS-A and SS-B and Schirmer test (tear test)

67
Q

diagnosis

A

Sjogren syndrome

68
Q
A

histopathology of primary Sjogren syndrome

69
Q

treatment for dry eyes for Sjogren syndrome

A

Cyclosporine eye drops

70
Q

treatment for dry mouth for Sjogren syndrome

A

Cevimeline

71
Q

main diagnostic test to establish Sjogren syndrome diagnosis

A

Anti-Ro/SS-A antibodies

72
Q

Which of the following is the best option for improving a patient’s ocular dryness in Sjogren syndrome?

A

cyclosporine eye drops

73
Q

A 55-year-old woman is referred to your department for polysynovitis and palpable purpura with a Dx of Sjögren syndrome. Which of the following treatments would you recommend to treat these systemic manifestations?

A

Rituximab (b/c Sjogren deals with T and B cell infiltration)

74
Q

this type of vasculitis is immune complex mediated

A

small vessel vasculitis

75
Q

this type of vasculitis deals with immune complexes and anti-endothelial cell Ab’s

A

medium vessel vasculitis

76
Q

this type of vasculitis deals with granulomatous disease

A

large vessel vasculitis

77
Q

giant cell arteritis and polymyalgia rheumatica are what type of vasculitis

A

large vessel

78
Q

elevated ESR (inflammation) and temporal artery affected that can lead to blindess

A

large vessel vasculitis symptoms (specifically polymyalgia rheumatica)

79
Q

to treat giant cell arteritis and polymyalgia rheumatica (large vessel vasculitis)

A

prednisone

80
Q

pulseless disease and has multinucleated giant cell (large vessel vasculitis)

A

Takayasu arteritis

81
Q

example of medium vessel vasculitis

A

Kawasaki disease

82
Q

presents in children < 5 yrs, bilateral conjunctival congestion, buccal erythema, and strawberry tongue (can resemble toxic shock syndrome and scarlet fever)

A

Kawasaki disease

83
Q

to treat Kawasaki disease

A

IVIG and ASPIRIN!!

84
Q

medium vessel vasculitis

A

Polyarteritis Nodosa

85
Q

Non-atherosclerotic, small and medium arteries, veins and nerves, <50 years old tobacco user, raynaud’s (can lose your toes)

A

Thromboangitis obliterans (Buerger disease)

86
Q

best treatment for Thromboangitis obliterans (Buerger Disease)

A

NO TOBACCO

87
Q

Immunosuppressive therapy is known to be futile for which disease associated with vascular inflammation?

A

Buerger disease (thromboangitis obliterans)

88
Q

ANCA associated vasculitis, strawberry gums, palpable purpura (small vessel vasculitis)

A

Granulomatous w/ polyangitis (Wegener Granulomatosis) GPA

89
Q

ANCA associated vasculitis, tissue inflammation from eosinophils, targets peripheral nerves (small vessel vasculitis)

A

Eosinophilic Granulomatosis w/ polyangitis (Churg-Strauss syndrome) EGPA

90
Q

to diagnose ANCA vasculitis GPA

A

c-ANCA (cytoplasmic)

91
Q

to diagnose ANCA vasculitis (EGPA)

A

p-ANCA (perinuclear)

92
Q

children, IgA vasculitis, palpable purpura

A

Henoch-Schonlein purpura

93
Q

triad of oral and genital ulcerations and ocular involvement-uveitis (variable vessel vasculitis)

A

Behcet’s syndrome

94
Q

apthous ulcerations in the mouth (extremely painful); (variable vessel vasculitis)

A

Behcet’s syndrome

95
Q

to treat Behcet’s syndrome

A

glucocorticoids and adalimumab

96
Q

patient with Behcet’s syndrome and already on anticoagulation meds and glucocorticoids should add what to his treatment

A

tumor necrosis factor inhibition (TNF)—adalimumab

97
Q

blanching, cyanosis, and rubor of fingers or toes; vascular reactivity disorder

A

Raynaud disease

98
Q

to treat Raynaud disease

A

sympathectomy (b/t T1 and T2)

MSK Biweekly 3 (5 decks)

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