CT Diseases - McGowan Flashcards

1
Q

PAN peripheral nerve involvement is what?

GI?

A

Mononeuritis multiplex - foot drop

Postprandial ABD pain

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2
Q

What malignancies are associated w/dermatomyositis?

What imaging is needed?

A

Ovarian, lung, pancreatic, stomach, colorectal, NHL

Transvaginal US, CT abd/pelvis, CA-125

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3
Q

Inclusion body myositis age group and sex?

What is weak?

Tx?

A

> 40-50, Males more common

Finger flexion, quadriceps

Supportive

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5
Q

Diffuse scleroderma has what serology?

A

(+) Anti-Scleroderma 70 aka Anti-DNA topoisomerase I

(+) Anti-RNA Polymerase III

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6
Q

What are the characteristic features of inflammatory myopathies?

A

Symmetrical b/l proximal muscle weakness

I.e. Difficulty rising from chair/bathtub

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6
Q

Secondary Raynaud occurs in whom?

Pattern?

Clinical course?

A

> 30 y/o due to some drug or disorder

U/l, Thoracic outlet, CTS

More severe, ischemia

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7
Q

SLE tx?

A

Avoid sun
NSAID
Corticosteroids
Hydroxychloroquine

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8
Q

GERD, Barrett’s, GAVE, PBC (anti-Mitochondrial Ab) are GI complications of what?

A

Diffuse scleroderma

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9
Q

GERD, Telangiectasias, or cutaneous calcinosis seen in what?

A

Limited cutaneous systemic sclerosis (Scleroderma)

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10
Q

Arthraliga, muscle weakness, CTS with renal crisis seen in what?

A

Diffuse scleroderma

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11
Q

SLE 4 important serologies?

A

(+)ANA
(+)Anti-dsDNA
(+)Smith
Decreased C3 or C4

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12
Q

CKD is a renal complication common in what?

May manifest how?

A

Diffuse SSc

Malignant HTN, hemolytic anemia, progressive renal insuff

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13
Q

Serology for Dermatomyositis?

A

Elevated CK, Adolase

(+) Anti Jo-1, anti-Mi2, anti-MDA5, anti-P155/P140

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14
Q

What presents w/prox severe, symmetrical morning and daylong stiffness, soreness, and pain in the shoulder, neck, and pelvic girdles?

What does biopsy show?

Elevated what?

Tx?

A

Polymyalgia Rheumatica

NORMAL

ESR and CRP

Corticosteroids

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15
Q

What shows endomysial inflammation, rimmed vacuoles, invasion of non-necrotic muscle fibers?

What autoantibodies are present?

A

Inclusion body myositis

Anti-cN1A

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16
Q

Type 3 antiphospholipid antibody causes what?

A

Anti-cardiolipin antibodies

Beta2GPI

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16
Q

What has a biopsy of perimysial and perivascular inflammation w/perifascicular atrophy?

Increased risk for what?

A

Dermatomyositis

Occult MALIGNANCY

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17
Q

What causes mucocutaneous LN syndrome?

What else is seen?

A

Kawasaki

Strawberry tongue

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18
Q

Localized scleroderma affects who?

Has what symptoms?

A

Children

Patches (morphea)
Discrete areas of discolored skin

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21
Q

Cotton wool spots are seen in what diseases for this section?

A

SLE/APS

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22
Q

How do you treat Sjogren’s?

A

Symptomatic

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23
Q

Neonatal lupus affects what children?

What is the biggest complication that may result?

A

Born of mothers w/SSA, SSB antibodies

Complete heart block

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24
Q

Diffuse scleroderma involves what organs?

Prognosis?

A

Kidney, cardiac, ILD

Worst

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24
Q

Giant Cell Arteritis affects what arteries?

What main sx?

What assoc?

Tx?

A

Cranial arteries (temporal, facial, ophthalmic), aortic arch

HA, jaw claudication, PMR, Inc ESR > 50

HLA-DR4

Corticosteroids before biopsy

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25
Q

Kawasaki disease occurs in whom?

Death from what?

Treatment?

A

< 5 y/o Asians

Coronary aneurysm or MI (many years later)

IVIG, high dose ASA

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26
Q

What is pANCA?

A

MPO-ANCA

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27
Q

What shows NO skin changes and an elevated serum CK w/Anti Jo-1 antibodies?

A

Polymyositis

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28
Q

Wegener’s (GPA) occurs in what age/sex?

Serology?

Involves what most commonly?

Tx?

A

Males over 40

(+)c-ANCA / PR3-ANCA

Nasal (saddle nose) and kidney

Cyclophosphamide, high dose glucocorticoids, Rituximab

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29
Q

Drug-induced lupus has what serology?

A

(+)ANA

(+) Anti-histone antibodies

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29
Q

What is pathergy?

What causes it?

Neuro involvement can mimic what?

A

Pustules at site of sterile needle prick

Behcet

M.S.

30
Q

What serology used to dx Sjogren’s?

A

(+)ANA, RF
Hypergammaglobulinemia
(+)Anti SSA,B

30
Q

What biopsy shows infiltration and destruction of blood vessels by inflammatory cells -> fibrinoid necrosis but no granulomas?

Associated w/what?

A

PAN

HBV

32
Q

What are the possible complications of Sjogren’s syndrome?

A

Increased incidence of oral infx (candida)
Dental caries
Parotid/salivary gland enlargement

33
Q

Chung-Strauss hallmark?

Serology?

Tx?

A

Asthma + eosinophilia -> vasculitis

(+)MPO-ANCA

Glucocorticoids

35
Q

What type of scleroderma are vascular manifestations more pronounced?

A

Limited

37
Q

Clinical course of scleroderma?

Occurs mostly in whom?

Tx?

A

Progressive

Women, AA’s, 30-60 y/o

No cure, just control sx

38
Q

IgA deposits seen in what?

A

HSP

41
Q

What is the primary cause of morbidity and mortality in scleroderma?

A

Pulmonology –> aspiration pneumonia and ILD

42
Q

Limited scleroderma has what serology?

A

(+) Anti-centromere

45
Q

What pregnancy complications can lupus cause?

A

GHTN
FGR
Fetal distress –> fetal loss/premature delivery

46
Q

Type 1 antiphospholipid antibody causes what?

A

False-positive RPR test for syphilis

47
Q

What does Takayasu Arteritis affect?

Clinical course?

Dx how?

Treatment?

A

Large vessels - aorta, subclavian, inominate

Relapsing/remitting

MRI or CT angiography

Glucocorticoids

49
Q

What causes obliteration of eccrine sweat and sebaceous glands causing dry itchy skin?

A

Scleroderma

50
Q

What imaging for PAN?

What is spared from disease?

Tx?

A

Angiogram - micro-aneurysm

Lungs

Corticosteroids

51
Q

Thromboangiitis Obliterans (Buerger’s) occurs in whom?

Dx?

Tx?

A

Young males who smoke

Angiography - corkscrew appearance

Stop smoking

52
Q

Primary Raynaud occurs in whom?

Pattern?

Clinical course?

A

W > M, 15-30 y/o

Benign, symmetric

Episodic, no thumb involvement
Pallor -> cyanosis -> erythema

53
Q

What MSK/other complications occur in diffuse scleroderma?

A

CTS

Hypothyroid due to fibrosis

54
Q

Increased incidence of bronchoalveolar carcinoma seen in what?

A

Diffuse scleroderma

55
Q

Limited scleroderma involves what organs?

Clinical course?

Prognosis?

A

Pulmonary HTN, CREST

Indolent

Good

56
Q

How do you treat Dermatomyositis/polymyositis?

A

Corticosteroids

57
Q

Behcet Syndrome has what complications?

Treatment?

A

Large vessel aneurysms, DVT, ulcers in distal ileum or cecum

Low dose corticosteroids

58
Q

Drug-induced lupus has what causes?

A
Hydralazine
Isoniazid
Methyldopa
Procainamide
Sulfa antibiotics
59
Q

What is the gold standard of biopsy for Ginat cell arteritis?

If no tx what happens?

A

Temporal a. Biopsy of at least 1 cm segment

Blindness

60
Q

Dermatomyositis age group affected?

Characteristics?

A

7-15 and 30-60

Weakness w/o sensory sxs

61
Q

What is the hallmark of systemic sclerosis?

A

Thickening and hardening of the skin

Microangiopathy and fibrosis of the skin and visceral organs

64
Q

Nailfold capillaroscopy that is distorted w/widened and irregular loops, dilated lumen and areas of vascular dropout seen in what?

Normal capillaroscopy?

A

Secondary Raynaud

Primary

65
Q

What causes long smooth tapered stenosis?

Aka?

A

Takayasu Arteritis

Pulseless disease - obliterates UE pulses

66
Q

1/3 of SLE pts also have what?

A

Secondary anti-phospholipid antibody syndrome

67
Q

What condition shows endomysial inflammation w/invasion of non-necrotic muscle fibers?

Age group?

A

Polymyositis

30-50

69
Q

What titer for ANA is clinically significant?

A

1:160

71
Q

Type 2 antiphospholipid antibody causes what?

A

Lupus anticoagulant, prolonged aPTT

72
Q

What is cANCA?

A

PR3-ANCA

73
Q

What is the Schirmer test used for?

A

Measures quantity of tears secreted - Sjogren’s

76
Q

What causes necrotizing vasculitis and segmental glomerulonephritis?

CXR would show what?

A

Wegener’s (GPA)

Cavitary lesions

78
Q

What has the histology of granuloma w/some giant cells, fibrosis in chronic stages?

A

Takayasu Arteritis

80
Q

What has a triad of recurrent mouth ulcers, genital ulcers, eye inflammation (uveitis)

Associated w/what?

A

Behcet syndrome

HLA-B51 and the Silk route (Turkey, Asia, Mid East)

84
Q

What has skin changes of livedo reticularis, sq nodules, ulcers, and digital gangrene?

A

PAN

87
Q

Copper wiring is seen in what?

A

Takayasu Arteritis

93
Q

How do you dx Sjogren’s?

What does it show?

A

Lip biopsy

Lymphoid foci in accessory salivary glands

94
Q

What has a strong association w/B cell NHL?

A

Sjogren’s

95
Q

Shawl sign for what?

A

Dermatomyositis

106
Q

What are Sicca symptoms?

A

Immune mediated dysfunction of lacrimal and salivary glands

107
Q

What is elevated CK and adolescent found in?

A

Inflammatory myopathy