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
Kawasaki disease occurs in whom? Death from what? Treatment?
< 5 y/o Asians Coronary aneurysm or MI (many years later) IVIG, high dose ASA
26
What is pANCA?
MPO-ANCA
27
What shows NO skin changes and an elevated serum CK w/Anti Jo-1 antibodies?
Polymyositis
28
Wegener's (GPA) occurs in what age/sex? Serology? Involves what most commonly? Tx?
Males over 40 (+)c-ANCA / PR3-ANCA Nasal (saddle nose) and kidney Cyclophosphamide, high dose glucocorticoids, Rituximab
29
Drug-induced lupus has what serology?
(+)ANA | (+) Anti-histone antibodies
29
What is pathergy? What causes it? Neuro involvement can mimic what?
Pustules at site of sterile needle prick Behcet M.S.
30
What serology used to dx Sjogren's?
(+)ANA, RF Hypergammaglobulinemia (+)Anti SSA,B
30
What biopsy shows infiltration and destruction of blood vessels by inflammatory cells -> fibrinoid necrosis but no granulomas? Associated w/what?
PAN HBV
32
What are the possible complications of Sjogren's syndrome?
Increased incidence of oral infx (candida) Dental caries Parotid/salivary gland enlargement
33
Chung-Strauss hallmark? Serology? Tx?
Asthma + eosinophilia -> vasculitis (+)MPO-ANCA Glucocorticoids
35
What type of scleroderma are vascular manifestations more pronounced?
Limited
37
Clinical course of scleroderma? Occurs mostly in whom? Tx?
Progressive Women, AA's, 30-60 y/o No cure, just control sx
38
IgA deposits seen in what?
HSP
41
What is the primary cause of morbidity and mortality in scleroderma?
Pulmonology --> aspiration pneumonia and ILD
42
Limited scleroderma has what serology?
(+) Anti-centromere
45
What pregnancy complications can lupus cause?
GHTN FGR Fetal distress --> fetal loss/premature delivery
46
Type 1 antiphospholipid antibody causes what?
False-positive RPR test for syphilis
47
What does Takayasu Arteritis affect? Clinical course? Dx how? Treatment?
Large vessels - aorta, subclavian, inominate Relapsing/remitting MRI or CT angiography Glucocorticoids
49
What causes obliteration of eccrine sweat and sebaceous glands causing dry itchy skin?
Scleroderma
50
What imaging for PAN? What is spared from disease? Tx?
Angiogram - micro-aneurysm Lungs Corticosteroids
51
Thromboangiitis Obliterans (Buerger's) occurs in whom? Dx? Tx?
Young males who smoke Angiography - corkscrew appearance Stop smoking
52
Primary Raynaud occurs in whom? Pattern? Clinical course?
W > M, 15-30 y/o Benign, symmetric Episodic, no thumb involvement Pallor -> cyanosis -> erythema
53
What MSK/other complications occur in diffuse scleroderma?
CTS | Hypothyroid due to fibrosis
54
Increased incidence of bronchoalveolar carcinoma seen in what?
Diffuse scleroderma
55
Limited scleroderma involves what organs? Clinical course? Prognosis?
Pulmonary HTN, CREST Indolent Good
56
How do you treat Dermatomyositis/polymyositis?
Corticosteroids
57
Behcet Syndrome has what complications? Treatment?
Large vessel aneurysms, DVT, ulcers in distal ileum or cecum Low dose corticosteroids
58
Drug-induced lupus has what causes?
``` Hydralazine Isoniazid Methyldopa Procainamide Sulfa antibiotics ```
59
What is the gold standard of biopsy for Ginat cell arteritis? If no tx what happens?
Temporal a. Biopsy of at least 1 cm segment Blindness
60
Dermatomyositis age group affected? Characteristics?
7-15 and 30-60 Weakness w/o sensory sxs
61
What is the hallmark of systemic sclerosis?
Thickening and hardening of the skin | Microangiopathy and fibrosis of the skin and visceral organs
64
Nailfold capillaroscopy that is distorted w/widened and irregular loops, dilated lumen and areas of vascular dropout seen in what? Normal capillaroscopy?
Secondary Raynaud Primary
65
What causes long smooth tapered stenosis? Aka?
Takayasu Arteritis Pulseless disease - obliterates UE pulses
66
1/3 of SLE pts also have what?
Secondary anti-phospholipid antibody syndrome
67
What condition shows endomysial inflammation w/invasion of non-necrotic muscle fibers? Age group?
Polymyositis 30-50
69
What titer for ANA is clinically significant?
1:160
71
Type 2 antiphospholipid antibody causes what?
Lupus anticoagulant, prolonged aPTT
72
What is cANCA?
PR3-ANCA
73
What is the Schirmer test used for?
Measures quantity of tears secreted - Sjogren's
76
What causes necrotizing vasculitis and segmental glomerulonephritis? CXR would show what?
Wegener's (GPA) Cavitary lesions
78
What has the histology of granuloma w/some giant cells, fibrosis in chronic stages?
Takayasu Arteritis
80
What has a triad of recurrent mouth ulcers, genital ulcers, eye inflammation (uveitis) Associated w/what?
Behcet syndrome HLA-B51 and the Silk route (Turkey, Asia, Mid East)
84
What has skin changes of livedo reticularis, sq nodules, ulcers, and digital gangrene?
PAN
87
Copper wiring is seen in what?
Takayasu Arteritis
93
How do you dx Sjogren's? What does it show?
Lip biopsy Lymphoid foci in accessory salivary glands
94
What has a strong association w/B cell NHL?
Sjogren's
95
Shawl sign for what?
Dermatomyositis
106
What are Sicca symptoms?
Immune mediated dysfunction of lacrimal and salivary glands
107
What is elevated CK and adolescent found in?
Inflammatory myopathy