05. Collagen Disorders % Injury Flashcards

1
Q

Ankylosing spondylitis is associated with which gene in whites?

A

HLA B27

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2
Q

Ankylosing spondylitis is associated with which gene in blacks?

A

HLA B7

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3
Q

Ankylosing spondylitis is characterized by inflammation of what?

A

Skeleton

Large peripheral joints

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4
Q

What are the 3 main signs/symptoms of ankylosing spondylitis?

A

Back pain
Stiffness of lower back
Peripheral joint pain

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5
Q

What is the typical finding on X-ray of ankylosing spondylitis?

A

Bamboo spine

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6
Q

What are the 3 hallmark signs of AS?

A

Symmetrical sacro-ilitis
Joints affected asymmetrically
Sciatica and compression radiculitis

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7
Q

What is the major ocular finding associated with AS?

A

Non-granulomatous uveitis

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8
Q

What is vasculitis?

A

Inflammation of blood vessels

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9
Q

What size of arteries of CGA affect?

A

Large arteries

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10
Q

What 3 effects does GCA have on tissue?

A

May lead to aneurym formation
Leads to vascular occlusion
Leads to tissue ischemia

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11
Q

What is the typical demographic for GCA?

A

White females over 50 (most common over 80)

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12
Q

What are the 4 most commonly affected arteries in GCA?

A

Temporal
Ophthalmic
Posterior ciliary
Vertebral

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13
Q

What are the 4 constitutional sypmtoms of GCA?

A

Anorexia/weight loss
Malaise
Fatigue
Fever and night sweats

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14
Q

Systemic manifesatations of GCA may include what?

A

Aneurysm

Stroke

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15
Q

What is occult GCA, and what percent of patients does it affect?

A

Ocular manifestations, but no systemic manifestations

21%

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16
Q

How urgent is GCA?

A

Emergent

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17
Q

Which ocular artery is always involved in GCA?

A

Posterior ciliary arteries

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18
Q

What are the common EOM disorders associated with GCA?

A

Palsies of III, IV, and VI

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19
Q

Which lab test is 100% specific for GCA?

A

ESR over 50mm/hr

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20
Q

What is the appropriate treatment if GCA is suspected?

A

Start steroids immediately

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21
Q

What is polyarteritis nodosa?

A

Inflammation and necrosis of medium sized muscular arteries

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22
Q

What are the cutaneous signs of polyarteritis nodosa?

A

*Osler’s nodules
Petechiae
Urticaria

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23
Q

What is the major renal sign of polyarteritis nodosa?

A

Glomerulonephritis

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24
Q

What are the important ocular signs of polyarteritis nodosa?

A
Necrotizing scleritis
Irido-cyclitis
Occlusive retinal peri-arteritis
BRVA
CRAO
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25
What is the most important lab investigation of polyarteritis nodosa?
Nodule biopsy
26
What is relapsing polychondritis?
Genetically determined autoimmune response to type II collagen in sclera and cartilage
27
What vessels does relapsing polychondritis affect?
Small vessels
28
What are the ear signs of relapsing polychondritis?
``` Cauliflower ear Pain/inflammation Vestibular dysfunction Tinnitus Vertigo ```
29
What are the 3 ocular findings of relapsing polychondritis?
Scleritis Uveitis BRVO CRAO
30
What is the common finding in the blood vessels in relapsing polychindritis?
Vasculitis
31
What does the McAdam's criteria help diagnose?
Relapsing polychondritis
32
What is the Susac syndrome triad?
Encephalopathy BRAO Hearing loss
33
What is Susac syndrome?
Retino-Cochleo-Cerebral vasculopathy
34
What characterizes Susac syndrome?
Localized arteriolar vasculitis secondary to anti-endothelial cell antibodies
35
What is the typical onset of RA?
30-40 years
36
What is RA?
Chronic, multisystem inflammatory disease
37
RA typically affects what gender?
Female (3:1)
38
Rheumatoid factor is positive in 70% of RA courses and indicates what?
More severe course, worse prognosis
39
RA typically affects which joints?
Small joints (hand, knee, neck)
40
What is the typical deformity of the fingers in RA?
Swan neck deformity
41
What is a common finding in RA that can cause strokes?
Vasculitis
42
What phenomenon is typical in RA?
Raynauds'
43
What are the common pulmonary findings in RA?
Pleurisy without effusions
44
Amyloidosis is a secondary complication of what condition?
RA
45
What are the common ocular signs of RA?
``` KCS Sjogren's Filamentary keratitis Sclerosing keratitis Peripheral corneal furrowing Corneal melt Episcleritis Scleritis ```
46
What labs are used to investigate RA?
ESR (elevated) | RF
47
When does type 1 Juvenile RA set in?
Early
48
When does type 2 Juvenile RA set in?
Later onset
49
What joints does type 2 JRA affect?
Hips | Sacroiliac joints
50
Still's triad is associated with what?
Type 1 JRA
51
What is Still's triad?
Early to moderate cataract formation Chronic uveitis Band keratopathy
52
Type 2 JRA is associated with what ocular condition?
Acute uveitis
53
RA is generally associated with what 4 ocular conditions?
``` Dry eye Filamentary keratitis Episcleritis Scleritis Scleromalacia perforans ```
54
Which type of Sjogren's is an autoimmune disorder?
Secondary Sjogren's
55
Secondary Sjogren's has an association with what gene?
HLA-DR3
56
What happens to salivary and lacrimal glands in Sjogren's?
Become infiltrated with CD4+T cells
57
What do the CD4+T cells do in Sjogrens?
Produce inflammatory cytokines that damage the secretory epithelium of the glands
58
The parotid gland becomes enlarged in what condition?
Sjogren's
59
What causes the extreme oral dryness in Sjogren's?
Salivary gland atrophy
60
What are the common ocular signs/symptoms of Sjogren's?
``` Dryness/redness of conj KCS Ulcers Neo of cornea Keratitis ```
61
Ocular investigations should be done in Sjogren's?
Shirmer (normal is about 15mm/5m) Rose Bengal (grade 4 stain) Lissamine green staining
62
What does a biopsy of the lip often reveal in Sjogren's?
Large foci of multiple lymphocytes
63
What is SLE?
Chronic inflammatory autoimmune CT disorder
64
What is Discoid lupus?
Chronic skin or scalp rash
65
What are the most common hallmark findings of ocular SLE?
Dry eye | Lupus retinopathy
66
What is seen in lupus retinopathy?
``` CWS Intraretinal hemes Macular ischemia Roth spots CSR ```
67
What labs are found to be high in SLE?
ANA ELISA ESR
68
What labs should be done to investigate SLE?
``` ANA ELISA ESR C-Reactive protein Anti-SM antibody ```
69
Where is fibrotic damage found in scleroderma?
Skin | Cells lining arteries
70
What does scleroderma mean?
Hardened skin
71
Systemic scleroderma signs/symptoms follow the CREST acronym. What are they?
``` Calcinosis Raynaud's Esophageal dysfunction Sclerodactyly Telangiectasia ```
72
What are the most frequent ocular signs of FAS?
Optic nerve hypoplasia | Increased retinal vessel tortuosity