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Systemic Disease > 05. Collagen Disorders % Injury > Flashcards

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
Q

What is the most important lab investigation of polyarteritis nodosa?

A

Nodule biopsy

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26
Q

What is relapsing polychondritis?

A

Genetically determined autoimmune response to type II collagen in sclera and cartilage

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27
Q

What vessels does relapsing polychondritis affect?

A

Small vessels

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28
Q

What are the ear signs of relapsing polychondritis?

A 
Cauliflower ear
Pain/inflammation
Vestibular dysfunction
Tinnitus
Vertigo
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29
Q

What are the 3 ocular findings of relapsing polychondritis?

A

Scleritis
Uveitis
BRVO
CRAO

30
Q

What is the common finding in the blood vessels in relapsing polychindritis?

A

Vasculitis

31
Q

What does the McAdam’s criteria help diagnose?

A

Relapsing polychondritis

32
Q

What is the Susac syndrome triad?

A

Encephalopathy
BRAO
Hearing loss

33
Q

What is Susac syndrome?

A

Retino-Cochleo-Cerebral vasculopathy

34
Q

What characterizes Susac syndrome?

A

Localized arteriolar vasculitis secondary to anti-endothelial cell antibodies

35
Q

What is the typical onset of RA?

A

30-40 years

36
Q

What is RA?

A

Chronic, multisystem inflammatory disease

37
Q

RA typically affects what gender?

A

Female (3:1)

38
Q

Rheumatoid factor is positive in 70% of RA courses and indicates what?

A

More severe course, worse prognosis

39
Q

RA typically affects which joints?

A

Small joints (hand, knee, neck)

40
Q

What is the typical deformity of the fingers in RA?

A

Swan neck deformity

41
Q

What is a common finding in RA that can cause strokes?

A

Vasculitis

42
Q

What phenomenon is typical in RA?

A

Raynauds’

43
Q

What are the common pulmonary findings in RA?

A

Pleurisy without effusions

44
Q

Amyloidosis is a secondary complication of what condition?

A

RA

45
Q

What are the common ocular signs of RA?

A 
KCS
Sjogren's
Filamentary keratitis
Sclerosing keratitis
Peripheral corneal furrowing
Corneal melt
Episcleritis
Scleritis
46
Q

What labs are used to investigate RA?

A

ESR (elevated)

RF

47
Q

When does type 1 Juvenile RA set in?

A

Early

48
Q

When does type 2 Juvenile RA set in?

A

Later onset

49
Q

What joints does type 2 JRA affect?

A

Hips

Sacroiliac joints

50
Q

Still’s triad is associated with what?

A

Type 1 JRA

51
Q

What is Still’s triad?

A

Early to moderate cataract formation
Chronic uveitis
Band keratopathy

52
Q

Type 2 JRA is associated with what ocular condition?

A

Acute uveitis

53
Q

RA is generally associated with what 4 ocular conditions?

A 
Dry eye
Filamentary keratitis
Episcleritis
Scleritis
Scleromalacia perforans
54
Q

Which type of Sjogren’s is an autoimmune disorder?

A

Secondary Sjogren’s

55
Q

Secondary Sjogren’s has an association with what gene?

A

HLA-DR3

56
Q

What happens to salivary and lacrimal glands in Sjogren’s?

A

Become infiltrated with CD4+T cells

57
Q

What do the CD4+T cells do in Sjogrens?

A

Produce inflammatory cytokines that damage the secretory epithelium of the glands

58
Q

The parotid gland becomes enlarged in what condition?

A

Sjogren’s

59
Q

What causes the extreme oral dryness in Sjogren’s?

A

Salivary gland atrophy

60
Q

What are the common ocular signs/symptoms of Sjogren’s?

A 
Dryness/redness of conj
KCS
Ulcers
Neo of cornea
Keratitis
61
Q

Ocular investigations should be done in Sjogren’s?

A

Shirmer (normal is about 15mm/5m)
Rose Bengal (grade 4 stain)
Lissamine green staining

62
Q

What does a biopsy of the lip often reveal in Sjogren’s?

A

Large foci of multiple lymphocytes

63
Q

What is SLE?

A

Chronic inflammatory autoimmune CT disorder

64
Q

What is Discoid lupus?

A

Chronic skin or scalp rash

65
Q

What are the most common hallmark findings of ocular SLE?

A

Dry eye

Lupus retinopathy

66
Q

What is seen in lupus retinopathy?

A 
CWS
Intraretinal hemes
Macular ischemia
Roth spots
CSR
67
Q

What labs are found to be high in SLE?

A

ANA
ELISA
ESR

68
Q

What labs should be done to investigate SLE?

A 
ANA
ELISA
ESR
C-Reactive protein
Anti-SM antibody
69
Q

Where is fibrotic damage found in scleroderma?

A

Skin

Cells lining arteries

70
Q

What does scleroderma mean?

A

Hardened skin

71
Q

Systemic scleroderma signs/symptoms follow the CREST acronym. What are they?

A 
Calcinosis
Raynaud's
Esophageal dysfunction
Sclerodactyly
Telangiectasia
72
Q

What are the most frequent ocular signs of FAS?

A

Optic nerve hypoplasia

Increased retinal vessel tortuosity

Systemic Disease (12 decks)

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