Autoimmune Disease3 Flashcards

1
Q

Poliosis is characterized by immune reseponse to melanin. What are a few causes?

A

Staph blepharitis, albinism, VKH syndrome

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2
Q

What is the name of the condition characterized by loss of pigmentation of the skin?

A

Vitiligo

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3
Q

Vitiligo usually presents before what age?

A

20

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4
Q

What perentage of the world’s population has vitiligo?

A

1-2%

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5
Q

What are some treatment options for vitiligo?

A

Topical steroids, PUVA, grafting depigmentation

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6
Q

VKH is a multisystemic disorder more common in what populations?

A

Darker people; ages 20-50

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7
Q

What are the four phases of VKH?

A

Prodromal (meningitis, encephalopathy, auditory disturbances); Acute Uveitis Phase (anterior or posteroid uveitis, can lead to retinal detachment); Convalescent phase (alopecia, poliosis, vitiligo, fundus lesions, depigmented limbal lesions); Chronic-recurrent phase (anterior uveitis with exacerbations)

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8
Q

In the chronic-recurrent phase of VKH, there are mutton fat (clumps of white blood cells) in the corneal endothelium. What other sign might accompany this phase?

A

Posterior synechiae

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9
Q

In order to diagnose VKH, you need to match symptoms in three of four of which categories?

A

Bilateral chronic anterior uveitis, posterior uveitis, neurological features, cutaneous lesions

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10
Q

What is a hallmark sign of VKH?

A

Acute serous retinal detachment

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11
Q

Comparing Vogt-Koyanagi to Harada, which has the worse visual prognosis?

A

Vogt-Koyanagi (Hurray I have harada; Shoutout to Nikki.)

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12
Q

If treatment of VKH with steroids is not sufficient, what other types of medications may be used?

A

Immunosuppressive agents: methotrexate, cyclosporine, chlorambucil

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13
Q

Ocular cicatricial pemphigoid is ________ (chronic/acute), progresses __________ (quickly/slowly), ___________(bilateral/unilateral), potentially blinding, systemic, autoimmune disease.

A

Chronic, slowly, bilateral

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14
Q

OCP involves what types of tissue?

A

Skin, mucous membranes (conjunctiva)

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15
Q

OCP is what type of a hypersensitivity reaction?

A

Type II

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16
Q

True or false: OCP is very common

A

False; it is rare

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17
Q

Conjunctival disease is seen in how many cases of oral involvement? What about skin involvement?

A

75% oral, 25% skin

18
Q

True or false: OCP is bilateral, each eye starts at the same time, and each eye progresses at the same rate

A

False; time of onset, severity, and rate of progression can vary between eyes

19
Q

Which gender has a higher prevalence of OCP?

A

Female

20
Q

True or false: OCP is more common in Caucasians

A

False; it actually has no racial predilection

21
Q

When is OCP typically diagnosed?

A

Later in life (50-60 years), though it probably originates earlier

22
Q

What are the four stages of OCP?

A

Conjunctivitis (particularly if associated with subconjunctival fibrosis); Conjunctival scarring and shrinkage (occurs over the next 3-4 years; conjunctivitis starts in other eye); Symblepheron formation (connection between palpebral and bulbar conjunctivas); End stage with corneal scarring and ankyloblepheron (including dry eye, vision loss)

23
Q

In advanced cases of OCP, what are some corneal signs?

A

Vascularization, limbal inflammation (stem cell destruction), stromal thinning and ulceration), opacification

24
Q

OCP can be treated with topical, and probably oral medications. Which medications would you use?

A

Prednisone, dapsone, cyclophosphamide, azathioprine

25
Q

Erythema multiforme is divided into what categories?

A

Minor (typical targets or raised, edematous papules in extremities) and major (typical targets or raised, edematous papules distributed to the extremities with involvement of one or more mucous membranes, epidermal detachment less than 10% of TBSA)

26
Q

Erythema multiforme is typified by what sort of skin lesions?

A

Target or bull’s eye lesion in extremeties: erythematous center with a pale surrounding ring with a larger erythematous ring

27
Q

What are the clinical features of SJS/TEN (Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis?

A

Widespread blisters on trunk and face, with erythematous or pruritic macules and one or more mucous membrane erosions.

28
Q

Stevens-Johnson Syndrome, if not overlapping with TEN, involves how much of the total body surface area?

A

Less than 10%

29
Q

If Stevens-Johnson Syndrome Toxic Epidermal Necrolysis overlap, how much of the total body surface area is affected?

A

Between 10-30%

30
Q

Toxic epidermal necrolysis, if not overlapping with SJS, involves how much of the total body surface area?

A

More than 30%

31
Q

SJS is a mild form of TEN (or TEN is a severe form of SJS, not sure which). How do these two conditions differ?

A

SJS is less common than TEN; SJS mortality is low at 1-5% while TEN is higher at 25-35%

32
Q

True or false: the onset of SJS/TEN is slow

A

False; onset is acute

33
Q

What age group is usually affected by SJS/TEN?

A

Children are 20% of patients, though the peak is between 10-30.

34
Q

Which gender has a higher prevalence of SJS/TEN?

A

Men

35
Q

It is thought that SJS/TEN is caused by…?

A

Delayed hypersensitivity response to drugs (sulfonamides, tetracycline, penicillin, NSAIDs, etc), response to epithelial cell antigen, microbial infection (myoplasma pneumoniae, bacteria, viruses such as HSV**)

36
Q

What are the systemic features of SJS/TEN?

A

Fever, malaise, HA, loss of appetite, N&V, cough, sore throat, URTI

37
Q

Diagnosis of SJS is partially based on skin involvement. What sort of signs might you find in a SJS patient?

A

Vesicles, bullae on trunk

38
Q

Diagnosis of SJS is partially based on mucous membrane involvement. Which membranes are commonly involved?

A

Mouth and eyes

39
Q

What are some of the ocular symptoms of SJS/TEN?

A

Lids (swelling, crusting, ulceration, entropion, trichiasis); conjuntiva (papillary or pseudomembranous conjunctivitus, fibrosis, necrosis, keratinization), lacrimal dysfunction, keratopathy

40
Q

What are some systemic treatment options for SJS/TEN?

A

No specific treatment, though steroids have been used, as have oral antibiotics and antivirals.

41
Q

How would you treat ocular SJS/TEN?

A

Broad spectrum antibiotics, topical steroids, soft CLs, surgery (lid scrubs, epilation, cool compresses as adjunct therapy)