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(J2) Skin > Autoimmune Disorders > Flashcards

Autoimmune Disorders Flashcards

1
Q

What is systemic lupus erythematosus?

What is its mechanism?

A

Autoimmune disorder (type III hypersensitivity)

caused by immune complexes fromed antinuclear antibodies (ANAs) which deposit in tissue causing damage and activate complement

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2
Q

What are the clinical features of SLE?

A

comes in flares and has periods of remission

  • fever, fatigue, malaise, weight loss
  • malar “butterfly” rash, or discoid rash that are worse with sun exposure
  • arthritis
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3
Q

What is the epidemiology of SLE?

A
  • significanlty more common in females
  • young to middle aged adults (20-40)
  • more common in minority populations
  • more common in poor SES
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4
Q

What findings are used to diagnose SLE?

(11)

A

RASH OR PAIN

Rash (malar or discoid) Arthritis Serositis Hematologic

Oral Renal

Photosensitivity ANA Immunologic disorder (anti-dsDNA/Sm) Neurologic

4 of the following 11

Skin:

  1. malar rash
  2. discod rash
  3. photosensitivity
  4. oral/nasopharyngeal ulcers

Organs

  1. symmetric, nonerosive arthritis
  2. serositis (pleurisy, caridac rub, pericarditis on EKG, etc.)
  3. nephritits
  4. neuro symptoms (seizures or psychosis)

Labs

  1. some or all components of pancytopenia
  2. positive serum ANA (most commonly dsDNA; anti-Sm and antiphospholid also found)
  3. ANAs on immunofluorescence
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5
Q

What is the association (statistical) between positive ANA and SLE?

What particular Abs​ are associated with SLE?

A

ANAs are sensitive (~95%) but not specific (negative ANA = no lupus; positive ANA = possible lupus or something else)

  • anti-dsDNA is specific (70%); also coorelates with flares
  • anti-Sm (highly specific, poorly sensitive ~30%)
  • antiphospholipid Ab can be found
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6
Q

Other than ANA, what are additional expected lab findings with SLE?

A

Blood:

  • elevated ESR
  • hemolytic anemia/thrombocytopenia/leukopenia/lymphopenia
  • decreased C3/C4
  • decreased CH50

Urine:

  • proteinuria
  • casts
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7
Q

What are possible complications of SLE?

A

Early:

-infection

Late:

  • atherosclerosis -> CVD (most common CoD)
  • nephritits -> ESRD (worst prognosis)
  • Libman-Sacks endocarditis
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8
Q

What is Libman-Sachs endocarditis?

A

non-infectious endocarditis

  • caused by formation of sterile vegetations on heart valves
  • highly associated with SLE
  • embolization is common
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9
Q

What is the treatment/management for SLE?

A

Treatment:

  • hydroxychloroquine
  • glucocorticoids (immune supression)
  • NSAIDs
  • other immunosupressants

Management:

  • avoid sun exposure; wear sunscreen
  • influenza and pneumococcal vaccines
  • cancer screenings (increased risk)
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10
Q

What is antiphospholipid antibody syndrome?

What is its mechanism?

A

Autoimmune disorder

caused by antiphospholipid Abs which typically target molecules that regulate coagulation

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11
Q

What are the clinical features of antiphospholipid antibody syndrome?

A

recurrent thrombosis (both arterial and venous)

  • DVT
  • PE
  • stroke
  • MI
  • miscarriage (placental arteries)
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12
Q

How does antiphospholipid antibody syndrome relate to SLE?

A

Primary APS:

-more common than secondary

Secondary APS

-occurs in 1/3 of SLE cases

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13
Q

What specific antibodies are associated with antiphospholipid antibody syndrome?

What features are noted with each antibody?

A
  • lupus anticoagulant -> prolonged aPTT
  • anticardiolipin -> false positive for syphilis (positive VDRL/RPR)
  • anti-β2-glycoprotein
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14
Q

What is the treatment for antiphospholipid antibody syndrome?

A

-life long anticoagulation

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15
Q

What is drug-induced lupus and how does it compare to SLE?

A
  • positive ANA but with antihistone antibodies (no dsDNA antibodies)
  • typically caused by certain drugs; remvoing drugs resolves symptoms

More mild over all:

  • systemic symptoms less common
  • renal and neuro symptoms absent
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16
Q

What medications cause drug-induced lupus?

A

SHIPPE

  • Sulfa
  • Hydralazine
  • Isoniazid
  • Procainamide
  • Phenytoin
  • Etanercept (TNF inhibitors)
17
Q

What is neonatal lupus and how does it compare to SLE?

A

-affects newborns whose mothers have anti-Ro/anti-La (ANAs)

Can result in permanent heart block

Other symptoms are transient

  • rash
  • arthritis
  • hemolytic anemia/thrombocytopenia
18
Q

What is discoid lupus and how does it compare to SLE?

A

ANA is frequently negative but symptoms are caused by similar deposits of immune complexes in affected skin

  • mostly limited to skin involvement; systemic/organ system symptoms absent
  • well-defined erythematous plaques -> discoid rash (central atrophy w/ peripheral erythema) which resolve into disfiguring scars
  • most common appears on the head
19
Q

What is Sjögren syndrome?

What is its mechanism?

A

Autoimmune T-cell mediated (type IV HSR) destruction of lacrimal and salivary glands

20
Q

What are the clinical features of Sjögren syndrome?

A

Sicca syndrome (and other glandular):

  • dry eyes (keratoconjunctivitis)
    • foriegn body sensation
  • dry mouth (xerostomia)
    • dental carries
    • difficulting eating dry foods (dysphagia)
  • parotitis
  • vaginal dryness -> dyspareunia

Extraglandular

  • arthritis
21
Q

What is the epidemiology of Sjögren syndrome?

A
  • significanlty more common in females
  • older adults (40-60)
22
Q

What is the association (statistical) between positive ANA and Sjögren syndrome?

What particular Abs are associated with Sjögren syndrome?

A

ANAs are sensitive (~97%) but not specific (negative ANA = no Sjögren; positive ANA = could be something else)

-anti-Ro/SSA Ab and anti-La/SSB Ab (70%); not specific

23
Q

Other than ANA, what are additional expected lab findings with Sjögren syndrome?

A
  • elevated ESR
  • rheumatoid factor (w/ or w/o rheumatoid arthritis)
  • anemia of chronic disease
24
Q

What is used to diagnose Sjögren syndrome?

A

-lip biopsy (looking at minor salivary glands) looking for lymphocytic sialadenitis

25
Q

What is the treatment/management for Sjögren syndrome?

A

Immunomodulation ineffective

AVOID atropine related drugs and decongestants

Supportive only:

  • follow dentist and ophthalmologist
  • artificial tears (dry eyes)
  • frequent sips of water (dry mouth)
  • hydroxychloroquine (arthritis)
26
Q

What other diseases are associated with Sjögren syndrome?

A

Secondary Sjögren syndrome:

  • particularly rheumatoid arthritis (frequently RF+)
  • B-cell marginal zone lymphoma (associated w/ chronic inflammation) in the parotid gland
27
Q

What is scleroderma?

What is its mechanism?

A

Autoimmune disorder resulting in increased fibroblast activity/collagen deposition -> sclerosis

28
Q

What are the clinical features common of scleroderma?

A

-thickening/hardening of skin

-hyper/hypopigmentation (common first symptom in African Americans)

  • Raynaud’s phenomenon (common first symptom in caucasians; not present in localized)
  • positive ANA
29
Q

What are the subtypes of systemic sclerosis?

A
  • localized
  • limited
  • diffuse
30
Q

What is the epidemiology of systemic sclerosis​?

A

Limited and diffuse:

  • women
  • mid to older adult (30-60)

Localized:

-children

31
Q

What are the clinical features of localized scleroderma?

A
  • discreet, localized patches called morphea
  • no systemic organ involvement
  • no Raynauds
32
Q

What are the clinical features of limited systemic sclerosis​?

A

CREST syndrome

  • Calcinosis cutis (Ca2+ deposition in skin)
  • Raynaud’s (early symptom)
  • Esophageal dysmotility (Barrett and GERD)
  • Sclerodactyly (fibrosis/tightening of skin of fingers/hands -> limited movement and smoothing of surface)
  • Telangiectasia
  • typically LIMITED to face and distal extremities
  • prominent vascular involvement (early Raynaud’s)
  • pulmonary HTN
  • can have late systemic organ involvement
33
Q

What are the clinical features of diffuse systemic sclerosis?

A
  • diffuse, can starts in trunk and proximal extremities and spreads distally
  • hyperpigmentation with
  • prominent systemic involvement of organs (early symptom)
  • renal crisis (malignant HTN, hemolytic anemia, renal insufficiency)
  • interstitial lung disease (rales on exam)
  • esophageal dysmotility (Barrett and GERD)
  • Raynaud phenomenon
34
Q

What is the association (statistical) between positive ANA and scleroderma?

What particular Abs are associated with sclerodrma?

A

ANAs are sensitive (~90%) but not specific (negative ANA = no systemic sclerosis; positive ANA = coud be something else)

Diffuse systemic sclerosis:

  • anti-Scl 70/anti-topoisomerase I
  • anti-RNA polymerase III

Limited systemic sclerosis:

  • anti-centromere
  • C in CREST can also stand for Centromere
35
Q

What life threatening event can occur with systemic sclerosis?

What findings are associated with it?

A

Renal crisis:

  • malignant hypertension
  • microangiopathic hemolytic anemia
  • renal insufficiency/oligouria
36
Q

What is the treatment/management for systemic sclerosis​?

A

AVOID glucocorticoids (increases risk of renal crisis)

Supportive only

(J2) Skin (7 decks)

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