Immunological And Multi-System Disorders

Front 1

who are the pro-inflammatory cytokines?

Back 1

TNF-alpha
IL-1

Front 2

Systemic Autoimmune Diseases

Back 2

SLE
RA
Systemic Sclerosis
Sjogren's Syndrome
Polymyositis/Dermatomyositis
(maybe vasculitic disorders)

Front 3

SLE
Sx's

Back 3

Malar Rash (butterfly)
Flu-like illness
Fever of unknown origin
polyarthralgias/polyarthritis

Front 4

SLE
different areas of disease manifestation?

Back 4

Vascular: thrombosis
Cytotoxic Ab's:AIHA, thrombocytopenia
Disrupted Cell Fxn: Anti-neuronal Ab's

Front 5

Who most commonly gets SLE?

Back 5

women 14-40
Blacks, Asians, Latinos

Front 6

what is discoid lupus?

Back 6

scarring rash
scarring alopecia
occasionally papular

Front 7

SLE Hands?

Back 7

Jaccoud's Arthropathy
bilateral swelling
palmar erythema
digital infarcts
Raynaud's

Front 8

what is Jaccoud's Arthropathy?

Back 8

reducible ulnar drift (i.e. pt can move them back w/o other hand)

Front 9

what is Raynaud's phenomenon?

Back 9

vasospastic disorder causing discoloration of the fingers, toes, and occasionally other extremities.

Front 10

Who is associated w/ SSA antibody?

Back 10

Subacute Cutaneous Lupus
Neonatal Lupus
Sjogren's

Front 11

Sx's of Neonatal Lupus?

Back 11

Surprised look
fever
rash
joint swelling
thrombocytopenia
3rd degree heart block (BAD)

Front 12

besides discoid lupus, who else is scarring?

Back 12

Lupus Profundus (lobular panniculitis)

Front 13

Sx of Livedo Reticularis?

Back 13

Lacey reticular pattern

Front 14

other cause of Livedo Reticularis besides lupus?

Back 14

SE of amantadine

Front 15

What is the "hallmark of the lupus disease process?"

Back 15

Immune complex deposition

Front 16

what is the "hallmark of systemic autoimmune diseases?"

Back 16

ANA's

Front 17

who is the kicker for SLE ANA testing?

Back 17

Human Laryngeal Epithelioma nuclie (Hep-2)

Front 18

besides ANA testing with Hep-2, how else can you diagnose SLE?

Back 18

Indirect Immunofluorescence

Front 19

Which IFI ANA pattern is indicative of SLE?

Back 19

Macrospeckled
Microspeckled is more for CREST

Front 20

nuclear antigens associated w/ SLE?

Back 20

dsDNA
Sm (smith)

Front 21

Nuclear Ag associated w/ systemic sclerosis?

Back 21

Scl-70

Front 22

Hallmark of Glomerulonephritis?

Back 22

RBC Cast

Front 23

Dx of renal involvement of SLE?

Back 23

Renal Immune Complex Deposits seen via:
Fluorescent IgG deposits
EM subendothelial deposits

Front 24

Cardiac involvement w/ SLE?

Back 24

Pericardium usually
Atypical Verrucous Endocarditis = Libman-Sachs

Front 25

What does Libman-Sachs Endocarditis lead to?

Back 25

Valvular incompetence, usually mitral regurg
maybe a source of emboli

Front 26

Rx of Lupus?

Back 26

Corticosteroids (judiciously)
Preventive (vaccines, TB testing)
Education (UV, diet, exercise)

Front 27

Life expectancy of SLE?

Back 27

80-90% at 10 years

Front 28

ANTIPHOSPHOLIPID ANTIBODY SYNDROME
path?

Back 28

hypercoagulability w/ recurrent thromboses

Front 29

ANTIPHOSPHOLIPID ANTIBODY SYNDROME
sx's?

Back 29

preggers complications
digital ischemia
livedo reticularis

Front 30

MIXED CT DISEASE
gender?

Back 30

women mostly

Front 31

MIXED CT DISEASE
clinical features?

Back 31

combo of SLE, polymyositis, scleroderma

Front 32

MIXED CT DISEASE
marker?

Back 32

Anti-RNP Ab's

Front 33

POLYMYOSITIS/DERMATOMYOSITIS
kickers

Back 33

autoimmune infl muscle disorder
mostly women
weaker proximal muscles than distal
high CK
dermato when skin is involved
dermato ass w/ lung, breast, and colon tumors

Front 34

POLYMYOSITIS/DERMATOMYOSITIS
marker for lung involvement?

Back 34

Jo-1 Ab

Front 35

POLYMYOSITIS/DERMATOMYOSITIS
typical rash?

Back 35

Gottron's Papules on hands
(fine scaling)
Heliotrope Rash (upper and lower eyelids swell)

Front 36

POLYMYOSITIS/DERMATOMYOSITIS
lower extremity kicker?

Back 36

Calf/Thigh Calcinosis

Front 37

SJOGREN'S SYNDROME
Kickers

Back 37

Autoimmune against tears and spit=dry mouth and eyes
Positive ANA, SSA, SSB, rheumatoid factor

Front 38

Difference between Raynaud's Disease and Phenomenon?

Back 38

Disease is primary form and benign

Phenomenon is secondary and can cause digital ulceration or gangrene

Front 39

typically who has raynaud's?

Back 39

women 15-40

Front 40

SCLERODERMA
types of localized?

Back 40

Morphea
Linear (one leg)
Coup de sabre (cut of saber)

Front 41

What is CREST?

Back 41

Limited (though systemic) Scleroderma
Calcinosis (digits)
Raynaud's Phen
Esophageal Dilation
Sclerodactyly (tight skin-->bent)
Telangiectasia (blanchable)

Front 42

Which is the Bad Bad Scleroderma?

Back 42

Diffuse Systemic

Front 43

Sx's of Diffuse Scleroderma?

Back 43

Mouse Facies (small nose, ridge lines)
Salt and Pepper skin
Digital pitting
Auto-amputation
Pulmonary Fibrosis
CREST
Duodenal Dilation
Colon-wide, mouthed Diverticula

Front 44

BEHCET'S
Sx's

Back 44

Oral and Genital ulcers (painful)
Ocular Disease: Hypopyon, Post/Ant Uveitis

Front 45

BEHCET'S
other system involvement?

Back 45

vascular: popliteal/femoral aneurysms
Neuro:cerebral hemorrhage (strokes)

Front 46

Which size vessels does BEHCET'S hit on?

Back 46

all of 'em

Front 47

which size vessels does Polyarteritis nodosa hit on?

Back 47

Medium size vessel vasculitis

Front 48

POLYARTERITIS NODOSA
kickers?

Back 48

Hep B correlation (30%)
Multiple microaneurysms
Multiple Constrictions on angiogram (beaded)
Typically renal and visceral
Necrotizing immune complex deposit infl

Front 49

what is Kawasaki's?

Back 49

Infantile Polyarteritis Nodosa

Front 50

Who does Kawaski go after?

Back 50

Medium and small size vessels

Front 51

Kawasaki kickers?

Back 51

Mucocutaneous involvement
strawberry tongue
Desquamation of skin
Fever
Coronary Artery Aneurysms

Front 52

Who goes's after larger vessels?

Back 52

Giant Cell Arteritis:
Temporal or Takayasu's Arteritis
and
Polymyalgia Rheumatica (PMR)

Front 53

Polymyalgia Rheumatica
kickers

Back 53

Old pts (70)
Sudden onset of hip and shoulder pain
Fever of unknown origin
No weakness
Prednisone is miraculous Rx

Front 54

Relationship between PMR and temporal arteritis?

Back 54

15-20% of PMR develops temporal arteritis
50% of temporal arteritis will manifest PMR sx's

Front 55

Temporal Arteritis
kickers

Back 55

Affects branches of Aortic Arch
Bad news if visual loss
Older women mostly
BIG inc in ESR
pain w/ chewing
Wt. loss, fever, malaise, HA
Temporal Artery Bulge and positive biopsy

Front 56

What do you see on fundoscopic exam of a temporal arteritis pt?

Back 56

Cotton wool exudates

Front 57

Rx for Temporal Arteritis?

Back 57

Corticosteroids

Front 58

TAKAYASU'S ARTERITIS
kickers?

Back 58

Asian women under 40
Med and Large arteries
Pulseless/Aortic Arch disease
Different upper and lower BP's

Front 59

TAKAYASU'S ARTERITIS
rx

Back 59

corticosteroids
immunosuppressives

Front 60

WEGENER'S GRANULOMATOSIS
kickers?

Back 60

Triad of Necrotizing Vasculitis
=necrotizing granulomas in:
1. lung
2. upper airway
3. and necrotizing glomerulonephritis

MARKER: C-ANCA
Eye involvement sometimes
Saddle nose

Front 61

P-ANCA marks who?

Back 61

Microscopic Polyangitis
Pauci-Immune Crescentic Glomerulonephritis
Churg-Strauss (maybe)

Front 62

Microscopic Polyangiits
kickers?

Back 62

non-granulomatous vasculitis of small vessels

Front 63

HENOCH-SCHONLEIN PURPURA
kickers?

Back 63

Small vessel vasculitis (hypersensitivity)
Most common childhood systemic vasculitis
All the lesions are at the same stage
Palpable Purpura
densest near ankles

Front 64

RELAPSING POLYCHONDRITIS
kickers

Back 64

inflammatory destructive lesions of cartilaginous structures (ears, nose, trachea, larynx)
40% associated w/ other condition

Front 65

PRIMARY ANGIITIS OF CNS

Back 65

small and medium vessels
CNS issues...duh

Front 66

ADULT STILL'S DISEASE

Back 66

not vasculitis
Fever
Rash (salmon-pink, transient w/ fever)
Achy and Swollen Joints
Muscle pain