autoimmune disorders

Question 1

SLE

Answer 1

immune complexes of self antigens - deposit

complement deficiency

Question 2

SLE HLA association

Answer 2

DR2, DR3

Question 3

SLE

Answer 3

deficiency in complement pathway proteins - Cq1, C2, C4

Question 4

SLE predisposition

Answer 4

uv rays, cigarettes, hormones, drugs (procainamide, hydralazine)

Question 5

procainamide

Answer 5

SLE

Question 6

hydralazine

Answer 6

SLE

Question 7

TLR 9

Answer 7

recognition of self DNA in SLE

Question 8

TLR-7

Answer 8

recognition of self RNA in SLE

Question 9

SLE abnormalities

Answer 9

plasmacytoid DC - INF alpha
B cells - activation by nuclear ag
Failure of tolerance

Question 10

SLE antibodies

Answer 10

antinuclear
antiphospholipids
antibodies against blood substances

Question 11

SLE hypersensitivity

Answer 11

type iii

Question 12

organs affected

Answer 12

vessels (fibrinoid necrosis), kidneys (glomerulonephritis), skin, joints, spleen (onion skin lesion), heart (libman sac’s endocarditis), serosa

Question 13

lupus nephritis

Answer 13

minimal mesangial 
mesangial proliferative 
focal
diffuse
membranous
advanced sclerosing

Question 14

lupus nephritis classes

Answer 14

minimal mesnagial - immune complexes
mesnagial proliferative - mesangium hypercellularity and matrix increase
focal - mesangial swelling and proliferation, neutrophils and fibrinoid deposits (in capillaries) hematuria, proteinuria/ red cell casts/ renal insufficiency
diffuse - half or more glomeruli affected, subendothelial immune complexes, thickened wire loop capillary walls/ glomerulosclerosis/ hematuria, proteinuria/ hypertension/ renal insufficiency
membranous - subendothelial immune complexes in capillaries, proteinuria, NEPHROTIC syndrome
advanced - 90% glomeruli - end stage

Question 15

SLE symptoms

Answer 15

skin - malar maculopapular butterfly rash
photosensitivity
dermoepidermo immune complexes
joints - swelling, mononuclear cells
cns - atherosclerosis/ thrombosis - cerebral angiopathy - infarcts - neuropsychiatric disorders

spleen - onion skin lesions (penicillary artery fibrosis)

pericardial and pleural serositis

libman sacks endocarditis - mononuclear cells infiltrate, - verrucous endocarditis (warty deposits on leaflets)

Question 16

SLE treatment

Answer 16

anti CD20 antibody - rituximab

blocking growth factors

Question 17

CREST

Answer 17

limited scleroderma

calcinosis, Raynaud’s phenomenon, esophgeal dysmotility, sclerodactyly, telangiectasia

Question 18

systemic sclerosis

Answer 18

limted

diffuse

Question 19

Sjorgen syndrome

Answer 19

lacrimal and salivary gland effected -

keratoconjunctivitis sicxa and xerostomia

Question 20

systemic sclerosis - skin

Answer 20

epidermis: skin capillaries thicken then fibrosed - occlude - skin epidermal atrophy - extremities - progress centrally.

dermis: edema of dermis - dermis fibrosis (epidermis thins and appendages atrophy occurs) - dermal vessels fibrosis
CD 4 T cells

joint motion limitations
drawn mask face

Question 21

git scleroderma

Answer 21

mostly esophagus affected
inflexibility
GERD
atrophy and fibrosis of muscularis
barret's metaplasia

small intestine - loss of villi, microvilli, malabsorption syndrome

Question 22

musculoskeletal system in scleroderma

Answer 22

synovial hyperplasia and inflammation

inflammatory myositis

Question 23

Lungs

Answer 23

endothelial activation

pulmonary vessels - vasospasm - hypertension

fibrosis - interstitium

Question 24

kidneys scleroderma

Answer 24

intimal thinkening (gp, mucopolysaccharides) intralobular arteries - hypertension in 30% people
thrombosis, infarction - fibrinoid necrosis

Question 25

heart scleroderma

Answer 25

patchy myocardial fibrosis

thickened myocardial arterioles corpulmonale

Question 26

mixed connective tissue disease

Answer 26

arthritis
swelling of hand
Raynaud's phenomenon
esophagus dysmotility, myositis
leukopenia
fever
lymphadenopathy
hyeprgammaglobulinemia

Question 27

IgG4 related

Answer 27

increased IgG4 producing B cells
all organs involved
B cells - organised germinal centers
T cells- diffused

storiform fibrosis
obliterative phlebitis, tissue eosinophilia

Question 28

diseases associated with IgG4

Answer 28

mikulicz - salivary and lacrimal
reidel’s thyroiditis retroperitoneal fibrosis
autoimmune pancreatitis
inflammatory pseudotumors