Gen Path-Autoimmune Disorders Part Ii

Front 1

scleroderma/progressive systemic sclerosis

Back 1

scleroderma/progressive systemic sclerosis

Front 2

Definition of sleroderma is?

Back 2

autoimmune disease byVASCULITIS and EXCESSIVE DEPOSITION OF COLLAGEN throughout the body, mainly in the skin ending in FIBROSIS & ATROPHY

Front 3

possible mechanisms that lead to systemic sclerosis is?

Back 3

PDGF- platelet derived growth factor
FGF- fibroblast growth factor

Front 4

pathological changes include?

Back 4

vasculitis

marked fibrosis and atrophy

restricted to skin first, then to viscera

Front 5

Clinical features: SKIN
starts by?

Face will present with?

Back 5

fingers then extends proximally to arms, shoulder, neck and face
Face present with restricted movement
-skin is edematousfollowed by progressive fibrosis

decrease range of motion
tapered
dec blood supply-ulceration and gangrene

Front 6

fingers present with a typical _____ necroses and ulceration of fingertips...

face presents with a ________ look/

Back 6

rat bite

masklike- lost lines, stretched, shiny skin- looks younger than actual age

Front 7

GI tract.
Mucosa presents?
submucosa presents?
muscle layer?

Back 7

1. thin, ulcers,loss of villi
2. collagenized
3. atrphies, replaced by collagen, deficient paristalsis

Front 8

Esophagus in 50% dyspahgia due to?
Intestines -malabsorption

Back 8

narrowing of lower portion- rubber hose look

Front 9

Joints present with?

Back 9

synovitis: proliferation of synovial membrane, then fibrosis
-resembles RA but destruction of articular cartridge

Front 10

Muscles-
focal inflammatory infiltrates and edema followed by?

Back 10

fibrosis and atrophy

Front 11

clinical features of the kidneys:
small arterioles present with?

Back 11

vasculitis, fibrosis, ischemia---> malignant hypertension
- decrease in GFR and renal failure

Front 12

clinical features of lungs are?

Back 12

diffuse interstitial PNEUMONITIS and FIBROSIS
-progressive THICKENING OF WALLS of small vessels

Front 13

Sjogrens Disease

Back 13

Sjogrens disease

Front 14

sjogrens is defined as?

Back 14

autoimmune - destruction of salivary glands, lacrimal glands

Front 15

happens more in ?
age?

Back 15

females 9;1
40-60 yrs

Front 16

pathological lesions include?

-marked infiltration w/ lymphocytes and plasma cells ....

hyperplasia of duct epithelium leading to?

Back 16

replacing the normal acinar cells

lumen destruction

Front 17

clinical findings of Sjogrens:

keratoconjuctivitis sicca presents?

Back 17

(dry eye)
dec tears
blur vision
itchy eyes
corneal ulceration

Front 18

clinical finding of Sjogrens:

xerostomia presents?

Back 18

dec salivary secretion
(dry mouth)
atrophy and ulceration of oral mucosa
dec taste
dysphagia

Front 19

clinical findings of respiratory tract present?

Back 19

dry/crusty nasal mucosa
laryngitis, bronchitis, pneumonia

Front 20

Polymyositis/Dermatomyositis

Back 20

Polymyositis/Dermatomyositis

Front 21

polymyositis defined as?

Back 21

autoimmune that affects skeletal muscles and skin

incidence
females 2;1
age-two peaks, 5-15 yrs and 50-60yrs

Front 22

in early stages muscle fibers show? Later stage will present?

Back 22

edema, lymphocytes, fatty change and necrosis
-later stage- atrophy, fibrosis

PROXIMAL MUSCLE GROUPS-shoulder pelvic
neck/pharynx
ics/diaphragm

Front 23

Skin is 50% of cases, and presents?

Back 23

Vasculitis w/ edema and cellular infiltration
Later on, atrophy,fibrosis, calcification

Front 24

dermatomyositis of the hands presents?

Back 24

violaceous erythema of the dorsum of the hands and fingers over mcp and interphalang joints

Front 25

Clinical features:
Muscles present?

Back 25

symmetrical weakness, pain and ternderness
stiff fingers
dysphagia-pahryngeal muscles

Front 26

clinical features:
Skin presents?

Back 26

erythematous rash on:
malar of face(butterfly rash)
V area of neck
forehead
shoulder chest
HELIOTROPE RASH -upper eyelid
atrophy

Front 27

AMYLOIDOSIS

Back 27

AMYLOIDOSIS

Front 28

amyloidosis definition?

Back 28

extracellular accumulation of fibrillar proteins ----> pressure atrophy of adjacent parenchyma

Front 29

Etiology of Amyloidosis is?

Back 29

increase production or decrease utilization.

Front 30

Clinical forms:
Systemic amyloidosis, Primary is associated with?

Back 30

-multiple myeloma, most common form in US.
Amyloid proteins; type AL(amyloid light chain) acuum in tissue and pass in urine(BENCE JONES PROTEIN)

Front 31

SECONDARY (REACTIVE) AMYLOIDOSIS:
Chronic inflam disease which are?

Autoimmune diseases are?

Neoplasms?

Back 31

TB, bronchiectasis, osteomyelitis

2. crohns disease, ulcerative colitis, scleroderma, RA

3. hodgkins lymphoma
renal cell carcinoma

Front 32

Hemodialysis-related amyloidosis
assoc w/ renal failure affect 70% of pt maintained on hemodialysis, the amyloid protein _________ acumulates in joints and synovium.

Back 32

B2-micorglobulin

Front 33

Hereditary amyloidosis-
Familial mediterranean fever is?

Back 33

recurrent inflammation of serosal linings and fever--- Type AA amyloid protein

Front 34

LOCALIZED AMYLOIDOSIS;
1. Senile amyloiamyloid proteindosis occurs?

Back 34

old age 80-90
-amyloid protein accumulates in HEART, BRAIN-as in Alzheimers

Front 35

Amyloid in neoplasms have local deposits in?

Back 35

the stroma of neoplasms of endocrine

Front 36

gross morphology presents?

Back 36

enlarged organ, tissue appears pale, waxy, gray and firm in consistency