LAB 9 - Autoimmunity

Question 1

Compare and contrast hypersensitivity and autoimmunity.

Answer 1

Hypersensitivity: excessive response to a previous sensitization/exposure to antigen

Autoimmunity: not recognizing self antigens

Question 2

Systemic sclerosis

Answer 2

Etiology: antigen activates CD4+ T cell -> cytokines released -> activating fibroblasts & collagen deposition

Dense Collagen deposits in skin

Thickening of skin
Fibrosis - Pulmonary, esophageal, GI, endothelium
Raynaud's Phenomenon
Polyneuropathy
Swollen Hands/forearms/face/trunk
Ulcers

Female:Male = 3:1 50-60 y.o.

EARLY = distal extremity skin changes
LATE = edema/fibrosis

Limited: only 1 organ/tissue system
Diffuse: starts diffues/widespread - everywhere; more than 1 organ system; rapid progression

CREST Syndrome:
C - Ca deposits
R - Raynaud’s
E - Esophageal dysmotility (collagen deposits in smooth muscle)
S - Sclerodactyly (tough fingers)
T - Telangestasia (dilated BV resulting from vascular injury)

Question 3

Sjogren Disease

Answer 3

most often in Women (50-60 y.o)
Salivary & Lacrimal - other exocrine glands too
synovitis, diffuse pulmonary fibrosis, and peripheral neuropathy

Primary (alone) vs Secondary (with other Autoimmune disorders - RA & SLE)

• Eyes: cornea inflamed, eroded, and ulcerated
• Mouth: atrophy of mucosa with inflammatory fissuring and ulceration; dental caries.
• Nose: dryness and crusting leading to ulcerations and even perforation of the nasal septum.
• Parotid gland enlargement in 50% of patients (unilateral or bilateral).

Tests:
rheumatoid factor (RF) +
anti-Ro +
anti-La +
ESR=42 mm/hr
Schirmer’s test + (lacrimal gland secretion flow rate)

Question 4

keratoconjunctivitis

Answer 4

Inflammation of cornea and conjunctiva

Dry eyes

Question 5

xerostomia

Answer 5

dry mouth

Question 6

Mikulicz syndrome

Answer 6

abnormal enlargement of glands in head & neck

Lymphoma, Sjorgren’s, sarcoidosis, Goiter

Question 7

Amyloidosis

Answer 7

Deposition of mis-folded proteins (in the cross-linked Beta pleated sheet form)

Primary - on its own => MI
Secondary - result of another disease - chronic infection/inflammation

Congo Red dye => Apple Green under polarized LM

Transthyretin-derived (senile, AS)
Amyloid Light-chain (AL)

Alzheimer’s Chronic inflammation, Cancer, Type I Diabetes

Dx with Rectal Biopsy

Question 8

RF

Answer 8

Rheumatoid factor
Autoantibody related to Rheumatoid Arthritis (& other autoimmune diseases)

Antibody agains Fc portion of IgG

Question 9

Anti-Ro

Answer 9

Anti-Ro - + in SLE & Sjorgrens

Question 10

Anti-La

Answer 10

Anti-La - Sjorgren’s Syndrome

Question 11

ESR

Answer 11

Erythrocyte Sedimentation Rate

the rate at which red blood cells sediment in a period of one hour

Men = 12-19
Women = 18-23

Question 12

ANA

Answer 12

Anti-nucleate antibodies

Anti-Ro - + in SLE & Sjorgrens
Anti-La - Sjorgren's Syndrome
Anti-Sm - specific for SLE
Anti-nRNP -  SLE
Anti-Scl-70 - scleroderma
Anti-dsDNA - polymyositis & dermatomyositis
Anti-histone - drug-induced Lupus
Anti-centromere - CREST syndrome & cutaneous systemic sclerosis

Question 13

Non-Hodgkin’s B Cell Lymphoma

Answer 13

types of cancer derived from blood cells (all except for Hodgkin’s Lymphoma)

Question 14

Raynaud’s Phenomenon

Answer 14

excessively reduced blood flow in response to cold or emotional stress, causing discoloration of the fingers, toes, and occasionally other areas

stays white
i.e Christina Tsoriano

Question 15

anti-topoisomerase antibody

Answer 15

autoantibodies directed against topoisomerase and found in several diseases, most importantly scleroderma

Question 16

BUN

Answer 16

Blood urea nitrogen

Checks kidney function

Normal = 6-20 mg/dL

Urea nitrogen is what forms when protein breaks down

Question 17

Creatinine

Answer 17

Serum = Blood creatinine levels
Urine = Urine creatinine levels

Checks kidney function

Normal = 0.6-1.3 mg/dL (blood)

Question 18

Total Protein

Answer 18

Total amount of protein in blood
Albumin & Globulins

Nutritional problems, kidney/liver disease

Normal = 6-8g/dL

Question 19

Orthopnea

Answer 19

ortho = straight
pnoia = breath

SOB when lying flat

Has to sleep propped up in bed or sitting in a chair

Question 20

Nocturia

Answer 20

having to get up at night to urinate

Question 21

Albumin

Answer 21

Albumin (from Liver) circulating in blood serum

Decreased = Kidney/Liver diseases

Increased = High protein diet, dehydration

Normal = 3.5-5.5g/dL

Question 22

Waldenström’s macroglobulinemia

Answer 22

cancer affecting B cells that produce excessive IgM, thickening of the blood

• Unknown cause that causes adenopathy, hepatosplenomegaly, peripheral neuropathy and hyperviscocity (too much stuff in blood -> thickens)

Question 23

Globulin

Answer 23

Liver function

Increased:
Acute infection
Multiple myeloma
Chronic inflammatory disease
           (RA or SLE)
Waldenstrom macroglobulinemia

Question 24

Polymyositis/Dermatomyositis

Answer 24

many muslces

muscle destruction b CD8+ T cell attack

Dermatomyositis = muscle & Skin
Perivascular muscular atrophy with mononuclear cell infiltrate

Immunifluorescence = patchy deposits of Ig

Question 25

CPK

Answer 25

Creatine phosphokinase

Enzyme found in heart, brain, and skeletal muscle (non regenerative tissues)

Elevated levels indicate tissue damage to heart, brain or skeletal muscle

Normal = 10 - 120 mcg/L

Question 26

Grotten’s lesions

Answer 26

Bilateral red papules on elbows, knuckles and knees

Characteristic antibody for dermatomyositis

Anti-Jo-1

Question 27

Juvenile dermatomyositis (JDM)

Answer 27

Pediatric counterpart of dermatomyositis

S/s:

1) distinctive rash - Grotten’s lesions
2) vasculitis from muscle inflammation