Micro - Autoimmune Respones

Front 1

HLA B27

Back 1

Ankylosing spondylitis
Acute anterior uveitis

Front 2

HLA DR3

Back 2

Addison's
Graves
Myasthenia gravis
Systemic lupus erythematous

Front 3

HLA DQ6 - most risk

Back 3

Narcolepsy
Multiple Sclerosis

Front 4

HLA DQ6 - less risk

Back 4

IDDM (HLA DQ8 & DQ2)

Front 5

HLA DR4
HLA DR6

Back 5

Rheumatoid Arthritis
Juvenile RA

Front 6

HLA B35

Back 6

Subacute thyroiditis

Front 7

HLA Cw6

Back 7

Psoriasis vulgaris

Front 8

HLA DQ2, DQ8

Back 8

Celiac Dz

Front 9

HLA DR2

Back 9

Goodpastures
Multiple sclerosis

Front 10

HLA DR3/DR4

Back 10

Type 1 DM

Front 11

HLA B27

Back 11

Ankylosing spondylitis
Acute anterior uveitis

Front 12

HLA DR3

Back 12

Addison's
Graves
Myasthenia gravis
Systemic lupus erythematous

Front 13

HLA DQ6 - most risk

Back 13

Narcolepsy
Multiple Sclerosis

Front 14

HLA DQ6 - less risk

Back 14

IDDM (HLA DQ8 & DQ2)

Front 15

HLA DR4
HLA DR6

Back 15

Rheumatoid Arthritis
Juvenile RA

Front 16

HLA B35

Back 16

Subacute thyroiditis

Front 17

HLA Cw6

Back 17

Psoriasis vulgaris

Front 18

HLA DQ2, DQ8

Back 18

Celiac Dz

Front 19

HLA DR2

Back 19

Goodpastures
Multiple sclerosis

Front 20

HLA DR3/DR4

Back 20

Type 1 DM

Front 21

HLA DR4

Back 21

RA
Pemphigus vulgaris

Front 22

HLA DR5

Back 22

Hashimoto's thyroiditis

Front 23

Odd thing about type 1 insulin-dependent DM & multiple sclerosis

Back 23

associatin is w/ HLA-DQ (which is associated w/ HLA-DR) but not detected in serotyping

Front 24

Non-MHC genes in autoimmunity

Back 24

Complement proteins - C2, C4
Fas, FasL
AIRE

Front 25

Lupus-like dz

Back 25

Gene: Complement protein - C2, C4
Mechanism: Defective clearance of immune complexes, defects in B cell tolerance

Front 26

Lpr, gld mouse strains
Human ALPS

Back 26

Gene: Fas, FasL
Mechanism: Defective elimination of self-reactive T and lymphocytes by AICD

Front 27

Autoimmune polyendocrinopathy w/ candiadisis
Ectodermal dysplasia

Back 27

Gene: AIRE
Mechanism: Defective elimination of self-reactive T cells in the thymus

Front 28

MC cause of diarrhea in the US

Back 28

Campylobacter jejuni

Front 29

Reiter's syndrome

Back 29

HLA association: HLA-B27
Infection: Chlamydia trachomatis

Front 30

Reactive arthritis

Back 30

HLA association: HLA-B27
Infection: shigella, salmonella, yersinia, campylobacter

Front 31

Lyme Dz - chronic arthritis

Back 31

HLA association: HLA-DR2, DR4
Infection: Borrelia burgdorferi

Front 32

IDDM

Back 32

HLA Association: HLA-DQ2, DQ8, DR4
Infection: Coxsackie A, B, echoviruses, rubella

Front 33

Central tolerance

Back 33

tolerance to self antigens when lymphocytes encounter these ag in the primary lymphoid organs

NEGATIVE SELECTION of lymphocytes in thymic medulla

Front 34

AIRE (autoimmune regulator gene)

Back 34

Encodes a protein that is a TF that induces 100s of genes in peripheral tissue
Transcribes peripheral cellular proteins in a subpopulation of epithelial cells in the medull of thymus
Negative selection makes sure T cells don't recognize these antigens

Front 35

Autoimmune Polyglandular Disease (APD)
Autoimmune Polyendocrinopathy-candidiasis-ectodermal dystrophy

Back 35

Cause: Defective AIRE gene --> autoimmunity

Front 36

APCED

Back 36

MC affected endocrine glands: Hypoparathyroidism, adrenal failure, ovarian failure
MC conditions that affect other tissues: candiasis, dental enamel hypoplasia, nail dystrophy, tympanic membrane calcification, alopecia, keratopathy

MC in Finns, Sardinians, Iranian Jews

Front 37

Peripheral tolerance

Back 37

tolerance when mature lymphocytes encounter self antigens in peripheral tissues via Anergy, Suppression by Treg cells, Clonal deletion, Ag sequestration

Front 38

Anergy

Back 38

prolonged or irreversible functional inactivation of lymphocytes

induced by encounter w/ ag in absence of a second signal (CD28/B7) OR inhibitory signal mediated via CTLA4/B7 interaction

Front 39

Principal factors in development of autoimmunity

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Genetic & Environmental triggers ( infection, trauma, toxic irritants)

Front 40

Immunologic tolerance

Back 40

unresponsiveness to self antigens

Front 41

Peripheral tolerance

Back 41

development of tolerance when mature lymphocytes encounter self antigens in peripheral tissues

Front 42

Treg cells

Back 42

CD4 T cells w/ CD25 + IL-2R, but may lack CD24
Regulatory effect mediated via IL-10 or TGF-b secretion
Induced either in thymus (recognition of self ag) or induced in the periphery

Front 43

Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-linked (IPEX)

Back 43

Mutation in Foxp3 gene - affects T reg cells

Front 44

Foxp3

Back 44

required for development of CD4, CD25 Treg cells

Front 45

Suppression of autoreactive T cells by Treg cells

Back 45

Tregs recognize autoag presented by MHCII --> suppress proliferation of naive T cells responding to autoag --> Tregs engage B7 on APC via CTLA-4

IL4, IL10, TGF-b mediates the effects of Treg

Front 46

Clonal deletion by activation-induced cell death

Back 46

Fas mediated apoptosis - Fas on lymphocytes reacts w/ FasL on activated T cells --> apoptosis of T cells

Front 47

Autoimmune Lymphoproliferative Syndrome (ALPS)

Back 47

Fas gene mutation --> induction of apoptosis of unwanted lymphocytes does not occur --> pts cannot control lymphocyte population or autoimmune cells --> 2ndary lymph organs become swollen
Autoimmune rxn against blood cells, platelets, liver cells

Front 48

Common cause of lymphadenopathy

Back 48

Lymphoma, malignant dz, tb, leukemia

Front 49

Antigen sequestration

Back 49

exposure to ag of immune privileged site tissues
Ex: post-traumatic orchitis, uveitis, sympathetic opthalmia

Front 50

Immune privileged sites

Back 50

testes, eye, brain

Front 51

Sympathetic Opthalmia

Back 51

Cause - trauma to one eye --> release of intraocular protein ag --> goes to lymph nodes to activate T cells --> effector T cells attack both eyes

Sx: impaired vision in both eyes

Front 52

B cell tolerance

Back 52

Central B cell tolerance
Peripheral B cell tolerance

Front 53

Central B cell tolerance

Back 53

Clonal deletion - induced apoptosis for immature B cells that interact strongly w/ self (can also become anergic by migrating to the periphery and change from IgM to IgD expressing B cells)

Front 54

Peripheral B cell tolerance

Back 54

Autoreactive B cells fail to enter primary lymphoid follicle to receive T cell help --> entrapped in T cell zone and undergo apoptosis or can become anergic by migrating to periphery and expressing IgD

Anergic B cells + Fas -- FasL + T cell --> apoptosis

Front 55

Autoimmune dz (type II hypersensitivity) - ab against cell surface or matrix

Back 55

Hemolytic anemia, thrombocytopenia purpura, Goodpasture's, Pemphigus vulgaris, Acute rheumatic fever, Graves dz, Myasthenia gravis, Insulin-resistant diabetes, Hypoglycemia

Front 56

Autoimmune hemolytic anemia

Back 56

IgG & IgM ab bind to erythrocyte surfaces --> classical complement activation --> destruction of RBC
Occurs in spleen

Front 57

Goodpasture syndrome

Back 57

Autoab against noncollagenous domain of alpha-3 chain of collagen IV
Destroys basement membranes of renal glomeruli and lung alveoli
Epi: teens-twenties, M>F
Dx: linear IgG deposition on GBM & alveolar membrane - immunofluorescence; serology
Tx: plasmapheresis, immunosuppressive drugs - Prednisone, cyclophosphamide

Front 58

Pemphigus

Back 58

Epid: M>F, 40s-60s
Types: vulgaris, vegetans, foliaceus, erythematous

Front 59

Pemphigus vulgaris

Back 59

@ the mucosa and skin - scalp, face, axilla, groin, trunk, points of pressure
Primary lesions are superficial vesciles and bullae that rupture easily

Front 60

Pemphigus vegetans

Back 60

large, moist, verrucous vegetating plaques studded w/ pustules
@ axilla, groin, flexural surfaces

Front 61

Pemphigus foliaceus

Back 61

Benign form
@ scalp, face, chest, and back

Front 62

Pemphigus erythematosus

Back 62

Less severe than foliaceus
May selectively involve malar area of the face in a lupuserythamatosus-like fashion
Confused w/ SLE

Front 63

Acantholysis

Back 63

detachment between keratinocytes due to deposition of autoab against desmosomes or inherited dysfunction of desmosomal structure
cells become polyhedral --> circular

Front 64

Pemphigus Dx

Back 64

Immunofluorescence showing fish net-like pattern of ab deposition
Autoab against desmoglein 1 --> superficial layers of epidermis in folliaceus
Autoab against desmo 3 --> deeper layer of epidermis in vulgaris

Front 65

Intramolecular Epitope Spreading

Back 65

immune response against one part of antigenic molecule and progresses to other non-cross-reactive epitope on same molecule
Example Pemphigus
EC desmoglein - EC1-4 similar, EC-5 different
Initially ab is against EC5 - nonpathogenic
After onset pts have ab to EC1 and EC2

Front 66

Graves dz

Back 66

Epid: F>M, 20-40yrs, high risk for other autoimmune dz, HLA-DR3
hyperthyroid state (little tissue destruction)
Autoimmune response (TH2) @ TSH receptor (80%)
Other Autoab - ANA, Antithyroperoxidase, Antithyroglobulin

Front 67

Microscopy - Graves

Back 67

hyperplastic infoldings in the colloid
clear vaculoesnext to epithelium - increased activity --> increased hormone --> scalloping out of colloid

Front 68

Sx - Graves

Back 68

heat intolerance
Nervousness
Irritability
warm moist skin
weight loss
*bulging eyes (infiltrative ophthalmopathy: exophthalmos)
*enlargement of thyroid (goiter; typical)
Pretibial myxedema (infliltrative dermopathy; less common)

Front 69

Exopthalmos

Back 69

increased volume of retro-orbital connective tissue and extraocular muscles due to accumulation of proteoglycans and hyaluronic acid push eyeball forward

sympathetic overstimulation also causes wide-eyed, staring gaze

Front 70

Labs - Graves

Back 70

increased T3/T4, decreased TSH
diffuse uptake of radioactive iodine
increased TSH-R levels (80% cases), ANA levels without evidence of SLE or other collagen-vascular diseases, antithyroglobulin and, antithyroperoxidase antibody (nonspecific)

Front 71

Treatment - Graves

Back 71

Propranolol, Thyourea drugs (methimazole, propylthiouracil), thyroidectomy, ablation

Front 72

Hashimoto's Thyroiditis
Epidemiology

Back 72

MC thyroid disorder in US
White>Mexican>Black
F>M
Genetic and maybe drug induced (amiodarone)

Front 73

Hashimoto's
Pathophys

Back 73

Infection or nonspecific inflamaation --> IFN-gamma induces HLA-II expression on thyroid cells --> thyroid peptides present to T cells --> induce response --> Autoab (TH2 + effector T cell (TH1)) against thyroid cells --> Infiltration of lymphocytes into tissue --> destruction

Front 74

Hashimoto's
Sx

Back 74

Diffuse goiter - firm and finely nodular
Systemic - Depression
Chronic fatigue
Dry mouth (xerostomia)
Dry eyes (keratoconjunctivitis sicca)
May be associated with other autoimmune condtions including IBD or Celiac disease
Patients of Turner’s syndrome have a 15% incidence of significant thyroid dysfunction by age of 40 years

Front 75

Hashimoto's
Labs

Back 75

Antithyroid peridase (90%)
Antithyroglobulin (40%)
Increased TSH

Front 76

Hashimoto's
Tx

Back 76

Synthetic thyroid hormone
T4 in goiter pts or increased TSH levels

Front 77

Myasthenia gravis - pathophys

Back 77

autoab against acetylcholine receptor --> endocytosis of receptor --> degradation

Front 78

Myasthenia gravis - Sx

Back 78

Early symptoms: droopy eyelids - ptosis, double vision

Subsequently: Weakness of other facial muscles, upper and lower extremities, Involvement of chest muscles cause breathing difficulty and make these patients prone to respiratory infections

Front 79

Myasthenia gravis - Dx

Back 79

Improvement of weakness after edrophonium (cholinesterase inhibitor) administration
Diagnosis is confirmed by detection of antibody to Ach receptor

Front 80

Myasthenia gravis - Tx

Back 80

Pyridostigmine - enzyme cholinesterase inhibitor
Azathioprine - immunosuppressive drug that inhibits DNA synthesis - affects B cell and T cell proliferation --> inhibits immune response

Front 81

SLE - pathophys

Back 81

circulating IgG ab specific for cell surfaces, cytoplasm, and nucleus (including NA & nucleoproteins) --> inflammation --> tissue destruction due to soluble tissue ag releaseSL

Front 82

SLE - epidemiology

Back 82

African/Asian
F>M (10:1)

Front 83

SLE - presentation

Back 83

Butterfly shaped skin rash (due to immune complex deposition in skin)
Immune complex may deposit in blood vessels, **kidneys, **joints and other tissues

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SLE - histology

Back 84

thickened basement membrane
Glomerular hypercellularity (prolif of mesengial and endothelial cells w/ infiltration of neutrophils due to cellular injury from immune complexes)

Front 85

SLE - manifestations

Back 85

hematological
arthritis
skin
fever
fatigue
weight loss
renal
CNS

Front 86

SLE - dsDNA ab

Back 86

a/w active renal dz
40-60%
diagnostic

Front 87

SLE - anti-SS-B ab

Back 87

low risk of nephritis

Front 88

SLE - ANA

Back 88

95%
less specific and found in other autoimmune dz (drug induced LE, systemic sclerosis, scleroderma, sjogren, inflammatory myopathies)

Front 89

SLE - anti-sm

Back 89

Smith ag - core protein of small nuclear ribonucleoprotein particle
Diagnostic

Front 90

SLE - other autoab

Back 90

anti-ribonucleoprotein Ab
anti-SS-A ab
anti-SS-B ab

Front 91

SLE - how are autoab formed?

Back 91

Genetic predisposition - HLA-DQ, deficiency in C2, C4, C1
Non-genetic (environmental) factors - drugs, UV light, sex hormones
Fundamental abnormality in the immune system

Front 92

Drug inducing SLE

Back 92

Hydrallazin – BP/ACE inh
Procainamide - antiarrhythmic
D- penicillamine
Isoniazid – tb/prophylaxis

Front 93

SLE - tx & px

Back 93

Prednisone
Px: common cause of deat from SLE - renal failure & intercurrent infections

Front 94

Chronic Discoid Lupus Erythematosus

Back 94

Systemic manifestations are rare
Characteristic skin lesions usually affect face and scalp (Edema, Erythema, Scaliness, Follicular plugging)
Skin atrophy

Front 95

CDLE - ab

Back 95

+ ANA (35%)
rare to have anti-dsDNA ab
Skin bx --> Ig & C3 deposition

Front 96

Subacute Cutaneous Lupus Erythematosus

Back 96

Skin rashes are widespread, superficial and nonscarring
Most patients have mild systemic symptoms consistent with SLE
Strong a/w ab to the SS-A antigen and with HLA-DR3 genotype

Front 97

Drug-induced Lupus Erythematosus

Back 97

Genetics: HLA-DR4
Sx: affects organs (renal & CNS uncommon)
Antibodies: + anti-histone ab; Procainamide: + ANA w/ 33% manifesting clinical sx

Front 98

Nephritogenic GAS

Back 98

12, 4, 1

Front 99

Polyarteritis Nodosa

Back 99

Sx: Fibrinoid necrosis of renal & visceral vessels affected (small-medium arteries)
Associations: HBV, CMV
Ab: P-ANCA (Wegners - C-ANCA)

Front 100

PAN histology

Back 100

Inflammation of cellular infiltrate --> fibrous thickening of vessels
Occurs segmentally

Front 101

PAN - clinical presentation

Back 101

Young adults
MC: Malaise, fever of unknown cause, and weight loss
Other: Hypertension, Abdominal pain and melena (bloody stool), Muscle pain (diffuse), Peripheral neuritis (predominantly motor), Renal involvement (no GN)

Front 102

PAN - tx

Back 102

corticosteroids
cyclophosphamide

Front 103

T cell mediated Autoimmune Dz

Back 103

Insulin-dependent diabetes mellitus
RA
MS
Celiac dz

Front 104

IDDM

Back 104

destruction of insulin producing b cells in the pancreatic islets --> against insulin, glutamic acid decarboxylase, and other specialized protein of the pancreatic b cells --> insulitis
Sx show when 90% b cells destroyed

Front 105

Multiple Sclerosis

Back 105

Injury exposes CNS --> Autoag: structural protein of myelin (myelin basic protein, proteolipid protein, and myelin oligodendrocyte glycoprotein) --> demyelination of neurons in white matter of CNS --> Th1 differentiation releasing IFN-gamma + plasma cells secreting IgG (90%) --> inflammation

Front 106

MS - Tx

Back 106

Initial - Immunosuppressive drugs
(Corticosteroids); injection of IFN-b to prevent progression of the disease (the mechanism is unknown)
Late Stage: Stronger immunosuppressives (cyclophosphamide)

Front 107

Rhematoid arthritis

Back 107

Characteristic: nonsuppurative proliferative and inflammatory synovitis & ankylosis of the joints

Chronic systemic inflammatory disorder that may affect many tissue and organs - skin, blood vessels, heart, lungs, and muscles

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RA - epid

Back 108

1-3% of US
F>M 3:1
20-40

Front 109

RF

Back 109

Rheumatoid factors are the anti-Ig autoAb (IgG, IgM, and IgA) against the Fc portion of human IgG

Front 110

Cellular infiltrate

Back 110

CD4, CD8 T cells; B cells, lymphoblast, plasma cells, neutrophils and macrophages --> prostaglandin, leukotriens, proteinase and collagenase --> tissue damage

Front 111

RA joint change

Back 111

Infiltration of synovium by inflammatory cells
Increased vascularity due to vasodilatation and angiogenesis
Aggregation of organized fibrin
Accumulation of neutrophils in the synovial fluid
Osteoclastic activity in subchondral bone resulting in bone destruction
**Pannus formation

Front 112

RA - pathogenesis

Back 112

Nature of the autoimmune reaction - CD4 T cells + B cells
Mediators of tissue injury - TNF & IL-1 (stimulate synovial cells --> inflammation (e.g. prostaglandin) and matrix metalloproteinases); T cells --> produce RANKL --> bone destruction
Genetic susceptibility
The arthritogenic antigen (s)

Front 113

RA - x-ray

Back 113

Decreased joint space
Bony erosions
Joint deformities

Front 114

RA - genetics

Back 114

HLA-DRB1 (*0401, *0404, *0405, and *0408 ) --> allele (w/ basic residues) may bind and display the arthrtogenic antigen to T cells
HLA-DRB1*0402 --> a/w predisposition (difference among aa sequence)

Front 115

RA - Dx

Back 115

4/7 sx
1. morning stiffness
2. arthritis in 3 or more joint areas
3. arthritis of typical hand joints
4. symmetric arthritis
5. Rheumatoid nodules
6. serum rheumatoid factor
7. typical radiographic changes

Front 116

RA - Tx

Back 116

1. Anti-inflammatory drugs
2. Immuno-suppressive drugs
3. Antibody against TNF-a
4. Physiotherapy

Front 117

Celiac Dz

Back 117

abnormal immune responses to gluten protein present in wheat and wheat-related products
Loss of oral tolerance to gluten ag

Front 118

Celiac - Epid

Back 118

Caucasians

Front 119

Celiac - Genetics

Back 119

HLA-DQ2 (approx. 80%) and HLA-DQ8 (approx. 20%)

Front 120

Celiac - pathogenesis

Back 120

Gluten/Gliadin degraded into fragments in gut --> fragment enters gut and deaminated by transglutaminase --> CD4 Tcell responds to peptides presented by HLA-DQ on APC --> T cells cause inflammation

Front 121

Celiac - Autoab

Back 121

IgG or IgA autoantibodies specific for tissue transglutaminase
Anti-gliadin antibodies

Autoab are made by - B cells that gobble transglutaminase, gliadin, gluten --> present to T cells --> T cells activate B cells into plasma cells --> produce autoab
Dx: small intestine biopsy

Front 122

Celiac - presentation

Back 122

Classical: Diarrhea, Flatulence, Weight loss, Fatigue
Extraintestinal: skin, neuro manifestations
Increased risk to non-Hodgkin lymphoma, adenocarcinoma of small intestine and squamous cell carcinoma of esophagus

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Celiac Tx

Back 123

Gluten free diet

Front 124

Sjogrens

Back 124

keratoconjunctivitis sicca & xerostomia caused by immunologically-mediated destruction of lacrimal and salivary glands by CD4+ T cells, and B cells including plasma cells

Front 125

Sjogrens - associations

Back 125

Etiology: EB virus, hepatitis C virus and HTLV-1 virus
HLA-B8, HLA-DR3,and DRW52, as well as, HLA-DQA1, HLA-DQB1

Front 126

Sjogrens Autoab

Back 126

RF (75%)
ANA (50-80%)
SS-A, **SS-B (60-90%): (not dx)

Autoag - a-fodrin: cytoskeletal protein

Front 127

Systemic Sclerosis (Scleroderma)

Back 127

Abnormal accumulation of fibrous tissue in the skin and multiple organs
Skin (MC), GI tract, kidneys, heart, muscles, and lungs

Front 128

Diffuse scleroderma

Back 128

widespread skin involvement at the onset with rapid progression and early visceral involvement (lung, kidney, heart and alimentary tract)

Front 129

Limited scleroderma
CREST

Back 129

skin involvement is often confined to fingers, forearms and face and visceral involvement is late

CREST syndrome: Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, & Telangiectasia

Front 130

Scleroderma - pathogenesis

Back 130

CD4+T cells respond to ag accumulate in skin --> release cytokines --> recruit inflammatory cells --> release mediators (histamine, heparin, IL-1, IL-2, IL-13, TNF, PDGF, and TGF-b) --> fibroblast transcription for collagen and ECM (fibronectin) --> fibrosis

Front 131

Scleroderma - Autoab

Back 131

ANA antibodies (almost always present)
Scleroderma antibody (SCL 70; against topoisomerase III)
Anti-centromere Ab
Anti-RNA polymerase Ab (RNAP I, II, III)

Front 132

Scleroderma - Tx

Back 132

Vasodilators (nifidipine and iloprost) <-- ischemic attack and skin ulcers
H2 blocker, antacids and proton pump inhibitors <-- reflux esophagitis
Antibiotics (tetracycline) <-- infection
ACE inhibitor <-- Secondary hypertension due to renal involvement
Cyclophosphamide <--severe interstitial lung disease

Front 133

Reiters syndrome

Back 133

Urethritis
Conjunctivitis (or less commonly uveitis)
Mucocutaneou lesions
Septic arthritis

Front 134

Reiters - etiology

Back 134

1. dysenteric infection (with Salmonella, Shigella, Yersinia, or Campylobacter)
2. sexually transmitted infection (with Chlamydia trachomatis, or perhaps Ureaplasma urealyticum)

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Reiters - presentation

Back 135

HLA-B27- 80% of whites & 50-70% of blacks
Arthritis: asymmetric - involves weight bearing joints (knee and ankle)
Systemic sx: (fever, weight loss)
Mucocutaneous lesion: balanitis, stomatitis, keratoderma blennorhagicum

Front 136

Reiters - Tx

Back 136

NSAIDs*
Tetracycline w/ C. trachomatis
Sulfasalazine and anti-TNF agents are tried in patients refractory to NSAID and tetracycline

Front 137

Amyloidosis

Back 137

deposition of an abnormal protein, amyloid, in various tissues and organs of the body in a wide variety of clinical settings

Front 138

Amyloid

Back 138

pathogenic proteinaceous substance --> pressure atrophy of the adjacent cells

Front 139

Amyloid protein types:
AL (amyloid light chain)

Back 139

derived from plasma cells and contains immunoglobulin light chain

Front 140

Amyloid protein types:
AA (amyloid-associated)

Back 140

unique non-immunoglobulin protein synstesized by the liver

Front 141

Amyloid protein types:
**Ab amyloid

Back 141

found in the cerebral lesion of Alzheimer disease

Congo red stain + beta amyloid stain = alzheimers

Front 142

Systemic amyloidosis

Back 142

Immunocyte discrasias with amyloidosis (primary amyloidesis)
Reactive systemic amyloidosis (secondary amyloidosis)
Hemodialysis-associated amyloidosis
Hereditary amyloidosis
Familial mediterranian fever
Familial amyloidotic neuropathies
Systemic senile amyloidosis

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Localized amyloidosis

Back 143

Senile cerebral
Endocrine - Medullary carcinoma of thyroid, Islet of Langerhans
Isolated atrial amyloidosis
Prion diseae

Front 144

Amyloid histology - H&E

Back 144

amyloid appears as an amorphous, eosinophillic, hyaline extracellular substance

Front 145

Primary amyloidosis

Back 145

Amyloidosis w/ immunocyte dyscrasias
Systemic in distribution
Most common form
Many cases have some form of plasma cell dyscrasia (classical example: multiple myeloma)

No overt B cell neoplasm representing the classic primary amyloidosis, yet will have monoclonal Ig and/or free light chain in serum or in urine
Bone marrow also show a modest increase in plasma cells