Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Patho > 15. autoimmune diseases > Flashcards

15. autoimmune diseases Flashcards

1
Q

immunologic tolerance

A

Inability of immune system to set immune response to a specific self-antigen is immunologic tolerance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

2 type of tolerance

A

central and peripheral tolerance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Mechanisms of development central tolerance

A

T cell central tolerance, additional sources of antigen are made available in the thymus by the action of the transcription factor AIRE (autoimmune regulator).
Positive selection
Negative selection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Mechanisms of development of peripheral tolerance

A

colonal anergy
colonal deletion
peripheral suppresion by t cells
ag sequestration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Clonal anergy;

A

Prolonged or irreversible inactivation of lymphocyte
Costimulator deficient antigen-presenting cells (APCs)

B7 - CD28

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Clonal deletion by activation-induced cell death

A

Fas by Fas L induces apoptosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Peripheral suppression by T cells

A

Regulatory T cells produce anti inflammatory cytokine
IL4, IL10 and TGFβ

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Antigen sequestration

A

Tissues not communicate with the blood and lymph, eg, testis, eye and brain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

organ specific autoimmune diseases

A

autoimmune hemolytic anemia
autoimmune thrombocytopeni
autoimmune pernicous anemia
myasthenia gravis
graves disease
goodpasture sydrome
type 1 DM
multiple scelerosis
crohns diesease
primary biliary cirrhosis
AI hepatatis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

systemic

A

SLE
rhematoid arthriitis
sclerodemra
sjogrens sydrome
polyarteritis nodosa
inflammatory myopathies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Mechanisms of Development of Autoimmunity

A

Breakdown of T cell anergy ; express costimulatory molecules

Failure of activation-induced cell death; congenital or acquired defects in the Fas–Fas ligand system

Molecular mimicry; cross-reaction between antibodies against infective agents with self-antigens

Polyclonal lymphocyte activation

Failure of T cell-mediated suppression

Release of sequestered antigens (anatomic sequestration)

Exposure of cryptic self-antigens and
epitope spreading (molecular sequestration)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

The systemic diseases tend to involve blood vessels and connective tissues . they are often called

A

collagen vascular diseases or connective tissue diseases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Systemic lupus erythematosus(SLE)

A

Classical prototype of a multisystem autoimmune disease
Chronic, remitting and relapsing illness
M:F = 1:9
Peak incidence is age 20-45 years
Type II and III hypersensitivity reactions.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

anti what antibodies

A

Antinuclear antibody (ANA) (>95%);
anti-dsDNA (40 - 60%);
anti-Sm (20-30%);

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

……………..and ……………..are virtually diagnostic of SLE.

A

Anti-Sm and ds DNA are virtually diagnostic of SLE.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

pathogenesis of SLE

A

In this hypothetical model, susceptibility genes interfere with the maintenance of self-tolerance, and external triggers lead to persistence of
nuclear antigens. The result is an antibody response against self nuclear antigens, which is amplified by the action of nucleic acids on dendritic cells (DCs) and B cells, and the production of type 1 interferons. IFN, Interferon; TLRs, Toll-like receptors.

ag ab complex deposit in tissue
inflamation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

…………….. impairs removal of circulating immune complexes

A

Inherited deficiency of early complement components

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

……….. are linked to the production of anti-ds DNA, anti-Sm and AP antibodies

A

HLA-DQ

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

drugs induce sle

A

Drugs (hydralazine and procainamide)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

clinical manifestations specific 4

A

butterfly rash
discoid rash
alopecia
raynauld syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

lupus erythematous cell

A

neutrophils
engulfed ag ab comples
nucleus pushed to periphery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

kidney sle

A

lupus nephritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

classification of lupus nephritis 6

A

class 1 minimal - normal
class 2 mesangial prolerative - microscopic hematuria
class 3 focal lupus nephritis- hematuria proteinuria

class 4 diffuse- like 3 but with decresed c3 and highdsDNA

class 5 membranous nephropathy-nephrotic syndrome

class 6 advanced sclerosing lupus - renal fail

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

class 3 and 4 present with

A

wire loop lesions thickening of capill
hematoxylin bodies - LE cells
hyaline thrombi - aggregation of complex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

spleen sle

A

Capsular thickening,
follicular hyperplasia,
increased plasma cells and
thickening of penicilliary arteries (onion skinning)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

skin sle

A

liquefactive degeneration of the basal layer of the epidermis and

edema at the DEJ -derm epiderm junct

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

heart sle and whts the special name

A

vegetation on mitarl valve

libman sacks endocarditis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Drug-Induced Lupus Erythematosus

A

Hydralazine,
procainamide (80% positive for ANAs, 1/3 become symptomatic),
isoniazid, and
D-penicillamine
Anti-TNF therapy

Renal and CNS involvement is uncommon

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Drug-Induced Lupus Erythematosus
serologic finding

A

Anti-histones Ab&raquo_space;> Anti dsDNA Ab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Antiphospholipid antibody syndrome

A

1° or 2° autoimmune disorder (30% of SLE)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

…………….. can cause false positive VDRL (syphilis test) …. type of antiphospholipid

A

Anticardiolipin antibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

……………. can cause prolonged PTT that is not corrected by mixing study … type of antiphospholipid

A

Lupus anticoagulant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

complications of antiphospholipid ab syndrome

A

Arterial and venous thrombosis

(DVT, hepatic vein thrombosis,
placental thrombosis (recurrent pregnancy loss), and stroke)

32
Q

triggers for APA syndrome

A

trauma
infection
non immune procoagulant

33
Q

what antibody exerts protective effect

A

anti lamin b1 ab

34
Q

Sjögren’s syndrome
triad

A

dry mouth
dry eye
inflammatory arthritis

1ᵒ = sicca syndrome and 2ᵒ associated with other autoimmune disease (RA)

35
Q

what antibodies plus types

A

~80% anti-ribonucleoprotein antibodies

SS-A (Ro) and SS-B (La)

36
Q

An increased incidence of ………. seen in sjogren

A

Marginal zone lymphoma (low grade B-cell lymphoma)
non hodgkins type

37
Q

manifestations or sicca syndrom

A

dry eye
dry vagina
dry skin
dry mouth
palpable pupura
raynauld sydrome
enlarged salivary gland (bilateral parotid swelling)

38
Q

Systemic sclerosis character

A

Characterized by: (1) chronic inflammation (autoimmunity) (2) damage to small blood vessels, and (3) progressive interstitial and perivascular fibrosis in the skin and multiple organs.

39
Q

pathogenesis

A

external stimuli

endothelial damage

vasculopathy

ischemia repair fibrosis

or

genetic susceptability plus external stimuli

t and b activation

profibrotic cytokines

fibrosis

40
Q

profibrotic cytokines

A

TGF B
IL14
PDGF

41
Q

2 types of scleroderma

A

diffuse and limited

42
Q

Diffuse scleroderma —

A

widespread skin involvement,
rapid progression,
early visceral involvement.
Anti-Scl-70 antibody (anti-DNA topoisomerase-I antibody) and anti-RNA polymerase III.

43
Q

Limited scleroderma and crest syndrome

A

limited skin involvement confined to fingers and face.

anti-Centromere antibody

CREST syndrome:
Calcinosis cutis C ,
Raynaud phenomenon,
Esophageal dysmotility,
Sclerodactyly, and
Telangiectasia.

44
Q

dermatomyositis

A

inflammatory cells around border of muscle fibers

both skin and muscle

perimysial inflammation

cd4 t cells

45
Q

manifestations

A

heliotrope - eyelid rash purple like flower

malar or butterfly rash

shawl sigh

guttrons papule

calcinosis cutis

ground glass opacity of lungs

46
Q

polymyocitis

A

inflammatory cells invade muscle fibers

endomysial inflammaion

cd8 t cells

47
Q

manifestations

A

trouble raising hand and leg

48
Q

Mixed connective tissue disease

A

An overlap condition with features of SLE, systemic sclerosis, and polymyositis

Typically presents with synovitis of the fingers, Raynaud phenomenon, myositis, and renal involvement

Anti- U1 ribonucleoprotein are nearly always positive

49
Q

IgG4-Related Disease(IgG4-RD)

A

Constellation of disorders characterized by tissue infiltrates dominated by IgG4 producing plasma cells and lymphocytes (particularly T cells), fibrosis and obliterative phlebitis
Usually increased serum IgG4
Middle age and older men
Ideopathic; It is unclear if the disease is truly autoimmune in nature, and no target autoantigens have been identified.
Rituximab provided clinical benefit

50
Q

IgG4-Related Disease(IgG4-RD)
manifestation mnemonic

A

pairs
pancreatitis
interstitial nephritis
riedel thyroditis
retroperitoneal fibrosis
scelerosing sialadentis = salivary

51
Q

what syndrome seen in IgG4 related disease

A

mikulicz syndrome
lacri sali enlargement

52
Q

Immunodeficiency classifications

A

defect on first line second or third line defense

53
Q

first line deficinecy

A

burn
viral infection
primary ciliary dyskinesia

54
Q

Primary ciliary dyskinesia (PCD)

A

.PCD is inherited in an autosomal recessive manner with the exception of FOXJ1-PCD (which is autosomal dominant) and PIH1D3-PCD and OFD1-PCD (which are X-linked).

55
Q

Kartagener syndrome (50% of PCD) is a subgroup of the immotile cilia syndrome
triad

A

dextro cardia
bronchiectasis
sinusitis

56
Q

Abnormalities of cilia include

A

absence of both inner and outer dynein arms, absence of spoke heads, and absence of one or both central microtubules.

57
Q

defect in second line defense

A

leukocyte adhesion deficiency

Chédiak-Higashi syndrome

Deficiencies of oxygen-dependent killing

complement defect

58
Q

leukocyte adhesion deficiency 1 special

A

AR
deficiency of B2 integrin or CD18

recurrent bacterial infection and umlical doesnt heal

59
Q

Chédiak-Higashi syndrome 1 special

A

AR
Characterized by neutropenia defect in chemotaxis and degranulation (LYST mutation)

partial albinism

60
Q

What is seen in all chediak higashi syndrome WBCs

A

abnormal granules

61
Q

Deficiencies of oxygen-dependent killing
2 types

A

Chronic granulomatous disease of childhood:

Myeloperoxidase deficiency

62
Q

Chronic granulomatous disease of childhood: ntt result

A

XR/AR

Absence/deficiency of NADPH oxidase

Recurrent bacterial infections with catalase-positive organisms (S. aureus)

NTT will be -Ve nitro tetrazolium test

63
Q

Myeloperoxidase deficiency ntt result

A

AR
Infections with Candida
NTT will be +Ve

64
Q

complement defects plus types

A

C5, C6, C7, C8, Factor B and properdin deficiencies  increased Neisseria infections

C1q, C1r, C1s, C2, C3 and C4 deficiencies  increases in immune complex diseases and recurrent bacterial infections

2 types
Hereditary angioedema
paroxysmal nocturnal hemoglobinuria

65
Q

Hereditary angioedema

A

defect in C1 esterase inhibitor

c1 esterase produce bradykinin
which increase permeability
so edema

66
Q

PNH

A

defect in PIGA
leads to complements not being inhibited so hemolysis
black urine

67
Q

third line defense defect list 7

A

X linked agammaglobulinaemia - XLA

Common variable immunodeficiency (CVID)

selective IgA deficiency

DiGeorges syndrome

SCID

Hyper IgM syndrome

wiskotts aldrich syndrome

68
Q

X-linked agammaglobulinaemia (XLA )

A

= Bruton disease
Failure of B cell maturation and absence of gammaglobulin in the blood

Mutations on Bruton’s Tyrosine Kinase (BTK) gene

Manifests by about 6 months of age

Recurrent bacterial or viral infections and giardia lamblia infections

69
Q

Common variable immunodeficiency (CVID)

A

Heterogeneous group of disorders

Defect in B-cell differentiation and antibody production due to unknown cause

Plasma cells are absent, perhaps due to a block in antigen-stimulated B cell differentiation

May present in childhood but usually diagnosed after puberty (onset in second decade)

↑risk of autoimmune disease, bronchiectasis, lymphoma, sinopulmonary infections

70
Q

selective IgA deficiency

A

most common primary

low IgA but others all normal

high penetration of allergens through the mucosa due to defective serum and mucosal IgA

71
Q

DiGeorge syndrome

A

Absence of the parathyroid glands and thymus

hypocalcemia and tetany

T-cell deficiency  viral and fungal organisms

Deletion of chromosome 22q11.2

72
Q

DDiGeorge syndrome mnemonic

A

catch 22
cardiac problems
abnormal facies
thymic aplasia
cleft palate
hypocalcemia

72
Q

Severe combined immunodeficiency (SCID)

A

Deficiency of cell-mediated and humoral immunity
Often caused by a progenitor-cell defect
Recurrent viral, bacterial, fungal, and protozoal infections
Most infants die of infection within a year

73
Q

types of SCID

A

Types

IL-2R gamma chain (most common,XR);

Adenosine deaminase deficiency (AR);- purines defect toxic

RAG mutation - VDJ recombination defect

74
Q

Hyper-IgM syndrome

A

Most commonly due to defective

CD40L(X-linked recessive) on Th cells

Class switching defect; Normal or ↑IgM and↓IgG, IgA, IgE

Failure to make germinal centers

75
Q

Wiskott–Aldrich syndrome

A

Due to mutations in WASP gene so defect in APC

Age related depletion of T lymphocytes

Inability to synthesize antibodies to polysaccharide antigens (encapsulated pyogenic organisms)

↑risk of autoimmune disease and NHL

76
Q

Ab number

A

IgM decreased
IgG same
IgE and IgA high

77
Q

wiskott aldrich triad
water

A

thrombocytopenia
eczema
recurrent infection

78
Q

Hv

A

Fg

Patho (33 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions