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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

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2
Q

2 type of tolerance

A

central and peripheral tolerance

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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

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4
Q

Mechanisms of development of peripheral tolerance

A

colonal anergy
colonal deletion
peripheral suppresion by t cells
ag sequestration

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5
Q

Clonal anergy;

A

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

B7 - CD28

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6
Q

Clonal deletion by activation-induced cell death

A

Fas by Fas L induces apoptosis

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6
Q

Peripheral suppression by T cells

A

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

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7
Q

Antigen sequestration

A

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

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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

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9
Q

systemic

A

SLE
rhematoid arthriitis
sclerodemra
sjogrens sydrome
polyarteritis nodosa
inflammatory myopathies

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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)

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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

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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.

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13
Q

anti what antibodies

A

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

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14
Q

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

A

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

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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

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16
Q

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

A

Inherited deficiency of early complement components

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17
Q

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

A

HLA-DQ

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18
Q

drugs induce sle

A

Drugs (hydralazine and procainamide)

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19
Q

clinical manifestations specific 4

A

butterfly rash
discoid rash
alopecia
raynauld syndrome

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20
Q

lupus erythematous cell

A

neutrophils
engulfed ag ab comples
nucleus pushed to periphery

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21
Q

kidney sle

A

lupus nephritis

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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

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23
Q

class 3 and 4 present with

A

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

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24
spleen sle
Capsular thickening, follicular hyperplasia, increased plasma cells and thickening of penicilliary arteries (onion skinning)
25
skin sle
liquefactive degeneration of the basal layer of the epidermis and edema at the DEJ -derm epiderm junct
26
heart sle and whts the special name
vegetation on mitarl valve libman sacks endocarditis
27
Drug-Induced Lupus Erythematosus
Hydralazine, procainamide (80% positive for ANAs, 1/3 become symptomatic), isoniazid, and D-penicillamine Anti-TNF therapy Renal and CNS involvement is uncommon
27
Drug-Induced Lupus Erythematosus serologic finding
Anti-histones Ab >>> Anti dsDNA Ab
28
Antiphospholipid antibody syndrome
1° or 2° autoimmune disorder (30% of SLE)
29
................. can cause false positive VDRL (syphilis test) .... type of antiphospholipid
Anticardiolipin antibodies
30
................ can cause prolonged PTT that is not corrected by mixing study ... type of antiphospholipid
Lupus anticoagulant
31
complications of antiphospholipid ab syndrome
Arterial and venous thrombosis (DVT, hepatic vein thrombosis, placental thrombosis (recurrent pregnancy loss), and stroke)
32
triggers for APA syndrome
trauma infection non immune procoagulant
33
what antibody exerts protective effect
anti lamin b1 ab
34
Sjögren’s syndrome triad
dry mouth dry eye inflammatory arthritis 1ᵒ = sicca syndrome and 2ᵒ associated with other autoimmune disease (RA)
35
what antibodies plus types
~80% anti-ribonucleoprotein antibodies SS-A (Ro) and SS-B (La)
36
An increased incidence of .......... seen in sjogren
Marginal zone lymphoma (low grade B-cell lymphoma) non hodgkins type
37
manifestations or sicca syndrom
dry eye dry vagina dry skin dry mouth palpable pupura raynauld sydrome enlarged salivary gland (bilateral parotid swelling)
38
Systemic sclerosis character
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
pathogenesis
external stimuli endothelial damage vasculopathy ischemia repair fibrosis or genetic susceptability plus external stimuli t and b activation profibrotic cytokines fibrosis
40
profibrotic cytokines
TGF B IL14 PDGF
41
2 types of scleroderma
diffuse and limited
42
Diffuse scleroderma —
widespread skin involvement, rapid progression, early visceral involvement. Anti-Scl-70 antibody (anti-DNA topoisomerase-I antibody) and anti-RNA polymerase III.
43
Limited scleroderma and crest syndrome
limited skin involvement confined to fingers and face. anti-Centromere antibody CREST syndrome: Calcinosis cutis C , Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia.
44
dermatomyositis
inflammatory cells around border of muscle fibers both skin and muscle perimysial inflammation cd4 t cells
45
manifestations
heliotrope - eyelid rash purple like flower malar or butterfly rash shawl sigh guttrons papule calcinosis cutis ground glass opacity of lungs
46
polymyocitis
inflammatory cells invade muscle fibers endomysial inflammaion cd8 t cells
47
manifestations
trouble raising hand and leg
48
Mixed connective tissue disease
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
IgG4-Related Disease(IgG4-RD)
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
IgG4-Related Disease(IgG4-RD) manifestation mnemonic
pairs pancreatitis interstitial nephritis riedel thyroditis retroperitoneal fibrosis scelerosing sialadentis = salivary
51
what syndrome seen in IgG4 related disease
mikulicz syndrome lacri sali enlargement
52
Immunodeficiency classifications
defect on first line second or third line defense
53
first line deficinecy
burn viral infection primary ciliary dyskinesia
54
Primary ciliary dyskinesia (PCD)
.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
Kartagener syndrome (50% of PCD) is a subgroup of the immotile cilia syndrome triad
dextro cardia bronchiectasis sinusitis
56
Abnormalities of cilia include
absence of both inner and outer dynein arms, absence of spoke heads, and absence of one or both central microtubules.
57
defect in second line defense
leukocyte adhesion deficiency Chédiak-Higashi syndrome Deficiencies of oxygen-dependent killing complement defect
58
leukocyte adhesion deficiency 1 special
AR deficiency of B2 integrin or CD18 recurrent bacterial infection and umlical doesnt heal
59
Chédiak-Higashi syndrome 1 special
AR Characterized by neutropenia defect in chemotaxis and degranulation (LYST mutation) partial albinism
60
What is seen in all chediak higashi syndrome WBCs
abnormal granules
61
Deficiencies of oxygen-dependent killing 2 types
Chronic granulomatous disease of childhood: Myeloperoxidase deficiency
62
Chronic granulomatous disease of childhood: ntt result
XR/AR Absence/deficiency of NADPH oxidase Recurrent bacterial infections with catalase-positive organisms (S. aureus) NTT will be -Ve nitro tetrazolium test
63
Myeloperoxidase deficiency ntt result
AR Infections with Candida NTT will be +Ve
64
complement defects plus types
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
Hereditary angioedema
defect in C1 esterase inhibitor c1 esterase produce bradykinin which increase permeability so edema
66
PNH
defect in PIGA leads to complements not being inhibited so hemolysis black urine
67
third line defense defect list 7
X linked agammaglobulinaemia - XLA Common variable immunodeficiency (CVID) selective IgA deficiency DiGeorges syndrome SCID Hyper IgM syndrome wiskotts aldrich syndrome
68
X-linked agammaglobulinaemia (XLA )
= 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
Common variable immunodeficiency (CVID)
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
selective IgA deficiency
most common primary low IgA but others all normal high penetration of allergens through the mucosa due to defective serum and mucosal IgA
71
DiGeorge syndrome
Absence of the parathyroid glands and thymus hypocalcemia and tetany T-cell deficiency  viral and fungal organisms Deletion of chromosome 22q11.2
72
DDiGeorge syndrome mnemonic
catch 22 cardiac problems abnormal facies thymic aplasia cleft palate hypocalcemia
72
Severe combined immunodeficiency (SCID)
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
types of SCID
Types IL-2R gamma chain (most common,XR); Adenosine deaminase deficiency (AR);- purines defect toxic RAG mutation - VDJ recombination defect
74
Hyper-IgM syndrome
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
Wiskott–Aldrich syndrome
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
Ab number
IgM decreased IgG same IgE and IgA high
77
wiskott aldrich triad water
thrombocytopenia eczema recurrent infection
78
Hv
Fg

Patho (33 decks)

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