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MSK week 2 > immunology > Flashcards

immunology Flashcards

1
Q

WHAT IS autoimmunity?

A

The presence of immune response against self-tissue/cells

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2
Q

What is the pathogenesis of autoimmune disease?

A

Genetic susceptibility
(Irritating event)
Breakdown of self-tolerance-loss of immune regulation and generation of auto reactive T and B cells
This leads to autoimmune phenomena and disease

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3
Q

What are monogenic disorders

A

single gene defect causing autoimmune disease - very rare

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4
Q

What is IPEX syndrome

A

presents in early childhood

Characterised by overwhelming systemic immunity

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5
Q

in harmless autoimmunity there is…

A

low titres of auto-antibodies or auto-reactive T cells

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6
Q

in harmful autoimmunity there is…

A

high titres of auto-antibodies or auto-reactive T cells

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7
Q

most autoimmune diseases result from complex genetic interplay between..

A

HLA genes
Genes determining sex
Other immune response genes

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8
Q

What does IPEX syndrome stand for?

A

Immune dysregulation
Polyendocrinopathy
Enteropathy
X-linked inheritance syndrome

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9
Q

What is the pathogenesis of IPEX syndrome

A

Pathogenesis- mutation in FOXP3 gene which is essential for development of regulatory T cells

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10
Q

What are the symptoms of IPEX syndrome?

A

Symptoms include- severe infections, intractable d, eczema, very early onset insulin onset diabetes and autoimmune manifestations

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11
Q

What is the treatment of IPEX syndrome?

A

Treatment

cure- Haematopoetic stem cel transplantation (HSCT)

supportive care- immunosuppressive drugs plus total parental nutrition

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12
Q

What gives rise to hugely diverse B cells and T cell populations (allowing for recognition of millions of different antigens)

A

Random rearrangement of TCR a and b chain gene segments (or Ig heavy and light chain gene segments) occurs in developing T cells and B cells

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13
Q

What are the two specific tolerance mechanisms required

A

Removal of self-reactive lymphocytes in primary lymphoid tissues (central tolerance)

Inactivation of self-reactive lymphocytes that escape central tolerance (peripheral tolerance) e.g. Regulatory T cells

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14
Q

What percentage of regulatory T cells make up the normal CD4+ T cell population

A

5-10%

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15
Q

_ _____ __ express Class 1 MHC on the cell surface

A

all nucleated cells

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16
Q

________ __ also express Class 11 on the cell surface

A

Specialised APC

17
Q

What is Molecular mimicry?

A

cross-reactivity between antigens expressed by pathogen and self

18
Q

What type of hypersensitivity reaction is SLE?

A

type III

19
Q

What type of hypersensitivity reaction is RA?

A

type IV

20
Q

What type of hypersensitivity is blood transfusions?

A

Type II

21
Q

What is SLE

A 
rare
type 111 hypersensitivity disease
increased risk of CVD
peak age of onset in 2nd and 3rd decades
high female preponderance
genetic predisposition
22
Q

What is the pathogenesis of SLE?

A

Infection –> ROS production
UV radiation

mutations in C1q(C2,C4)
Mutations in Mac-1

B cell activation–> production of auto-antibodies

23
Q

A person has SLE if any 4 out of these 11 features are present serially or simultaneously

A

SOAP BRAIN MD

Serositis
Oral Ulcers
Arthritis
Photosensitivity

Blood (Haematological disorder)
Renal disorder
ANA disorder
Immunological disorder
Neurological disorder

Malar rash
DIscoid rash

24
Q

Where is ANA highly useful in diagnosis?

A

SLE
Mixed CTD
Autoimmune hepatitis

25
Q

What is anti-dsDNA, anti-Sm present in?

A

SLE

26
Q

What are anti-Ro and anti-La associated with?

A

Sjogren’s syndrome

27
Q

What is Scl-70 and anti U3-RNP found in?

A

SS

28
Q

What is jo-1 found in?

A

PM

29
Q

Type III hypersensitivity reactions:management

A

Limit sun exposure
analgesia
hydrochloroquinine
Decrease inflammation- corticosteroids ( prednisone)
Decrease production of auto-antibodies (Immunosuppresive agents and inhibit B cell proliferation)

30
Q

What is the major susceptibility halotype in most ethnic groups in RA

A

HLA-DR4

31
Q

What are the two pro-inflammatory cytokines involved in the pathogenesis of RA

A

TNFa

IL-1

32
Q

What pro-inflammatory cytokine plays a critical role within the joint in RA

A

IL-6

33
Q

What is RF?

A

A rheumatoid factor is an auto-antibody directed against the common (Fc) region of human IgG

(only 70% of patients with RA are +ve for RF at the time of diagnosis

34
Q

What makes ACPA particularly wonderful in the diagnosis of RA?

A

Highly specific for RA, occurring in many patients before clinical onset of the disease

MSK week 2 (11 decks)

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