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Clinical Lab > autoimmune juju > Flashcards

autoimmune juju Flashcards

1
Q

What specific autoimmune disease can affect the brain?

A

MS, Guillian Barre Syndrome, Autism

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2
Q

What specific autoimmune disease can affect the blood?

A

Leukemia, Lupus erythematous, Hemolytic Dysglycemia

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3
Q

What specific autoimmune disease can affect the GI tract?

A

Celiac Disease Crohns Disease Ulcerative Colitis DM Type 1

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4
Q

What specific autoimmune disease can affect the skin?

A

Vitiligo Eczema Scleroderma Psoriasis

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5
Q

What specific autoimmune disease can affect the muscles?

A

Muscular Dystrophy Fibromyalgia

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6
Q

What specific autoimmune disease can affect the bones?

A

Rheumatoid Arthritis Polymyalgia Rheumatica Amylosing Spondylosis

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7
Q

Receptors on __________ of the immune system are antigen specific with diversity derived from ____________

A

T & B cells gene recombination

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8
Q

Autoimmune Diseases = immune responses to _________.

A

Immune response to self antigens occur in the context of a genetic predisposition resulting in disease expression. Can be organ or system

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9
Q

Primary Immunodefiency

A

A direct consequence of structural or functional derangement in the immune network.

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10
Q

Secondary immunodeficiency

A

A consequence of an alteration in the immune system resulting from a ds process (HIV, CMV, cancer, myelomas)

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11
Q

What is Systemic Lupus Erythematosus?

A

Multisystem autoimmune disease, associated with the production of antibodies to a variety of nuclear and cytoplasmic antigens

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12
Q

What do pts with systemic lupus erythematous have antibodies to?

A

antibodies to: Double stranded DNA
-causes inflammation to skin, joints, kidney, cardiovascular system, nervous system, lung and hemopoietic cells.

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13
Q

What are diagnostic signs of systemic lupus erythematous?

A

Butterfly rash on cheeks + forehead
- arthritis
- oral ulcers
- proteinuria
- cytopenia

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14
Q

What are the labs for systemic lupus erythematous?

A
  • Positive anti-ds DNA
  • Positive antiphospholipid antibody
  • Positive anti-Sm antibody
  • false positive test for syphilis
  • abnormal/”speckled ANA”
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15
Q

With systemic lupus erythematous what false positive test?

A

False positive syphillis test

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16
Q

Sjogren Syndrome description

A
  • Systemic connective tissue disorder
  • more common in women
  • an autoimmune exocrinopathy: lacrimal glands, salivary glands and less often the pancreas (moisture producing glands)
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17
Q

What are symptoms of sjogren syndrome?

A

Inflammation contributes to sicca syndrome, dry eyes and mouth
-glandular swelling
- Dry mouth, skin, throat, eyes , dry nose, dry vagina
- Joint /muscle pain
- recurrent bronchitis
- abnormal liver function
- digestive problems

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18
Q

What are the labs for Sjogren Syndrome

A

Positive ANA Positive SS-A Positive SS-B Positive rheumatoid factor

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19
Q

What diagnostic tests are performed for Sjogren Syndrome

A
  • Test for sicca.
  • Dry eye Schirmer test. (tissue paper
  • should have a certain mm of tears)
  • Bx of salivary gland
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20
Q

What can 2.5% of individuals with sjrogen syndrome develop?

A

Non hodgkins lymphoma

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21
Q

What is Scleroderma and 3 main characterisitics

A
  • Excessive and widespread deposition of collagen in many organ systems of the body
  • common in old ppl, age 70+ characteristics:
  • tissue fibrosis
  • Proliferative and occlusive vasculopathy of the small blood vessels
  • Specific autoimmune response w distinctive autoantibody profile
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22
Q

Scleroderma sx

A

CREST Calcinosis: calcium deposits in skin Raynaud’s Phenomenum: spasm of blood vessels in response to cold/stress Esophageal Motility Dysfunction: acid reflux,decreased motility Sclerodactyly: thickening +tightening of skin on fingers/hands Telangiectasias: dilation of capilarries-red marks on skin

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23
Q

What are the labs for scleroderma

A

Positive ANA Antibody Scl-70

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24
Q

What is involved in scleroderma

A
  • Skin
  • GI tract
  • kidney(renal ischemia)
  • lung
  • muscles
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25
Q

What are the 3 types of inflammatory muscle disease

A

Dermatomyositis. (derm signs) Polymyositis. (t cell mediated muscle injury) Inclusion body myositis. (older pts) (10% have pulmonary interstitial fibrosis)

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26
Q

What is dermatomyositis

A

Heliotrope rash shawl sign: redness where a shawl is Gottron papules. Complement vasculopathy

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27
Q

What are the labs for inflammatory muscle disease

A

positive Anti Jo Positive ANA “not your average jo, he has (inflamed) muscles”

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28
Q

What are the criteria for inflammatory muscle disease? How much of criteria must be met?

A

3/5 criteria:
- Proximal muscle weakness
- Muscle pain and tenderness on palpation
- EMG abnormal
- Elevated serum/plasma concentrations of muscle enzymes
- Muscle bx with cellular inflammation

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29
Q

Mixed Connective Tissue Disease involves combined features of

A
  • SLE
  • sclerosis,
  • polymyositis (t cell mediated muscle injury)
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30
Q

What symptoms of mixed connective tissue disease?

A
  • Arthralgias/myalgias
  • fatigue
  • Raynaud phenomenon
  • Interstitial pneumonitis
  • Pulmonary HTN
  • Interstitial Fibrosis
  • diaphragm & esophagus disfunction
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31
Q

What are labs for mixed connective tissue disease? how do you dx?

A

Positive ANA Anti-U1 RNP Dx: by clinical features + positive ANA, positive anti-U1 RNP

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32
Q

Rheumatoid Arthritis definition

A

Systemic autoimmune connective tissue disorder
- primarily affects the synovial joints often starting as a synovitis
- MC: young women
- stiffness in the morning-> as the joints lubricate during the day it improves
- >

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33
Q

You must meet ______ criteria for Rheumatoid Arthritis

A

3/7 criteria with morning stiffness

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34
Q

Rheumatoid arthritis is most common in

A

young women

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35
Q

What are the labs for Rheumatoid Arthritis

A
  • Increased serum Rheumatoid Factor
  • Anti- CCP
36
Q

What is Amyloidosis?

A
  • Extracellular deposition of low-molecular-weight fibrils giving a waxy appearance to the infiltrated organs
  • multiorgan involvement
  • factor x coagulopathy
  • MC: males (2:1); 40s onset
37
Q

Who is amyloidosis most common in? Typical Onset

A

Male 2:1, onset 40’s

38
Q

What are symptoms of amyloidosis

A

sx due to bone marrow disorder: misfolded proteins can accumulate in the body tissues, nerves and organs:
- Fatigue
- SOB
- Proteinuria
- Stiff Heart
- Diarrhea/Consipation
- Kidney Failure
- BP Changes
- Dizzy
- Weight Loss abdominal fat pad

39
Q

What testing for amyloidosis?

A

+ Congo red stain on microscopy abdominal fat pad “amy loves congo red lipstick”

40
Q

In amyloidosis , _____involvement Factor _____

A

Multiorgan involvement Factor X coagulopathy

41
Q

Cryoglobulinemia: immunoglobulins in the serum precipitate at a temp…

A

immunoglobulins in the serum precipitate at a temp… Below 37C, resolves on warming Immune complex vascultitis, arthritis, neuropathy, renal involvement

42
Q

How to diagnose cryoglobulinemia?

A

Wintrobe tube collects cyroglubulin reported as a cryocrit

43
Q

What is X-linked agammaglobulinemia?

A

Absence of B lymphocyte in males
-causes recurrent Infections

44
Q

What is common variable immunodeficency and its characteristics

A
  • Affects both male and female adults
  • B cells dysfunction characteristics:
    -Recurrent infections
  • Neutropenic
  • Immune Hemolytic Anemia
  • Pernicious anemia
  • B cell Lymphoma
45
Q

What are frequent symptoms of common variable immunodeficency?

A
  • chronic cough
  • swollen lymph nodes
  • breathing troubles
  • ear pain
  • n/v/d
  • wt loss
46
Q

What is hyper-IgM syndrome?

A

Normal to High IgM with:
- Low IgG + IgA
- normal B cells: but can’t undergo antibody class switching
- cant make antibodies

47
Q

What is the most common primary immunodeficency syndrome?

A

Selective IgA deficency

48
Q

What is the IgA for selective IgA deficency

A

IgA <5 MG.DL with normal IgG and IgM

49
Q

Selective IgA deficiency characteristics

A
  • Asymptomatic to Allergies
  • Autoimmune disorders
  • GI disease
  • Pulm Ds
  • Recurrent infections
50
Q

What is DiGeorge syndrome?

A

Chromosome 22 deletion
- T + B cell abnormalities
- Abnormal parathyroid CATCH 22
- Cardiac abnormality
- Abnormal facies
- Thymic hypoplasia
- Cleft Palate
- Hypocalcemia

51
Q

What is severe combined immunodeficiency (SCID)?

A

Cellular and humoral immunity defects
-> bubble babies

52
Q

severe combined immunodeficiency (SCID) screen/dx and tx

A

Dx: Neonate heel prick test tx: Bone marrow transplant
-Bubble babies

53
Q

What does complement protect against

A
  • Pathogens and non self antigens
  • cytokine release
  • stimulation of phagocytes
  • inflammation
    -cell killing attack deficiencies = susceptible to infections and predisposes to autoimmune ds
54
Q

What would a deficency in complement result in

A
  • Susceptibility for infections
  • predispose to autoimmune disease
55
Q

What test is performed for deficencies in complement?

A

CH50 test
-Uses hemolyze antibody coated sheep red cells complement deficient or congenitally deficienct:
- low

56
Q

What is AH50 test?

A

screening test for complement abnormalities in the alternative pathway:
- Addition of complement restore hemolytic activity

57
Q

What is organ rejection primarily a consequence of ?

A

The interactions between the immune system of the transplant recipient and the histocompatibility antigens present on the transplanted cells.

58
Q

Histocompatibility antigens are encoded by a complex of closely linked genes called

A

Major Histocompatibility Complex (MHC)
- found on the short arm of chromosome 6
- Encodes for the human leukocyte antigen system (HLA )

59
Q

HLA Class I genes include what 3 types?

A

HLA-A HLA-B HLA-C

60
Q

HLA Class 2 genes include what 3 types?

A

HLA-DP HLA-DQ HLA-DR

61
Q

What do Class I genes interact with

A
  • T lymphocytes (cytotoxic t cells, t suprressor cells) and CD8 cells
  • Found on surface of all cells w nucleus
    -> NOT RBCs
  • Antigen of infection binds to class I HLA surface antigen and triggers immune reaction
62
Q

What do Class 2 genes interact with

A
  • T lymphocytes, (T helper cells) and CD4 cells
  • Found on surface of B lymphocytes, macrophages dendritic cells and t cells
  • Help immune cells communicate with each other through IL-1 and IL-2
    —— When a macrophage attacks an organism the antigen binds from the pathogen which releases interleukin 1, calls helper t cells which release interleukin 2, which calls b cells-antibodies and t cells- cytotoxic
63
Q

What do HLA Class 3 include

A

Cytokines Complement Proteins (prone to hemachromatosis)

64
Q

What are HLA-A 3 prone to

A

Hemachromatosis A3
-> Fe3+ overload

65
Q

What are HLA-B 8 prone to**

A

Graves disease, Addisons disease, Myasthenia gravis GAM = B8

66
Q

What are the HLA-B27-associated disorders?***

A

Psoriasis Ankylosing spondylitis IBS Reiter syndrome PAIR = B27***
-> pair by 27 yrs old

67
Q

What are HLA-D R2 associated disorders?

A

MS SLE

68
Q

What are HLA-D R3 +R4 associated disorder?

A

DM type 1

69
Q

What are HLA-D R5 associated disorder?

A

Hashimoto

70
Q

___ chance of two siblings being identical, ____ of sharing one haplotype

A

25%,50% currently, 17,500 HLA alleles= bone marrow match difficult needs to be identical

71
Q

What are HLA-D Q2 associated disorder?

A

Celiac Disease

72
Q

Matching HLA with Siblings

A

Each person has two sets of HLA genes, called haplotypes—one inherited from each parent
- currently 17,500 HLA alleles
- HLA match between siblings or no match at all is 25%
- 50% chance of siblings sharing one haplotype

73
Q

Histocompatibility Testing Assays include

A

HLA Typing HLA Antibody Screening Crossmatching

74
Q

What is HLA typing?

A

Uses microlymphocytotoxicity assay: T lymphocytes and B lymphocytes are used to detect Class I (t) and Class II (b) HLA antigens,
- Antibodies in the serum bind to these antigens, causing cell lysis, which is observed to check for matches. Uses DNA polymerase chain reaction: for specific antigens

75
Q

What is HLA antibody screening

A
  • Serum screened for antibodies from prior exposure
  • Percent panel reactive antibody (PRA)
  • High PRA: indicates a high risk of rejection, necessitating waiting for a donor with a 0% PRA (no detected HLA antibodies)
    —— Percent Panel Reactive Antibody (PRA): Indicates the percentage of antibodies against a panel of known HLA types
76
Q

What is crossmatching?

A

Ensure that the recipient’s serum does not contain antibodies that react against the donor’s lymphocytes, which would predict transplant rejection For Renal, heart, lung transplants

77
Q

If recipient has HLA antibodies against donor antigens

A

No transpant match! The cells will be lysed

78
Q

Organ Transplant Requirements: kidney, liver, pancreas, heart, lungs, cornea

A

Kidney and Pancreas:
- ABO compatible
- crossmatch lymphocytes Lungs and Heart:
- ABO compatible
- additional virtual crossmatch needed if high HLA antibodies are present in the recipient Liver:
- ABO compatible
- Donor specific HLA antibodies not a contraindication Cornea:
- no HLA matching
- No ABO matching

79
Q

Histocompatibility: Solid Organ Transplants Kidney +Pancreas

A
  • ABO compatible
  • crossmatch lymphocytes
80
Q

Histocompatibility: Solid Organ Transplants Liver

A
  • ABO blood group compatibility
  • Donor specific HLA antibodies are NOT contraindication.
81
Q

Histocompatibility: Solid Organ Transplants Heart+ Lung

A
  • ABO compatible
  • additional virtual crossmatch needed if high HLA antibodies are present in the recipient
82
Q

Histocompatibility: Solid Organ Transplants Cornea

A

MC tissue transplant doesnt need any match NO HLA or ABO matching

83
Q

Normal hematopoietic cell transplantation

A
  • Hematopoietic transplantation is a therapeutic option for patients undergoing cytotoxic therapy for malignancy
  • Harvested from the bone marrow or from the peripheral blood after giving the donor growth factors
    ——- Hematopoietic transplantation involves the infusion of hematopoietic stem cells to restore bone marrow function in patients whose marrow has been destroyed
84
Q

Even with perfect HLA matching, what can occur

A

2% graft failure

85
Q

HLA typing can be used to help identify increased risk of a drug hypersensitivity to

A

Hypersensitivity to :
- Carbamazepine
- allopurinol
- abacavir seizure med, anti virals

Clinical Lab (17 decks)

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