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S3B5 Pathology > Systemic Autoimmune Diseases > Flashcards

Systemic Autoimmune Diseases Flashcards

1
Q

What are the two basic mechanisms of self-tolerance?

A
  • Central tolerance
    • immature lymphocytes that recognize self-antigens in the central lymphoid organs are normally triggered to die via apoptosis
  • Peripheral tolerance
    • mature lymphocytes that recognize self-antigens in peripheral tissue either become functionally inactive (anergic), are suppressed by Treg cells, or are triggered to die by apoptosis
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2
Q

What is the fundamental problem with autoimmune disorders?

A

breakdown in self-tolerance

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3
Q

What are factors that lead to autoimmunity?

A

susceptible genes (HLA & non-HLA)

infections & tissue injury - expose self antigens & activate antigen presenting cells/lymphocutes in those tissues

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4
Q

What are the 6 organ specific autoimmune disorders mediated by antibodies?

A
  1. autoimmune hemolytic anemia
  2. autoimmune throbocytopenia
  3. atrophic gastritis of pernicious anemia
  4. myasthenia gravis
  5. graves disease
  6. goodpasture syndrome
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5
Q

What is the systemic autoimmune disease mediated by antibodies?

A

Systemic Lupus Erythematous

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6
Q

What are the 2 organ specific autoimmune disorders mediated by T-cells?

A

Type 1 Diabetes Mellitus

Multiple Sclerosis

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7
Q

What are the 3 systemic autoimmune diseases mediated by T-cells?

A

Rheumatoid arthritis

systemic sclerosis (scleroderma)

Sjogren syndrome

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8
Q

What are the 3 organ specific diseases that are postulated to be autoimune?

A

inflammatory bowel disease

primary biliary cholangitis

autoimmune hepatitis

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9
Q

What are the 2 systemic diseases postulated to be autoimmune?

A

polyarteritis nodosa

inflammatory myopathies

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10
Q

SLE is particularly associated with what autoantibodies?

A

Antinuclear Antibodies (ANA)

  • DNA
  • Histones
  • Non-histone proteins bound to RNA
  • Nucleolar antigens
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11
Q

What is the typical clinical presentation of a patient with SLE?

A

Female patient in 20s/30s

more common in black & hispanic patients

acute or insidious onset

injury to skin, joints, kidneys, & sclerosal membranes

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12
Q

What is the mechanism of injury seen in SLE?

A

deposition of immune complexes & binding of antibodies to various cells and tissues

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13
Q

What is the pneumonic associated with the diagnostic criteria for SLE?

A

SOAP BRAIN MD

4+ either serially or simultaneously

  • S:erositis
  • O:ral ulcers
  • A:rthritis
  • P:hotosensitivity
  • B:lood disorders
  • R:enal involvement
  • A:ntinuclear Antibodies
  • I:mmunologic phenomena
  • N:eurologic disorder
  • M:alar Rash
  • D:iscoid rash
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14
Q

The provided image is an example of what SLE criteria?

A

Serositis (pleural effusion)

(also includes pericarditis)

chronic interstitial fibrosis & secondary pulmonary hypertension sen in some cases

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15
Q

The provided image is an example of what SLE criteria?

A

Oral ulcers

(usually painless)

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16
Q

The provided image is an example of what SLE criteria?

A

arthritis

(non-erosive, involving two or more peripheral joints)

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17
Q

The provided image is an example of what SLE criteria?

A

Photo sensitivity

(rash in reaction to light exposure)

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18
Q

The provided image is an example of what SLE criteria?

A

Blood disorders

  • neutropenia
  • anemia
  • lymphocytopenia
  • thrombocytopenia
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19
Q

The provided image is an example of what SLE criteria?

A

Renal Involvement

(persistent proteinuria / cellular casts)

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20
Q

What are the immunologic phenomena associated with SLE?

A

anti-dsDNA

anti-Smith [Sm] antibodies

anti-phospholipid antibodies

LE cell (outdated test)

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21
Q

The provided image is an example of what SLE criteria?

A

Antinuclear antibodies (in absence of drugs known to cause lupus-like syndromes)

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22
Q

What neurologic disorders are associated with SLE?

A

seizure

psychosis

(non-inflammatory occlusion of small vessels – NO vasculitis has been found in CNS)

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23
Q

The provided image is an example of what SLE criteria?

A

Malar rash

(characteristic “butterfly rash”; fixed erythema, either raised or flat, over malar eminences, usually sparing nasolabial folds)

  • similar rash can also be seen on extremities & trunk
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24
Q

The provided image is an example of what SLE criteria?

A

Discoid Rash

(focal, raised, red patches, with adherent keratotic scaling and follicular plugging; atrophic scarring in older lesions)

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25
Q

Briefly describe the proposed pathogenesis model for SLE

A
  • Susceptibility genes interfere with maintenance of self-tolerance
  • External trigger → persistence of nuclear antigens
    • antibody response agains self nuclear antigen
    • amplified by dendritic cells & B cells
    • production of type 1 interferon

**not a testing point, but good to understand

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26
Q

What are the possible contributing factors that lead to the development of SLE?

A
  • Genetic Factors
    • MHC & non-MHC
  • Environmental factors
    • UV light
    • Drugs
  • Sex hormones & gene on X chromosome
  • Immunologic factors
    • Self-tolerance failure for nucleosomal antigens
    • TLR engagement by nuclear DNA / RNA
    • Type 1 interferons - lymphocyte activation
    • other cytokines
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27
Q

What drugs are most commonly associated with drug-induced lupus?

A

hydralazine

isoniazid

procainamide

d-penicillamine

Anti-hypertensive & anti-fungal medicines are the most common culprits

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28
Q

What is the most commonly-use method to detect antibodies?

A

Indirect Immunofluorescence - positive for some type of ANA is positive in virtually every patient with SLE

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28
Q

What is the most commonly-use method to detect antibodies?

A

Indirect Immunofluorescence

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29
Q

What protozoan is us commonly used for test substrate in immunofluorescence testing? Why?

A

Crithidia luciliae

kinetoplast stains positive in patients with antibodies to dsDNA - ideal for testing due to its high concentration of dsDNA and lack of other human antigens

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30
Q

Are tests for ANA sensitive or specific for SLE?

A

sensitive - positive in almost all

but not specific because they are also positive in patients with other autoimmune conditions

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31
Q

SLE is what type of hypersensitivity reaction? Leading to what type of injury?

A

Type III

tissue injury is caused mainly by deposition of immune complexes & binding of antibodies

acute necrotizing vasculitis & fibroid deposits

32
Q

What is the pathogenesis involved with peripheral blood cytopenias in patients with SLE?

A

autoantibodies specific for erythrocytes, leukocytes, & platelets - will opsonize these cells & promote their phagocytosis and lysis

33
Q

What is the difference between LE bodies & LE cells?

A
  • LE bodies
    • nuclei damaged cells react with ANAs - lose chromatin pattern & become homogeneous
    • in vivo phenomenon
  • LE cells
    • any phagocytic leukocyte that has engulfed & denatured nucleus of an injured cell
    • ONLY seen in vitro when blood is agitated - Outdated
34
Q

What is antiphospholipid syndrome?

This syndrome is associated with what risks?

A

autoantibodies (lupus anticoagulants) are directed against phospholipid-associated proteins

may raise the risk of venous & arterial thrombosis

35
Q

What substances are commonly found in the blood vessels in patients with SLE?

A

immunoglobulin, dsDNA, C3

perivascular lymphoid infiltrate

36
Q

The provided image is a characteristic feature of what autoimmune condition?

A

SLE

fibrous thickening and luminal narrowing

37
Q

Where are SLE immune deposits found within the kidney?

A

mesangium

basement membrane

38
Q

What kidney problems are seen in patients with SLE & how does this present clinically?

A

cellular infiltration, microvascular thrombosis, vascular wall deposition

hematuria, proteinuria, hypertension, renal insufficiency

** don’t have to differentiate the types via images

39
Q

SLE can cause what effect on the cardiovascular system?

A
  • pericarditis (common)
  • myocarditis (less common)
  • valvular involvement
  • Coronary artery disease due to atherosclerosis
40
Q

SLE can cause what effects on the spleen?

A

moderate splenomegaly

follicular hyperplasia

41
Q

What are the most common causes of death in patients with SLE?

A

renal failure & incurrent infection

42
Q

What the provided immunofluorescence image is characteristic of what condition?

A

Skin manifestation of SLE

Ig & complement along DE junction

43
Q

What type of skin manifestations are seen in SLE?

A
  • liquefactive degeneration of the basal layer
  • Dermis
    • edema
    • perivascular mononuclear infiltrates
    • vasculitis and fibrinoid necrosis of the vessels
44
Q

What condition is shown in the provided image? It is most common on what areas of the body?

A

Discoid Lupus Erythematosus

face & scalp

plaques with varying degrees of edema, erythema, scaliness, follicular plugging & sin atrophy - surrounded by erythematous, elevated border

rare to have systemic symptoms

45
Q

What is a laboratory test that can help differentiate SLE from chronic discoid lupus erythematosus?

A

antibodies to dsDNA are rarely present in discoid lupus

46
Q

What pathology is is shown int he provided image?

A

Discoid Lupus Erythematosus

Hyperkeratosis, flattened epidermis, perivascular and periadnexal chronic inflammation

47
Q

What are the key difference in antinuclear antibodies seen in SLE compared to drug-induced lupus?

A

antibodies for dsDNA are rare in drug-induced

high frequency of antibodies for histones

48
Q

Do people with drug-induced lupus have the same systemic symptoms as people with SLE?

A

most do not, but some do

renal & CNS involvement is rare

49
Q

What genetic factors are associated with Rheumatoid arthritis?

A

HLA-DR4,

HLA-DR1,

HLA-DR10,

HLA-DR14

50
Q

What joints are most commonly affected by rheumatoid arthritis?

A

metacarpaophalangeal & proximal interphalangeal joints of hands & feet

elbows

knees

ankles

spine (uncommon)

51
Q

What percent of patients with RA are positive for RF?

A

80% (humoral immunity)

high titers are associated with more severe disease

52
Q

What infections agents are possibly associated with rheumatoid arthritis?

A

EBV, parvovirus B19

53
Q

How does RA affect joints?

A

non-suppurative proliferative synovitis & may progress to destruction of articular cartilage

54
Q

What clinical symptoms are commonly seen in RA?

A

joint pain, swelling, morning stiffness & decreased grip strength

joint deformities

anemia, fatigue, rheumatoid nodules, pleural or pericardial inflammation/effusion, neuropathy, splenomegaly, Sjogren syndrome, vasculitis, inflammation of slcera

55
Q

The provided histological slides are indicative of what condition?

A

Rheumatoid Arthritis

  • papillary synovial hyperplasia w/ dense inflammatory infiltrate of plasma cells & lymphocytes, and lymphoid nodules
    • later a pannus of synovium, inflammatory cells & fibroblasts encroach on the hyaline cartilage leading to destruction
  • neutrophils accumulate in synovial surface & in fluid
  • osteoclasts are activated, with resulting bone erosion, osteoporosis, and subchrondral cysts
56
Q

In addition to RF, what other antibodies are commonly seen in RA?

A
  • Antibody to citrullinated peptide (ACPA)
  • anti-CCP antibody (>90% specificity)
    • can also be seen in other acute inflammatory states
57
Q

What is the postulated mechanism by which cellular immunity contributes to RA?

A

CD4 initiate autoimmune response

they produce cytokines (ie TNF) that stimulate other inflammatory cells to cause tissue injury

58
Q

What are rheumatoid nodules & in what types of patients do they most commonly occur?

A

firm, non-tender nodules arising in subcutaneous tissue in areas subject to recurrent pressure & viscera

occur almost exclusively in seropositive (RA, ACPA) patients

59
Q

What autoimmune disorder is characterized by dry eyes & dry mouth?

A

Sjogren Syndrome

60
Q

What immunofluorescence pattern is seen with Sjogren?

A

speckled

61
Q

90% of patients with Sjogren have antibodies agains what serologic markers of the disease?

A

two ribonucleoprotein antigens

SS-A (Ro)

SS-B (La)

62
Q

What is the clinical manifestation of Sjogren Syndrome?

A
  • older woman
  • keratoconjunctivitis sicca
    • blurred vision, burning & itching
  • xerostomia
  • parotid gland enlargement
  • nosebleeds
  • bronchitis
63
Q

Patients with Sjogren’s have a 40-fold increase of developing what pathology?

A

lymphoma (usually MALT)

64
Q

What is the clinical picture of a patient with Systemic Sclerosis?

A
  • Woman 50-60
  • cutaneous fibrosis
  • dysphagia
  • Raynaud
  • RHF
  • GI issues
  • renal failure
65
Q

The etiology of systemic sclerosis is due to what 3 processes?

A

autoimmunity, vascular damage, fibrosis

66
Q

What are the two different types of Systemic Sclerosis?

A

Diffuse scleroderma

Limited scleroderma

67
Q

What are the characteristic symptoms associated with diffuse scleroderma?

A
  • skin (shiny) involvement at onset (expand proximally)
  • rapid progression to chronic inflammation (small vessels & fibrosis)
  • early visceral involvement
  • Raynaud (may appear years before)
68
Q

What are the characteristic symptoms associated with limited scleroderma?

A
  • skin is only on fingers, forearms, & face
  • late visceral involvement
  • CREST Syndrome
    • C:alcinosis
    • R:aynaud phenomenon
    • E:sophageal dysmotility
    • S:leroderma
    • T:elangiectasia
  • Centromeric patterin on immunofluorescence
69
Q

What immunofluorescence pattern is seen with Limited Scleroderma?

A

centromeric

70
Q

What ANAs are commonly seen in Diffuse Systemic sclerosis? Limited Lystemic Sclerosis?

A
  • Diffuse: DNA topoisomerase I
  • Limited: Anti-centromere antibody
71
Q

What pathology is shown in the provided image?

A

you see infiltration between the glands & within the glands themselves

72
Q

The skin manifestation shown in the provided image is characteristic of what disease?

A

Systemic Sclerosis

notice that it is tight & shiny

73
Q

What can be the result of extensive subcutaneous fibrosis?

A

claw-like flexion deformity

74
Q

The provided image shows what pathology?

A

Systemic Sclerosis

  • extensive deposition of dense collagen in the dermis with virtual absence of hair follicle & focal inflammation
  • increasing dermal fibrosis
75
Q

Mixed connective tissue disease is characterized by what high antibody titers?

A

U1 ribonucleoprotein

76
Q

What is the clinical manifestation of mixed connective tissue disease?

A

mixture of those other autoimmune diseases

(SLE, polymyositis, RA, Systemic Sclerosis)

77
Q

What are the possible complications associated with mixed connective tissue disease?

A

pulmonary hypertension,

interstitial lung disease,

progressive renal disease

S3B5 Pathology (13 decks)

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