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MSS 2 > Pathology of Autoimmune Disease > Flashcards

Pathology of Autoimmune Disease Flashcards

1
Q

What do autoimmune disorders tend to be, once induce?

A
  • progressive
  • sometimes marked by relapses and remissions
  • damage becomes inexorable (relentless) due to amplification mechanisms and epitope spreading.
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2
Q

What determines the pathological features of autoimmune diseases?

A
  • the nature of the underlying immune response.
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3
Q

*** With what are Th1 cells associated?

A
  • IFN-y and macrophage-rich inflammation.
  • production of opsonizing antibodies (make cells yummier for phagocytosis) that activate complement and bind via high affinity receptors for Fc region of IgG, to MACROPHAGES and neutrophils.
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4
Q

With what are Th17 cells associated?

A
  • production of IL-17 and inflammation that is rich in NEUTROPHILS
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5
Q

Do different autoimmune disorders show substantial clinical, pathological , and serological overlap?

A
  • YES

* this makes diagnosis DIFFICULT.

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6
Q

What type of hypersensitivies are most autoimmune diseases?

A
  • type II, III, IV, or a mix

* rarely type I

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7
Q

What type of hypersensitivity is SLE?

A
  • depends on the organs involved:
  • kidney= type III
  • leukopenia, thrombocytopenias= type II
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8
Q

What type of hypersensitivity is myasthenia gravis?

A
  • type II hypersensitivity (autoantibody against acetylcholine receptor; ANTAGONIST)
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9
Q

What type of hypersensitivity is graves disease?

A
  • type II (autoantibody against TSH receptor; AGONIST)
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10
Q

What type of hypersensitivity is Goodpastures syndrome?

A
  • type II (autoantibody against glomerular basement membrane type IV collagen; linear immunofluorescence).
  • contrast this to SLE, which is lumpy bumpy deposition.
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11
Q

What type of hypersensitivity is autoimmune hemolytic anemia?

A
  • type II (opsonization and phagocytosis of erythrocytes due to erythrocyte membrane proteins (Rh blood group antigens, I antigen).
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12
Q

What type of hypersensitivity is autoimmune (idiopathic) thrombocytopenic purpura?

A
  • type II (opsonization and phagocytosis of platelets).
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13
Q

What are the mechanisms of damage in type II hypersensitivity?

A
  • opsonization and phagocytosis
  • complement and Fc receptor-mediated inflammation
  • antibody-mediated cellular dysfunction
  • remember antigen is on cell surface.
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14
Q

What diseases are mediated by immune complexes?

A
  • systemic diseases= SLE

- polyarteritis nodosa (disease of small and medium sized arteries)

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15
Q

Do men or women tend to get lupus (SLE) more?

A
  • pre-menopausal WOMEN (9:1 ratio)
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16
Q

To what infection is polyarteritis nodosa linked?

A
  • hepatitis B
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17
Q

What is a type III hypersensitivity?

A
  • circulating antigen-antibody immune complexes (soluble) that deposit in various locations (especially vessel walls).
  • these antigen-antibody complexes then fix complement and bring in neutrophils via Fc receptor
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18
Q

What is the number 1 cause of death in lupus (SLE)?

A
  • heart disease
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19
Q

What autoimmune diseases are organ specific and mediated by T cells?

A
  • T1DM

- MS (myelin basic protein is targeted)

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20
Q

What autoimmune diseases are systemic and mediated by T cells?

A
  • systemic sclerosis
  • sjogren’s syndrome (salivary glands)
  • rheumatoid arthritis
21
Q

What autoimmune diseases may be caused by microbial antigens?

A
  • inflammatory bowel disorders (Crohn’s or UC)

- inflammatory myopathies

22
Q

What is the PROTOTYPE multi-system disease of autoimmune origin?

A
  • SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)= characterized by a bewildering array of autoantibodies (particularly ANTI-NUCLEAR ANTIBODIES; ANA).
23
Q

What are the salient features of SLE?

A
  • acute or insidious onset
  • chronic, often relapsing and remitting
  • often febrile
  • damage to SKIN, JOINTS, SEROSAL SURFACES, and KIDNEYYYYY.
  • weight loss, fatigue, headaches, myalgias, raynaud’s, neuropathy, and ocular problems are also common.
  • usually WOMAN of CHILDBEARING AGE
24
Q

Is the joint damage seen in lupus different from that seen in RA?

A
  • YES
25
Q

Why do women with lupus tend to be more susceptible to infections, when they have an overactive immune system?

A
  • bc some of the autoantibodies being generated are against leukocytes and cells of the immune system and complement is being used as fast as it’s made.
26
Q

What is the etiology of SLE?

A
  • unknown (probably a fundamental defect in self-tolerance (B and T cells).
  • antibodies against an array of nuclear and cytoplasmic cellular components (not organ specific, however another group is specific for surface antigens of blood cells; immunologic cells).
27
Q

** What are the 11 criteria for SLE?

A
  1. MALAR RASH (BUTTERFLY RASH)= fixed erythema, flat or raised over the malar eminences (spares the nasolabial fold).
  2. DISCOID RASH= erythematous raised patch with adherent keratotic scaling and follicular plugging.
  3. PHOTOSENSITIVITY
  4. ORAL ULCERS (painless)
  5. ARTHRITIS (non-erosive involving 2 or more peripheral joints)
  6. SEROSITIS= pleuritis or pericarditis.
  7. RENAL disorder= persistent proteinuria and casts in urine.
  8. NEUROLOGIC disorder= seizures or psychosis.
  9. IMMUNOLOGIC= hemolytic anemia, leukopenia, lymphopenia, or thrombocytopenia.
  10. IMMUNOLOGIC disorder= anti-dsDNA, anti-SM, anti-phospholipid.
  11. ANA
    * must fulfill 4 of 11 criteria.
28
Q

What are the ANA patterns of staining?

A
  • HOMOGENOUS= SLE
  • DIFFUSE= drug reaction, SLE, or systemic sclerosis
  • SPECKLED= MCTD, SLE, Sjogrens, or dermatomyositis.
  • NUCLEOLAR= systemic sclerosis
  • PERIPHERAL= SLE
  • CENTROMERE= systemic sclerosis
29
Q

What antibodies will most pts with lupus have?

A
  • antibodies to DNA, HISTONES, NON-HISTONE PROTEINS bound to RNA, and NUCLEOLAR antigens
30
Q

Is ANA sensitive?

A
  • YES but NOT specific.
31
Q

Since ANA is not specific for lupus, in what other autoimmune diseases is it found?

A
  • drug-induced lupus
  • systemic sclerosis
  • limited scleroderma (CREST)
  • Sjogren’s syndrome
  • inflammatory myopathies
32
Q

*** What 2 antibodies are virtually diagnostic in SLE?

A
  • antibodies to dsDNA
  • antibodies to SMITH antigen
  • note not all SLE pts have these, but if they do, then we can definitely say they have SLE.
33
Q

What antibody will you see most often in systemic sclerosis?

A
  • antibody against DNA topoisomerase I
34
Q

What antibody will you see most often in limited scleroderma (CREST)?

A
  • antibody against centromeres
35
Q

What antibody will you see most often in Sjogren’s syndrome?

A
  • RNP
36
Q

What other antibodies are seen in SLE?

A

antibodies against:

  • RBCs (accounts for the anemia).
  • platelets
  • lymphocytes
  • phospholipids
37
Q

What antibody binds to cardiolipin and what test will show up as a false positive?

A
  • phospholipid
  • syphilis will be false positive
  • intereferes with clotting (referred to as lupus anti-coagulant).
38
Q

Why do pts often become hypercoagulable if antiphospholipid antibody acts as an anticoagulant?

A
  • if hemorrhage occurs, there is always a possibility of venous and arterial thromboses
39
Q

What is secondary anti-phospholipid syndrome?

A
  • focal cerebral or ocular ischemia, recurrent spontaneous miscarriages, and thromboses.
  • seen in 28 y/o woman with a positive ANA and history of miscarriages.
40
Q

Why would a genetic complement deficiency predispose one to lupus?

A
  • bc the immune complexes can not clear.
41
Q

What medications can cause “drug-induced lupus”?

A
  • hydralazine
  • procainamide
  • D-penicillamine
42
Q

Why does UV light exacerbate SLE?

A
  • cells undergo apoptosis, leading to epitope spreading and enhanced inflammation.
43
Q

Do pts with lupus have polyclonal B ell activation?

A

YES

*also T cell defects, but regardless of the defects, autoantibodies are the major mediators of damage.

44
Q

What type of hypersensitivities are occurring in SLE?

A
  • autoantibodies to RBC, WBC, platelets= Type II

- visceral organ damage= type III

45
Q

*** What are the pathologic manifestations of SLE?

A
  • vascular degeneration of the basal layer of the epidermis.
  • dermal edema
  • perivascular inflammation
  • vasculitis with prominent fibrinoid necrosis.
  • non-erosive synovitis (remember in RA it is erosive).
  • PERICARDIAL INVOLVEMENT in up to 50% of pts.
  • LIBMAN-SACKS ENDOCARDITIS
  • mitral and aortic valve abnormalities.
  • coronary ATHEROSCLEROSIS.
  • RENAL DISEASE.
46
Q

Where will you deposition of immunoglobulin in the skin?

A
  • dermal-epidermal junction.
47
Q

** What is the major mechanism of renal disease in SLE?

A
  • GRANULAR immune complex deposition
48
Q

How many classes of mesangial lupus glomerulonephritis are there?

A

6:

  • I= minimal nephritis
  • II= mesangial proliferation (subENDOthelial immune complex deposition).
  • III= focal proliferative glomerulonephritis (segmental; only part of the glomerulus or global; affecting the entire glomerulus)
  • IV= diffuse proliferative glomerulonephritis (most severe; subENDOthelial).
  • V= membranous glomerulonephritis (subEPIithelial immune complexes).
  • VI= advanced sclerosing lupus nephritis
49
Q

What is the major killer of SLE?

A
  • cardiovascular or cerebrovascular disease

MSS 2 (15 decks)

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