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KN-Pathology Block 2 > Immunopathology - Nichols > Flashcards

Immunopathology - Nichols Flashcards

1
Q

Positive test for anti-cyclic citrullinated peptide (anti-CCP) antibody common with what disease?

A

Rhuematoid arthritis

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2
Q

Positive test for anti-centromere antibody common with what disease?

A

CREST syndrome (limited systemic sclerosis)

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3
Q

Positive test for anti-Jo-1 (an anti-synthetase) antibody common with what disease?

A

polymyositis/dermatomyositis

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4
Q

Positive test for anti-Scl70 (anti-DNA topoisomerase) antibody common with what disease?

A

systemic sclerosis (SS), diffuse

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5
Q

Positive test for anti-RNA polymerase (anti-U3 RNP) antibody common with what disease?

A

systemic sclerois (SS)

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6
Q

Positive test for anti-U1 RNP (anti-RNP) antibody common with what disease?

A

mixed connective tissue disease

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7
Q

Positive test for anti-Smith (anti-Sm) antibody common with what disease?

A

Lupus

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8
Q

Positive test for anti-double-stranded DNA (anti-dsDNA) antibody common with what disease?

A

Lupus

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9
Q

Positive test for anti-nuclear (ANA) antibody common with what disease?

A

Lupus and many other rheumatic diseases

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10
Q

Positive test for anti-SSA (anti-Ro) antibody common with what disease?

A

Sjogren syndrome, neonatal lupus, subcutaneous lupus

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11
Q

Positive test for anti-SSB (anti-La) antibody common with what disease?

A

Sjogren syndrome, neonatal lupus, subcutaneous lupus

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12
Q

Positive test for anti-myeloperoxidase (perinuclear anti-neutrophil cytoplasmic, P-ANCA) antibody common with what disease?

A

Microscopic polyangitis, eosinophilic granulomatosis with polyangitis (Churg Strauss)

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13
Q

Positive test for anti-proteinase-3 (diffuse cytoplasmic anti-neutrophil, C-ANCA) antibody common with what disease?

A

granulomatosis with polyangitis (Wegener’s)

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14
Q

If a person has a positive test for the presence of any of these rheumatological antibodies does it mean they have a disease?

A

NO. Cannot diagnose based on serology alone.

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15
Q

What is cyclosporine?

A

a calcineurin inhibitor given to transplant patients.

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16
Q

What is a calcineurin inhibitor?

A

bind to intracytoplasmic receptor proteins called immunophilins to form a complex which then binds to and inhibits a phosphatase called calcineurin

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17
Q

What is calcineurin?

A

a phosphatase that normally dephosphorylates intracytoplasmic nuclear regulatory proteins in lymphocytes and promotes T lymphocyte activation an secretion of TNF, IFN-gamma, IL-2 and IL-4.

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18
Q

What is Mycophenolate mofetil?

A

a reversible inhibitor of inosine monophosphate dehydrogenase in purine biosynthesis (specifically guanine synthesis) to inhibit DNA replication and inhibit lymphocyte proliferation.

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19
Q

What is azathioprine?

A

purine analog that prevents DNA replication

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20
Q

What is hydroxychloroquine?

A

anti-malarial antibiotic that is used to treat systemic lupus erythematosus and rheumatoid arthritis because of its anti-inflammatory activity and low toxicity

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21
Q

What do infliximab, etanercept, and adalimumab do?

A

block TNF-alpha

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22
Q

What is belimumab do?

A

is an antibody to B-cell activating factor

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23
Q

What is Rituximab?

A

an antibody to CD20 on B lymphoctyes

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24
Q

Which of these is true?
ANA is present in 100% of patients with systemic lupus erythematosus, but also present in some healthy people.
OR
ANA is present in some cases of systemic lupus erythematosus, but very rarely in healthy people.

A

ANA is always present in diseased patients, but cannot use this as a diagnosis by itself because up to 15% of healthy people also have them.

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25
Q

What is PTLD?

A

post-transplant lymphoproliferative disorder. a spectrum ranging from a proliferatoin of lymphocytes that halts with decreasing immunosuppressive therapy to a full blown malignant lymphoma that can be resistant to chemotherapy. Usually infected with EBV.

26
Q

What is tacrolimus?

A

(FK506) given to transplant patients to prevent transplant rejection. Calcineurin inhibitor. Binds to intracytoplasmic receptors called immunophilins, which in turn bind to and inhibit calcineurin, a phosphatase.

27
Q

What is alumtuzumab?

A

antibody to CD52, which is highly expressed in both normal and malignant B and T cells. CD52 is also lowly expressed on monos, macros and eosinos. Alemtuzumab is an especially powerful immunosupressant that can cause permanent AIDS-like effects with a single dose

28
Q

What is systemic lupus erythematosus?

A

multisystem autoimmune disease that is more common in women and africans/asians. It is a failure of self-tolerance of antinuclear antibodies. Will cause Libman-Sacks (vegetations on heart valves), and splenomegaly, peritonitis and synovitis. Fibrinoid depoisits and nephritis visible microscopically as well as IgG deposits. Symptoms of joint poain, fever, fatigue and weight loss. Skin rash in butterfly patter (malar), edema, hematuria, psychiatric disorders and alopecia.
Diagnosed with ANA, anti double stranded DNA or anti-Sm abs.
Treat with corticosteroids and immunosuppression.

29
Q

What is sjogren syndrome?

A

T cell-mediated autoimmune attack of lacrimal and salivary glands. Intense infiltration of CD4 T cells to these glands and eventual destruction. Diagnosed by Anti-SSA or -SSB antibodies (SS=sjogren syndrome)
Treat with hydroxychloroquine and rituximag.
Often leads to lymphoma.

30
Q

What is systemic sclerosis?

A

chronic accumulation of fibrous tissue in skin and organs. Often involves CREST syndrome. More common in females and 50-60 yr olds. Abnormal immune response and vascular damage cause increased growth factors. CD4 T cellls respond to antigens and stimulate fibroblasts to produce collagen. HLA-2 and fibrillin1 genes implicated. GI tract and esophagus also have fibrous replacement of muscular walls. Causes Raynauds and peripheral numbness. Eearliest signs are edema of hands and feet. Diagnose with Anti-Scl (diffuse sclerosis) or anticentromere Ab (limited sclerosis).
No good treatment. Exercise, splint, avoid cold.

31
Q

What type of transplant rejection occurs within minutes and what is it mediated by?

A

hyperacute rejection. mediated by pre-formed Abs.

32
Q

What is CD4 immunostaining in a linear pattern along peri-tubular capillaries indicative of after a kidney transplant?

A

transplant rejection, when this information is combined with histological evidence of rejection.

33
Q

What is of special concern when using calcineurin inhibitors to treat rejection of kidney transplants?

A

calcinuerin inhibitors are nephrotoxins that can damage the transplant by itself.

34
Q

What is What is the most common type of cell that leaves a transplant and sets up residence elsewhere in the host body?

A

lymphocytes. They can cause GVHD

35
Q

What is XLA of Bruton?

A

congenital immunodeficiency with failure of B cells to produce Abs.

36
Q

What wlll IgA deficiency cause?

A

suceptibility to anaphylaxis with blood transfusion.

37
Q

What is Wiskott-Aldrich syndrome?

A

congenital X-linked disorder with immunodeficiency, eczema and thrombocytopenia. Typically low IgM levels and high IgE and A.

38
Q

What are the four categories of systemic amyloidosis?

A
  1. primary
  2. secondary
  3. induced
  4. hereditary
39
Q

Describe the pathogenesis of amyloidosis?

A

disease of abnormal folding of proteins into Beta sheets. Often involves light chains (bence-Jones proteins).

40
Q

What is primary amyloidosis?

A

most common form. Involves heart, gut, nerves, skin and tongue. eposition of AL (light chains - Bence-jones). 10% of pateints have myeloma.

41
Q

What is secondary amyloidosis?

A

kidneys, liver, spleen, lymph nodes, adrenals and thyroid. Most commonly associated with rheumatoid arthritis, chronic injection drug abuse, renal carcinoma and Hodgkin’s. Deposition of AA (amyloid A), which complexes with HDL.

42
Q

What is induced amyloidosis?

A

involves nerves, joints, bone, gut and tongue. deposition of AB2M (beta-2 microglobulin light chain of HLA1).

43
Q

What is hereditary amyloidosis?

A

Examples: Mediterranean fever - AA deposition in liver, spleen kidneys and adrenals.
Amylooid poly-neuropathy - peripheral and autonomic neurophatyy from deposition of ATTR.

44
Q

What is localized (senile) cardiac amyloidosis?

A

deposition of ATTR in ventricles or AANF deposition in atria. AB deposits in brain. In thyroid calcitonin is deposited. In pancreas amyloids are deposited in islets of langerhans. Cutaneous deposits of AL and AA.
Treat with transplantation of affected organs. Poor prognosis.

45
Q

What can Congo Red stian diagnose?

A

amyloidosis.

46
Q

What is systemic sclerosis?

A

chronic accumulation of fibrous tissue in skin and organs. Often involves CREST syndrome. More common in females and 50-60 yr olds. Abnormal immune response and vascular damage cause increased growth factors. CD4 T cellls respond to antigens and stimulate fibroblasts to produce collagen. HLA-2 and fibrillin1 genes implicated. GI tract and esophagus also have fibrous replacement of muscular walls. Causes Raynauds and peripheral numbness. Eearliest signs are edema of hands and feet. Diagnose with Anti-Scl (diffuse sclerosis) or anticentromere Ab (limited sclerosis).
No good treatment. Exercise, splint, avoid cold.

47
Q

What type of transplant rejection occurs within minutes and what is it mediated by?

A

hyperacute rejection. mediated by pre-formed Abs.

48
Q

What is CD4 immunostaining in a linear pattern along peri-tubular capillaries indicative of after a kidney transplant?

A

transplant rejection, when this information is combined with histological evidence of rejection.

49
Q

What is of special concern when using calcineurin inhibitors to treat rejection of kidney transplants?

A

calcinuerin inhibitors are nephrotoxins that can damage the transplant by itself.

50
Q

What is What is the most common type of cell that leaves a transplant and sets up residence elsewhere in the host body?

A

lymphocytes. They can cause GVHD

51
Q

What is XLA of Bruton?

A

congenital immunodeficiency with failure of B cells to produce Abs.

52
Q

What wlll IgA deficiency cause?

A

suceptibility to anaphylaxis with blood transfusion.

53
Q

What is Wiskott-Aldrich syndrome?

A

congenital X-linked disorder with immunodeficiency, eczema and thrombocytopenia. Typically low IgM levels and high IgE and A.

54
Q

What are the four categories of systemic amyloidosis?

A
  1. primary
  2. secondary
  3. induced
  4. hereditary
55
Q

Describe the pathogenesis of amyloidosis?

A

disease of abnormal folding of proteins into Beta sheets. Often involves light chains (bence-Jones proteins).

56
Q

What is primary amyloidosis?

A

most common form. Involves heart, gut, nerves, skin and tongue. eposition of AL (light chains - Bence-jones). 10% of pateints have myeloma.

57
Q

What is secondary amyloidosis?

A

kidneys, liver, spleen, lymph nodes, adrenals and thyroid. Most commonly associated with rheumatoid arthritis, chronic injection drug abuse, renal carcinoma and Hodgkin’s. Deposition of AA (amyloid A), which complexes with HDL.

58
Q

What is induced amyloidosis?

A

involves nerves, joints, bone, gut and tongue. deposition of AB2M (beta-2 microglobulin light chain of HLA1).

59
Q

What is hereditary amyloidosis?

A

Examples: Mediterranean fever - AA deposition in liver, spleen kidneys and adrenals.
Amylooid poly-neuropathy - peripheral and autonomic neurophatyy from deposition of ATTR.

60
Q

What is localized (senile) cardiac amyloidosis?

A

deposition of ATTR in ventricles or AANF deposition in atria. AB deposits in brain. In thyroid calcitonin is deposited. In pancreas amyloids are deposited in islets of langerhans. Cutaneous deposits of AL and AA.
Treat with transplantation of affected organs. Poor prognosis.

61
Q

What can Congo Red stian diagnose?

A

amyloidosis.

KN-Pathology Block 2 (10 decks)

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