immunopathology

Question 1

55. Hypersensitivity reactions can be easily remembered by the ABCD pneumonic, what do ABCD stand for?

Answer 1

Type I: Anaphylaxis–> IgE
Type II: Cytotoxic-mediated–>IgM, IgG
Type III: Immune-complex mediated (Abs, glomerulonephritis)
Type IV: Delayed hypersensitivity (T cells, celiacs)

Question 2

56. What are the features of SLE? What is needed for its diagnosis?

Answer 2

characterized by loss of self-tolerance & production of autoantibodies.
-Abs against nuclear DNA: Antinuclear antibody (ANA) (>95%) • Anti-dsDNA (40-60%)
-Type II & III hypersensitivity (skin III)
Hematologic: Hemolytic anemia, Thrombocytopenia, Neutropenia, Lymphopenia
-Arthritis
Malar ‘butterfly” rash, bullae formation

Question 3

57. Which hypersensitivity reactions are involved in the pathogenesis of SLE?

Answer 3

Type II & III hypersensitivity
Skin--> III
Kidney--> III
Heart-->Libman-Sacks endocarditis -->III
Serosal surfaces-->III
Neurological--> III
ALL 3

Question 4

58. What are ANA’s and why do they form?

Answer 4

Anti-nuclear antibodies

failure of self-tolerance

Question 5

59. Sjogren Syndrome causes what symptoms? Does it always occur as a single disease?

Answer 5

characterized by destruction of the lacrimal and salivary glands (cant make saliva or tears)
• Anti-ribonucleoprotein antibodies

Often associated with RA and other autoimmune dz, incr. chance of hodgkins lymphoma

Question 6

60. What is the pathogenesis of scleroderma, and which visceral organs can be involved?
    61.

Answer 6

characterized by fibroblast stimulation and deposition of collagen in the skin and internal organs.
Activation of fibroblasts by cytokines which leads to fibrosis:
• Interleukin 1 (IL-1), (PDGF), (FGF)
Anti-DNA topoisomerase I antibodies (Scl-70) (70%)
• Widespread skin involvement
• Early involvement of the visceral organs (Esophagus, GI Tract ,Pulmonary, Cardiac,Kidney – renal insufficiency

Question 7

What is CREST syndrome?

Answer 7

Localized Scleroderma 
Anti-centromere antibodies
• Skin involvement of the face and hands
• Late involvement of visceral organs
C-alcinosis R-aynauds E-sophageal dysfunction S-clerodactyly T-elengiactiasis

Question 8

62. How can Polymyositis be differentiated from fibromyalgia on physical exam?

Answer 8

POLYMIOSITIS does NOT HAVE SKIN involvement and is due to T cell-mediated damage. women who have muscle pain and atrophy. increased serum creatine kinase and sometimes positive ANA

DERMATOMYOSITIS Dermatomyositis HAS SKIN involvement and is due to anti-body-mediated damage

Question 9

63. What is Bruton’s agammaglobulinemia? What does the mutation result in ?

Answer 9

mutation of X-LINKED B-cell Bruton tyrosine kinase (btk)
male infants, recurrent infetictions

Inherited immunodeficiency characterized by a developmental failure to produce mature B cells
and plasma cells, resulting in agammaglobulinemia

Question 10

64. Is common variable immunodeficiency a single disease?

Answer 10

Group of disorders characterized by B-cell maturation defect & hypogammaglobulinemia

Onset in childhood, Recurrent bacterial infections, Giardia

• Complications:
• Increased autoimmune diseases
• Increased non-Hodgkin lymphoma and gastric cancer

Question 11

65. What fails to develop in DiGeorge Syndrome, and what does it result in?
66. Why do males usually get SCID? How does treatment differ now than from the past?
67. What are the common systemic diseases that cause a functional immunosuppression?
68. What 3 type of rejection occur in renal transplantation?
69. Which countries have the highest incidence of HIV?
70. Be able to describe the replication cycle of HIV
71. Why do 10% of hemophiliacs not progress to AIDS?
72. What are the phases of HIV infection that lead to AIDS?
73. What 2 neoplasms are most often seen in AIDS?
74. What is the diagnostic criteria of AIDS, and what lab tests are used to test for HIV?
75. What lab tests are used to monitor HIV infection?

Answer 11

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