Inflammation (High Yield)

Question 1

What is the sign of nephritic renal damage in Lupus?

Answer 1

Diffuse proliferative glomerulonephritis

Question 2

What is the sign of nephrotic renal damage in Lupus?

Answer 2

Membranous glomerulonephritis

Question 3

What disease can result from an adenosine deaminase deficiency?

Answer 3

Severe Combined Immunodeficiency (SCID)

Question 4

What causes X-linked agammaglobulinemia?

Answer 4

Mutated Bruton tyrosine kinase

Question 5

What conditions/diseases might arise secondary to Common Variable Immunodeficiency (CVID)?

Answer 5

Autoimmune disease, lymphoma

Question 6

Cause and symptoms of hyper-IgM syndrome

Answer 6

Mutated CD40 causes deficiency of cytokines necessary for Ig class switching (low IgA, IgG and IgE), resulting in recurrent pyogenic infections at mucosal sites

Question 7

C5-C9 deficiencies may predispose an individual to what condition(s)?

Answer 7

Recurrent Neissaria infections

Question 8

What disease results from a C1 inhibitor deficiency? What are the symptoms?

Answer 8

Hereditary angioedema; skin edema, especially periorbital and at mucosal surfaces

Question 9

Describe the manifestation of neonatal lupus. What is the clinical significance?

Answer 9

If anti-SSA and anti-SSB are present in a Lupus patient, they can cross the placental membrane and cause neonatal lupus, which may result in a congenital heart block; Important to screen pregnant women who have lupus for anti-SSA and anti-SSB

Question 10

Clinical indications of mixed connective tissue disease

Answer 10

ANA with serum antibodies against U1 ribonucleoprotein (RNP)

Question 11

Under what conditions will impermanent tissues (e.g. labile or stable) undergo repair instead of regeneration?

Answer 11

Loss of regenerative stem cells

Question 12

TGF-a function

Answer 12

Epithelial and fibroblast growth factor

Question 13

TGF-b function

Answer 13

Fibroblast growth factor, inhibits inflammation

Question 14

PDGF function

Answer 14

Endothelium, smooth muscle, fibroblast growth factor

Question 15

FGF (fibroblast growth factor) function

Answer 15

Angiogenesis; skeletal development

Question 16

VEGF function

Answer 16

Angiogenesis

Question 17

Where do keloids commonly occur? What causes them?

Answer 17

Earlobe (most common), face and upper extremities; Excess production of scar tissue (containing type III collagen) relative to wound size

Question 18

What is CD-14, where is it found and what does it do?

Answer 18

Toll-like receptor; on macrophages; recognizes LPS on gram-negative bacteria

Question 19

Effect of TLR activation

Answer 19

Upregulation of NF-kB, which triggers the acute inflammatory response, causing multiple immune mediators to be released

Question 20

Describe the delayed response of Mast cells

Answer 20

Production of arachidonic acid metabolites, particularly leukotrienes

Question 21

Where in the body do all steps (margination, adhesion, transmigration, etc.) of the acute inflammatory response take place?

Answer 21

Postcapillary venules

Question 22

What immune system cell can prolong acute inflammation, and how?

Answer 22

Macrophages; secretion of IL-8

Question 23

Cause and symptoms of Chediak-Higashi syndrome

Answer 23

Autosomal recessive mutation in LYST gene causes microtubule defect, resulting in poor phagolysosome formation (trafficking defect); increased risk of pyogenic infections, neutropenia, giant granules in leukocytes, defective primary hemostasis, albinism, peripheral neuropathy

Question 24

Cause and symptoms of Leukocyte Adhesion Deficiency

Answer 24

Type 1: autosomal recessive defect in CD18 (beta-2 chain) subunit of interns (Mac-1 and LFA-1); type 2: absence of Sialyl Lewis X, ligand for E- and P- selectins, d/t a defect in fucosyl transferase; delayed separation of umbilical cord, neutrophilia, recurrent bacterial infection w/o pus formation

Question 25

What molecule activates both coagulation and fibrinolytic pathways

Answer 25

Hageman factor (factor XII)

Question 26

What is the defining characteristic of a granuloma?

Answer 26

Presence of epithelioid histiocytes (macrophages with abundant pink cytoplasm)

Question 27

Describe the steps present in granuloma formation

Answer 27

Macrophages present antigens to CD4+ helper T cells via MHCII, and secrete IL-12 to convert the T cell into the TH1 subtype, which in turn secretes IFN-gamma, converting the macrophage into an epithelioid histiocyte

Question 28

Describe mechanisms by which CD8+ cytotoxic T cells kill other cells

Answer 28

Secrete granzyme and performs which induce apoptosis, also FasL (Fas ligand), which binds to Fas on the target cell membrane to induce apoptosis