alloimmunity/autoimmunity pt 3

Question 1

amyloidosis characteristics

Answer 1

condition classified by abnormal protein deposits (fibrils)

may be systemic, localized, or hereditary

RF: older adults, M>F

Question 2

amyloidosis symptomatology

Answer 2

fatigue
unintentional weight loss

nephrotic syndrome
restrictive cardiomyopathy
peripheral neuropathy
hepato/splenomegaly
macroglossia , shoulder pad sign
skin involvement - purpura, easy bruising, cutaneous nodules
bleeding

Question 3

circumstances to suspect amyloidosis

Answer 3

• unexplained non-diabetic proteinuria
• restrictive cardiomyopathy/unexplained CHF
• increased BNP w/o heart disease
• unexplained edema, hepatosplenomegaly, or carpal tunnel syndrome
• unexplained facial/neck purpura
• macroglossia
• suspicion in pt w multiple myeloma, Waldenstrom macroglobulinemia or lymphoma, fam hx of amyloidosis

Question 4

amyoloidosis diagnostic criteria

Answer 4

• presence of amyloid-related systemic syndrome
• presence of amyloid staining by Congo red in fat aspirate, bone marrow, or organ biopsy
• evidence that amyloid is light-chain related
• evidence of a monoclonal plasma cell proliferative disorder

Question 5

amyloidosis treatment

Answer 5

chemo (bortezomib-based regimen)

or chemo & SCT

consider specific symptom guidelines (HF)

Question 6

scleroderma characteristics

Answer 6

present of thickened, hardened skin due to an excess of collagen fibers

hallmark feature of systemic sclerosis

systemic sclerosis - fibrosis of skin and internal organs w vascular dysfunction

autoimmune

RF: 30-50, F>M

Question 7

CREST syndrome

Answer 7

associated w limited cutaneous systemic sclerosis

calcinosis cutis
Raynaud phenomenon
esophageal dismotility
sclerodactyly (hardening of the skin)
telangiectasia

Question 8

scleroderma symptomatology

Answer 8

skin thickening

CREST syndrome

puffy hands/fingers

risk for PH

Question 9

diagnosis of systemic sclerosis (limited or diffuse)

Answer 9

skin thickening of the fingers of both hands extending proximal to the MCP joints

Question 10

scleroderma treatment

Answer 10

• focus on organ systems involved
• skin involvement - immunosuppression, consider IVIG or rituximab
• kidney involvement - ACE
• cardiac involvement - see HF guidelines

Question 11

vasculitis characteristics

Answer 11

systemic rheumatic disorder characterized by the inflammation of blood vessels

Question 12

large vessel vasculitis examples

Answer 12

giant cell arteritis
Takayasu arteritis

Question 13

vasculitis treatment

Answer 13

glucocorticoids

rheum/nephrology consults

consider PLEX

immunosuppression

Question 14

precautions for long term high dose steroids

Answer 14

GI prophylaxis
prophylactic antibiotics/antivirals/PJP
ostoporosis prevention
DM prevention

Question 15

IgM function

Answer 15

• main antibody of primary responses
• best at fixing complement
• the monomer form of IgM serves as the B cell receptor

Question 16

IgG function

Answer 16

• main blood antibody of secondary responses
• neutralizes toxins
• opsonization

Question 17

IgA function

Answer 17

• secreted into mucus, tears, saliva, colostrum

Question 18

IgE function

Answer 18

• antibody of allergy and antiparasitic activity