ICM - Rheum 5

Question 1

this inflammatory myopathy is due to problems with the humoral immune system

Answer 1

dermatomyositis

Question 2

these inflammatory myopathies are due to cell-mediated response

Answer 2

polymyositis and inclusion-body

Question 3

rash seen over knuckles/elbows in dermatomyositis

Answer 3

Gottron’s papules

Question 4

this inflammatory myopathy is at increased risk for malignancies

Answer 4

dermatomyositis

Question 5

inflammatory infiltrate seen in dermatomyositis biopsy

Answer 5

perivascular and perimysial

Question 6

inflammatory infiltrate seen in polymyositis and inclusion-body myositis biopsy

Answer 6

endomysial

Question 7

treatment for inflammatory myopathies

Answer 7

corticosteroids, immunosuppresion (methotrexate, cyclophosphamide)

Question 8

muscle most affected in inflammatory myopathies (symmetrical proximal muscle weakness)

Answer 8

neck flexors, shoulder girdle, pelvic girdle

Question 9

enzymes that are elevated in inflammatory myopathies

Answer 9

CPK, LDH, aldolase, AST, ALT

Question 10

inflammation seen in inflammatory myopathies

Answer 10

lymphocytes

Question 11

pathology: perivascular and perifascicular lymphocytic infiltrate, immune complex deposition in vessels, perifascicular atrophy and fibrosis

Answer 11

dermatomyositis

Question 12

pathology: cellular infiltrate within fascicle, inflammatory cells invade individual muscle fibers

Answer 12

polymyositis

Question 13

pathology: perivascular exudates rare, characteristic intracellular lined vacuoles; intracytoplasmic or intranuclear tubular or filamentous inclusions

Answer 13

inclusion body myositis

Question 14

MCTD is overlap of these 3 conditions –> high ANA and anti-RNP

Answer 14

polymyositis, scleroderma, SLE

Question 15

myositis specific Ab –> association with interstitial lung disease, Raynaud’s, arthritis, mechanics hands

Answer 15

anti-histidyl tRNA synthetase (anti-Jo1)

Question 16

myositis specific Ab –> rapid onset of classical dermatomyositis

Answer 16

anti Mi2

Question 17

pathognomic for dermatomyositis or scleroderma

Answer 17

nail fold capillary changes

Question 18

5 diagnostic criteria for inflammatory myopathies

Answer 18

proximal muscle weakness, elevation serum muscle enzymes, EMG, biopsy, rash

Question 19

possible pulmonary problems associated with inflammation myopathies

Answer 19

interstitial lung disease, infection

Question 20

peak incidence (time) for inflammatory myopathy related malignancy

Answer 20

within 2 years

Question 21

top 3 malignancies related to inflammatory myopathies

Answer 21

breast, lung, ovary

Question 22

mainstay of therapy for inflammatory myopathies

Answer 22

corticosteroids

Question 23

patient population affected mostly by scleroderma

Answer 23

female, 30-40 yo

Question 24

DDX for Raynaud’s phenomenon

Answer 24

scleroderma, SLE, MCTD, vasculitis, medications

Question 25

most common cause of death related to scleroderma

Answer 25

interstitial fibrosis/pulmonary HTN

Question 26

CREST --\> variant of limited scleroderma (symptoms)

Answer 26

calcinosis of digits, raynaud's, esophageal dysmotility, sclerodatyly, telangiectases (digits and under nails)

Question 27

Ab found in most limited scleroderma patients

Answer 27

anticentromere

Question 28

markers for renal problems associated with scleroderma

Answer 28

proteinuria, HTN, azotemia, microangiopathic hemolytic anemia

Question 29

DDX for scleroderma

Answer 29

sclerederma, scleromyxedema, eosinophilic fasciitis, toxic syndroms

Question 30

specific test for the diffuse scleroderma form

Answer 30

antitopoisomerase I

Question 31

most common cause of death in Sjogrens

Answer 31

malignancy

Question 32

possible test used to diagnose Sjogrens...measures lacrimal gland output --\> highly sensitive and specific

Answer 32

Schirmer test

Question 33

Rx treatments for Sjogrens --\> to enhance oral and ocular secretions

Answer 33

pilocarpine or Cevimeline

Question 34

these NT are in lower levels in serum and CSF in fibromyalgia

Answer 34

serotonin and NE

Question 35

this NT is elevated in CSF in fibromyalgia patients

Answer 35

substance P

Question 36

type of oligarticular JRA --\> female, 50% ANA+, uveitis, long term risk of developing polyarticular

Answer 36

I

Question 37

type of oligoarticular JRA --\> male, 90% B27+, long term risk of ankylosing spondylitis

Answer 37

II

Question 38

hallmark for systemic JRA onset

Answer 38

spiking fever

Question 39

post-infectious arthritis that are on DDX for JRA

Answer 39

yersinia and rheumatic fever

Question 40

anemia common in JRA

Answer 40

normocytic hypochromic

Question 41

characteristic change of cervical spine in Xray of JRa --\> complication in polyarticular JRA

Answer 41

atlantoaxial subluxation

Question 42

main treatments for JRA

Answer 42

NSAIDs, local corticosteroids, methotrexate (if don't respond to NSAIDs)

Question 43

triad of Reiter's syndrome

Answer 43

urethritis, conjunctivitis, arthritis

Question 44

primary site of inflammation in seronegative spondyloarthropathies

Answer 44

enthesis

Question 45

possible extra-articular manifesations of ankylosing spondylitis

Answer 45

prostitis, iritis and conjunctivitis, upper lobe fibrosis (lung), CV, cauda equina inflammation

Question 46

will see dacytlitis (sausage fingers) and "lovers heel" in this arthritis

Answer 46

reiter's

Question 47

distribution of Reiter's syndrome arthritis

Answer 47

pauciarticular, large joints, lower extremity

Question 48

characteristic skin lesions seen on soles, glans penis and toes in Reiter's

Answer 48

keratoderma blenorrhagica

Question 49

these forms of arthritis can have accompanying "sausage fingers" (dactylitis)

Answer 49

Reiter's and psoriatic

Question 50

deficiency in these enzymes can cause overproduction of uric acid

Answer 50

HGPRT, G6PD, F1P aldolase

Question 51

overactivity of this enzyme can cause overproduction of uric acid

Answer 51

PRPP

Question 52

primary reasonse for under excretion of urate

Answer 52

deficiency urate exporter, medullary cystic kidney disease (kids)

Question 53

drugs that promote hyperuricemia

Answer 53

diuretics, organic acids (salicylates, pyrazinamide), cyclosporine, ehtambutol

Question 54

treatment of choice in acute gouty arthritis

Answer 54

NSAIDs (indomethacin)

Question 55

alternative to NSAID treatment of acute gouty arthritis...but does have side effect of N/V, abdominal cramps, severe diarrhea

Answer 55

colchicine

Question 56

drugs for prophylaxis of gouty attack

Answer 56

uricosuric drugs or allopurinol

Question 57

what does allopurinol inhibit? (\*used when uric acid is \>800 mg/day\*)

Answer 57

xanthine oxidase

Question 58

prophylaxis for gout if uric acid is undersecretion of urate (\*drug works to increase excretion\*)

Answer 58

uricosuric drugs

Question 59

will see this on Xray of pseudogout --\> cartilage calcification

Answer 59

chondrocalcinosis

Question 60

only potent uricosuric agent in US (increases uric acid secretion)

Answer 60

probenecid

Question 61

these crystal deposition in articular cartilage is closely tied to OA --\> can also cause chondrocalcinosis

Answer 61

basic calcium phosphate

Question 62

most common BCP crystal --\> amorphous not visible with polarized light, chronic low grade inflammation and cartilage/bone damage

Answer 62

hydroxyapatite

Question 63

most commonly affected by hydroxyapatite (BCP) crystals --\> can cause Milwaukee shoulder in elderly women

Answer 63

knee, shoulders, hips, fingers

Question 64

deposition disease associated with patients in end-stage renal disease who need hemodialysis and received ascorbic acid supplements

Answer 64

calcium oxalate deposition