Rheum D/O 4

Question 1

triple symptoms of Bahcet’s dz

Answer 1

recurrent uveitis
oral aphthous ulcers
genital ulcers

Question 2

where is Bahcet’s dz MC

Answer 2

ancient Silk Road

easter EU to the Mediterranean

Turkish, Chinese, Japanese, Korean, Saudi Arabian

Question 3

Bahcet’s dz patho

Answer 3

infectious pathogen triggers a hyperactive immune response in genetically susceptible person

possible heat shock proteins driving the reaction

Question 4

proposed infectious agents of Bahcet’s dz

Answer 4

herpes simplex
Strep 
Staph
H. Pylori 
E. coli

Question 5

Bahcet’s dz levels of immunity affected

Answer 5

B and T cells
autoantibody production
activation of coagulation cascade

Question 6

vessels Bahcet’s dz

Answer 6

vasculitis lesions of small, medium, and larger arteries and veins

VESSELS of ALL SIZES

Question 7

epidemiology Bahcet’s dz

Answer 7

25-35 yrs

Question 8

Bahcet’s dz spares

Answer 8

liver
kidneys
heart

Question 9

clinical manifestations

Answer 9

1. oral ulcers
2. genital ulcers
3. cutaneous lesions
4. ocular disease
5. neurologic dz
6. arterial dz

Question 10

Bahcet’s dz oral ulcers

Answer 10

canker sores

more extensive than typical

multiple, painful. limit eating

heal 1-3 weeks+

Question 11

most specific sign for Bahcet’s dz

Answer 11

genital ulcers

painful, heal with scarring, most often on scrotum/vulva

Question 12

cutaneous lesions (list)

Answer 12

Acneiform
pathergy
dermaographism
erythema nodosum 
pyoderma hangrenosum

Question 13

acneiform Bahcet’s dz

Answer 13

arthritis, looks like regular acne

Question 14

pathergy Bahcet’s dz

Answer 14

formation of papule 48 hrs after skin prick

cutaneous reaction following skin stimulation

Question 15

dermographism

Answer 15

light scratching produces pathology

Question 16

erythema nodosum

Answer 16

medium vessel vasculitis that ULCERATES

Question 17

Bahcet’s dz making diagnosis

Answer 17

3 episodes of oral herpetiform or apthous ulceration in 12 months (observed or reported)

2 recurrent/painful genital ulcers and ophthalmic lesions

and
2 skin lesions or positive potherb skin testing

Question 18

pharm tx Bahcet’s dz

Answer 18

gdlucocritcoids 
colchicine (mucocutaneous dz)
azathiprine 
severe immunosuppressive agents 
TNF Inhibitors

Question 19

polymyositis

Answer 19

idiopathic autoimmune inlammatory myopathy

1. symmetrical, proximal muscle weakness
2. elevated CK levels
3. characteristic EMG and muscle bx findings

Question 20

dermatomyositis is different from polymyositis

Answer 20

due to associated rash

Question 21

dermatomyositis polymyositis epidemiology

specific agents

Answer 21

MC in African American

2x as common in women

dermatomyositis: children 5-14, adults 50+
polymyositis: adults, 45-60s

Question 22

etiology polymyositis

Answer 22

altered CELLULAR immunity

T cell mediated cytotoxic process against Ag on myocytes

Question 23

associated dz polymyositis

Answer 23

MC with other autoimmune dz

Question 24

dermatomyositis etiology

Answer 24

altered HUMERAL immunity

directed against muscle capillaries and small arteriole

complement activation = mircoinfarction

Question 25

dermatomyositis polymyositis association

Answer 25

``` underlying malignancy infection medicaitons rheumatology disease HLA subtypes ```

Question 26

infection of dermatomyositis polymyositis

Answer 26

HIV coxsackievirus HBV influenza

Question 27

dermatomyositis polymyositis presentation

Answer 27

symmetrical and proximal painless myopathy weakness of large muscles of hip and shoulder girdles develop over 3 - 6 months weak neck muscles of neck flexion = difficult to hold head up sensation and DTRs preserved dysphagia constitutional symptoms interstitial lung disease

Question 28

dermatomyositis pathognomonic

Answer 28

heliotrope rash

Question 29

dermatomyositis polymyositis work up

Answer 29

1. CPK (elevated 5-50x) 2. EMG abnormal 3. muscle biopsy 4. malignancy screening

Question 30

dermatomyositis polymyositis tx (direct dz)

Answer 30

initial tx corticosteroids (prednisone) 1mg/kg/day x6-8 weeks and tapered typically responds well, but if not give cytotoxic vitamin D and Ca for osteoporosis

Question 31

dermatomyositis polymyositis CA

Answer 31

incidence of lung, bladder, non-Hodgkin lymphoma are all increased in 1st year after diagnosis

Question 32

prognosis dermatomyositis polymyositis

Answer 32

mortality is associated to malignancy and pulmonary complication 5 yr survival >80%

Question 33

inclusion body myositis

Answer 33

idiopathic autoimmune inflammatory myopathy more common in men, >50 proximal and distal muscles affected