Rheum PassMed 2

Question 1

what is dermatomyositis?

Answer 1

an inflammatory disorder causing symmetrical, proximal muscle weakness and characteristic skin lesions

Question 2

what is dermatomyositis associated with?

Answer 2

connective tissue disease

underlying malignancy

Question 3

what is sjogrens syndrome

Answer 3

autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces

Question 4

what is there a high risk of with sjogrens syndrome?

Answer 4

lymphoid malignancy

Question 5

manage sjogrens syndrome?

Answer 5

artificial saliva and tears

pilocarpine may stimulate saliva production

Question 6

what is osteogenesis imperfecta?

Answer 6

known as brittle bone disease, is a group of disorders of collagen metabolism, resulting in bone frailty and fractures. The most common form is type 1, which is a mild form

Question 7

how does osteogenesis imperfecta present ?

Answer 7

fractures following minor trauma, blue sclera, deafness secondary to otosclerosis and dental imperfection

Question 8

what is pagets disease

Answer 8

disease of increased but uncontrolled bone turnover

disorder of osteoclasts, with excessive osteoclastic resorption followed by increased osteoblastic activity.

Question 9

what is the classical presentation of pagets disease

Answer 9

the stereotypical presentation is an older male with bone pain and an isolated raised ALP