Autoimmune - Sjogrens, polymyositis, scleroderma

Question 1

SJOGRENS SYNDROME
i) what is it? what does it primarily affect? what happens?
ii) what is F:M? when is the most common age of onset?
iii) name three conditions it may be secondary to
iv) name four symptoms? what cancer are patients at increased risk of?
v) what is there increased risk of in the mouth? how can this be treated

Answer 1

i) chronic progressive AI disorder affecting exocrine glands - lymphocytic infiltrates replace functional epithelium = decreased exocrine secretion
ii) 9:1 common between 40-60yrs
iii) RA, SLE, polymyositis
iv) sicca (dry symp) = dry eyes and mouth
salivary gland enlargement
non erosive polyarthritis hands and wrists
raynauds
risk of lymphoma
v) peridontal disease and dental caries
tx with water, salivary flow stimulands, oral hygiene

Question 2

SJOGRENS SYMPTOMS AND DIAGNOSIS
i) name two tests for the eyes that can be used to indicate sjogrens?
ii) name three things used in diagnosis? name three antibodies tested
iii) what is mainstay drug tx?

Answer 2

i) schirmer test - filter paper into eyes and wetting of paper <5mm in 5 mins indicates SS
rose bengal test - stain into eye and it is retained in the cornea
ii) minor salivary gland biopsy, acute phase reac, B cell activation (hypergammaglob)
ABs - RF, ANA, ENA (Ro and La)
iii) supportive meas for sicca and hydroxychlorquine

Question 3

POLYMYOSITIS/DERMATOMYOSITIS
i) what is it? what is F:M? what may it preceed
ii) what is polymyositis? which two areas show pain and weakness? what causes hoarsness, dysphona and difficulty swallowing?
iii) name three clinical features of dermatomyositis

Answer 3

i) idiopathic inflam myopathy
2:1
may preceed malignancy (espec in males >45yrs)
ii) poly = just muscles affected
symmetrical proximal hip and shoulder pain.weak
bulbar and intercostal muscle weakness
iii) heliotrope rash (purple discolour of eyelids), gottrons papules (red over ext surf of PIP, DIPs), calcinosis of PIPs

Question 4

MYOSITIS DIAGNOSIS
i) which two muscle enzymes are elevated? what is seen on electromyography?
ii) what is seen on muscle histology (3) is it auto antibody positive?
iii) how is it treated?

Answer 4

i) CK and ALT
electro - see polyphasic action potentials
ii) lots of inflam cells, variation in fibre size and degen/necrosis
auto AB positive
iii) prednisolone, hydroxychloroquinine, cyclo, rituximab

Question 5

SCLERODERMA
i) what phenomenon is seen? what is it
ii) what is the crest syndrome of limited cutaneous systemic sclerosis? what antibody is positive?

Answer 5

i) raynauds
hardening and thickening of skin
ii) calcification, raynauds, oes dysmot, sclerodactyly, telengiectasia