scleroderma and sjogrens syndrome Flashcards
location of limited and diffuse scleroderma
limited: head, neck, distal upper extremities
diffuse: torso, abdomen, proximal upper extremity/shoulder
scleroderma characteristics
age
gender
symmetric deposition of collagen in skin.(less common hrt, kidney,lungs,stomach). affects women(4:1) and ages 30-50yrs old
raynauds phenomenon
vasospasm of digital arteries, seen in 75% scleroderma pts
CREST
calcinosis, raynauds phenomenon, esophageal dysfunction, sclerodactyly, telangiectasias. (sclerodema)
limited one
labs in diffuse vs limited scleroderma
ANA positive in diffuse. anticentromere antibody in limited.
anti-SCL-70 in diffuse and portends a poor prognosis
monitor what in scleroderma
HTN, heralding kidney involvment.
tx for scleroderma
none. tx aimed at organ specific disease processes
penicillamine for skin changes
ACE inh for renal pts
CCB for raynauds
immunosupressive drugs for pulm HTN
what is sjogrens syndrome
autoimmune disorder that destroys the salivary and lacrimal glands(exocrine)
sjogrens can be secondary to what
SLE, RA, polymyositis, scleroderma
age of occurence of sjogrens
middle aged females
xerostomia and xerophthalmia, enlarged parotid glands
dry mouth and eyes in sjogrens.
schirmers tear test
evaluates secretions of lacrimal glands.
wetting of less than 5mm of filter paper placed in lower eyelid for 5 minutes is positive for decreased secretions
main test for sjogrens
bx of lower lip mucosa confirms lymphocytic infiltrate and gland fibrosis
labs for sjogrens
RA 70%, ANA 60%, anti RO antibodies 60%, anti LA antibodies 40%
sjogrens tx
keep mucosal surfaces moist!
pilocarpine may increase saliva flow
cyclosporine may improve ocular symptoms
