8/10 - Diseases of Immunity Flashcards
cause of systemic sclerosis
unkown
what does systemic sclerosis likely result from
interrelated processes:
1. autoimmne responses
2. vascular damage
3. collagen deposition
how does autoimmunity in systemic sclerosis work
CD4+ T cells respond to unknown antigen, accumulate in skin and release cytokines that activate inflammatory cells and fibroblasts
cytokines stimulate transcription of genes encoding collagen and other extracellular matrix proteins
describe vascular damage of pt with systemic sclerosis
microvascular disease could be present early on resulting from chronic inflammation
widespread narrowing of microvascular leads to ischemic injury and scarring
describe fibrosis in systemic sclerosis
fibroblasts of pt have intrinsic abnormality to produce excess collagen
sex and age of ppl with systemic sclerosis
women (3-5 times more likely than men)
50-60 years
distinctive feature of systemic sclerosis
striking skin changes
- insidious onset w/ cutaneous changes
clinical features of systemic sclerosis/scleroderma
- Raynaud phenomenon
- dysphagia
- respiratory difficulty
- myocardial fibrosis
what clinical feature of systemic sclerosis is seen in virtually all patients
Raynaud phenomenon
what is manifested as numbness and tingling of fibers and toes caused by episodic vasoconstriction of arteries and arterioles
Raynaud phenomenon
what is atrributed to esophageal fibrosis and its resultant hypomobility is present in more than 50% of pt w/ scleroderma
dysphagia
what does dysphagia in ssystemic sclerosis lead to
destruction of esophageal wall leading to atony and dilation
___ is caused by pulmonary fibrosis and may result in right-sided cardiac dysfunction of 40-50% of systemic sclerosis pt
respiratory difficulty
___ my cause cardiac failure in 40-50% of systemic scerosis pt
myocardial fibrosis
what is often the first sign of systemic scerosis
raynaud phenomenon
what triggers raynaud phenomenon
vasoconstrictive event:
1. emotional distress
2. exposure to cold
T/F: Raynaud phenomenon is specific to systemic sclerosis
FALSE! it is NOT SPECIFIC and can be seen in healthy individuals and other autoimmune disease
what is the color sequence of Raynaud phenomenon
white -> blue -> red
skin of systemic sclerosis
develops diffuse and hard texture and its surface is usually smooth
facial skin of systemic sclerosis
subcutaneous collagen deposition results in characteristic smooth, taut, masktlike facies; nasal alae becomes atrophied
hands of pt with systemic sclerosis
fingers fixed in claw-like position w/ resoprtion of terminal phalanges (acro osteolysis)
internal organ fibrosis of pt w/ systemic sclerosis
(and or vascular damage) involving lung, heart, GI tract, and or kidneys
what is present on the face of systemic sclerosis pt in the atrophic phase of disease
telangiectasias
what is present on the face of systemic sclerosis pt in later stage of disease
radial furrowing around the mouth
oral manifestation of pt with systemic sclerosis
- microstomia
- purse string mouth
- loss of attached gingival mucosa and gingival recession
- ! diffuse widerning of PDL
- resorption of posterior ramus of mandible, coronoid process, chin, and condyle
what develops as result of deposition of collagen in perioral tissues of 70% of patients in systemic sclerosis pt
microstomia
! diffuse widening of PDL space is often throughout dentition of what disease
systemic sclerosis
resorption of posterior ramus of mandible, coronoid process, chin, and condyle are detected in what percent of pt with systemic sclerosis
10-20%
limited cutaneous systemic sclerosis was previously referred to as what?
CREST syndrome
what does CREST stand for
C = calcinosis
R = raynaud phenomenon
E = esophageal dysmotility
S = sclerodactyly
T = telangiectasis
T/F: virtually all patients react to variety of nuclear antigens (ANAs)
trUE
2 ANA strongly associated w/ systemic sclerosis? what percent of patients?
- anti-scl 70 - directed against topoisomerase I - 10-20%
- anticentromere antibody - 20-30%
treatment for systemic sclerosis pt
difficult
1. penicillamine (systemic med) - inhibits collagen production
2. controlling symptoms
3. dental strategies = collapsible dentures
how can immunodeficiency diseases be divided
- primary/congenital
- secondary/acquired