Connective Tissue Disease Part 2 Flashcards
what generally causes autoimmune disease?
breakdown of immunological tolerance to autoreactive immune cells
genetic, environmental and infectious pre-disposing mechanisms
what type of immune response is typically involved in autoimmunity?
adaptive
- cell mediated
- humoral
can autoimmune conditions coexist?
yes
overlap syndrome
- has features of more than 1 disease but cant be clearly defined as one type
who is autoimmune disease more common in?
women
2 types of autoimmune disease?
primary
secondary (due to another condition)
what are autoimmune diseases associated with?
auto antibody formation
when do you suspect autoimmune disease?
young women of joint and muscle problems photosensitivity/malar rash raynauds sicca symptoms (dryness in mucosal areas) - in absence of other causes dysphagia, SOB neurological symptoms pregnancy problems unprovoked thrombosis
how are antibodies used in diagnosis of autoimmune disease?
more specific then sensitive
ANA = general test for all autoimmune test
- higher titre = more significant
if ANA positive, sample analysed for other antibodies
what is raynauds?
in 90% of systemic sclerosis
vasospasm causing white fingers in cold
types of raynauds?
primary (benign, gets better in summer)
secondary = with autoimmune, doesn’t improve
3 phases of raynauds?
1 = ischaemia (become pure white) 2 = cyanosis (blueish) 3 = rubor = (redness as blood returns)
what indicates secondary raynauds?
ulcers or gangrene in hands
how is raynauds treated?
keep warm vasodilation - CCBs, PDE5 inhibitors prostacycline inhibitors for finger ulcers botox injections endothelin receptor antagonists
systemic sclerosis vs scleroderma?
scleroderma = thickening of skin (symptom of systemic sclerosis)
what are the 2 main features of systemic sclerosis?
scleroderma
raynauds
what are the 2 types of systemic sclerosis?
diffuse
limited
what is diffuse systemic sclerosis?
skin on extremities above and below elbows and knees plus face and trunk