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
what is limited systemic sclerosis?
skin on extremities only below elbows and knees plus face
pathogenesis of systemic sclerosis?
genetic predisposition + etiologic agent > endothelial cell and vascular alterations > chronic inflammation > fibroblasts, fibrocytes, endothelial cells activated > myofibroblasts > vasculopathy and tissue fibrosis
what causes skin thickening in systemic sclerosis?
fibrosis
what is CREST?
symptoms of systemic sclerosis
C = calcinosis
R = raynauds
E = esophageal dysmotility
S = sclerodactyly (thickening/tightening of hand skin)
T = telangiectasias (dilated blood vessels)
why is lung fibrosis common in SS?
fibroblasts are activated
how can SS cause pulmonary hypertension?
causes pulmonary fibrosis which restricts blood flow in pulmonary arteries
what does lupus cause in the kidney?
glomerulonephritis (inflammation)
what does SS cause in the kidneys?
fibrosis increases pressure
no inflammation
2 main antibodies of SS? how do they appear?
Anticentromere
antitopoisomerase
if you have one you don’t have the other
does everyone with SS have autoantibodies present?
no
how is SS managed?
treat raynauds and hand ulcers
treat reflux
immunosuppression for pulmonary fibrosis and skin involvement
tight control of BP - ACE inhibitors
pulmonary hypertension - prostacyclin analogues, endothelin receptor antagonists, PDE5 inhibitors
what is sjogrens syndrome?
multisystem autoimmune disease more common in men
lymphocytic infiltration of exocrine glands
main symptom of Sjogrens disease?
Main symptom = sicca
ask about dryness in eyes, mouth and vagina
what cancer are people with autoimmune diseases more at risk of?
lymphoma
other symptoms of sjogrens?
blepharitis salivary gland inflammation tooth decay lymphoma dry cough multisystem involvement
sjogrens specific antibodies?
anti Ro
anti La
how is sjogrens diagnosed?
usually ANA positive anti La and anti Ro high ESR/plasma viscosity raised IgG cytopenia if antibodies are negative - salivary gland biopsy
how is sjogrens treated?
artificial tears salivary supplements vaginal lubricants strong fluoride toothpaste Hydroxyquinone for fatigue and arthralgia immunosuppression if organ involvement
what is antiphospholipid antibody syndrome?
antiphospholipid antibodies cause the blood to become more coagulable
- anticardiolipin antibody
- lupus anticoagulant
- beta 2 glycoprotein
what must be present for antiphospholipid SYNDROME?
antibodies present on 2 occasions 12 weeks apart
pregnancy problems early or late loss)
thrombosis
when do you suspect antiphospholipid syndrome?
young person with unprovoked thrombosis
several pregnancy losses
primary vs secondary antiphospholipid syndrome?
primary = no underlying cause secondary = often due to lupus
how is antiphospholipid syndrome treated?
lifelong anticoagulation if thrombosis
LMWH and aspirin pre and post pregnancy
how does Antiphosphlipid syndrome look?
mottling on legs
= levido reticularis
why is anti Ro dangerous?
can cross the placenta into the foetus and cause congenital heart block
when is immunosuppression used in autoimmune disease?
if there is major organ involvement
if not - HCQ involvement
