Systemic rheum disease Flashcards
4 features of collagen vascular diseases
1 inflammation - causes disease activity
2 immune activation - leads to loss of tolerance
3 vasculopathy - leads to tissue ischemia and necrosis
4 fibrosis - end stage from chronic inflammation - organ dysfunction
how do systemic diseases present
generally undifferentiated and then become more classic over time
mech. of SLE
loss of tolerance>auto-antibodies>
- cytotoxic ABs>tissue inflammation and damage
- immune complex formation>low complement and inflammation/damage
3 ways to help diagnose SLE
- bloods - cytopenia, renal involvemnet
- ANAs
- possibly kidney biopsy
Sx in SLE
- rash
- scalp/alopecia
- glomerulonephritis (nephrotic syndrome)
what is seen on serology in SLE (5)
- cytopenia
- ANA
- dsDNA
- anti smith
- low c3/c4
2 key features of SLE
- immune activation
2. inflammation
prominent features of scleroderma
- NOT inflammation
- vasculopathy dominant
- thickening and fibrosis predominant
4 common manifestations of vasculopathy
- raynauds
- fingertip ulcers
- nailbed hemmorages
- telangiectasias
3 consequences of fibrotic changes in scleroderma
- sclerodactyly
- facial changes
- interstitial lung disease
CREST form of sclerodrma
Calcinosis Raynauds Eso dysmotility Scerodactyly Telantectasia
typical presentation of inflammatory myositis
- PAINLESS proximal muscle weakness
2 types
polymyositis - primarily in muscles
dermatomyositis - in skin too
2 tests to help diagnose myositis
- CK in blood
2. muscle biopsy
2 features at work in mysotis
- immune inactivation
2. inflammation
3 other collagen vasc. disorders
- sjorgens
- mixed CT disease
- overlap syndromes
def. of vasculitis
inflammation and necrosis of blood vessesl
- dilations and rupture
- ischemia and thrombosis
classification of vasculitis
by size
- small
- medium
- large
4 constitutional Sx of vasculitis
very unwell
- fever
- weight loss
- anorexia
- fatigue
skin manifestations
small - pupura - urticaria - splinter hemmorage med - infacrts - gangrene
other organ involvment
joints - arthritis kidneys - glomeruloneph and infarcts brain - strokes and seizures lungs - hemmorage heart - MI , myopathy bowel - infarcts
3 tests for vasculitis
- blood work
- ESR
- organ dysfunction - tissue biopsy
- of vessel - imaging
Sx of giant cell arteritis
- constitutional Sx
- new onset headache
- jaw claudication
- shoulder stiffness
- large and dilated tempral vein
what to test for in GCA
1 ESR
2. temporal arteryr biopsy
treat of GCA
high does prednisone
