Connective Tissue Disorders Flashcards
Define systemic lupus erythematosus (SLE)
an autoimmune disease in which the body’s immune system mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys, brain, and other organs.
Define Limited scleroderma (=CREST)
one subtype of scleroderma — a condition that literally means “hardened skin.” The skin changes associated with limited scleroderma typically occur only in the lower arms and legs and sometimes the face and throat. Limited scleroderma can also affect your digestive tract.
Define Diffuse scleroderma
a type of systemic sclerosis (scleroderma). Systemic scleroderma can affect any part of the body. It often affects the skin, blood vessels, and internal organs, especially the lungs, heart, kidneys, and gastrointestinal tract.
Define polymyositis
(“inflammation of many muscles”) is a type of chronic inflammation of the muscles (inflammatory myopathy) related to dermatomyositis and inclusion body myositis.
Define dermatomyositis
an uncommon inflammatory disease marked by muscle weakness and a distinctive skin rash.
What is the HLA associated with SLE?
HLA-DR3
What is the HLA associated with scleroderma (limited & diffuse)?
HLA-DR5 & DRw8
What is the main clinical feature of scleroderma?
Skin fibrosis/hard skin
Polymyositis & dermatomyositis is associated with?
Underlying malignancy
What hypersensitivity reaction is SLE?
Type III -immune complex (tissue damage caused by the activation of complement in response to antigen-antibody (immune) complexes that are deposited in tissues.)
What are the 4 key features of connective tissue disease?
Inflammation Fibrosis/scarrign Vasospasm (including Reynaud's) Vascular thrombosisInflammation Fibrosis/scarrign Vasospasm (including Reynaud's) Vascular thrombosis
Risk factors for SLE?
Drug-induced
Increased in classical complement deficiencies
In afrocarribean
FemalesDrug-induced
Increased in classical complement deficiencies
In afrocarribean
Females
Autobodies associated with SLE:
ANA (95%) Anti dsDNA Anti-Sm Drug-induced Anti-histoneANA (95%) Anti dsDNA Anti-Sm Drug-induced Anti-histone
Autobodies associated with Limited scleroderma CREST:
Anti-centromere
Autobodies associated with diffuse scleroderma:
Anti Scl-70 Fibrillarin RNA pol I, II, III PM-SclAnti Scl-70 Fibrillarin RNA pol I, II, III PM-Scl