Connective Tissue Diseases (R) Flashcards
What can connective tissue disease be broken down into
2 groups
autoimmune diseases
vasculitic diseases
What are connective tissue autoimmune diseases
multisystem vasculitic inflammatory diseases
The tissues themselves are inflamed (whereas in vasculitic the vessels are the target of inflammation)
What are the 4 CT autoimmune diseases
systemic lupus erythematosis
systemic sclerosis
sjrogens syndrome SS
undifferentiated connective tissue disease
What do the autoimmune diseases have in common
they have associated blood antibodies
Do the antibodies cause the disease
no, they are found in normal people and the pattern varies from disease to disease
What causes the tissue damage in the autoimmune diseases
complement activation
What are examples of vasculitic diseases
large vessel disease
medium vessel disease
small vessel disease
What is an example of large vessel disease
giant cell (temporal) arteritis
What are examples of medium vessel disease
polyartertitis nodosa
kawasaki disease
What is an example of smalll vessel disease
wegener’s granulomatosis
What is the general management for connective tissue disease
dependent on disease activity
analgesic NSAIDs (for joint/muscle symtoms)
immune modulating treatment
systemic steroids
What are analgesic NSAIDs given for?
for joint and muscle symptoms
What are the possible immune modulating treatment
hydroxychloroquine methotrexate azathioprine mycophenolate biologic medications
What are biologic medications
these can be used if immune modulators are not enough and they are synthetic antibodies against certain inflammatory markers or lymphocytic markers
What systemic steroid is used in connective tissue disease management and what can it be used for?
prednisolone
can be used at any stage short term to reduce the inflammatory process but due to their long term side effects they are not used regularly
Describe lupus
lupus has every system involved
it can have features of any of the other diseases as well as inflammatory organ diseases (this part is characteristic to lupus)
What are the antibodies that are commonly found in connective tissue disease
anti-nuclear antibody (ANA)
anti-double strand DNA (dsDNA)
anti-Ro antibody (Ro)
anti-la antibody (La)
What antibody is commonly found in scleroderma
anti-centromere antibody
anti-scl-70 antibody
anti neutrophil cytoplasmic antibody (ANCA)
What is systemic lupus erythematosis?
tissue changes without blood autoantibodies called ‘discoid lupus’ (because you get discoid lupus and systemic lupus erythematosis)
seen in the skin and mouth and looks similar to lichen planes
What are the circulating immune complexes with systemic lupus erythematosis
ANA
dsDNA
Ro antibodies
What are the systems effected by SLE
joints, skin, kidney, muscles, blood
CVS, RS, CNS
renal involvement previously major cause
enhanced cardiovascular risk
What are the features of SLE
particularly genetic - seen in twins
environmental trigger
females of child bearing age
may see a photosensitive rash
What are dental aspects of SLE
chronic anaemia (GA risk and oral ulceration)
bleeding tendency (thrombocytopenia - need platelets checked before an extraction)
renal disease (impaired drug metabolism)
drug reactions - can trigger photosensitivity
steroid and immunosuppressive therapy
lichenoid oral reactions
oral pigmentation from hydroxychloroquine use
What is a lupus anticoagulant?
it is a marker founds in the blood of some px with lupus
it is not a true anticoagulant but indicates a subtype of lupus px (it anticoagulates blood in a test tube but NOT in the patient)