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)
What is the subtype of px with lupus anticoagulant called
antiphospholipid antibody syndrome
What is the primary form of antiphospholipid antibody syndrome
it has no other associated disease
What is the secondary form of antiphospholipid syndrome
found in some patients with chronic inflammatory conditions such as SLE
What antibodies are seen in the antiphospholipid antibody syndrome
antiphospholipid (aPL)
anticardiolipin (aCl)
What is antiphospholipid antibody syndrome characterized by
-recurrent thrombosis (DVT with pulmonary embolism)
are at risk of venous and arterial thrombosis so usually require anticoagulant
Note: DO NOT STOP the patients anticoagulant without talking to the physician
What is sjogrens syndrome
inflammatory disease associated with (but not caused by) circulating autoantibodies (ANA, Ro and La)
What symptoms are mainly associated with sjogrens
dry eyes
dry mouth
can be multisystem
What is sicca syndrome in sjogrens
dry eyes dry mouth
What is primary sjogrens
not associated with any other disease
What is secondary sjogrens
associated with another connective tissue disease e.g RA or SLE
What is the diagnostic criteria for sjogrens
no one test that proves it
some signs and symptoms are more important than other
Look at dry eyes/mouth, autoantibody findings
imaging findins
histopathology findings
positive criteria for diagnosis often a clinical judgement
What are the oral and dental implications of sjogrens
oral infection caries risk functional loss denture retention sialosis salivary lymphoma - unilateral gland size change - usually after years
What is systemic sclerosis
excessive collagen deposition
connective tissue fibrosis
loss of elastic tissue
local or generalized forms
What antibody is associated with local systemic sclerosis
anticentromere antibodies
What antibody is associated with general systemic sclerosis
anti Scl-70 antibodies
What gender predominantly suffer SS
women
What is the gradual onset of systemic sclerosis
raynoud’s phenomenon
then renal failure due to vasculitic damage
malabsorption as there is GI involvement
slow progression - no treatment to prevent
What is the dental aspect of systemic sclerosis
involvement of perioral tissues provide provision for patient ahead (slow progression) may be compounded by sjrogens dysphagia and reflux oesophagitis cardiac and renal vasculitic disease widening of periodontal ligament space
What does the involvement of perioral tissues in Ss result in
limited mouth opening
progressively poor oral access
limited tongue movements
What does dysphagia and reflux esophagitis result in
swallowing difficulties
dental erosion
What is significant about the widening of the PDL in SS
no dental mobility
What is vasculitis
inflammation of blood vessels
can result in infraction of tissue
vessel wall thickens with inflammation narrowing the lumen & reducing BF
How may the infarction of tissue present in vasculitis
oral inflammatory masses
ulcers (tissue necrosis)
What is giant cell arteritis
known as temporal arteritis as commonly the temporal artery is involved
involves other carotid branches
How can giant cell arteritis present
headache/facial pain
What can involvement of carotid branches in GCA result in
chewing claudication
occlusion of central retinal artery - blindness
If someone comes in with facial pain emergency what can be tested to see if GCA is expected
Erythrocyte sedimentation rate is raised
c-reactive protein and plasma viscosity
acute phase reactants
What is polymayalgia rheumatic
disease of the elderly
large BV effected
What are symptoms of polymyalgia rheumatica
pain and morning stiffness of muscles
non specific systemic features e.g malaise and weight loss
responds well to steroids
What is kawasaki disease
mainly a disease of children
What does kawasaki disease clinically give
fever and lymphadenopathy crusting/cracked tongue strawberry tongue & erythematous mucosa peeling rash on hands and feet coronary vessel aneurysms - may need Ab cover
What is wegener granulomatosis
inflammatory condition that can lead to destruction of hard and soft size fo the face and oral cavity giving look of spongy red tissue
What is wegener granulomatosis associated with
ACNA
level correlates with clinical activity
What is most affected in WG
renal and resp tract
What is fibromyalgia
non specific collection of musculoskeletal symptoms
What are the symptoms of FM
joint pain
muscle pain
What are the dental aspects of vasculitides
steroid precaution may be needed
diseases that may present to the dentist are GCA, WG and kawasaki disease
