connective tissue diseases Flashcards
name autoimmune connective tissue diseases
Systemic lupus erythematosis (SLE)
Systemic sclerosis (Scleroderma)
Sjogrens syndrome (SS)
Undifferentiated connective tissue disease (UCTD)
special fact about multi system vasculitic inflammatory diseases
they have associated blood autoantibodies but do not cause the disease itself
can be present in “normal” people
disease can only be caused if there are certain other antibodies present
name vasculitic diseases
Large vessel Disease - Giant cell (temporal) arteritis - maxillary or temporal arteries
Medium Vessel Disease
Polyarteritis nodosa
Kawasaki disease
Small vessel Disease - Wegener’s Granulomatosis
management of connective tissue disease
NO CURE AS NO CLEAR CAUSE
Immune suppression is main one
Analgesic NSAIDs (joint/muscle symptoms)
Immune modulating treatment:
Hydroxychloroquine
Methotrexate
Azathioprine
Mycophenolate
3rd step - Biologic Medication – Cytokine inhibitors and lymphocyte depleting drugs
Systemic steroids - prednisolone = at any stage
antibodies in autoimmune diseases
Anti-nuclear antibody (ANA)
Anti-double-strand DNA (dsDNA)
Anti-Ro antibody (Ro)
Anti-La antibody (La)
Anti-Centromere antibody
Anti-Scl-70 antibody
Anti-Neutrophil Cytoplasmic Antibody (ANCA)
systemic lupus erythematosis
Tissue changes without blood autoantibodies called ‘Discoid Lupus’
Seen in the skin and the mouth – LOOKS SIMILAR TO LICHEN PLANUS
Circulating immune complexes (ANA, dsDNA & Ro antibodies)
Multi-system
joints, skin, kidney, muscles, blood
CVS, RS & CNS
Renal invlovement previously major cause
Enhanced cardiovascular risk
features of systemic lupus
genetic base
common in twins
common in females of child bearing age
one of common symptoms is butterfly zygomatic rush on the face
dental aspect of lupus
Chronic anaemia -> oral ulceration and
GA risk
Bleeding tendency - Thrombocytopenia
renal disease
impaired drug metabolism
drug reactions – photosensitivity
steroid & immunosuppresive therapy
Possible increased malignancy risk
lichenoid oral reactions
oral pigmentation from hydroxychloroquine use
lupus anticoagulant
Marker found in the blood of some patients with Lupus
anticoagulates blood in a test tube but NOT in a patient
Indicates a ‘subtype’ of lupus patient
Antiphospholipid Antibody Syndrome (APS)
they are HYPERCOAGULABLE
have recurrent thrombosis = DVT with pulmonary embolism
often on warfarin
do not stop anticoagulant !!
sjogrens syndrome
Inflammatory disease ASSOCIATED with circulating autoantibodies (ANA, Ro and La)
Mainly associated with dry eyes and dry mouth
Major involvement in Salivary glands
Leads to Xerostomia and oral disease
3 types of Sjogren’s Syndrome
Sicca Syndrome
Dry eyes or Dry mouth
Primary Sjogrens
Not associated with any other disease
Secondary Sjogrens
Associated with another Connective Tissue disease – Rheumatoid Arthiritis, SLE, etc.
diagnostic criteria for sjogrens syndrome
No one test that ‘proves’ Sjögren’s syndrome
Some signs/symptoms more important than others
Dry eyes/mouth
Autoantibody findings
Imaging findings = depends on severity
Histopathology findings
Positive criteria for diagnosis often a clinical judgement
BUT might not get any visible symptoms first as changes in lacrimal glands take about 30 years
sjogrens syndrome oral implications
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
can’t stretch or bend
Local - anticentromere antibodies
Generalised – anti Scl-70 antibodies
signs and symptoms - sclerodactyly, raynauds, nailfold capillaroscopy, telangiectasia
happens mostly in women
gradual onset and slow progression
causes renal failure and malabsorbtion (GI involvement)
dental aspects of systemic sclerosis
involvement of perioral tissues
limited mouth opening - can’t stretch skin to the sides
progressively poor oral access
limited tongue movement
PLAN TREATMENT 10yrs AHEAD!
May be compounded by sjogrens!
dysphagia & reflux oesophagitis = swallowing difficulties + dental erosion
cardiac and renal vasculitic disease -> watch drug metabolism
widening of periodontal ligament space
so no dental mobility!
what is vasculitis
inflammation of blood vessels
infarction of tissue
May present as oral inflammatory masses
May present as ulcers (tissue necrosis)
Vessel wall thickens with inflammation
Narrowing of lumen reduced blood flow
giant cell arteritis
Known as ‘temporal’ arteritis as commonly the Temporal Artery is involved
May present with Headache/facial pain
Involves other carotid branches
“Chewing claudication”
Occlusion of central retinal artery (blindness)
A facial pain emergency!
No specific test
ESR (Erythrocyte sedimentation rate) is RAISED
Also C-reactive protein & plasma viscosity
“Acute Phase” reactants
polymyalgia rheumatica
Disease of the elderly
Large blood vessels affected
Pain & morning stiffness of muscles
Non-specific systemic features
Malaise, weight loss, profound fatigue
Responds well to steroids
what is kawasaki disease
Mainly a disease of Children
AKA “mucocutaneous lymph node syndrome”
Clinically gives
Fever & lymphadenopathy
Crusting/cracked tongue
Strawberry tongue & erythematous mucosa
Peeling rash on hands and feet
Coronary vessel aneurysms
May need antibiotic cover – see cardiologist!
wegener’s granulomatosis
Inflammatory condition
Can lead to destruction of hard and soft tissues of the face and oral cavity
Spongy red tissue
Associated with ANCA -> Level correlates with clinical activity
Renal and respiratory tract most affected
dental aspect of vasculitides
Steroid precautions may be needed
May present to the dentist
Giant cell arteritis
Wegener’s Granulomatosis
Kawasaki Disease
