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)
