4 - Connective tissue disease Flashcards
Describe connective tissue disease.
- multisystem inflammation disease
- tissues are the target of autoimmune disease
- complement activation of antibodies (found in healthy individuals) cause the tissue damage, not antibodies themselves
Describe multisystem vasculitic inflammatory disease.
Autoimmune disease that targets vessels
What are examples of connective tissue disease?
- systemic lupus erythematosis (SLE)
- systemic sclerosis (scleroderma)
- Sjorgen’s syndorme
- undifferentiated connective tissue disease (UCTD)
What are examples of vasculitic connective tissue disease?
- large vessel disease (giant cell temporal arteritis)
- medium vessel disease (polyarteritis nodosa, Kawasaki disease)
- small vessel disease (Wegener’s granulomatosis)
What is the general management of connective tissue disease?
- dependant on disease activity and immune suppression
- analgesia via NSAIDs for joint/muscle symptoms
- immune modulating treatment
- systemic steroids
Give an example of a systemic steroid used to treat connective tissue disease.
Prednisolone (short term)
Give an example of immune modulating drugs used to treat connective tissue disease.
- hydroxychloroquine
- methotrexate
- axathioprine
- mycophenolate
- biologic (cytokine inhibitors or lymphocyte depleting drugs)
What antibodies are associated with SLE and its associated diseases?
Overlap symptoms
- anti-nuclear antibody (ANA)
- anti-double-strand DNA (dsDNA)
- anti-Ro antibody (Ro)
- anti-Scl-70 antibody
- anti-neutrophil cytoplasmic antibody (ANCA)
Scleroderma
- anti-La antibody (La)
- anti-centromere antibody
Describe SLE.
- systemic lupus erythematosis
- circulating imune complexes include ANA, dsDNA and Ro antibodies (most common)
- multisystem disease affecting joints, skin, kidney, muscles, blood, CVS, RS, CNS
- renal involvement is major cause of death
- enhanced CV risk
What is discoid lupus?
- tissues change that are seen without autoantibodies
- seen in skin and mouth (appearance is similar to lichen planus)
- localised effect
How does SLE present?
- photosensitivity is common (butterfly zygomatic rash)
- raynauds phenomenon
- lupus nephritis
- CV problems
- pleural effusions
What are the dental aspects of SLE?
- chronic anaemia results in oral ulceration, can be a GA risk
- bleeding tendency, thrombocytopenia
- renal disease impairs drug metabolism
- steroid and immunosuppressive therapy increases malignancy risk
- lichenoid reaction presently similarly in the mouth
- oral pigmentation (hydroxychloroquine)
What is APS?
- antiphospholipid antibody syndrome
- marker in the blood a subtype of patients with lupus
- characterised by recurrent thrombosis (DVT with pulmonary embolism)
- venous and arterial thrombosis therefore patients are anticoagulated (usually warfarin)
What is Sjogren’s syndrome?
- inflammatory disease associated with ANA, Ro and La antibodies (not caused by)
- can either be primary (only Sjorgens symptoms) or secondary (associated with other connective tissue disease)
- presents with dry eyes and mouth (Sicca syndrome)
- major involvement of salivary glands leads to xerostomia and oral disease
How do you diagnose Sjogrens syndrome?
- signs/symptoms and clinical judgement are main diagnostic keys
- autoantibody findings
- imaging findings
- histopathology findings (labial gland biopsy)
What are the oral implications of Sjogrens syndrome?
- oral infection
- caries risk due to xerostomia
- functional loss
- denture retention poor
- sialosis
- salivary lymphoma due to chronic activation of lymphoid tissue
What is systemic sclerosis?
- excessive collagen deposition
- connective tissue fibrosis
- lost of elastic tissue (internally organs are affected eg peristalsis, externally joints and skin are less mobile)
- localised = anticentromere antibodies
- generalised = anti-Scl-70 antibodies
What are some symptoms of systemic sclerosis?
- can’t straighten fingers
- occlusion of blood vessels (raynauds) leads to ischaemic necrosis of fingers
Describe the progression of systemic sclerosis.
- gradual onset with slow progression but no treatment to prevent
- raynauds phenomenon
- renal failure
- malabsorption (GI involvement)
What are the dental aspects of systemic sclerosis?
- limited mouth opening (tissue does not stretch and will tear if retracted too far)
- progressively poor oral access
- limited tongue movement
- plan treatment 10 years ago
- compounded by sjogrens syndrome (higher caries risk)
- dysphagia
- reflux (dental erosion)
- cardiac and renal vasculitic disease affects drug metabolism
What is vasculitis?
- inflammation of blood vessels
- infarction of tissues occurs when inflammation occludes the vessel
- can present in oral inflammatory masses or oral ulceration (tissue necrosis)
What is giant cell arteritis?
- temporal arteritis (when temporal artery involved)
- presents with headache or facial pain
- involves carotid branches, can cause blindness if central retinal artery occluded
- a facial pain emergency, no specific test but ESR is raised
- prednisolone should be started asap
What is polymyalgia rheumatica?
- disease of elderly that affects large vessels
- pain and morning stiffness of muscles
- nonspecific systemic features (malaise, weight loss, fatigue)
- steroids used to treat
What is Kawasaki disease?
- disease of children
- presents clinically with fever, lymphadenopathy, crusty tongue, strawberry tongue and erythematous mucosa, peeling rash on hands and feet
- coronary vessel aneurysms that may need antibiotics
What is Wegener’s granulomatosis?
- inflammatory condition which can destroy hard and soft tissues of face and oral cavity
- spongy red tissue (oral ulceration)
- associated with ANCA
- renal and respiratory tract most affected
What is fibromyalgia?
- non specific collection of musculoskeletal problems
- joint pain
- muscle pain
- functional disorders
- no active disease or clinical findings
- pain relief rather than immunosuppression
