14-HD Rheumatology 2020 PDF

Introduction to Rheumatology HUD02-3005 Professor Jane Setterfield Department of Oral Medicine & St John’s Institute of Dermatology, Guy’s & St Thomas’ NHS Foundation Trust Hospitals King’s College London Autoimmune diseases Rheumatoid arthritis (RA) Sjogren’s Syndrome (SS) Systemic lupus erythemato...

Introduction to Rheumatology HUD02-3005 Professor Jane Setterfield Department of Oral Medicine & St John’s Institute of Dermatology, Guy’s & St Thomas’ NHS Foundation Trust Hospitals King’s College London Autoimmune diseases Rheumatoid arthritis (RA) Sjogren’s Syndrome (SS) Systemic lupus erythematosus (SLE) Scleroderma Osteoarthritis Learning outcomes By the end of this lecture students should be able to: Describe the clinical presentation and complications of rheumatoid arthritis, systemic lupus erythematosus, Sjogren’s syndrome, scleroderma and osteoarthritis. Outline the pathogenesis of rheumatoid arthritis specifically and explain how autoantibody profiles can assist with diagnosis of connective tissue disease. Discuss in brief the types of treatment used in disease management and their impact on dental treatment. GDC Learning outcomes CLINICAL 1.1 Foundations of practice (1.14, 1.15, 1.18, 1.19). 1.2 Comprehensive patient assessment (1.2.1, 1.2.2, 1.2.3, 1.2.4, 1.2.5,1.2.6,1.2.7). 1.4 Diagnosis (1.4.1, 1.4.2). 1.5 Treatment planning (1.5.1, 1.5.3). 1.7 Patient management (1.7.1, 1.7.2, 1.7.3, 1.7.4). 1.8 Patient and public safety (1.8.5, 1.8.6, 1.8.7,). 1.9 Treatment of acute oral conditions (1.9.1, 1.9.2, 1.9.3,1.9.4). 1.10 Health promotion and disease prevention (1.10.3, 1.10.4,). COMMUNICATION 3. Patients, their representatives and the public (3.1, 3.2, 3.3). 4. Team and the wider healthcare environment (4.1). 5. Generic communication skills (5.1). PROFESSIONALISM 6. Patients and the public (6.1, 6.2, 6.3). 9. Development of self and others (9.1, 9.3 and 9.4). 12. Managing the clinical and working environment (12.1 and 12.2) Aims of presentation §Clinical features §Radiological and pathological characteristics §Medical management §Impact on dental care Autoimmune disorders Rheumatoid arthritis (RA) Sjogren’s Syndrome (SS) Systemic lupus erythematosus (SLE) Scleroderma Aetiopathogenesis Unknown cause Combination of genetic and environmental factors leading to breakdown of immune tolerance Associated with MHC: Rheumatoid Arthritis (RA): HLA-DR4 (more frequent in smokers) Systemic Lupus Erythematosus (SLE): HLA-DR3, complement components Progressive systemic sclerosis (PSS): HLA-DR3 Immunological associations: serology often positive for a variety of autoantibodies Histology shows evidence of immune involvement The auto-antibodies you should know RA: : RF (Rheumatoid factor) non-specific Anti CCP (anti-citrullinated protein antibodies) 95% specific SLE: ANA + dsDNA (antinuclear antibodies) PSS: Scl 70 Sjogren’s: ENA (Ro, La) RF Rheumatoid factor in rheumatic diseases May be any isotype but is directed to Fc IgG RA 80% Sjogren’s 70% Cryoglobulinaemia >90% antibodies to citrullinated peptides CCP antibodies 95% specific for RA Rheumatoid factor in rheumatic diseases RA 80% Sjogren’s 70% Cryoglobulinaemia >90% Systemic lupus erythematosus (SLE) Sjögren syndrome Interstitial pulmonary fibrosis Hepatitis B, chronic liver disease, and chronic hepatitis Essential mixed cryoglobulinemia Primary biliary cirrhosis Infectious mononucleosis and any chronic viral infection Bacterial endocarditis Leprosy Sarcoidosis Tuberculosis Syphilis Visceral leishmaniasis Malaria Leukemia Dermatomyositis Systemic sclerosis After vaccination/transfusion in normal individuals Rheumatoid arthritis Clinical features § 1% population F:M 2.5:1 § Symmetrical polyarthritis often starts in hands and wrists § Systemic disease which may have extra-articular involvement § Symptoms: pain, swelling and morning stiffness malaise non-articular symptoms § Signs: Swelling warmth tenderness limited movement deformity Rheumatoid arthritis subluxed MCP joints, ulnar drift, Z shaped thumb) Normal hand Rheumatoid arthritis Meta-tarsal phalangeal joints become subluxed (dislocated) Rheumatoid arthritis Pathology granulation tissue forms at the edges of the synovial lining (pannus) Complement activation and release of destructive enzymes RA The synovium thickens (pannus) Villous hypertrophy Villous fronds Pannus: eroding cartilage and bone pannus bone cartilage Cytokines and chemokines attract and accumulate immune cells, i.e. activated Tand B cells, monocytes and macrophages in the joint space Osteoclast production results in degradation of bone tissue Progressive osteopenia, loss of joint space and bone loss in RA Knee joint damage - Rheumatoid arthritis Complications of RA § Rheumatoid nodules § Tendon rupture § Normochromic, normocytic anaemia § Nerve entrapment e.g. median nerve § Vasculitis § Atlanto-axial subluxation § Eye complications Rheumatoid nodule Nodules are necrotising granulomas Rheumatoid nodule Subcutaneous nodule Rheumatoid nodules Digital gangrene secondary to vasculitis Episcleritis Perforating episcleritis in RA Site of Atlanto-axial subluxation Occurs due to erosion of the odontoid process and / or the transverse ligaments connecting the cervical spine to the skull. Treatments specific for RA Analgesia (e.g. NSAIDs reduce pain and swelling) DMARDs especially methotrexate, hydroxychloroquine for mild to moderate TNFa blockade e.g. Infliximab, Etanercept Anti-B cell monoclonal ab e.g. rituximab Relevance to dentistry § Hand deformity – oral hygiene, blister packs § Carpal tunnel syndrome § Atlanto-axial subluxation during GA § TMJ dysfunction § Sjogren’s syndrome § Anaemia § Complications of systemic treatment Learning points so far - RA Common autoimmune disease The joint complications are not usually seen nowadays due to improved therapy Multisystem disease – remember the extra-articular complications Remember the DMARD /immunosuppressive treatment may impact your dental care Sjogren’s syndrome Sjogren’s syndrome Primary (dry eyes, mouth, vagina) Secondary Rheumatoid arthritis Progressive systemic sclerosis SLE Sjogren’s Syndrome Autoimmune sialadenitis F > M (9:1) 2 Peaks of onset mid 30s & postmenopausal 45-60 Sicca symptoms (95%) - Dryness of mouth, eyes, skin, vagina Dental carries; loss of teeth, candida Excessive fatigue (75%), arthralgia & myalgia Swollen salivary glands/lachrymal glands Increased risk of lymphoma/Maltoma B cell lymphoma Mucosal Associated Lymphoid Tissue Swollen parotid gland Smooth, lobulated tongue, angular stomatitis (candidal infection) What Investigations are required? Unstimulated whole salivary flow (Normal > 0.2 ml/min) Stimulated parotid flow (Normal > 0.4ml/min) Schirmer test Anaesthetise eye first then apply filter paper to lower lid Sjogren’s - moistens less than 5 mm in 5 minutes. Normal is ≥15 mm wetting of the paper after 5 minutes. Bloods FBC, inflammatory markers Auto antibodies, Ro & La Others as indicated Investigations Ultrasound has largely replaced Sialography & Scintigraphy Labial gland biopsy – dense focal lymphocytic infiltrate UCSF-SICCA Focal lymphocytic sialadenitis (FLS) Relevance to dentistry §Dry mouth - Dental carries, loss of teeth, candida §Salivary gland enlargement §Complications of systemic treatment Lupus erythematosus Variants Discoid lupus (DLE) – scarring skin lesions /oral DLE Subacute cutaneous (SCLE) (rash, systemic disease) SLE Anti-phospholipid syndrome SCLE Drug-induced lupus Aetiology §SLE is a multi-system disease §Pathology – widespread vasculitis of capillaries, arterioles and venules §Aetiology – genetic, complement deficiency §Triggers – UV light, infection §Drug-induced lupus-like syndrome Investigations FBC - anaemia ESR raised CRP (normal) ANA + Double stranded DNA ab+ RF+ Complement – reduced Skin biopsy Photosensitivity rash Vasculitis with gangrene in SLE Cerebral micro-infarcts lead to: headaches, dizziness, behaviour changes, hallucinations, and even strokes or seizures. Many people with lupus experience memory problems and may have difficulty expressing their thoughts. Lupus nephritis 5% patients with SLE will develop some degree of renal involvement. This may manifest as: Foamy urine due to increased protein in the urine Brownish urine due to blood in the urine Elevated creatinine in the blood High blood pressure Swelling (oedema) of the feet, lower legs and occasionally the eyelids Anti-phospholipid syndrome Anti-Phospholipid Syndrome Increased tendency to clotting Venous thrombosis Arterial thrombosis Recurrent foetal loss Thrombocytopenia Livedo Reticularis Relevance of lupus erythematosus to dentistry § Oral ulceration § Complications of systemic treatment § Candida § +/-Dry mouth § Main differential is lichen planus Scleroderma Scleroderma Sub-types: §Systemic fibrosis (Diffuse systemic sclerosis) § Lungs § Kidneys § Gastrointestinal tract: malabsorption § Thickening of skin starts distally Endothelial damage ultimately leading to vasoconstriction Fibroblast activation §Limited systemic/ CREST syndrome §Localised to skin - morphoea Diffuse systemic sclerosis Scleroderma :skin induration, flexion fingers Scleroderma: acrosclerosis and terminal tuft resorption Scleroderma: hand arteriogram Scleroderma: skin changes PSS skin Normal skin Scleroderma: facial and mouth changes Scleroderma: late facial changes Scleroderma: diverticula (malabsorption) Scleroderma: intimal arteriolar proliferation (kidney) Scleroderma: pulmonary fibrosis C.R.E.S.T – limited systemic disease §Calcinosis §Raynaud’s phenomenon §Oesophagus §Sclerodactyly §Telangiectasia CREST: telangiectesiae face and tongue Raynaud’s phenomenon Reversible skin colour change - White to blue to red Due to Vasospasm Induced by cold or emotion Raynaud’s progressing to cyanosis, gangrene CREST: oesophageal involvement CREST: calcinosis CREST: calcinosis, acrolysis Treatments Analgesics Glucocorticoids Immunosuppressive: cyclophosphamide, azathioprine, mycophenolate mofetil Biologics Treatment of Raynaud’s: Physical (gloves) Nifedipine (calcium channel antagonist) Losartan (angiotensin II antagonist) Relevance to dentistry § Microstomia § Widened periodontal membrane § Severe sclerodactyly – difficulty with OH § Immunosuppression Osteoarthritis population over 60 yrs 10% genetic predisposition Pathogenesis: degenerative disease of cartilage Stimuli: mechanical insults, biochemical abnormalities of cartilage Primary OA: Majority of cases Strong family history Onset around 50 Secondary: Congenital abnormality of joints, structural disorders in children, trauma, Heberden’s nodes Clinical features Symptoms Pain – worse with use Morning stiffness