Rheumatology

1. Rheumatology By Amy Kitchen

2. Aims • An overview of Rheumatology conditions • Specific ways in which it can appear in your OSCEs • Progress Test knowledge + Kahoot

3. Conditions To Cover • Osteoarthritis • Rheumatoid arthritis • Spondylarthritides (Ankylosing spondylitis, reactive/enteric/psoriatic arthritis) • Crystal Arthropathies (gout/pseudo-gout) • Septic Arthritis • Connective Tissue Disease (SLE, Systemic Sclerosis, Myositis) • Vasculitis (GCA, Takayasu, Kawasaki etc.) • Antiphospholipid syndrome

4. Osteoarthritis • Degenerative changes to joints, increases with age. • 10% in over 60s. • Local: often affects hips & knees with pain & crepitus on movement. Worse with prolonged activity and better with rest. • Generalised: hands affected. Joint tenderness, bony swelling, Heberdens & Bouchard’s nodes.

5. Osteoarthritis: Imaging • LOSS • Loss of Joint Space • Osteophytes • Subchondrial Cysts • Subchondrial Sclerosis

6. Osteoarthritis: Management • Exercise to improve muscle strength and aerobic fitness. Weight loss. • Analgesia: paracetamol & topical NSAIDs. Codeine. Oral NSAID & PPI. Steroid injections. (Glucosamine & chondroitin are not recommended although do not do any harm). • Always use an MDT approach. OT & physiotherapists. • Surgery: joint replacement

7. Rheumatoid Arthritis • Typically: symmetrical, swollen, painful, stiff small joints of the hands and feet. Often feel worse in the morning. • Extra-articular manifestations (40% with RA) - Rheumatoid nodules, lung disease (fibrosis, bronchiolitis obliterans organizing pneumonia), cardiac disease (pericardial disease, IHD, pericardial effusion), eye disease (episcleritis, scleritis, scleromalacia)

8. Rheumatoid Arthritis: Investigations • Blood Tests • FBC, CRP, ESR, LFTs, U&Es • Rheumatoid factor, anti-CCP • X ray of affected joints • Chest X ray if lungs affected

9. Rheumatoid Arthritis: Imaging • X ray changes: LOSE • Loss of joint space • Osteopenia • Soft tissue swelling • Erosions • USS/MRI may identify synovitis and erosions more accurately.

10. Rheumatoid Arthritis: Diagnostic Criteria (ACR/EULAR) • C: Acute phase reactants • Normal CRP and ESR = 0 • Abnormal CRP or ESR = 1 • D: Duration of symptoms • <6 weeks = 0 • >6 weeks = 1 SUSPECT RA WHEN: • >1 swollen joint • Score >6 is diagnostic • A: Joint involvement • 1 Large Joint=0 • 2-10 Large Joints = 1 • 1-3 Small Joints = 2 • 4-10 Small Joints = 3 • >10 Joints (at least 1 small) = 5 • B: Serology • Negative RF & anti-CCP = 0 • Low +ve RF or Anti-CCP = 1 • High +ve RF or Anti-CCP = 3

11. Rheumatoid Arthritis: Management In GP: urgent (2 week) referral to rheumatology team – as early treatment improves outcomes. • Newly diagnosed RA: offer a combination of DMARDs (methotrexate + one other) and a short course of glucocorticoid treatment for flare-up. Ideally within 3 months of onset. • Biological drugs: Sarilumab can be added if insufficient response to previous DMARDS. Rituximab can be considered. • Symptom control: analgesia. • MDT: physiotherapy & occupational therapy for aids/splints etc. • Surgery: may improve pain & function and prevent deformity.

12. Rheumatoid Arthritis: Monitoring • Measure CRP & DAS28 monthly until treatment has controlled the disease • DAS28 involves: 1) counting swollen joints /28 2) tender joints /28 3)ESR/CRP 4) global assessment on health: mark a line between very good/very bad) • Otherwise offer annual review to: • Assess disease activity • Check for comorbidities • Assess symptoms of possible complications • Assess need for surgery • Organise appropriate MDT input • Assess effect of disease on patient’s life

13. Spondylarthritides • Seronegativity (-ve rheumatoid factor) • HLA B27 association • Axial arthritis (pathology in spine and sacroiliac joints) • Asymmetrical large-joint oligoarthritis (i.e. <5 joints) or monoarthropathies • Enthesitis: inflamed site of tendon/ligament insertion (plantar fasciitis/Achilles tendonitis) • Dactylitis – “sausage” digits • Extra-articular manifestations: iritis, rashes, aortic valve incompetence, oral ulcers, IBD • CONDTIONS: Ankylosing spondylitis, Enteric arthropathy, Psoriatic arthritis, Reactive arthritis.

14. Ankylosing Spondylitis • Chronic inflammatory disease of the spine and sacroiliac joints. Men present earlier. • Typical patient: male, <30yrs, with gradual onset of lower back pain that radiates to buttocks and improves throughout the day. Morning stiffness. Relieved by exercise. • Progressive loss of spinal movement – eventually leads to reduced chest expansion • As of AS • Anterior uvetitis • Atlanto-axial subluxation • Apical Fibrosis • Aortic insuffiency • Amyloidosis • Autoimmune bowel disease • Achilles Tendonitis

15. Ankylosing Spondylitis: Diagnosis • Clinical diagnosis • Blood • Raised ESR/CRP, positive HLA B27+ • Normocytic anaemia • Imaging • X-rays: SI joint narrowing or widening, sclerosis, erosions, ankylosis/fusion. Bamboo spine is a late sign of ankylosis & calcification • MRI: detecting active inflammation

16. Ankylosing Spondylitis: Management • Exercise – ideally with help from physiotherapist • NSAIDs for analgesia and reduced inflammation • Severe: TNF-alpha blockers • Local injections can be considered for symptom relief • Hip involvement may require hip replacement PROGNOSIS • Worse if: ESR>30, onset <16y, early hip involvement or poor response to NSAIDs

17. Psoriatic Arthritis • Can present before skin changes. • Symmetrical polyarthritis without DIP sparing. Spinal involvement. Arthritis mutilans. • Nail changes in 80% (pitting, onycholysis, subungual keratosis), dactylitis, acneiform rash, palmoplantopustolosis • Imaging: erosive changes, pencil in cup deformity • Management: NSAIDs, DMARDs, Anti-TNF agents.

18. Reactive Arthritis • Can’t see (conjunctivitis), can’t pee (urethritis), can’t climb a tree (arthritis) – Reiter’s syndrome. • Arthritis as a result of infection elsewhere in the body: typically GI or GU. • Test: ESR/CRP. Culture stool if diarrhoea. Infectious serology. Sexual health review. • Manage: No specific cure. Splint affected joints. NAIDs. Local steroid injections. Consider DMARDs if symptomatic >6 months.

19. Enteric Arthritis • Associated with inflammatory bowel disease • Arthropathy improves with treatment of bowel symptoms • Beware NSAIDs. Consider DMARDs for resistant cases.

20. Crystal Arthropathies: • Gout • Calcium pyrophosphate deposition (CPPD)

21. Gout: Clinical Features • Acute monoarthropathy with severe joint inflammation. • >50% joint occurs at first MTP joint. Other joints include ankle, foot, small hand joints, wrist, elbow or knee, • Risk Factors: reduced urate excretion. Excess production (dietary, genetic, medications etc.) • Investigation: synovial fluid shows negatively birefringent urate crystals. Serum urate often high (sometimes normal). Radiograph show early tissue swelling but late punched out erosions in juxta-articular bone.

22. Gout: Management • Acute treatment: High-dose NSAID or if contraindicated colchicine. Steroids also an option. Rest and elevate joint. Ice packs. • Prevention: • Reduce weight, avoid: prolonged fasts, alcohol excess, purine-rich meats and low-dose aspirin. • If >1 attack in 12 months, tophi or renal stones start allopurinol (3 weeks post attack and with NSAID or colchicine cover). Do not stop in acute attacks once established. Also consider Febuxostat as alternative (inhibits xanthine oxidase and reduces uric acid)

23. Calcium pyrophosphate deposition (CPPD) • Acute CPPD crystal arthritis: Acute monoarthropathy of larger joints in the elderly • Chronic CPPD: Inflammatory RA-like polyarthritis/synovitis • Risk Factors: Old age, hyperparathyroidism, haemachromotosis, hypophophataemia • Test: Positively birefringement crystals in synovial fluid. Soft tissue calcium deposit on x-ray • Management: Acute attacks use cool packs, rest aspiration and joint steroid injection. NSAIDs/colchicine may prevent acute attacks. Consider DMARDs

24. Septic Arthritis • Any hot or swollen joint is septic arthritis until proven otherwise! • >50% cases affect the knee. • Important because: • a joint can be destroyed rapidly • mortality is 11% • Risk factors: Pre-existing joint disease (RA), diabetes, immunosuppression, chronic renal failure, recent joint surgery, prosthetic joint, IV drug abuse, age > 80yr. • Investigate: Urgent joint aspiration: microscopy & culture. • Treat: Empirical antibiotics IV (after aspiration).

25. Connective Tissue Diseases • SLE • Systemic Sclerosis • Sjogren’s syndrome • Polymyositis, dermatomyositis

26. SLE (Systemic Lupus Erythematous) • Multi-system autoimmune disease • Prevalence 0.2%. Female:Male = 9:1. Typically women of child-bearing age. • Can be non-specific with malaise, fatigue, myalgia & fever. Can also have specific features. Associations with other autoimmune conditions. • Drug-induced form • Monitoring activity: Anti-dsDNA, Complement and ESR. • Jaccoud’s arthritis is a progressive deforming arthropathy following SLE

27. SLE: Criteria Laboratory Criteria Positive ANA Anti-dsDNA Anti-Smith antibodies present Antiphospholipid antibodies present Low complement (C3,4 or 50) +ve Direct Coombs Test Clinical Criteria • Acute cutaneous lupus: Malar rash/butterfly • Chronic cutaneous lupus: Discoid rash • Non-scarring alopecia: absence of other causes • Oral/nasal ulcers: absence of other • Synovitis: involving 2+ tender joints with >30minutes morning stiffness • Serositis: pleural or pericardial disease • Urinanalysis: Proteinuria or red casts • Neurological features • Haemolytic anaemia • Leucopenia: WCC<4 • Thrombocytopenia: platelets <100 Diagnosis: >4 with at least one clinical Criteria & one laboratory criteria OR biopsy-proven lupus nephritis with positive ANA or anti-DNA

28. SLE: Management • General: sunblock. Hydroxychloroquine, topic steroid in skin flares • Maintenance: NSAIDs (unless renal disease), hydroxychloroquine for joint/skin problems. Other DMARDs. • Mild flare: hydroxychloroquine or low-dose steroids • Moderate flare (organ involvement): DMARDs or mycophenolate • Severe flare: high dose steroid & DMARDs • Lupus nephritis: More intense immunosuppression

29. Systemic Sclerosis • Scleroderma, internal organ fibrosis and microvascular abnormalities • Limited: CREST syndrome (image) • Calcinosis (calcium deposits in the skin) • Raynaud’s phenomenon – spasm in blood vessels • oEsophageal dysfunction – acid reflex and less oesophagus motility • Sclerodactyly – thickening and tightening of the skin on fingers and hands • Telangiectasias – dilation of capillaries causing red marks on surface of the skin. • Diffuse: whole body SCL-70. Poor prognosis. • Manage: no cure. Immunosuppression • Can be mixed

30. Sjogren’s Syndrome • Autoimmune lymphocytic infiltration/fibrosis of exocrine glands. • Dry eyes & mouth (sicca), parotid swelling. Fatigue. • Autoimmune associations • Anti-Ro and Anti-La autoantibodies • Treat: artificial tears/saliva. NSAIDs/hydroxychloroquine for arthralgia. Immunosuppression in severe disease.

31. Polymyositis & Dermatomyositis • Rare conditions. Myositis: Progressive symmetrical proximal muscle weakness and inflamed striated muscle. May cause dysphagia, dysphonia or respiratory weakness • Dermatomyositis: myositis plus skin signs of macular “shawl sign” rash, purple helio-trope rash on eyelids, nailfold erythema, Gottran’s papules (red papules on knuckles/elbows/knees). • Test: muscle enzymes increased, muscle biopsy to diagnose. Anti-Mi2 and anti-Jo1 • Manage: prednisolone. Immunosuprresion. Hydroxycloroquine/topical tacrolimus for skin disease

32. Vasculitis • Large • Giant cell arteritis/temporal arteritis • Takayasu • Medium • Kawasaki disease • Polyarteritisnodosa • Small (ANCA-associated) • Microscopic polyangiitis • Granulomatous with polyangiitis: c-ANCA & PR3 +ve • Eosinophilic granulomatous with polyangiitis: pANCA & MPO +ve

33. Giant cell arteritis • Common in elderly • Symptoms: headache, temporal artery & scalp tenderness, tongue/jaw claudication, amaurosis fugax or sudden unilateral blindness. • Risk of irreversible, bilateral vison loss • Test: ESR and CRP raised. Temporal artery biopsy – beware skip lesions • Manage: high dose prednisolone immediately. • Prognosis: typically a 2-year course then complete remission. Reduce prednisolone once symptoms resolve. Main cause of morbidity/mortality is overuse of steroids so balance risk. Give PPI, bisphosphonate, calcium with colecalciferol.

34. Antiphospholipid syndrome • Association with SLE 20-30% of cases. Often primary disease. • Antiphospholipid antibodies • CLOTS: Coagulation, Livedo reticularis, Obstetric, Thrombocytopenia. • Diagnosis: clinical features and persistent antibodies • Treat: anticoagulation. Seek expert advice in pregnancy.

35. Autoantibodies Summary • Rheumatoid factor: RA (70%), Sjogrens (<100%), SLE (40%). Normal (2-10%). • Anti-CCP – rheumatoid specific • ANA – SLE (>95%), systemic sclerosis (96%), Autoimmune hepatitis (75%), Sjogren’s (68%), RA (30%) • Anti-dsDNA – SLE specific • Antihistone Ab – drug induced SLE • Antiphospholipid Ab (anti-cardiolipin Ab) – antiphopholipid syndrome, SLE • Anticentromere – limited systemic sclerosis • Anti-extractable nuclear antigen (ENA) • Anti-Ro & -La – Sjogrens • Anti-Sm – SLE • Anti-RNP – mixed, SLE • Anti Jo-1, Anti-Mi-2 – Polymyositis dermatomyositis • Anti-Scl70 – diffuse systemic sclerosis.

36. Autoantibodies Summary • Gastrointestinal • Antimitochondrial Ab (AMA) – primary biliary cholangitis • Anti-smooth muscle Ab (ASMA) – autoimmune hepatitis • Gastric parietal cell Ab + Intrinsic factor Ab – pernicious anaemia • Anti-TTG, anti-gliadin, anti-endomysial Ab – coeliac • Endocrine • Thyroid peroxidase – Hashimoto, Grave’s. • Renal • Anti-GBM – Goodpastures • C-ANCA/PR3 – granulomatosis with polyangiitis • P-ANCA/MPO – eosinophilic granulomatosis with polyangiitis • Neurological • Acetylcholine receptor Ab – myasthenia gravis

37. The OSCE Stuff • Examinations • Hand • Back/Spine • Joints • Histories • Back Pain • Joint Pain/ Stiffness/ Polyarthritis • Temporal Arteritis/Headache • Explanations • Osteoarthritis • Osteoporosis • Methotrexate • Bisphosphonates • Rheumatoid arthritis • Prescribing • Steroids • ?Methotrexate

38. Methotrexate: the sell • Disease – Rheumatoid arthritis is a disease where the body’s own immune system goes into overdrive and attacks the joints. This causes the joints to become red, inflamed, painful and stiff. It can also affect other areas of the body. • Action – Disease modifying drug that reduced inflammation and suppresses immune system. Early use improves symptoms and outcomes

39. Methotrexate: the tablet • Timeline – Once weekly on the same day each week (with folic acid on a different day) (Methotrexate Mondays & Folic Fridays) • How to take – Tablet (injection also available) • Length of treatment – long term • Effects (time before) – 4-6months • Tests – FBCs, LFTS, U&Es. Before starting then fortnightly until stabilised and then 2-3monthly

40. Methotrexate: drawbacks & supplementary information • Important side effects – alopecia, headache, GI upset. Myelosuppression = infection, bruising, anaemia • Complications/contraindications – Myelosuppression, liver toxicity, pulmonary toxicity. Do not use in pregnancy! Or hepatic impairment, breast-feeding, acute infection, immunodeficiency. • Supplementary advice – NO NSAIDS/aspirin, get annual flu jab. Use arthritisresearch.org.uk and patient.co.uk. Treatment breaks – but we can worry about that in the future.

41. Bisphosphonates: the sell • Disease – Bone is a living tissue. It is continually being built up and broken down. Sometimes the balance of this process shifts so there is more breaking down than building. Over time, the bones become, less dense or “thinner”, which leads to an increased risk in fractures. • Action – prevents bone from being broken down and strengthens bone to help to prevent fractures,

42. Bisphosphonates: the tablet • Timeline – Once weekly or smaller daily dose. Tablet. • How to take – swallow with a full glass of water. Take 30 mins before food/drink other than water. Be upright for 30 minutes. • Length of treatment – long term. • Effects (time before) –6-12 months although patient won’t really notice a difference. • Tests – Dental check-ups before and then regularly

43. Bisphosphonates: drawbacks & supplementary information • Important side effects – Headache, heartburn, bloating, indigestion, GI upset • Complications/contraindications – Osteonecrosis of the jaw. Avoid in: pregnancy, dysphagia, peptic ulcers, severe renal impairment. • Supplementary advice – patient.org or NOS.org.uk

44. Any Questions?