Rheumatology. Overview Osteoarthritis Rheumatoid arthritis Psoriatic arthropathy Chronic tophaceous gout Systemic lupus Scleroderma

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1 Rheumatology This document is based on the handout from the Medicine for Finals course. The notes provided here summarise key aspects, focusing on areas that are popular in clinical examinations. They will complement more detailed descriptions and are not intended to be comprehensive. Overview Osteoarthritis Rheumatoid arthritis Psoriatic arthropathy Chronic tophaceous gout Systemic lupus Scleroderma The commonest exam cases in rheumatology are probably rheumatoid arthritis and scleroderma. Psoriatic arthropathy comes into the differential of rheumatoid as it can present with a rheumatoid pattern of arthritis. Chronic tophaceous gout is rare- except in clinical exams; every medical school seems to have a few patients with this condition who are regularly invited to help in OSCEs and short cases. Arthritis vs arthralgia Arthralgia is a symptom only- pain in a joint without any signs Signs of arthritis include: - Soft tissue swelling - Heat and redness - Reduced range of movement; painful to move - Deformity of joint Inflammatory arthropathy Symptoms: early morning STIFFNESS Worse after resting, eased by movement Signs: soft tissue swelling eg loss of valleys between knuckles Tests: raised ESR, CRP Osteoarthropathy typically presents with mechanical symptoms- pain worse with movement and eased by rest. Inflammatory arthropathies, by contrast, are dominated by stiffness as well as by pain. Both pain and stiffness are worse after resting and often improve a little after exercising. Patients with rheumatoid arthritis often complain they have pain and difficulty getting out of a chair if they have been sitting too long. They typically experience early morning stiffness (EMS) of longer than 20 minutes, which eventually eases as they get going. It is the combination of symptoms, signs and blood markers that helps to make the diagnosis. Dr R Clarke 1

2 Spindling of proximal interphalangeal joints in rheumatoid arthritis A spindle Loss of valleys between the hills of knuckles Implies soft tissue swelling around mcp joints Prominence of ulnar styloid due to palmar subluxation at the wrist Rheumatoid nodule Rheumatoid arthritis Symmetrical, inflammatory arthritis Small joints of hands and feet Also hips, elbows and knees Young adults, female:male = 3:1 HLA DR4 associated Dr R Clarke 2

3 Z deformity of thumbs Prominence of ulnar styloid Swelling and palmar subluxation at mcp joints with ulnar deviation Rheumatoid hands What is rheumatoid factor? IgM against your own IgG Lots of normal people have it Lots of people with classic rheumatoid do not have it It is not a diagnostic test but helps with prognosis - high titres associated progressive disease I want the arthritis test, doctor OK, I ll check your blood to see if the sedimentation rate has been affected And I ll request an x-ray of your hands and feet to make sure you haven t got any serious damage to the joints (erosions) But I don t think a rheumatoid factor test will help us because the results are so often misleading Seronegative rheumatoid Identical disease presentation to seropositive in most respects One third of patients are seronegative They are most unlikely to have nodules Less likely to be rapidly progressive Most do have some non classical rheumatoid factors if you look carefully- usually IgG vs IgG, unlike classical rheumatoid factor (IgM vs IgG) Note that seronegative rheumatoid is quite separate from seronegative arthritis. The former is clinically similar to seropositive rheumatoid, apart from the absence of nodules and a generally less aggressive clinical course. The latter is a whole family of illnesses involving an asymmetrical oligoarthropathy- a few joints only. Examples include Reiter s syndrome, ankylosing spondylitis and the arthropathies which accompany inflammatory bowel disease. Dr R Clarke 3

4 Disease modifying anti-rheumatic drugs (DMARD S) Sulfasalazine- marrow suppression, oligospermia, hepatitis, rashes Gold- nephrotic syndrome, marrow suppression Penicillamine- disturbance of taste, nephrotic syndrome, myasthenia Chloroquine- retinopathy, tinnitus Steroids- diabetes, truncal obesity; azathioprine- marrow suppression Cytotoxic drugs eg methotrexate- hepatic toxicity, marrow suppression Newer immunosuppressants eg leflunomide- marrow suppression Tumour necrosis factor antagonists eg infliximab- infusion reactions including anaphylaxis, infections including re-activation of TB Atlanto-axial subluxation spinal cord compression atlas Head flexion axis 4mm odontoid peg This complication of rheumatoid arthritis is often asked about. For example, What precautions would you take before sending this patient to theatre for hand surgery? Intubation involves flexion and extension of the neck. It is most important that the anaesthetist is made aware of any neck involvement. A lateral cervical spine x-ray in flexion will show narrowing of the space between the back of the anterior arch of the atlas and the odontoid peg if there is instability with subluxation (due to involvement of the ligaments at the top of the spine). Rheumatoid hands: palms Palmar erythema Wasting of thenar eminence- carpal tunnel syndrome Fixed flexion contracture Specific abnormalities- swan neck, button hole, z thumb Dr R Clarke 4

5 Rheumatoid hands: dorsal aspect Symmetrical, peripheral destructive arthropathy Soft tissue swelling with spindling of proximal joints and loss of valleys between knuckles Palmar subluxation and ulnar deviation at metacarpo-phalangeal joints Nodules- otherwise rheumatoid pattern Gouty tophus Rheumatoid nodules Examine the hands means Examine the hands and the elbows and the ears Dr R Clarke 5

6 Psoriatic arthropathy Oligoarthritis (commonest- 70%) Distal (classical- 15%) Rheumatoid pattern (surprisingly common- 10%) Arthritis mutilans Sacroiliitis Seronegative arthritis Different from seronegative rheumatoid Asymmetrical oligoarthritis Sacroiliitis Enthesopathy (where tendons insert into bone) HLA B27 associated with ankylosing spondylitis Anterior uveitis (acute iritis) Bone Joint capsule Enthesis Tendon Dr R Clarke 6

7 Bony ankylosis with bamboo spine Syndesmophyte bridging Fusion of sacroiliac joints Forward flexion deformity Ankylosing spondylitis Hyperuricaemia Commonly asymptomatic Is associated with hypertriglyceridaemia and hypertension Usually idiopathic; also thiazides, cytotoxics, renal failure, neoplasms May cause acute gout (treated with NSAID; alternatives colchicine or steroids) Some patient suffer repeated episodes of acute gout A few suffer from accumulation of urate deposits: tophi Tophi Ear- tophi in cartilage of helix Kidney- radiolucent stones Soft tissues- eg some cases of olecranon bursitis Small joints- chronic tophaceous gout Chronic tophaceous gout Destruction of articular cartilage Punched out erosions on x-ray Asymmetrical small joint arthropathy of hands and feet Indicates need for prophylaxis Allopurinol - several weeks after acute attack settled With initial NSAID cover Systemic lupus Classical immune complex disease Non specific presentation- eg fever, weight loss, anaemia Light sensitive rash Nail fold vasculitis- nail fold infarcts and splinter haemorrhages Small joint arthritis Dr R Clarke 7

8 Systemic lupus Hypertension Renal failure / Nephrotic syndrome Pleurisy Pericarditis Cerebral lupus Sclerodactyly Scleroderma affecting the hands May be part of systemic sclerosis or one of the milder variants, such as CREST syndrome Raynaud s phenomenon Tight, cold, waxy skin with telangiectasia Pulp atrophy may lead to ulceration Subcutaneous calcification Tethering of skin over forehead and nose Telangiectasia Radial furrows around mouth Limited mouth opening microstomia Scleroderma Systemic sclerosis More extensive scleroderma with internal organ involvement Mask like face, wrinkled skin around small mouth (microstomia) Tethering of skin over nose ( beaking ) Interstitial pulmonary fibrosis (with restrictive ventilatory defect) Renal involvement (hypertension, renal failure) Atonic oesophagus-reflux and aspiration Dr R Clarke 8

9 CREST syndrome Calcinosis Raynaud s Esophageal involvement Sclerodactyly Telangiectasia Three questions to ask Do your hands change colour in the cold? (white, blue, red) Do you get breathless? (scleroderma affecting the chest wall or interstitial fibrosisboth cause restrictive defect) Do you get indigestion or heartburn? Dr R Clarke 9