RHEUMATOID ARTHRITIS
Case vignette
A 44-year-old female presents with severe depression. She was diagnosed with rheumatoid arthritis 3 years ago. She has experienced painful upper- and lower-extremity small joint arthritis despite therapy with multiple agents. The disease has progressed despite therapy, leading to deformed fingers, and she has had to give up her job as a designer. Lately she has experienced extreme fatigue and progressive dyspnoea. In the wake of these symptoms she has gone through the break-up of her 10-year marriage.
On examination the patient appears cushingoid and has a flat facial affect. Upper limb examination shows mild ulnar deviation of her fingers bilaterally. Proximal interphalangeal joints and metacarpophalangeal joints are tender, warm and boggy to palpation. On auscultation of her lung fields there are fine crepitations distributed widely. Her JVP is elevated and she has peripheral oedema.
Approach to the patient
History
Ask about:
• joint pain, stiffness, swelling and tenderness
• distribution of the joint symptoms
• onset, duration and frequency of symptoms—age of onset is important
• pain on moving the neck—would suggest involvement of the cervical spine, particularly the upper segment
• whether the patient has experienced any neurological deficit in the way of localised weakness and/or sensory loss
• dyspnoea or exertional dyspnoea
• any extraarticular features (as listed in the box below)
• whether the patient suffers from any constitutional symptoms, such as lethargy/ fatigue, anorexia, weight loss or fever
• the impact of the disease on the patient’s social life, occupational life, finances and overall quality of life
• the various treatment modalities tried, and their effects and side effects
• toxicities the patient has experienced with NSAIDs and slow-acting anti-rheumatoid arthritis drugs (SAARD)—should be especially enquired into
• detailed history of the psychosocial impact of the disease.
Examination
The examination needs to be detailed and meticulous. Look for wasting or cushingoid body habitus. Note any skin bruises, oral ulcers, oral candidiasis or surgical scars. Look for features that are characteristic of rheumatoid arthritis, such as symmetrical distribution of deforming arthropathy, involvement of the proximal interphalangeal joint, metacarpophalangeal joint and the wrist (Fig 13.1, overleaf), Baker’s cyst of the popliteal fossa, and involvement of the upper cervical spine and the joints of the feet. Warm, tender and stiff joints may suggest active arthritis.
Assess the JVP and feel the precordium for a right ventricular heave. Auscultate the lung fields for fine crepitations of fibrosis. Listen to the heart for muffling of heart sounds due to pericardial effusion and loud P2 due to pulmonary hypertension. Palpate the abdomen, looking for splenomegaly. Perform a detailed eye examination and a neurological examination.
Hand examination is important. The following features should be looked for:
• radial deviation of the wrist
• ulnar deviation of the digits, swan-neck deformity and boutonnière deformity of the fingers, palmar subluxation of proximal phalanges
• ‘Z’ deformity of the thumb
• wasting of intrinsic musculature
• Tinel’s sign and neurological deficit in the distribution of the median nerve.
Assess the manual functional ability by asking the patient to pick up a pen and write their name, hold a cup, button and unbutton a shirt, and comb their hair.
Examination of the foot is also significant. Features to look for include:
Extraarticular manifestations of rheumatoid arthritis
• Rheumatoid nodules in the elbow, Achilles tendon and the occiput (Fig 13.2)
• Vasculitic features, such as nailfold infarcts (Fig 13.3), necrotic leg ulcers, mononeuritis multiplex
• Pleural effusion and bibasilar fine crepitations of pulmonary fibrosis (Fig 13.4)
• Signs of pericardial effusion—elevated JVP, peripheral oedema etc (Fig 13.5)
• Ocular signs of scleritis, episcleritis, scleromalacia perforans (Figs 13.6–13.8)
• Splenomegaly, if Felty’s syndrome is suspected
Investigations
1. Serum rheumatoid factor—this is present in only two-thirds of patients suffering from rheumatoid arthritis. It has more prognostic than diagnostic value, in that rheumatoid factor positivity is associated with more severe disease and the development of extraarticular manifestations. Human leucocyte antigen (HLA) haplotyping may have diagnostic and prognostic utility. Anti-CCP (anti-citrullinated cyclic peptide) is a very important disease-specific marker and may be detected several years prior to disease onset.
2. Full blood count—looking for anaemia of chronic disease or iron deficiency anaemia associated with NSAID therapy and/or thrombocytopenia and leucopenia of Felty’s syndrome. Anaemia and thrombocytosis correlate with disease activity.
3. Inflammatory markers such as ESR and C-reactive protein level—to monitor disease activity
4. Synovial fluid analysis—looking for features that suggest active arthritis, such as low viscosity, white cell count 20000–50000/mL with a predominance of polymorphs
5. X-rays of involved joints—looking for periarticular soft tissue swelling, joint deformity, joint space narrowing, juxtaarticular osteopenia, loss of articular cartilage and bony erosions, in chronic cases (Fig 13.9, overleaf)
6. Chest X-ray—looking for pleural effusion, rheumatoid nodules, features suggestive of pulmonary fibrosis and cardiomegaly suggesting pericardial effusion
7. Echocardiogram—looking for pericardial effusion, pulmonary hypertension (usually secondary), right heart strain and any suggestion of tamponade, which is a particularly rare phenomenon
8. Urine analysis—looking for proteinuria, and renal function indices looking for evidence of renal failure due to therapy-related toxicity
9. Liver function indices—looking for evidence of methotrexate or sulfasalazine toxicity
Management
Management of rheumatoid arthritis has three objectives: 1) control the symptoms, 2) optimise function and 3) retard progression.
Symptom control
This requires an interdisciplinary approach. Pain and stiffness can be managed with physical therapeutic modalities, antiinflammatory agents such as aspirin, NSAIDs, cyclo-oxygenase-2 inhibitors and parenteral glucocorticoid injections. Systemic glucocorticoid therapy is helpful in high-dose pulses at times of severe exacerbation and in low doses for the maintenance of remission. Severe deformity may need corrective surgery. The patient may need physical therapy for joint stiffness, and occupational therapy to improve functionality. Psychological and social help should be facilitated as required.
Disease-modifying agents
These drugs prevent disease progression and joint damage. They should be commenced early in the disease for maximum benefit. However, their onset of action is usually delayed by about 2–4 months. Some commonly used agents are as follows:
1. Methotrexate—given at weekly doses that vary between 7.5 mg and 25 mg per week. The maximum benefit may take up to 6 months to manifest. Patients should be monitored for the side effects of oral ulcers, hepatotoxicity, pneumonitis, lung fibrosis, anaemia and bone marrow suppression. Patients should be administered folic acid supplements concurrently. As this agent is teratogenic, female patients of reproductive age should take contraceptive measures while being treated.
2. Sulfasalazine (in male patients this agent can cause mild azoospermia)
3. Hydroxychloroquine—be aware of ocular complications
4. Gold
5. Leflunomide
6. Anti-tumour necrosis factor (TNF) agents play a major role in the management of rheumatoid arthritis—infliximab, an antibody against TNF-alpha, or etanercept, a soluble TNF receptor/Fc fusion protein. These agents are expensive and also have the side effects of tumour formation, sepsis and precipitating tuberculosis in susceptible individuals.
7. Anti-CD 20 agent—rituximab (selectively depletes CD 20+ B cells), for those not responding to anti-TNF therapy, usually given with methotrexate
8. T-cell modulator (costimulation modulator agent)—abatacept, for those not responding to anti-TNF therapy. Be cautious: in lung disease, it can worsen chronic obstructive pulmonary disease.
Combination therapy
Methotrexate can be given in combination with infliximab, etanercept or rituximab. Other effective combinations include methotrexate and leflunomide or methotrexate and sulfasalazine together with hydroxychloroquine. The latter triple combination is becoming popular.
SYSTEMIC SCLEROSIS
Case vignette
A 55-year-old woman with a background history of systemic sclerosis presents with progressive exertional dyspnoea of 1 month’s duration. She complains of significant debilitation recently—she can barely walk up a flight of stairs without experiencing significant dyspnoea. On examination there is evidence of sclerodactyly and pitting scars on her fingerpads. Tightness and cutaneous fibrosis is also evident in her face, with loss of skin creases and microstomia. There is a parasternal heave. On auscultation of the precordium there is a significant systolic murmur and a loud P2
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