Early presentation
Around 400,000 people in the UK are thought to have rheumatoid arthritis (RA), with 10,000 new cases presenting each year. RA occurs most commonly in the over-70s. The importance of early recognition and urgent referral for specialist investigation and treatment is recognised. Any patient with a persistent synovitis of unknown cause in the small joints of the hands and feet with symmetrical swelling and stiffness of the interphalangeal and metacarpophalangeal joints of the hand, should be referred.
Long-term consequences
Long-term consequences are potentially severe, painful and disabling. About one third of patients with RA have to give up work within two years of its development. This patient, with long standing disease, developed the complication of swan neck deformity, in which there is hyperextension at the proximal interphalangeal joint and a fixed flexion deformity at the terminal interphalangeal joint. Treatment includes physiotherapy with stretching, massage and mobilisation of the joints, splints or surgery.
Complications
Advise patients to seek help in the event of any flare up, complication or progressive deformity. This patient developed RA 30 years ago; now aged 62, her activity is limited by deformities in her feet - bilateral hallux valgus and clawed toes, which occur due to contraction of the muscles. Walking is hampered and it is difficult to find suitable shoes; corns and calluses are likely in traumatised areas. Early treatment for clawed toes offers a better chance of straightening them. Surgery with arthroplasty or arthrodesis may be the only option once the soft tissues that fasten the claw have tightened.
Rheumatoid vasculitis
Rheumatoid vasculitis occurs in about 1% of patients with severe, long standing RA. It involves small and medium sized arteries and may manifest in a number of ways, for example, scleritis, cutaneous ulceration or, as in this patient, small nail infarcts. Note that nail infarcts are not necessarily a sign of systemic vasculitis and will not indicate any need to change the treatment regimen. Recent evidence suggests that incidence of rheumatoid vasculitis has decreased and this may be due to better, earlier treatment of RA.
Investigations
Although the most common presentation is in the hands, other joints may be affected, as in this patient with painful swelling of the wrist. Shoulders, hips, elbows, knees, ankles, midtarsal joints and the upper cervical spine can also be affected. In patients with synovitis and possible RA, blood should be tested for rheumatoid factor. If negative, the anti-cyclic citrullinated peptide test should be performed as it is more specific for RA and will indicate the need for combination therapy. X-ray of those with persistent symptoms should be performed in the early stages of the disease.
Treatment
In the days when effective treatment for RA was less available, this patient had required bilateral knee replacements. Now, RA may be treated with DMARDs including methotrexate and at least one other (for example, penicillamine, gold, chloroquine, sulfasalazine), and short-term glucocorticoid therapy. Ideally, this should be started within three months of persistent symptoms. It is vital to quickly reach an effective dose and to monitor it while control is achieved; then, it may be possible to reduce the dose, with care, to lower maintenance levels.
Rheumatoid nodules
These occur in 20-35% of patients with RA, most often on pressure points, such as over the extensor surface adjacent to joints including the elbow, fingers and heel. Nodules may be 2-5cm in diameter, are firm and mobile but not tender. Specific treatment is not necessary unless nodules ulcerate, cause pain or compressive neuropathies, interfere with function, or the patient is concerned about appearance. Steroid injection into the lesion may reduce its size or surgical removal can be done, although recurrence is quite common.
Pyoderma gangrenosum
This is an uncommon problem, where the most typical presentation is a deep ulcer, usually on the leg, with a violaceous border that overhangs the ulcer bed. Less typically there is a more superficial ulceration, usually on the back of the hand, extensor surface of the forearm or the face. Pyoderma gangrenosum is also seen in association with ulcerative colitis, Crohn's disease and multiple myeloma. Treatment relies on the most effective therapy for the underlying disease, and care of the wound, which might include topical and systemic treatments. Surgery is best avoided.