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A state of confusion

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Q1: What is illustrated in fig 1 (see p 666)?

Scleromalacia—this condition results from severe scleritis and can lead to permanent localised thinning of the sclera, which develops a blue tinge. The sclera may go on to perforate—scleromalacia perforans; this complication results in blindness.

Q2: What is the skin lesion illustrated in fig 2 (see p 666)?

This is a vasculitic ulcer of the lower leg. It is most likely to be caused by rheumatoid arthritis in this patient, and is relatively uncommon in this condition.

Q3: What is the unifying diagnosis?

This patient had rheumatoid vasculitis, manifesting with mononeuritis multiplex of the left ulnar nerve, larger vessel involvement in the form of leg ulceration, and multiple areas of cerebral ischaemic change that resulted in the clinical presentation of a stroke. The scleromalacia affecting his eye was also another extra-articular manifestation of rheumatoid arthritis. Involvement of the larger arteries, in this case, the brain, is seen rarely. In this patient, a lumbar puncture revealed raised cerebrospinal fluid protein. His erythrocyte sedimentation rate and C reactive protein were also high, all indicators of active rheumatoid disease.

Q4: What treatment would you now offer the patient?

Aggressive treatment with cyclophosphamide is often required for the treatment of rheumatoid vasculitis involving medium to large sized vessels. Cyclophosphamide may be administered as a daily oral dose or an intermittent intravenous bolus infusion. Concomitant treatment with moderate doses of prednisolone are also sometimes required. The length of treatment is usually approximately six months, although some patients require maintenance therapy for years. Antibiotic treatment should be given for cutaneous infections. Foot and wrist drop are treated with splints.

Q5: What is his prognosis?

Prognosis depends on the organ system involved (see table 1). Patients with only limited cutaneous fingertip lesions generally do well. Involvement of major nerves or arteries in organs such as the heart are associated with a poorer prognosis.

Table 1

Range of organ involvement in rheumatoid vasculitis

Discussion

Fourteen previous cases have been reported in the literature of cerebral infarction complicating rheumatoid vasculitis. These cases have mostly been described as showing multiple large vessel infarctions in the cerebral hemisphere. Other large vessel involvement in rheumatoid vasculitis can result in infarction of the myocardium, bowel, lungs and very rarely, the kidneys.1 A diagnosis of rheumatoid vasculitis should be considered even if the patient does not have severe seropositive rheumatoid arthritis. The diagnosis of rheumatoid arthritis is often longstanding. Rheumatoid vasculitis is commoner in smokers. In cases of specific organ or tissue involvement such as a mononeuritis syndrome biopsy of the affected tissue (in this case the nerve) can be very useful in establishing the diagnosis.2 The aetiology of this condition lies in immune complexes, which probably contain rheumatoid factor. Immune complexes and are deposited in the walls of blood vessels. All patients with rheumatoid vasculitis have strongly positive titres for IgM rheumatoid factor. Most of the rheumatoid factor containing complexes can fix complement, which contributes to tissue damage.

Final diagnosis

Rheumatoid vasculitis.

References

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