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Rheumatoid vasculitis (RV) is a severe complication of rheumatoid arthritis (RA), which like a primary necrotising vasculitis can affect any organ but characteristically presents with painful cutaneous ulcers and systemic inflammation.1,2 Usually patients with refractory RA are affected who, therefore, had already undergone extensive immunosuppressive treatment. The prognosis is poor and most patients die from infectious complications, cardiac failure, or cerebral insult.3–6 Cyclophosphamide (CYC) is the preferred treatment but is often not well tolerated and does not contain the synovitis.3 We report the cases of three patients with RV who could not sufficiently be treated by CYC and steroids but responded very well to infliximab infusion therapy.
CASE REPORTS
Case 1
A 48 year old male patient with longlasting RA was admitted because of general malaise, dyspnoea, oedema, pleural and pericardial effusions, increased creatinine and liver enzymes, leucocytopenia, thrombocytopenia, decreased C4 and CH50, and increased levels of circulating immune complexes. The diagnosis of RV was established. The patient’s condition did not sufficiently improve after treatment with steroid pulses, high dose CYC, or even plasmapheresis. He developed a life threatening heart failure caused by a “swinging heart” due to the pericardial effusion, which required immediate and repeated drainage (fig 1). As a last resort we decided to start infliximab treatment at 3 mg/kg. After the first infusion his condition improved rapidly and the pericardial effusion, in particular, disappeared within two weeks. All laboratory findings returned to normal. He continues to receive infliximab every eight weeks and is in good clinical condition. Even the activity of his RA, previously not sufficiently controlled by methotrexate alone, has decreased significantly.
Case 1: chest computed tomographic scan showing the pericardial effusion due to RV.
Case 2
A 60 year old woman with a history of aggressive seropositive RA for 36 years suddenly developed painful ulcers on her left leg. The diagnosis of RV was established by biopsy from the ulcer rim. CYC bolus therapy was started and steroids also had to be increased. She responded partially to CYC, but the ulcers did not heal completely. Because of infections and leucopenia the dose and interval of the infusions often had to be adjusted. After 17 boli within 22 months the CYC therapy was stopped because of severe leucopenia. The lesions worsened after an ineffective trial of cyclosporin A (fig 2A). Infliximab was given at 3 mg /kg in weeks 0, 2, and 6 and thereafter every eight weeks. The ulcers improved soon after the second infusion and were completely healed after nine months (fig 2B). Her RA activity also improved greatly.
Case 2: leg ulcer before infliximab treatment was started (A) and the same lesion after seven infliximab infusions (B).
Case 3
A 64 year old male patient with RA was admitted to our hospital with a fistula between the colon and the urine bladder, which required immediate surgery because of imminent perforation. After surgery the wound did not heal. In addition, painful ulcers appeared on both legs, the scrotum, and other skin areas. All biopsies including that from the fistula, revealed necrotising vasculitis. CYC bolus therapy was started. The scrotal ulcer healed and the belly wound gradually improved, but the leg ulcers remained unchanged and his RA activity increased sharply. CYC had to be stopped because of leucopenia. Infliximab was started at 3 mg/kg at weeks 0, 2, and 6, and thereafter every eight weeks. The treatment was immediately effective for his synovitis and after a while the belly wound and the leg ulcers healed also.
CONCLUSION
These cases demonstrate a very rapid and sustained improvement not only of the vasculitic symptoms but also of the inflammatory joint activity, suggesting that infliximab may be a valuable alternative to standard CYC/steroid therapy for severe RV.