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Despite advances in understanding the immunopathogenesis of Sjögren’s syndrome, successful therapeutic interventions are extremely limited.
With this in mind we began a immunoadsorption treatment of a 38 year old woman, diagnosed 1.5 years ago with Sjögren’s syndrome. She reported dry eyes and mouth for several months and intermittent arthralgias, especially of the small finger joints and elbows, as well as swollen joints of the hands, elbows, and ankles for six years. During the six months before treatment her joint symptoms had increased significantly and considerably impaired everyday activities. A Schirmer’s test was positive. The antinuclear antibodies and rheumatoid factor were raised, autoantibodies against Ro/SSA and La/SSB were positive. Thus, the patient fulfilled four of six revised criteria of primary Sjögren’s syndrome.1
Previous corticoid treatment (prednisolone 20 mg a day for four weeks) had led to oropharyngeal candida mycosis, and methotrexate (25 mg a week) did not have a therapeutic effect. Because chloroquine had side effects in her family, the patient refused to take hydroxychloroquine. Upon presentation in our clinic, the patient took 5 mg a day of prednisolone. Because the severe arthralgias and sicca symptoms did not respond to conventional treatment we started to treat the patient with immunoadsorption, to improve the symptoms by reducing IgG to 10–20% of its initial level.
Approval of the ethics committee and informed consent by the patient were obtained, and we started immunoadsorption therapy according to a previous protocol used for patients with dilated cardiomyopathy.2,3
Immunoadsorption treatment took place in two consecutive cycles with an interval of four weeks. The first cycle comprised three treatment days and the second, two. The plasma filtration was similar to haemodialysis. The IgG-Therasorb Adsorber and a Mirosorb treatment unit (Plasmaselect, Teterow, Germany) treated 7 litres plasma a day. Plasma IgG, antibody complexes, and fragments of antibodies were bound to the Fc fragment of polyclonal sheep antihuman antibodies, bound in turn to Sepharose.4
After the first treatment cycle, the patient showed remarkable clinical improvement, with lessening of arthalgias and articular swelling, and subsequent increased joint mobility. The score for tender/swollen joints5 reduced from a value of 29 at the beginning to 0 at the end of the study. Complement factors C3 and C4 fell to 67% and 78% of baseline (0.8–0.6 g/l and 0.19–0.15 g/l), respectively. In addition, circulating immune complexes reduced to 29% (3.3–1.0 g/l) and the rheumatoid factor to 55% of initial value (2.5–1.4 g/l). The treatment reduced IgG from 24.31 to 4.88 g/l after the first cycle and from 22.72 to 10.04 g/l after the second cycle. The IgG level increased within 16 months to 34.70 g/l.
The changes during the second treatment cycle were less striking in their effect on circulating immune complexes (reduced to 53% (1.55–0.83 g/l)) and the rheumatoid factor (reduced to 92% of the initial level (6.56–6.09 g/l)). Increases were seen in the values of circulating immune complexes (from 0.83 to 0.96 g/l) and of rheumatoid factor (from 6.09 to 6.24 g/l) after 16 months.
As a result of the striking and sustained clinical benefit achieved, the treatment was discontinued after the second cycle of immunoadsorption.
To prevent a rebound and infection after immunoadsorption, the treatment protocol requires intravenous IgG substitution (0.5 g per kg body weight Venimmun) after each cycle, indicating that the patient’s IgG has a significant role in the disease. We cannot exclude the possibility that immunoglobulin infusion may have had some effect on the outcome. This needs to be examined by additional studies.
This case report suggests the potential advantages of immunoadsorption in severe Sjögren’s syndrome which is refractory to conservative treatment. After 16 months the patient remains free of tender and swollen joints, and the sicca symptoms remain stable. A current open trial will provide further data, allowing better assessment of the value of immunoadsorption in patients with Sjögren’s disease.