Longlasting effects of immunoadsorption in severe Sjögren's syndrome

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ls of the Rheumatic Diseases 2004;63:214-215

© 2004 by BMJ Publishing Group Ltd & European League Against Rheumatism

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LETTER

Longlasting effects of immunoadsorption in severe Sjögren's syndrome

M Böhm1, T Dörner2, F Knebel1, A Bruns2, N Jochmann1 and G Baumann1

1 Department of Medicine, Cardiology, Angiology and Pneumology, Charité

University Medical Centre, Berlin, Germany

2 Department of Medicine, Rheumatology and Clinical Immunology, Charité

University Medical Centre, Berlin, Germany

Correspondence to:

Dr M Böhm

Department of Medicine, Cardiology, Angiology, and Pneumology,

University Hospital Charité, Schumannstr 20/21, D-10098 Berlin, Germany;

marko.boehm@...

Accepted 22 May 2003

Keywords: immunoadsorption; autoimmunity; autoantibodies; Sjögren's

syndrome

Despite advances in understanding the immunopathogenesis of Sjögren's

syndrome, successful therapeutic interventions are extremely limited.

CASE REPORT

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.

DISCUSSION

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.

REFERENCES

1.. Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, EL,

Carsons SE, et al. Classification criteria for Sjögren's syndrome: a

revised version of the European criteria proposed by the

American-European Consensus Group. Ann Rheum Dis

2002;61:554-8.[Abstract/Free Full Text]

2.. Felix S, Staudt A, Dörffel WV, Stangl V, Merkel K, Pohl M, et al.

Hemodynamic effects of immunoadsorption and subsequent immunoglobulin

substitution in dilated cardiomyopathy: three-month results from a

randomized study. J Am Coll Cardiol 2000;35:1590-8.[CrossRef][Medline]

3.. Staudt A, Schaper F, Stangl V, Plagemann A, Böhm M, Merkel K, et

al. Immunohistological changes in dilated cardiomyopathy induced by

immunoadsorption therapy and subsequent immunoglobulin substitution.

Circulation 2001;103:2681-6.[Abstract/Free Full Text]

4.. Koll RA. Prinzip und Leistung der Ig-Therasorb®- Immunadsorption.

Klinische Immunadsorption 2000:23-25.

5.. Felson DT, JJ, Boers M, Bombardier C, Furst D, Goldsmith

C, et al. American College of Rheumatology. Preliminary definition of

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