H G Kraetsch, C Antoni, J R
Kalden, B Manger
Department for Internal Medicine III with Institute for Clinical
Immunology,
Friedrich-Alexander University of Erlangen, Nuremberg, Germany
Correspondence to: Dr H Kraetsch, Department of Internal Medicine III
with
Institute for Clinical Immunology, Krankenhausstr 12, D-91054 Erlangen,
Germany
hans-georg.kraetsch@med3.imed.uni-erlangen.de
Accepted for publication 28 June 2001
OBJECTIVE To test the efficacy of infliximab in the treatment of
patients
with severe and active adult onset Still's syndrome (AOSD) despite
conventional immunosuppressive therapy.
PATIENTS AND METHODS Six patients with the diagnosis of AOSD according
to the Yamagushi criteria of 1992 were treated with infliximab. All
patients had
severe disease with high clinical and serological activity. Patients
were
treated initially with high dose steroids or more intensive
immunosuppressive therapy.
Two patients had a history
of multiple disease
modifying antirheumatic drug (DMARD) treatments. One patient had a
history
of three years of AOSD with fever, chills, pleural and pericardial
effusions, and hepatosplenomegaly. Despite these treatments, he
developed
increasing serological signs of inflammation and arthritis of both hips
and
peripheral joints. Another patient had a history of five years of AOSD
with
oligoarthritis, myalgias, and recurrent fever despite multiple DMARD
treatment, including cyclophosphamide pulse therapy. Our patients with
AOSD
presented with massive polyarthralgias, polyarthritis, splenomegaly or
hepatomegaly, the typical rash, sore throat, weight loss, serositis,
continuing fever, leucocytosis, and raised C reactive protein (CRP),
erythrocyte sedimentation rate (ESR), and ferritin levels. Four patients
with early onset of the disease, fulfilling the diagnostic criteria for
AOSD
and a clinical and serological high disease activity, were included in
our
pilot study without any further DMARD treatment apart from the initial
steroid treatment. Reduction of established treatment, mainly with
steroids,
caused a relapse of the disease in all our patients. Patients then
received
3-5 mg/kg infliximab on weeks 0, 2, and 6, continuing with intervals of
6-8
weeks depending on the patient's individual disease activity.
RESULTS In all patients, fever, arthralgias, myalgias,
hepatosplenomegaly,
and the rash resolved after the first courses of treatment with
infliximab.
All serological variables (CRP, ESR, hyperferritinaemia) returned to
normal.
After three courses of infliximab infusions, splenomegaly could not be
detected in any of our patients. One patient still had severe pain in
the
left hip caused by hip postarthritic osteoarthrosis, requiring hip
replacement. After three courses of treatment with infliximab,
splenomegaly
could not be detected in any of our patients. Up to now, our patients
have
received infliximab infusion treatment for between five and 28 months.
Throughout this period all patients have continued to benefit from this
treatment, with improvement in their clinical symptoms, joint counts,
and
serological disease activity. One of our patients had a moderate
infusion
reaction during the second treatment. The infusion was discontinued for
one
hour and then was resumed with no further problems.
CONCLUSION The disease improved remarkably in all six patients with AOSD
after treatment with infliximab, also in the early stage of AOSD. These
preliminary data suggest the potential therapeutic benefit of anti-tumour
necrosis factor treatment in AOSD.
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