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[Adult onset Still's disease with small vessel vasculitis]
P Hoff1, B F Hoyer, U Schneider
1Med. Klinik m. S. Rheumatologie und Klinische Immunologie, Charité Universitätsmedizin, Charitéplatz 1, 10117, Berlin, Deutschland, paula.hoff@charite.de.
Zeitschrift Fur Rheumatologie
|August 2, 2015
Summary
Adult onset Still's disease with small vessel vasculitis is severe but treatable. Tocilizumab combined with IVIG achieved remission in a difficult-to-treat case, showing promise for managing this rare condition.
Area of Science:
- Rheumatology
- Internal Medicine
- Immunology
Background:
- Adult onset Still's disease (AOSD) is a rare systemic inflammatory disorder.
- Small vessel vasculitis can be a severe complication of AOSD, posing treatment challenges.
Observation:
- This case details a severe presentation of AOSD with refractory small vessel vasculitis.
- Conventional immunosuppressants including methotrexate, cyclophosphamide, and rituximab showed limited efficacy.
Findings:
- Tocilizumab, an IL-6 receptor inhibitor, in combination with intravenous immunoglobulin (IVIG) successfully induced remission.
- Maintenance therapy with tocilizumab alone sustained the remission for the observed period.
Implications:
- This case highlights tocilizumab and IVIG as a potential therapeutic strategy for severe AOSD with vasculitis.
- Effective management of refractory AOSD-associated vasculitis may require targeted biologic therapies.
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