Anti-TNF therapy can have a lasting beneficial effect on the course of AOSD. Five of eight patients remained in remission even after termination of therapy.

Induction of factor VIII (FVIII) inhibitors sometimes occurs in patients with hemophilia due to frequent supplementation of FVIII. The inhibitor is rarely detected in non-hemophilic patients; however, an association has been described in patients with chronic inflammatory diseases, such as autoimmune diseases (e.g. SLE and rheumatoid arthritis), malignant tumors and drug allergies, and also to pregnant or aged individuals without underlying disease. We report on an 82-year-old patient who was transferred to our hospital after the diagnosis of acquired FVIII inhibitor. On admission the laboratory results showed no detectable FVIII activity (0%, normal range 70-100%), prolongation of coagulation time (APTT 102.4 s), and severe anemia 7.8 g/dL (normal range 12-16 g/dL). On physical examination multiple subcutaneous hematomas were detected and further bleeding in his left pectoralis muscle was observed. Despite extensive investigation no underlying disease was detected. Thirty-six courses of plasma exchange with 360 units of fresh frozen plasma replacement daily were conducted. High dose steroids and mycophenolate mofetil (MMF) were given throughout the course, and cyclophosphamide was administered once. Thirty-four units of erythrocytes were applied during this time. After 36 courses of plasma exchange in combination with high dose steroids, FVIII activity and coagulation time normalized and bleeding could be stopped. The patient was discharged in good health 48 days after admission. Hence, we present a case of severe idiopathic FVIII inhibitor-positive hemophilia successfully treated with the combination of plasma exchange, corticosteroids, cyclophosphamide and MMF.

Objective.To analyze the effect of a risk-stratified disease-modifying antirheumatic drug (DMARD)–tapering algorithm based on multibiomarker disease activity (MBDA) score and anticitrullinated protein antibodies (ACPA) on direct treatment costs for patients with rheumatoid arthritis (RA) in sustained remission.Methods.The study was a posthoc retrospective analysis of direct treatment costs for 146 patients with RA in sustained remission tapering and stopping DMARD treatment, in the prospective randomized RETRO study. MBDA scores and ACPA status were determined in baseline samples of patients continuing DMARD (arm 1), tapering their dose by 50% (arm 2), or stopping after tapering (arm 3). Patients were followed over 1 year, and direct treatment costs were evaluated every 3 months. MBDA and ACPA status were used as predictors creating a risk-stratified tapering algorithm based on relapse rates.Results.RA patients with a low MBDA score (< 30 units) and negative ACPA showed the lowest relapse risk (19%), while double-positive patients showed high relapse risk (61%). In ACPA-negative and MBDA-negative (< 30 units), and ACPA or MBDA single-positive (> 30 units) groups, DMARD tapering appears feasible. Considering only patients without flare, direct costs for synthetic and biologic DMARD in the ACPA/MBDA-negative and single positive groups (n = 41) would have been €372,245.16 for full-dose treatment over 1 year. Tapering and stopping DMARD in this low-risk relapse group allowed a reduction of €219,712.03 of DMARD costs. Average reduction of DMARD costs per patient was €5358.83.Conclusion.Combining MBDA score and ACPA status at baseline may allow risk stratification for successful DMARD tapering and cost-effective use of biologic DMARD in patients in deep remission as defined by the 28-joint count Disease Activity Score using erythrocyte sedimentation rate.

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Vor 10 Jahren wurden erstmals Berichte veröffentlicht, wonach intravenös verabreichte Immunglobuline (ivig) bei einer durch Autoantikörper vermittelten Erkrankung, der idiopathischen Thrombozytopenie, erfolgreich eingesetzt wurden. Seither kamen ivig im Rahmen von Pilotstudien bei nahezu alien Autoimmunopathien zur Anwendung. Als anerkannte Therapie gelten ivig heute jedoch nur bei der idiopathischen Thrombozytopenie und beim Kawasaki-Syndrom. In den letzten Jahren wurde von verschiedenen Arbeitsgruppen viel Mühe aufgewendet, die möglichen Wirkmechanismen einer ivIg-Therapie besser zu verstehen. Fallbeschreibungen über die Behandlung des systemischen Lupus erythematodes (SLE) berichten fast immer über positive Erfahrungen mit der ivIg-Therapie, insbesondere bei Patienten mit Zytopenien und kutaner Vaskulitis. Andererseits häufen sich auch Mitteilungen über ernsthafte Nebenwirkungen der ivIg-Therapie bei Patienten mit SLE und Nierenbeteiligung. Wir behandelten bislang 6 SLE-Patienten mit einer hochdosierten ivIg-Therapie. Bei 1 Patientin stellte sich eine 6 Monate, bei einer anderen eine bislang 3 Jahre anhaltende Remission ein; bei 2 Patientinnen beobachteten wir direkt nach Beendigung der Therapie ein akutes Nierenversagen; bei einer weiteren Patientin verschlechterte sich die Nierenfunktion nach 1 Monat. Diese vorläufigen Daten unter-streichen die Notwendigkeit kontrollierter Studien zum Nachweis einer klinischen Wirksamkeit, zur Eingrenzung der Indikation und zur Dosisfindung sowie zur Unter-suchung der möglichen Wirkmechanismen. Es ist zu hoffen, daβ die weitere Erforschung der möglichen Wirkmechanismen bei Autoimmunerkrankungen in der Zukunft zu Behandlungsansätzen führen wird, die sowohl zytotoxische als auch immunsuppressive durch immunmodulatorische Therapien ersetzen und zum Verständnis des Übergangs von physiologischer Autoreaktivität zu pathologischer Autoimmunität beitragen.