Adjuvant treatment of severe/refractory bullous pemphigoid with protein A immunoadsorption.

BACKGROUND: While depletion of circulating autoantibodies using immunoadsorption (IA) is an established therapeutic approach in patients with pemphigus vulgaris, IA has only sporadically been used in other autoimmune bullous disorders. Although bullous pemphigoid (BP) usually responds well to topical and systemic corticosteroids, rapid depletion of serum autoantibodies may be an effective adjuvant treatment option in patients with severe and/or refractory disease. PATIENTS AND METHODS: Case series of 20 patients (13 women, 7 men; mean age 78.6 years; range 56-94 years) with severe or refractory BP. In addition to oral prednisolone (0.25-0.5 mg/kg/day), dapsone (1.0-1.5 mg/kg/day), and clobetasol propionate 0.05 % ointment (lesional application, twice daily), treatment consisted of protein A IA (three sessions on consecutive days). The mean follow-up period was 33.6 months (1-84 months). RESULTS: The majority of patients showed a rapid and sustained response. One month after treatment, eight patients (42 %; 19 patients were included in the follow-up) were in complete remission; at the last follow-up visit (after 1 to 84 months), that number was 13 (68 %). Not only was there an initial drop in anti-BP180 autoantibodies (by 92 %), the effect also continued after one and three months, with mean autoantibody levels at 26 % and 13 % of baseline, respectively (p < 0.001). Both previously treated and treatment-naive patients showed a significant reduction in anti-BP180NC16A antibody levels throughout the observation period. Adverse events occurred in 13 of the 20 patients (65 %). Three were severe of which two were likely or probably related to IA. CONCLUSION: Immunoadsorption is an effective adjuvant treatment option for (the usually elderly) patients with severe and/or refractory BP.

Adjuvante Behandlung des schweren/refraktären bullösen Pemphigoids mit Protein-A-Immunadsorption.

HINTERGRUND: Mittels Immunadsorption (IA) können Immunglobuline und Immunkomplexe aus dem Plasma entfernt werden. Während dieses therapeutische Verfahren beim Pemphigus vulgaris bereits etabliert ist, wird es bei anderen blasenbildenden Autoimmundermatosen bislang nur sporadisch eingesetzt. Das bullöse Pemphigoid (BP) spricht zwar meist gut auf eine Therapie mit topischen und systemischen Kortikosteroiden an, jedoch könnte bei Patienten mit ausgedehnten Läsionen oder bei einem Rezidiv die rasche Reduktion der pathogenen Autoantikörper eine effektive adjuvante Therapie darstellen. PATIENTEN UND METHODIK: Fallserie mit 20 Patienten (13 Frauen, 7 Männer; mittleres Alter 78,6 Jahre; 56-94 Jahre) mit schwerem oder refraktärem BP, die zusätzlich zur Basistherapie bestehend aus Prednisolon (0,25-0,5 mg/kg/d), Dapson (1,0-1,5 mg/kg/d) und Clobetasolpropionat 0,05 % Salbe (läsional 2 x/d) mit Protein-A-IA (3 IAs an aufeinander folgenden Tagen) behandelt wurden. Die durchschnittliche Nachbeobachtungszeit betrug 33,6 Monate (1-84 Monate). ERGEBNISSE: Bei der Mehrzahl der Patienten zeigte sich ein rascher und langandauernder Therapieeffekt. Nach einem Monat befanden sich acht Patienten (von 19 nachbeobachteten, 42 %) und zum Zeitpunkt des letzten Kontakts (nach 1-84 Monaten) 13 Patienten (68 %) in kompletter Remission. Die Anti-BP180-Autoantikörper wurden nicht nur initial (um 92 %) gesenkt, sondern lagen auch nach ein und drei Monaten im Mittel bei 26 % und 13 % des Wertes vor Therapiebeginn (p < 0,001). Bei Differenzierung in vortherapierte bzw. therapienaive Patienten zeigten sich in beiden Subgruppen signifikante Absenkungen der Anti-BP180NC16A-Antikörper-Spiegel zu allen Zeitpunkten. Unerwünschte Ereignisse traten bei dreizehn (65 %) der 20 Patienten auf, Drei der Ereignisse waren schwer und zwei davon wahrscheinlich oder möglicherweise in Zusammenhang mit der IA. SCHLUSSFOLGERUNGEN: Die IA ist eine effektive adjuvante Therapieoption bei den in aller Regel älteren Patienten mit einem schweren und/oder therapierefraktären BP.

IgE-Selective Immunoadsorption for Severe Atopic Dermatitis.

INTRODUCTION: Recent reports proposed the application of immunoadsorption (IA) for patients with recalcitrant atopic dermatitis (AD) and high-serum IgE levels. However, experience with this novel treatment approach, especially with the newly available IgE-specific adsorber, is limited and recommendation for its use in clinical practice awaits evidence from more studies. MATERIALS AND METHODS: Patients with severe AD (SCORAD ≥ 60) and total serum IgE levels ≥750 kU/L were included in this study. The treatment protocol consisted of two cycles of five consecutive treatments with IgE-selective IA 3 weeks apart. RESULTS: Ten patients were enrolled and four patients completed the study. The mean SCORAD was significantly improved by up to 43% within a few weeks and until the end of a 6-month follow-up period, with 50% of patients achieving an at least 50% individual reduction of the baseline SCORAD. Each IA cycle induced a temporal average decrement of total serum levels of IgE, IgM, IgA, and IgG by 92, 43, 38, and 35%, respectively. Except for one case of Staphylococcus aureus septicemia, no major adverse events occurred. CONCLUSION: Although limited by a considerable withdrawal rate, our observations strengthen our and other recent results further suggesting that IgE-selective IA is an effective treatment option for patients severely affected by AD with highly elevated IgE levels.

Diagnostics of autoimmune bullous diseases in German dermatology departments.

BACKGROUND: No consistent data are available on the currently employed diagnostic tools for autoimmune bullous diseases in Germany. The aim of this survey was to describe currently performed diagnostic methods for bullous autoimmune diseases in German dermatology departments. METHODS: A standardized questionnaire evaluated the available diagnostic methods i. e. direct immunofluorescence microscopy (IFM), indirect IFM, commercial ELISA systems, and non-commercial serological tests as well as the number of samples per year in all 34 university and 39 non-university dermatology departments. RESULTS: The overall return rate was 89 %, 100 % and 79 % for the university and non-university departments, respectively. Direct IFM was the most frequently used method and was applied in 98 % of the responding departments. In 74 % of the responding departments, indirect IFM was used mainly on monkey esophagus and human salt-split skin. Commercial ELISA systems were employed in 58 % of the clinics; all of them used anti-desmoglein ELISA, while anti-BP180 and anti-BP230 ELISA were established in 49 % and 48 % of departments, respectively. Non-commercial analytic methods were only performed in 22 % of the departments. CONCLUSIONS: The high return rate of this survey allows a relatively precise description of the current diagnostic methods used in German dermatology departments. Standard diagnostic tests are available nationwide and in bullous pemphigoid and pemphigus, the antigen-specific detection of autoantibodies is routinely performed in half of the departments. Rare disorders may be diagnosed by cooperation with some specialized centers.