Supplementary Material for: Successful Treatment of Autoimmune Urticaria with Low-Dose Prednisolone Therapy Administered for a Few Months: A Case Series of 42 Patients

<p><b><i>Background:</i></b> Chronic spontaneous urticaria (CSU) is defined as symptoms of urticaria persisting for 6 weeks or more without obvious cause. Autologous serum skin test (ASST) positivity in patients with CSU is considered to be associated with autoimmune urticaria (AIU). <b><i>Methods:</i></b> In this retrospective study we retrieved the medical records of 1,073 urticaria patients seen at the Department of Dermatology and Allergology of Szeged University between January 2005 and February 2014. Forty-two patients (36 female and 6 male) met the study criteria by having CSU and giving positive results in the ASST. Our aim was to assess the clinical efficacy and safety of low-dose oral prednisolone therapy administered to patients with antihistamine-refractory ASST-positive CSU for a few months. Patients were given an initial dose (40 mg/day) of prednisolone until the complete resolution of the symptoms, usually 7-10 days, and then the dose was gradually decreased, as in other autoimmune diseases. <b><i>Results:</i></b> Prednisolone therapy lasted for an average of 3.6 months and a complete long-lasting response was achieved in 35 of 42 AIU patients (83.3%). The follow-up period was at least 36 months (3 years) for each AIU patient; the longest follow-up time was 139 months (11.5 years). None of the patients reported any considerable side effects. <b><i>Conclusion:</i></b> Based on our results, we suggest that the use of this treatment could be an alternative for the treatment of AIU. Our present results also highlight the need for other therapies in a small percentage of AIU patients. Our results suggest that AIU represents a transient autoimmunity that can be successfully treated with low-dose steroid therapy administered for a few months.</p

Treatment of pemphigus vulgaris and foliaceus with efgartigimod, a neonatal Fc receptor inhibitor: a phase II multicentre, open-label feasibility trial

Background Pemphigus vulgaris and pemphigus foliaceus are potentially life-threatening autoimmune disorders triggered by IgG autoantibodies against mucosal and epidermal desmogleins. There is an unmet need for fast-acting drugs that enable patients to achieve early sustained remission with reduced corticosteroid reliance. Objectives To investigate efgartigimod, an engineered Fc fragment that inhibits the activity of the neonatal Fc receptor, thereby reducing serum IgG levels, for treating pemphigus. Methods Thirty-four patients with mild-to-moderate pemphigus vulgaris or foliaceus were enrolled in an open-label phase II adaptive trial. In sequential cohorts, efgartigimod was dosed at 10 or 25 mg kg$^{-1}$ intravenously with various dosing frequencies, as monotherapy or as add-on therapy to low-dose oral prednisone. Safety endpoints comprised the primary outcome. The study is registered at ClinicalTrials.gov (identifier NCT03334058). Results Adverse events were mostly mild and were reported by 16 of 19 (84%) patients receiving efgartigimod 10 mg kg$^{-1}$ and 13 of 15 (87%) patients receiving 25 mg kg−1, with similar adverse event profiles between dose groups. A major decrease in serum total IgG and anti-desmoglein autoantibodies was observed and correlated with improved Pemphigus Disease Area Index scores. Efgartigimod, as monotherapy or combined with prednisone, demonstrated early disease control in 28 of 31 (90%) patients after a median of 17 days. Optimized, prolonged treatment with efgartigimod in combination with a median dose of prednisone 0·26 mg $^{-1}$ per day (range 0·06–0·48) led to complete clinical remission in 14 of 22 (64%) patients within 2–41 weeks. Conclusions Efgartigimod was well tolerated and exhibited an early effect on disease activity and outcome parameters, providing support for further evaluation as a therapy for pemphigus

S2k guideline for treatment of cutaneous lupus erythematosus - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV).

Cutaneous lupus erythematosus (CLE) is a rare inflammatory autoimmune disease with heterogeneous clinical manifestations. To date, no therapeutic agents have been licensed specifically for patients with this disease entity, and topical and systemic drugs are mostly used 'off-label'. The aim of the present guideline was to achieve a broad consensus on treatment strategies for patients with CLE by a European subcommittee, guided by the European Dermatology Forum (EDF) and supported by the European Academy of Dermatology and Venereology (EADV). In total, 16 European participants were included in this project and agreed on all recommendations. Topical corticosteroids remain the mainstay of treatment for localized CLE, and further topical agents, such as calcineurin inhibitors, are listed as alternative first-line or second-line topical therapeutic option. Antimalarials are recommended as first-line and long-term systemic treatment in all CLE patients with severe and/or widespread skin lesions, particularly in patients with a high risk of scarring and/or the development of systemic disease. In addition to antimalarials, systemic corticosteroids are recommended as first-line treatment in highly active and/or severe CLE. Second- and third-line systemic treatments include methotrexate, retinoids, dapsone and mycophenolate mofetil or mycophenolate acid, respectively. Thalidomide should only be used in selected therapy-refractory CLE patients, preferably in addition to antimalarials. Several new therapeutic options, such as B-cell- or interferon α-targeted agents, need to be further evaluated in clinical trials to assess their efficacy and safety in the treatment of patients with CLE