Background Chronic urticaria is a frequent and debilitating skin disease. Its symptoms commonly fluctuate considerably from day to day. As of yet, the only reliable tool to assess disease activity is ...the Urticaria Activity Score, which prospectively documents the signs and symptoms of urticaria for several days. Objective We sought to develop and validate a novel patient-reported outcome instrument to retrospectively assess urticaria control, the Urticaria Control Test (UCT). Methods Potential UCT items were developed by using established methods (literature research and expert and patient involvement). Subsequently, item reduction was performed by using a combined approach, applying impact and regression analysis. The resulting UCT instrument was then tested for its validity, reliability, and screening accuracy. Results A 4-item UCT with a recall period of 4 weeks was developed based on 25 potential UCT items tested in 508 patients with chronic urticaria. A subsequent validation study with the 4-item UCT in 120 patients with chronic urticaria demonstrated that this new tool exhibits good convergent and known-groups validity, as well as excellent test-retest reliability. In addition, the screening accuracy to identify patients with urticaria with insufficiently controlled disease was found to be high. Conclusions The UCT is the first valid and reliable tool to assess disease control in patients with chronic urticaria (spontaneous and inducible). Its retrospective approach and simple scoring system make it an ideal instrument for the management of patients with chronic urticaria in clinical practice.
Background Schnitzler syndrome is an adult-onset autoinflammatory disease characterized by urticarial exanthema and monoclonal gammopathy accompanied by systemic symptoms such as fever, bone, and ...muscle pain. Up to now, approved treatment options are not available. Objective We assessed effects of the anti–IL-1β mAb canakinumab on the clinical signs and symptoms of Schnitzler syndrome. Methods In this phase II, randomized placebo-controlled multicenter study, 20 patients with active disease enrolled in 4 German study centers. Patients were randomly assigned to receive single subcutaneous canakinumab 150 mg or placebo injections for 7 days, followed by a 16-week open-label phase with canakinumab injections on confirmed relapse of symptoms. The primary end point was the proportion of patients with complete clinical response evaluated by physician global assessment at day 7. Key secondary end points included changes in patient-reported disease activity (Schnitzler activity score), inflammation markers (C-reactive protein and serum amyloid A), and quality-of-life assessments (Dermatology Life Quality Index and 36-item short form health survey). Results The proportion of patients with complete clinical response at day 7 was significantly higher ( P = .001) in the canakinumab-treated group (n = 5 of 7) than in the placebo group (n = 0 of 13). Levels of inflammation markers C-reactive protein and serum amyloid A and quality-of-life scores were significantly reduced in canakinumab-treated but not in placebo-treated individuals. Positive effects continued up to 16 weeks. Adverse events were manageable and included respiratory tract infections, gastrointestinal symptoms, and hypertension. Conclusions In this first placebo-controlled study, canakinumab was effective in patients with Schnitzler syndrome, and thus canakinumab may be further evaluated as a therapeutic option for this rare disease.
Secondary end points included changes in the physician and patient global assessment of disease activity (10-point visual analog scale VAS) and changes in inflammation markers C-reactive protein ...(CRP) and erythrocyte sedimentation rate (ESR) during the 16 weeks following canakinumab treatment as compared to baseline. Methods Eligibility criteria To be included in the study, patients had to fulfill the following diagnostic criteria of UV: (1) urticarial rash with individual lesions persisting for more than 24 hours and (2) UV confirmed by biopsy from lesional skin showing signs of leukocytoclasis (fragmentation of neutrophils with nuclear dust) and/or fibrinoid deposits in perivascular and interstitial locations.E1 Participation in the study required active UV and insufficient response to treatment with antihistamines, nonsteroidal anti-inflammatory drugs, and/or immunomodulating or immunosuppressive drugs.
...anti-IL-1 treatment has been reported to be effective in all cases, without a single treatment failure.4-7 Here, we report 3 cases of SchS that did not respond to anti-IL-1 and multiple other ...treatments but showed rapid and complete remission after initiation of anti-IL-6 treatment with tocilizumab. Surprisingly, anakinra treatment (100 mg/d) also did not result in any improvement in the symptoms or in the reduction of markers of inflammation, and anakinra was discontinued after 8 weeks. Since anti-IL-1 treatment had been reported to be successful in all patients with SchS ever treated, the patient then received rilonacept (loading dose 320 mg, followed by weekly doses of 160 mg), another IL-1-targeted therapy.
Abstract Rare systemic autoinflammatory diseases (sAIDs) are driven by cytokine-mediated uncontrolled inflammation that results from activation of innate immune pathways. sAIDs present with recurrent ...fever episodes, fatigue, musculoskeletal symptoms, gastrointestinal, neurologic, and skin manifestations. They include hereditary monogenic and acquired multifactorial disorders, show a significant morbidity and usually persist for life. Due to the limited awareness of sAIDs, they are often associated with a considerable delay in diagnosis. Within the last decade, the use of cytokine-neutralizing therapies has been shown to improve the clinical symptoms of many patients with different sAIDs. Because skin involvement, such as urticarial, pustular, or ulcerative eruptions, is common in a variety of autoinflammatory disorders, dermatologists should be aware of the most important diseases and their skin phenotypes. This review gives an overview on prototype sAIDs with focus on cutaneous manifestations, clinical clues, and diagnostic approaches. Effective treatment options, such as anti–interleukin-1–targeted therapies, are discussed.
To the Editor: Chronic urticaria is a severe skin disease characterized by itchy wheals and/or angioedema1,2 with an estimated lifetime prevalence of 3% to 5% in the general population.3,4 The ...current European Academy of Allergy and Clinical Immunology (EAACI)/Global Allergy and Asthma European Network (GA2LEN)/European Dermatology Forum (EDF)/World Allergy Organization (WAO) treatment guidelines recommend nonsedating H1-antihistamines as first-line treatment, increasing their dosage as second-line treatment, and switching to another such antihistamine or adding a leukotriene antagonist as the third-line treatment.5 Thereafter, among several other fourth-line options including cyclosporine A,6 the guidelines suggest the possibility of using omalizumab, a humanized mAb that blocks the IgE receptor.7 Since September 2008, our specialized university clinic has used omalizumab (Xolair; Genentech & Novartis, San Francisco & Basel) to treat 8 patients with chronic spontaneous urticaria patients (Table I) who had not benefited from the recommended first-line, second-line, and third-line treatments.
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