Maurer, M. (Marcus)

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    Autoimmune diseases are linked to type IIb autoimmune chronic spontaneous urticaria
    (Korean Academy of Asthma, Allergy and Clinical Immunology, Korean Academy of Pediatric Allergy and Respiratory Disease, 2021) Altrichter, S. (Sabine); Sussman, G. (Gordon); Kinaciyan, T. (Tamar); Makris, M. (Michael); Zhang, K. (Ke); Asero, R. (Riccardo); Daschner, A. (Alvaro); Kolkhir, P. (Pavel); Metz, M. (Martin); Kromminga, A. (Arno); Skov, P.S. (Per Stahl); Jakob, T. (Thilo); Konstantinou, G.N. (George N.); Maurer, M. (Marcus); Hawro, T. (Tomasz); Gimenez-Arnau, A. (Ana); Staubach, P. (Petra); Ferrer-Cardona, M. (Marta)
    Purpose: Patients with chronic spontaneous urticaria (CSU) have an increased risk for comorbid autoimmune diseases. In this retrospective multicenter study of CSU patients, we evaluated clinical and laboratory features of CSU associated with a higher risk of comorbid autoimmune diseases. Methods: We analyzed records of CSU patients (n = 1,199) for a history or presence of autoimmune diseases. Patients were diagnosed with type IIb autoimmune CSU (aiCSU) if all 3 tests were positive: autologous serum skin test (ASST), basophil histamine release assay (BHRA) and/or basophil activation test (BAT), and IgG autoantibodies against FcεRIα/IgE detected by immunoassay. Results: Twenty-eight percent of CSU patients had at least 1 autoimmune disease. The most prevalent autoimmune diseases were Hashimoto's thyroiditis (HT) (≥ 21%) and vitiligo (2%). Two percent of CSU patients had ≥ 2 autoimmune diseases, most frequently HT plus vitiligo. Comorbid autoimmune diseases, in patients with CSU, were associated with female sex, a family history of autoimmune diseases, and higher rates of hypothyroidism and hyperthyroidism (P < 0.001). Presence of autoimmune diseases was linked to aiCSU (P = 0.02). The risks of having autoimmune diseases were 1.7, 2.9 and 3.3 times higher for CSU patients with a positive ASST, BHRA and BAT, respectively. In CSU patients, markers for autoimmune diseases, antinuclear antibodies and/or IgG anti-thyroid antibodies were associated with non-response to omalizumab treatment (P = 0.013). Conclusions: In CSU, autoimmune diseases are common and linked to type IIb autoimmune CSU. Our results suggest that physicians assess and monitor all adult patients with CSU for signs and symptoms of common autoimmune diseases, especially HT and vitiligo.
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    Urticaria: Collegium Internationale Allergologicum (CIA) update 2020
    (Karger AG, 2020) Pejler, G. (Gunnar); Hartmann, K. (Karin); Weller, K. (Karsten); Kolkhir, P. (Pavel); Metz, M. (Martin); Eyerich, S. (Stefanie); Sánchez-Borges, M. (Mario); Eyerich, K. (Kilian); Jakob, T. (Thilo); Simon, D. (Dagmar); Simon, H.U. (Hans-Uwe); Maurer, M. (Marcus); Schäkel, K. (Knut); Zuberbier, T. (Torsten); Park, H.S. (Hae-Sim); Gutermuth, J. (Jan); Larenas-Linnemann, D. (Désirée); Kapp, A. (Alexander); Ferrer-Cardona, M. (Marta)
    This update on chronic urticaria (CU) focuses on the prevalence and pathogenesis of chronic spontaneous urticaria (CSU), the expanding spectrum of patient-reported outcome measures (PROMs) for assessing CU disease activity, impact, and control, as well as future treatment options for CU. This update is needed, as several recently reported findings have led to significant advances in these areas. Some of these key discoveries were first presented at past meetings of the Collegium Internationale Allergologicum (CIA). New evidence shows that the prevalence of CSU is geographically heterogeneous, high in all age groups, and increasing. Several recent reports have helped to better characterize two endotypes of CSU: type I autoimmune (or autoallergic) CSU, driven by IgE to autoallergens, and type IIb autoimmune CSU, which is due to mast cell (MC)-targeted autoantibodies. The aim of treatment in CU is complete disease control with absence of signs and symptoms as well as normalization of quality of life (QoL). This is best monitored by the use of an expanding set of PROMs, to which the Angioedema Control Test, the Cholinergic Urticaria Quality of Life Questionnaire, and the Cholinergic Urticaria Activity Score have recently been added. Current treatment approaches for CU under development include drugs that inhibit the effects of signals that drive MC activation and accumulation, drugs that inhibit intracellular pathways of MC activation and degranulation, and drugs that silence MCs by binding to inhibitory receptors. The understanding, knowledge, and management of CU are rapidly increasing. The aim of this review is to provide physicians who treat CU patients with an update on where we stand and where we will go. Many questions and unmet needs remain to be addressed, such as the development of routine diagnostic tests for type I and type IIb autoimmune CSU, the global dissemination and consistent use of PROMs to assess disease activity, impact, and control, and the development of more effective and well-tolerated long-term treatments for all forms of CU.