The Diagnostic Workup in Chronic Spontaneous Urticaria—What to Test and Why

Metz, Martin; Altrichter, Sabine; Buttgereit, Thomas; Fluhr, Joachim W.; Fok, Jie Shen; Hawro, Tomasz; Jiao, Qingqing; Kolkhir, Pavel; Krause, Karoline; Magerl, Markus; Pyatilova, Polina; Siebenhaar, Frank; Huichun, Su; Terhorst‐Molawi, Dorothea; Weller, Karsten; Xiang, Yi-Kui; Maurer, Marcus

doi:10.1016/j.jaip.2021.03.049

Cited by 34 publications

(50 citation statements)

References 136 publications

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“…They are to confirm the diagnosis and exclude differential diagnoses; to look for the underlying causes; to identify relevant conditions that modify disease activity; to check for comorbidities; to identify the consequences of CSU; to assess predictors of the course of disease and response to treatment; and to monitor disease activity, impact, and control (Table 9). 34 …”

Section: Diagnosis Of Urticariamentioning

confidence: 99%

“…to check for comorbidities; to identify the consequences of CSU; to assess predictors of the course of disease and response to treatment; and to monitor disease activity, impact, and control (Table 9). 34 In all CSU patients, the diagnostic workup includes a thorough history, physical examination (including review of pictures of wheals and/ or angioedema), basic tests, and the assessment of disease activity, impact, and control. The basic tests include a differential blood count and CRP and/or ESR, in all patients, and total IgE and IG-anti-TPO, in patients in specialist care.…”

Section: Diagnostic Workup In Csumentioning

confidence: 99%

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The international EAACI/GA²LEN/EuroGuiDerm/APAAACI guideline for the definition, classification, diagnosis, and management of urticaria

Zuberbier

Latiff

Abuzakouk

et al. 2021

Allergy

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655

646

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This update and revision of the international guideline for urticaria was developed following the methods recommended by Cochrane and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) working group. It is a joint initiative of the Dermatology Section of the European Academy of Allergology and Clinical Immunology (EAACI), the Global Allergy and Asthma European Network (GA²LEN) and its Urticaria and Angioedema Centers of Reference and Excellence (UCAREs and ACAREs), the European Dermatology Forum (EDF; EuroGuiDerm), and the Asia Pacific Association of Allergy, Asthma and Clinical Immunology with the participation of 64 delegates of 50 national and international societies and from 31 countries. The consensus conference was held on 3 December 2020. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). Urticaria is a frequent, mast cell–driven disease that presents with wheals, angioedema, or both. The lifetime prevalence for acute urticaria is approximately 20%. Chronic spontaneous or inducible urticaria is disabling, impairs quality of life, and affects performance at work and school. This updated version of the international guideline for urticaria covers the definition and classification of urticaria and outlines expert‐guided and evidence‐based diagnostic and therapeutic approaches for the different subtypes of urticaria.

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Section: Diagnosis Of Urticariamentioning

confidence: 99%

Section: Diagnostic Workup In Csumentioning

confidence: 99%

The international EAACI/GA²LEN/EuroGuiDerm/APAAACI guideline for the definition, classification, diagnosis, and management of urticaria

Zuberbier

Latiff

Abuzakouk

et al. 2021

Allergy

Self Cite

655

646

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“…The exact underlying pathomechanisms of skin mast cell activation in CSU are not fully understood. Based on recent evidence, three subtypes of CSU have been described, type I autoimmunity (or “autoallergy”), type IIb autoimmunity (“classical autoimmunity”), and CSU due to unknown cause ( 180 ). In addition, other factors such as acute infections, certain drugs or stress modulate mast cell activation and drive exacerbations or worsening of CSU.…”

Section: Autoimmune Skin Diseasesmentioning

confidence: 99%

“…The current guideline on the definition, classification, diagnosis, and management of urticaria recommends a detailed patient history, physical examination (including pictures from patients) and a basic diagnostic workup consisting of a complete blood count with differential, CRP, IgG anti-TPO and total IgE ( 178 ). The questions and investigations are mainly aimed at ruling out rare differential diagnoses, for example urticaria vasculitis, autoinflammatory syndromes or bradykinin-mediated angioedema, assessing patients for underlying causes and modifying conditions, and identifying comorbid diseases and consequences of having CSU ( 180 ). Based on the answers to the respective questions, additional investigations such as histological examination of a skin biopsy or further laboratory analyses may be necessary.…”

Section: Autoimmune Skin Diseasesmentioning

confidence: 99%

Unmet Medical Needs in Chronic, Non-communicable Inflammatory Skin Diseases

et al. 2022

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An estimated 20–25% of the population is affected by chronic, non-communicable inflammatory skin diseases. Chronic skin inflammation has many causes. Among the most frequent chronic inflammatory skin diseases are atopic dermatitis, psoriasis, urticaria, lichen planus, and hidradenitis suppurativa, driven by a complex interplay of genetics and environmental factors. Autoimmunity is another important cause of chronic skin inflammation. The autoimmune response may be mainly T cell driven, such as in alopecia areata or vitiligo, or B cell driven in chronic spontaneous urticaria, pemphigus and pemphigoid diseases. Rare causes of chronic skin inflammation are autoinflammatory diseases, or rheumatic diseases, such as cutaneous lupus erythematosus or dermatomyositis. Whilst we have seen a significant improvement in diagnosis and treatment, several challenges remain. Especially for rarer causes of chronic skin inflammation, early diagnosis is often missed because of low awareness and lack of diagnostics. Systemic immunosuppression is the treatment of choice for almost all of these diseases. Adverse events due to immunosuppression, insufficient therapeutic responses and relapses remain a challenge. For atopic dermatitis and psoriasis, a broad spectrum of innovative treatments has been developed. However, treatment responses cannot be predicted so far. Hence, development of (bio)markers allowing selection of specific medications for individual patients is needed. Given the encouraging developments during the past years, we envision that many of these challenges in the diagnosis and treatment of chronic inflammatory skin diseases will be thoroughly addressed in the future.

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“…Future perspectives, barriers and facilitators5.2.1 | Precision medicine using endotyping and multiple upstream targetsThe separation into type I and type IIb autoimmunity and further validation of biomarkers is of utmost importance to select responders to omalizumab 19,25,[108][109][110]. In addition, a better understanding of the 'non-canonical' mechanisms of action of omalizumab, such as effects on mast cell releasability or the coagulation cascade or targeting membrane-IgE in IgE+B cells, reducing IL4R expression and IgE synthesis and decreasing the number of these cells, possibly by causing B-cell anergy, should be further prioritized 111,112.…”

mentioning

confidence: 99%

EAACI Biologicals Guidelines—Omalizumab for the treatment of chronic spontaneous urticaria in adults and in the paediatric population 12–17 years old

Agache

Akdiş

Brockow

et al. 2021

Allergy

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Chronic spontaneous urticaria (CSU) imposes a significant burden on patients, families and healthcare systems. Management is difficult, due to disease heterogeneity and insufficient efficacy of classical drugs such as H 1 R-antihistamines. Better understanding of the mechanisms has enabled a stratified approach to the management of CSU, supporting the use of targeted treatment with omalizumab. However, many practical issues including selection of responders, the definition of response, strategies to enhance the responder rate, the duration of treatment and its regimen (in the clinic or home-based) and its cost-effectiveness still require further clarification. The EAACI Guidelines on the use of omalizumab in CSU follow the GRADE approach in formulating recommendations for each outcome. In addition, future therapeutic approaches and perspectives as well as research priorities are discussed.

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The Diagnostic Workup in Chronic Spontaneous Urticaria—What to Test and Why

Cited by 34 publications

References 136 publications

The international EAACI/GA²LEN/EuroGuiDerm/APAAACI guideline for the definition, classification, diagnosis, and management of urticaria

The international EAACI/GA²LEN/EuroGuiDerm/APAAACI guideline for the definition, classification, diagnosis, and management of urticaria

Unmet Medical Needs in Chronic, Non-communicable Inflammatory Skin Diseases

EAACI Biologicals Guidelines—Omalizumab for the treatment of chronic spontaneous urticaria in adults and in the paediatric population 12–17 years old

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