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Chronic spontaneous urticaria

Chronic spontaneous urticaria (CSU) is the most common form of chronic urticaria. A considerable amount of data supports an immunological basis for CSU. Some research has focused on the association between chronic urticaria and specific human leukocyte antigen (HLA) alleles. Î’ozek et al (2010) demonstrated that HLA alleles may be involved in CSU development or play a protective role in CSU[1].

Chronic spontaneous urticaria (CSU) lasting more than 6 weeks is one of the most disabling types of urticaria and often results in severely impaired quality of life. Patients with CSU are often unsatisfied with the standard treatment. Another treatment option recommended for patients with so-called nonresponding CSU according to the newest guidelines is intravenous immunoglobulin (IVIG).

IL-31 serum levels may play a role in the pathophysiology of CU. This is supported by the finding that not all patients with CU respond to antihistamine treatment but to the treatment with immunosuppressive drugs [3].

Chronic spontaneous urticaria is a common skin disorder characterized by recurrent, transitory, itchy wheals. It is defined as the daily or almost daily occurrence of wheals for at least 6 weeks[4].

Chronic spontaneous urticaria is an extremely distressing and disabling condition for the patient. A study has reported that the degree of disability experienced by a chronic urticaria patient -personal, social and occupational - is comparable with that of patients with severe coronary artery disease awaiting surgery  [5].

The diagnosis "chronic spontaneous urticaria" (CSU) is used in situations in which urticarial vasculitis and predominant physical urticarias have been excluded '. CSU is affecting at least 0.1-3.0% of the population and has a chronic relapsing course with approximately 20% of patients still having the disease after 10 years [6].

High-dose IVIG represents an important therapeutic option in patients with severe CSU [2].

About 30-35%) of CSU patients including children may have circulating autoantibodies against IgE or the α subunit of the high-affinity IgE receptor (FceRla) demonstrated by HR-Urticaria Test [7, 8].

Serum induced histamine release is considered as the only laboratory method to demonstrate not only mast cell activating autoantibodies but also other mast cell activating signals.

References

1. Bozek A, Krajewska J, Filipowska B, Polanska J, Rachowska R, Grzanka A, Jarzab J. HLA Status in Patients with Chronic Spontaneous Urticaria. Int Arch Allergy Immunol. 2010 Jun 18;153(4):419-423. [Epub ahead of print].

2. Mitzel-Kaoukhov H, Staubach P, Müller-Brenne T. Effect of high-dose intravenous immunoglobulin treatment in therapy-resistant chronic spontaneous urticaria. Ann Allergy Asthma Immunol. 2010 Mar;104(3):253-8.

3. Raap U, Wieczorek D, Gehring M, Pauls I, Ständer S, Kapp A, Wedi B.Increased levels of serum IL-31 in chronic spontaneous urticaria.Exp Dermatol. 2010 May;19(5):464-6. Epub 2010 Feb 16.

4. Zuberbier T, Asero R, Bindslev-Jensen C, Walter Canonica G, Church MK, Giménez-Arnau AM, Grattan CE, Kapp A, Maurer M, Merk HF, Rogala B, Saini S, Sánchez-Borges M, Schmid-Grendelmeier P, Schünemann H, Staubach P, Vena GA, Wedi B; Dermatology Section of the European Academy of Allergology and Clinical Immunology; Global Allergy and Asthma European Network; European Dermatology Forum; World Allergy Organization. EAACI/GA(2)LEN/EDF/WAO guideline: management of urticaria.Allergy. 2009 Oct;64(10):1427-43.

5. Greaves MW, O'Donnell BF.Not all chronic urticaria is "idiopathic"!Exp Dermatol. 1998 Feb;7(1):11-3.

6. Sabroe RA, Seed PT, Francis DM, Barr RM, Black AK, Greaves MW.Chronic idiopathic urticaria: comparison of the clinical features of patients with and without anti-FcepsilonRI or anti-IgE autoantibodies.J Am Acad Dermatol. 1999 Mar;40(3):443-50.

7. Platzer MH, Grattan CE, Poulsen LK, Skov PS. Validation of basophil histamine release against the autologous serum skin test and outcome of serum-induced basophil histamine release studies in a large population of chronic urticaria patients.Allergy. 2005 Sep;60(9):1152-6.

8. Brunetti L, Francavilla R, Miniello VL, Platzer MH, Rizzi D, Lospalluti ML, Poulsen LK, Armenio L, Skov PS.High prevalence of autoimmune urticaria in children with chronic urticaria.J Allergy Clin Immunol. 2004 Oct;114(4):922-7.