Chronic Spontaneous Urticaria in Patients with Common Variable Immunodeficiency (original) (raw)
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International archives of allergy and immunology, 2016
Immunoglobulin (Ig) A deficiency is a primary immunodeficiency in which autoimmunity is frequently observed. Thirty to fifty percent of patients with spontaneous chronic urticaria have autoantibodies that are able to cross-link FcεRI on mast cells and basophils. We investigated whether spontaneous chronic urticaria in patients with IgA deficiency meets the criteria for autoimmunity. Four patients were screened for positivity to a skin prick test and an autologous serum skin test and for the presence of other autoimmune diseases. Patient sera were tested for the ability to activate basophils and mast cells in vitro by measuring surface CD63 expression and β-hexosaminidase release, respectively. The autologous serum test was positive in all patients, and patient sera were found to induce CD63 upregulation on basophils and degranulation of an LAD2 mast cell line. Moreover, all patients were affected by other autoimmune disorders. For the first time, these data point out chronic autoimm...
Autoimmune Diseases Are Linked to Type IIb Autoimmune Chronic Spontaneous Urticaria
Allergy, Asthma & Immunology Research
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
2002
Background: Circulating autoantibodies against FcεRI, IgE, or both occur in approximately one third of patients with chronic idiopathic urticaria (CIU), but not all autoantibodies initiate histamine release. Objective: We sought to classify patients with CIU into subsets on the basis of serum bioactivity and immunoreactivity and to examine the relationship between newly defined subtype and disease severity. Methods: Sera from patients with CIU (n = 78), dermographism (n = 15), and cholinergic urticaria (n = 10) and sera from healthy subjects (n = 39) were analyzed by means of Western blot analysis for anti-FcεRI autoantibodies and for histamine release from basophils and dermal mast cells. In vivo reactivity of autologous serum was tested by means of intradermal injection, and CIU severity was determined on the basis of clinical interview. Results: We classified sera from patients with CIU into 5 subsets: immunoreactive histamine-releasing anti-FcεRI autoantibodies (n = 20 [26%]); immunoreactive anti-FcεRI autoantibodies without histamine-releasing activity (n = 12 [15%]); anti-IgE-like autoantibodies (n = 7 [9%]); serum containing a mast cell-specific histamine-releasing factor (n = 7 [9%]); and sera with no identifiable factor (n = 32 [41%]). Patients with serum histamine-releasing activity had more severe urticaria than patients without such activity. Positive skin test responses to autologous sera were associated with histamine-releasing anti-FcεRI autoantibodies but not with non-histamine-releasing anti-FcεRI autoantibodies. Neither healthy subjects nor patients with dermographism or cholinergic urticaria had histamine-releasing anti-FcεRI autoantibodies. Conclusion: These data support the specificity of functional
Journal of the American Academy of Dermatology, 1999
Background: Previous studies defining the clinical features of patients with chronic idiopathic urticaria (CIU) were performed before the identification of functional autoantibodies against FcεRI and/or IgE, now known to be present in approximately 30% of patients with CIU. Objective: Our purpose was to determine whether there are differences between patients with and those without autoantibodies in the clinical features or severity of CIU. Methods: The clinical features of 107 patients with CIU were evaluated prospectively. Patients were identified as having functional autoantibodies on the basis of the serumevoked histamine release in vitro from the basophils of 2 healthy donors. Results: Patients with autoantibodies (31%) had more wheals (P = .005), a wider distribution of wheals (P = .009), higher itch scores for the most severe episodes of itching (P = .002), more systemic symptoms (P = .03), and lower serum IgE levels (P < .0005) than patients without autoantibodies.
Autoimmune chronic spontaneous urticaria: What we know and what we do not know
The Journal of allergy and clinical immunology, 2017
Chronic spontaneous urticaria (CSU) is a mast cell-driven skin disease characterized by the recurrence of transient wheals, angioedema, or both for more than 6 weeks. Autoimmunity is thought to be one of the most frequent causes of CSU. Type I and II autoimmunity (ie, IgE to autoallergens and IgG autoantibodies to IgE or its receptor, respectively) have been implicated in the etiology and pathogenesis of CSU. We analyzed the relevant literature and assessed the existing evidence in support of a role for type I and II autoimmunity in CSU with the help of Hill's criteria of causality. For each of these criteria (ie, strength of association, consistency, specificity, temporality, biological gradient, plausibility, coherence, experiment, and analogy), we categorized the strength of evidence as "insufficient," "low," "moderate," or "high" and then assigned levels of causality for type I and II autoimmunity in patients with CSU from level 1 (cau...
Chronic Autoimmune Urticaria: Frequency and Association With Immunological Markers
■ Abstract Background: Chronic autoimmune urticaria (CAU), a subgroup of chronic idiopathic urticaria (CIU), is characterized by severe and persistent wheals accompanied by redness and itching. Diagnosis is almost completely based on clinical suspicion and the results of the autologous serum skin test (ASST). Objectives: To determine the frequency of CAU and compare the clinical and laboratory parameters of patients with positive and negative ASST results. Patients and Methods: A total of 165 patients with chronic urticaria (CU) were enrolled; 31 were excluded (known causes and pregnancy/ breastfeeding), leaving 134 patients with CIU. A clinical evaluation and routine and specifi c laboratory tests were performed. Results: The cause of CU was identifi ed in 18.9% of patients; 81.2% patients were considered to have CIU. The ASST result was positive in 39.6% of patients with CIU, who had more frequent urticaria attacks than patients with a negative ASST result. Patients with positive results had a higher urticaria activity score than those with negative results, although the difference was not statistically signifi cant. As for immunological markers, the absolute eosinophil count and serum immunoglobulin (Ig) E titer were lower in patients with a positive ASST result than in those with a negative ASST result, although, again, the difference was not statistically signifi cant (P=.07). Antithyroid antibody titer and B-cell percentage were higher in patients with a positive ASST result than in those with a negative result, and the difference was statistically signifi cant (P=.04 and .004, respectively). Conclusions: ASST remains a baseline diagnostic test for CAU. Patients with CAU had more frequent attacks and higher antithyroid antibody titers and peripheral B-cell percentages, as well as lower absolute eosinophil counts and serum IgE concentrations.
Journal of Allergy and Clinical Immunology, 2000
Background: Intradermal injection of autologous serum elicits a wheal-and-flare response in about 60% of patients with chronic idiopathic urticaria (CIU). This reactivity has been attributed to the presence of IgG autoantibodies directed against IgE or the α-chain of the high-affinity IgE receptor (FcεRIα) expressed on basophils and mast cells, leading to the hypothesis that at least some forms of CIU could be sustained by an autoimmune process. Objectives: The aim of this study was to investigate the relationship between the presence of anti-IgE or anti-FcεRI antibodies and the ability to induce wheal-and-flare responses in CIU sera selected for the capacity to give a positive skin test response. Methods: Fifteen patients with CIU and a positive skin test response to autologous serum were injected intradermally with native serum and with serum heated at 56°C for 30 minutes and then adsorbed on Sepharose-protein G to obtain IgG depletion. Serum levels of anti-IgE and anti-FcεRIα antibodies were measured by ELISA by using purified IgE and recombinant RIα-soluble double-fusion protein RIα-human serum albumin-RIα, respectively. The histamine-releasing activity of sera was tested by using ELISA with whole human blood from a healthy donor. Results: All patients had positive cutaneous responses to native serum injection. Anti-FcεRIα antibodies were present in 14 of 15 native sera, only two of which were able to induce in vitro basophil degranulation. On the contrary, detectable amounts of anti-IgE antibodies were not found in any serum. IgG depletion by protein G resulted in complete (10/14 samples) or considerable (4/14 samples) removal of anti-FcεRIα antibodies. The two sera endowed with functional activity lost their capacity to trigger histamine release from basophils after heating and protein G adsorption. Nonetheless, heat-decomplemented/ IgG-depleted sera elicited wheal-and-flare reactions comparable with those observed with untreated sera. Conclusions: These results strongly suggest that skin reactivity to autologous serum could be due to as yet unidentified non-Ig reactants present in the sera of patients with CIU. (J Allergy Clin Immunol 2000;106:567-72.)
Assessment of Autoimmunity in Urticaria
International journal of pharmaceutical sciences review and research, 2023
Urticaria is distinguished by the sudden onset of temporary, pruritic skin swellings (wheals). It is believed that autoimmunity, or more specifically, autoimmune mechanisms of cutaneous mast cell activation, is a prevalent underlying cause of CSU. An elevated odds ratio for hypothyroidism, hyperthyroidism, and the presence of thyroid autoantibodies was linked to a diagnosis of CU. About 50% of patients have positive "autoimmune" tests in vivo and/or in vitro, and there is a substantial quantity of circumstantial evidence indicating that chronic urticaria is an autoimmune illness. While there are still many unanswered questions regarding CSU, it is becoming more and more obvious that both autoimmunity (an IgG-mediated disease) and autoallergy (an IgE-mediated disease) can play a role in its pathogenesis and predispose individuals to the emergence of other autoimmune diseases.
Journal of Allergy and Clinical Immunology, 2005
Background: Approximately 40% to 50% of patients with chronic idiopathic urticaria (CIU) have functional IgG autoantibodies against FceRIa or IgE, which induce histamine release from basophils and cutaneous mast cells. A positive autologous serum skin test response is believed to reflect the presence of these autoantibodies. Objective: We sought to further define the functional properties of and develop a sensitive functional assay for detection of autoantibodies in patients with CIU. Methods: Sera from patients with CIU (n = 61) and sera from healthy control subjects (n = 23) were incubated with donor basophils. Activation of basophils was determined on the basis of CD63 surface expression, as analyzed on a FACScan flow cytometer. Results: A positive basophil activation test result was found in 51% of patients with CIU, and basophil-activating properties were present in the IgG fractions of sera. When both the in vitro test and the autologous serum skin test were considered, basophil/mast cell-activating autoantibodies were present in 62% of the patients. Patients with a positive basophil activation test result had a significantly higher prevalence of other autoantibodies, had more severe urticaria, and were more likely to have angioedema. Conclusion: The results demonstrate the presence of basophilactivating autoantibodies in about 50% of patients with CIU. The data support the autoimmune cause of the disease and provide a simple test for detection of these autoantibodies. (J Allergy Clin Immunol 2005;116:662-7.)
Indian Journal of Dermatology, Venereology and Leprology, 2008
Background: Chronic idiopathic urticaria (CIU), in its extremely severe form, can pose a therapeutic challenge to the treating physician. It has been noted that in one third of such patients, autoantibodies against the IgE receptor are seen and such patients have more severe and unremitting urticaria. Aim: To compare clinical features of autoimmune urticaria with those of other CIU patients. Methods: We conducted a prospective study in an attempt to correlate the clinical features with autoantibodies, indirectly detected via the autologous serum skin test (ASST), which is the simplest and the best in vivo clinical test for detection of basophil histamine-releasing activity. Discussion: Out of 100 patients with chronic idiopathic urticaria, 34 showed a positive reaction to the autologous serum skin test and it was found that the frequency and severity of attacks was higher in these patients. Conclusion: ASST may be used as a simple and cost-effective test for the classifi cation of chronic urticaria, which has proven to be a therapeutic challenge to the treating physician.