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Hereditary Angioedema Due to C1 Inhibitor Deficiency: New Findings Concerning Symptoms and Affected Organs
S122 Abstracts
Comparison of Autologous Serum Test (AST) Between Healthy Volunteers and Patients with Chronic Idiopathic Urticaria (CIU). PK Vedanthan* PA Mahesh^, AD Holla^, AK Prabhakar^. *Univ. of Colorado Health Sciences Center, Denver, Colorado USA; ^ Allergy, Asthma and Chest Center, Mysore, INDIA P. K. Vedanthan; Medicine, Univ. of Colorado Health Sciences Center, Lakewood, CO. RATIONALE: AST is a test to demonstrate an autoimmune phenomenon in patients with CIU. We have established a normogram in healthy volunteers for AST. We wanted to compare the time-reaction relationship in patients with CIU and rule out an irritant phenomenon. METHODS: 30 patients with CIU (19 females, 11 males) with the mean age of 24.6 years participated in the study. AST was performed on all the subjects as per the accepted protocol. The test was read and recorded every 5 minutes. The results were compared with the normogram developed by testing 20 healthy volunteers. RESULTS: The peak reaction of the AST was noted at 30 minutes in patients with CIU, as compared to the healthy volunteers, in whom the peak values were recorded at the 10th. minute posttest. At the end of 30 minutes, in all subjects with CIU, the difference in the wheal size between the saline control and the serum or plasma was more than 3 mm, and in healthy volunteers it was 1.02 mm for the serum and 2.22 mm for the plasma. CONCLUSIONS: 1. 1. AST peaks at 30 minutes posttest in patients with CIU, compared to 10 minutes in normal controls. 2. A positive AST in patients with CIU read at thirty minutes is unlikely to be an irritant phenomenon.
476
SUNDAY
Serum Concentration of Dehydroepiandrosterone Sulphate (DHEA-S) in Female Patients with Chronic Idiopathic Urticaria (CIU) A. Kasperska-Zajac, Z. Brzoza, B. Rogala; Chair and Clinical Department of Internal Diseases, Allergology and Clinical Immunology, Medical University of Silesia, Zabrze, POLAND. RATIONALE: The aetiology of chronic urticaria is unknown, but in some cases an autoimmune pathogenic mechanism is proposed. It has been reported that an adrenal androgen, DHEAs shows immunomodulatory properties and its deficiency has been associated with immune-mediated diseases. The question raised by the data is whether there appears any role for DHEA-S in CIU. Therefore, the aim of this study was to evaluate serum concentration of DHEA-S in female patients suffering from CIU. METHODS: Serum DHEA-S concentration was determined by ELISA method in 22 CIU female patients showing negative wheal response to autologous serum skin test (ASST), 12 CIU female patients with strong reactivity to ASST, and 32 non-atopic healthy women. The statistical analysis of the results was made using the Kruskal-Wallis test as well as the U Mann-Whitney test. RESULTS: In CIU patients serum concentration of DHEA-S was significantly lower than the control values (median value: 180.5 and 268.3 ug/dl, respectively; p = 0.003). No difference was observed in DHEA-S concentration between the two patient subgroups. CONCLUSIONS: It is difficult to draw a firm conclusion from our results due to the relatively small number of patients studied, but these data reveal that the circulating level of DHEA-S is lower in CIU patients. Our observation could provide a basis for further research into androgen secretion and respective role in urticaria as well as for new concept of therapy of this disease, such as dehydroepiandrosterone administration.
477
J ALLERGY CLIN IMMUNOL FEBRUARY 2006
Sulfasalazine is Beneficial in the Treatment of Recalcitrant Chronic Idiopathic Urticaria L. Y. McGirt, L. A. Beck; Medicine - Clinical Immunology, Johns Hopkins School of Medicine, Baltimore, MD. RATIONALE: Chronic idiopathic urticaria (CIU) does not always adequately respond to anti-histamines, and there have been efforts to identify steroid-sparing treatments for those with recalcitrant CIU. In this study we present the largest case series evaluating the efficacy of sulfasalazine as treatment for CIU. METHODS: We performed a retrospective chart review of people who received sulfasalazine for CIU at the Johns Hopkins Bayview Medical Center between 2002-2005. We recorded demographic data, subjective response to sulfasalazine, change in steroid use, and side effects. RESULTS: Nineteen patients with recalcitrant CIU were treated with sulfasalazine between 2002 and 2005. Fourteen (74%) patients reported significant improvement in their urticaria, and four (21%) patients reported minimal improvement, but were not satisfied. One patient (5%) reported a worsening of urticaria while on sulfasalazine. Of the nine patients requiring systemic steroids for control of their urticaria, all were able to reduce or completely stop steroid use while on sulfasalazine. Four patients were able to come off all other urticaria medications, and one patient tapered off of sulfasalazine and remains free of urticaria. Although seven (37%) patients reported side effects felt to be secondary to sulfasalazine, such as gastrointestinal discomfort and nausea, they all continued their use of sulfasalazine. CONCLUSIONS: We demonstrate that sulfasalazine is an effective treatment for CIU that has not responded adequately to antihistamines, and can also reduce the need for systemic steroids. Prospective trials are needed to further evaluate its efficacy.
478
Hereditary Angioedema Due to C1 Inhibitor Deficiency: New Findings Concerning Symptoms and Affected Organs K. Bork, P. Staubach, G. Meng; Department of Dermatology, University of Mainz, Mainz, GERMANY. RATIONALE: We examined a temporal and spatial pattern of the edema episodes of hereditary angioedema due to C1 inhibitor deficiency by evaluating the long-term course in order to establish a specific swelling pattern. METHODS: Data were generated from 221 patients with C1 inhibitor deficiency by asking them about symptoms they experienced during their edema episodes. Documentation was accomplished through the use of standardized questionnaires. RESULTS: A total of 131,110 episodes of skin swellings, abdominal pain attacks, or other symptoms were observed. Clinical symptoms started at a mean age of 11.2 (SD 7.7) years. During the following cumulative 5,736 years, only 370 (6.5%) symptom-free years occurred. Skin swellings, including extremity, facial, genital, and trunk swellings, and abdominal attacks occurred in 97.4% of all edema episodes of the disease. The other episodes were laryngeal edema (0.9%); edema of the soft palate (0.6%); tongue swellings (0.3%); headache episodes (0.7%); episodes affecting urinary bladder (0.3%), chest (0.2%), muscles (0.4%), joints (0.1%), kidneys (0.1%), and esophagus (0.05%).The per-patient analysis and the per-episode analysis revealed markedly discrepant results. CONCLUSIONS: The described swelling pattern is specific for hereditary angioedema and allows a tentative diagnosis based on clinical symptoms and the course of the disease. The analysis also revealed a number of affected organs hitherto unrecognized as being affected by hereditary angioedema.
479
Comparison of Autologous Serum Test (AST) Between Healthy Volunteers and Patients with Chronic Idiopathic Urticaria (CIU). PK Vedanthan* PA Mahesh^, AD Holla^, AK Prabhakar^. *Univ. of Colorado Health Sciences Center, Denver, Colorado USA; ^ Allergy, Asthma and Chest Center, Mysore, INDIA P. K. Vedanthan; Medicine, Univ. of Colorado Health Sciences Center, Lakewood, CO. RATIONALE: AST is a test to demonstrate an autoimmune phenomenon in patients with CIU. We have established a normogram in healthy volunteers for AST. We wanted to compare the time-reaction relationship in patients with CIU and rule out an irritant phenomenon. METHODS: 30 patients with CIU (19 females, 11 males) with the mean age of 24.6 years participated in the study. AST was performed on all the subjects as per the accepted protocol. The test was read and recorded every 5 minutes. The results were compared with the normogram developed by testing 20 healthy volunteers. RESULTS: The peak reaction of the AST was noted at 30 minutes in patients with CIU, as compared to the healthy volunteers, in whom the peak values were recorded at the 10th. minute posttest. At the end of 30 minutes, in all subjects with CIU, the difference in the wheal size between the saline control and the serum or plasma was more than 3 mm, and in healthy volunteers it was 1.02 mm for the serum and 2.22 mm for the plasma. CONCLUSIONS: 1. 1. AST peaks at 30 minutes posttest in patients with CIU, compared to 10 minutes in normal controls. 2. A positive AST in patients with CIU read at thirty minutes is unlikely to be an irritant phenomenon.
476
SUNDAY
Serum Concentration of Dehydroepiandrosterone Sulphate (DHEA-S) in Female Patients with Chronic Idiopathic Urticaria (CIU) A. Kasperska-Zajac, Z. Brzoza, B. Rogala; Chair and Clinical Department of Internal Diseases, Allergology and Clinical Immunology, Medical University of Silesia, Zabrze, POLAND. RATIONALE: The aetiology of chronic urticaria is unknown, but in some cases an autoimmune pathogenic mechanism is proposed. It has been reported that an adrenal androgen, DHEAs shows immunomodulatory properties and its deficiency has been associated with immune-mediated diseases. The question raised by the data is whether there appears any role for DHEA-S in CIU. Therefore, the aim of this study was to evaluate serum concentration of DHEA-S in female patients suffering from CIU. METHODS: Serum DHEA-S concentration was determined by ELISA method in 22 CIU female patients showing negative wheal response to autologous serum skin test (ASST), 12 CIU female patients with strong reactivity to ASST, and 32 non-atopic healthy women. The statistical analysis of the results was made using the Kruskal-Wallis test as well as the U Mann-Whitney test. RESULTS: In CIU patients serum concentration of DHEA-S was significantly lower than the control values (median value: 180.5 and 268.3 ug/dl, respectively; p = 0.003). No difference was observed in DHEA-S concentration between the two patient subgroups. CONCLUSIONS: It is difficult to draw a firm conclusion from our results due to the relatively small number of patients studied, but these data reveal that the circulating level of DHEA-S is lower in CIU patients. Our observation could provide a basis for further research into androgen secretion and respective role in urticaria as well as for new concept of therapy of this disease, such as dehydroepiandrosterone administration.
477
J ALLERGY CLIN IMMUNOL FEBRUARY 2006
Sulfasalazine is Beneficial in the Treatment of Recalcitrant Chronic Idiopathic Urticaria L. Y. McGirt, L. A. Beck; Medicine - Clinical Immunology, Johns Hopkins School of Medicine, Baltimore, MD. RATIONALE: Chronic idiopathic urticaria (CIU) does not always adequately respond to anti-histamines, and there have been efforts to identify steroid-sparing treatments for those with recalcitrant CIU. In this study we present the largest case series evaluating the efficacy of sulfasalazine as treatment for CIU. METHODS: We performed a retrospective chart review of people who received sulfasalazine for CIU at the Johns Hopkins Bayview Medical Center between 2002-2005. We recorded demographic data, subjective response to sulfasalazine, change in steroid use, and side effects. RESULTS: Nineteen patients with recalcitrant CIU were treated with sulfasalazine between 2002 and 2005. Fourteen (74%) patients reported significant improvement in their urticaria, and four (21%) patients reported minimal improvement, but were not satisfied. One patient (5%) reported a worsening of urticaria while on sulfasalazine. Of the nine patients requiring systemic steroids for control of their urticaria, all were able to reduce or completely stop steroid use while on sulfasalazine. Four patients were able to come off all other urticaria medications, and one patient tapered off of sulfasalazine and remains free of urticaria. Although seven (37%) patients reported side effects felt to be secondary to sulfasalazine, such as gastrointestinal discomfort and nausea, they all continued their use of sulfasalazine. CONCLUSIONS: We demonstrate that sulfasalazine is an effective treatment for CIU that has not responded adequately to antihistamines, and can also reduce the need for systemic steroids. Prospective trials are needed to further evaluate its efficacy.
478
Hereditary Angioedema Due to C1 Inhibitor Deficiency: New Findings Concerning Symptoms and Affected Organs K. Bork, P. Staubach, G. Meng; Department of Dermatology, University of Mainz, Mainz, GERMANY. RATIONALE: We examined a temporal and spatial pattern of the edema episodes of hereditary angioedema due to C1 inhibitor deficiency by evaluating the long-term course in order to establish a specific swelling pattern. METHODS: Data were generated from 221 patients with C1 inhibitor deficiency by asking them about symptoms they experienced during their edema episodes. Documentation was accomplished through the use of standardized questionnaires. RESULTS: A total of 131,110 episodes of skin swellings, abdominal pain attacks, or other symptoms were observed. Clinical symptoms started at a mean age of 11.2 (SD 7.7) years. During the following cumulative 5,736 years, only 370 (6.5%) symptom-free years occurred. Skin swellings, including extremity, facial, genital, and trunk swellings, and abdominal attacks occurred in 97.4% of all edema episodes of the disease. The other episodes were laryngeal edema (0.9%); edema of the soft palate (0.6%); tongue swellings (0.3%); headache episodes (0.7%); episodes affecting urinary bladder (0.3%), chest (0.2%), muscles (0.4%), joints (0.1%), kidneys (0.1%), and esophagus (0.05%).The per-patient analysis and the per-episode analysis revealed markedly discrepant results. CONCLUSIONS: The described swelling pattern is specific for hereditary angioedema and allows a tentative diagnosis based on clinical symptoms and the course of the disease. The analysis also revealed a number of affected organs hitherto unrecognized as being affected by hereditary angioedema.
479