- Email: [email protected]
Association of Chronic Idiopahic Urticaria with Autoimmunity and IgE levels
S200 Abstracts
J ALLERGY CLIN IMMUNOL JANUARY 2007
785
Association of Chronic Idiopahic Urticaria with Autoimmunity and IgE levels A. Chomiciene1, L. M. DuBuske2, A. Blaziene1; 1Vilnius University, Vilnus, LITHUANIA, 2Immunology Research Institute of New England, Gardner, MA. RATIONALE: Approximately one-third of patients with chronic idiopathic urticaria (CIU) have circulating functional auto-antibodies directed against the high affinity IgE receptor (FceRI) or against IgE. The only in vivo method for detection of these antibodies is the autologous serum skin test (ASST). CIU is often associated with autoimmune thyroiditis. This study assesses the relationship between CIU and functional autoantibodies against FceRI or IgE and the association with autoimmune thyroiditis. METHODS: 39 patients with CIU were studied (32 females, 7 males), age range 20-70 years old. Total IgE, thyroid peroxidase antibodies (ATPO) and ASST were performed in all patients. RESULTS: Positive ASST were seen in 9 (23%) patients. Elevated ATPO were found in 7 (18%) patients, 5 (71%) of whom had a positive ASST. Increased total IgE was found in 15 (38.5%) patients, 12 (80%) having a negative ASST, and 3 (20%) having a positive ASST. CONCLUSIONS: Most patients with CIU are middle-aged females. About one-fourth of patients had a positive ASST, most of them having autoimmune thyroid disease. Increased total IgE, seen in two-fifths of patients, was more often noted in patients without functional auto-antibodies. Thus two distinct subgroups of patients, those with autoimmune etiology featuring functional auto-antibodies and those with elevated IgE generally without auto-antibodies could be identified as comprising nearly twothirds of CIU patients, suggesting a heterogeneous pathophysiologic etiology for this condition.
787
786
788
MONDAY
Investigation Of Complement Activation In Patients with Chronic Idiopathic Urticaria S. N. Sharif, B. Goldberg; Kaiser Permanente Los Angeles, Los Angeles, CA. RATIONALE: To determine if a sensitive assay for complement activation (Quidel iC3b EIA) can detect the presence of complement activation in patients with chronic idiopathic urticaria (CIU). METHODS: Blood from 14 patients with CIU was drawn into tubes containing EDTA, and Futhan was added to minimize spontaneous complement activation. Two control groups were used; 15 patients without urticaria but taking similar antihistamines for other disorders and 10 healthy individuals on no medications. iC3b levels were measured in the plasma of patients and controls using the Quidel iC3b EIA. Mean iC3b levels were compared between the 3 groups using an unpaired t-test. RESULTS: The mean iC3b concentration was 5.27 6 1.08 mcg/ml in patients with CIU and 4.48 6 0.54 mcg/ml in healthy controls (p50.029). The mean iC3b concentration for non-urticaria patients taking antihistamines was 5.16 6 0.93 mcg/ml. A significant difference was observed between the non-urticaria patients taking antihistamines and healthy controls (p50.031) but not between the non-urticaria patients taking antihistamines and urticaria patients (p50.76). The mean iC3b concentration for the subgroup of CIU patients not taking antihistamines was 6.25 6 0.94 mcg/ml. This was significantly different from the mean iC3b levels in healthy controls (p50.004). CONCLUSIONS: Patients with CIU, including those with mild CIU not on antihistamines, have increased iC3b levels compared to healthy controls. As patients with more severe CIU on antihistamines have similar levels of iC3b as patients without urticaria but taking antihistamines for other disorders, we cannot rule out complement activation in the latter group. Funding: Kaiser Permanente Southern California Regional Research Committee
Colchicine Use in Resistant Urticaria and Angioedema Patients: A Case Series B. A. Martinez, I. C. Hanson, C. M. Davis; Baylor College of Medicine, Houston, TX. RATIONALE: Controlling chronic idiopathic urticaria/angioedema (CIUA) can be challenging, with typical treatments including H1 and H2 receptor antagonists and, in severe cases, immunosuppressive agents. Although colchicine may be efficacious in urticarial vasculitis and pressure induced urticaria, reports of its efficacy in CIUA are limited, especially in children. METHODS: We present a case series of three pediatric patients, ages 6-15 y with a history of CIUA from 6 weeks to over 3 years. None had evidence of urticarial vasculitis. All had a negative autoimmune evaluation. Patients #2 and #3 were given combination therapy with H1 and H2 receptor antagonists, doxepin, montelukast, dapsone and or cyclosporine with continued symptoms. Patient #1 failed H1 and H2 receptor antagonists and montelukast. All patients received intermittent corticosteroids for severe symptoms. Patients were started on colchicine 0.6 mg or 1.2 mg daily. RESULTS: Each patient had substantial improvement of symptoms with the addition of colchicine. Patient #1 has had no recurrence on low dose cetirizine and colchicine for 1 year. Patient #2 is outbreak free on colchicine, loratadine and famotidine for 3 months. Patient #3 has been controlled on cetirizine, ranitidine and colchicine for 3 months. CONCLUSIONS: Treatment of CIUA resistant to traditional therapy may require immunosuppressive agents to control symptoms. Here we present three pediatric patients uncontrolled on typical management but with a clinical response to colchicine. In children who fail usual therapy, colchicine may be an effective, inexpensive and safer alternative to more potent immunosuppressive mediators in children.
Eosinophilic Fasciitis Masquerading as Angioedema A. M. Dowden1,2, Z. K. Ballas1,2; 1University of Iowa, Iowa City, IA, 2Veterans Affairs Medical Center, Iowa City, IA. RATIONALE: Eosinophilic fasciitis (EF) is a rare disorder which may be overlooked given its rarity. METHODS: Case report. RESULTS: An 81 year-old female presented with a two month history of painful swelling of her extremities. Although episodic, it progressively worsened to involve all four extremities and was occasionally associated with erythema. Treatment with diuretics and antibiotics failed to improve her symptoms. We were consulted to evaluate for angioedema. On physical exam, she had diffuse angioedema and mild erythema bilaterally from her elbows to wrists and from knees to ankles. Of significance, the induration seemed to follow the muscle planes. The remainder of the physical exam was negative. She denied exposure to toxins, drugs or nutritional supplements but had a history of collagenous colitis diagnosed by biopsy in 2005. She had an eosinophil count of 1009 and CRP of 5.6, but had a negative ANA and RF, normal TSH and ESR of 16. She had a monoclonal gammopathy but a normal bone marrow biopsy. An MRI of the left forearm revealed extensive subcutaneous edema infiltrating into the muscle planes with rim enhancement. A muscle biopsy revealed lymphoplasmacytic fasciitis consistent with a diagnosis of EF. She was started on prednisone and within ten days exhibited significant edema improvement and her eosinophil count plummeted to 127. CONCLUSIONS: Although the patient’s presentation looked like angioedema, thorough examination suggested that the edema followed the muscle planes and thus led to the proper diagnosis. EF should be considered in the differential diagnosis of angioedema. Funding: University of Iowa Hospitals and Clinics
J ALLERGY CLIN IMMUNOL JANUARY 2007
785
Association of Chronic Idiopahic Urticaria with Autoimmunity and IgE levels A. Chomiciene1, L. M. DuBuske2, A. Blaziene1; 1Vilnius University, Vilnus, LITHUANIA, 2Immunology Research Institute of New England, Gardner, MA. RATIONALE: Approximately one-third of patients with chronic idiopathic urticaria (CIU) have circulating functional auto-antibodies directed against the high affinity IgE receptor (FceRI) or against IgE. The only in vivo method for detection of these antibodies is the autologous serum skin test (ASST). CIU is often associated with autoimmune thyroiditis. This study assesses the relationship between CIU and functional autoantibodies against FceRI or IgE and the association with autoimmune thyroiditis. METHODS: 39 patients with CIU were studied (32 females, 7 males), age range 20-70 years old. Total IgE, thyroid peroxidase antibodies (ATPO) and ASST were performed in all patients. RESULTS: Positive ASST were seen in 9 (23%) patients. Elevated ATPO were found in 7 (18%) patients, 5 (71%) of whom had a positive ASST. Increased total IgE was found in 15 (38.5%) patients, 12 (80%) having a negative ASST, and 3 (20%) having a positive ASST. CONCLUSIONS: Most patients with CIU are middle-aged females. About one-fourth of patients had a positive ASST, most of them having autoimmune thyroid disease. Increased total IgE, seen in two-fifths of patients, was more often noted in patients without functional auto-antibodies. Thus two distinct subgroups of patients, those with autoimmune etiology featuring functional auto-antibodies and those with elevated IgE generally without auto-antibodies could be identified as comprising nearly twothirds of CIU patients, suggesting a heterogeneous pathophysiologic etiology for this condition.
787
786
788
MONDAY
Investigation Of Complement Activation In Patients with Chronic Idiopathic Urticaria S. N. Sharif, B. Goldberg; Kaiser Permanente Los Angeles, Los Angeles, CA. RATIONALE: To determine if a sensitive assay for complement activation (Quidel iC3b EIA) can detect the presence of complement activation in patients with chronic idiopathic urticaria (CIU). METHODS: Blood from 14 patients with CIU was drawn into tubes containing EDTA, and Futhan was added to minimize spontaneous complement activation. Two control groups were used; 15 patients without urticaria but taking similar antihistamines for other disorders and 10 healthy individuals on no medications. iC3b levels were measured in the plasma of patients and controls using the Quidel iC3b EIA. Mean iC3b levels were compared between the 3 groups using an unpaired t-test. RESULTS: The mean iC3b concentration was 5.27 6 1.08 mcg/ml in patients with CIU and 4.48 6 0.54 mcg/ml in healthy controls (p50.029). The mean iC3b concentration for non-urticaria patients taking antihistamines was 5.16 6 0.93 mcg/ml. A significant difference was observed between the non-urticaria patients taking antihistamines and healthy controls (p50.031) but not between the non-urticaria patients taking antihistamines and urticaria patients (p50.76). The mean iC3b concentration for the subgroup of CIU patients not taking antihistamines was 6.25 6 0.94 mcg/ml. This was significantly different from the mean iC3b levels in healthy controls (p50.004). CONCLUSIONS: Patients with CIU, including those with mild CIU not on antihistamines, have increased iC3b levels compared to healthy controls. As patients with more severe CIU on antihistamines have similar levels of iC3b as patients without urticaria but taking antihistamines for other disorders, we cannot rule out complement activation in the latter group. Funding: Kaiser Permanente Southern California Regional Research Committee
Colchicine Use in Resistant Urticaria and Angioedema Patients: A Case Series B. A. Martinez, I. C. Hanson, C. M. Davis; Baylor College of Medicine, Houston, TX. RATIONALE: Controlling chronic idiopathic urticaria/angioedema (CIUA) can be challenging, with typical treatments including H1 and H2 receptor antagonists and, in severe cases, immunosuppressive agents. Although colchicine may be efficacious in urticarial vasculitis and pressure induced urticaria, reports of its efficacy in CIUA are limited, especially in children. METHODS: We present a case series of three pediatric patients, ages 6-15 y with a history of CIUA from 6 weeks to over 3 years. None had evidence of urticarial vasculitis. All had a negative autoimmune evaluation. Patients #2 and #3 were given combination therapy with H1 and H2 receptor antagonists, doxepin, montelukast, dapsone and or cyclosporine with continued symptoms. Patient #1 failed H1 and H2 receptor antagonists and montelukast. All patients received intermittent corticosteroids for severe symptoms. Patients were started on colchicine 0.6 mg or 1.2 mg daily. RESULTS: Each patient had substantial improvement of symptoms with the addition of colchicine. Patient #1 has had no recurrence on low dose cetirizine and colchicine for 1 year. Patient #2 is outbreak free on colchicine, loratadine and famotidine for 3 months. Patient #3 has been controlled on cetirizine, ranitidine and colchicine for 3 months. CONCLUSIONS: Treatment of CIUA resistant to traditional therapy may require immunosuppressive agents to control symptoms. Here we present three pediatric patients uncontrolled on typical management but with a clinical response to colchicine. In children who fail usual therapy, colchicine may be an effective, inexpensive and safer alternative to more potent immunosuppressive mediators in children.
Eosinophilic Fasciitis Masquerading as Angioedema A. M. Dowden1,2, Z. K. Ballas1,2; 1University of Iowa, Iowa City, IA, 2Veterans Affairs Medical Center, Iowa City, IA. RATIONALE: Eosinophilic fasciitis (EF) is a rare disorder which may be overlooked given its rarity. METHODS: Case report. RESULTS: An 81 year-old female presented with a two month history of painful swelling of her extremities. Although episodic, it progressively worsened to involve all four extremities and was occasionally associated with erythema. Treatment with diuretics and antibiotics failed to improve her symptoms. We were consulted to evaluate for angioedema. On physical exam, she had diffuse angioedema and mild erythema bilaterally from her elbows to wrists and from knees to ankles. Of significance, the induration seemed to follow the muscle planes. The remainder of the physical exam was negative. She denied exposure to toxins, drugs or nutritional supplements but had a history of collagenous colitis diagnosed by biopsy in 2005. She had an eosinophil count of 1009 and CRP of 5.6, but had a negative ANA and RF, normal TSH and ESR of 16. She had a monoclonal gammopathy but a normal bone marrow biopsy. An MRI of the left forearm revealed extensive subcutaneous edema infiltrating into the muscle planes with rim enhancement. A muscle biopsy revealed lymphoplasmacytic fasciitis consistent with a diagnosis of EF. She was started on prednisone and within ten days exhibited significant edema improvement and her eosinophil count plummeted to 127. CONCLUSIONS: Although the patient’s presentation looked like angioedema, thorough examination suggested that the edema followed the muscle planes and thus led to the proper diagnosis. EF should be considered in the differential diagnosis of angioedema. Funding: University of Iowa Hospitals and Clinics