Decrease in Fc Epsilon R1 Antibodies with IVIG

Abstract

RationaleChronic idiopathic urticaria (CIU) can be extremely difficult to treat. Several reports show efficacy with intravenous immunoglobulin (IVIG). But there have been no records of Fc Epsilon R1 (FceR1) antibodies in these. I present a case where such measurement had been done, suggesting a possible mechanistic connection.MethodsA case of refractory CIU treated with IVIG is presented.ResultsA 52 year-old female with a history of hypothyroidism, chronic sinusitis, and asthma presented initially with 1 week of recurrent urticarial lesions. Over the next several months, her symptoms went on to include angioedema of the lips. Initial labs, including complete blood count with differential, blood chemistries, liver function testing, C3, C4, and thyroid stimulating hormone level were normal. Over 4 months, several antihistamines, colchicine, doxepin, dapsone, and montelukast offered no relief. Multiple oral steroid courses also failed. She had an FceR1 antibody level drawn, which was 22.1 units. Azathioprine and cyclosporine were then each tried with minimal benefit. She underwent a humoral immunodeficiency survey due to repeated sinus infections and was diagnosed with specific antibody deficiency. She was started on IVIG. After 2 doses of IVIG, she had no hives or edema. She stopped all her urticarial-related medication except cyclosporine and had no recurrence. Her FceR1 antibody level was repeated, and it had dropped to 2.65 units.ConclusionsIVIG may reduce the symptoms of urticaria and angioedema in CIU by decreasing Fc Episilon R1 antibodies. Further studies are needed to further investigate this. RationaleChronic idiopathic urticaria (CIU) can be extremely difficult to treat. Several reports show efficacy with intravenous immunoglobulin (IVIG). But there have been no records of Fc Epsilon R1 (FceR1) antibodies in these. I present a case where such measurement had been done, suggesting a possible mechanistic connection. Chronic idiopathic urticaria (CIU) can be extremely difficult to treat. Several reports show efficacy with intravenous immunoglobulin (IVIG). But there have been no records of Fc Epsilon R1 (FceR1) antibodies in these. I present a case where such measurement had been done, suggesting a possible mechanistic connection. MethodsA case of refractory CIU treated with IVIG is presented. A case of refractory CIU treated with IVIG is presented. ResultsA 52 year-old female with a history of hypothyroidism, chronic sinusitis, and asthma presented initially with 1 week of recurrent urticarial lesions. Over the next several months, her symptoms went on to include angioedema of the lips. Initial labs, including complete blood count with differential, blood chemistries, liver function testing, C3, C4, and thyroid stimulating hormone level were normal. Over 4 months, several antihistamines, colchicine, doxepin, dapsone, and montelukast offered no relief. Multiple oral steroid courses also failed. She had an FceR1 antibody level drawn, which was 22.1 units. Azathioprine and cyclosporine were then each tried with minimal benefit. She underwent a humoral immunodeficiency survey due to repeated sinus infections and was diagnosed with specific antibody deficiency. She was started on IVIG. After 2 doses of IVIG, she had no hives or edema. She stopped all her urticarial-related medication except cyclosporine and had no recurrence. Her FceR1 antibody level was repeated, and it had dropped to 2.65 units. A 52 year-old female with a history of hypothyroidism, chronic sinusitis, and asthma presented initially with 1 week of recurrent urticarial lesions. Over the next several months, her symptoms went on to include angioedema of the lips. Initial labs, including complete blood count with differential, blood chemistries, liver function testing, C3, C4, and thyroid stimulating hormone level were normal. Over 4 months, several antihistamines, colchicine, doxepin, dapsone, and montelukast offered no relief. Multiple oral steroid courses also failed. She had an FceR1 antibody level drawn, which was 22.1 units. Azathioprine and cyclosporine were then each tried with minimal benefit. She underwent a humoral immunodeficiency survey due to repeated sinus infections and was diagnosed with specific antibody deficiency. She was started on IVIG. After 2 doses of IVIG, she had no hives or edema. She stopped all her urticarial-related medication except cyclosporine and had no recurrence. Her FceR1 antibody level was repeated, and it had dropped to 2.65 units. ConclusionsIVIG may reduce the symptoms of urticaria and angioedema in CIU by decreasing Fc Episilon R1 antibodies. Further studies are needed to further investigate this. IVIG may reduce the symptoms of urticaria and angioedema in CIU by decreasing Fc Episilon R1 antibodies. Further studies are needed to further investigate this.