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P095 Transarterial chemoembolization (TACE) procedure-induced development of acquired angioedema
Abstracts: Poster Sessions / Ann Allergy Asthma Immunol 117 (2016) S22eS124
wall thickening and a degree of stasis with no evidence for a primary focal obstruction (Figure A). ACE-I-induced small bowel angioedema was made as a diagnosis of exclusion. Conclusion: Intestinal angioedema related to ACE-I is a relatively rare but likely under-recognized condition characterized by abdominal pain, nausea, and/or diarrhea. CT findings consist of small bowel wall thickening, dilatation, and stiffening, with or without ascites.
P093 NOT JUST A VIRUS: DIFFICULT DIAGNOSIS OF TYPE I HEREDITARY ANGIOEDEMA IN A TODDLER S. Hwang*1, H. Gordon1, T. Craig2, M. DeFelice3, 1. Philadelphia, PA; 2. Hershey, PA; 3. Garnet Valley, PA. Introduction: Hereditary angioedema (HAE) is a rare, genetic condition characterized by recurrent episodes of cutaneous and/or potentially fatal mucosal angioedema. Type I HAE, due to C1 inhibitor (C1INH) deficiency, is the most common form. Typical laboratory findings include C4 level <50% of normal and C1INH protein and functional levels <30% of normal. Methods: The patient is a 2-year-old male presenting with 1 day of fever, emesis, erythematous rash, and minimal symmetric extremity edema. Influenza A testing was positive. Swelling improved in 24 hours. He has a strong family history of type I HAE. Results: Initial labs revealed C4 <8 mg/dL (9-36 mg/dL), C1INH protein 12 mg/dL (19-37 mg/dL) and normal C1INH function (ELISA). Baseline labs demonstrated normal C4 level, C1INH protein 12 mg/dL (19-37 mg/dL) and normal C1INH function (ELISA). The patient presented 5 months later with URI symptoms, emesis, and minimal pedal edema. Swelling resolved in 24 hours. Studies revealed low C4 level <8 mg/dL (11-61 mg/dL), C1INH protein 5.4 mg/dL (8-19.5 mg/dL), and C1INH function 42% (74-174%, chromogenic). Conclusion: This young patient presented with characteristic viral symptoms, indiscernible extremity swelling, and a family history of HAE. Baseline C4 was normal, C1INH protein levels were consistently >60% of normal, and C1INH function was normal on ELISA. Chromogenic testing for C1INH function provided increased sensitivity and specificity compared to the routine ELISA assay. Due to heterogeneity of presentation and potential variability in lab findings, clinicians must maintain a high index of suspicion for HAE in patients with a family history of the disorder.
P094 DEPRESSION AND ANXIETY IN HEREDITARY ANGIOEDEMA COMPARED TO PRIMARY IMMUNODEFICIENCY AND HYMENOPTERA ALLERGY G. Sabharwal*1, T. Craig2, 1. Camp Hill, PA; 2. Hershey, PA. Introduction: Hereditary angioedema (HAE) is a rare debilitating illness with recurrent attacks. Primary Immunodeficiency (PID) is a rare inherited disorder that often has significant drug burden. Hymenoptera sting allergy (HA) is associated with the fear of anaphylactic reaction. In light of above features of PID and HA, it was our objective to compare depression and anxiety associated with them to HAE. Methods: Three studies, which were IRB approved, were compared using Hamilton Depression Rating Scale (HAM-D) and the Hamilton Anxiety Rating Scale (HAM-A) to determine the extent of anxiety and depression. Results: Patients with HAE, 39% of participants were identified as having depression of mild (50%), moderate (40%) or severe (10%) levels. Fifteen percent had anxiety of mild (50%), moderate (25%) and severe (25%) levels. PID patients had a median score of 2 with upper and lower quartiles of (0,4) on HAM-D, which corresponds to normal degree of depressive symptoms. On HAM-A they had a
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median score of 2 with upper and lower quartiles of (1,5), which corresponds to none to mild degree of anxiety. Twenty percent of patients with HA had depression of which 50% were in epinephrine-only treatment group. Mild Anxiety was present in 93.3% patients, 5.6% showed mild-moderate anxiety and they were epinephrine users whereas only 1.1 % showed moderate to severe anxiety and were in non-treatment group. Conclusion: Depression and anxiety in HAE far exceeds that noted in PID and HA. Appropriate care of HAE should include assessment of depression and anxiety and treatment when necessary.
P095 TRANSARTERIAL CHEMOEMBOLIZATION (TACE) PROCEDURE-INDUCED DEVELOPMENT OF ACQUIRED ANGIOEDEMA T. Patel*1, S. Sur2, J. McCracken1, 1. League City, TX; 2. Galveston, TX. Introduction: Acquired angioedema (AAE) is a very rare disorder with just over 100 reported cases world-wide. AAE consists of hyper-activation of the classical complement pathway and acquired C1 inhibitor deficiency causing recurrent angioedema symptoms. Lymphoproliferative disorders have been implicated in the development of AAE. Here we present a case of a procedure-induced development of AAE without any prior or subsequent episodes of angioedema. Case: A 67 year old female with a history of hepatocellular carcinoma experienced delayed onset angioedema following TACE. The patient developed upper extremity, facial, and severe laryngeal edema 12-18 hours post-TACE procedure, and required intensive care unit admission. Initial laboratory studies revealed low levels of C3 (36 mg/dL, normal 86-184 mg/dL), C4 (2 mg/dL, normal 20-59 mg/dL ), and C1 esterase inhibitor (7 mg/dL, normal 21-39 mg/dL). Her symptoms were unresponsive to histamine receptor 1 antagonists and corticosteroid therapy, but gradually resolved over the course of 3-5 days. On repeat evaluation two months later, the patient had normalization of complement levels (C3 97 mg/dL, C4 24 mg/dL, and C1-esterase inhibitor 36 mg/dL with 115% function). Prior to TACE, the patient never experienced any angioedema symptoms, nor has she developed any subsequent episodes of angioedema. Conclusion: Given the delayed onset, absence of any previous or subsequent angioedema, low C1-inhibitor levels that normalized, and unresponsiveness to antihistamine and corticosteroid therapy, we hypothesize that the patient has AAE triggered by TACE. For future TACE procedures, the patient may benefit from administration of plasma-derived C1-inhibitor and bradykinin inhibitor for prevention and treatment of angioedema.
P096 HEREDITARY ANGIOEDEMA IN THE PUERTO RICAN POPULATION: EPIDEMIOLOGIC CHARACTERISTICS AND BURDEN OF DISEASE Y. Arce Ayala*1, E. Arroyo-Flores2, S. Nazario2, C. Ramos2, 1. Bayamon, Puerto Rico; 2. San Juan, Puerto Rico. Introduction: Hereditary angioedema (HAE) is a life-threatening condition characterized by episodes of subcutaneous or submucosal edema. Despite numerous HAE studies published in the literature, only one has been performed in Puerto Rico (PR). This study describes clinical characteristics of HAE patients, but no data regarding quality of life has ever been published in this population. The aim of this study was to assess epidemiologic characteristics and burden of this disease in the Puerto Rican population. Methods: A retrospective study was performed. Data on sociodemographic characteristics, symptoms, and treatment was collected by means of a survey administered to HAE patients. To evaluate health-related quality of life, the SF-36v2 questionnaire was used.
wall thickening and a degree of stasis with no evidence for a primary focal obstruction (Figure A). ACE-I-induced small bowel angioedema was made as a diagnosis of exclusion. Conclusion: Intestinal angioedema related to ACE-I is a relatively rare but likely under-recognized condition characterized by abdominal pain, nausea, and/or diarrhea. CT findings consist of small bowel wall thickening, dilatation, and stiffening, with or without ascites.
P093 NOT JUST A VIRUS: DIFFICULT DIAGNOSIS OF TYPE I HEREDITARY ANGIOEDEMA IN A TODDLER S. Hwang*1, H. Gordon1, T. Craig2, M. DeFelice3, 1. Philadelphia, PA; 2. Hershey, PA; 3. Garnet Valley, PA. Introduction: Hereditary angioedema (HAE) is a rare, genetic condition characterized by recurrent episodes of cutaneous and/or potentially fatal mucosal angioedema. Type I HAE, due to C1 inhibitor (C1INH) deficiency, is the most common form. Typical laboratory findings include C4 level <50% of normal and C1INH protein and functional levels <30% of normal. Methods: The patient is a 2-year-old male presenting with 1 day of fever, emesis, erythematous rash, and minimal symmetric extremity edema. Influenza A testing was positive. Swelling improved in 24 hours. He has a strong family history of type I HAE. Results: Initial labs revealed C4 <8 mg/dL (9-36 mg/dL), C1INH protein 12 mg/dL (19-37 mg/dL) and normal C1INH function (ELISA). Baseline labs demonstrated normal C4 level, C1INH protein 12 mg/dL (19-37 mg/dL) and normal C1INH function (ELISA). The patient presented 5 months later with URI symptoms, emesis, and minimal pedal edema. Swelling resolved in 24 hours. Studies revealed low C4 level <8 mg/dL (11-61 mg/dL), C1INH protein 5.4 mg/dL (8-19.5 mg/dL), and C1INH function 42% (74-174%, chromogenic). Conclusion: This young patient presented with characteristic viral symptoms, indiscernible extremity swelling, and a family history of HAE. Baseline C4 was normal, C1INH protein levels were consistently >60% of normal, and C1INH function was normal on ELISA. Chromogenic testing for C1INH function provided increased sensitivity and specificity compared to the routine ELISA assay. Due to heterogeneity of presentation and potential variability in lab findings, clinicians must maintain a high index of suspicion for HAE in patients with a family history of the disorder.
P094 DEPRESSION AND ANXIETY IN HEREDITARY ANGIOEDEMA COMPARED TO PRIMARY IMMUNODEFICIENCY AND HYMENOPTERA ALLERGY G. Sabharwal*1, T. Craig2, 1. Camp Hill, PA; 2. Hershey, PA. Introduction: Hereditary angioedema (HAE) is a rare debilitating illness with recurrent attacks. Primary Immunodeficiency (PID) is a rare inherited disorder that often has significant drug burden. Hymenoptera sting allergy (HA) is associated with the fear of anaphylactic reaction. In light of above features of PID and HA, it was our objective to compare depression and anxiety associated with them to HAE. Methods: Three studies, which were IRB approved, were compared using Hamilton Depression Rating Scale (HAM-D) and the Hamilton Anxiety Rating Scale (HAM-A) to determine the extent of anxiety and depression. Results: Patients with HAE, 39% of participants were identified as having depression of mild (50%), moderate (40%) or severe (10%) levels. Fifteen percent had anxiety of mild (50%), moderate (25%) and severe (25%) levels. PID patients had a median score of 2 with upper and lower quartiles of (0,4) on HAM-D, which corresponds to normal degree of depressive symptoms. On HAM-A they had a
S49
median score of 2 with upper and lower quartiles of (1,5), which corresponds to none to mild degree of anxiety. Twenty percent of patients with HA had depression of which 50% were in epinephrine-only treatment group. Mild Anxiety was present in 93.3% patients, 5.6% showed mild-moderate anxiety and they were epinephrine users whereas only 1.1 % showed moderate to severe anxiety and were in non-treatment group. Conclusion: Depression and anxiety in HAE far exceeds that noted in PID and HA. Appropriate care of HAE should include assessment of depression and anxiety and treatment when necessary.
P095 TRANSARTERIAL CHEMOEMBOLIZATION (TACE) PROCEDURE-INDUCED DEVELOPMENT OF ACQUIRED ANGIOEDEMA T. Patel*1, S. Sur2, J. McCracken1, 1. League City, TX; 2. Galveston, TX. Introduction: Acquired angioedema (AAE) is a very rare disorder with just over 100 reported cases world-wide. AAE consists of hyper-activation of the classical complement pathway and acquired C1 inhibitor deficiency causing recurrent angioedema symptoms. Lymphoproliferative disorders have been implicated in the development of AAE. Here we present a case of a procedure-induced development of AAE without any prior or subsequent episodes of angioedema. Case: A 67 year old female with a history of hepatocellular carcinoma experienced delayed onset angioedema following TACE. The patient developed upper extremity, facial, and severe laryngeal edema 12-18 hours post-TACE procedure, and required intensive care unit admission. Initial laboratory studies revealed low levels of C3 (36 mg/dL, normal 86-184 mg/dL), C4 (2 mg/dL, normal 20-59 mg/dL ), and C1 esterase inhibitor (7 mg/dL, normal 21-39 mg/dL). Her symptoms were unresponsive to histamine receptor 1 antagonists and corticosteroid therapy, but gradually resolved over the course of 3-5 days. On repeat evaluation two months later, the patient had normalization of complement levels (C3 97 mg/dL, C4 24 mg/dL, and C1-esterase inhibitor 36 mg/dL with 115% function). Prior to TACE, the patient never experienced any angioedema symptoms, nor has she developed any subsequent episodes of angioedema. Conclusion: Given the delayed onset, absence of any previous or subsequent angioedema, low C1-inhibitor levels that normalized, and unresponsiveness to antihistamine and corticosteroid therapy, we hypothesize that the patient has AAE triggered by TACE. For future TACE procedures, the patient may benefit from administration of plasma-derived C1-inhibitor and bradykinin inhibitor for prevention and treatment of angioedema.
P096 HEREDITARY ANGIOEDEMA IN THE PUERTO RICAN POPULATION: EPIDEMIOLOGIC CHARACTERISTICS AND BURDEN OF DISEASE Y. Arce Ayala*1, E. Arroyo-Flores2, S. Nazario2, C. Ramos2, 1. Bayamon, Puerto Rico; 2. San Juan, Puerto Rico. Introduction: Hereditary angioedema (HAE) is a life-threatening condition characterized by episodes of subcutaneous or submucosal edema. Despite numerous HAE studies published in the literature, only one has been performed in Puerto Rico (PR). This study describes clinical characteristics of HAE patients, but no data regarding quality of life has ever been published in this population. The aim of this study was to assess epidemiologic characteristics and burden of this disease in the Puerto Rican population. Methods: A retrospective study was performed. Data on sociodemographic characteristics, symptoms, and treatment was collected by means of a survey administered to HAE patients. To evaluate health-related quality of life, the SF-36v2 questionnaire was used.