The Complex Interaction Between Polycystic Ovary Syndrome and Hereditary Angioedema: Case Reports and Review of the Literature

Show simple item record

dc.contributor Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.author IAHN-AUN, Marina FMUSP-HC
AUN, Marcelo Vivolo FMUSP-HC
MOTTA, Antonio Abilio FMUSP-HC
KALIL, Jorge FMUSP-HC
GIAVINA-BIANCHI, Pedro FMUSP-HC
HAYASHIDA, Sylvia Asaka FMUSP-HC
BARACAT, Edmund Chada FMUSP-HC
MACIEL, Gustavo Arantes FMUSP-HC
dc.date.issued 2017
dc.identifier.citation OBSTETRICAL & GYNECOLOGICAL SURVEY, v.72, n.7, p.417-424, 2017
dc.identifier.issn 0029-7828
dc.identifier.uri http://observatorio.fm.usp.br/handle/OPI/21258
dc.description.abstract Importance Hereditary angioedema (HAE) is a rare but severe disease, with high risk of death, and attacks have been associated to high estrogen levels. Polycystic ovary syndrome (PCOS) is a common hyperandrogenic condition, which is frequently treated with combined oral contraceptives. Objective The aim of this study was to describe 2 clinical cases of young women diagnosed as having PCOS who developed HAE attacks after the introduction of combined estrogen-progestin pills to treat PCOS symptoms. Evidence Acquisition Literature review of sex hormones' role in genesis of HAE attacks and possible mechanisms involved. Results In the cases reported, after initiation of combined contraceptives, patients presented with facial swelling with airway involvement (laryngeal edema) and abdominal pain. They had a familial history of angioedema and normal C1 inhibitor (C1-INH) levels, leading to the diagnosis of HAE with normal C1-INH (HAEnC1-INH) or HAE type III. After suspension of exogenous estrogen, patients remained asymptomatic from HAE. Conclusions and Relevance HAEnC1-INH is an estrogen-dependent form of HAE. It is well established that exogenous estrogen triggers attacks of all types of HAE. However, this is the first description of the association between PCOS and HAE, in which PCOS could be masking HAE symptoms. We propose that PCOS might have a protective role regarding HAE attacks, because of its particular hormonal features, that is, hyperandrogenism and relative stable levels of estradiol. The use of combined estrogen-progestin compounds in women with PCOS and HAE must be avoided, and treatment must be individualized. Target Audience Obstetricians and gynecologists, family physicians. Learning Objectives After completing this activity, the learner should be better able to evaluate for hereditary angioedema (HAE) in patients with recurrent angioedema attacks and abdominal pain, particularly when they are under exogenous estrogen treatment; ask patients about personal or familial history of angioedema attacks before prescribing exogenous estrogen; recall that isolated progestins can be used as long-term prophylaxis to HAE patients; and explain that hyperandrogenism of polycystic ovary syndrome could mask HAE symptoms.
dc.language.iso eng
dc.publisher LIPPINCOTT WILLIAMS & WILKINS
dc.relation.ispartof Obstetrical & Gynecological Survey
dc.rights restrictedAccess
dc.subject.other hormone replacement therapy; molecular-weight kininogen; angioneurotic-edema; c1 inhibitor; postmenopausal women; normal c1-inhibitor; contact activation; in-vivo; management; diagnosis
dc.title The Complex Interaction Between Polycystic Ovary Syndrome and Hereditary Angioedema: Case Reports and Review of the Literature
dc.type article
dc.rights.holder Copyright LIPPINCOTT WILLIAMS & WILKINS
dc.description.group LIM/19
dc.description.group LIM/58
dc.description.group LIM/60
dc.identifier.doi 10.1097/OGX.0000000000000457
dc.identifier.pmid 28715060
dc.type.category original article
dc.type.version publishedVersion
hcfmusp.author IAHN-AUN, Marina:HC:ICHC
hcfmusp.author AUN, Marcelo Vivolo:FM:
hcfmusp.author MOTTA, Antonio Abilio:HC:ICHC
hcfmusp.author KALIL, Jorge:FM:MCM
hcfmusp.author GIAVINA-BIANCHI, Pedro:FM:MCM
hcfmusp.author HAYASHIDA, Sylvia Asaka:HC:ICHC
hcfmusp.author BARACAT, Edmund Chada:FM:MOG
hcfmusp.author MACIEL, Gustavo Arantes:HC:ICHC
hcfmusp.origem.id WOS:000405330200012
hcfmusp.origem.id 2-s2.0-85025444698
hcfmusp.publisher.city PHILADELPHIA
hcfmusp.publisher.country USA
hcfmusp.relation.reference · Azziz R, 2004, J CLIN ENDOCR METAB, V89, P2745, DOI 10.1210/jc.2003-032046
· Azziz R, 2003, OBSTET GYNECOL, V101, P995, DOI 10.1016/S0029-7844(02)02725-4
· Azziz R, 2009, FERTIL STERIL, V91, P456, DOI 10.1016/j.fertnstert.2008.06.035
· Betschel S, 2014, ALLERGY ASTHMA CL IM, V10, DOI 10.1186/1710-1492-10-50
· Binkley KE, 2000, J ALLERGY CLIN IMMUN, V106, P546
· Bork K, 2003, AM J MED, V114, P294, DOI 10.1016/S0002-9343(02)01526-7
· Bork K, 2006, AM J MED, V119, P267, DOI 10.1016/j.amjmed.2005.09.064
· Bork K, 2000, LANCET, V356, P213, DOI 10.1016/S0140-6736(00)02483-1
· Bork K, 2015, ALLERGY, V70, P1004, DOI 10.1111/all.12648
· Bouillet Laurence, 2010, Allergy Asthma Clin Immunol, V6, P17, DOI 10.1186/1710-1492-6-17
· Broekmans FJ, 2006, BJOG-INT J OBSTET GY, V113, P1210, DOI 10.1111/j.1471-0528.2006.01008.x
· Caballero T, 2011, J INVEST ALLERG CLIN, V21, P333
· Caccia S, 2014, PEDIAT ALLER IMM PUL, V27, P159, DOI 10.1089/ped.2014.0425
· Christiansen SC, 2015, ALLERGY ASTHMA PROC, V36, P145, DOI 10.2500/aap.2015.36.3831
· Cicardi M, 2014, ALLERGY, V69, P602, DOI 10.1111/all.12380
· Cicardi M, 1996, NEW ENGL J MED, V334, P1666, DOI 10.1056/NEJM199606203342510
· Craig Timothy, 2012, World Allergy Organ J, V5, P182, DOI 10.1097/WOX.0b013e318279affa
· DAVIS AE, 1988, ANNU REV IMMUNOL, V6, P595
· Davis AE, 2008, MOL IMMUNOL, V45, P4057, DOI 10.1016/j.molimm.2008.06.028
· de Maat S, 2014, INT J LAB HEMATOL, V36, P374, DOI 10.1111/ijlh.12222
· Dewald G, 2006, BIOCHEM BIOPH RES CO, V343, P1286, DOI 10.1016/j.bbrc.2006.03.092
· DONALDSON VH, 1963, AM J MED, V35, P37, DOI 10.1016/0002-9343(63)90162-1
· DONALDSON VH, 1966, PEDIATRICS, V37, P1017
· Eckes B, 1999, EUR J INTERN MED S1, V10, P187
· FARSETTI A, 1995, ENDOCRINOLOGY, V136, P5076, DOI 10.1210/en.136.11.5076
· Fauser BCJM, 2004, HUM REPROD, V19, P41, DOI 10.1093/humrep/deh098
· FRANK MM, 1976, ANN INTERN MED, V84, P580
· GELFAND JA, 1976, NEW ENGL J MED, V295, P1444, DOI 10.1056/NEJM197612232952602
· Giavina-Bianchi P, 2015, CLIN EXP ALLERGY, V45, P1142, DOI 10.1111/cea.12551
· Giavina-Bianchi P, 2011, CLINICS, V66, P1627, DOI 10.1590/S1807-59322011000900021
· Goodarzi MO, 2011, NAT REV ENDOCRINOL, V7, P219, DOI 10.1038/nrendo.2010.217
· Han ED, 2002, J CLIN INVEST, V109, P1057, DOI 10.1172/JCI200214211
· Huang A, 2010, FERTIL STERIL, V93, P1938, DOI 10.1016/j.fertnstert.2008.12.138
· Jayasena CN, 2014, NAT REV ENDOCRINOL, V10, P624, DOI 10.1038/nrendo.2014.102
· Joseph K, 2013, J ALLERGY CLIN IMMUN, V132, P470, DOI 10.1016/j.jaci.2013.03.026
· Joseph K, 2009, J ALLERGY CLIN IMMUN, V124, P143, DOI 10.1016/j.jaci.2009.02.006
· LANDERMAN NS, 1962, J ALLERGY, V33, P330, DOI 10.1016/0021-8707(62)90032-1
· LEVI M, 1991, J CLIN INVEST, V88, P1155, DOI 10.1172/JCI115416
· Nussberger J, 1998, LANCET, V351, P1693, DOI 10.1016/S0140-6736(97)09137-X
· Osler W, 1888, AM J MED SCI, V95, P362, DOI 10.1097/00000441-188804000-00004
· PERRICONE R, 1992, CLIN EXP IMMUNOL, V90, P401
· PROUDLER AJ, 1995, LANCET, V346, P89, DOI 10.1016/S0140-6736(95)92114-1
· Renne T, 2005, J IMMUNOL, V175, P3377
· Riedl MA, 2013, J ALLER CL IMM-PRACT, V1, P427, DOI 10.1016/j.jaip.2013.06.004
· ROSEN FS, 1971, J CLIN INVEST, V50, P2143, DOI 10.1172/JCI106708
· SCHAPIRA M, 1983, NEW ENGL J MED, V308, P1050, DOI 10.1056/NEJM198305053081802
· Schunkert H, 1997, CIRCULATION, V95, P39
· Stein IF, 1935, AM J OBSTET GYNECOL, V29, P181
· Yip J, 1992, Australas J Dermatol, V33, P35, DOI 10.1111/j.1440-0960.1992.tb00050.x
· Zawadski JK, 1992, POLYCYSTIC OVARY SYN, P377
· Zuberbier T, 2014, ALLERGY, V69, P868, DOI 10.1111/all.12313
· Zuraw BL, 2008, NEW ENGL J MED, V359, P1027, DOI 10.1056/NEJMcp0803977
dc.description.index MEDLINE
dc.identifier.eissn 1533-9866
hcfmusp.citation.scopus 1
hcfmusp.scopus.lastupdate 2021-07-14


Files in this item

This item appears in the following Collection(s)

Show simple item record

Search DSpace



Browse

My Account

Statistics