Obwodzenie
Etiologia i przyczyny

Obrzęk naczynioruchowy (obwodzenie) to stan charakteryzujący się obrzękiem tkanek głęboko pod skórą lub błonami śluzowymi, wynikający ze zwiększonej przepuszczalności naczyń i wycieku płynu do tkanek. Etiologia dzieli się na obrzęk mediowany histaminą (alergiczny, często z pokrzywką) oraz bradykininą (niedobór/dysfunkcja inhibitora C1 esterazy lub leki, np. inhibitory ACE). Klinicznie wyróżnia się obrzęk alergiczny, idiopatyczny, polekowy (ok. 0,1-2,5% pacjentów na inhibitorach ACE) i dziedziczny (HAE, często mutacje w genie SERPING1 lub F12). Alergiczny obrzęk pojawia się zwykle w ciągu 30 min do 2 h po ekspozycji na alergen (orzechy, owoce morza, jaja, mleko, soja, pszenica, czekolada, pyłki, sierść, lateks, czynniki fizyczne, stres). Obrzęk polekowy najczęściej wywołują inhibitory ACE, NLPZ, antybiotyki, ARB i inne leki, z mechanizmem opartym na nadprodukcji bradykininy lub leukotrienów.

Etiologia obrzęku naczynioruchowego (obwodzenia)

Obrzęk naczynioruchowy, w języku polskim określany również jako obwodzenie, to stan charakteryzujący się obrzękiem tkanek położonych głęboko pod skórą lub pod błonami śluzowymi. Mechanizm powstawania obrzęku wiąże się z zwiększoną przepuszczalnością naczyń krwionośnych i wyciekiem płynu z małych naczyń do otaczających tkanek, co powoduje obrzęk12. Etiologia obrzęku naczynioruchowego jest złożona i może być spowodowana różnymi czynnikami w zależności od typu schorzenia.

Klasyfikacja przyczyn obrzęku naczynioruchowego

Przyczyny obrzęku naczynioruchowego można sklasyfikować według mechanizmu patofizjologicznego na dwie główne kategorie12:

  1. Obrzęk mediowany histaminą – związany z reakcjami alergicznymi, często współwystępuje z pokrzywką
  2. Obrzęk mediowany bradykininą – niezależny od histaminy, związany z niedoborem lub dysfunkcją inhibitora C1 esterazy lub stosowaniem niektórych leków

Podział kliniczny obrzęku naczynioruchowego

Ze względów klinicznych obrzęk naczynioruchowy dzieli się na cztery główne typy12:

  • Obrzęk alergiczny – spowodowany reakcją alergiczną, często współwystępujący z anafilaksją
  • Obrzęk idiopatyczny – bez znanej przyczyny, choć czynniki takie jak stres czy infekcja mogą wyzwalać objawy
  • Obrzęk polekowy – występujący jako skutek uboczny stosowania niektórych leków, najczęściej inhibitorów konwertazy angiotensyny (ACE)
  • Obrzęk dziedziczny – spowodowany defektem genetycznym dziedziczonym od rodziców

Obrzęk alergiczny – przyczyny i mechanizmy

Najczęstszą przyczyną obrzęku naczynioruchowego są reakcje alergiczne12. W tym typie obrzęku układ odpornościowy błędnie rozpoznaje nieszkodliwe substancje jako zagrożenie i uruchamia reakcję zapalną. Dochodzi wówczas do produkcji histaminy, która powoduje rozszerzenie naczyń krwionośnych w danym obszarze, prowadząc do obrzęku skóry1.

Alergeny pokarmowe

Wśród najczęstszych pokarmowych przyczyn alergicznego obrzęku naczynioruchowego znajdują się123:

  • Orzechy (zwłaszcza orzechy ziemne i drzewne)
  • Owoce morza i ryby
  • Jajka
  • Mleko
  • Soja
  • Pszenica
  • Czekolada

Czynniki środowiskowe

Obrzęk alergiczny może być również spowodowany przez12:

  • Pyłki roślin
  • Sierść zwierząt i łupież naskórkowy
  • Ukąszenia i użądlenia owadów
  • Lateks
  • Czynniki fizyczne (zimno, ciepło, nacisk, wibracje, promieniowanie UV)
  • Stres fizyczny (intensywny wysiłek)

Alergiczny obrzęk naczynioruchowy pojawia się zazwyczaj w ciągu 30 minut do 2 godzin po ekspozycji na alergen1. Często towarzyszy mu pokrzywka i świąd skóry, co może pomóc w rozróżnieniu go od innych typów obrzęku naczynioruchowego2.

Obrzęk polekowy – przyczyny i mechanizmy

Obrzęk naczynioruchowy może być również wywołany przez leki, zarówno poprzez mechanizm alergiczny, jak i niealergiczny1. Reakcje polekowe stanowią około 30% przypadków ostrego obrzęku naczynioruchowego obserwowanych na oddziałach ratunkowych1.

Inhibitory konwertazy angiotensyny (ACE)

Najczęstszą przyczyną polekowego obrzęku naczynioruchowego są inhibitory ACE stosowane w leczeniu nadciśnienia tętniczego i niewydolności serca12. Mechanizm powstawania obrzęku związany jest z bezpośrednim zakłóceniem degradacji bradykininy, co wzmacnia jej działanie naczyniowo aktywne1. Szacuje się, że obrzęk naczynioruchowy występuje u około 0,1-1,0% pacjentów przyjmujących inhibitory ACE1, a według niektórych źródeł nawet u 2,5% osób stosujących te leki1. Najczęściej dotyka twarzy i górnych dróg oddechowych, ale może również wpływać na jelita1.

Inne leki wywołujące obrzęk naczynioruchowy

Poza inhibitorami ACE, obrzęk naczynioruchowy mogą wywoływać123:

  • Niesteroidowe leki przeciwzapalne (NLPZ), w tym aspiryna, ibuprofen i naproksen
  • Antybiotyki (szczególnie penicyliny i sulfonamidy)
  • Blokery receptorów angiotensyny (ARB)
  • Leki zwiotczające mięśnie
  • Opiaty
  • Środki fibrynolityczne
  • Blokery kanałów wapniowych

NLPZ mogą wywoływać obrzęk naczynioruchowy poprzez hamowanie cyklooksygenazy-1 (COX-1), co prowadzi do nadprodukcji leukotrienów1. Reakcje na NLPZ często występują w ciągu godziny od przyjęcia leku1.

Obrzęk dziedziczny – przyczyny genetyczne

Dziedziczny obrzęk naczynioruchowy (HAE) jest rzadkim schorzeniem genetycznym, występującym u około 1 na 50 000 osób12. Jest to choroba dziedziczona autosomalnie dominująco, co oznacza, że dzieci mają 50% szans na odziedziczenie schorzenia, jeśli jedno z rodziców jest dotknięte tą chorobą12.

Typy dziedzicznego obrzęku naczynioruchowego

Wyróżnia się trzy główne typy dziedzicznego obrzęku naczynioruchowego12:

  • HAE typu I – stanowi około 80-85% przypadków HAE, charakteryzuje się znacznie obniżonym poziomem białka inhibitora C1 esterazy z powodu wadliwego genu na chromosomie 11 (SERPING1)
  • HAE typu II – stanowi około 15-20% przypadków HAE, charakteryzuje się prawidłowym lub podwyższonym poziomem inhibitora C1, który jednak nie funkcjonuje prawidłowo
  • HAE typu III – niezwykle rzadka forma choroby, w której poziom i funkcja inhibitora C1 są prawidłowe, ale występują mutacje w innych genach, np. genie F12 kodującym czynnik krzepnięcia XII

Mechanizm genetyczny HAE

Dziedziczny obrzęk naczynioruchowy typu I i II jest spowodowany mutacjami w genie SERPING1, który koduje inhibitor C1 esterazy (C1-INH)12. Białko to odgrywa kluczową rolę w regulacji układu dopełniacza, kaskady kinin-kalikreiny i kaskady krzepnięcia. Brak lub dysfunkcja inhibitora C1 prowadzi do nadmiernej produkcji bradykininy, która powoduje rozszerzenie naczyń krwionośnych i zwiększenie ich przepuszczalności12.

W przypadku HAE typu III, mutacje w genie F12 powodują, że białko czynnika XII jest łatwiejsze do aktywacji niż normalnie1. Prowadzi to do nadmiernej aktywacji układu kinin i nadprodukcji bradykininy1.

Co istotne, mimo uwarunkowań genetycznych, około 25% przypadków HAE wynika ze spontanicznej mutacji genu inhibitora C1 w momencie poczęcia, bez wcześniejszej historii rodzinnej1.

Czynniki wyzwalające ataki HAE

Ataki dziedzicznego obrzęku naczynioruchowego mogą być wywołane przez123:

  • Łagodne urazy (np. zabiegi stomatologiczne)
  • Stres emocjonalny
  • Infekcje (w tym przeziębienia i grypa)
  • Zabiegi chirurgiczne
  • Menstruację
  • Leki zawierające estrogeny (w tym antykoncepcja hormonalna)
  • Inhibitory ACE
  • Ekspozycję na zimno

W przypadku dziedzicznego obrzęku naczynioruchowego, ataki mogą trwać od 2 do 5 dni i często nie mają znanej przyczyny1. W odróżnieniu od obrzęku alergicznego, zazwyczaj nie towarzyszy mu pokrzywka ani świąd1.

Obrzęk nabyty (AAE) – przyczyny i mechanizmy

Nabyty obrzęk naczynioruchowy (AAE) związany z niedoborem inhibitora C1 jest bardzo rzadkim schorzeniem1. W przeciwieństwie do formy dziedzicznej, rozwija się on w trakcie życia, a nie jest dziedziczony1.

Typy nabytego obrzęku naczynioruchowego

Wyróżnia się dwa typy nabytego obrzęku naczynioruchowego12:

  • Typ 1 – związany z chorobami limfoproliferacyjnymi lub nowotworowymi
  • Typ 2 – związany z obecnością autoprzeciwciał przeciwko inhibitorowi C1

Choroby związane z nabytym obrzękiem naczynioruchowym

Nabyty obrzęk naczynioruchowy najczęściej występuje w związku z123:

  • Zaburzeniami limfoproliferacyjnymi, takimi jak:
    • Chłoniak złośliwy (zwłaszcza chłoniak strefy brzeżnej śledziony)
    • Przewlekła białaczka limfocytowa
    • Gammapatia monoklonalna o nieokreślonym znaczeniu (MGUS)
    • Makroglobulinemia Waldenströma
  • Chorobami autoimmunologicznymi, takimi jak:
    • Toczeń rumieniowaty układowy (SLE)
    • Zapalenia stawów o podłożu zapalnym
  • Innymi schorzeniami:
    • Choroby tarczycy
    • Różnego rodzaju nowotwory

W AAE komórki chłoniaka mogą zubażać lub powodować zużycie inhibitora C1, a możliwość leczenia obrzęku naczynioruchowego poprzez wyleczenie podstawowego chłoniaka wiąże chorobę limfoproliferacyjną z patogenezą AAE1. Co ciekawe, objawy kliniczne obrzęku naczynioruchowego mogą poprzedzać rozpoznanie choroby limfoproliferacyjnej nawet o wiele lat1.

Mechanizm patofizjologiczny AAE jest podobny do HAE – niedobór funkcjonalnego inhibitora C1 prowadzi do nadmiernej produkcji bradykininy, co skutkuje zwiększoną przepuszczalnością naczyń i obrzękiem1.

Obrzęk idiopatyczny – przyczyny i czynniki ryzyka

Idiopatyczny obrzęk naczynioruchowy to typ, w którym nie można zidentyfikować konkretnej przyczyny obrzęku1. Według niektórych badań ponad 40% przypadków przewlekłego obrzęku naczynioruchowego ma charakter idiopatyczny1.

Potencjalne czynniki wyzwalające

Chociaż dokładna przyczyna idiopatycznego obrzęku naczynioruchowego pozostaje nieznana, zidentyfikowano kilka czynników, które mogą wyzwalać lub nasilać epizody123:

  • Stres emocjonalny
  • Infekcje (szczególnie wirusowe – najczęstsza przyczyna pokrzywki i obrzęku naczynioruchowego u dzieci)
  • Urazy
  • Zabiegi chirurgiczne
  • Ciąża
  • Tabletki antykoncepcyjne
  • Intensywny wysiłek fizyczny
  • Ekstremalne warunki temperatury (zimno, gorąco)

Mechanizm idiopatycznego obrzęku naczynioruchowego

Dokładny mechanizm powstawania idiopatycznego obrzęku naczynioruchowego nie jest w pełni zrozumiały, choć podejrzewa się niespecyficzną aktywację i degranulację komórek tucznych1. W niektórych przypadkach może wynikać z problemu z układem odpornościowym, który czasami błędnie się uruchamia1.

U niektórych osób, u których obrzęki utrzymują się przez lata bez wyraźnej przyczyny, odpowiedzialny może być proces autoimmunologiczny1. Badania wskazują, że 30-50% przypadków tego typu obrzęku naczynioruchowego może być związanych z chorobami autoimmunologicznymi, w tym z toczniem rumieniowatym układowym (SLE)1.

W przypadku przewlekłego idiopatycznego obrzęku naczynioruchowego (utrzymującego się ponad 6 tygodni), przyczyną może być również nieumyślne, powtarzające się spożywanie substancji, takich jak penicylina w mleku lub konserwanty czy barwniki w żywności1.

Inne przyczyny obrzęku naczynioruchowego

Infekcje jako przyczyna obrzęku naczynioruchowego

Infekcje mogą wyzwalać obrzęk naczynioruchowy u niektórych osób1. Do najczęstszych należą12:

  • Infekcje dróg oddechowych (np. paciorkowcowe zapalenie gardła)
  • Infekcje dróg moczowych
  • Choroby wirusowe:
    • Mononukleoza zakaźna
    • Zakażenia wirusem cytomegalii
    • Zakażenia wirusem Epsteina-Barr
    • Wirusowe zapalenie wątroby typu B i C
    • Przewlekłe zakażenia wirusowe
  • Zakażenia bakteryjne, grzybicze i pasożytnicze

Szczególnie u dzieci infekcje wirusowe są najczęstszą przyczyną pokrzywki i obrzęku naczynioruchowego trwającego dłużej niż 24 godziny1.

Obrzęk naczynioruchowy indukowany czynnikami fizycznymi

Czynniki fizyczne mogą wywoływać obrzęk naczynioruchowy poprzez bezpośrednie działanie na skórę i naczynia krwionośne1. Do najczęstszych należą12:

  • Zimno
  • Ciepło
  • Nacisk
  • Wibracje
  • Promieniowanie ultrafioletowe
  • Kontakt z wodą

Obrzęk naczynioruchowy w chorobach układu pokarmowego

Obrzęk naczynioruchowy może również dotyczyć układu pokarmowego, powodując silne bóle brzucha i mogąc prowadzić do niepotrzebnej laparotomii1. Jest to szczególnie częste w dziedzicznym i nabytym obrzęku naczynioruchowym1.

Rzadkie przyczyny obrzęku naczynioruchowego

Do rzadszych przyczyn obrzęku naczynioruchowego należą12:

  • Zespół cytokino-zależnego obrzęku naczynioruchowego (zespół Gleicha lub epizodyczny obrzęk naczynioruchowy z eozynofilią)
  • Zespół Wellsa lub eozynofilowe zapalenie tkanki łącznej (ziarniniakowe zapalenie skóry z eozynofilią)
  • Spożycie pokarmów, które same są wazodylatatorami, takich jak napoje alkoholowe czy cynamon – może zwiększać prawdopodobieństwo epizodu obrzęku naczynioruchowego u podatnych pacjentów1

Podsumowanie przyczyn obrzęku naczynioruchowego

Etiologia obrzęku naczynioruchowego jest złożona i obejmuje szerokie spektrum czynników – od reakcji alergicznych, przez działania niepożądane leków, po rzadkie zaburzenia genetyczne i choroby autoimmunologiczne1. Zrozumienie przyczyn jest kluczowe dla właściwego leczenia i zapobiegania nawrotom obrzęku naczynioruchowego.

W praktyce klinicznej identyfikacja konkretnej przyczyny może być trudna, szczególnie w przypadkach przewlekłego obrzęku naczynioruchowego, gdzie przyczyna pozostaje nieustalona u znacznego odsetka pacjentów1. Niemniej jednak dokładna diagnostyka, uwzględniająca wywiad, badanie fizykalne i odpowiednie badania laboratoryjne, może pomóc w ustaleniu etiologii i wdrożeniu odpowiedniego leczenia1.

Warto podkreślić, że różne typy obrzęku naczynioruchowego wymagają odmiennego podejścia terapeutycznego. Przykładowo, obrzęk indukowany inhibitorami ACE wymaga odstawienia leku i nie reaguje na standardowe leczenie przeciwhistaminowe, sterydowe czy adrenalinę, ponieważ jest mediowany przez bradykininę, a nie histaminę12.

Kolejne rozdziały

Zapraszamy do dalszego czytania naszego leksykonu.

Wybierz kolejny rozdział z menu poniżej, aby otworzyć nową podstronę kompedium wiedzy i uzyskać szczegółowe informację o leku, substancji lub chorobie.

  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Angioedema: Causes, Symptoms, Types & Treatments
    https://my.clevelandclinic.org/health/diseases/22632-angioedema
    Angioedema is a reaction to a trigger that causes swelling in the tissue below the inner layer of your skin called the dermis or the layer below a mucous membrane. Angioedema often happens at the same time as hives (urticaria) and for similar reasons. Both angioedema and hives happen when liquid from small blood vessels escapes and fills up tissues, causing swelling. […] The causes of angioedema depend on what type of angioedema you have. Allergies are probably the main cause of the swelling of angioedema. There are many types of allergies that can cause it, including: […] Other causes of angioedema include inherited and acquired problems with the C1 inhibitor protein, drug reactions that arent standard allergy reactions (theres no itchiness and no hives) and vibrational movements.
  • #1 Angioedema – Wikipedia
    https://en.wikipedia.org/wiki/Angioedema
    Angioedema is an area of swelling (edema) of the lower layer of skin and tissue just under the skin or mucous membranes. The underlying mechanism typically involves histamine or bradykinin. The version related to histamine is due to an allergic reaction to agents such as insect bites, foods, or medications. The version related to bradykinin may occur due to an inherited problem known as C1 esterase inhibitor deficiency, medications known as angiotensin-converting enzyme inhibitors, or a lymphoproliferative disorder. […] In hereditary angioedema (HAE), often no direct cause is identifiable, although mild trauma, including dental work and other stimuli, can cause attacks. There is usually no associated itch or urticaria, as it is not an allergic response. […] Bradykinin plays a critical role in all forms of hereditary angioedema. Various mechanisms that interfere with bradykinin production or degradation can lead to angioedema. ACE inhibitors block ACE, the enzyme that among other actions, degrades bradykinin. In hereditary angioedema, bradykinin formation is caused by continuous activation of the complement system due to a deficiency in one of its prime inhibitors, C1-esterase.
  • #1 Angioedema | NHS inform
    https://www.nhsinform.scot/illnesses-and-conditions/skin-hair-and-nails/angioedema/
    The cause of angioedema depends on the type you have. There are 4 main types of angioedema: […] allergic angioedema the swelling is caused by an allergic reaction, such as a reaction to peanuts, and sometimes occurs in combination with anaphylaxis […] idiopathic angioedema there is no known cause for the swelling (although certain factors, such as stress or infection, may trigger the symptoms) […] drug-induced angioedema the swelling is a side effect of certain medications, most often angiotensin-converting enzyme (ACE) inhibitors […] hereditary angioedema the swelling is caused by faulty genes that are inherited from a persons parents. […] Angioedema is often caused by a problem with the immune system, although there are many cases where no cause can be identified. […] In allergic angioedema, your immune system mistakenly attacks harmless substances in your blood. Your body produces the chemical histamine, which causes the blood vessels in the area to expand, leading to swelling of the skin.
  • #1 Hives and angioedema – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/hives-and-angioedema/symptoms-causes/syc-20354908
    Angioedema can arise with hives or alone. It causes swelling in the deeper layers of skin, often around the face and lips. […] For most people who experience acute hives and angioedema, the exact cause can’t be identified. The conditions are sometimes caused by: […] Many foods can trigger reactions in people with sensitivities. Shellfish, fish, peanuts, tree nuts, soy, eggs and milk are frequent offenders. […] Many medications may cause hives or angioedema, including penicillins, aspirin, ibuprofen (Advil, Motrin IB, others), naproxen sodium (Aleve) and blood pressure medications. […] Other causes of acute hives and angioedema are insect bites and infections. […] Severe angioedema can be life-threatening if swelling of the tongue or in the throat blocks the airway.
  • #1 Angioedema Symptoms & Treatment | Frontier Allergy Austin
    https://www.frontierallergist.com/conditions/angioedema/
    Angioedema can have a variety of causes, including: […] Food allergies. Foods such as shellfish, fish, peanuts, tree nuts, eggs, dairy, soy, and wheat are common offenders. […] Medication allergies or medical treatments. Medications such as penicillin, aspirin, ibuprofen, naproxen sodium, and certain blood pressure medications are common triggers. Medical treatments such as blood transfusions may cause angioedema. […] Airborne allergens, such as pollen or other allergens that are in the air, can trigger an allergic reaction including angioedema. […] Environmental factors, such as sunlight, vibration or pressure on the skin, hot showers or baths, stress, insect bites, or exercise. […] Underlying conditions, such as infections from bacteria or viruses. […] Sometimes, no specific cause can be identified. In this case, it is known as idiopathic angioedema.
  • #1 Angioedema: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/885100-overview
    More than 40% of chronic angioedema is idiopathic. Trauma, surgical procedures, and stress are common nonspecific triggers for angioedema attacks. […] Angioedemas with identifiable etiologies include those caused by the following: Hypersensitivity (eg, food, drugs, or insect stings), Physical stimuli (eg, cold or vibrations), Autoimmune disease or infection, ACE inhibitors, NSAIDs, C1-INH deficiency (hereditary and acquired). […] Angioedema has also been associated with certain conditions or syndromes, such as the following: Cytokine-associated angioedema syndrome (ie, Gleich syndrome or episodic angioedema with eosinophilia), Well syndrome or eosinophilic cellulitis (ie, granulomatous dermatitis with eosinophilia). […] Hypersensitivity (allergic) angioedema is often associated with urticaria. It is typically observed within 30 minutes to 2 hours after exposure to the allergen.
  • #1 Drug-induced nonallergic angioedema | Angioedema NewsEnvelope icon
    https://angioedemanews.com/non-allergic-angioedema/
    Drug-induced nonallergic angioedema is a type of angioedema that occurs due to an adverse reaction to certain medications. […] Drug-induced nonallergic angioedema is caused by an adverse reaction to certain medications. […] Specifically, acute allergic angioedema is triggered by an aberrant immune reaction against a harmless substance that’s wrongfully recognized as a potential threat. By contrast, the immune system is not activated in drug-induced nonallergic angioedema. Instead, this form of angioedema typically arises when certain medications lead to higher than normal levels of bradykinin, a signaling molecule that promotes swelling. […] Several different types of medications can act as drug-induced nonallergic angioedema causes. The most notable are angiotensin-converting enzyme (ACE) inhibitors, which are used to treat high blood pressure.
  • #1 Angioedema – Immunology; Allergic Disorders – Merck Manual Professional Edition
    https://www.merckmanuals.com/professional/immunology-allergic-disorders/allergic-autoimmune-and-other-hypersensitivity-disorders/angioedema
    Angioedema is usually acute but sometimes is a chronic mast cell-mediated reaction caused by exposure to a medication (eg, angiotensin-converting enzyme inhibitors), venom, dietary, pollen, or animal dander allergens, or it can be idiopathic. […] Several causes (eg, calcium channel blockers, fibrinolytic agents) have no identified mechanism; sometimes a cause (eg, muscle relaxants) with a known mechanism is overlooked clinically. […] Angiotensin-converting enzyme (ACE) inhibitors cause about 30% of cases of acute angioedema seen in emergency departments. […] The cause of chronic ( 6 weeks) angioedema is usually unknown. […] A few cases are due to hereditary or acquired C1 inhibitor deficiency. […] Hereditary angioedema and acquired angioedema are disorders that are characterized by abnormal complement responses and caused by deficiency or dysfunction of C1 inhibitor.
  • #1 Acquired Angioedema – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK430889/
    Angioedema is characterized by one or more areas of well-demarcated, non-pitting edema of deep subcutaneous tissues. […] There are several etiologies of angioedema, most notably angiotensin-converting enzyme inhibitor (ACEI) use. With tens of millions of people taking ACEIs, the incidence of ACEI angioedema is rising. […] Other etiologies include IgE-mediated allergic reactions (such as to food, drug, or environmental triggers), nonsteroidal anti-inflammatory drug (NSAID) use (including aspirin), and chemically-induced histamine release (most commonly opiates, highly cationic antibiotics, and muscle relaxants). […] A less common cause is hereditary angioedema (HAE) and acquired angioedema (AAE), both caused by a C1-inhibitor (C1-INH) deficiency. […] AAE is due to an acquired deficiency of (C1-INH), caused by either consumption (type 1) or inactivation (type 2). […] This increased catabolism can be related to an autoimmune disorder (e.g., systemic lupus erythematous) or a malignant tumor (e.g., lymphoma). […] Acquired angioedema may have underlying autoimmune connective tissue disease or a lymphoproliferative disorder.
  • #1 Angioedema: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/885100-overview
    Pseudoallergic angioedema (PAE) is not mediated by IgE; that is, the angioedema is caused by a nonallergic or nonimmunologic reaction. […] Nonallergic angioedema does not involve IgE or histamine and is generally not associated with urticaria. […] Hereditary angioedema (HAE), a rare autosomal dominant disorder, is perhaps the prototype of nonallergic angioedema. […] Acquired angioedema, now known as C1-INH-AAE is a rare disorder caused by accelerated consumption of C1-INH or the production of autoantibodies to C1-INH. […] ACE inhibitors can precipitate attacks of angioedema by directly interfering with the degradation of bradykinin, thereby potentiating its vasoactive effect. […] Physically induced angioedema is caused by physical agents, such as cold, heat pressure, vibration, and ultraviolet radiation. […] The causes of idiopathic angioedema are, by definition, not identifiable. Furthermore, the exact mechanisms are unclear, though nonspecific mast cell activation and degranulation are suspected. […] The link between infection and angioedema is vague at best.
  • #1 Radiologic manifestations of angioedema | Insights into Imaging | Full Text
    https://insightsimaging.springeropen.com/articles/10.1007/s13244-014-0329-1
    The majority of cases of angioedema are idiopathic. When a trigger is identified, most often it is a medication, allergen, or a physical agent such as pressure or cold. Many medications can cause angioedema but those most commonly implicated are angiotensin-converting enzyme (ACE) inhibitors and nonsteroidal anti-inflammatory drugs (NSAIDs. A less common, but important cause of angioedema is deficiency or inactivation of C1 esterase inhibitor (C1-INH) in the complement system, which can be either hereditary or acquired. Hereditary angioedema (HAE) is autosomal dominant, and is classified into three types. Acquired angioedema (AAE), a very rare condition is associated mainly with lymphoproliferative disorders and autoimmune diseases. The mechanism of angioedema is the overproduction or failure to inactivate vasoactive stimulants such as histamine, bradykinin, and leukotrienes, which lead to increased vascular permeability. Allergen-induced angioedema is due to histamine mediated by immunoglobulin E (IgE). ACE inhibitor-induced angioedema is not entirely understood, but it is postulated that the use of ACE inhibitors increases bradykinin levels, which is a potent vasodilator. Given the estimated incidence of ACE inhibitor-induced angioedema of 0.1 to 1.0 % and the large number of patients on ACE inhibitors, one theory is that affected individuals have an underlying partial deficiency of C-1 esterase. NSAIDs inhibit cyclooxygenase 1 (Cox 1), which results in overproduction of leukotrienes. HAE types 1 and 2 and AAE (deficiency or inactivation of C1-INH) are characterised by uncontrolled complement activation and resultant increased bradykinin production. In addition, a rare form of HAE (type 3) mainly noted in female patients is associated with a mutation in the coagulation factor XII gene, which also ultimately results in increased levels of bradykinin. Angioedema attacks can last from 1 to 5 days depending on the causes and symptoms. Importantly, acute attacks of HAE and AAE do not respond to epinephrine, antihistamines, or steroid. Consequently, treatment is often mainly supportive, consisting of intravenous fluids and upper airway management. Allergen and drug induced angioedema are treated with elimination of causative agents. For acute attacks of HAE and AAE, C1-INH concentrate, kallikrein inhibitor ecallantide, bradykinin B2 antagonist icatibant, or fresh frozen plasma are administered. Angioedema involving the gastrointestinal tract may present as an acute abdominal pain resulting in unnecessary laparotomy.
  • #1 Drug-induced nonallergic angioedema | Angioedema NewsEnvelope icon
    https://angioedemanews.com/non-allergic-angioedema/
    Drug-induced nonallergic angioedema is estimated to occur in as many as 2.5% of people who take these drugs. […] Another class of blood pressure-lowering medicines that can also cause drug-induced nonallergic angioedema is angiotensin receptor blockers, or ARBs. […] In some cases, other types of medications, including nonsteroidal anti-inflammatory drugs — ones commonly used to ease inflammation and pain — can also trigger drug-induced nonallergic angioedema.
  • #1 Angioedema – Immune Disorders – Merck Manual Consumer Version
    https://www.merckmanuals.com/home/immune-disorders/allergic-reactions-and-other-hypersensitivity-disorders/angioedema
    Angioedema can be a reaction to a medication or other substance (trigger), a hereditary disorder, a rare complication of cancer, or an immune disorder, but sometimes the cause is not known. […] There are rare hereditary or acquired forms of angioedema. These forms are caused by a deficiency or malfunction of a substance called C1 inhibitor, which is part of the immune system. […] Angioedema can be chronic, recurring over weeks or months. The cause is usually unknown, but it may be repeated, unintentional intake of a substance, such as penicillin in milk or a preservative or dye in foods. Use of certain medications, such as aspirin, other NSAIDs, or opioids, can also cause chronic angioedema. […] However, usually when angioedema without hives recurs, no specific cause is identified. Angioedema with no identified cause is called idiopathic angioedema. […] Angioedema without hives is sometimes caused by ACE inhibitors, which are used to treat high blood pressure and heart failure. When ACE inhibitors are the cause, the face and upper airways are most commonly affected, but the intestine may be affected.
  • #1 Angioedema: Symptoms, Causes, Treatment, Types
    https://www.webmd.com/skin-problems-and-treatments/angioedema-overview
    Angioedema is swelling beneath your skin. […] There are four types of angioedema, and they all have different causes. […] Allergic angioedema: This is the most common kind. Your allergic reaction can be to foods, like: […] Drug-induced angioedema: Some medicines can trigger angioedema. […] Certain blood pressure medicines called angiotensin-converting enzyme (ACE) inhibitors can also cause flare-ups that can happen quickly. […] Hereditary angioedema (HAE): This is rare. It happens when your body doesnt make enough of a blood protein called C1 esterase inhibitor. […] Theres another very rare type called acquired angioedema that has the same symptoms as HAE. […] Idiopathic angioedema: Idiopathic means there isnt a known cause for your swelling. […] Possible culprits can include: […] Allergic and drug-induced angioedema usually happen within an hour of exposure to your trigger. Hereditary and acquired types usually happen over many hours, but it can feel much faster if you wake up and suddenly discover swelling.
  • #1 Angioedema – Australasian Society of Clinical Immunology and Allergy (ASCIA)
    https://www.allergy.org.au/patients/skin-allergy/angioedema
    Angioedema is a condition where small blood vessels leak fluid into the tissues under the skin, causing swelling in different parts of the body. It can develop because of an allergic reaction and is usually accompanied by hives (urticaria). […] A viral infection is usually the most common cause of hives (urticaria) and angioedema in children, especially if they last for more than 24 hours. […] Swellings due to allergic reactions to foods or drugs are sometimes severe and dramatic, but usually resolve within 24 hours. […] Around 1 in 200 people who take these medications for heart and blood pressure, develop angioedema, usually appearing within the first few months of treatment. […] HAE is a rare genetic condition occurring in approximately 1 in 50,000 people, who have low levels (deficiency) or reduced effectiveness of C1-inhibitor enzyme.
  • #1 US Hereditary Angioedema Association
    https://www.haea.org/pages/p/what_is_hae
    Hereditary Angioedema, or HAE, is a very rare and potentially life-threatening genetic condition that involves recurrent attacks of severe swelling (angioedema) in various parts of the body, including the hands, feet, genitals, stomach, face and/or throat. […] The vast majority of people with HAE have a defect in the gene that controls a blood protein called C1-Inhibitor. This defect causes a biochemical imbalance that produces swelling. HAE is also known as C1-Inhibitor Deficiency – Type I and Type II. […] HAE is hereditary and children have a 50% chance of inheriting HAE if one of their parents has the condition. However, the absence of a family history does not rule out the diagnosis of HAE, as scientific reports indicate that as many as 25% of HAE cases result from a spontaneous mutation of the C1-Inhibitor gene at conception.
  • #1 Hereditary angioedema: MedlinePlus GeneticsLock
    https://medlineplus.gov/genetics/condition/hereditary-angioedema/
    Hereditary angioedema is a disorder characterized by recurrent episodes of severe swelling (angioedema). […] Hereditary angioedema is broadly divided into two types, which are distinguished by levels of a protein called C1 inhibitor (C1-INH) in the blood. […] Variants (also called mutations) in the SERPING1 gene cause hereditary angioedema due to C1-INH deficiency. […] SERPING1 gene variants that cause hereditary angioedema due to C1-INH deficiency type I lead to reduced levels of C1-INH in the blood, while variants that cause type II result in the production of a C1-INH protein that functions abnormally. […] When the body does not have enough normal C1-INH protein, it ends up with excessive amounts of a protein fragment (peptide) called bradykinin. […] Variants in the F12 gene cause most cases of hereditary angioedema with normal C1-INH.
  • #1 Angioedema: Causes, Appearance and Treatment — DermNet
    https://dermnetnz.org/topics/angioedema
    Decreased C1 inhibitor activity leads to excessive kallikrein, which in turn produces bradykinin, a potent vasodilator. […] Acquired during life rather than inherited. […] May be due to B-cell lymphoma or antibodies against C1 inhibitor. […] Associated with lymphoproliferative and autoimmune disease e.g. SLE.
  • #1 Hereditary angioedema: MedlinePlus GeneticsLock
    https://medlineplus.gov/genetics/condition/hereditary-angioedema/
    In people with hereditary angioedema with normal C1-INH, variants in the F12 gene cause cells to produce a factor XII protein that is easier to activate than normal. […] Variants in a few other genes are responsible for a small number of additional cases of hereditary angioedema with normal C1-INH.
  • #1 What Is Hereditary Angioedema?
    https://www.icliniq.com/articles/genetic-disorders/hereditary-angioedema
    Type III: F12 gene mutation is associated with type III hereditary angioedema. This gene acts as an instructor in making the coagulation factor XII protein. Coagulation factor XII plays an essential role in the blood clotting mechanism and the production of bradykinin. Bradykinin is a peptide that promotes inflammation. With the genetic mutation in F12, the coagulation factor production increases, which leads to increased bradykinin production. This results in leaky blood vessels and gives rise to inflammatory episodes in the body.
  • #1 Hereditary angioedema – UF Health
    https://ufhealth.org/conditions-and-treatments/hereditary-angioedema
    Hereditary angioedema (HAE) is caused by a low level or improper function of a protein called the C1 inhibitor. It affects the blood vessels. An HAE attack can result in rapid swelling of the hands, feet, limbs, face, intestinal tract, larynx (voicebox), or trachea (windpipe). […] There is usually a family history of the condition. But relatives may be unaware of previous cases, which may have been reported as an unexpected, sudden, and premature death of a parent, aunt, uncle, or grandparent. […] Dental procedures, sickness (including colds and the flu), and surgery may trigger HAE attacks.
  • #1 Angiodema: What It Is, Symptoms, and Treatments
    https://www.verywellhealth.com/angioedema-overview-83233
    Angioedema is often caused by an allergy but can also be triggered by a non-allergic drug reaction, an infection, cancer, genetics, and even stress. […] From a broad perspective, angioedema is caused by an abnormal response of the immune system in which chemicals known as histamine or bradykinins are released into the bloodstream. […] Histamine and bradykinin each cause blood vessels to dilate. When released abnormally, either on their own or together, these compounds can cause the swelling we recognize as angioedema. […] Hereditary angioedema (HAE) is usually an autosomal dominant disorder, meaning that you can inherit the problematic gene from just one parent. The gene mutations typically result in the overactivation of bradykinin. […] While HAE can be triggered by stress or injury, most attacks have no known cause. Recurrence is common and can last anywhere from two to five days. ACE inhibitors and estrogen-based contraception, both of which can affect bradykinin levels, are known to increase the frequency and severity of attacks. […] Angioedema can result from allergic or drug reactions, an infection, cancer, genetics, and stress events but sometimes no cause can be found.
  • #1 Angioedema due to acquired C1-Inhibitor deficiency | British Society for Immunology
    https://www.immunology.org/public-information/bitesized-immunology/immune-dysfunction/angioedema-due-acquired-c1-inhibitor
    Acquired angioedema (AAE) due to deficiency of C1-inhibitor is a relatively infrequently occurring but serious disorder, resulting in severe, sometimes life-threatening, episodes of angioedema. […] The main causes of acquired C1-inhibitor deficiency are lymphoproliferative disease and autoimmune disease. Most cases of acquired C1-inhibitor deficiency are associated with an underlying B-cell disorder, ranging from auto-immune anti-C1-inhibitor auto-antibodies to lymphoproliferative disease (usually a low grade or splenic marginal zone lymphoma). […] There are two mechanisms which can lead to this condition: consumption due to complement activation, or (rarely) due to an autoantibody directed against C1-inhibitor. […] Interestingly, many patients with lymphoproliferative disorders may have anti-C1 inhibitor antibodies but without any clinical evidence of angioedema.
  • #1 Acquired angioedema | Allergy, Asthma & Clinical Immunology | Full Text
    https://aacijournal.biomedcentral.com/articles/10.1186/1710-1492-6-14
    Acquired angioedema (AAE) is characterized by acquired deficiency of C1 inhibitor (C1-INH), hyperactivation of the classical pathway of human complement and angioedema symptoms mediated by bradykinin released by inappropriate activation of the contact-kinin system. […] AAE occurred in presence of lymphoma. This association has been repeatedly confirmed in subsequent patients and even if lymphoma is not the only disease associated with AAE, it remains the preeminent disease association. […] Along with lymphoma, other benign forms of lymphoproliferation, namely monoclonal gammopathy of uncertain significance (MGUS), have been reported with high frequency in association with AAE. […] Capacity of lymphoma cells to deplete C1-INH or to cause its consumption through C1 activation, and the possibility to treat AAE by curing the underlying lymphoma, linked the lymphoproliferative disease to the pathogenesis of AAE.
  • #1 Angioedema due to acquired C1-Inhibitor deficiency | British Society for Immunology
    https://www.immunology.org/public-information/bitesized-immunology/immune-dysfunction/angioedema-due-acquired-c1-inhibitor
    The clinical manifestations of angio-edema may precede the diagnosis of a lymphoproliferative disorder in some cases by many years. […] As the underlying causes of acquired C1 inhibitor deficiency in most, if not all, patients are related to B-cell dysfunction, anti-CD20 targeted treatments are thought to be a rational strategy.
  • #1 Angioedema: A Case Report And Review Of The Literature
    https://acquaintpublications.com/article/angioedema_a_case_report_and_review_of_the_literature
    Acquired Angioedema It is even rarer and is related to other disorders, often being a consequence of autoimmune disorders such as the presence of anti- C1 inhibitor antibodies, lymphoproliferative diseases, in studies from the United Kingdom according to the frequency of chronic lymphocytic leukemia, non-Hodgkin’s lymphoma, B-cell lymphoma, marginal zone lymphoma, follicular lymphoma, and Waldenstrom’s macroglobulinemia. […] Triggers: It is for all forms of angioedema, both hereditary and acquired: Minor trauma (e.g., dental repair, tongue piercing). […] Aetiology: The common mechanism is that C1 inhibitor deficiency or dysfunction affects complement activation and increases bradykinin concentrations by not blocking kallikrein activated in the synthesis pathway.
  • #1 Angioedema causes: Allergies, medications, and more | Angioedema NewsEnvelope icon
    https://angioedemanews.com/causes-of-angioedema/
    Acquired angioedema is a very rare condition marked by abnormally low C1-INH levels, leading to the excessive production of bradykinin and swelling, much like HAE type 1. […] There also are some cases in which the underlying cause of angioedema cannot be determined. This specific form of angioedema, known as idiopathic angioedema, is diagnosed only when all other potential explanations for swelling have been ruled out.
  • #1 Angioedema | NHS inform
    https://www.nhsinform.scot/illnesses-and-conditions/skin-hair-and-nails/angioedema/
    Cases of angioedema without an identifiable cause are known as idiopathic angioedema. It may be that a problem with the immune system sometimes causes it to misfire. […] Some medications can cause angioedema. Angiotensin-converting enzyme (ACE) inhibitors, which are used to treat high blood pressure (hypertension), are usually responsible. […] Hereditary angioedema is caused by a genetic mutation (a change in the DNA) in the C1 esterase inhibitor (C1-inh) gene.
  • #1 Angioedema – Australasian Society of Clinical Immunology and Allergy (ASCIA)
    https://www.allergy.org.au/patients/skin-allergy/angioedema
    In even rarer cases, low levels of C1-inhibitor occur with some cases of lymphoma, malignancies and in autoimmune diseases such as Systemic Lupus Erythematosus (SLE). […] Thyroid gland disease and some types of inflammatory arthritis are more common in people with hives (urticaria) and angioedema. […] In some people whose swellings persist for years without an obvious cause, an autoimmune process may be responsible. […] Allergic disease is almost never the cause of swellings that last for days or recur for weeks at a time.
  • #1 Angioedema: Causes, Appearance and Treatment — DermNet
    https://dermnetnz.org/topics/angioedema
    Angioedema is caused by an increase in local capillary permeability and plasma extravasation, usually mediated by mast cells, histamine, or bradykinin release. […] Angioedema is most commonly histamine-mediated; mast cell and basophil stimulation results in histamine release. Angioedema with urticaria tends to suggest a histaminergic form, which includes acute allergic angioedema and histaminergic idiopathic angioedema. The exact cause depends on the type of angioedema a patient has. […] In most cases, the cause of angioedema is unknown. […] Recent research indicates that 30-50% of this type of angioedema may be associated with some types of autoimmune disorders including systemic lupus erythematosus (SLE). […] Inherited abnormal gene that causes a deficiency of a normal blood protein.
  • #1
    https://www.wyndly.com/blogs/learn/angioedema?srsltid=AfmBOoqemp5cfe7mTCTda–K00cPkPCHGkqiFy-CQuDo8vkr8mcea_1H
    Some medications can also cause angioedema as a side effect. Common culprits include angiotensin-converting enzyme inhibitors (used to treat high blood pressure), nonsteroidal anti-inflammatory drugs (NSAIDs), and certain antibiotics. […] Certain infections can trigger angioedema in some individuals. These include respiratory infections such as strep throat and urinary tract infections and viral illnesses like mononucleosis or hepatitis B and C. […] In some cases, angioedema may be caused by an underlying medical condition such as lupus or thyroid disease. […] In some cases, angioedema may be hereditary, which means that it runs in your family and is passed down from generation to generation through genes. Hereditary angioedema usually occurs without any known trigger but can sometimes be triggered by stress or strenuous activity.
  • #1 Urticaria and Angioedema: A Practical Approach | AAFP
    https://www.aafp.org/pubs/afp/issues/2004/0301/p1123.html
    Urticaria (i.e., pruritic, raised wheals) and angioedema (i.e., deep mucocutaneous swelling) occur in up to 25 percent of the U.S. population. […] Chronic urticaria and angioedema tend to be idiopathic, with no identifiable cause, or to be precipitated by a multitude of endogenous or exogenous factors that can be immunologic or nonimmunologic. […] Immunologic urticaria and angioedema are a result of IgE antibody-mediated reactions that usually occur within one hour of exposure to the allergen. […] A rarer cause of acute urticaria is a type II hypersensitivity reaction mediated by cytotoxic antibodies and complement activation. […] Urticaria also has been associated with herpes virus, cytomegalovirus, Epstein-Barr virus, and chronic hepatitis infections, and with bacterial, fungal, and parasitic infections. […] Angioedema has been reported in patients taking angiotensin-converting enzyme inhibitors. […] Patients with a rare inherited or acquired disorder linked to C1 esterase deficiency or autoimmune consumption may present with recurrent angioedema without urticaria.
  • #1 Angioedema: MedlinePlus Medical EncyclopediaLock
    https://medlineplus.gov/ency/article/000846.htm
    Angioedema may be caused by an allergic reaction. Angioedema typically is not itchy while hives are very itchy. During the reaction, histamine and other chemicals are released into the bloodstream. The body releases histamine when the immune system detects a foreign substance called an allergen. […] However, in most cases, the cause of angioedema is never found. […] The following may cause angioedema: […] Allergies due to animal dander (scales of shed skin), foods, insect bites or pollen […] Exposure to water, sunlight, cold or heat […] Medicines (drug allergy) such as antibiotics (penicillin and sulfa drugs), nonsteroidal anti-inflammatory drugs (NSAIDs), and certain blood pressure medicines (ACE inhibitors) […] Autoimmune diseases such as lupus […] Hives and angioedema may also occur after infections. Angioedema may occur alone or with other illnesses (including autoimmune disorders such as lupus, leukemia, and lymphoma). […] A rare form of angioedema runs in families and has different triggers, complications, and treatments. This is called hereditary angioedema.
  • #1 Hereditary angioedema
    https://dermnetnz.org/topics/hereditary-angioedema
    Hereditary angioedema is a familial disease characterised by recurrent attacks of self-limiting oedema. […] The gene coding for C1-INH is located on chromosome 11 and nearly 200 mutations of this gene have been described. […] The most common type of hereditary angioedema is associated with a deficiency of functional C1-esterase inhibitor (C1-INH), a serine protease inhibitor that regulates the activation of the classic complement pathway and suppresses spontaneous activation of complement component C1. […] A deficiency of functional C1-INH results in excessive bradykinin. […] Bradykinin can increase vascular permeability by binding to its receptor on vascular endothelial cells, causing angioedema. […] Type III hereditary angioedema is caused by at least three known gene mutations, including a mutation of the F12 gene, which codes for factor XII to activation by plasmin. […] Acquired angioedema results from a lymphoproliferative disorder such as a B-cell lymphoma (type I) or from the development of mainly immunoglobulin G antibodies against C1-INH (type II). […] ACE inhibitor-induced angioedema occurs because the inhibition of ACE allows bradykinin to accumulate.
  • #1 Angioedema – Wikipedia
    https://en.wikipedia.org/wiki/Angioedema
    Angioedema can be due to antibody formation against C1INH; this is an autoimmune disorder. This acquired angioedema is associated with the development of lymphoma. […] Consumption of foods that are themselves vasodilators, such as alcoholic beverages or cinnamon, can increase the probability of an angioedema episode in susceptible patients. The use of ibuprofen or aspirin may increase the probability of an episode in some patients.
  • #1 An overview of angioedema: Pathogenesis and causes – UpToDate
    https://www.uptodate.com/contents/an-overview-of-angioedema-pathogenesis-and-causes
    An overview of angioedema: Pathogenesis and causes […] The pathogenesis and causes of angioedema will be reviewed here. […] Causes of angioedema classified by mechanism […] Mast cell-mediated etiologies […] Allergic reactions […] Direct mast cell release […] Aspirin and NSAIDs […] Chronic urticaria with or without angioedema […] Bradykinin-mediated etiologies […] ACE inhibitors […] Hereditary and acquired angioedema due to C1 inhibitor deficiency […] Hereditary angioedema with normal C1 inhibitor […] Fibrinolytic agents […] Etiologies of unknown mechanism […] Idiopathic angioedema […] Infections […] Calcium channel blockers […] Other drugs […] Herbal medicines […] Other rare causes.
  • #1 Urticaria and angioedema – Symptoms, diagnosis and treatment | BMJ Best Practice US
    https://bestpractice.bmj.com/topics/en-us/844
    Angioedema is swelling involving the deeper layers of the subdermis and occurs in association with urticaria in about 40% of cases. […] Angioedema can also occur in the absence of urticaria. This is a separate clinical entity, requiring a different diagnostic approach. Patient history, physical exam, and laboratory investigations are used to determine whether angioedema is drug-induced, hereditary, or acquired.
  • #1 Angioedema • LITFL Medical Blog • CCC Airway
    https://litfl.com/angioedema/
    Angioedema may be life-threatening, depending on the underlying cause and the body location affected. […] Hereditary angioedema (HAE) (type 1 and type 2) is caused by C1 esterase inhibitor deficiency (functionally abnormal C1-INH leads to bradykinin over-production). […] Acquired angioedema can be due to medications such as ACE Inhibitors, which have an incidence of up to 1%. […] Angioedema may be histamine-mediated or non-histamine-mediated: histamine-mediated angioedema may co-exist with urticaria and is mast-cell mediated, while non-histaminergic (bradykinin-mediated) angioedema tends to be more severe, more prolonged and less responsive to adrenaline. […] Idiopathic angioedema is thought to be histamine-mediated in most cases. […] Acquired C1 esterase inhibitor deficiency can occur due to an underlying lymphoproliferative disorder and/or an antibody directed against C1-INH. […] The role of adrenaline, steroids and antihistamines is unlikely to be effective for ACEI-related angioedema, as this is a bradykinin-mediated condition, not related to mast cell degranulation.
  • #2 Angioedema: Causes, Appearance and Treatment — DermNet
    https://dermnetnz.org/topics/angioedema
    Angioedema is caused by an increase in local capillary permeability and plasma extravasation, usually mediated by mast cells, histamine, or bradykinin release. […] Angioedema is most commonly histamine-mediated; mast cell and basophil stimulation results in histamine release. Angioedema with urticaria tends to suggest a histaminergic form, which includes acute allergic angioedema and histaminergic idiopathic angioedema. The exact cause depends on the type of angioedema a patient has. […] In most cases, the cause of angioedema is unknown. […] Recent research indicates that 30-50% of this type of angioedema may be associated with some types of autoimmune disorders including systemic lupus erythematosus (SLE). […] Inherited abnormal gene that causes a deficiency of a normal blood protein.
  • #2 Angioedema – Allergy & Immunology | UCLA Health
    https://www.uclahealth.org/medical-services/allergy/disorders-treated/angioedema
    What causes angioedema? There are two main biological pathways that lead to angioedema. One pathway is mediated by histamine. Specific cells that reside in the skin, called mast cells, produce and secrete histamine, which in turn leads to inflammation of surrounding tissue, creating hives and swelling. Common causes of histamine-mediated angioedema include foods, drugs, infections, and insect bites; however, in many cases the cause cannot be established. […] The second major cause of angioedema is much less common than histamine-mediated angioedema. This pathway involves the release of bradykinin, a molecule that acts on blood vessels and causes fluid leakage into the surrounding tissues. The causes of bradykinin-mediated angioedema are usually due to a genetic defect (hereditary angioedema) or a medication side effect (such as ACE inhibitors). In hereditary angioedema (HAE), a molecule named C1 inhibitor is either deficient or dysfunctional.
  • #2 Angioedema | nidirect
    https://www.nidirect.gov.uk/conditions/angioedema
    There are several different types of angioedema, each of which has a different cause. The different types are: […] allergic angioedema – an allergic reaction, such as a food allergy […] drug-induced angioedema an allergic reaction to a medication, such as angiotensin-converting enzyme (ACE) inhibitors for high blood pressure […] hereditary angioedema – a genetic condition that you inherit from your parents this is a rare, lifelong condition that usually starts in childhood […] idiopathic angioedema – with this type of angioedema there is no clear cause.
  • #2 Angioedema: Symptoms, Causes, Treatment, Types
    https://www.webmd.com/skin-problems-and-treatments/angioedema-overview
    Angioedema is swelling beneath your skin. […] There are four types of angioedema, and they all have different causes. […] Allergic angioedema: This is the most common kind. Your allergic reaction can be to foods, like: […] Drug-induced angioedema: Some medicines can trigger angioedema. […] Certain blood pressure medicines called angiotensin-converting enzyme (ACE) inhibitors can also cause flare-ups that can happen quickly. […] Hereditary angioedema (HAE): This is rare. It happens when your body doesnt make enough of a blood protein called C1 esterase inhibitor. […] Theres another very rare type called acquired angioedema that has the same symptoms as HAE. […] Idiopathic angioedema: Idiopathic means there isnt a known cause for your swelling. […] Possible culprits can include: […] Allergic and drug-induced angioedema usually happen within an hour of exposure to your trigger. Hereditary and acquired types usually happen over many hours, but it can feel much faster if you wake up and suddenly discover swelling.
  • #2 Angioedema: Causes, Symptoms, Types & Treatment – Allergyfree
    https://www.allergyfree.co.in/en-gb/know-your-allergy/skin-allergies/angioedema
    There are several types of Angioedema and each type is caused by different triggers. […] Allergic Angioedema can be caused by insect bites, certain medications such as aspirin and penicillin, latex and food allergies, especially to nuts, shellfish, milk and eggs. […] Drug-induced Angioedema can be caused by angiotensin-converting enzyme (ACE) inhibitors. […] Hereditary Angioedema can be caused by the presence of a gene that leads to low levels of proteins in the body. […] In many cases, the cause of Angioedema is not clear, and this situation is called as idiopathic Angioedema. […] Other triggers like pregnancy, pollen, animal dander, contraceptive pills, trauma or infection can also cause Angioedema in some people. […] Depending on its type, Angioedema can be caused through any of the following triggers insect bites, pollen, pet dander, certain medications like aspirin, penicillin and ACE inhibitors containing medicines, latex and certain types of foods.
  • #2 Urticaria and angioedema | Allergy, Asthma & Clinical Immunology | Full Text
    https://aacijournal.biomedcentral.com/articles/10.1186/s13223-018-0288-z
    Urticaria (hives) is a common disorder that often presents with angioedema (swelling that occurs beneath the skin). […] Angioedema can occur in the absence of urticaria and can be broadly divided into histamine-mediated and non-histamine-mediated angioedema. Histamine-mediated angioedema can be allergic, pseudoallergic or idiopathic. Non-histamine mediated angioedema is largely driven by bradykinin and can be hereditary, acquired or drug-induced, such as with angiotensin-converting enzyme inhibitors. […] The first section of this article will focus on the causes, diagnosis and management of the most common types of urticaria (with or without angioedema). The second section will review the work-up and management of angioedema without urticaria. This type of angioedema can be broadly divided into histamine-mediated and non-histamine-mediated angioedema. Distinguishing between these two types of angioedema is important as investigations and management differ considerably.
  • #2 Angioedema – Immune Disorders – Merck Manual Consumer Version
    https://www.merckmanuals.com/home/immune-disorders/allergic-reactions-and-other-hypersensitivity-disorders/angioedema
    Angioedema can be a reaction to a medication or other substance (trigger), a hereditary disorder, a rare complication of cancer, or an immune disorder, but sometimes the cause is not known. […] There are rare hereditary or acquired forms of angioedema. These forms are caused by a deficiency or malfunction of a substance called C1 inhibitor, which is part of the immune system. […] Angioedema can be chronic, recurring over weeks or months. The cause is usually unknown, but it may be repeated, unintentional intake of a substance, such as penicillin in milk or a preservative or dye in foods. Use of certain medications, such as aspirin, other NSAIDs, or opioids, can also cause chronic angioedema. […] However, usually when angioedema without hives recurs, no specific cause is identified. Angioedema with no identified cause is called idiopathic angioedema. […] Angioedema without hives is sometimes caused by ACE inhibitors, which are used to treat high blood pressure and heart failure. When ACE inhibitors are the cause, the face and upper airways are most commonly affected, but the intestine may be affected.
  • #2 What Causes Angioedema? Symptoms
    https://www.emedicinehealth.com/what_causes_angioedema/article_em.htm
    Angioedema is the swelling of tissue under the skin. Allergies are a common cause. […] Allergies are a common cause of angioedema. Common allergens include: Foods – commonly nuts, shellfish, or eggs, Insect bites, Something that comes in contact with the skin such as latex, animal saliva or dander, or plants, Exercise, Medications such as Antibiotics, Medicines used to treat high blood pressure (hypertension) or heart disease called angiotensin-converting enzyme (ACE) inhibitors, Over-the-counter (OTC) nonsteroidal anti-inflammatory drugs (NSAIDs) for pain and fever, such as aspirin, ibuprofen (Motrin, Advil), and naproxen (Aleve). […] Angioedema can also be caused by rare genetic diseases such as hereditary angioedema.
  • #2 Hereditary Angioedema Types, Treatment, Symptoms, Diagnosis
    https://www.medicinenet.com/hereditary_angioedema_hae/article.htm
    Hereditary angioedema (HAE) is a rare hereditary disease that causes swelling with a fluid accumulation of the skin and tissues just beneath the skin (subcutaneous tissue). […] The cause of hereditary angioedema is due to a problem with a gene that produces a protein termed C1 inhibitor (C1-INH). The problem with the autosomal dominant gene is that it does not produce enough C1 inhibitors (a protein that inhibits the complement system from self-activation) so that the body under certain triggers allows fluid to escape from tiny blood vessels into many different areas. […] Males and females have about a 50-50 chance of inheriting the disease if one of their parents has HAE. […] The most dangerous complication of hereditary angioedema is laryngeal edema, which can result in a rapidly advancing shortness of breath to complete airway obstruction within a few hours.
  • #2 Hereditary Angioedema Causes
    https://www.firazyr.com/understanding-hae/hae-causes
    Hereditary angioedema (HAE) is called „hereditary” because literature suggests that about 75% of cases are passed down through families. About 25% of people with HAE have no family history at all. These cases result from a genetic change that happens spontaneously. […] Since the gene responsible for HAE is a dominant gene, the person only needs one HAE gene from one parent to inherit the HAE condition. […] If a person does have HAE, there is a 50% chance he or she will pass it on to the next generation. […] If the person does not have HAE, he or she will not pass on HAE to his or her own children.
  • #2 Understanding Hereditary Angioedema: Causes, Symptoms, and Management
    https://www.everydayhealth.com/hereditary-angioedema/triggers-what-watch/
    Hereditary angioedema (HAE) is caused by a genetic defect that is inherited or developed at conception. These genetic changes may cause one of three types of HAE: HAE type 1, HAE type 2, or HAE type 3. […] All of the genetic changes that cause HAE ultimately affect how your body controls inflammation, and all can lead to severe swelling. […] Hereditary angioedema occurs as a result of a genetic change. The mechanism of an HAE attack varies depending on which genetic change you have. […] There are two known mutations in the SERPING1 gene and the F12 gene but scientists suspect there are also other as yet unidentified genes that cause HAE. […] Changes in the C1NH gene, also known as the SERPING1 gene, cause type 1 and type 2 HAE. […] In type 1 HAE, your body does not make enough C1 inhibitor. With type 2, your body makes enough of the protein, but it doesn’t work properly.
  • #2 What Is Hereditary Angioedema?
    https://www.icliniq.com/articles/genetic-disorders/hereditary-angioedema
    Hereditary angioedema is a rare genetic condition that causes recurrent swelling in limbs, face, intestinal tract, and airway. This disorder causes swelling under the skin and at the lining of the guts and lungs. […] It is an autosomal recessive condition caused by either lack of C1 inhibitor protein or a dysfunctional inhibitor protein. […] The following are the etiological factors associated with hereditary angioedema: […] Type I and Type II: SERPING 1 gene mutation is responsible for the occurrence of type I and type II hereditary angioedema. This gene works as an instructor for making C1 inhibitor protein. This protein is responsible for controlling the inflammatory response of the body. Type I causes a low level of this inhibitor, whereas type II causes an abnormal level. Without the normal level of this inhibitor, the protein fragment called bradykinin is generated. This accelerates the fluid leakage through the blood vessel walls in the tissue and promotes the inflammation reaction in the body.
  • #2 C1 Esterase inhibitor deficiency
    https://www.rch.org.au/clinicalguide/guideline_index/c1_esterase_inhibitor_deficiency/
    (Hereditary Angioedema, HAE) HAE causes recurrent episodes of angioedema in the upper respiratory, gastrointestinal tract or in subcutaneous tissues. […] Acute episodes of angioedema may be triggered by infection, stress, menstruation, surgery, dental work, trauma and some medicines (including oestrogen-containing contraceptives and ACE-inhibitors) or may have no clear trigger. […] HAE is a rare autosomal dominant condition in which C1 esterase inhibitor levels are reduced (HAE type I) or poorly functional (HAE type II). HAE is diagnosed by the finding of low C1 esterase inhibitor level or function. C4 level is also low during episodes of angioedema.
  • #2 Autoimmune angioedema – Autoimmune Association
    https://autoimmune.org/disease-information/autoimmune-angioedema/
    Acquired angioedema (AAE) is a rare disorder that causes recurrent episodes of swelling (edema) of the face or body, lasting several days. […] Swelling episodes may have various triggers, such as mild trauma (such as dental work), viral illness, cold exposure, pregnancy, certain foods, or emotional stress. […] There are two forms of AAE. Type 1 is associated with various other diseases including lymphoproliferative disorders, and autoimmune diseases that may not become apparent until years after the angioedema begins. Type 2 is associated with an autoimmune abnormality in which a person has autoantibodies against a protein in the blood called C1-INH. […] When an underlying disease is present, episodes may stop if the underlying disease is treated, but some people continue to experience episodes despite treatment.
  • #2 Angioedema: Causes, Appearance and Treatment — DermNet
    https://dermnetnz.org/topics/angioedema
    Decreased C1 inhibitor activity leads to excessive kallikrein, which in turn produces bradykinin, a potent vasodilator. […] Acquired during life rather than inherited. […] May be due to B-cell lymphoma or antibodies against C1 inhibitor. […] Associated with lymphoproliferative and autoimmune disease e.g. SLE.
  • #2 Angioedema | NHS inform
    https://www.nhsinform.scot/illnesses-and-conditions/skin-hair-and-nails/angioedema/
    Cases of angioedema without an identifiable cause are known as idiopathic angioedema. It may be that a problem with the immune system sometimes causes it to misfire. […] Some medications can cause angioedema. Angiotensin-converting enzyme (ACE) inhibitors, which are used to treat high blood pressure (hypertension), are usually responsible. […] Hereditary angioedema is caused by a genetic mutation (a change in the DNA) in the C1 esterase inhibitor (C1-inh) gene.
  • #2 11 Causes Of Angioedema And Its Prevention! – By Dr. Manju Keshari | Lybrate
    https://www.lybrate.com/topic/11-causes-of-angioedema-and-its-prevention/14ddb1bd8b32aaf2d75540b09b351380
    Allergic reactions are the bodys way of responding to foreign substances. […] Angioedema is caused due to the following: Food allergies including eggs, peanuts, shellfish, milk, tree nuts, chocolates, etc. […] Insect bites or stings […] Pollen, latex, animal dander, poison ivy, and other common allergens […] Medications like aspirin, penicillin, ibuprofen, and some blood pressure medicines […] In response to blood transfusions […] Auto-immune disorders like lupus […] Conditions like leukemia and thyroid disorders […] Infections like hepatitis, cytomegalovirus infections, Epstein-Barr infections, etc. […] Weather conditions like extremes of cold, heat, or pressure […] Extreme emotional stress […] Genetic angioedema, passed on from parents to children, which could last forever.
  • #2 Hives and angioedema – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/hives-and-angioedema/symptoms-causes/syc-20354908
    Angioedema can arise with hives or alone. It causes swelling in the deeper layers of skin, often around the face and lips. […] For most people who experience acute hives and angioedema, the exact cause can’t be identified. The conditions are sometimes caused by: […] Many foods can trigger reactions in people with sensitivities. Shellfish, fish, peanuts, tree nuts, soy, eggs and milk are frequent offenders. […] Many medications may cause hives or angioedema, including penicillins, aspirin, ibuprofen (Advil, Motrin IB, others), naproxen sodium (Aleve) and blood pressure medications. […] Other causes of acute hives and angioedema are insect bites and infections. […] Severe angioedema can be life-threatening if swelling of the tongue or in the throat blocks the airway.
  • #2 Angioedema: Treatments, types, and symptoms
    https://www.medicalnewstoday.com/articles/216095
    Angioedema is normally caused by an allergic reaction, but it may also be hereditary. […] The cause of angioedema depends on its type. […] Allergic angioedema is most commonly caused by an allergen, such as certain foods, medications, or environmental factors. […] Certain medications may cause angioedema. […] Angioedema may be inherited from family members. […] The condition typically involves a person having dysfunctional C1 esterase inhibitor (C1-INH) protein or having low blood levels of properly functioning C1-1NH protein. […] For this type of angioedema, the doctor may not be able to identify a specific cause for angioedema after checking for typical causes. […] Other triggers may include: pregnancy, contraceptive pills, infection, trauma. […] In some cases, the cause may not be identified.
  • #2 Angio-oedema: Causes, Symptoms, and Treatment | Doctor
    https://patient.info/doctor/angio-oedema-pro
    Angio-oedema can be classified according to cause: Allergic angio-oedema, Non-allergic drug reaction, Idiopathic angio-oedema, Hereditary C1 esterase inhibitor deficiency (or hereditary angio-oedema), Acquired C1-INH deficiency (or acquired angio-oedema). […] The oedema is caused by increased vascular permeability and leakage of plasma into the deeper skin layers. This is due to a weakening of the endothelial cell barrier and is mediated by mast cell-derived mediators, particularly histamine, or by bradykinin. […] There are a number of causes of angio-oedema and this affects clinical presentation, investigation and subsequent management. […] ACE inhibitor-induced angio-oedema is bradykinin-mediated and antihistamines, steroids and adrenaline (epinephrine) are not effective or recommended.
  • #3 Angioedema: Causes, Symptoms, Types & Treatment – Allergyfree
    https://www.allergyfree.co.in/en-gb/know-your-allergy/skin-allergies/angioedema
    There are several types of Angioedema and each type is caused by different triggers. […] Allergic Angioedema can be caused by insect bites, certain medications such as aspirin and penicillin, latex and food allergies, especially to nuts, shellfish, milk and eggs. […] Drug-induced Angioedema can be caused by angiotensin-converting enzyme (ACE) inhibitors. […] Hereditary Angioedema can be caused by the presence of a gene that leads to low levels of proteins in the body. […] In many cases, the cause of Angioedema is not clear, and this situation is called as idiopathic Angioedema. […] Other triggers like pregnancy, pollen, animal dander, contraceptive pills, trauma or infection can also cause Angioedema in some people. […] Depending on its type, Angioedema can be caused through any of the following triggers insect bites, pollen, pet dander, certain medications like aspirin, penicillin and ACE inhibitors containing medicines, latex and certain types of foods.
  • #3 Drug-induced nonallergic angioedema | Angioedema NewsEnvelope icon
    https://angioedemanews.com/non-allergic-angioedema/
    Drug-induced nonallergic angioedema is estimated to occur in as many as 2.5% of people who take these drugs. […] Another class of blood pressure-lowering medicines that can also cause drug-induced nonallergic angioedema is angiotensin receptor blockers, or ARBs. […] In some cases, other types of medications, including nonsteroidal anti-inflammatory drugs — ones commonly used to ease inflammation and pain — can also trigger drug-induced nonallergic angioedema.
  • #3 Autoimmune angioedema – Autoimmune Association
    https://autoimmune.org/disease-information/autoimmune-angioedema/
    Acquired angioedema (AAE) is a rare disorder that causes recurrent episodes of swelling (edema) of the face or body, lasting several days. […] Swelling episodes may have various triggers, such as mild trauma (such as dental work), viral illness, cold exposure, pregnancy, certain foods, or emotional stress. […] There are two forms of AAE. Type 1 is associated with various other diseases including lymphoproliferative disorders, and autoimmune diseases that may not become apparent until years after the angioedema begins. Type 2 is associated with an autoimmune abnormality in which a person has autoantibodies against a protein in the blood called C1-INH. […] When an underlying disease is present, episodes may stop if the underlying disease is treated, but some people continue to experience episodes despite treatment.
  • #3 Angioedema – Australasian Society of Clinical Immunology and Allergy (ASCIA)
    https://www.allergy.org.au/patients/skin-allergy/angioedema
    In even rarer cases, low levels of C1-inhibitor occur with some cases of lymphoma, malignancies and in autoimmune diseases such as Systemic Lupus Erythematosus (SLE). […] Thyroid gland disease and some types of inflammatory arthritis are more common in people with hives (urticaria) and angioedema. […] In some people whose swellings persist for years without an obvious cause, an autoimmune process may be responsible. […] Allergic disease is almost never the cause of swellings that last for days or recur for weeks at a time.
  • #3 Angioedema: Treatments, types, and symptoms
    https://www.medicalnewstoday.com/articles/216095
    Angioedema is normally caused by an allergic reaction, but it may also be hereditary. […] The cause of angioedema depends on its type. […] Allergic angioedema is most commonly caused by an allergen, such as certain foods, medications, or environmental factors. […] Certain medications may cause angioedema. […] Angioedema may be inherited from family members. […] The condition typically involves a person having dysfunctional C1 esterase inhibitor (C1-INH) protein or having low blood levels of properly functioning C1-1NH protein. […] For this type of angioedema, the doctor may not be able to identify a specific cause for angioedema after checking for typical causes. […] Other triggers may include: pregnancy, contraceptive pills, infection, trauma. […] In some cases, the cause may not be identified.