Materiały źródłowe

• #1 Hives (Urticaria) and Angioedema Overview

  https://www.aaaai.org/tools-for-the-public/conditions-library/allergies/hives-(urticaria)-and-angioedema-overview

  Hives or welts, also known as urticaria, are itchy, raised, reddish areas on the skin. Angioedema is swelling below the surface of the skin and fatty tissue. Throat swelling can be life threatening and requires immediate medical attention. Understanding the underlying mechanism of swelling or the specific disease is critical in determining the best treatment. […] Doctors will classify your hives based on how long you have had them. This helps doctors think about possible causes of your hives. When hives occur for less than 6 weeks, we call this acute urticaria. When hives last longer than 6 weeks, we change the name to chronic urticaria. Sometimes the hives in chronic urticaria can occur with angioedema (swelling). […] Talk to your doctor if swelling occurs without hives. This may suggest a special situation requiring additional evaluation. Sometimes high dose antihistamines may not improve your swelling. This is because the mechanism (cause) of swelling may be different than that of hives.

• #2 Urticaria and angioedema – Symptoms, diagnosis and treatment | BMJ Best Practice US

  https://bestpractice.bmj.com/topics/en-us/844?locale=no

  Urticaria is a skin condition characterized by erythematous, blanching, edematous, nonpainful, pruritic lesions that typically resolve within 24 hours and leave no residual markings. […] Diagnosis of urticaria and angioedema is based on history and physical exam; diagnosis of chronic urticaria and hereditary angioedema may involve additional laboratory testing, depending on the history. […] 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.

• #3 Diagnosis and Treatment of Urticaria and Angioedema: A Worldwide Perspective

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3651155/

  Urticaria and angioedema are common clinical conditions representing a major concern for physicians and patients alike. […] The Scientific and Clinical Issues Council of WAO proposed the development of this global Position Paper to further enhance the clinical management of these disorders through the participation of renowned experts from all WAO regions of the world. […] This position paper provides updates on recent advances in the understanding of etiologic factors, pathogenic mechanisms, diagnostic methods, and medical management of acute and chronic urticaria (CU) and angioedema. […] The objectives of this WAO position paper on urticaria and angioedema are to provide updated information on the assessment and treatment that should be applied in health care settings worldwide to obtain a better symptom control, improve patients’ quality of life, contribute to patient education, and enhance accessibility to more effective therapies.

• #4 Hives (Urticaria) and Angioedema Overview

  https://www.aaaai.org/tools-for-the-public/conditions-library/allergies/hives-(urticaria)-and-angioedema-overview

  Hives or welts, also known as urticaria, are itchy, raised, reddish areas on the skin. Angioedema is swelling below the surface of the skin and fatty tissue. Throat swelling can be life threatening and requires immediate medical attention. Understanding the underlying mechanism of swelling or the specific disease is critical in determining the best treatment. […] Doctors will classify your hives based on how long you have had them. This helps doctors think about possible causes of your hives. When hives occur for less than 6 weeks, we call this acute urticaria. When hives last longer than 6 weeks, we change the name to chronic urticaria. Sometimes the hives in chronic urticaria can occur with angioedema (swelling). […] Talk to your doctor if swelling occurs without hives. This may suggest a special situation requiring additional evaluation. Sometimes high dose antihistamines may not improve your swelling. This is because the mechanism (cause) of swelling may be different than that of hives.

• #5 Acute urticaria and angioedema: diagnostic and treatment considerations – PubMed

  https://pubmed.ncbi.nlm.nih.gov/19489657/

  Urticaria is defined as wheals consisting of three features: (i) central swelling of various sizes, with or without surrounding erythema; (ii) pruritus or occasional burning sensations; and (iii) the skin returning to normal appearance, usually within 1-24 hours. Angioedema is defined as: (i) abrupt swelling of the lower dermis and subcutis; (ii) occasional pain instead of pruritus; (iii) commonly involving the mucous membranes; and (iv) skin returning to normal appearance, usually within 72 hours. Acute urticaria and angioedema is defined by its duration (6 weeks) compared with chronic urticaria and angioedema. The best tools in the evaluation of these patients are a comprehensive history and physical examination. […] This review focuses on the value of a comprehensive clinical evaluation at the onset of symptoms. It underscores the importance of coordination of care among physicians, and the development of an action plan for evidence-based investigations, diagnosis, and therapy.

• #6 Evaluation, diagnosis and management of chronic urticaria

  https://www.racgp.org.au/afp/2014/september/evaluation-diagnosis-and-management-of-chronic-urt

  Chronic urticaria is characterised by the occurrence of weals, which occur on a daily basis for more than 6 weeks. Clinical history and physical examination alone are sufficient to establish a diagnosis. […] A detailed history is usually sufficient to establish a diagnosis of urticaria and the type of urticaria. Chronic urticaria persists for more than 6 weeks. […] Patients report weals that are associated with itch, often worse at night. This pruritus can be debilitating enough to affect activities of daily living and quality of life. […] In 80-90% of cases no identifying trigger for chronic urticaria can be established. However, it is thought that many of these idiopathic urticarias may be attributed to autoimmune causes. […] Chronic urticaria is associated with autoimmune disorders, most commonly hypothyroidism, but also coeliac disease, systemic lupus erythematosus (SLE) and type 1 diabetes mellitus.

• #7 Acute and Chronic Urticaria: Evaluation and Treatment | AAFP

  https://www.aafp.org/pubs/afp/issues/2017/0601/p717.html

  Urticaria commonly presents with intensely pruritic wheals, sometimes with edema of the subcutaneous or interstitial tissue. […] Diagnosis is made clinically; anaphylaxis must be ruled out. […] The diagnosis of urticaria is usually clinical. The first step in evaluating urticaria and angioedema is a history and physical examination to characterize the lesions and help identify causes. […] It is critical to rule out anaphylaxis, which has findings or symptoms involving other organ systems beyond the skin, such as the pulmonary (wheezing, stridor), cardiovascular (tachycardia, hypotension), gastrointestinal (diarrhea, vomiting, abdominal pain), or nervous system (dizziness). […] Laboratory workup in the absence of indications of an underlying cause is not necessary. […] If the history or physical examination suggests a specific cause or underlying disease, targeted testing is appropriate. […] Allergy testing is not recommended unless there is specific indication of an allergic cause.

• #8 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). […] The authors will review the causes, diagnosis and management of urticaria (with or without angioedema) and isolated angioedema. […] The diagnostic and therapeutic approaches to these two conditions are considerably different, and this review is designed to highlight these differences to the reader. […] The diagnosis of urticaria, with or without angioedema, is based primarily on a thorough clinical history and physical examination. Based on the history and physical exam, diagnostic tests may also be considered to help confirm a diagnosis of acute, chronic or inducible urticaria. […] Certain diagnostic tests and assessments can be helpful in the diagnosis and differential diagnosis of CSU, including: a complete blood count (CBC), and erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) as markers of inflammation.

• #9 Angioedema Workup: Approach Considerations, Laboratory Studies, Allergy Testing

  https://emedicine.medscape.com/article/135208-workup

  The great majority of cases of angioedema can be diagnosed on the basis of the history and physical examination alone; extensive diagnostic workup and laboratory testing are rarely indicated. […] Most mild cases of angioedema do not require any laboratory tests. Screening for suspected allergies to food, stinging insects, latex, and antibiotics can be performed. […] For angioedema without urticaria (especially those with recurrent episodes), diagnostic tests should include the following: C4 level, C1 esterase inhibitor (C1-INH) quantitative and functional measurements, C1q level. […] These studies will help to establish or rule out C1-INH deficiency associated angioedema, either hereditary or acquired. […] The Joint Taskforce on Practice Parameters 2013 update of the 2000 angioedema practice parameters include diagnostic testing to differentiate among subtypes of angioedema without urticaria.

• #10 Acute and Chronic Urticaria: Evaluation and Treatment | AAFP

  https://www.aafp.org/pubs/afp/issues/2017/0601/p717.html

  Urticaria commonly presents with intensely pruritic wheals, sometimes with edema of the subcutaneous or interstitial tissue. […] Diagnosis is made clinically; anaphylaxis must be ruled out. […] The diagnosis of urticaria is usually clinical. The first step in evaluating urticaria and angioedema is a history and physical examination to characterize the lesions and help identify causes. […] It is critical to rule out anaphylaxis, which has findings or symptoms involving other organ systems beyond the skin, such as the pulmonary (wheezing, stridor), cardiovascular (tachycardia, hypotension), gastrointestinal (diarrhea, vomiting, abdominal pain), or nervous system (dizziness). […] Laboratory workup in the absence of indications of an underlying cause is not necessary. […] If the history or physical examination suggests a specific cause or underlying disease, targeted testing is appropriate. […] Allergy testing is not recommended unless there is specific indication of an allergic cause.

• #11 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. […] Diagnosis can be challenging, especially if symptoms are chronic or minimally responsive to therapy. A thorough medical history, physical examination, and methodical investigation are necessary to uncover diagnostic clues. […] Diagnostic testing can be expensive and may not determine a cause. […] A recent systematic review of more than 6,000 patients with urticaria and angioedema found that routine laboratory screening tests independent of the patient’s history and physical examination should be discouraged, because these tests are of little value in discovering the cause of the reactions. […] Evaluation of patients with urticaria begins with a thorough history that details travel, recent infection, occupational exposure, medications (prescription drugs and herbal and vitamin supplements), ingestion of foods, timing and onset of lesions, morphology, and associated symptoms.

• #12 Diagnosis and Treatment of Urticaria and Angioedema: A Worldwide Perspective

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3651155/

  The goal of diagnostic measures is to (1) identify urticaria type and subtype and (2) identify underlying causes (in long-standing or severe chronic spontaneous urticaria only). […] In patients with CSU, it is necessary to obtain a thorough history, including all possible eliciting factors, and to identify the significant aspects of the disease. […] The ASST is the only generally available test to screen for autoantibodies against either IgE or the high-affinity IgE receptor. […] The diagnostic workup should include physical stimulation tests if physical urticaria is suspected. […] The presence of these antibodies does not prove causality, although their role as a pathogenic mechanism is debated with evidence pro and con. […] The remaining 55 to 60% of patients lacking such autoimmunity are considered to have chronic nonautoimmune or idiopathic (but nevertheless spontaneous) urticaria. […] The prognosis of AU is excellent, with most cases resolving within days; however, the prognosis of CU is variable. […] The prognosis of urticaria and angioedema is improved with prompt and proper treatment.

• #13 Angioedema diagnosis | Angioedema NewsEnvelope icon

  https://angioedemanews.com/angioedema-diagnosis/

  Angioedema is characterized by swelling, or edema, in the tissues underneath the skin that can usually be diagnosed by a doctor after examining the affected area and discussing other symptoms a person may be experiencing. […] Thus, a detailed review of symptoms along with specific lab tests may be needed to correctly diagnose the specific type of angioedema. […] Swiftly and accurately identifying the cause and type of angioedema a person has is crucial because different angioedema types require distinct treatment approaches. […] A quick and early angioedema diagnosis allows appropriate medication to be given as rapidly as possible to control the acute manifestations of the condition, and facilitate the adoption and implementation of strategies to prevent future swelling episodes. […] The first step in diagnosing and determining the underlying cause of angioedema is assessing a patient’s symptoms and reviewing their clinical and family history.

• #14 Angioedema | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/skin-hair-and-nails/angioedema/

  Your doctor can diagnose angioedema by examining the affected skin and discussing symptoms. […] However, further tests, such as blood tests or allergy tests, may be required to determine the type of angioedema. […] Idiopathic angioedema is only diagnosed if no cause can be found. […] Angioedema is usually diagnosed by your doctor examining the affected skin and discussing your symptoms. […] It can be difficult to find the exact cause and identify which type of angioedema you have. There is no single test available, but you may have allergy tests or blood tests if a specific cause is suspected. […] If allergic angioedema is suspected, you are likely to be referred to a specialist allergy or immunology clinic for further testing. Tests may include: a skin prick test your skin is pricked with a tiny amount of the suspected allergen to see whether there is a reaction, a blood test a sample of your blood is tested to determine whether your immune system reacts to a suspected allergen.

• #15 Angioedema Differential Diagnoses

  https://emedicine.medscape.com/article/135208-differential

  Special consideration should be given to those who experience angioedema without urticaria. In such cases, hereditary and acquired angioedema (AAE) must be differentiated. […] When angioedema is associated with urticaria, the diagnostic algorithm is almost identical to that of urticaria patients. For recurrent angioedema without urticaria, it is strongly recommended to rule out hereditary angioedema (HAE), angiotensin-converting enzyme inhibitor induced angioedema (ACEI-AAE, or AIIA), and acquired C1 esterase inhibitor deficiency angioedema (C1-INH-AAE). […] Therefore, for angioedema without urticaria, and once C1 INH deficiency and ACEI-AAE are ruled out, empirical treatment with high doses of a second generation antihistamine (up to 4 times more than the conventional dose) can help further categorize the patients (histaminergic vs. nonhistaminergic).

• #16 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). […] The authors will review the causes, diagnosis and management of urticaria (with or without angioedema) and isolated angioedema. […] The diagnostic and therapeutic approaches to these two conditions are considerably different, and this review is designed to highlight these differences to the reader. […] The diagnosis of urticaria, with or without angioedema, is based primarily on a thorough clinical history and physical examination. Based on the history and physical exam, diagnostic tests may also be considered to help confirm a diagnosis of acute, chronic or inducible urticaria. […] Certain diagnostic tests and assessments can be helpful in the diagnosis and differential diagnosis of CSU, including: a complete blood count (CBC), and erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) as markers of inflammation.

• #17 Urticaria and angioedema | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-018-0288-z

  The presence of thyroid autoantibodies supports the autoimmune process in CSU. […] The diagnosis of HAE and AAE is based upon a suggestive clinical history, and there is significant overlap in their clinical presentation. […] The most common presentation is that of non-emergent angioedema resulting in impairment in QOL with discomfort, immobility and disfigurement, and the inability to attend work or school. […] The diagnosis of HAE and AAE should include the assessment of C4, C1q, and C1-INH function and antigenic levels.

• #18 Angioedema Workup: Approach Considerations, Laboratory Studies, Allergy Testing

  https://emedicine.medscape.com/article/135208-workup

  The great majority of cases of angioedema can be diagnosed on the basis of the history and physical examination alone; extensive diagnostic workup and laboratory testing are rarely indicated. […] Most mild cases of angioedema do not require any laboratory tests. Screening for suspected allergies to food, stinging insects, latex, and antibiotics can be performed. […] For angioedema without urticaria (especially those with recurrent episodes), diagnostic tests should include the following: C4 level, C1 esterase inhibitor (C1-INH) quantitative and functional measurements, C1q level. […] These studies will help to establish or rule out C1-INH deficiency associated angioedema, either hereditary or acquired. […] The Joint Taskforce on Practice Parameters 2013 update of the 2000 angioedema practice parameters include diagnostic testing to differentiate among subtypes of angioedema without urticaria.

• #19 Angioedema – Wikipedia

  https://en.wikipedia.org/wiki/Angioedema

  The diagnosis is made on the clinical picture. Routine blood tests (complete blood count, electrolytes, kidney function, liver enzymes) are typically performed. Mast cell tryptase levels may be elevated if the attack was due to an acute allergic (anaphylactic) reaction. When the patient has been stabilized, particular investigations may clarify the exact cause; complement levels, especially depletion of complement factors 2 and 4, may indicate deficiency of C1-inhibitor. HAE type III is a diagnosis of exclusion consisting of observed angioedema along with normal C1 levels and function. […] The hereditary form (HAE) often goes undetected for a long time, as its symptoms resemble those of more common disorders, such as allergy or intestinal colic. An important clue is the failure of hereditary angioedema to respond to antihistamines or steroids, a characteristic that distinguishes it from allergic reactions. It is particularly difficult to diagnose HAE in patients whose episodes are confined to the gastrointestinal tract. Besides a family history of the disease, only a laboratory analysis can provide final confirmation. In this analysis, it is usually a reduced complement factor C4, rather than the C1-INH deficiency itself, that is detected. The former is used during the reaction cascade in the complement system of immune defense, which is permanently overactive due to the lack of regulation by C1-INH. […] Angioedema is classified as either hereditary or acquired.

• #20 Urticaria and angioedema | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/1710-1492-7-S1-S9

  The ASST is currently one of the most useful tests for confirming a diagnosis of chronic autoimmune urticaria. […] The diagnosis of HAE and AAE is based upon a suggestive clinical history (i.e., episodic angioedema in the absence of urticaria affecting the skin, gastrointestinal and upper respiratory tracts) and the presence of abnormalities in specific complement proteins. […] The management of HAE and AAE involves both prophylactic strategies to prevent attacks of angioedema as well as pharmacologic interventions for the treatment of acute attacks. […] Diagnosis of HAE and AAE should include the assessment of C4, C1q, C1 inhibitor function and antigenic levels.

• #21 Hives: Causes, Symptoms, Diagnosis, Treatment & Prevention

  https://my.clevelandclinic.org/health/diseases/8630-hives

  Hives are a type of allergic reaction that creates itchy bumps on your skin. […] Your healthcare provider can diagnose hives and angioedema by looking at your skin. Allergy tests can help identify whats triggering a reaction, but this is true primarily for acute hives. […] Allergy tests to diagnose hives include: Skin tests: During this test, healthcare providers test different allergens on your skin. If your skin turns red or swells, it means youre allergic to that substance. […] A blood test checks for specific antibodies in your blood. Your body makes antibodies to fight off allergens. If your body makes too many antibodies, you can develop hives and swelling.

• #22 Angioedema | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/skin-hair-and-nails/angioedema/

  Your doctor can diagnose angioedema by examining the affected skin and discussing symptoms. […] However, further tests, such as blood tests or allergy tests, may be required to determine the type of angioedema. […] Idiopathic angioedema is only diagnosed if no cause can be found. […] Angioedema is usually diagnosed by your doctor examining the affected skin and discussing your symptoms. […] It can be difficult to find the exact cause and identify which type of angioedema you have. There is no single test available, but you may have allergy tests or blood tests if a specific cause is suspected. […] If allergic angioedema is suspected, you are likely to be referred to a specialist allergy or immunology clinic for further testing. Tests may include: a skin prick test your skin is pricked with a tiny amount of the suspected allergen to see whether there is a reaction, a blood test a sample of your blood is tested to determine whether your immune system reacts to a suspected allergen.

• #23 Angioedema Workup: Approach Considerations, Laboratory Studies, Allergy Testing

  https://emedicine.medscape.com/article/135208-workup

  Screening laboratory studies have limited value in most cases. […] If the initial laboratory tests yield abnormal results or if a specific medical condition is suspected, additional tests may be needed. […] When the CH50 or C4 level is low, additional tests for C1-INH function and C1q level should be considered. […] The diagnosis of IgE-mediated angioedema is usually made on the basis of the history. […] However, epicutaneous skin testing or radioallergosorbent tests (RASTs) for foods may be confirmatory. […] Most angioedema patients do not need any imaging studies. […] When internal organ involvement is suspected, during acute attacks, the following studies can be performed: Plain abdominal radiography, Abdominal ultrasonography, CT of the abdomen, Radiography of the chest, Radiography of cervical soft tissue.

• #24 Diagnosis and Treatment of Urticaria and Angioedema: A Worldwide Perspective

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3651155/

  The goal of diagnostic measures is to (1) identify urticaria type and subtype and (2) identify underlying causes (in long-standing or severe chronic spontaneous urticaria only). […] In patients with CSU, it is necessary to obtain a thorough history, including all possible eliciting factors, and to identify the significant aspects of the disease. […] The ASST is the only generally available test to screen for autoantibodies against either IgE or the high-affinity IgE receptor. […] The diagnostic workup should include physical stimulation tests if physical urticaria is suspected. […] The presence of these antibodies does not prove causality, although their role as a pathogenic mechanism is debated with evidence pro and con. […] The remaining 55 to 60% of patients lacking such autoimmunity are considered to have chronic nonautoimmune or idiopathic (but nevertheless spontaneous) urticaria. […] The prognosis of AU is excellent, with most cases resolving within days; however, the prognosis of CU is variable. […] The prognosis of urticaria and angioedema is improved with prompt and proper treatment.

• #25 Urticaria and angioedema | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/1710-1492-7-S1-S9

  The ASST is currently one of the most useful tests for confirming a diagnosis of chronic autoimmune urticaria. […] The diagnosis of HAE and AAE is based upon a suggestive clinical history (i.e., episodic angioedema in the absence of urticaria affecting the skin, gastrointestinal and upper respiratory tracts) and the presence of abnormalities in specific complement proteins. […] The management of HAE and AAE involves both prophylactic strategies to prevent attacks of angioedema as well as pharmacologic interventions for the treatment of acute attacks. […] Diagnosis of HAE and AAE should include the assessment of C4, C1q, C1 inhibitor function and antigenic levels.

• #26 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. […] Diagnosis can be challenging, especially if symptoms are chronic or minimally responsive to therapy. A thorough medical history, physical examination, and methodical investigation are necessary to uncover diagnostic clues. […] Diagnostic testing can be expensive and may not determine a cause. […] A recent systematic review of more than 6,000 patients with urticaria and angioedema found that routine laboratory screening tests independent of the patient’s history and physical examination should be discouraged, because these tests are of little value in discovering the cause of the reactions. […] Evaluation of patients with urticaria begins with a thorough history that details travel, recent infection, occupational exposure, medications (prescription drugs and herbal and vitamin supplements), ingestion of foods, timing and onset of lesions, morphology, and associated symptoms.

• #27 Angioedema diagnosis | Angioedema NewsEnvelope icon

  https://angioedemanews.com/angioedema-diagnosis/

  A healthcare provider also will review whether a patient is taking any medications that may cause angioedema as a side effect. […] Whether a person has acute or chronic angioedema, with recurrent swelling episodes, will also help guide the diagnosis. […] If someone shows signs of HAE, such as recurrent swelling episodes, and/or has a family history of the disease, laboratory tests can be used to confirm the diagnosis. […] Typically, the first angioedema test performed when someone shows signs of HAE is designed to measure the levels of a protein called C4 in the bloodstream. […] Specialized blood tests to measure the levels and activity of the C1-INH protein also can be performed to help identify HAE types 1 or 2. […] A diagnosis of HAE can be confirmed by genetic testing to identify the disease-causing mutation(s) a patient may be carrying.

• #28 Diagnosis and screening of patients with hereditary angioedema in prim | TCRM

  https://www.dovepress.com/diagnosis-and-screening-of-patients-with-hereditary-angioedema-in-prim-peer-reviewed-fulltext-article-TCRM

  The most significant hurdle to diagnosis of HAE in the primary care clinic and the emergency department is low initial suspicion. […] Once a clinician suspects HAE, however, laboratory tests and medical management can reliably confirm or exclude the diagnosis of C1-INH-deficient HAE. […] A complete physical examination along with a detailed family history is the best way to raise suspicion of HAE in the primary care clinic. […] It is important to distinguish HAE from other forms of angioedema, particularly mast cell-mediated reactions, which are much more common and resolve with epinephrine, antihistamine, and glucocorticoid treatment. […] The diagnosis of HAE should be confirmed through laboratory testing. […] Most patients with HAE due to C1-INH deficiency have persistently low antigenic C4 levels.

• #29 Diagnosis and screening of patients with hereditary angioedema in prim | TCRM

  https://www.dovepress.com/diagnosis-and-screening-of-patients-with-hereditary-angioedema-in-prim-peer-reviewed-fulltext-article-TCRM

  When low C4 levels are found in combination with low C1-INH function, the results had a 98% specificity for HAE due to C1-INH deficiency across a multicenter evaluation. […] To confirm a diagnosis of HAE, it is recommended that physicians work closely with an HAE expert. […] The laboratory diagnosis of HAE-FXII relies on genetic testing, while no confirmatory laboratory tests have been developed for HAE-U. […] HAE with normal C1-INH levels is much more challenging to diagnose, and requires a history of recurrent angioedema without urticaria, documented normal or near-normal C4, C1-INH protein, and C1-INH functional levels, and either a demonstrated F12 mutation or a positive family history.

• #30

  https://link.springer.com/article/10.1007/s12016-025-09027-4

  The diagnosis of HAE should be considered in all patients with recurrent angioedema without hives. Proper diagnosis utilizes four modes of investigation: (1) clinical presentation (phenotype), (2) pathomechanism, (3) biomarkers, and (4) genetics. […] The diagnostic process is complicated by the reality that in clinical practice, many angioedema patients will have an unknown pathomechanism while validated biomarkers and genetic studies are either unavailable or uninformative. […] In the absence of helpful laboratory data, clinicians must assess the patients responses over time to empiric treatment with medications targeting potential pathogenic pathways. […] The algorithm below and Fig. 1 summarize the recommended approach for evaluating these patients. […] Confirm a clinical history of documented recurrent angioedema without hives.

• #31

  https://link.springer.com/article/10.1007/s12016-025-09027-4

  Measure C4, C1INH antigen, and C1INH function (if available), even if the patient is taking a medication that may cause angioedema. […] If acquired C1INH deficiency is suspected based on age of symptom onset, C1q level and anti-C1INH antibodies should be measured. […] A detailed family history for evidence of recurrent angioedema or a diagnosis of HAE in other family members is essential in all patients with recurrent angioedema. […] Evaluate for mast cell-mediated angioedema based on clinical symptoms as well as response to mast cell-directed therapy. […] Targeted sequencing (next-generation sequencing [NGS] or Sanger sequencing), for known HAE pathogenic variants is recommended if accessible. […] In the absence of a known pathogenic variant, patients with a reliable positive family history of angioedema are classified as HAE-UNK while those without a family history are classified as INMA.

• #32 Angioedema – Wikipedia

  https://en.wikipedia.org/wiki/Angioedema

  The diagnosis is made on the clinical picture. Routine blood tests (complete blood count, electrolytes, kidney function, liver enzymes) are typically performed. Mast cell tryptase levels may be elevated if the attack was due to an acute allergic (anaphylactic) reaction. When the patient has been stabilized, particular investigations may clarify the exact cause; complement levels, especially depletion of complement factors 2 and 4, may indicate deficiency of C1-inhibitor. HAE type III is a diagnosis of exclusion consisting of observed angioedema along with normal C1 levels and function. […] The hereditary form (HAE) often goes undetected for a long time, as its symptoms resemble those of more common disorders, such as allergy or intestinal colic. An important clue is the failure of hereditary angioedema to respond to antihistamines or steroids, a characteristic that distinguishes it from allergic reactions. It is particularly difficult to diagnose HAE in patients whose episodes are confined to the gastrointestinal tract. Besides a family history of the disease, only a laboratory analysis can provide final confirmation. In this analysis, it is usually a reduced complement factor C4, rather than the C1-INH deficiency itself, that is detected. The former is used during the reaction cascade in the complement system of immune defense, which is permanently overactive due to the lack of regulation by C1-INH. […] Angioedema is classified as either hereditary or acquired.

• #33 Angioedema diagnosis | Angioedema NewsEnvelope icon

  https://angioedemanews.com/angioedema-diagnosis/

  There are different diagnostic tests available to help clinicians identify the various forms of angioedema other than HAE. […] If a patient shows signs of acute allergic angioedema, such as swelling that’s accompanied by itching or hives, clinicians will first try to determine whether there has been exposure to substances that may potentially trigger an allergic reaction. […] Acquired angioedema is caused by a disruption in the function of the immune system that ultimately causes the levels and activity of the C1-INH protein to dip excessively low. […] Drug-induced nonallergic angioedema is typically diagnosed when a patient is showing signs of angioedema after being exposed to a medication that can trigger the condition as a side effect. […] Idiopathic angioedema is diagnosed when a person experiences angioedema-like swelling, but its underlying cause cannot be identified.

• #34 Angioedema – Immune Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/immune-disorders/allergic-reactions-and-other-hypersensitivity-disorders/angioedema

  Angioedema is swelling of areas of tissue under the skin, sometimes affecting the face and throat. […] The cause of angioedema is often obvious, and tests are seldom needed because the reactions usually resolve and do not recur. […] If angioedema recurs and the cause is not clear, doctors ask people about all the medications they are taking and all food and drink they are consuming. […] If the cause is still not clear, particularly if people have no hives or if family members have angioedema, doctors may do tests for hereditary or acquired forms of angioedema. […] For angioedema without hives (as when caused by an ACE inhibitor or a hereditary form of angioedema), antihistamines, corticosteroids, and epinephrine may not help. […] If the cause is an ACE inhibitor, symptoms usually resolve about 24 to 48 hours after stopping the medication.

• #35 How Angioedema Is Diagnosed

  https://www.verywellhealth.com/how-angioedema-is-diagnosed-4163523

  Commonly used tests in the evaluation of angioedema include: Allergy Test: There are a variety of allergy tests. […] Blood Test: Blood tests can detect elevated levels of immunological activity. […] Sometimes, there is no allergen identified and angioedema may be a diagnosis of exclusion after other causes of the symptoms have been ruled out. […] A blood and/or genetic test can help identify non-histaminergic angioedema. However, it is more common to have the blood test. […] Non-histaminergic angioedema can be inherited directly from parents with an autosomal dominant pattern, which means that if a person has the gene for this condition, symptoms of the disease will develop. […] Imaging is not normally helpful in diagnosing angioedema. […] There are a few other conditions that may produce symptoms similar to those of angioedema.

• #36 Angioedema Workup: Approach Considerations, Laboratory Studies, Allergy Testing

  https://emedicine.medscape.com/article/135208-workup

  Screening laboratory studies have limited value in most cases. […] If the initial laboratory tests yield abnormal results or if a specific medical condition is suspected, additional tests may be needed. […] When the CH50 or C4 level is low, additional tests for C1-INH function and C1q level should be considered. […] The diagnosis of IgE-mediated angioedema is usually made on the basis of the history. […] However, epicutaneous skin testing or radioallergosorbent tests (RASTs) for foods may be confirmatory. […] Most angioedema patients do not need any imaging studies. […] When internal organ involvement is suspected, during acute attacks, the following studies can be performed: Plain abdominal radiography, Abdominal ultrasonography, CT of the abdomen, Radiography of the chest, Radiography of cervical soft tissue.

• #37 Angioedema Workup: Approach Considerations, Laboratory Studies, Allergy Testing

  https://emedicine.medscape.com/article/135208-workup

  Screening laboratory studies have limited value in most cases. […] If the initial laboratory tests yield abnormal results or if a specific medical condition is suspected, additional tests may be needed. […] When the CH50 or C4 level is low, additional tests for C1-INH function and C1q level should be considered. […] The diagnosis of IgE-mediated angioedema is usually made on the basis of the history. […] However, epicutaneous skin testing or radioallergosorbent tests (RASTs) for foods may be confirmatory. […] Most angioedema patients do not need any imaging studies. […] When internal organ involvement is suspected, during acute attacks, the following studies can be performed: Plain abdominal radiography, Abdominal ultrasonography, CT of the abdomen, Radiography of the chest, Radiography of cervical soft tissue.

• #38 Hives (Urticaria) | Causes, Symptoms & Treatment

  https://acaai.org/allergies/allergic-conditions/skin-allergy/hives/

  In cases where vasculitis may be the cause, your allergist may conduct a skin biopsy and send it to a specialist to examine under a microscope. […] If your hives last more than a month or if they recur over time, see an allergist, who will take a history and perform a thorough physical exam to try and determine the cause of your symptoms.

• #39 Evaluation, diagnosis and management of chronic urticaria

  https://www.racgp.org.au/afp/2014/september/evaluation-diagnosis-and-management-of-chronic-urt

  A skin biopsy can be performed if the diagnosis is in question. […] Diagnosis of chronic urticaria can be established with a history and physical examination. […] An alternative diagnosis should be considered in the context of accompanying symptoms. […] Histamine H1 receptor antagonists are the mainstay of treatment. […] Patients who are unresponsive to treatments should be referred to a specialist for alternative treatment options.

• #40 Chronic Spontaneous Urticaria: Diagnosis and Treatment Strategies

  https://www.dermatologytimes.com/view/chronic-spontaneous-urticaria-diagnosis-and-treatment-strategies

  Dr Rodrigues discussed the characteristics of CSU, which are wheals (or hives) and/or angioedema present for more than 6 weeks. […] Accurately diagnosing CSU involves a thorough history and physical examination. In addition to assessing the presence of wheals or hives and angioedema, providers should also review family history and all patient symptoms to rule out other possible conditions. There is a CSU diagnostic algorithm, shared Dr Rodrigues. This tool allows physicians to explore the multiple pathways that can lead to a diagnosis of CSU and also exclude other differential diagnoses. […] Delayed diagnosis is common among patients with CSU, who experience an average delay of 24 months from symptom onset to established diagnosis. […] Overall, insufficient knowledge of CSU among health care providers in both primary and secondary care can contribute to diagnostic delays.

• #41 Chronic Spontaneous Urticaria: Diagnosis and Treatment Strategies

  https://www.dermatologytimes.com/view/chronic-spontaneous-urticaria-diagnosis-and-treatment-strategies

  Dr Rodrigues discussed the characteristics of CSU, which are wheals (or hives) and/or angioedema present for more than 6 weeks. […] Accurately diagnosing CSU involves a thorough history and physical examination. In addition to assessing the presence of wheals or hives and angioedema, providers should also review family history and all patient symptoms to rule out other possible conditions. There is a CSU diagnostic algorithm, shared Dr Rodrigues. This tool allows physicians to explore the multiple pathways that can lead to a diagnosis of CSU and also exclude other differential diagnoses. […] Delayed diagnosis is common among patients with CSU, who experience an average delay of 24 months from symptom onset to established diagnosis. […] Overall, insufficient knowledge of CSU among health care providers in both primary and secondary care can contribute to diagnostic delays.

• #42 Chronic Spontaneous Urticaria: Diagnosis and Treatment Strategies

  https://www.dermatologytimes.com/view/chronic-spontaneous-urticaria-diagnosis-and-treatment-strategies

  Dr Rodrigues discussed the characteristics of CSU, which are wheals (or hives) and/or angioedema present for more than 6 weeks. […] Accurately diagnosing CSU involves a thorough history and physical examination. In addition to assessing the presence of wheals or hives and angioedema, providers should also review family history and all patient symptoms to rule out other possible conditions. There is a CSU diagnostic algorithm, shared Dr Rodrigues. This tool allows physicians to explore the multiple pathways that can lead to a diagnosis of CSU and also exclude other differential diagnoses. […] Delayed diagnosis is common among patients with CSU, who experience an average delay of 24 months from symptom onset to established diagnosis. […] Overall, insufficient knowledge of CSU among health care providers in both primary and secondary care can contribute to diagnostic delays.

• #43 Testing for Hives (Urticaria): Getting a Diagnosis

  https://www.health.com/hives-diagnosis-7092859

  Blood tests may be another step for confirming a diagnosis of urticaria. Your healthcare provider will take your blood sample and send it to a lab for testing. Blood tests are generally indicated for chronic cases in which symptoms persist longer than six weeks. […] The diagnosis of urticaria, commonly called hives, primarily involves an assessment of medical history, as well as a physical examination of affected areas. In chronic cases, additional tests may be needed. These include allergy tests, blood tests, as well as tests to identify any triggers for urticaria.

• #44 Angioedema | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/skin-hair-and-nails/angioedema/

  Hereditary angioedema can be diagnosed using a blood test to check the level of proteins regulated by the C1-inh gene. A very low level would suggest hereditary angioedema. […] Idiopathic angioedema is usually confirmed by a diagnosis of exclusion. This means a diagnosis of idiopathic angioedema can only be made after all the above tests have been carried out and a cause has not been found.

• #45 Evaluation, diagnosis and management of chronic urticaria

  https://www.racgp.org.au/afp/2014/september/evaluation-diagnosis-and-management-of-chronic-urt

  Chronic urticaria is characterised by the occurrence of weals, which occur on a daily basis for more than 6 weeks. Clinical history and physical examination alone are sufficient to establish a diagnosis. […] A detailed history is usually sufficient to establish a diagnosis of urticaria and the type of urticaria. Chronic urticaria persists for more than 6 weeks. […] Patients report weals that are associated with itch, often worse at night. This pruritus can be debilitating enough to affect activities of daily living and quality of life. […] In 80-90% of cases no identifying trigger for chronic urticaria can be established. However, it is thought that many of these idiopathic urticarias may be attributed to autoimmune causes. […] Chronic urticaria is associated with autoimmune disorders, most commonly hypothyroidism, but also coeliac disease, systemic lupus erythematosus (SLE) and type 1 diabetes mellitus.

• #46 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Angioedema-Diagnosis.aspx

  Angioedema diagnosis more often than not depends on the history of recent exposure to a triggering agent and presentation with typical clinical symptoms. […] There are some tests that may be prescribed to determine the type of angioedema according to cause. […] Diagnosis is based on the symptoms of angioedema. […] The diagnosis of each type of angioedema according to its cause includes:- […] The first step in diagnosing an allergy is a detailed history of an exposure to a possible allergen or triggering agent. […] Further tests include the skin prick test and blood tests. […] Diagnosing this type of angioedema involves taking a detailed history of all the drugs consumed. […] This form of angioedema is commonly inherited. […] Idiopathic angioedema is diagnosed and confirmed when no other cause is found.

• #47 Angioedema | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/skin-hair-and-nails/angioedema/

  Hereditary angioedema can be diagnosed using a blood test to check the level of proteins regulated by the C1-inh gene. A very low level would suggest hereditary angioedema. […] Idiopathic angioedema is usually confirmed by a diagnosis of exclusion. This means a diagnosis of idiopathic angioedema can only be made after all the above tests have been carried out and a cause has not been found.

• #48 Hives and angioedema – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hives-and-angioedema/symptoms-causes/syc-20354908

  Hives and angioedema are usually treated with antihistamine medication. Angioedema can be life-threatening if swelling of the tongue or in the throat blocks the airway. […] Angioedema is a reaction similar to hives that affects deeper layers of the skin. It can appear with hives or alone. […] If you think your hives or angioedema was caused by a known allergy to food or a medication, your symptoms may be an early sign of an anaphylactic reaction. Seek emergency care if you feel your tongue, lips, mouth or throat swelling or if you’re having trouble breathing. […] Severe angioedema can be life-threatening if swelling of the tongue or in the throat blocks the airway.

• #49 Hives: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/hives-treatment

  Hives can require emergency care. Go to urgent care or the nearest emergency room if you have any of the following: Swelling on your face, inside your mouth, or in your throat. Problems swallowing or breathing. Feel light-headed or faint. Racing heart. […] A dermatologist can often diagnose hives by looking at your skin. While your dermatologist may diagnose you during your first office visit, finding the exact cause of hives can take time. […] Your dermatologist may also require one or more of the following medical tests: Allergy tests (on the skin or blood test). Blood work (to rule out an illness or infection). Skin biopsy. […] While having answers to these questions and test results is helpful, its important to know that the cause of hives cannot always be found. Without knowing the cause, your dermatologist can successfully treat hives.

• #50 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. […] It may be difficult to tell what kind of angioedema you have. Your provider will begin with a physical examination, though in many cases the swelling will be easy to see. Theyll ask you questions about: […] In addition to the questions, your provider may order blood or skin tests for allergies or blood tests to find out if you have angioedema related to the C1 inhibitor protein. […] Treating angioedema depends on what kind of angioedema you have. For severe allergic reactions, youll often have injectable epinephrine to carry. You should administer this while calling 911.

• #51 Angioedema: Causes, Symptoms, Types & Treatments

  https://my.clevelandclinic.org/health/diseases/22632-angioedema

  For allergic angioedema, your provider may suggest antihistamines or steroids. You may get them either in oral (pill or liquid) or intravenous (in the vein) form. […] If you have hereditary, idiopathic or acquired C1 inhibitor deficiency angioedema, youll probably be referred to a specialist. Some medications that treat or prevent heredity angioedema include: […] If you have allergy-related angioedema, you can prevent occurrences by avoiding the food, medication or other triggers that cause allergic reactions. […] For some people, allergic angioedema can cause anaphylaxis severe swelling of the airways and lungs. People with this life-threatening condition should carry injectable epinephrine (EpiPen, Auvi-Q, Adrenaclick and other brand names) to treat severe allergic reactions. Angioedema that affects the airways, no matter what the cause, is always a medical emergency and you should seek treatment immediately.

• #52 Hives (Urticaria) and Angioedema Overview

  https://www.aaaai.org/tools-for-the-public/conditions-library/allergies/hives-(urticaria)-and-angioedema-overview

  Antihistamines are the best initial medication to treat your hives. About 50% of chronic spontaneous urticaria (hives over 6 weeks with no identifiable cause) will respond to antihistamine as discussed above. For those who do not improve on antihistamines, 65% respond to omalizumab. Omalizumab is an FDA approved treatment of chronic urticaria. Your doctor will outline a treatment plan that allows you to increase treatment during an outbreak of hives or swelling and reduce medications when the hives or angioedema are not as bothersome.

• #53 Angioedema: Causes, Symptoms, Types & Treatments

  https://my.clevelandclinic.org/health/diseases/22632-angioedema

  For allergic angioedema, your provider may suggest antihistamines or steroids. You may get them either in oral (pill or liquid) or intravenous (in the vein) form. […] If you have hereditary, idiopathic or acquired C1 inhibitor deficiency angioedema, youll probably be referred to a specialist. Some medications that treat or prevent heredity angioedema include: […] If you have allergy-related angioedema, you can prevent occurrences by avoiding the food, medication or other triggers that cause allergic reactions. […] For some people, allergic angioedema can cause anaphylaxis severe swelling of the airways and lungs. People with this life-threatening condition should carry injectable epinephrine (EpiPen, Auvi-Q, Adrenaclick and other brand names) to treat severe allergic reactions. Angioedema that affects the airways, no matter what the cause, is always a medical emergency and you should seek treatment immediately.

• #54 Angioedema: Causes, Symptoms, Types & Treatments

  https://my.clevelandclinic.org/health/diseases/22632-angioedema

  You should call 911 if you have a severe allergic reaction and the swelling affects your airways. […] In less severe cases, contact your healthcare provider if you have repeated instances of angioedema. You may be able to work out preventive measures. Other treatment options include oral antihistamines and steroid medications. Some providers have used omalizumab (Xolair), a monoclonal antibody, in difficult-to-treat idiopathic angioedema.

• #55

  https://link.springer.com/article/10.1007/s12016-025-09027-4

  The treatment strategies used for HAE-nC1INH are similar to those of HAE-C1INH and include the use of on-demand treatment (ODT), short-term prophylaxis (STP), and long-term prophylaxis (LTP). […] There are no data from controlled clinical trials, and the limited information available comes from case reports and small case series. […] Since HAE-nC1INH is a group of conditions that are similar in clinical manifestation but differ in their pathogenesis, differences in treatment responses across the different subtypes of HAE-nC1INH must be expected.

• #56 Clinical Practice Guideline: Initial Evaluation and Management of Patients Presenting with Acute Urticaria or Angioedema – AAEM

  https://www.aaem.org/statements/clinical-practice-guideline-initial-evaluation-and-management-of-patients-presenting-with-acute-urticaria-or-angioedema/

  Unless hereditary angioedema is suspected from the history and physical examination, there is no role for laboratory studies in the evaluation of acute urticaria and/or angioedema. […] The pathophysiology of ACE inhibitor angioedema remains controversial. […] Clinically, ACE inhibitor angioedema presents without urticaria. […] Emergency department treatment of the patient with acute urticaria and/or angioedema centers on antihistamines and corticosteroids. […] Regardless of whether an etiology is discovered, patients should avoid drugs that exacerbate urticaria or angioedema. […] Diagnosis of HAE requires laboratory investigation. […] Acute episodes of HAE typically do not respond to antihistamines, corticosteroids, or epinephrine. […] Disposition of the patient with angioedema can be challenging for the emergency physician.

• #57 Hereditary Angioedema (HAE) – Australasian Society of Clinical Immunology and Allergy (ASCIA)

  https://www.allergy.org.au/patients/immunodeficiencies/hae

  Diagnosis of HAE is usually considered due to typical swellings or a family history suggestive of the condition. […] There are three blood tests that are used to confirm HAE – a screening test called C4 and specific tests of the level and function of the C1-inhibitor protein. […] It is important to note that acute HAE attacks do not respond to antihistamines, corticosteroids or adrenaline.

• #58 Hives (Urticaria) and Angioedema Overview

  https://www.aaaai.org/tools-for-the-public/conditions-library/allergies/hives-(urticaria)-and-angioedema-overview

  Antihistamines are the best initial medication to treat your hives. About 50% of chronic spontaneous urticaria (hives over 6 weeks with no identifiable cause) will respond to antihistamine as discussed above. For those who do not improve on antihistamines, 65% respond to omalizumab. Omalizumab is an FDA approved treatment of chronic urticaria. Your doctor will outline a treatment plan that allows you to increase treatment during an outbreak of hives or swelling and reduce medications when the hives or angioedema are not as bothersome.

• #59 Urticaria and angioedema – BAD Patient Hub

  https://www.skinhealthinfo.org.uk/condition/urticaria-and-angioedema/

  The aim of treatment is to suppress the symptoms of the condition until it becomes better naturally rather than cure it. […] Antihistamine tablets block the effect of histamine by reducing itch and the rash in most people but may not relieve urticaria completely. […] If antihistamine tablets are not helpful, your doctor may recommend other medicines, including montelukast, that is usually used as an add-on treatment of asthma. […] A biological injection called omalizumab has been approved for severely affected chronic spontaneous urticaria patients in whom other treatments have been ineffective. This is only available in specialist clinics. […] Angio-oedema occurring without weals can be due to medicines (e.g. aspirin, ACE inhibitors). When angio-oedema occurs without weals, hereditary angio-oedema should be excluded by testing. […] The condition is due to an inherited deficiency of a blood protein or a non-functional version of C1 inhibitor and can be identified by a blood test.

• #60 Urticaria and angioedema – BAD Patient Hub

  https://www.skinhealthinfo.org.uk/condition/urticaria-and-angioedema/

  The aim of treatment is to suppress the symptoms of the condition until it becomes better naturally rather than cure it. […] Antihistamine tablets block the effect of histamine by reducing itch and the rash in most people but may not relieve urticaria completely. […] If antihistamine tablets are not helpful, your doctor may recommend other medicines, including montelukast, that is usually used as an add-on treatment of asthma. […] A biological injection called omalizumab has been approved for severely affected chronic spontaneous urticaria patients in whom other treatments have been ineffective. This is only available in specialist clinics. […] Angio-oedema occurring without weals can be due to medicines (e.g. aspirin, ACE inhibitors). When angio-oedema occurs without weals, hereditary angio-oedema should be excluded by testing. […] The condition is due to an inherited deficiency of a blood protein or a non-functional version of C1 inhibitor and can be identified by a blood test.

• #61 Diagnosis and Treatment of Urticaria and Angioedema: A Worldwide Perspective

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3651155/

  The goal of diagnostic measures is to (1) identify urticaria type and subtype and (2) identify underlying causes (in long-standing or severe chronic spontaneous urticaria only). […] In patients with CSU, it is necessary to obtain a thorough history, including all possible eliciting factors, and to identify the significant aspects of the disease. […] The ASST is the only generally available test to screen for autoantibodies against either IgE or the high-affinity IgE receptor. […] The diagnostic workup should include physical stimulation tests if physical urticaria is suspected. […] The presence of these antibodies does not prove causality, although their role as a pathogenic mechanism is debated with evidence pro and con. […] The remaining 55 to 60% of patients lacking such autoimmunity are considered to have chronic nonautoimmune or idiopathic (but nevertheless spontaneous) urticaria. […] The prognosis of AU is excellent, with most cases resolving within days; however, the prognosis of CU is variable. […] The prognosis of urticaria and angioedema is improved with prompt and proper treatment.

• #62 Urticaria and angioedema – Symptoms, diagnosis and treatment | BMJ Best Practice US

  https://bestpractice.bmj.com/topics/en-us/844?locale=no

  Urticaria is a skin condition characterized by erythematous, blanching, edematous, nonpainful, pruritic lesions that typically resolve within 24 hours and leave no residual markings. […] Diagnosis of urticaria and angioedema is based on history and physical exam; diagnosis of chronic urticaria and hereditary angioedema may involve additional laboratory testing, depending on the history. […] 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.

• #63 Hives (Urticaria) and Angioedema Overview

  https://www.aaaai.org/tools-for-the-public/conditions-library/allergies/hives-(urticaria)-and-angioedema-overview

  Hives or welts, also known as urticaria, are itchy, raised, reddish areas on the skin. Angioedema is swelling below the surface of the skin and fatty tissue. Throat swelling can be life threatening and requires immediate medical attention. Understanding the underlying mechanism of swelling or the specific disease is critical in determining the best treatment. […] Doctors will classify your hives based on how long you have had them. This helps doctors think about possible causes of your hives. When hives occur for less than 6 weeks, we call this acute urticaria. When hives last longer than 6 weeks, we change the name to chronic urticaria. Sometimes the hives in chronic urticaria can occur with angioedema (swelling). […] Talk to your doctor if swelling occurs without hives. This may suggest a special situation requiring additional evaluation. Sometimes high dose antihistamines may not improve your swelling. This is because the mechanism (cause) of swelling may be different than that of hives.

• #64 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). […] The authors will review the causes, diagnosis and management of urticaria (with or without angioedema) and isolated angioedema. […] The diagnostic and therapeutic approaches to these two conditions are considerably different, and this review is designed to highlight these differences to the reader. […] The diagnosis of urticaria, with or without angioedema, is based primarily on a thorough clinical history and physical examination. Based on the history and physical exam, diagnostic tests may also be considered to help confirm a diagnosis of acute, chronic or inducible urticaria. […] Certain diagnostic tests and assessments can be helpful in the diagnosis and differential diagnosis of CSU, including: a complete blood count (CBC), and erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) as markers of inflammation.

• #65 Urticaria and Angioedema: A Practical Approach | AAFP

  https://www.aafp.org/pubs/afp/issues/2004/0301/p1123.html

  Physicians should ensure that proper health maintenance testing is up to date and consider diagnostic testing directed by history and physical examination findings, especially in patients with chronic urticaria. […] Challenge testing, which reproduces exposure to the suspected stimuli in a supervised clinical environment, can confirm the diagnosis.

• #66 Hereditary Angioedema

  https://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/allergy/hereditary-angioedema/

  The diagnosis of HAE should be considered in individuals presenting with recurrent episodes of swelling, particularly if the swelling is not responsive to antihistamines or steroid therapy. The diagnosis should also be considered if swelling episodes are not associated with hives. Published guidelines for the diagnosis of HAE are available. […] Unlike HAE, the main mediator of angioedema in the setting of hypersensitivity reactions or urticaria/angioedema syndromes is histamine, not bradykinin. Bradykinin is responsible for recurrent episodes of swelling seen in HAE and in acquired angioedema and ACE inhibitor-induced angioedema syndromes. […] Defects in C1 inhibitor lead to unregulated breakdown of the complement cascade including low levels of C4, C1 inhibitor, and/or C1 inhibitor function, seen at baseline and during an acute attack. […] There are no laboratory tests that can confirm HAE with normal C1 inhibitor or ACE inhibitor-induced angioedema.

• #67 Hives (Urticaria) | Causes, Symptoms & Treatment

  https://acaai.org/allergies/allergic-conditions/skin-allergy/hives/

  Hives, also known as urticaria, affects about 20 percent of people at some time during their lives. […] If you think you might have hives, then it’s best to speak with an allergist. […] The best way to identify your symptoms is to talk to an allergist who can diagnose and treat both hives and angioedema. […] A single episode of hives does not usually call for extensive testing. […] Chronic hives should be evaluated by an allergist, who will ask about your and your family’s medical history, substances to which you are exposed at home and at work, exposure to pets or other animals and any medications you’ve taken recently. […] Your allergist may want to conduct skin tests, blood tests and urine tests to identify the cause of your hives. […] If a specific food is the suspected trigger, your allergist may do a skin-prick test or a blood test to confirm the diagnosis; once the trigger is identified, you’ll likely be advised to avoid that food and products made from it.

• #68 Diagnosis and Treatment of Urticaria and Angioedema: A Worldwide Perspective

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3651155/

  Urticaria and angioedema are common clinical conditions representing a major concern for physicians and patients alike. […] The Scientific and Clinical Issues Council of WAO proposed the development of this global Position Paper to further enhance the clinical management of these disorders through the participation of renowned experts from all WAO regions of the world. […] This position paper provides updates on recent advances in the understanding of etiologic factors, pathogenic mechanisms, diagnostic methods, and medical management of acute and chronic urticaria (CU) and angioedema. […] The objectives of this WAO position paper on urticaria and angioedema are to provide updated information on the assessment and treatment that should be applied in health care settings worldwide to obtain a better symptom control, improve patients’ quality of life, contribute to patient education, and enhance accessibility to more effective therapies.

• #69 Angioedema – Australasian Society of Clinical Immunology and Allergy (ASCIA)

  https://www.allergy.org.au/patients/skin-allergy/angioedema

  Symptoms usually start to settle within one to two hours after taking antihistamines and it is best to take them on a regular basis rather than when symptoms appear. […] The aim of treatment is to stop the episodes of angioedema from happening, make them less frequent or make the symptoms less severe.

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