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What is Angioedema - You must know about it

What is Angioedema


In simple words, Angioedema can be described as a type of swelling inside the deeper part of the skin’s inner layer and beneath. The swelling is often caused by an accumulation of fluid over a period of several minutes to a few hours.

Some other names of Angioedema are:

  1. Angiooedema
  2. Quincke's edema,&
  3. Angioneurotic edema
In normal cases, the symptoms of Angioedema can affect any of the body parts of the patient. However, the swelling takes place in the following body parts mostly.

  • Hands
  • lips
  • genitals
  • eyes
  • feet

In some of the cases, it has been observed that the swelling occurs along with the advent of hives. Therefore, angioedema is also known as giant hives as well.



These hives are prickly and raised as well in nature. They are red welts that develop on the surface of the skin and it involves only two layers of skin. Hives are also known as Urticaria as well.

It has been observed that both hives and angioedema can be effected through:

  • Intolerance towards certain types of food or through an allergic reaction.
  • Allergen in the surroundings of the person such as venom from insect bites, pollen, pet dander, etc.
  • Allergy/ Side effect towards a particular medicine.


In certain unusual cases, swelling can be an indication of a much bigger health complication like non- Hodgkin B-cell Lymphoma. Certain body parts, such as the eyelids, tongue, lips are more likely to get affected by angioedema than other parts.

History


Even though there are certain prescriptions written in the early part of the 17th century, yet it was Heinrich Quincke (26-August 1842 to 19 May 1922), the famous German surgeon who was the first person to create a clinical picture of angioedema in the year 1882.

In the year 1888, William Osler, (a Canadian physician and one of the founders of John Hopkins Hospital) derived the fact that certain cases of angioedema might be hereditary in nature. Therefore, it was he who coined the term ‘hereditary angioneurotic edema’.

In the year 1963, it was established that angioedema had a link with C1 esterase inhibitor deficiency.

Who all are affected by Angioedema?


This particular disease is a very common disease which affects around 10 to 20% of people in their entire life. In the coming stages of this blog, we shall study about 4 types of angioedema. You need to understand that certain types of angioedema are more common than others.

Out of the 4 types, Hereditary angioedema is one of the rarest forms of angioedema as it affects only 1 in 50,000 people worldwide.

Symptoms of Angioedema


The main symptom of angioedema is the ‘swelling’ which happens below the deeper part of the skin’s inner layer and beneath.

a) Swollen Skin

As already mentioned, the reason behind the swelling is an accumulation of fluid under the skin. Normally it affects the eyes, lips, hands, feet, genitals.

However, if the situation turns out to be critical, one can also see swelling inside the throat lining. Thus, both eating, breathing and bowel movement could be affected and if not treated immediately could be fatal in nature.

As far as the nature of the swelling is concerned, it comes up without any warning most of the time and is more serious in nature when compared to normal hives.

Sometimes the patient could feel a pricking sensation in the affected body part just before the swelling appears. The duration of the swelling is normally between 1 to 3 days.

Note – Even in the case of swelling, the skin color of the patient may not change and on the top of it, the swelling is not itchy. But there is an exception to this situation.

If the patient is suffering from allergic or idiopathic angioedema, there is a high probability that he would also contact hives. And hives, as we all know, is itchy by nature.

Some Other Symptoms of Angioedema


  • Along with the swellings, patients of angioedema could also show some of these symptoms:
  • Swelling inside the throat, tongue, and windpipe. This makes eating and breathing extremely difficult.
  • Painful feeling in the swollen body parts.
Additionally, if any patient is suffering from hereditary angioedema, they may also face the following complications:

  • The swelling of the urethra or bladder can be responsible for problematic behavior as the patient would suffer from difficulties in passing the urine and bladder problems as well.
  • The patient can be suffering from vomiting, diarrhea, nausea which is caused by swelling and pain in the bowel and stomach.

Causes of Angioedema




In this case, the cause of angioedema is directly linked to the type of angioedema from which the patient is suffering.

Therefore, there are basically 4 types of angioedema and their related causes.

  • Hereditary angioedema
  • Idiopathic angioedema
  • Allergic angioedema
  • Drug-induced angioedema

Let us discuss them one by one.

Hereditary angioedema (HAE)

In simple words, in a situation where angioedema is passed from one generation to another, it is known as hereditary angioedema.

This disease is caused by a change in the DNA in the C1 esterase inhibitor (C1-INH) gene. These genes are the ones that decide upon the code for the features of the next generation such as hair color, eye color, etc.

Since there is a change (mutation) in the C1-in gene, the body of the patient suffering from this disease will not make enough amount of C1-INH protein.

If there is a lack of C1-INH protein, the immune system would then produce the symptoms of angioedema as the protein has a very vital role in the regulation of the immune system of the body.

In the case of HAE although there is no direct cause which can be zeroed upon yet there are certain aspects which are believed to activate the hereditary angioedema, such as:

  • Oral contraceptive pills
  • Mild trauma including infection or surgery
  • Pregnancy
The patient who is suffering from HAE suffers from intense abdominal pain which is known as attacks. This is followed by complications like non-itchy rashes, weakness, watery diarrhea, and intense vomiting as well.

These abdominal attacks can increase the WBC count in the range of 13,000 to 30,000. Also, the pain can be unbearable and would require hospitalization for the patient in most of cases.

As and when the symptoms begin to loosen up, the WBC count returns to the normal range.

Important – It must be noted especially by the medical students that the symptoms and diagnostic tests are almost similar when HAE is compared to Perforated Appendicitis.Therefore, if not diagnosed properly, the HAE patients may be mistakenly advised to go for laparotomy or laparoscopy without any reason.This would put unnecessary emotional and financial pressure on the patient and also the efforts of the medical team engaged in such kind of operations would be wasted.

There are basically 3 types of hereditary angioedema.

1. Type I – In this type of HAE, the levels of C1-in decreases (85 %.)
2. Type II – In this type of HAE, the level of C1-in is stable but the function is decreased (15%)
3. Type III – There is no detectable anomaly in C1-in.

This happens in an X-linked dominant fashion. As a result, it affects the womenfolk.

Note – In Type III, it can be exacerbated by the usage of hormonal contraception and pregnancy. Also, this has been attached to the mutations (Factor XII gene).

Idiopathic angioedema


These are the cases of angioedema where the cause of angioedema has not been figured out yet by the medical community. It is assumed that a certain type of problem with the immune system of the patient may have caused it to ‘misfire’(that’s the best word to explain it)

In such cases of idiopathic angioedema, there could be certain possible prompts that may lead to swelling in the patients.

Some of them could be


  • Excessive hot or cold temperatures
  • Certain exercises
  • High levels of anxiety or stress
  • Some minor infections

The best way out of this situation is to try and avoid these prompts as much as possible so that you can see a positive impact on the symptoms of angioedema.

Allergic Angioedema


In order to understand this angioedema- let us visit the basics of the immune system once again. Under normal conditions, our bodies are always protected by the immune system inside us from various diseases, illnesses, and germs.

But if you happen to get in contact with allergic angioedema, your immune system would attack the purely naïve things in your blood.

As a result, your body would start producing histamine which would make the blood vessels in the affected area to expand and your skin would start swelling.

Let us quickly go through a list of things that are in-famous for prompting allergic angioedema, such as:

Insect bites – for example, wasps, honeybees. etc.
Medicines – For example, Aspirin, NSAIDs such as ibuprofen and penicillin.
Latex – It is a very common thing that is used to manufacture condoms, medical gloves, etc.
Food – Some people are allergic to eggs, prawns, peanuts, butter, and milk as well.

Drug-induced angioedema


It is no big surprise that many medicines come with certain side effects. Similarly, there are certain medicines that have turned out to be the reason for angioedema.

For example, Angiotensin-converting enzyme (ACE) inhibitors which are commonly used to treat hypertension amongst patients have been found to be the reason for drug-induced angioedema.

Reports also suggest that certain races like the black people, when treated with Angiotensin-converting enzyme (ACE) inhibitors are 3-4 times more likely to get affected by this particular side effect thing as compared to the white community people.

Further, it is projected that around 1 to 5 % of the total population who have been prescribed Angiotensin-converting enzyme (ACE) inhibitors are most likely to suffer from the effects of drug-induced angioedema.

Diagnosis of Angioedema


At the outset, the medical staff can easily diagnose angioedema by examining the affected body part and the related swelling.

However, in order to ascertain which particular type of angioedema has affected the patient, one needs to conduct allergy and blood tests to determine the type.

Note – As already mentioned above, Idiopathic angioedema is the only type of angioedema which is diagnosed if no cause can be found.

For a detailed diagnosis, the doctor needs to rely upon the clinical checklist. Tests related to kidney function, blood count, liver enzymes, electrolytes are basically done.

In case of serious situations, the Mast cell tryptase levels may be raised up, if the doctor figures out that the attack (stomach pain) as a result of an acute anaphylactic reaction.

When the condition of the patient improves, then the specific diagnosis may reveal the exact cause. Particularly reduction of complement factors may specify the deficiency of C1-inhibitor.

In this regard, it should be noted that HAE type III is actually a diagnosis of exclusion which consists of observed angioedema accompanied with normal C1 levels and function.

As already mentioned in the section of hereditary angioedema, the HAE can remain undetected for a longer period of time because of its striking similarity with respect to symptoms, when compared to intestinal colic or allergies.

So how do we figure out what has exactly happened?


When a patient is administered antihistamines and hereditary angioedema does not respond to that, we can clearly see the difference from allergic reactions.

It is a well-known fact that it becomes difficult for doctors to diagnose the hereditary angioedema in patients whose chapters are confined to the gastrointestinal tract itself.

Now if the family history of HAE is not available immediately, then only a detailed analysis by the pathological lab can confirm the status of HAE.

In the due process of this analysis, it has been observed that it is normally a reduced complement factor C4, instead of C1-INH deficiency itself, which is detected.


How is angioedema treated?



It is a common practice to avoid using medicines immediately if the situation is very minor in nature as in most cases of angioedema, the patient starts to feel better after a couple of days.

However, for cases of idiopathic angioedema and allergic ones, Oral steroid tablets and antihistamines are recommended and used in order to reduce the swelling.

Whereas in case of drug-induced angioedema, a patient can be treated by prescribing alternative medication in order to treat the underlying condition that the patient has.

In the case of hereditary angioedema, there is no permanent solution. This particular angioedema does not respond to steroids, antihistamines or adrenaline.

A number of different kinds of medicines are used in this case with the objective of stabilizing the protein levels in the blood of the patient.

Note for Medical Students - Serious angioedema can be life-threatening if swelling causes your throat or tongue to block your airway.

Let us learn about two more important medications here – Antihistamines and Corticosteroids.
Antihistamines

Histamine is a chemical compound present inside our body. This is one of the key chemicals which causes our skin to swell. Antihistamines' role is to stop the effects of histamine so that the swelling can be controlled.




What is the process?


for cases of idiopathic angioedema and allergic ones, a two-week course of Antihistamines (non-sedating type is recommended.

This includes two well know names – cetirizine and fexofenadine. Out of these two, one can easily buy cetirizine without going to a doctor for medical prescription. Whereas in the case of antihistamines, you can inject the patent at the hospital or GP.

Note – If your patient is suffering from Hives and is not able to stay in peace, ask your GP to prescribe an older kind of antihistamines before the patient goes to sleep. The idea behind it is that since the older one is a sedating type; it could help the patient is sleeping.

However. If the patient has been prescribed the new types of antihistamines, then some of them would still feel drowsy. Therefore, as a precautionary measure, the patient must avoid working with complex machines, driving any sorts of vehicles and avoid drinking alcohol.

Also, there are certain short-lived side effects of newer antihistamines that go away very soon, such as the dry nose, dry mouth as well as a mild headache.


Corticosteroids


This type of medicine is a type of steroid. In most of the cases, the doctors recommend going for a three to five-day course of steroid tablets. These steroids show their effect by blocking the patient’s immune system’s actions. In most of the cases, prednisolone is prescribed by the doctors.

It may be noted that the patient might feel drowsiness after consuming prednisolone. Therefore, as a precautionary measure, just like in the case of antihistamines, the patient must avoid working with complex machines, driving any sorts of vehicles and avoid drinking alcohol.

There could be certain side effects of prednisolone such as tiredness, drowsiness, stomach pain, nausea, oral thrush, etc.

Please note that Steroid injections must be administered only by an allergy specialist or immunologist and must be used only during extremely severe cases.

Medication for Hereditary Angioedema


The particular medicine which is used to treat hereditary angioedema would come under the purview of these two sub-sections.

  1. Medicines are used to provide relief from the symptoms.
  2. Medicines that are used for preventing Angioedema.

Let us take a quick tour of some medications.

Icatibant


Icatibant is administered inside the patient through an injection under the skin. In this case, it has been observed that a second injection is often required for better results.

It is a new medication used to treat short term duration of swelling. Icatibant works by stopping the effects of some of the chemicals which can be held responsible for swelling in cases of HAE.



  • Some of the common side effects of Icatibant include:
  • Itchiness in other certain areas of the skin  
  • Dizziness.
  • A series of adverse skin reactions at the site of the injection – such as redness, pain, itching or swelling.

Danazol

This particular medicine helps boost levels of the C1-INH protein. This means that it is a synthetic hormone that is pretty effective in preventing the symptoms of angioedema.

However, there could be certain side effects such as high blood pressure, irregular or absent periods in women. liver issues, excessive body hair growth, more muscle tone, flattening of the chest (all things associated with men but found in women), depression as well as weight gain.

It is because of all these side effects; a lot of people are not comfortable with danazol and prefer taking Stanozolol which has better tolerance against all these factors.

Tranexamic Acid

Preferred by women mostly, it is used as an alternative to Danazol. It does not have so many side effects as compared to Danazol.

C1 inhibitors


This particular medicine is needed to prevent attacks that are caused by surgical trauma. The purified C1-inhibitor concentrate is used for substituting the protein which is missing in the blood of the patient.

It is a common practice for many patients to keep a stock of inhibitors at their homes and offices in case of an emergency.

Note – Both C1-inhibitor concentrate and Icatibant are prescribed by a specialist immunology unit. This is done while treating patients who are suffering from hereditary angioedema.

Seeking Medical Advice


You need to decide what kind of medical advice you need at a particular moment of time. There could be two instances:

a) Time to seek normal medical advice

You may contact the GP, if the patient in front of you, is not getting issues with his breathing. You need to conduct various types of tests to ascertain the type of angioedema the patient is suffering from.

b) Time to seek emergency medical advice

If you see that the patient is suffering from a severe allergic reaction, then you must immediately inform the authorities and also inform the person (emergency ward) that you are suspecting anaphylaxis.

How to analyze the symptoms of anaphylaxis?


You can check for two things in particular:

  • Whether there is any type of swelling of the tongue and throat
  • Whether you can feel stiffness in the chest leading to breathing problems 

FAQs 

1. Is angioedema serious in nature?


While it has been observed that angioedema does not cause any type of serious damage to the heart, kidney-pancreas, etc.

However, if the swelling takes place inside the throat or tongue, then it results in severe blockage of the respiratory system, essentially causing difficulty in breathing. And if timely treatment is not provided to the patient then it may be fatal in nature.

2. How do you test for angioedema?


There are several ways that one can use for the diagnosis of angioedema. Some of them you can do by yourself as well.


  • You may check for any type of unexplainable swelling on eyes, lips, tongue, arms, legs or any other part of your body. Also,  Discoloration and blanching are a sign of angioedema.
  • In case of swelling, you can check out the food that the patient has consumed and whether the patient has a previous record of being allergic to that particular food. This will help you in understanding the cause of angioedema. Also, you can check the family history of the patient to know about the possible chances of the younger generation being allergic to the same kind of food.
  • You can conduct a blood test in a laboratory to detect elevated levels of immunological activity as such.
  • You can also go for a genetic test to check out for any specific genetic mutations that may cause angioedema.


3. Sometimes the lips of a patient swell overnight. What could be the possible reasons?


It may happen that the patient is allergic to certain kinds of foods such as peanuts, eggs, milk, etc or certain types of medicines like penicillin.

These things may cause allergic reactions inside a patient due to which the lips might be swollen. The best way is to stay away from such things if the patient has a known history of allergic Angioedema.

4. What is the best antihistamine for angioedema?


 Medicines such as loratadine (Claritin), cetirizine (Zyrtec) and diphenhydramine (Benadryl), are proven antihistamine drugs for angioedema. These drugs act by subduing the hyperactive immune system and helps in reducing the swelling in the due process and course of time.

5. Does angioedema ever go away completely?


It has been observed that cases related to idiopathic angioedema, allergic angioedema & drug-induced angioedema, the symptoms and swelling can go away by themselves or through properly prescribed antihistamine drugs or steroids.

However, in the case of hereditary angioedema, there is no permanent cure as this particular angioedema does not respond to steroids, antihistamines or adrenaline.

6. How long does ACE inhibitor angioedema last?


 As it is well known that in the year 1992, after Israeli and HalP reviewed the entire literature on cough and angioedema, particularly related to ACE inhibitors, they came to an understanding that angioneurotic edema happens within certain hours or can appear after 7 days after the ACE inhibitor was started.

However, according to one report, it was observed that after long therapy was developed with an ACE inhibitor as well.

7. How long does it take for angioedema to get resolved completely?


Depending upon the type of angioedema, from which the patient is suffering it can take up to 1 to 3 days or even can go up to 5 days. In most of the mild cases, angioedema is self-healing as the body immunity takes care of it with due course of time.

8. What kind of doctor treats angioedema?


Since it is a special type of diseases and it is really important to find out the root cause of the problem, therefore hospitals have certain trained allergy specialists or immunologists. Under their supervision, any kind of antihistamine,  steroids or injections is administered.

9. Can stress cause swollen eyes?


While it may sound strange to common people but if due to stress, you are not getting proper sleep at night, you may experience puffiness around your eyes in the morning. The reason being - the salt proportion inside your body gets disturbed due to stress.

This, in turn, leads to retaining of water in your eyes and your eyes would end up swelling in such a case.

10. Is angioedema always an emergency?


If the patient is not facing any issues with his/her breathing then, you have some time in hand before you start the treatment. In mild cases of angioedema, it normally takes 1 to 5 days to self-heal.

But in case the patient is suffering from breathing problems, then it can be considered as a case of anaphylaxis and this is a matter of emergency where you need professional medical assistance from the doctors and hospital.



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