Chronic Granulomatous Disease

Chronic Granulomatous Disease

Chronic granulomatous disease, also known as Bridges-Good syndrome or Quie syndrome, is a joint name for a large group of genetic disorders. However, all these disorders have something in common. In each of them some cells within the immune system fail to produce the reactive oxygen compounds whose purpose is to eliminate certain viruses, bacteria, fungus, protozoa, prions or other microorganisms which can cause diseases.

Lack of these compounds results in the formation of granuloma in many organs. It is important to understand what granuloma is.

Granuloma is a form of inflammation developed from macrophages, cells within the immune system which digest anything that does not match their version of a healthy cell. Macrophages eliminate cancer cells, microbes, cellular debris, dead cells and other things that a healthy body does not need.

Granulomas are formed when immune system fails to eliminate some foreign substances. In this case, granulomas are used to form a wall between these foreign substances and the surrounding healthy cells.

This is why, when the immune system fails to create the compounds which should eliminate viruses, bacteria, fungus, protozoa, prions or other microorganisms, granulomas are formed to try and wall off the unwanted foreign substances.

Other forms of granuloma-related diseases

Aside from this, there are many other forms of inflammatory granuloma. For example, calcified granuloma which forms from calcium deposits on the areas affected by old inflammation. A common example is calcified granuloma which can be caused by tuberculosis that has been treated years earlier.

Granuloma inguinale, also known as granuloma genitoinguinale or donovanosis is a form of ulcerative genital lesions often mistaken for syphilis and is often left untreated which makes it dangerous since it can cause destruction of tissue.

Lung granuloma often stays undetected because in many cases it produces no symptoms, but chest x-ray will show it. Similar to it due to the location is eosinophilic granuloma, which is a lung disease related to smoking whose prognosis greatly depends on the patients’ cessation of smoking.

Symptoms of Chronic Granulomatous Disease

Having in mind the variety of foreign organisms and their diverse effects, chronic granulomatous disease is actually a name used to represent a large number of immunodeficiencies.

One of the common issues is recurrent infection. It occurs because the immune system is weakened and it can’t fend off the attacks. However, these infections are easy to notice, since they always appear in certain forms.

Chronic Granulomatous Disease Symptoms
Chronic Granulomatous Disease Symptoms
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For example, pneumonia is the most common one. The other are osteomyelitis, abscesses of the organs and skin, bacteremia, suppurative arthritis, fungemia and various skin infections.

However, the overall weakening of the immune system also means that some atypical infections may occur. These are the ones which do not normally occur with the people with healthy immune system.

Treatment of Chronic Granulomatous Disease

There are several different courses of treatment.

Antibiotics are used to prevent bacterial infections, most commonly trimethoprim-sulfamethoxazole. However, it can cause severe hepatic failure and liver damage, renal insufficiency, nausea, depression, bone marrow depression and uremia for patients suffering from renal problems. Moreover, it is advised against using with some medicines such as ACE inhibitors, dapsone, rifampicin and many more.

Itraconazole and voriconazole are used for fungal infections. Both of them are better tolerated than trimethoprim-sulfamethoxazole, but they can also cause nausea, abdominal pain, fatigue, jaundice, fever, rash and itching, sepsis, peripheral edema, changes in urine color, pale stool and headache. Voriconazole is also phototoxic.

Immunomodulaiton is performed using interferon. It reduces infections by up to 70%.

Hematopoietic stem cell transplantation can cure chronic granulomatous disease, but it requires a matched donor and comes with a number of risks.

Palmitoylethanolamide (PEA) is another solution for patients suffering from chronic granulomatous disease. It helps relieve inflammation and infections and it greatly helps the immune system. It affects several different receptors which play an important role in different parts of the body.

For example, PEA works with anandamide, which is naturally present in the body and this is the way it affects CB2 receptor. This receptor is spread throughout the immune system and it affects B cells, which secretes cytokines and antibodies and presents antigens. It also helps hematopoietic stem cells, which we have already said can actually cure chronic granulomatous disease. They give rise to all types of blood cells. The same receptor is responsible for regulating the performance of macrophages, which are cells responsible for cleaning the body of various kinds of cellular debris, microbes and cancer cells.

As we have already said, granuloma is a form of inflammation and PEA is very effective for relieving and even eliminating excessive inflammation. This is done through its effects on other receptors including PPAR-alpha, GPR55 and GPR119. In fact, the first effects of palmitoylethanolamide (PEA) were discovered when scientists noticed that it relieves inflammation.

The same mechanism sits behind its positive effects against allergies. The benefits come from calming mast and glia cells, as well as controlling the influx of neutrophils.

However, the overall improvement of the immune system is not the only benefit of PEA. Just like the mentioned anandamide, palmitoylethanolamide is endogenous – naturally produced in the body. It is completely safe in any amount and can be used with any drug that you might be using to treat chronic granulomatous disease or any other condition for that matter.