Is orbital inflammatory pseudotumor a tumor? How is it treated?

Author: Xie Like, Chief Physician, Eye Hospital, China Academy of Chinese Medical Sciences

Reviewer: Wei Junping, Chief Physician, Guang'anmen Hospital, China Academy of Chinese Medical Sciences

Orbital inflammatory pseudotumor is common in adults, mainly in the age group of 20-50 years old. Once it occurs, it has a great impact on the family, society and individuals.

So, what exactly is orbital inflammatory pseudotumor? Is it a tumor?

1. What is orbital inflammatory pseudotumor?

Orbital inflammatory pseudotumor is a nonspecific, benign inflammatory change, or inflammatory lesion, that occurs in the eye. It is mainly characterized by lymphocyte infiltration and is an immune inflammation. The cause is currently unclear, so it is called nonspecific inflammation.

When orbital inflammatory pseudotumor occurs, the location is relatively limited, and even some tumor-like changes are formed locally. From the appearance or CT scan, it looks like a tumor, so it is called a pseudotumor, but it is not a real tumor.

Figure 1 Original copyright image, no permission to reprint

Orbital inflammatory pseudotumor is divided into lymphocytic infiltration type and fibrous tissue proliferation type according to morphological changes. The coexistence of both types is called mixed type. According to the characteristics of the disease, it is divided into acute phase, subacute phase, chronic phase and remission phase. According to the site of disease onset, it is divided into eye muscle type, lacrimal gland type, orbital tissue type and periocular type.

Orbital inflammatory pseudotumor is usually seen in one eye, but it can also occur in both eyes. Some patients develop inflammatory pseudotumor in one eye during the entire course of the disease, while some patients develop it in both eyes. There are also some patients who develop it in both eyes one after another, and it may be the right eye in the early stage, and then the left eye will gradually develop the same disease during the course of the disease.

Orbital inflammatory pseudotumor ranks third among orbital diseases, accounting for 7%-12% of the incidence.

2. What causes orbital inflammatory pseudotumor?

It is now believed that orbital inflammatory pseudotumor is caused by both infectious and non-infectious factors.

Infectious factors, the most common of which is bacterial infection, but after a large number of case studies, it was found that infectious factors rarely cause it. Most of them are immune, called non-infectious inflammation. The pathogen cannot be found and there is no clear cause. The onset of the disease may be related to fatigue and decreased resistance.

Figure 2 Original copyright image, no permission to reprint

If the cause of the disease is clear, the treatment effect will generally be very good. If the cause is unclear, immune and functional diseases will be more difficult to treat.

3. What are the symptoms of orbital inflammatory pseudotumor?

The lymphocytic infiltration type often presents with local pain, swelling, discomfort when opening the eyes, and even headache, irritability, etc. in the early stages.

In the fibrous tissue hyperplasia type, the pain is not very severe, and symptoms such as protrusion of the eyeball or eye movement disorder and double vision may occur.

Mixed type: the redness and swelling are not too severe, and the pain is not that intense. Sometimes the pain lasts for two hours this afternoon and may last for one hour tomorrow. The symptoms are relatively mild.

Inflammation usually manifests itself as redness, swelling, heat, and pain. The same is true for inflammatory pseudotumors. The most common symptoms are red eyes, eye pain, swelling, and edema. These are the most common symptoms.

Of course, inflammatory pseudotumors in different parts of the body also have their own characteristics. For example, eye muscle inflammatory pseudotumors are mainly painful, while lacrimal gland inflammatory pseudotumors can cause swelling of the lacrimal glands. The lacrimal glands are located above and outside the eye sockets, and swelling in this area is the main symptom. Pulling open the eyelids will reveal swelling and prolapse of the lacrimal glands. Under normal circumstances, the lacrimal glands cannot be seen with the naked eye.

Diagnosis of orbital inflammatory pseudotumor is based on the patient's symptoms, physical examination, specific signs, and imaging examinations and laboratory tests, including CT, MRI, B-ultrasound, etc. In recent years, some patients have been found to have hematological changes with increased IgG4. Through concentrated analysis of these aspects, a more accurate diagnosis of inflammatory pseudotumor can be made.

4. How to treat orbital inflammatory pseudotumor?

The treatment principles of orbital inflammatory pseudotumor are, on the one hand, to eliminate symptoms and on the other hand, to reduce recurrence.

Western medicine treatment measures include hormones, immunosuppressants, radiotherapy, and surgical resection for localized lesions. The most commonly used treatment is hormone therapy, or hormone combined with immunosuppressant therapy.

There are several ways of hormone treatment. The first is systemic intravenous infusion of hormones, the second is oral administration, the third is local injection of hormones, and the fourth is to use hormone eye drops and apply hormone eye ointment on the eyelids.

The efficacy of systemic medication is very obvious. If you use hormones today, you will see obvious effects tomorrow. However, systemic hormones also have disadvantages. When the hormone reaches a certain dose, it will relapse if you suddenly stop taking it or reduce the dose. The course of the disease is very long. In addition, the side effects of systemic hormones are also very large.

Orbital inflammatory pseudotumor is a non-infectious inflammation or immune inflammation. The cause is not very clear, which leads to the lack of good specific treatment methods in Western medicine.

In addition to Western medicine, there is actually another treatment method, which is traditional Chinese medicine. Traditional Chinese medicine has a history of thousands of years and has formed a very effective method for treating orbital inflammatory pseudotumor.

Whether it is through syndrome differentiation or early, middle and late stage treatment, TCM has provided us with a lot of strong evidence for treatment, and the treatment effect is relatively ideal. Some patients who take hormones can gradually reduce the amount of hormones after TCM treatment until they stop taking the medicine, preventing recurrence. Many patients stop taking hormones and use TCM treatment completely, maintaining very good results.

The most commonly used method of TCM treatment for orbital inflammatory pseudotumor is oral Chinese medicine. Of course, there are also acupuncture and physical therapy. Oral Chinese medicine is a very effective treatment measure.