Relapsing polychondritis can be life-threatening and requires high attention!

Author: Wang Guangfa, Chief Physician, Peking University First Hospital

Reviewer: Wang Lixiang, Chief Physician, Third Medical Center, PLA General Hospital

The 10th Chairman of the Science Popularization Branch of the Chinese Medical Association

Relapsing polychondritis is a relatively rare disease, but it is easily misdiagnosed or missed. Its main manifestation is recurrent inflammatory reactions of cartilage tissue. Its systemic manifestations may include low-grade fever and fatigue, but these symptoms are particularly hidden, and many patients do not have systemic manifestations.

The local manifestations of relapsing polychondritis actually depend on the affected part. The most prominent and common symptom is the involvement of the auricle. When the auricle is involved, there will be local pain, tenderness, and skin erythema, which are typically bilaterally symmetrical. However, if the nose is involved, it will be pain and tenderness in the bridge of the nose, which is a manifestation of the acute phase.

Over time, the ears may shrink and collapse, looking a bit like cauliflower stuck on the head. Some people call it cauliflower ears. The nose is collapsing in common parlance, and the scientific name is saddle nose, which means that the nose is like a saddle with a collapse in the middle. This is a late stage manifestation.

Figure 1 Original copyright image, no permission to reprint

In addition, the inner ear may also have some symptoms. For example, it may cause the Eustachian tube to be blocked, which may lead to otitis media, pain, hearing loss, and even perforation in some people. For example, if the cochlea is affected, tinnitus, hearing loss, dizziness, etc. may occur.

If the heart is affected, the patient may have some cardiac symptoms, such as cardiac enlargement and heart failure in severe cases, and heart murmurs may be heard during physical examination. If the large blood vessels are affected, aneurysms may occur, which may rupture over time, and rupture is very dangerous, with the possibility of massive bleeding and life-threatening.

Of course, some patients may develop rashes if the skin is affected; conjunctivitis, iritis, iridocyclitis, and uveitis may occur if the eyes are affected, and the patient may have vision problems at this time; if the nervous system is affected, such as cranial nerves, it will manifest as diplopia.

These are some of the manifestations it may cause.

What you need to know is that relapsing polychondritis is very different from ordinary chondritis:

First, in terms of the mechanism of occurrence, relapsing polychondritis is often an autoimmune mechanism, that is, one's own immune cells destroy one's own tissues, and one's own people fight one's own people. However, compared with some other autoimmune diseases, its mechanism is relatively unclear, because there is no specific autoantibody against one's own cartilage, so this type of disease is more difficult to diagnose. Ordinary cartilage inflammation is more common, such as viral infection may cause costochondritis, which is quite common clinically, and it may be cured after one infection, unlike relapsing polychondritis, which recurs repeatedly.

Second, it is polychondritis, which means that the cartilage in multiple parts is damaged instead of just one part, from the cartilage in the auricle, to the cartilage in the nose, to the cartilage in the airway, to the cartilage in the joints. In contrast, in common chondritis, only one part is affected.

Of course, not all cartilage parts will show signs of damage in relapsing polychondritis, only some parts with obvious damage will show signs of damage. And the degree of cartilage damage is not necessarily the same, some parts may be more severe, some parts may be less severe, some parts may appear first, and some parts may appear later, there are differences.

Third, it is prone to relapse, which is a clear difference from other chondritis.

For relapsing polychondritis, it is very important to solve the problem of early detection and early diagnosis.

After early detection and early diagnosis, the most important thing is to treat with glucocorticoids. Generally, 30-60 mg of hormones are enough for an adult. However, although hormones can quickly relieve symptoms, the characteristic of relapsing polychondritis is relapse. If the hormone dosage is reduced too quickly, it will relapse, so the hormone dosage should be reduced slowly while observing the changes in the condition.

Figure 2 Original copyright image, no permission to reprint

There is a test that is considered to be valuable for monitoring the condition of relapsing polychondritis, which is PET/CT. What is PET/CT? We inject glucose into the patient, and this glucose is labeled with isotopes. After labeling, it is injected into the body. Any part with inflammation will absorb more glucose, and the signal in this place will be high, so it helps us observe the activity of the disease.

So at this time, we can be of great help to the doctor. Whether the hormone dosage should be further reduced or increased, this PET/CT examination can help us decide.

In addition, hormone therapy is only one aspect, and relapsing polychondritis is prone to relapse, so on the basis of using hormones to relieve acute inflammation, we should also consider using some immunosuppressants, such as cyclophosphamide, methotrexate, and azathioprine.

These drugs are used for a long time, at least three months, before you can consider stopping hormones or immunosuppressants. Even if the condition is stable and the drugs are stopped, long-term follow-up is required because you don't know when the disease will relapse.

There are some other drugs now, such as anti-TNF-α (anti-tumor necrosis factor α) antibodies, which are antibodies against tumor necrosis factor α. There are also some antibodies like interleukin 6 receptor, which can be considered for use, but this type of drug is relatively expensive and the efficacy is not very certain, so it can only be tried for patients who are difficult to control.