Li Yuchun's "immortal cancer", why is the diagnosis time delayed by an average of 6 years in China?

"My spine has not been very good, and I had problems with my spine during rehearsals for the 2018 "Pop" concert and at one point I needed to travel
in a wheelchair.
Last year, a new condition began to appear, the body is like petrified, it is difficult to lie down smoothly, and there is no way to ensure
sleep.
Recently, Li Yuchun revealed for the first time in a talk show that he was diagnosed with ankylosing spondylitis
last year.
After falling ill, she also composed the song "Five Visceral Organs"
from the perspective of a patient.

Ankylosing spondylitis is an autoimmune disease
characterized by chronic inflammation and abnormal ossification.
Because it cannot be cured and the frequency of recurrence is high, the disease is also called "immortal cancer"
.
In addition to Li Yuchun, stars including Jay Chou, Zhang Jiayi, Cai Shaofen and others also suffer from the disease
.
In China, the prevalence of ankylosing spondylitis is about 0.
3% ~ 0.
4%, and about 5 million patients are suffering
from this disease.

Source: Screenshot of the "My Bronze Age" video

"Tonic pain is the 'surface' and bone damage is the 'inside', the latter being a more worry
.
" Zeng Xiaofeng, director of the National Clinical Research Center for Skin and Immune Diseases and director of the Department of Rheumatology and Immunology of Peking Union Medical College Hospital, told China Newsweek that most of the patients showed low back pain in the early stage of the disease, and if left unchecked, it would cause the disease to deteriorate to the "bamboo-shaped spine", that is, the phenomenon of spinal fusion in the late stage, and even the basic actions of lowering the head, bending over, and turning around could not be completed
.

The rate of misdiagnosis is high, and it is not uncommon for women to get sick

"Most of the pain of ankylosing spondylitis is manifested in the waist and hips in the early stage, resulting in gradual restriction of lumbar mobility; It extends posteriorly to the thoracic spine, affecting the patient's normal breathing; As the course of the disease progresses, bone damage progresses to a certain extent, and the pain further climbs to the cervical spine and head, resulting in the inability of the entire head and neck to move; Eventually, a healthy and flexible spine will stiffen like bamboo, and over time the physical activity function will be lost, and gradually deformed and even disabled
.
Weng Xisheng, a professor of orthopedics at Peking Union Medical College Hospital, said that this is what everyone often says that the body is gradually "petrified"
.

Zeng Xiaofeng said that in the late stage of the patient, the entire spine is highly hardened, and the body cannot be twisted flexibly, which is easy to cause fractures
.
This is the worst-case scenario and cannot be recovered from
.
Imaging can also see similar "bamboo-like" changes, that is, the entire cervical, thoracic, lumbar and other parts grow together, which used to be called "bamboo pole disease"
.
"If effective intervention is carried out after the onset of the disease, it will basically not develop to such an extreme situation
.
" He said
.

Cai Qing, chief physician of the Department of Rheumatology and Immunology of Changhai Hospital affiliated to Shanghai Second Military Medical University, co-edited the book "100 Questions about Ankylosing Spondylitis", which pointed out that epidemiological studies reported that about 50% of patients with mandatory spondylitis had inflammatory back pain as the first symptom
.
Low back pain is extremely common in the population, in addition to related to inflammatory symptoms, including mechanical low back pain, that is, the symptoms of lumbar disc herniation, 20%~30% of patients will have both kinds of low back pain
.

People usually have the impression that most patients with ankylosing spondylitis are men, in fact, female groups also have the disease, and it is not uncommon
.
"Now the ratio of male to female incidence is about 2~4:1
.
Overall, female patients have slightly milder symptoms than male patients, the disease is less destructive to women's joints, and fewer
patients eventually develop rigidity.
Zeng Xiaofeng said
.

Zhang Ge, director of the rheumatology and immunology department of Beijing Haidian Hospital, told China Newsweek that the number of female critically ill patients is lower than that of male patients, and the incidence of knee, elbow, wrist and other parts of the former is relatively higher
.

"In the past, it was thought that ankylosing spondylitis in women was rare, on the one hand, doctors did not pay enough attention to and understand female patients, did not carefully inquire or found relevant signs and symptoms, on the other hand, the clinical manifestations of female patients were different from those of men and ignored
.
" Cai Qing once wrote
.
In Cai Qing's view, many clinical features of ankylosing spondylitis in women are the same as those in men, but they are also somewhat different
.
The most common symptoms in men are lower back pain and spinal pain, and the onset symptoms in women are more peripheral arthritis, acute iridocyclitis, and some neck discomfort
at the onset.

The cause of ankylosing spondylitis is not clear, and the mainstream view in the industry is that the disease is related
to genetics, environmental factors, autoimmunity, pathogenic bacterial infection and other factors.
At present, there are many hypotheses for this disease, among which the "human leukocyte antigen B27 (HLAB27) genetic hypothesis" is more representative
.
Specifically, antigens in vivo bind to leukocyte antigen B27 to form a complex and are attacked by autoimmune cells, triggering a series of immune reactions leading to disease
.
Other studies have also shown that genes such as ERAP1 are also involved in the pathogenesis process
.

Ankylosing spondylitis is more likely to target young people, and the age of onset is usually 15~40 years old
.
"The misdiagnosis rate of this disease is relatively high, most people have a hidden onset, and it is easy to be mistaken for common diseases
such as lumbar strain, lumbar disc herniation, and air conditioning disease.
" Zhang Ge said that many young people encounter low back pain in daily life, and the symptoms will be significantly reduced after activity, so they think that it is only a temporary minor problem, and it will pass after a while
.
Even if they go to the hospital, many patients usually choose the pain department or massage department at first, and will not think of going to the rheumatology and immunology department, which will also cause misdiagnosis and delay
.

Zhang Ge said that ankylosing spondylitis will affect the eyes, toes, skin and other parts, when patients have symptoms in these parts, they generally go to ophthalmology, dermatology and other departments for examination, and will not say all these symptoms
.
It is also difficult for doctors trained in rheumatology and immunology to associate these symptoms, and most patients are treated
on a case-by-case basis.
Zeng Xiaofeng said that his team's previous research data showed that through the data of epidemiological investigations, it was predicted that the average delay time of ankylosing spondylitis in China from the first appearance of symptoms to the diagnosis was 6 years
.

Source: Australian Musculoskeletal Society

In patients with ankylosing spondylitis, the positive rate of HLAB27 can reach about 90%, but healthy people also have a positive rate
of about 5%.
"That is, not everyone who has this genetic factor will develop the disease
.
" Zeng Xiaofeng said
.

Zhang Ge analyzed that ankylosing spondylitis needs to be classified and diagnosed, that is, there must be clear sacroiliitis on imaging, and it is also necessary to check the symptoms of spondyloarthritis, such as arthritis, uveitis, toeitis, etc
.

Zeng Xiaofeng explained that enthesitis is a pathological feature
of ankylosing spondylitis.
The early symptoms of the onset are low back pain, patients often describe the back weight increase, heaviness, tightness, pain symptoms after the evening rest aggravated, after work or morning activity body stiffness, pain but reduced, "this is the main point to distinguish ankylosing spondylitis from other diseases"
.

The misdiagnosis of ankylosing spondylitis is related to
the shortage of talents in rheumatology and immunology.
Zeng Xiaofeng said that there are only more than 7,000 rheumatologists in China, and many tertiary hospitals do not have special rheumatology and immunology departments
.
On October 31, 2019, the National Health Commission issued the Guidelines for the Construction and Management of Rheumatology and Immunology in General Hospitals (Trial), which proposed that qualified tertiary general hospitals should, in principle, establish independent rheumatology and immunology departments, and qualified secondary general hospitals and other types of medical institutions should establish independent rheumatology and immunology departments
.
The "Basic Standard Guidelines for Rheumatology and Immunology in General Hospitals (Trial)" issued on the same day proposed that hospitals should also have independent laboratory departments, radiology, ophthalmology and other departments to support routine examination
of rheumatic immune diseases.

How to treat "immortal cancer"?

"This disease has been a medicine shortage
for a long time.
" Zeng Xiaofeng said
.
In the past, the clinical treatment of ankylosing spondylitis mainly relied on non-steroidal anti-inflammatory drugs and anti-rheumatic drugs
.
In recent years, new drugs have changed the treatment landscape
for ankylosing spondylitis.

Zeng Xiaofeng said that in the past 10 years, the drug treatment of ankylosing spondylitis has progressed rapidly
.
According to different targets, there are two main types of biological agents for therapeutic indications in China, one is anti-tumor necrosis factor antagonists, and the second is monoclonal antibody drugs
.

"The onset of the disease is accompanied by multiple inflammatory responses, and existing therapies are targeted therapies against these inflammatory mediators
.
" Zeng Xiaofeng said that when the disease is in the early stage and joint destruction has not yet occurred, the anti-inflammatory and analgesic effect of non-steroidal anti-inflammatory drugs is very important
.
When such drugs cannot control the progression of inflammation, biologics and small molecule targeted therapies can come in handy to relieve inflammation while also controlling inflammation and inhibiting pathological new bone formation
.

"The earlier the diagnosis, the earlier the treatment, the better
the recovery.
" In Zeng Xiaofeng's view, even if these drugs can alleviate symptoms and disease progression, the current forced spondylitis is still incurable, and the damage caused cannot be reversed
.
For example, he said, some patients who have advanced stages always have their heads raised to the sky and cannot lower their heads
.
There are also patients whose gaze is always only on the ground
.
After the surgical correction, from the physical point of view, only from the hunchback to the body straightened, the sight can look at the front normally, but the movements such as turning and bending are still limited
.

According to reports, on December 13, 2019, Shenzhen University General Hospital announced the successful treatment of the first case of "3-on folding human" ankylosing spondylitis in
China.
The degree of deformity is extremely rare, the lower jaw is close to the sternum, the sternum is close to the pubic bone, the face is close to the femur, and the whole person is as if it is folded
.
From June to December 2019, after half a year, Shenzhen University General Hospital carried out a total of 4 operations for this patient, cutting the patient's bones many times, reconnecting and correcting them, and finally successfully straightening the spine
.

"Generally, ankylosing spondylitis develops to the point where there is obvious inflammation or fusion of the joints, or spinal deformities, and orthopedic surgery
is required.
Because there are many drug treatment methods now, in recent years, there have been fewer and fewer
tonic patients undergoing surgery in hospitals.
Li Zijian, chief physician of the Department of Joint Surgery of Peking University Third Hospital
, told China Newsweek.

"One to two hundred patients with ankylosing spondylitis come to the hospital every month
.
The proportion of particularly mild and severe patients is small, and most of the patients are moderately developed, but have had a certain impact
on their daily life.
Zhang Ge said that basically after diagnosis and entering the treatment stage, the patient's symptoms can achieve complete clinical relief, that is, the pain basically disappears, and bone damage such as bone hardening and deformation does not continue to develop
.

According to the Practice Guidelines for Patients with Ankylosing Spondylitis/Spondyloarthritis published by the Chinese Journal of Internal Medicine in 2020, taking NSAIDs may cause potential cardiovascular risks, leading to arrhythmias, increased blood pressure, etc
.
Patients taking it for a long time should be regularly assessed for cardiovascular disease risk
.
Because it is a chronic disease, most patients still need to take long-term medication after diagnosis and go to the hospital for follow-up regularly
.
"Many patients who need to inject biologics need three months to check blood routines, liver and kidney function and other indicators
.
" Zhang Ge said
.

"The consensus on targeted treatment of spinal and joint diseases written by the National Clinical Research Center for Skin and Immune Diseases will be launched
soon.
" Zeng Xiaofeng said that in recent years, the treatment of spinal and joint diseases has made subversive progress, but many grassroots doctors lack experience and cannot accurately use medicine
.
Accelerating this consensus will help rheumatologists and orthopedic surgeons across the country to accurately carry out clinical diagnosis and treatment
.