Ankylosing spondylitis is a chronic progressive spondyloarthropathy that mainly affects the medial bones and is characterized by ossification of the ligamentous attachments of the spine, eventually leading to ankylosis, stiffness and deformity of the spine, which used to be confused with rheumatoid joints. Patients rheumatoid factor 95% negative, so also known as seronegative spondyloarthropathy, the onset of 15-30 years old, more men than women, male: female = 14:1.
Pathogenesis: The etiology is unknown and may be related to genetics, infection, and injury.
I. Medical history and symptoms
(I) Onset of disease
The onset of the disease is usually insidious. In the early stages, there may be symptoms such as anorexia, low-grade fever, weakness, wasting and anemia, but they are generally not severe except in children. Some symptoms of ankylosing spondylitis can occur after trauma, exertion, shock, and infection and are noteworthy.
In addition, there is significant family aggregation of the disease. The risk of first-degree relatives of patients with this disease has been reported in the West to be 20…-40 times higher than the general population, and a 35% prevalence in first-degree relatives has also been reported. In a group of HLA-B27-positive patients with ankylosing spondylitis, the authors found that the prevalence of first-degree relatives was 24%, and that ankylosing spondylitis segregated with B27.
(II) First symptoms
1. Low back pain
Low back pain or discomfort is the most common symptom of the disease. The incidence is about 90%. The occurrence is insidious, often vague and difficult to locate. At the beginning, the patient often feels pain or discomfort in the deep hip area, and the pain is often located in the sacroiliac joint in severe cases, and sometimes it can be radiated to the iliac crest or the back of the thigh. The pain may be aggravated by coughing, sneezing or other movements that involve the lower back. Low back pain may start unilaterally or intermittently and progress to bilateral and persistent with stiffness. Some patients have low back pain and stiffness at the beginning. The pain at night may affect sleep, and in severe cases, the patient may wake up in the middle of sleep with pain, and even have to get out of bed and move around before he can fall back to sleep, which is one of the indications of disease activity. When the back pain is severe, the patient may find it difficult to get out of bed and needs to move to the side of the bed first to avoid the pain being aggravated by bending and rotating the back.
This is one of the key points to differentiate inflammatory low back pain from mechanical low back pain, which cannot be relieved by rest but can be improved by activity.
2. Morning stiffness
It is also one of the common early symptoms of ankylosing spondylitis. The patient wakes up early and feels stiffness in the lower back, which can be relieved after activity. Morning stiffness is also one of the indicators of disease activity, and can last all day in severe cases. In addition to activity, hot compresses and hot baths can also relieve morning stiffness.
3.Tendon attachment point lesion
Inflammation of the tendon/ligament bone attachment point is a characteristic pathological change of ankylosing spondylitis and has received increasing attention in recent years.
Patients may present with chest pain that worsens with coughing or sneezing, sometimes misdiagnosed as “pleurisy”, or they may see a cardiovascular surgeon or thoracic surgeon because the chest pain resembles “pericarditis” or “atypical angina”. The chest pain may be similar to “pericarditis” or “atypical angina” and the patient may visit a cardiovascular doctor or a chest physician. Due to the limitation of thoracic expansion during inspiration, early cases may also have mild to moderate reduction in the outline mobility of the Qu. However, because of compensatory abdominal breathing, ventilation is rarely impaired and responds well to NSAIDs. Cervical stiffness and pain usually occur several years after the onset of the disease, but a few cases may also show such symptoms in the early stage.
4. Peripheral joint symptoms
In more than half of the cases, peripheral joint symptoms appear during the course of the disease. It is generally believed that about 20% of the cases have peripheral joint involvement as the first symptom. The peripheral joints are mostly involved in the large joints of the lower extremities, such as hip, knee and manic, but also in the large joints of the upper extremities, such as shoulder and wrist, while the involvement of small joints at the end, such as fingers and toes, is less common. Among the 295 cases of ankylosing spondylitis diagnosed at Shantou University School of Medicine, the frequency of peripheral joint involvement as the first symptom was hip, knee, manic, toe, upper extremity, and heel, with incidence rates of 18.5%, 15.5%, 7.8%, 2.6%, 2.2%, and 1.3%, respectively.
The peripheral joint involvement in ankylosing spondylitis is less likely to be persistent and destructive, one of the features that distinguishes it from rheumatoid arthritis.
Calin et al. found that the younger the age of onset, the higher the incidence of hip involvement and the poorer the prognosis for the 1,500 patients of the British Ankylosing Spondylitis Society. As the age of onset increases, the incidence of hip involvement decreases, as does the severity. The incidence of hip involvement is reported to be 17–36% in foreign countries and up to about 60% in China, but it is not as severe as in Westerners. The incidence of other peripheral joint involvement is also high in the Chinese.
(As the disease progresses, the entire spine may become straightened from the bottom up: first, the anterior convex curve of the lumbar spine disappears, and then the thoracic spine becomes posteriorly convex and has a hunchback deformity; with the involvement of the cervical spine, the movement of the cervical spine is limited, and the patient’s posture changes to a forward-facing head, a flattened thorax, a protruding abdomen, and breathing by diaphragm movement. The patient can only see a limited section of the road in front of him when walking. At this stage, pain and morning stiffness are not obvious, and there is still pain only in certain areas where there is still inflammatory activity. However, due to the ankylosis of the entire spine, the patient is prone to trauma because of the difficulty in self-balancing when changing posture, and trauma is likely to be the cause of the sudden increase in pain during this stage.
Fortunately, complete ankylosis is a minority of cases, and about 80% of patients are able to perform general work and take care of themselves, and the lesion is limited to part of the spine, or even to the sacroiliac joint for life.
(IV) Extra-articular manifestations
As a systemic chronic inflammatory disease, in addition to involving the spine, peripheral joints and tendon/ligament attachments, ankylosing spondylitis can also involve other organs.
1. Systemic symptoms
Most often seen in the early stages, except for Chen, which is usually not serious. The main symptoms are weakness, weight loss, anemia, and acute temporal reactions such as elevated blood sedimentation and c-reactive protein. Generally speaking, systemic symptoms are milder in those with predominantly medial joint symptoms.
In contrast, those with more severe peripheral joint involvement have more prominent systemic symptoms.
2. Acute anterior uveitis or iritis
Some people believe that this disease is part of ankylosing spondylitis, while others believe that it is a separate disease related to ankylosing spondylitis and HLA-B27. The incidence of iritis in ankylosing spondylitis is 4-33%, with some cases of iritis preceding the onset of ankylosing spondylitis. The clinical presentation is an acute attack, often unilateral in onset. Symptoms such as pain, lacrimation, and photophobia are present. Physical examination reveals pericorneal congestion and iris edema. If the iris is adherent, the pupil is seen to be constricted with irregular margins. Slit lamp last examination reveals massive exudate and corneal deposits in the anterior chamber. Each episode lasts about 4-8 weeks, usually without sequelae, but often recurring.
3.Cardiovascular manifestations
Cardiovascular involvement is rare though. However, it is also an important category of manifestations of ankylosing spondylitis. Clinically, this includes episodic aortitis, subaortic fibrosis, aortic valve closure insufficiency, mitral valve prolapse and mitral valve closure insufficiency, cardiac enlargement, atrioventricular block and bundle branch block, dilated cardiomyopathy and pericarditis. It has been reported that cardiovascular involvement in ankylosing spondylitis is closely related to HLA-B27 and is much more frequent in men than in women, with a higher incidence in Europe and the United States than in China and Japan.
The incidence of cardiac conduction system disorders in ankylosing spondylitis also increases with the course of the disease. According to some statistics, 2.7% of patients with 15 years of disease and 8.5% of patients with 30 years of disease can develop conduction system dysfunction, and its incidence is also high in peripheral joint involvement, about two times the general case. In some cases, complete atrioventricular block may occur and As syndrome may develop. It is thought that ankylosing spondylitis may be an important cause of cardiac conduction system dysfunction, and its real occurrence string may be much higher than previous statistics, which is worth noting.
4. Pulmonary manifestations
A common extra-articular manifestation of the disease in its later stages, usually occurring in those with a disease duration of 20 years or more. There may be no obvious clinical symptoms, but there may be cough, sputum, shortness of breath and even hemoptysis. With the development of the disease, the thoracic movement is limited and both upper lungs, especially the apical lung, may become fibrotic, cystic, or even cavernous, further impairing lung function. The disease is often complicated by opportunistic infections in the late stages.
In the later stages of ankylosing spondylitis, the thoracic contour is stiff and the posterior sternum is painful due to ossification of the spinal and sternoclavicular joints. Chest radiographs show narrowing and fusion of the sternoclavicular joint, fusion of the ribs and vertebrae and transverse processes, and reduced or even absent rib elevation in the sighing phase. At this time, the patient needs to be compensated by diaphragm for breathing, but abdominal breathing increases the abdominal pressure, and some patients may develop inguinal hernia. Therefore, early diagnosis, early treatment and medical sports are really important to improve the prognosis.
5. Nerve and muscle manifestations
In the past, not enough attention has been paid to the neurological and muscular manifestations of ankylosing spondylitis, and it is generally believed that many late secondary symptoms such as vertebral fractures and subluxations are caused.
After the ankylosis of the spine, the disease is usually complicated by severe osteoporosis, so it is very easy to fracture, that even minor trauma can cause. Vertebral fractures are most likely to occur in the cervical spine, especially in the 5 and 7 cervical spine, and are the most common complication with the highest mortality rate. Patients with neck and back pain or numbness of the limbs after trauma should be excluded from the possibility of spinal fracture.
6, kidney damage
Renal damage in ankylosing spondylitis is less common, mainly lgA nephropathy and renal amyloidosis. It is thought that IgA nephropathy is related to inflammatory bowel disease. And amyloidosis is usually secondary.
7, prostatitis
This disease is reported to be more common in chronic prostatitis than in the normal population.
The diagnosis: early diagnosis is difficult, lower back, hip pain, spinal activity is limited as the main feature, followed by thoracic vertebral kyphosis, chest expansion narrowing ≤ 2.5cm. x-ray examination is important, bilateral sacroiliac joints experience widening → erosion → sclerosis and ankylosis changes, the spine is bamboo-like ossification changes, laboratory tests patients mostly have mild anemia, it sinks fast, rheumatoid factor negative, HLA-B27 positive.
Auxiliary examination: X-ray examination, early lesions involving the sacroiliac joint, blurred joint contour, hairy glass-like bone density increase band under the articular cartilage, followed by joint fracture hyperplasia, joint gap narrowing, disappearance, occurrence of bony ankylosis, early spine with osteoporosis, then small joint lumen blurring, narrowing, ligament calcification, intervertebral disc fibrous ossification, intervertebral body bone bridge formation, extensive ligament calcification, the spine is The spine has a bamboo-like spine, with mild anemia, mildly elevated leukocytes, increased erythrocyte descent rate in 90% of patients, 95% negative rheumatoid factor, and 90% positive HLA-B27 in laboratory tests.
Ankylosing spondylitis in children
Ankylosing spondylitis that develops before the age of 14 is called ankylosing spondylitis in children. Ankylosing spondylitis in children is actually not uncommon, with Western reports accounting for about 10% of ankylosing spondylitis and 15 – 20% of chronic arthritis in children. The PLA General Hospital in China reported that of 24 cases originally diagnosed as rheumatoid arthritis oligoarticular and polyarticular in children, 19 were consistent with ankylosing spondylitis in children. In the three years from 1990 to 1992, 288 cases of ankylosing spondylitis were admitted to the Cantu University School of Medicine, and 36 cases of childhood type, accounting for 12.5%. The current clinical diagnosis of ankylosing spondylitis in children is based on retrospective studies, because most patients present with typical ankylosing spondylitis manifestations years after the onset of the disease.
(I) Clinical features
1. Morbidity
The disease is most common in older children, more males than females, with a male-to-female ratio of about 7.1. Of the 36 cases diagnosed at Shantou University School of Medicine, 33 were male and 3 were female, male:female = 11:1; the age of onset was 7-16 years, with an average of 13.4 years. According to statistics, only 12–24X had symptoms such as low back pain, morning stiffness, activity limitation or x-ray sacroiliac arthritis in the first year of onset. On the contrary, 80–90% of cases had peripheral arthritis or tendon attachment point lesions as the main symptoms, and generally the lower limbs were the main ones. The number of joints involved is usually less than 10, and oligoarticular joints are more common than polyarticular joints, often unilateral or asymmetric. Some patients may have persistent high or low fever, weight loss, muscle weakness, anemia, elevated leukocytes or hypogammaglobulinemia.
(1) Seronegative tendon attachment point lesions and arthritis syndrome: seronegative tendon attachment point lesions and arthritis syndrome (seronegativeenthesopathyandarthritissymdrome. SEA syndrome), refers to tendon attachment point lesions, as well as oligoarthrosis or arthritis that occurred before the age of 17, while rheumatoid factor, anti-nuclear antibody negative. SEA syndrome is a group of diseases that occur before the age of 17 years, as well as oligoarthropathy or arthritis, and rheumatoid factor, anti-nuclear antibody negative. These diseases differ from childhood rheumatoid arthritis in terms of age of onset, gender distribution, family history, and serum rheumatoid factor, antinuclear antibodies, and HLA-B27. The clinical manifestations are similar to those of childhood ankylosing spondylitis, and are generally considered to be early or mild manifestations of ankylosing spondylitis.
(2) HLA-B27-positive chronic arthritis in children: multi-series chronic arthritis in children with long-term (more than 10 years) follow-up results, more than 50% of HLA-B27-positive cases develop ankylosing spondylitis; while HLA-B27-negative individuals do not develop ankylosing spondylitis or x-ray sacroiliac arthritis. It is suggested that HLA-B27 is an important clue for the diagnosis of ankylosing spondylitis in children.
(3) Diagnosis of ankylosing spondylitis in children: mainly based on medical history. The x-ray examination is not usually the main basis.
This is the first time I’ve seen a woman with ankylosing spondylitis.
The first time I saw a woman, I was a little bit of a jerk. After the association with HLA-B27, a male-to-female ratio of 1:1 was reported, but subsequent studies continued to demonstrate a male prevalence. The authors found that in 288 cases, the male to female ratio was approximately 4:1. There is no satisfactory explanation for the difference in the prevalence of ankylosing spondylitis between men and women. The effect of occupation and pregnancy on the disease is not significant, and the role of sex hormones is not certain. The average age of onset for women is 26.8 years, 6 years later than for men at 20.8 years; the authors’ analysis of 285 cases showed that the average age of onset for women was 25.4 years, 3 years later than for men at 22.3 years.
Another characteristic of ankylosing spondylitis in women is the higher incidence of peripheral joint, especially knee joint involvement than in men, with Western reports of 50% and 25%, respectively, and a slightly higher incidence of peripheral joint involvement in women in the analysis of 200 cases at Peking Union Medical College Hospital in China, 57% compared to 48%, which is probably similar to the cases at Shantou University Medical College. In addition, the involvement of the pubic symphysis is more common in women than in men.
In terms of severity of disease, women are generally considered to have milder disease and less involvement of the entire spine. In the authors’ analysis of 201 cases, 37.2%, 4L 6%, and 2l.2% of the male group had grade I, -, and N IS iliac arthritis, while 54.3%, 34.3%, and 11.4% of the female group had grade I, -, and N IS iliac arthritis; 36.1% of the males had intervertebral bridge formation/bamboo-like changes in the spine, while only 8.6% of the female group had grade I, -, and N IS iliac arthritis.
The difference between ankylosing spondylitis in women and men is not only helpful for clinical diagnosis and differential diagnosis, but also a useful clue for in-depth study of the disease.
B27-negative ankylosing spondylitis
Although HLA-B27-negative and HLA-B27-positive ankylosing spondylitis share common clinical features, there are many differences: I. B27-negative patients generally have an older age of onset, and acute iritis is less common than in B27-positive patients, but is more common with psoriasis, ulcerative colitis, and clonorchiasis. On the other hand, B27-negative patients generally have a milder disease and rarely have familial aggregation. The difference in clinical manifestations between the two may be related to genetic qualities.
Prevention: Because the cause is unknown, it is not easy to prevent, and once the disease develops, it should be treated as early as possible to slow down the progression of the disease.
The main objectives of early and mid-term treatment are to
1. control inflammation and relieve symptoms
2.Prevent the stiffness of the spine and hip joint or maintain the best functional position
3, to avoid side effects caused by treatment
Outline of conservative treatment.
1, non-steroidal anti-inflammatory drugs: preferred, but emphasize individualized treatment. Symptoms improve after short-term administration, and if stopped too soon, it is not conducive to achieve anti-inflammatory effect and also easy to cause recurrence of symptoms
2.Sulfasalazine (SSZ): It is suitable for improving the synovitis of peripheral joints, but there is no conclusion on the role of spinal joint lesions. Currently, it is commonly used in China.
3.Methotrexate (MTX): It may be effective for patients in whom NSAIDs and salazosulfapyrimidine are ineffective.
Generally, surgery is not required. When hip dysfunction and spinal ankylosis affect movement, artificial hip replacement and spinal osteotomy are performed.