Ankylosing spondylitis, also known as Marie-strümpell disease, Von Bechterew disease, rheumatoid spondylitis, deforming spondylitis, and rheumatoid central, is now known as AS. AS is characterized by inflammation and ossification of the spinal joints and ligaments of the lumbar, cervical, and thoracic segments, as well as the sacroiliac joints, with frequent involvement of the hip and inflammation of other peripheral joints. The disease is generally rheumatoid factor-negative, and therefore is considered a seronegative spine disease along with Reiter’s syndrome, psoriatic arthritis, and enteropathic arthritis.
Zhang Ge, Department of Acupuncture, Workshop
Ankylosing spondylitis is actually a very ancient disease, with evidence of ankylosing spondylitis found thousands of years ago from the bones of ancient Egyptians. 2000 years ago, Hippocrates, a famous Greek physician, described a disease in which the affected person had pain in the sacrum, spine, and cervical spine.
AS is common in young people between 16 and 30 years of age, and is more common in men, with a rare first onset after age 40, accounting for about 3.3% of cases. The disease starts insidiously, progresses slowly, and has mild systemic symptoms. In the early stage, there is often lower back pain and morning stiffness, which decreases after activity, and can be accompanied by low fever, fatigue, loss of appetite, wasting and other symptoms. The pain is intermittent at the beginning and develops into persistent after several months and years. Later, the inflammatory pain disappears, the spine is partially or completely straightened from the bottom up, and a hunchback deformity appears. Peripheral joint invasion is more common in female patients, with slower progression and less severe spinal deformity.
Most of the patients with AS have arthropathy, and most of them first invade the sacroiliac joint and later progress upward to the cervical spine. In a small number of patients, the cervical spine or several spinal segments are invaded at the same time, and the surrounding joints may also be invaded. As the disease progresses, the joint pain decreases, while the movement of each spinal segment and joint is restricted and deformed, and in advanced stages the entire spine and lower limbs become strongly bowed and flexed forward.
(1) Sacroiliac arthritis: About 90% of AS patients first present with sacroiliac arthritis. Later, it progresses upward to the cervical spine and manifests as recurrent lumbar pain, lumbosacral stiffness, intermittent or alternating lumbar pain and hip pain on both sides, which may radiate to the thighs, without positive signs and negative extension and leg lift test. However, direct pressure or extension of the sacroiliac joint can cause pain, so it is not like sciatica. Some patients have no symptoms of sacroiliac arthritis and only abnormal changes are found on X-ray. About 3% of the AS cervical spine is involved at the earliest and later progresses down to the lumbosacral region, while 7% of the AS has several spinal segments involved at the same time.
(2) Lumbar spine lesions: When the lumbar spine is involved, most of them show restricted lower back anterior and lumbar activities. The lumbar anterior flexion, buckling base peculiar to the moisture scythe Liao Wei brake (11) cup guide hiring lies hiring stand stirrup about ∪ ration imitation dangerous slow deceitful wake up∥ entertainment
(3) Thoracic spine lesions: when the thoracic spine is involved, it manifests as back pain, anterior and lateral thoracic pain, and most commonly as hunchback deformity. If the cribriform joints, sternal stalk joints, sternoclavicular joints [19, 20] and intercostal cartilage joints are involved, there is a bundle-like chest pain, limited thoracic expansion, and the chest pain increases when inhaling and coughing or sneezing. In severe cases, the thorax remains in the state of exhalation, and the thoracic expansion is reduced by more than 50% compared with normal people, so that it can only be assisted by abdominal breathing. Due to the reduction of thoracic and abdominal cavity capacity, it causes cardiopulmonary and digestive dysfunction.
(4) Cervical spine lesions: A few patients first present with cervical spondylitis, with pain in the cervical spine first, radiating along the neck to the head and arms. The neck muscles start with spasm and later atrophy, and the lesion progresses to cervicothoracic posterior convexity deformity. Head movement is significantly restricted, often fixed in a forward-flexed position, unable to supinate, lateral bend or rotate. Severe cases can only see a small piece of ground in front of their toes, and cannot raise their heads to look flat.
(5) Peripheral arthropathy: About half of the patients with AS have transient acute peripheral arthritis, and about 25% have permanent peripheral joint damage. It usually occurs more often in the large joints and more in the lower extremities than in the upper extremities. Some statistics show that the rate of peripheral joint involvement is 40% for the hip and shoulder, 15,5 for the knee, 10% for the ankle, 5% for the foot and wrist, and very rarely for the hand. In 80 cases of AS, the involvement rate of hip joint was reported (100%); limitation of movement (64%), flexion contracture (38%), muscle atrophy (25%), and joint ankylosis (37%) were the main causes of disability in AS patients; 94% of the hip symptoms appeared within 5 years after the onset of the disease, suggesting that if the hip joint is not involved in the first 5 years of AS, it is unlikely to be involved later.
When the shoulder joint is involved, the pain is more pronounced when the joint is restricted, and activities such as combing the hair and lifting the hand are restricted. When the knee joint is involved, the joint is compensated for bending, making daily life such as walking and sitting more difficult. It rarely affects the elbow, wrist and foot joints, and is even rarer when it affects some of the joints.
In addition, the pubic symphysis may be involved, and the upper pelvic rim, sciatic tuberosity, greater trochanter of the femur, and heel of the foot may have osteoarthritic symptoms, with early manifestations of local soft tissue swelling and pain and later bony enlargement. Peripheral arthritis can generally occur before or after spondylitis, and local symptoms are not easily distinguishable from rheumatoid arthritis, but fewer deformities remain.
The extra-articular manifestations of AS mostly appear after spondylitis, and occasionally extra-articular symptoms occur months or years before skeletal muscle symptoms.
(1) Cardiac lesions: Aortic valve lesions are more common, and according to autopsy, about 25% of AS cases have aortic radiculopathy, and cardiac involvement can be clinically asymptomatic or significant. About 1% of the cases have varying degrees of aortic valve incompetence; about 8% have heart block, which can coexist with aortic valve incompetence and occur alone, or in severe cases, complete atrioventricular block and A.I. syndrome. Angina pectoris may occur when the lesion involves the coronary artery. Rarely, aortic myocardial aneurysm, pericarditis and myocarditis occur. Patients with AS with combined heart disease are generally older, have a longer medical history, more spondylitis and peripheral joint lesions, and more pronounced systemic symptoms. gould [24] et al. examined the cardiac function of 21 patients with AS and found that the cardiac function of AS patients was significantly lower than that of the control group.
(2) Ocular lesions: long-term with the room, 25% of AS patients have conjunctivitis, iritis, uveitis, or uveitis, the latter occasionally complicated by spontaneous anterior chamber hemorrhage. Iriditis is prone to recurrence, and the incidence increases the longer the disease is present, but is not related to the severity of spondylitis, which is common in those with peripheral arthropathy and may occur in a few cases before spondylitis. Ocular disease is often self-limiting, sometimes requiring corticosteroid treatment, and in some cases can lead to glaucoma or blindness without proper treatment.
(3) Ear lesions: Gamilleri et al. reported that chronic otitis media occurred in 1/2 of 42 patients (29%) with AS, which is 4 times more than normal controls, and that there were significantly more extra-articular AS patients with chronic otitis media than AS patients without chronic otitis media.
(4) Pulmonary lesions: A small number of patients with AS may be complicated by speckled irregular fibrotic lesions in the upper lobe of the lungs, manifested by coughing, shortness of breath, or even hemoptysis, and may be accompanied by recurrent pneumonia or pleurisy. x-ray examination shows diffuse fibrosis in the upper lobe of the lungs bilaterally, which may have cyst formation and parenchymal destruction, similar to tuberculosis, and needs to be differentiated.
(5) Neurological lesions: due to spinal ankylosis and osteoporosis, cervical dislocation and spinal fracture can easily occur and cause spinal cord compression; if intervertebral discitis occurs, it can cause severe pain; in the late stage of AS, it can invade the cauda equina and cauda equina syndrome can occur, resulting in neurogenic pain in the lower limbs or buttocks; loss of infection in the sacral nerve distribution area, weakened Achilles tendon reflex and motor dysfunction such as bladder and rectum.
(6) Amyloidosis: a rare complication of AS. Among 35 cases of AS, routine rectal mucosal biopsy has been reported to find deposits of amyloid in 3 cases, mostly without specific clinical manifestations.
Because ankylosing spondylitis is a relatively common disease with a lingering course and a tendency to cause disability, early diagnosis and treatment should be pursued. Young people aged 16-25 years, especially young men, should be particularly alert to the possibility of ankylosing spondylitis if they present with the following symptoms.
Initial symptoms
(1) Low back pain and lumbar stiffness for more than 3 months, which cannot be relieved by rest.
(2) Unilateral or bilateral sciatica without a history of significant trauma or sprain.
(3)Recurrent episodes of knee or ankle joint swelling and pain, joint effusion, without obvious history of trauma or infection.
(4)Recurrent episodes of heel node swelling and pain or heel pain.
(5) Recurrent episodes of iritis.
(6) No cough or other respiratory symptoms, chest pain and girdling sensation without history of trauma, and limited thoracic movement.
(7) Spinal pain, stiffness, and even limited movement function without any obvious history of trauma or sprain.
(8) Bilateral hip and hip pain without obvious history of trauma or strain.
(9) Sudden onset of pain, swelling, and activity dysfunction in the large joints of the spine and extremities.
Ankylosing spondylitis is generally insidious in its onset and can have no clinical symptoms in the early stages. Some patients may show mild systemic symptoms in the early stages, such as weakness, wasting, chronic or intermittent low-grade fever, anorexia, and mild anemia. Due to the mild disease, most patients cannot be detected early, resulting in delayed disease and loss of the best time for treatment.
Etiology and pathology
1.Heredity
Genetic factors play an important role in the development of AS. According to epidemiological surveys, the rate of HLA-B27 positivity in AS patients is as high as 90-96%, while
Ankylosing spondylitis
HLA-B27 positive rate in the general population is only 4% to 9%; the incidence of AS in HLA-B27 positive people is about 10% to 20%, while the incidence in the general population is 1‰ to 2‰, a difference of about 100 times. It has been reported that the risk of AS in a group of relatives is 20 to 40 times higher than that of the general population, and the prevalence of AS in first-degree relatives is 24.2% in a domestic survey, which is 120 times higher than that of the normal population. the chance of AS in relatives of HLA-B27-negative healthy people is much lower than that of relatives of HLA-B27-positive AS patients. All these indicate that HLA-B27 is an important factor in the development of AS.
However, it should be noted that, on the one hand, not all HLA-B27-positive individuals develop spondyloarthropathy, and on the other hand, approximately 5% to 20% of patients with spondyloarthropathy test negative for HLA-B27, suggesting that other factors besides genetics influence the development of AS. Therefore, HLA-B27 is an important genetic factor in the expression of AS, but it is not the only factor affecting the disease.
There are several hypotheses to explain the association of HLA-B27 with spondyloarthropathies.
(i), HLA-B27 acts as a receptor site for an infection factor;
②, HLA-B27 is an immune response gene of the earth change that determines susceptibility to environmental excitation factors;
③, HLA-B27 can cross-react with foreign antigens, thus inducing the development of tolerance to foreign antigens;
(iv) HLA-B27 enhances neutrophil activity [15].
With the help of monoclonal antibodies, cytotoxic lymphocytes, immunoelectrophoresis and restriction fragment length polymorphism, about seven or eight subtypes of HLA-B27 have been identified [1].
There may be genetic differences between healthy individuals positive for HLA-B27 and patients with spondylosis, for example, all HLA-B27 individuals have a constant HLA-B27M1 antigenic determinant cluster and antibodies against this antigenic determinant cluster can cross-react with HLA-B27. Most HLA-B27 molecules also have an M2 antigenic determinant. HLA-B27M2 negative molecules appear to be more strongly associated with AS than other HLA-B27 subtypes, especially in Asians, and HLA-B27M2-positive subtypes may have an increased susceptibility to Reiter syndrome. It has been shown that two antigenic determinants, HLA-B27M1 and M2, and the arthropogenic factors Saurobacter, Shigella, and Narsenia can cross-react. Those with low response seem to show mostly AS, while those with increased response develop reactive arthritis or Reiter’s syndrome.
2.Infection
Ebrimger et al. found that the detection rate of Klebsiella pneumoniae in the stool of AS patients was 79%, compared with <30% in controls; the carriage rate of Klebsiella pneumoniae in the intestinal tract and the IgA antibody titer against the bacterium in serum were higher in the active phase of AS than in controls, and were positively correlated with disease activity. It has been suggested that Klebsiella spp. may cross-react with HLA-B27 during antigenic residues or have a common structure, such as HLA-B27 (host antigen residues 72 to 77) and Klebsiella pneumoniae (residues 188 to 193) which have homologous oxyacid sequences, and whether other Gram-negative bacteria have antibodies that bind to this synthetic peptide sequence, with 29% of HLA-B27-positive AS patients compared to 5% of controls. Mason et al. showed that 83% of male AS patients had prostatitis and some authors found that about 6% had ulcerative colitis in combination with AS; other reports also confirmed that the incidence of ulcerative colitis and restrictive enterocolitis was much higher in AS patients than in the general population, so it was speculated that AS might be related to infection. The infection may be spread from the pelvis to the sacroiliac joints and then to the spine via the spinal venous plexus, but no infectious agent (bacterial or viral) can be found at the site of the lesion.
3. Autoimmunity
It has been found that 60% of AS patients have increased serum complement, most cases have IgA-type rheumatoid factor, significantly increased serum C4 and IgA levels, and circulating immune complexes (CIC) in the serum, but the nature of the antigen has not been determined. The above phenomena suggest that immune mechanisms are involved in the pathogenesis of this disease.
4, chronic enteritis is one of the causes of the pathogenesis. Experts at the Beijing Rheumatism Hospital remind us to pay attention to the treatment of chronic and recurrent diseases. “Like chronic enteritis, recurrent urinary tract infections, psoriasis, etc., in the eyes of some patients are easily not hurt people’s lives, but it is these diseases can cause inflammatory changes in the human sacroiliac joints, leading to the occurrence of ankylosing spondylitis. When there is pain in the body joints, do not rush to use drugs, anti-inflammatory drugs or analgesics and other drugs can temporarily relieve the pain, but the treatment of certain diseases can not play a fundamental role, and sometimes because of the nature of the disease to cover up the subsequent treatment difficulties.
5.Other
Trauma, endocrine, metabolic disorders and metabolic reactions are also suspected to be factors in the development of the disease. In conclusion, the cause of the disease is still unknown and there is no one theory that can fully explain all the manifestations of AS. It is likely that the disease is caused by environmental factors (including infection) based on genetic factors.
The white blood cell count is normal or elevated, the lymphocyte ratio is slightly increased, a few patients have mild anemia (orthocytic hypochromia), the sedimentation may be increased, but there is little correlation with disease activity, while C-reactive protein is more significant. Serum albumin is decreased, alpha1 and gamma globulins are increased, serum immunoglobulins IgG, IgA and IgM may be increased, and serum complement C3 and C4 are often increased. Alkaline phosphatase is elevated in about 50% of patients, and serum creatine phosphokinase is often elevated. Serum rheumatoid factor is negative. Although more than 90% to 95% of patients with AS are positive for LHA-B27, LHA-B27 is not generally relied upon for the diagnosis of AS, and LHA-B27 is not routinely examined. The diagnosis mainly relies on clinical manifestations and radiological evidence.
Related tests
X-ray examination is of great importance for the diagnosis of AS. About 98% to 100% of cases have X-ray changes of the sacroiliac joint at an early stage, which is an important basis for the diagnosis of the disease.
Early X-rays show sacroiliac arthritis, and the lesions usually start in the lower and middle part of the sacroiliac joint and are bilateral. The lesions usually start in the middle and lower part of the sacroiliac joint and are bilateral. They begin to invade the iliac side and then the sacral side. A patchy or lumpy bony side may be evident. Subsequently, the entire joint may be invaded, with jagged edges, subchondral osteosclerosis, osteophytes and narrowing of the joint space. Eventually the joint space disappears and bony ankylosis occurs. Sacroiliac arthritis under X-ray easy-to-use button is classified into 5 grades: grade 0 is normal sacroiliac joint, grade I is suspected sacroiliac joint inflammation on both sides; grade II is blurred sacroiliac joint margins, slight sclerosis and small erosion lesions, and mild narrowing of joint space; grade III is sclerosis on both sides of sacroiliac joint, blurred joint margins, and erosion lesions with disappearance of joint space; grade IV is complete fusion or ankylosis of joint with or without residual Sclerosis.
In the early stages of spinal lesions, there is generalized osteoporosis, blurring of the vertebral tuberosities and vertebral trabeculae (decalcification), destructive erosion of the superior and inferior angles of the vertebrae at the incidental part of the intervertebral disc fibrous ring, the vertebral body is “square vertebrae”, and the normal anterior curvature of the lumbar spine disappears and becomes straight, which may cause compression fractures of one or more vertebral bodies. The lesion progresses to the thoracic and cervical intervertebral joints, calcification of the intervertebral disc space, calcification and ossification of the annulus fibrosus and anterior longitudinal ligaments, and formation of ligamentous redundancies, causing adjacent vertebrae to join together and form intervertebral bridges, resulting in the most characteristic “bamboo-like spine”.
Ankylosing spondylitis [late stage]
The radiographic manifestations of primary AS and spondylitis secondary to inflammatory bowel disease, Reiter syndrome, and psoriatic arthritis are similar, but the latter is asymmetrical ankylosis. Bone and osteochondritis can be seen at the attachment of ligaments, tendons, and bursae, most often in the heel bone, sciatic tuberosity, and iliac crest. Similar radiographic changes can occur in other peripheral joints.
The National Symposium on Ankylosing Spondylitis, sponsored by the Chinese Journal of Internal Medicine and organized by the Department of Rheumatology, Shantou University School of Medicine, was held in Shantou on September 21-22, 2001. The following diagnostic criteria for AS were established at the symposium
2. imaging or pathology
(1) Bilateral X-ray SIJ inflammation ≥ grade III. (2) Bilateral CT SIJ ≥ grade II. (3) If the CT SIJ is less than grade II, MRI examination is feasible. If there is cartilage destruction, paracartilage edema and/or extensive fat deposition, especially if the enhancement intensity of the joint or paracartilage is >20% and the enhancement slope is >10%/min on dynamic enhancement examination. (4) Pathological examination of sacroiliac joint shows inflammation.
5. Surgical treatment
Severe spinal hunchback deformity can be corrected after the condition is stabilized, and vertebral osteotomy can be performed to correct hunchback in the case of lumbar deformity; cervical 7 thoracic 1 osteotomy can correct the severe deformity of cervical spine; Rowed [31] reported 21 cases of AS patients with cervical spine injury due to fall, half of them had good prognosis after conservative treatment; the other half had decompression and internal fixation due to recurrent displacement of cervical spine or worsening of symptoms due to spinal compression of nerves, and also The other half had good results with decompression and internal fixation due to recurrent displacement of the cervical spine or worsening of the spinal nerve compression. For severe hip flexion deformity, total hip arthroplasty or hip arthroplasty is feasible, but the results are not satisfactory and are prone to reanimation after surgery.
In addition, deep X-ray and 224 radium radiation therapy have certain effect on early AS patients to reduce symptoms and improve function, and the recent remission rate can reach 80%-96%, but cannot stop the progress of the disease, and there is a risk of inducing aplastic anemia, leukemia and transverse myelitis, which is not used now. Antimalarials, gold agents, penicillamine and azathioprine are ineffective against AS and are not used now.
For pulmonary lesions mainly symptomatic treatment, active prevention and treatment of secondary infections, cardiac lesions plus aortic valve closure insufficiency serious, feasible aortic valve surgery, for severe conduction block can be installed artificial pacemaker.