Bechterew’s disease (Ankylosing Spondylitis – AS)

Bechterew’s disease

Definition of the Disease and Its Causes:

Bechterew’s disease, also known as Strümpell-Bechterew-Marie disease or ankylosing spondylitis, is characterized by inflammation of the spinal joints with subsequent fusion, resulting in a rigid casing that significantly restricts movement. Gradually, the range of motion decreases, and the spine becomes immobile.

This disease has been known since ancient times, with the first historical mention dating back to 1559 when the Italian surgeon Realdo Colombo described its characteristic changes. Subsequent descriptions by physicians such as Bernard Connor in 1693, Vladimir Bechterew in 1893, Adolf Strümpell in 1897, and Pierre Marie in 1898 furthered our understanding.

Bechterew’s disease usually affects the spine, sacroiliac joints, and large joints in the lower extremities. In some cases, it may manifest as inflammation in the eyes, occurring in 10-50% of cases.

Causes of Bechterew’s Disease:

The causes of Bechterew’s disease remain largely unclear, with attention focused on various factors:

1. Genetics: Genetic predisposition, particularly the HLA-B27 gene, is found in up to 95% of patients.
2. Immune System Alteration: Changes in the immune system could trigger disease development.
3. Injuries: Injuries in the pelvic area or spine may contribute to disease development.
4. Hormonal Disorders and Inflammation: Hormonal disorders and chronic inflammations are presumed factors.
5. Infectious Diseases and Allergies: These are potential additional factors in disease development.

These factors indicate an improper immune system reaction to the musculoskeletal system, classifying Bechterew’s disease as an autoimmune disease. Tumor necrosis factor-alpha (TNF-α) plays a central role in disease development, with its highest concentration found in the hip and sacroiliac joints.

Symptoms of Bechterew’s Disease:

Early Stage Symptoms:

• Stiffness in the spine in the morning, gradually easing, especially after a hot shower.
• Weakness, fatigue, and rapid tiredness, indicating the impact of the disease.
• Eye involvement, persistent eye inflammation.
• Wandering pains in the lower back, not localized.
• Pain in the pelvic area worsened by coughing or active breathing, involving rib and spinal joints.
• Feeling of pressure in the chest, appearing as the disease progresses.
• Reduced range of motion in the head, indicating advancing movement restriction.
• Changes in walking and constant heel pain, indicating the disease’s impact on large foot joints.

Bechterew’s disease can start subtly with inflammation in the hands and feet joints or even symptoms affecting the heart. In some cases, the disease may begin with eye involvement. This disease is also characterized by silent progression, usually diagnosed based on radiographic studies to rule out other conditions.

Advanced Stage Symptoms:

The advanced stage of Bechterew’s disease is characterized by:

• Pain in the back and hips, especially at rest, with increasing movement restriction over time in the lower back.
• Alleviation of joint stiffness symptoms after mild loading or warm baths, worsening after long periods of rest.
• Loss of flexibility in the spine over time, making it difficult for the patient to bend forward.
• Difficulty breathing due to the disease’s impact on rib and spinal joints.
• Eye involvement in over 20% of cases, with inflammation in the iris. Pain and redness may occur in the eye area, but vision is not affected.
• Inflammation that may involve the upper sections of the spine with chest pain.

Late Stage Symptoms:

Progression of Bechterew’s disease is characterized by trunk movement restriction in all directions, and coughing or sneezing may elicit a painful reaction in the spine. As the disease progresses, pain increases when physical loading is restricted, while mild loading reduces pain. Without adequate treatment, the disease may lead to complete restriction of spinal movement, with the person assuming a distinctive position – the “stooped” position (elbows bent, back hunched, head tilted, and knees slightly bent), resulting in complete disability.

Mechanism of Bechterew’s Disease Occurrence:

• The occurrence mechanism of Bechterew’s disease is distinguished by the presence of the HLA B27 antigen, indicating a genetic predisposition to the disease. This antigen renders joint tissues akin to the aggressor. Upon infection penetration into the antigen carrier’s body, a reaction occurs. Currently, the presence of the HLA B27 antigen has been confirmed in almost all patients with the disease. However, not every carrier of this gene necessarily suffers from the disease.

Classification and Stages of Bechterew’s Disease Development:

• Clinical classifications (forms) of Bechterew’s disease include:
  1. Central Form: Involving only the spinal column.
  2. Arch Form: Changes in the cervical and thoracic regions of the spinal column leading to forward body inclination, constituting the “student” posture.
  3. Rigid Form: Flattening of all spinal curves, making the back flat, with the head slightly tilting backward, resembling the “rider” appearance.
  4. Root Form: Involving the spinal column and root joints (shoulders and pelvis).
  5. Peripheral Form: Involving the spinal column and peripheral joints (knees, ankles, etc.).
  6. Scandinavian Form: Involving small hand joints and the spinal column.
  7. Infiltrative Form: Including any of the above forms and involving infiltrative organs (heart, lungs, kidneys).

Diagnostic Signs of Ankylosing Spondylitis According to the Russian Rheumatism Institute Recommendations, 1997:

• Diagnostic signs of ankylosing spondylitis include:
  1. Persistent lower back pain not relieved by rest, decreasing with movement, and lasting for more than three months.
  2. Limitation of spinal column movement at the lumbar level in both dorsal and lateral directions.
  3. Restricted chest expansion (difference between chest circumference during full inhalation and maximum exhalation) compared to normal values for age and gender.
  4. Bilateral inflammation in the hip joint (sacroiliac joint) from Stage II to Stage IV.
• Diagnosis is considered reliable if there is a fourth sign along with any of the first three.

Complications of Bechterew’s Disease:

Serious and severe complications arise from Bechterew’s disease. The most prominent of these complications include:

1. Kidney Enlargement due to Protein and Carbohydrate Oxidation: Leads to the deposition of a specific insoluble protein – amyloid. This protein impairs kidney function and later leads to kidney failure.
2. Lung Inflammation due to Reduced Chest Movement: May occur due to decreased chest movement and can be serious.
3. Uveitis Inflammation in the Eye: Can lead to vision loss.
4. Vascular Damage: Increases the risk of heart attack and stroke.
5. Osteoporosis: Decreased bone strength and structural deterioration.
6. Cauda Equina Syndrome: Compression of the nerve root bundle at the bottom of the spinal cord, leading to loss of control over urination and defecation and paralysis of the legs.
7. Flattening of Spinal Curves (Rider Posture): Change in body posture.
8. Disability: Loss of mobility.

To prevent such complications, the disease must be detected, diagnosed, and treated as soon as possible.

Diagnosis of Bechterew’s Disease:

To diagnose spinal column and peripheral joint conditions, the doctor studies the patient’s complaints, collects medical history, performs a physical examination using specific tests, and requests instrumental examinations and laboratory tests.

• Patient Complaints Study: The doctor pays special attention to head rotation, numbness in the hands, heaviness and fatigue in the back, discomfort, and pain in various sections of the spine, which occur during movements, static loads, and other contexts. The degree and location of pain, its onset time, duration, severity, and the effect of external environment, treatments, and rest are determined.
• Medical History Collection: Identifies factors that triggered the onset of the disease, its duration, its status during periods of remission and exacerbation, main symptoms, and the effectiveness of treatments. It clarifies work and life conditions, physical burden tolerance, nature and degree of physical activity. Family history, sports activities, and the presence of injuries and influential psychological conditions are taken into account.
• Physical Examination: The doctor evaluates the patient’s self-care method, nature of movements, spine shape, position of the upper part of the body, head, hands, and legs. There are natural curvatures of the spine, and deformities cause deviation. Lordotic shape may occur when the spine is bent backward, or a straight (flat) shape – without natural curvatures. Recognition points are used during examination.

Diagnosis by X-ray and Magnetic Resonance Imaging (MRI):

Delay in diagnosing Bechterew’s disease is partly associated with general symptoms of rheumatic diseases. Radiological examination is necessary and is considered one of the most accurate diagnostic methods. The main criterion is changes in the sacroiliac region and disability.

Articular space narrowing with joint cavity widening is particularly important in the early stage of the process. Presence of erosion on joint surfaces is especially significant in the second stage. Partial fusion is important especially in the third stage. Complete fusion is determined in the fourth stage.

Tests for Bechterew’s Disease:

1. Genetic Testing to Confirm the Presence of HLA-B27 Antigen, although this antigen may not appear in about 10% of ankylosing spondylitis patients.
2. Complete Blood Count Analysis, where an elevated erythrocyte sedimentation rate (ESR) typically indicates 50 mm/hour, although increased ESR is common in any inflammatory process.

Bechterew’s disease is diagnosed based on a comprehensive examination that includes patient assessment, complaint analysis, clinic and laboratory diagnosis, and radiological and MRI examination data.

Treatment of Bechterew’s Disease:

• Rheumatological management is ongoing throughout the patient’s life and relies on a balance between pharmacological and non-pharmacological treatments.
• Treatment relies on three main principles: Immunosuppressants (medications that inhibit the immune system) take precedence.
• Hormone therapy serves as a secondary element to alleviate inflammation in the joints.
• The third component includes physical therapy sessions in conjunction with physiotherapy.

Patients with Bechterew’s disease are advised to understand that the primary goal of treatment is to slow disease progression.

Pharmacotherapy:

In the early 21st century, there was a revolution in the treatment of Bechterew’s disease when genetically engineered drugs appeared in rheumatologists’ arsenal. Modern Bechterew’s treatment strategy is based on the principle of “treat to target.” Genetic engineering has achieved tremendous success thanks to high-tech advances, and tumor necrosis factor-alpha (TNF-α) inhibitors became the first series of these drugs. Biologic modifiers of the inflammatory response such as TNF-α inhibitors (Infliximab, Adalimumab) and B-cell activation inhibitor (Rituximab) have been introduced. These drugs act at a molecular level to block the synthesis of inflammatory mediators without suppressing the immune system. Modern (targeted molecular) therapy effectively targets negative inflammatory substances and halts the inflammatory response cascade in Bechterew’s disease. This intervention effectively prevents inflammation progression, disease advancement, and thus preserves spinal and joint mobility.

In most cases, combination therapy (corticosteroids, nonsteroidal anti-inflammatory drugs) and basic drugs (Delagil, Plaquenil, Sulfasalazine) are necessary. Basic disease-modifying antirheumatic drugs (DMARDs) comprise a large and heterogeneous group of medications that combine general symptom relief and joint inflammation reduction, but also modify or alleviate or delay the progression of the disease itself, bone destruction, and other specific effects. Only the doctor determines the doses.

Features of nonsteroidal anti-inflammatory drug use in Bechterew’s disease. Bechterew’s disease is perhaps the only rheumatic condition where long-term nonsteroidal anti-inflammatory drug use is justified and effective, with no alternative, except for TNF-α inhibitor therapy.

Nonsteroidal anti-inflammatory drugs are the first-line medications in Bechterew’s disease treatment. They should be initiated immediately upon diagnosis, regardless of the disease stage. Continuous treatment with these drugs long-term is essential. The continuous use of these drugs is associated with disease development, while their “on-demand” use, i.e., when experiencing pain, has almost no effect on disease progression. When prescribing nonsteroidal anti-inflammatory drugs, the risks of cardiovascular and vascular events, stomach problems, and kidney diseases should be considered.

Physical Exercise:

Physical therapy is essential in cases of Bechterew’s disease, as physical activity maintains joint and spinal mobility. Daily morning fitness exercises should be performed regardless of health status. During sleep, inflammation and stiffness increase. Morning exercises help alleviate stiffness and restore mobility. Short sessions of 2 to 4 times a day during the day – “five-minute breaks” should be implemented. If you are required to work or sit in an uncomfortable position, these sessions should be conducted every hour.

Physical therapy is also necessary in cases of significant spinal mobility restriction with no hope of recovery. Exercise significantly improves lung ventilation, which decreases due to the impact on rib joints, chest joints, and vertebrae. The program includes muscle relaxation exercises and deep breathing to expand chest volume. Sessions should last at least 30 minutes. If the patient’s physical condition allows, Nordic walking and swimming should be practiced. Sleeping on a firm mattress without a pillow is preferred.

Furthermore, lung ventilation can be increased by inflating a ball 3-4 times a day and walking outdoors extensively.

Natural Therapy:

Natural therapies play a special role in treating Bechterew’s disease (ankylosing spondylitis). Natural therapies are mainly applied in the health and resort treatment stage to prolong periods of improvement. Patients with ankylosing spondylitis are recommended to receive various treatments such as cold therapy, heat therapy, and magnet therapy.

Magnetotherapy is a set of alternative medicine techniques involving the use of a static magnetic field or a variable magnetic field. These procedures help alleviate disease pain and improve spinal mobility.

Natural therapies are an additional means of treating Bechterew’s disease, preferably only implemented in hospital or resort therapy conditions. During exacerbations, electrical current is often used to deliver anti-inflammatory drugs (lithium chloride, calcium chloride) to the affected spinal area. Reduction in disease pain and anxiety is observed after the initial sessions. When joint contractures develop and spinal mobility is difficult, clay therapy is used. When pelvic joints are affected, magnetic laser therapy or ultrasound waves with hydrocortisone are used. During exacerbation remission, resort therapies can be implemented in specialized resorts for musculoskeletal system treatment (Pyatigorsk, Sochi, Saki, Matsesta). Positive effects can be obtained through radon baths, aquatic fitness sessions, and underwater massage. Natural therapy does not cancel existing anti-inflammatory treatment as prescribed.

Surgical Treatment:

Surgery is indicated in case of complications: severe spinal deviation, vertebral fractures, spinal canal narrowing, heart and joint impact, especially pelvic joints first.

Lifestyle Recommendations for Bechterew’s Disease:

Diet: In addition to drug therapy, a patient with Bechterew’s disease should maintain an ideal regimen: full sleep in the correct position, emotional rest, regular exercise, and elimination of chronic infection foci. Every patient should undergo annual health and recreational therapy. Natural therapy procedures should be refrained from during exacerbations.

Nutrition: There is no special diet for Bechterew’s disease. A healthy and balanced diet should be consumed without weight gain, as excess weight increases pressure on the spine and leg joints. Some scientists believe in the efficacy of the White Sea diet for rheumatic disease patients, which consists mainly of sea products, fish, fruits, and vegetables, with rare or no consumption of meat products.

Smoking cessation: Chronic bronchitis from smoking reduces lung ventilation, which is already low due to Bechterew’s disease. Inadequate lung ventilation promotes pulmonary infection development.

Article Author:

Dr. Ibrahim Mansour
A general practitioner and orthopedic specialist with 9 years of experience in the emergency department. Specializes in treating infectious diseases and gastrointestinal diseases, with a focus on skeletal aspects using therapeutic, surgical, and conservative methods for injuries, including sports-related ones, and major joint diseases in the extremities. His area of interest includes joint-preserving procedures in the knee and hip joints.

Sources:

• Diseases of Joints: A Guide for Physicians / edited by V.I. Mazurov. — St. Petersburg: SpecLit, 2008. — 397 p.
• Pyle, Kevin. Diagnosis and Treatment in Rheumatology. Problem-Oriented Approach / Kevin Pyle, Lee Kennedy. — Moscow: GEOTAR-Media, 2011. — 368 p.