What is Ankylosing Spondylitis (Behçet’s Disease)?

Ankylosing spondylitis (AS) is a systemic inflammatory disease that primarily affects the spine. The pathological process in the spine gradually leads to the fusion of vertebrae (ankylosis), resulting in limited mobility due to the formation of bony bridges between vertebrae. This is the origin of the disease’s name. At the same time, the ligaments surrounding the spine may ossify, causing the spine to lose flexibility and sometimes become solid bone.

There are four forms of Behçet’s disease:

Central form
This form affects only the spine. It develops slowly, often unnoticed by the patient. Pain initially appears in the sacral area and then ascends along the spine. Pain worsens with movement and exertion, often with night pain. The patient’s posture gradually changes: the cervical curve increases with forward protrusion, the thoracic curve protrudes backward, and the head tilts forward with the chin approaching the sternum. The thoracic spine is curved, and chest movements during breathing are limited. In advanced stages, spinal mobility is very restricted. Patients may experience shortness of breath, muscle spasms, and high blood pressure. All of these are accompanied by pain in all segments of the spine.

Rhizomelic form
This form combines spinal involvement with inflammation of large joints, especially shoulders and hips. The disease develops gradually. Pain location depends on the affected joint and may appear in the buttocks, thigh, or hip, radiating to the groin and knee, or in the shoulder, forearm, and arm.

Peripheral form
In this form, initial signs appear in the sacroiliac joints. After several months or years, inflammation develops in the knees and ankles, often accompanied by deforming arthrosis and nearby muscle contractures. This form is more common in adolescents.

Scandinavian form
Similar to the peripheral form, but smaller joints, such as those of the hands and feet, are also affected. Joint pain is mild.

Behçet’s disease mainly affects men aged 15–30. The exact causes are not fully understood, but most specialists consider it autoimmune. There is also a genetic predisposition, with 90–95% of patients carrying the HLA-B27 antigen.

Triggering factors can include:

  • Hypothermia
  • Spine or pelvic injuries
  • Infections
  • Allergic diseases
  • Hormonal disorders
  • Inflammatory diseases of the gastrointestinal tract
  • Inflammatory diseases of the genitourinary system

In Behçet’s disease, immune cells attack intervertebral discs, tendons, and joint ligaments. Inflammation develops in affected tissues, and elastic tissue is replaced by hard bone tissue, restricting spinal and joint mobility.

Diagnosis
Initial symptoms are similar to those of osteochondrosis, a degenerative condition of the intervertebral discs. Pain is similar, but Behçet’s disease has distinctive features:

  • Morning stiffness that disappears during the day
  • Rapid pain relief with nonsteroidal anti-inflammatory drugs (NSAIDs)

Diagnostic confirmation uses imaging studies, such as MRI and CT of the spine. Laboratory tests can identify genetic predisposition, marked by the HLA-B27 antigen, which indicates a very high likelihood of developing the disease (about 90% of cases).

Treatment
Currently, there is no cure for Behçet’s disease. Therapy aims to maintain minimal disease activity and preserve satisfactory joint and spinal function. It is a challenging task, but achievable with patience and consistency.

  • Continuous monitoring by a rheumatologist is necessary.
  • During disease activity, basic treatment (sulfasalazine or delagil/plaquenil) is prescribed under medical supervision.
  • Anti-inflammatory drugs, such as metindol (indomethacin), are used to reduce inflammation.
  • Daily therapeutic exercises (at least 1 hour) are very important to prevent spinal deformities, guided by a physiotherapy specialist. Exercises should include bending and rotation of the spine in various planes to increase range of motion daily.
  • Swimming is highly recommended.
  • Therapeutic spinal massage with traction elements is advised 2–3 times per year.
  • Manual therapy on the cervical spine is contraindicated due to the risk of subluxation of the second cervical vertebra.
  • Physiotherapy is recommended twice a year: laser therapy, magnetotherapy, and Dimexide electrophoresis on the spine and affected joints.
  • Once a year, if possible, spa treatment in specialized sanatoria with therapeutic baths and mud is recommended.
  • Prompt treatment of chronic infection foci, such as dental caries or nasopharyngeal infections, is essential. Urological consultation is also necessary, as chronic urogenital infections and chronic prostatitis worsen disease progression.

Following these recommendations strictly usually prevents the development of severe skeletal deformities.