Ankylosing spondylitis

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Classification according to ICD-10
M45 Ankylosing spondylitis
ICD-10 online (WHO version 2019)

Ankylosing spondylitis (from ancient Greek σπόνδυλος spondylos "fluidized" and ἄγκυλος Ankylos "bent, flexed"; latinisiert to spondylitis ankylosans "bending / stiffening vertebra inflammation"), or ankylosing spondylitis is a chronic inflammatory rheumatic disease with pain and stiffening of joints . It belongs to the group of diseases of the spinal joints ( spondyloarthritis ) and mainly affects the lumbar and thoracic spine and the sacrum and iliac joints . It can also cause inflammation of the iris of the eye and, rarely, other organs .

Synonyms are Bechterew's disease (after Wladimir Michailowitsch Bechterew , 1857–1927) or Bechterew-Strümpell-Marie disease or Strümpell-Bechterew-Pierre Marie disease , ankylosing spondylitis , rheumatoid spondylitis , ankylopoietic spondylitis and spondylarthritis ; the word Morbus is the Latin (medical) name for illness . Bechterew's disease has been a member of the axial spondyloarthritis ( axSpA ) family since 2009 , which also includes the early and less pronounced forms of Bechterew's disease .

Bechterew's kyphosis. The stiffened thorax forces "abdominal breathing" with the diaphragm

Epidemiology

Spondyloarthropathies, of which ankylosing spondylitis is one of the most common representatives, affect around 1.9% of the German population according to a study on blood donors in Berlin (prevalence in Germany). Many of the diseases associated with rather mild symptoms are never diagnosed, so that only a minority of the estimated 1.6 million people with spondyloarthropathies in Germany should know about them. The "German Association of Bechterew's Disease e. V. “speaks of 100,000–150,000 diagnosed cases in Germany. It used to be thought that men were affected three times as often as women. Today we know that both sexes are equally affected. As a result of the usually much milder course in women - at least with regard to the ossification of the spine - ankylosing spondylitis is diagnosed less often in women. In western industrialized countries, the first symptoms usually appear in young adulthood (20-25 years); in five percent of cases, the onset of the disease is after the age of 40.

causes

Although the causes of ankylosing spondylitis are not fully understood , they appear to be due to a disorder of the immune system . Since the symptoms are alleviated after the therapeutic inhibition of the tumor necrosis factor-α (TNF-α), it is reasonable to assume that this plays a central role in the development of the disease. In the inflamed sacrum and iliac joint, T helper cells ( CD4 + T lymphocytes), cytotoxic T cells ( CD8 + T lymphocytes) and phagocytes occur, as well as increased concentrations of TNF-α, although no trigger can be determined for this. One cause, however, could be autoimmune phenomena against the proteoglycan aggrecan, which is present in the cartilage and is partly responsible for its elasticity . Similarities in the antigens of proteoglycans could explain the distribution of the affected areas in the body. Many patients also occur in the blood increased antibody - titers against enterobacteria on, but there is so far no evidence that they play a role in the disease process.

A special feature is the close association of the disease with the presence of HLA-B27 , a histocompatibility antigen subtype of the membrane-bound protein HLA-B ( human leukocyte antigen ) present on almost all body cells . Like the other MHC genes, the HLA-B27 gene is located on the short arm of the sixth chromosome. The HLA-B27 gene product belongs to the class of MHC class I proteins, which bind fragments of intracellular pathogens (so-called antigens) and present them on the cell surface. Its occurrence, which varies depending on the ethnic group, is related to the frequency of the disease. It is now assumed that ankylosing spondylitis is largely genetic, with the HLA-B27 gene being by far the best known marker, but not the only causative genetic cause. The risk of developing ankylosing spondylitis is ninety-fold higher in HLA-B27 carriers than in the general population.

There are studies that indicate that IgA antibodies directed against the bacterium Klebsiella pneumoniae cross-react with components of the HLA-B27 molecule (precisely: B * 27-05 and subtypes derived from it), that is, antibodies , which are formed during a defense reaction against Klebsiella pneumoniae , are not only directed against this bacterium, but also against the body's own structures due to molecular mimicry and can thus trigger an autoimmune reaction, as it also occurs in ankylosing spondylitis.

pathology

The main process in ankylosing spondylitis is the inflammation of the tendon attachments, especially in the pelvis and spine. This is accompanied by edema and damage to the bone marrow , which then ossifies. Inflammation of the sacrum-iliac joint ( sacroiliitis ) is one of the first symptoms. Both the tendon insertion and the joint capsule are affected. Granulation tissue forms below the articular cartilage , with infiltration by lymphocytes and macrophages. The damaged edges of the joints are first replaced by fibrous cartilage , but then ossify, which stiffens the joint. This process leads to the formation of bone braces ( syndesmophytes ) in the spine , which bridge neighboring vertebrae. This leads to the formation of the so-called bamboo spine . Further damage to the spine is osteoporosis , wear and tear of the vertebral bodies at the edges and inflammation with subsequent destruction of the junctions between the intervertebral disc and bone.

Clinical picture

Changes in the cervical spine in ankylosing spondylitis

The first symptoms usually appear in late adolescence or early adulthood. At first they are expressed in dull pain in the lumbar and buttock regions. In addition, there is often stiffness in the morning, which eases with exercise or returns after periods of rest. Within a few months the pain is persistent and mostly bilateral.

About 25 to 35% of patients complain of arthritis in the shoulder, hip and sacrum-iliac joints (sacroiliitis). This usually occurs with restricted mobility and pain. Arthritis in other joints occurs in 30% of patients, often asymmetrically. This leads to painful inflammation of the tendon attachments ( enthesopathy ). The Achilles tendon , the plantar fascia in the sole of the foot and the tendon attachments on the thighbones and pelvis (trochanters, ischium, iliac crest ) are particularly affected . Furthermore, the spine loses mobility due to ossification ("ossification") of the intervertebral areas due to syndesmophytes .

The course of the disease is very variable: it ranges from slight stiffness to complete fusion of the vertebrae with the associated restriction of movement of the upper body, bilateral arthritis of the hip joint, arthritis of the joints in the limbs and manifestations outside the joints. In typically untreated cases, characteristic changes in the patient's posture occur. The lumbar lordosis (forward curvature) of the spine disappears, the glutes atrophy ( atrophy ) and the kyphosis (backward curvature ) of the thoracic spine becomes more pronounced.

A serious complication of the condition is a broken bone in the spine. The porous bones can break even with slight trauma , with the risk of injuring the spinal cord .

A common occurrence of ankylosing spondylitis outside the joints is acute anterior uveitis (inflammation of the middle skin of the eye). It usually only occurs on one side and is accompanied by photophobia and increased tear production. Concomitant symptoms are cataracts and glaucoma . The majority of patients also experience inflammation of the colon and ileum . These are usually asymptomatic. In five to ten percent of cases, however, they progress to inflammatory bowel disease . Less common side effects are damage to the lungs , aortic regurgitation and other functional disorders in the heart area .

Diagnosis

According to information from the German Association of Bechterew's Disease (DVMB), the time to diagnosis is on average five to seven years, but in some cases up to 15 years. Early diagnosis is important to avoid permanent deformation of the musculoskeletal system.

Ankylosing spondylitis is a member of the axial spondyloarthritis family of diseases . Axial spondyloarthritis can be divided into two classes:

  1. Radiological axial spondyloarthritis : synonymous with ankylosing spondylitis
  2. Non-radiological axial spondyloarthritis : This term includes both less severe forms of ankylosing spondylitis and early stages of ankylosing spondylitis.

While ankylosing spondylitis can be diagnosed by describing the radiological changes in the sacroiliac joints and spine , there are currently no direct tests (blood or imaging ) to unambiguously identify the early or less pronounced forms of ankylosing spondylitis ( non-radiological axial spondyloarthritis ) to diagnose. The diagnosis of non-radiological axial spondyloarthritis is therefore more difficult and is based on the diagnostic criteria of ASAS (Assessment of SpondyloArthritis International Society):

These criteria are:

  • Chronic inflammatory back pain: One speaks of chronic back pain if at least four of the following five parameters are present: (1) age under 40 years, (2) gradual onset, (3) pain improvement through movement, (4) pain not improved by rest, (5) awakening at night to pain that improves with standing and moving
  • Typical symptoms of spondylarthritis such as inflammation of the joints, tendons, fingers or toes
  • Family history of axial spondyloarthritis
  • Blood tests for the HLA-B27 gene
  • Responding well to treatment with nonsteroidal anti- inflammatory drugs (NSAIDs)
  • Signs of increased inflammation ( C-reactive protein and blood sedimentation )
  • Incidence of related diseases such as psoriasis ( psoriasis ), inflammatory bowel diseases ( Crohn's disease , ulcerative colitis ) or eye inflammation ( uveitis )

If these criteria still do not provide a convincing clinical picture, magnetic resonance imaging (MRI) can also be included in the diagnosis.

New York Criteria 1984

The modified New York criteria of 1984 have long been the gold standard for diagnosing ankylosing spondylitis. The following criteria apply here:

  • Deep back pain and stiffness for more than three months, improved with exercise
  • Limited mobility of the lumbar spine in the sagittal and frontal planes
  • Limited thoracic excursion
  • Grade 2-4 bilateral sacroiliitis
  • Grade 3–4 unilateral sacroiliitis

The sacrum-iliac joint is the "key joint" in ankylosing spondylitis . In around 99 percent of patients, the disease is first identified radiologically in the sacrum and iliac joints. Characteristic is bilateral sacrum-iliac joint arthritis with the coexistence of bone resorption and augmentation, subchondral sclerosis, and incipient ankylosis (native radiological grading 1–4). A confirmed SA exists if unilateral grade 3–4 sacroiliitis or bilateral sacroiliitis grade 2–4 and a clinical criterion exist. Laboratory tests are less helpful in SA. Elevations in sedimentation reactions or C-reactive protein are only found in around 30–40 percent of cases.

Movement restrictions associated with the disease can be determined more precisely through simple examinations ( Schober measure , Ott measure , Menell's sign , chin-sternum distance, back of the head-wall distance, breath-dependent change in chest circumference).

In terms of differential diagnosis , ankylosing spondylitis must be distinguished from other diseases of the musculoskeletal system such as osteoporosis , herniated discs, as well as bacterial inflammation and tumor diseases of the vertebral bodies.

Laboratory findings

There is no clear laboratory test for ankylosing spondylitis . 90% of those affected have the HLA-B27 - Gen , but this gene occurs in about 9% of the German population. The presence of this gene is only a risk factor that increases the likelihood of falling ill, but the vast majority of gene carriers remain healthy. The laboratory tests show signs of inflammation, i.e. the erythrocyte sedimentation rate (blood sedimentation rate), the concentration of C-reactive protein and immunoglobulin A are increased, and in severe cases occasionally also the activity of alkaline phosphatase . The rheumatoid factor is negative. Mild anemia may be present.

Imaging procedures

MRI for spondylitis of the left sacrum

Many of the changes in the musculoskeletal system are visible in X-rays . Sacroiliitis of the sacroiliac joint usually occurs first. In the early stages of ankylosing spondylitis, this is also the first reliable proof of diagnosis.

In later stages, there may be bone braces between adjacent vertebrae, spondylarthritis and ossification of the vertebral ligament apparatus. The ossification of the spine is easy to see on X-rays and is also known as the bamboo spine because of its distinctive shape . In MRI (T2-weighted) sacroiliitis for years is more recognizable than in the x-ray.

therapy

Exercise and physiotherapy

With ankylosing spondylitis , it is very important to exercise regularly and systematically take advantage of physiotherapy and to do stretching exercises such as yoga and Pilates as an extension to ankylosing spondylitis in order to keep the joints flexible and to avoid hyperkyphosis . This can often be very painful for those affected. In this way, however, the mobility of the body can often be sufficiently maintained.

Medication

As standard, non-steroidal anti-inflammatory drugs such as anti -inflammatory drugs are used against pain as well as causal, anti-inflammatory therapy . B. Indomethacin or Diclofenac used, in addition, sulfasalazine in certain forms . In addition, according to studies, pamidronate , a bisphosphonate , thalidomide (the active ingredient in Contergan, probably also works by inhibiting TNF-α) and the radioactive isotope radium 224 are effective as an infusion.

The first and only receptor fusion protein etanercept , which works according to the body's own active principle, has been approved since 2003 . The first fully human monoclonal antibody, adalimumab, was approved in 2006 . Both are so-called TNF-α blockers , which inhibit the inflammatory processes mediated by TNF-α. With these expensive preparations, good results are achieved in many cases, but it is not yet possible to foresee how the prognosis for affected patients will change as a result of anti-TNF-α therapy, as there is still no knowledge available for a period longer than six years . Disadvantages are the high annual therapy costs (more than € 20,000 for treatment with Etanercept and Adalimumab) and the side effects that result from the aggressive suppression of the immune system. These include the risk of (re-) activation of latent infections, e.g. B. tuberculosis , demyelinating diseases , blood formation disorders and an increased risk of lymphoma formation .

surgery

In very advanced stages of the disease, there are surgical therapy options, such as breaking the already stiffened spine in a complex and complicated operation in several places and fixing it in an upright position with metal plates ( spondylodesis ), or a wedge osteotomy . Although this does not improve the mobility of the spine, the result can be a significant increase in quality of life, because, among other things, the affected person's field of vision is significantly larger. If the hip joints are involved, surgery with the insertion of a hip prosthesis can also be helpful.

forecast

The course of the disease is often intermittent and varies between different patients. Invalidation can be prevented by doing ankylosing spondylum exercise. In women, ankylosing spondylitis is often milder, and stiffening of the spine is less common.

The influence of ankylosing spondylitis on life expectancy is controversial. Some, but not all, studies suggest that there is a reduction in life expectancy. Most deaths associated with ankylosing spondylitis are the result of spinal cord injury, respiratory failure , aortic regurgitation, or treatment side effects such as upper digestive tract bleeding .

Historical aspects

Spine with ankylosing spondylitis from the early 6th century, Heilbronn area; State Museum Württemberg
Leonard Trasks The Wonderful Invalid .

The ossification of joints and tendon attachments, especially in the axial skeleton, as an indication of ankylosing spondylitis was discovered in a 5000 year old Egyptian mummy.

In 1559, the anatomist and surgeon Realdo Colombo described a disease whose symptoms also apply to ankylosing spondylitis ; the first description of the pathological changes in the spine was made in 1691 using a skeleton found in France by the Irish medical student Bernard Connor . In a similar patient, an inflammation of the iris was documented by Benjamin Brodie in 1818 .

In 1858 David Tucker published a pamphlet describing the case of a patient named Leonard Trask who suffered from a severe spinal deformity due to ankylosing spondylitis . This was the first documented case of ankylosing spondylitis in the United States.

Only towards the end of the nineteenth century (1893–1898) was the first complete description of the disease by Vladimir Bechterew in Russia in 1893, Adolf von Strümpell in Germany in 1897 and Pierre Marie in France in 1898. For this reason, ankylosing spondylitis is also known as Bechterew's disease or Bechterew-Strümpell-Marie disease .

Comparative research shows a high correlation between ankylosing spondylitis and chronic erysipeloid ( red running ) in pigs. This disease is triggered by Erysipelothrix rhusiopathiae and, if it is chronic, leads to an almost identical picture. In veterinary medicine this is known as the carp or "fish spine". Corresponding studies in humans are still pending. Investigations on HLA-B27-positive transgenic test animals were able to show that these primarily gnotobiotic animals only become ill when they come into contact with the ubiquitous microflora and develop the full picture of Bechterew's disease .

Patient organizations

The following German-speaking patent client organizations offer information and assistance in dealing with Bechterew's disease and promote the exchange of experiences with other affected persons:

country Surname website
Germany German Association of Bechterew's Disease http://www.bechterew.de/
Austria Austrian Association of Bechterew's disease http://www.bechterew.at/
Switzerland Swiss Association of Bechterew's Disease http://www.bechterew.ch/

literature

Specialist literature

  • Albrecht Falkenbach (Ed.): Bechterew's disease. Advice, care, treatment. Springer-Verlag, Vienna et al. 2005, ISBN 3-211-00808-X .
  • Wolfgang Miehle: Ankylosing spondylitis (Bechterew's disease). Rheumamed-Verlag, Samerberg 2006, ISBN 3-9810960-5-3 .
  • Tinsley R. Harrison et al .: Harrison's Principles of Internal Medicine. 16th edition. Mcgraw-Hill Professional, New York NY 2005, ISBN 0-07-139140-1 .

Patient guide

  • Paul Schmied, Heinz Baumberger: Bechterew's disease. The inflammatory spinal rheumatism. A guide for patients and their relatives, for doctors, physiotherapists, nurses and social workers. 3. Edition. Urban & Fischer, Munich et al. 2003, ISBN 3-437-45706-3 .
  • Wolfgang Miehle: Ankylosing spondylitis - Bechterew's disease. Information about the tried and tested and new for diagnosis and therapy. Rheumamed-Verlag, Samerberg 2004, ISBN 3-9806607-2-9 .

Web links

Commons : Ankylosing Spondylitis  - Collection of images, videos and audio files

Individual evidence

  1. ↑ a b c d M Rudwaleit, D van der Heijde, R Landewe, J Listing, N Akkoc, J Brandt, J Braun, CT Chou, E Collantes-Estevez, M Dougados, F Huang, J Gu, MA Khan, Y Kirazli , WP Maksymowych, H Mielants, IJ Sorensen, S Ozgocmen, E Roussou, R Valle-Onate, U Weber, J Wei, J Sieper: The development of Assessment of SpondyloArthritis international Society classification criteria for axial spondyloarthritis (part II): validation and final selection . In: Annals of the Rheumatic Diseases . 68, No. 6, 2009, ISSN  0003-4967 , pp. 777-783. doi : 10.1136 / ard.2009.108233 .
  2. ↑ J. Braun, M. Bollow, G. Remlinger et al.: Prevalence of Spondylarthropathies in HLA-B27 positive and negative blood donors. In: Arthritis & Rheumatism 1998; 41, pp. 58-67. PMID 9433870
  3. ↑ Bechterew's disease. V.
  4. ^ J. Braun, J. Sieper: Therapy of ankylosing spondylitis and other spondyloarthritides: established medical treatment, anti-TNF-alpha therapy and other novel approaches. In: Arthritis Res . 2002; 4 (5): pp. 307-321. PMID 12223105 .
  5. ↑ J. Zou, Y. Zhang, A. Thiel, M. Rudwaleit, SL Shi, A. Radbruch, R. Poole, J. Braun, J. Sieper: Predominant cellular immune response to the cartilage autoantigenic G1 aggrecan in ankylosing spondylitis and rheumatoid arthritis. In: Rheumatology . 2003 Jul; 42 (7), pp. 846-855. PMID 12730543
  6. Jump up ↑ CG van Bohemen, AJ Nabbe, HS Goei The, AJ Dekker-Saeys, HC Zanen: Antibodies to Enterobacteriaceae in ankylosing spondylitis. In: Scand J Rheumatol . 1986; 15 (2), pp. 143-147. PMID 3092349 .
  7. ↑ More on HLA-B27: P. Bowness: HLA B27 in health and disease: a double-edged sword? In: Rheumatology. Volume 41, Number 8, August 2002, pp. 857-868, PMID 12154202 (review).
  8. ^ Abul K. Abbas: Diseases of Immunity. In: Vinay Kumar, Abul K. Abbas, Nelson Fausto: Robbins and Cotran - Pathologic Basis of Disease. 7th edition. Philadelphia, 2005, p. 205.
  9. ↑ More on Klebsiella pneumoniae vs. HLA-B27: M. Ogasawara, DH Kono, DT Yu: Mimicry of human histocompatibility HLA-B27 antigens by Klebsiella pneumoniae. In: Infection and Immunity . Volume 51, Number 3, March 1986, pp. 901-908, ISSN  0019-9567 . PMID 3512439 . PMC 260984 (free full text).
  10. ↑ T. Rashid, A. Ebringer: Anklyosing spondylitis is linked to Klebsiella-the evidence Springer, In: Clin Rheumatol. (2007) 26, pp. 858-864.
  11. ↑ A. Ebringer, T. Rashid, C. Wilson et al.: Ankylosing spondylitis, HLA B27 and Klebsiella-an overview: proposal for eraly diagnosis and treatment. In: Curr. Rheumatol. Rev. 2, 2006, pp. 55-68.
  12. ↑ Denis Poddubnyy, Astrid van Tubergen, Robert Landewé, Joachim Sieper, Désirée van der Heijde: Development of an ASAS-endorsed recommendation for the early referral of patients with a suspicion of axial spondyloarthritis . In: Annals of the Rheumatic Diseases . 74, No. 8, 2015, ISSN  0003-4967 , pp. 1483–1487. doi : 10.1136 / annrheumdis-2014-207151 .
  13. ↑ Dennis L. Kasper et al: Harrison's Principles of Internal Medicine. 16th edition. 2005, p. 1995 f.
  14. ↑ Active substance current 04/2006, a publication of the drug commission of the German medical profession (PDF; 82 kB).
  15. ↑ M. Ruf, R. Wagner, H. Merk, J. Harms: Preoperative planning and navigation-assisted wedge osteotomy in Bechterew's disease. In: Journal for orthopedics and their border areas. 144, 2006, p. 52, doi : 10.1055 / s-2006-921484 .
  16. ↑ A. Calin: Ankylosing spondylitis . In: Clinics in Rheumatic Diseases . tape 11 , 1985, pp. 41-60 .
  17. ^ Pierre Marie: Benoist M. - Historical Perspective . In: Spine . tape 20 , 1995, p. 849-852 .
  18. ↑ Wolfgang Miehle: Joint and spinal rheumatism. Eular Verlag, Basel 1987, ISBN 3-7177-0133-9 , p. 55.
  19. ↑ a b B. S. Blumberg :? In: Arch Rheum . tape 1 , 1958, p. 553 .
  20. ^ A Brief historical sketch of the life and sufferings of Leonard Trask, the wonderful invalid (1858). Retrieved February 19, 2013 .
  21. ↑ W. Bechterew: Stiffness of the spinal column and its curvature as a special form of disease . In: Neurol Centralbl . tape 12 , 1893, pp. 426-434 .
  22. ↑ A. Strümpell: Comment on the chronic ankylosing inflammation of the spine and the hip joints. In: Dtsch Z Nervenheilkd . tape 11 , 1897, p. 338-342 .
  23. ^ P. Marie: Sur la spondylose rhizomelique . In: Rev Med . tape 18 , 1898, p. 285-315 .
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