Ankylosing Spondylitis Remission
In childhood, AS usually presents in older boys as an asymmetric oligoarticular arthritis of the lower extremities, often predating back symptoms. Heel pain is a common complaint. However, with time, the child acquires more typical features of adult AS. Almost all children affected are positive for HLA-B27.
The most obvious finding is calcification of the interspinous ligaments causing a bamboo spine appearance on CXR, typical of ankylosing spondylitis. This disorder typically affects young males with predominant involvement of the axial spine and the sacroiliac joints (Fig. 39.2). Upper lobe fibrosis may also result. The lung function abnormality that results is usually restrictive. There is a very strong association with HLA-B27.
This chronic inflammatory disease is characterised by a progressive ligament calcification with hardening of the spine that reduces the function of the spine and therefore causes disability 13 . Although at present there are few clinical trials to confirm the benefits of therapeutic exercise in treating ankylosing spondylitis 14 , it is firmly believed that a rehabilitative program for patients with IBD should include
Analysis of the relationship between TAP binding affinity and HLA class I binding affinity across the full spectrum of HLA alleles is difficult because of the extensive polymorphism of HLA molecules. We addressed the problem by (a) generating a computational model, (b) combining the initial model with a selected set of laboratory experiments for model refinement, and (c) using the refined model to analyse the functional relationship between TAP and HLA class I molecules (Daniel et al 1998). The working ANN model was used to search for patterns of TAP-binding within sets of HLA-binding peptides. The proportion of HLA-binding peptides with affinity to TAP varied for each HLA class I allele with a range of 15 for HLA-B*5401 to 100 for HLA-B*2703 (Brusic et al 1999). On the basis of these results we hypothesize that HLA alleles constitute two separate classes those that are TAP-efficient for peptide loading (HLA-B27, -A3 and -A24) and those that are TAP-inefficient (HLA-A2, -B7 and -B8)....
The most common form of musculoskeletal tuberculosis is tuberculous spondylitis. isolated extraspinal bone infection by Mycobacterium tuberculosis is less common. The most frequent form of musculoskeletal involvement, excluding the spine, is within muscles and soft tissues 12 .
Fig. 2. (A) Sagittal T2-weighted image demonstrates fluid in the expected location of the T8-9 disc space with associated endplate erosion. This appearance could be misconstrued as indicative of discitis. (B) Sagittal CT reformation more clearly shows the bony fusion characteristic of ankylosing spondylitis, which in this case is complicated by fracture and pseudoarthrosis. Patients with DISH are at risk for similar injuries. Note again the association of a vacuum cleft with high T2 signal on MR. Fig. 2. (A) Sagittal T2-weighted image demonstrates fluid in the expected location of the T8-9 disc space with associated endplate erosion. This appearance could be misconstrued as indicative of discitis. (B) Sagittal CT reformation more clearly shows the bony fusion characteristic of ankylosing spondylitis, which in this case is complicated by fracture and pseudoarthrosis. Patients with DISH are at risk for similar injuries. Note again the association of a vacuum cleft with high T2 signal on...
Seronegative spondyloarthropathies (see section .I. ,B 5). In patients with psoriatic arthritis, Reiter's syndrome, or ankylosing spondylitis, a chronic phase may develop. Characteristic features include sacroiliitis, asymmetric oligoarthritis or polyarthritis of the lower extremities, and spondylitis. Even Reiter's disease, with its typical episodic flares of activity, becomes chronic in nearly 75 of patients.
Weight loss chronic cough change in bowel habits night pain or other constitutional symptoms may suggest an underlying
Similar pain or morning stiffness in other areas of the body would increase the suspicion that this represents a more generalized rheumatologic condition (e.g., ankylosing spondylitis, psoriatic arthritis, or endocrine disorder, such as hypothyroidism, hyperthyroidism, or hyperparathyroidism).
Other seronegative spondyloarthropathies. In any of the other seronegative spondyloarthropathies, skeletal inflammation may develop that is essentially identical to that of classic AS. Usually, the extraarticular manifestations of these diseases allow clinical differentiation, but there is probably an overlapping spectrum of features. The spondylitis of Reiter's syndrome and psoriatic arthropathy is usually less severe than that of typical AS, and syndesmophytes tend to be asymmetric. VIII. Prognosis. The course of AS varies. In some patients, the disease progresses relentlessly (often despite therapy), with fusion of axial and peripheral joints. In others, bony ankylosis may develop gradually with little pain or discomfort. In still others, skeletal involvement may be limited to only mild sacroiliitis and never progress to serious spondylitis or ankylosing disease.
Radiographic features considered classic for PA are destructive lesions involving predominantly the DIP joints of fingers and the IP joints of the toes. Bony ankylosis of the DIP joints of the hand and toes, along with bony proliferation of the base of the distal phalanx, and resorption of the tufts of the distal phalanges of hands and feet are also commonly seen. Other classic features are fluffy periostitis of large joints, pencil-in-cup appearance of DIP joints, an asymmetric joint pattern, and gross destruction of isolated small joints. Changes in the spine and sacroiliac (SI) joints may be similar to those seen in ankylosing spondylitis, but SI joint changes in PA are often unilateral, and syndesmophytes can sometimes be distinguished from those of ankylosing spondylitis.
Presentation is typically a chronic monarthritis or spondylitis. Skeletal tuberculosis is usually a combined osteomyelitis and arthritis, as infective lesions in epiphyseal bone invade the adjacent joint. The chest radiographic findings are often normal, and constitutional symptoms may not be present. The tuberculin skin test result is almost always positive. Synovial fluid analysis reveals an elevated WBC count (usually 10,000 to 20,000 pL) with neutrophil predominance (80 of cases). Acid-fast stains of joint effusions are positive in only 27 , and although joint fluid culture is positive in 83 , cultivation requires 4 to 6 weeks of incubation. Synovial biopsy is the procedure of choice for immediate diagnosis histopathology demonstrates granuloma formation in 95 , caseation in 55 , and the tubercle bacillus in 10 of cases.
Uveitis is etiologically classified as either infectious or non-infectious. The predominant form of the disease is felt to be non-infectious - specifically, autoimmune for AU. HLA-B27-associated acute AU is the most common form of non-infectious uveitis that occurs in genetically predisposed individuals. This allele is frequently associated with acute AU in conjunction with a spondyloarthropathy, such as anklyosing spondylitis. Although the B27 AU is felt to be autoimmune in origin, there is some evidence that a microbial trigger for the disease may exist - specifically, certain species of Klebsiella, Salmonella, Shigella, Yersinia and Chlamydia trachomatis have been implicated 9 . This has recently focused attention on the role of Toll-like receptors (TLRs) within the eye and the pathogen-associated molecular patterns on these and other
Patients with ankylosing spondylitis may have spinal or peripheral joint involvement and may require surgical intervention in the course of their illness. A variety of problems may arise, primarily as a result of severe spinal involvement. Patients with a rigid or ankylosed cervical spine may present the most challenging cases of endotracheal intubation to anesthesiologists. Fiberoptic techniques can be helpful, even mandatory, in these patients. If the patients are rigid and osteoporotic, there is a risk for spinal fracture and paraplegia with uncontrolled movement. In addition, restrictive lung disease often arises as a consequence of thoracic spinal involvement and increases the potential for postoperative pulmonary complications. Aggressive pulmonary toilet is mandatory in patients with anklyosing spondylitis, irrespective of the type of surgery that they are undergoing. A small percentage of these patients may also have underlying aortic valve disease...
Several types of studies have supported an association between expression of a particular MHC allele and susceptibility to autoimmunity, an issue covered in detail in Chapter 7. The strongest association between an HLA allele and an autoimmune disease is seen in ankylosing spondylitis, an inflammatory disease of vertebral joints. Individuals who have HLA-B27 have a 90 times greater likelihood of developing ankylosing spondylitis than individuals with a different HLA-B allele. However, the existence of such an association should not be interpreted to imply that the expression of a particular MHC allele has caused the disease, because the relationship between MHC alleles and development of autoimmune disease is complex. It is interesting to note that, unlike many other autoimmune diseases, 90 of the cases of ankylosing spondylitis are male.
1 It should be noted that these criteria were developed before the new classification of rheumatic diseases was adopted by the American Rheumatism Association in 1963, in which ankylosing spondylitis, psoriatic arthritis, and arthritis associated with ulcerative colitis and regional enteritis are listed as distinct from rheumatoid arthritis (Blumberg B, et al. ARA nomenclature and classification of arthritis and rheumatism. Arthritis Rheum 1964 7 93).
The examiner should first inspect the thorax from the foot of the bed with the subject supine, the legs horizontal, and the head and trunk elevated to approximately 300.205 The patient may have a barrel-shaped chest with an increased anteroposterior diameter, a straight-back syndrome, pectus excavatum, pectus carinatum, kyphoscoliosis, or ankylosing spondylitis. Each may produce or be associated with cardiac abnormalities. Asymmetry of the thorax due to convex bulging of the precordium suggests the presence of heart disease since childhood. Exaggerated movements of the cardiac apex often can be detected from this observation point.
A measured maximum chest expansion of 2 cm or less is definitely abnormal. Chest expansion is diminished in almost every type of diffuse bronchopulmonary disease, e.g. bronchial asthma, emphysema and pulmonary fibrosis, and in conditions which restrict movement of the ribs, such as ankylosing spondylitis. the bowel, a large ovarian cyst or pregnancy. If respiratory movements are exclusively abdominal, ankylosing spondylitis, intercostal paralysis or pleural pain may be responsible for the lack of chest expansion.
The CXR shows a right upper lobe ball within a cavity (air crescent sign) patho-gmonic of a mycetoma (also called aspergilloma). A lateral decubitus X-ray may demonstrate the fungal ball shifting position. In this condition, a preformed cavity becomes colonized, usually by the fungus Aspergillus fumigatus. Cavitary disease may be secondary to fibrotic lung disease, e.g. previous tuberculosis, sarcoidosis, or ankylosing spondylitis. Massive hemoptysis can result and bronchial angiogram with embolotherapy (using coils or gel foam) is temporizing. Surgical resection is definitive, but bronchopleural fistula may result. Unfortunately, most patients have insufficient pulmonary reserve to allow surgical resection.
Ankylosing spondylitis is a disease of the spine that appears mainly in males. Joint cartilage is destroyed eventually the disks between the vertebrae calcify and there is fusion of the bones (ankylosis) (Fig. 19-12). Changes begin low in the spine and progress upward, limiting mobility. FIGURE 19-12. Ankylosing spondylitis. Bone bridges fuse one vertebra to the next across the intervertebral discs and fuse the posterior portions of the vertebrae. There is osteoporosis from disuse. (Reprinted with permission from Rubin E, Farber JL. Pathology. 3rd Ed. Philadelphia Lippincott Williams & Wilkins, 1999.)
MRI plays a pivotal role in the evaluation of infectious spondylitis particularly because the diagnosis is often a difficult one to make clinically. Patients with spinal infections present with nonspecific symptoms such as malaise, focal tenderness, radiculopathy, and back pain (38-40). These symptoms may be attributable to other etiologies such as degenerative disorders, spinal stenosis, and neoplasms. In fact, back pain (the most common symptom in patients with infectious spondylitis 40,41 ) is also the second leading cause of physician visits affecting 5 of the population annually (42,43). Physicians therefore often rely on imaging modalities and on laboratory tests such as elevated erythrocyte sedimentation rate (ESR), white blood cell (WBC) count, or C-reactive protein (40). In a group of patients with pyogenic vertebral osteomyelitis evaluated by Caragee, 30 of immunocom-promised and 44 of immunocompetant patients had abnormal WBC counts while 89 of immunocompromised and 100 of...
Susceptibility of mice to infection with some viruses, for example, cytomegaloviruses, retroviruses, and lymphocytic choriomeningitis virus, has been shown to be linked to particular MHC genotypes. Many reports of such associations in humans have appeared in the literature, for example, affecting susceptibility resistance to AIDS or seroconversion following hepatitis B vaccination, but the correlations are generally not as dramatic as in the case of, for example, the association of ankylosing spondylitis with HLA B27.
Axial involvement is more frequent in Crohn's disease (5 to 22 ) than in ulcerative colitis (2 to 6 ). There is no male predominance, as there is in ankylosing spondylitis, and only 50 to 70 of patients are positive for HLA-B27. In fact, the presence of ankylosing spondylitis in patients negative for HLA-B27 should predict inflammatory bowel disease or psoriatic arthritis.
This group of diseases is characterized by presence of the class I histocompatibility antigen HLA-B27 axial arthritis, including spondylitis and sacroiliitis and inflammatory disease of the eye, skin, and ligamentous insertions (enthesopathy). The joint pattern is usually oligoarthritic and asymmetric, and large joints of the lower extremity are involved. Rheumatoid factor and antinuclear antibodies are not usually found in the serum of these patients. The specific diagnosis is usually defined by the associated clinical features psoriatic arthritis by the presence of psoriasis, Reiter's syndrome by the concomitant conjunctivitis and urethritis, and enteropathic arthritis by the presence of ulcerative colitis or Crohn's disease. a. Ankylosing spondylitis has perhaps the least association with peripheral joint involvement of the group it usually presents insidiously with symptoms in the axial spine. However, inflammatory involvement of the peripheral...
The spine is evaluated from the front, sides, and back. The curves of the spine are also assessed. Look for flattening of the lumbar curve. Assess for muscle spasms and decreased spinal mobility, which may be associated with ankylosing spondylitis. An accentuated lumbar lordosis
Prognosis for axial disease is similar to that of ankylosing spondylitis. III. Other spondyloarthritides Less prominent intestinal symptoms may occur in other forms of spondyloarthritis. In fact, endoscopic studies of the colon and ileum have shown macroscopic inflammatory lesions in 18 to 50 of patients with spondyloarthritis. The prevalence of such lesions is even higher by histologic criteria. Acute inflammatory changes resemble those of bacterial enteritis, and chronic inflammatory lesions are similar to those seen in Crohn's disease. Chronic gut inflammation is associated with more severe and persistent arthritis or spondylitis, and the condition occasionally evolves into clinically apparent Crohn's disease.
The CXR shows bilateral infiltrates and calcified nodules in both upper lobes. Differential diagnoses of upper lobe infiltrates include silicosis, tuberculosis, and ankylosing spondylitis. There is also egg-shell calcification of the hilar lymph nodes. The egg-shell calcification plus the upper lobe nodules are typical of silicosis. Differential diagnoses of egg-shell calcification include sarcoidosis, Hodgkin's lymphoma following radiotherapy, and coal-worker's pneumoconiosis.
The lumbar spine has a smooth lordosis which may be lost in certain disorders such as ankylosing spondylitis and lumbar disc protrusion (Disorders box). The main surface markings are the spinous processes of 1,4 5 which are level with the pelvic brim and the. 'dimples of Venus which lie over the sacroiliac joints.
EN develops in parallel with the colitis whereas PG may be concomitant or subsequent, even developing years after colectomy 45 . EN is characterised by sudden onset of multiple, bilateral, symmetrical, painful, red and warm nodules arising mainly on the extensor surfaces of the arms and legs. These nodules are often associated with systemic symptoms, such as fever, malaise and joint pain. The typical course of the disease lasts 3-6 weeks, but the residual bruiselike lesions may last for months. As for the presence of different EIM in the same patient (i.e. EN, anky-losing spondylitis and uveitis), there are numerous hypotheses to explain overlapping syndromes, one of which is based on the assumption of a common antigen, an isoform of tropomyosin, in the eyes, skin and joints. The likely aetiology is a hypersensitive response due to deposition of immune complex in and around venules in the septa of connective tissue in subcutaneous fat. Treatment for EN usually focuses on the...
The presenting symptoms of pathology in the thoracic spine are usually localised spinal pain (see Disorders box) or pain radiating round the chest wall or, less frequently, symptoms of paraparesis including sensory loss, leg weakness, and loss of bladder or bowel control. Disc lesions are rare and may be accompanied by girdle pain radiating around the chest, mimicking cardiac or pleural disease. Pain arising from joints at the thoracolumbar junction can occur in ankylosing spondylitis and again may be confused with pulmonary, renal or cardiac problems. The patient with osteoporosis may complain of becoming progressively stooped (Dowager hump) with loss of height but without neurological features. Acute osteoporotic and malignant vertebral collapse are common causes of acute thoracic pain, and the latter is not infrequently associated with spinal cord compression. Ankylosing spondylitis Back pain is an almost ubiquitous cxpcriencc and most adults will have experienced it by the time...
For example, in a patient with chronic low back pain and stiffness of an inflammatory character, ankylosing spondylitis should be considered. Here, the ESR may be raised or normal. Absence of the HLA-B27 tissue type makes the diagnosis unlikely but does not exclude it furthermore, because about 12 of the general population carry this haplotype, its presence is not diagnostic of spondylitis. A plain X-ray of the pelvis may demonstrate sacroiliitis, and of the lumbar and thoracic spine may demonstrate 'squaring' of the vertebrae (the normal concave anterior and posterior surfaces of the vertebral body appear flattened owing to sub-ligamentous calcification forming deep to the longitudinal ligaments). As the disease progresses, frank ossification of the longitudinal ligaments becomes apparent.
Image-guided spine biopsy procedures are usually performed to diagnose suspected primary or secondary neoplastic processes or to evaluate for the presence of infectious spondylitis.6 These procedures are less frequently performed to assess for other noninfectious inflammatory conditions that can affect the spine. The decision to perform a spine biopsy procedure is made after close communication between the radiologist and the referring clinician. Both individuals must be convinced that the benefit to be gained from the biopsy results firmly outweighs the risks of the procedure. To this end, as a prerequisite, there must be a thorough medical history and physical examination combined with a complete review of all prior imaging and laboratory examinations. This consultation will avoid unnecessary spine biopsies (when they are not indicated or when a more accessible bone biopsy site, such as the iliac bone, is available), ensure patient safety, and identify the optimal location and level...