Ankylosing Spondylitis Remission
Virtually all adult men with ankylosing spondylitis (AS), which is chronic inflammation of the spine and sacroiliac joints, are positive for the genetic marker HLA B27 positive, but we do not know how many boys who are positive for HLA B27 will never develop AS. Most individuals with AS do not fulfill criteria for the diagnosis until after they reach thirty years of age, and it is difficult to keep track of HLA B27 positive teenagers for the fifteen years or more of follow-up needed to answer that question. Therefore, I do not recommend diagnosing every boy who is positive for HLA B27 and has a spondyloarthropathy as having juvenile ankylosing spondylitis (JAS). Not only does it cause undue worry for many parents, but it may also make life and health insurance difficult to obtain. Although some will develop AS, not all will, and labeling them is unnecessary. However, an HLA B27 positive boy who has swollen joints and an elevated sedimentation rate is at risk of progressing to definite...
Surgical fusions to treat spinal deformity were first developed in the 1900s. In 1911, Hibbs 1 performed a noninstrumented, posterior spinal fusion for deformity related to tuberculous spondylitis. In 1962, Harrington 2 revolutionized spinal fusion for deformity with the use of his distraction rod instrumentation system. 25 Kim KT, Suk KS, Cho YJ, Hong GP, Park BJ Clinical outcome results of pedicle subtraction osteotomy in ankylosing spondylitis with kyphotic deformity. Spine 2002 27 612-618.
There is a reduced incidence of sacroiliitis, with 23.4 of enthesis-related JIA showing radiological changes at diagnosis, compared to 100 of patients with adult ankylosing spondylitis as demanded by New York classification criteria. However, there is also likely to be significant overlap of enthesitis-related JIA with adult undifferentiated spondyloarthropathy where radiological sacroiliitis is not a cardinal diagnostic feature. In juvenile enthesitis-related arthritis there is a higher rate of peripheral arthritis and enthesitis at presentation when compared to adult onset ankylosing spondylitis (19), and more enthesitis when compared to other subtypes of JIA (Table 4) (21). The rate of patients being HLA-B27 positive are similar in enthesis-related JIA and ankylosing spondylitis.
Several genetic loci are shown to be involved in the etiology of multiple autoimmune diseases in humans and support sharing of underlying disease mechanisms across related phenotypes. Associations of certain HLA loci with autoimmune diseases has been reported extensively in SS, SLE, RA, ankylosing spondylitis, psoriasis, multiple sclerosis, and type 1 diabetes.22 A growing list of non-HLA genes also has been implicated in multiple autoimmune diseases. Examples include associations of cytotoxic T-lymphocyte-associated antigen 4 (CTLA4) with AITD, type 1 diabetes mellitus (T1D), celiac disease, Wegener's granulomatosis, SLE, vitiligo, Addison's disease, and RA 23-30 Programmed Death 1(PD-1) with RA, T1D, and SLE 31 and protein tyrosine phosphatase nonreceptor type 22 (PTPN22) with SLE, RA, T1D, Graves' disease, and Hashimoto's thyroiditis.32-37 Interferon regulatory factor 5 (IRF5) and signal transducer and activator of transcription 4 (STAT4) are genes strongly associated with SLE for...
Crohn's disease is a chronic inflammatory disorder that can affect any part of the gastrointestinal tract involvement of the small bowel may be referred to as Crohn's enteritis. Crohn's disease is comparatively more common in North America and Europe which have the highest incidence the disease is relatively rare elsewhere, particularly in developing countries. To put it in context of all causes of enteropathy, recent data suggests the incidence of all childhood and adolescent Crohn's disease in northern hemisphere countries is estimated to be 2-3.7 cases per 100,000. The cause of Crohn's disease has not been defined, although the pathogenesis is complex involving an interrelation between genetic predisposition and environmental factors (fig. 2). The intestinal manifestations reflect the anatomic site of involvement, with Crohn's enteritis manifested in various combinations as diarrhea, fever, occult or gross blood in the stool, abdominal pain, aphthous ulcers, weight loss and...
We conducted the first anti-IFN-y therapy in Th-1-mediated diseases together with several clinics using a polyclonal anti-IFN-y antibody (both IgG and F(ab')2 antibody fragments) and in some cases, for comparison, a polyclonal anti-TNF-a. The antibodies were given intramuscularly once or twice daily for 5-6 days. Our studies include placebo-controlled, double-blind studies in patients with rheumatoid arthritis unresponsive to standard treatments (Sigidin et al. 2001) and in patients with secondary progressive multiple sclerosis (Skurkovich et al. 2001). In addition, we conducted smaller proof-of-principle studies in patients experiencing corneal transplant rejection (Skurkovich et al. 2002a), in psoriatic arthritis (Skurkovich et al. 1998), type I diabetes (Skurkovich et al. 2003a), uveitis (Skurkovich et al. 2003b), juvenile rheumatoid arthritis, auky-losing spondylitis and in several autoimmune skin diseases (Skurkovich et al. 2002b).
Cardiac involvement in adults with spondyloarthropathies is well recognized. Fortunately, it occurs in only a small number of children. The most common form of cardiac involvement is inflammation of the root of the aorta (the vessel carrying blood out of the heart), which is called aortitis. This inflammation can result in damage to the heart valves and a condition called aortic insufficiency. Children with aortic insufficiency complain of loss of energy and increasing shortness of breath with activity. The condition is easily detected by echocardiography, which is a painless and simple test in which sound waves are bounced off the heart to monitor the motion of the heart valves and muscle. Less frequent complications include involvement of the lungs or kidneys. These cardiac complications have been described in teenagers but are very rare. Most often these complications occur in adults with definite ankylosing spondylitis.
Extra-intestinal manifestations of IBD occur in approximately 15 of all Crohn's disease patients but in up to 30 of those with colonic disease (Jewell 2000b). The extraintestinal features present in Crohn's disease are similar to those experienced in ulcerative colitis. They include erythema nodosum, peripheral arthritis and ocular lesions. Less commonly ( 5 ), patients report pyoderma gangrenosum, primary sclerosing cholangitis, renal complications and anky-losing spondylitis. Some of these manifestations occur during active phases of the disease and respond to treatment of the bowel disorder, others (e.g. ankylosing spondylitis and hepatic complications) appear completely unrelated to disease activity.
Bifidobacteria and an atypical composition of bifidobacterial microflora. Also, aberrations in Clostridium content and composition have been reported to be important. Similar predisposing factors may also exist in the case of microflora and both inflammatory gut diseases and rotavirus diarrhea. Microflora aberrations have also been reported in rheumatoid arthritis, juvenile chronic arthritis, ankylosing spondylitis, and irritable bowel syndrome patients. A thorough knowledge of the intestinal microflora composition will offer a basis for future probiotic development and the search for new strains for human use. Many diseases and their prevention can be linked to the microflora in the gut.
More than 30 of skeletal TB cases involve the spine (tuberculous spondylitis or Pott's disease). The most commonly affected area is the lower thoracic spine, followed by the lumbar, cervical, and sacral areas. The mode of spread to the spine is usually hematogenous, but it also can result from contiguous disease or lymphatic spread from TB pleuritis. In contrast to common bacterial causes of spinal osteomyelitis that initially present as discitis with adjacent vertebral body involvement, TB spondylitis typically begins within the anterior vertebral body. With time, spread to the adjacent disc and vertebra occurs, and vertebral body wedging develops. A 12-month course of treatment is recommended for Pott's spondylitis. In a review of uncomplicated cases, response rates to systemic chemotherapy and bed rest until pain
Over-zealous attempts to force a joint to become fully functional in this situation will often lead to extreme reactions. Inflammation due to ankylosing spondylitis or Reiter's disease is a situation where excessive active treatment is best avoided. Gentle settling techniques can be of use to reduce the irritability enough so more powerful procedures can be used later. The timing and selection of when to treat can only be gained by thought, consideration and experience.
Adolescents with back pain related to JIA are most likely to have either enthesitis-related arthritis (ERA) or psoriatic arthritis. Back pain in these disorders results from inflammation of the spine and or sacroiliac (SI) joints. In contrast to the pattern seen with mechanical back pain, inflammatory back pain is worsened by rest (i.e., morning stiffness), and improved by activity. ERA is more common in adolescent Caucasian boys and in those with a family history of HLA-B27-associated diseases (57,58,84,85). These adolescents often present with enthesitis of the heel or knee, peripheral arthritis, and less commonly acute anterior uveitis (57,83,84). Although adults with ankylosing spondylitis (AS) frequently have inflammatory spinal pain at onset, only a minority of children with ERA (12.8-24 ) has been reported to have pain, stiffness, or limitation of movement of the lumbo-sacral or SI region during the first year of disease (57,83). Instead, most develop back symptoms after 5-10...
Spondyloarthropathies, including ankylosing spondylitis, are accompanied by acute anterior uveitis in about one-third of patients during the course of their disease. Anterior uveitis is typically acute in onset and unilateral it lasts four to eight weeks, and subsides without sequelae, if treated early (13). Cervical spondylitis is the other common head and neck manifestation of spondyloarthropathy. Examination of the neck may reveal decreased range of motion in all planes. Temporomandibular joints are affected in about 10 of patients with spondyloarthropathy. Other sites of involvement often include the thoracic and lumbar spine, sacroiliac joints, hip, and shoulder the smaller peripheral joints also can be affected. Spondyloarthropathy occurs in a subset of patients with psoriasis, inflammatory bowel disease, and reactive arthritis.