Aortic insufficiency and pelvospondylitis in a seropositive female with rheumatoid nodules

Aortic insufficiency appeared in a female patient with seropositive, nodular rheumatoid arthritis. X-ray changes of pelvospondylitis strongly suggested ankylosing spondylitis. Aortic insufficiency and pelvospondylitis are discussed as they relate to rheumatoid arthritis and ankylosing spondylitis.     

Upward subluxation of the axis in ankylosing spondylitis. A clinical pathologic report.

Upward subluxation of the axis associated with cord compression and death was noted in a patient with a long history of idiopathic ankylosing spondylitis. Upward subluxation of the axis has been recognized in up to 8 per cent of patients with rheumatoid arthritis but it is an exceedingly rare complication of ankylosing spondylitis. In this patient psoriasis and then psoriatic dactylitis developed 26 years after the onset of his ankylosing spondylitis. It is tempting to speculate that the unusual destruction of the joints around the atlas might be due to an added effect of psoriasis on idiopathic ankylosing spondylitis.     

New treatment options in spondyloarthropathies: increasing evidence for significant efficacy of anti-tumor necrosis factor therapy

Tumor necrosis factor-alpha is a major effector and regulatory cytokine, which seems to have an outstanding position in rheumatic and other inflammatory states. Because TNF-alpha has been detected in the inflamed gut and sacroiliac joints of patients with chronic inflammatory bowel diseases and spondyloarthritides, like ankylosing spondylitis, there was need for studies of the efficacy of the modern biologic anti-TNF agents infliximab and etanercept in these diseases. Infliximab is approved for the treatment of Crohn disease. In addition, there are now also positive data for infliximab in the treatment of ankylosing spondylitis and for etanercept and infliximab in the treatment of psoriatic arthritis.     

Ankylosing spondylitis and inflammatory bowel disease. II. Prevalence of peripheral arthritis, sacroiliitis, and ankylosing spondylitis in patients suffering from inflammatory bowel disease.

To establish the prevalence of peripheral arthritis, radiographic sacroiliitis, and ankylosing spondylitis in patients with inflammatory bowel disease, 58 consecutive patients suffering from ulcerative colitis (UC) and 51 with Crohn's disease (CD) underwent a detailed rheumatological examination. In addition, all patients were screened for the presence of the antigen HLA B27. Peripheral arthritis was found in 14 (8 UC, 6 CD) patients (12.8%); radiographic sacroiliitis was diagnosed in 11 (5 UC, 6 CD) (10.1%), of whom 10 were asymptomatic; and ankylosing spondylitis was diagnosed in 2 UC and 2 CD patients (3.7%). 18.9% of the UC and 3.9% of the CD patients were HLA B27 positive. One of the 11 patients with radiographic sacroiliitis and 2 of the 4 with ankylosing spondylitis had the HLA B27 antigen. Peripheral arthritis, radiographic sacroiliitis, and ankylosing spondylitis are apparently frequent manifestations in patients suffering from inflammatory bowel disease. Asymptomatic radiographic sacroiliitis in these patients appears to differ from idiopathic ankylosing spondylitis, both clinically and genetically. Evaluation of subjective rheumatological complaints, necessary for a confident diagnosis of ankylosing spondylitis, according to the New York criteria is difficult during a flare-up of the inflammatory bowel process, as was shown in 4 CD cases with marked limitation of lumbovertebral function and chest expansion, but no radiological abnormalities of the SI joints.     

Four cases of ankylosing spondylitis in medieval skeletal series from Croatia

Osteological changes consistent with ankylosing spondylitis were observed in three males and one female skeleton recovered from four medieval sites??Velim, Koprivno, Buje, and Rijeka??all situated on Croatia??s eastern Adriatic coast and its immediate hinterland. The skeletons present changes in the spine, ribs, sacrum, and innominates that are typical of ankylosing spondylitis that is a progressive, inflammatory disease of connective tissue calcification. The disease most commonly affects the sacroiliac joints, the joints of the spine, and the costovertebral joints. In the final stages of the disease, the vertebral bodies remodel and together with the associated syndesmophytes form a continuous, smooth bone surface that is sometimes referred to as ??bamboo spine.?? The prevalence of this disorder in the analyzed Croatian samples is 4/303 or 1.3% and thus corresponds with frequencies recorded in modern European populations. Differential diagnosis rules out the possibility of DISH, rheumatoid arthritis, and melorheostosis. These are the first cases of ankylosing spondylitis identified in Croatian archaeological series.     

The eye and inflammatory rheumatic diseases: The eye and rheumatoid arthritis,ankylosing spondylitis,psoriatic arthritis

Rheumatoid arthritis, ankylosing spondylitis and psoriatic arthritis are associated with potentially sight-threatening inflammatory eye disease. Although the ocular manifestations associated with ankylosing spondylitis and psoriatic arthritis are similar, such as anterior uveitis, this differs from rheumatoid arthritis where dry eye, peripheral ulcerative keratitis and scleritis are the major ocular complications. Apart from causing sight loss, these conditions are painful, debilitating, often recurrent or chronic and may require long-term therapy. Treatments such as ocular lubricant, topical corticosteroid, systemic corticosteroid and systemic immunosuppression are often similar for the underlying systemic disease. Yet for the treatment of the ocular complications, the evidence base is weak. Close collaboration with a rheumatologist is often essential, particularly in the management of these patients.     

Extreme atrophy of the shoulder muscles in juvenile ankylosing spondylitis as a (misleading) main symptom

An extreme unilateral muscular atrophy of the shoulder and upper arm region was a symptom of juvenile ankylosing spondylitis in a 20-year-old female patient. No pathological patterns were found in electromyographic, bioptic, and tomographic (CT, NMR) investigations. The muscular atrophy was caused by a shoulder arthritis with severe erosive damage. The false assumption of a neurological disorder and the disregard of anamnesis and low back pain delayed for several years an accurate diagnosis. After the onset of an arthritis of hip joints a collagen disease with myositis was supposed falsely in spite of normal electromyographic results. The unusual muscular atrophy around the shoulder joint probably must be interpreted as a consequence of reflex inhibition and partly due to inactivity. A real myositis seems to not be probable, because newer investigations in contrast to earlier findings show no evidence for inflammatory muscle disease in ankylosing spondylitis.     

Clinical aspects, outcome assessment, and management of ankylosing spondylitis and postenteric reactive arthritis

The cause of ankylosing spondylitis remains unclear. Proof that this disorder is an autoimmune disease attributable to crossreactivity between bacteria and HLA-B27 is still lacking. Differences in endogenous peptide presentation by HLA-B27 subtypes might be relevant in the etiopathogenesis. Fractures of the osteoporotic spine contribute to morbidity. Spinal cord injury may occur. MR imaging enables identifying sacroiliitis earlier than plain radiography. Sweet syndrome has now been described in patients with ankylosing spondylitis and Crohn disease. Progress has been made in the assessment of ankylosing spondylitis. There are now core sets for different settings and validated instruments for functioning and disease activity that will enable demonstrating efficacy of new therapeutic interventions.The debate continues on classification of postinfectious and reactive arthritis. Bacterial antigens may be found in the inflamed joints; occasionally 16S ribosomal RNA is also demonstrated. Antibiotics seem not to be effective in postenteric reactive arthritis.More than 25 years have now elapsed since the association between ankylosing spondylitis and HLA-B27 was first described in 1973. The cause of this disease is still unknown, but a lot of progress has been made regarding the molecular structure of HLA-B27, the spectrum of disease, the clinical and radiographic assessment of ankylosing spondylitis, and its treatment. Recent advances in research on ankylosing spondylitis are reviewed here.     

Diet and disease symptoms in rheumatic diseases — Results of a questionnaire based survey

Summary Experiences with food intake, diet manipulations and fast were registered in rheumatic patients. The study was a questionnaire-based survey in which 742 patients participated. It comprised 290 patients with rheumatoid arthritis, 51 patients with juvenile rheumatoid arthritis, 87 patients with ankylosing spondylitis, 51 patients with psoriatic arthropathy, 65 patients with primary fibromyalgia and 34 patients with osteoarthritis. One third of the patients with rheumatoid arthritis, ankylosing spondylitis and psoriatic arthropathy reported aggravation of disease symptoms after intake of certain foods while 43% of the patients with juvenile rheumatoid arthritis and 42% of the patients with primary fibromyalgia stated the same. Twenty-six percent of the patients with juvenile rheumatoid arthritis and 23% of the patients with rheumatoid arthritis, ankylosing spondylitis and primary fibromyalgia had previously tried certain diets in the attempt to alleviate disease symptoms, whereas 13% of the patients with sporiatic arthropathy and 10% with osteoarthritis had tried diet therapy. Less pain and stiffness were reported by 46% of the patients and 36% reported reduced joint swelling. Similar beneficial effects of diet were also reported in other rheumatic disease groups. Fifteen percent of the patients with rheumatoid arthritis and ankylosing spondylitis had been through a fasting period. Less pain and stiffness were reported by 2/3 of the patients in both groups and half of the patients in both groups reported a reduced number of swollen joints.     

Enthesitis in seronegative spondyloarthropathies with special attention to the knee joint by MRI: a step forward toward understanding disease pathogenesis

Seronegative spondyloarthropathies are a unique group of disorders sharing similar clinical characteristics (e.g., inflammatory back pain, spondylitis, sacroiliitis, uveitis, inflammatory bowel disease, skin rashes, and enthesitis). Clinical and genetic similarities suggest that they also share similar causes or pathophysiologies. Rheumatoid factor (RF) is characteristically negative in this group of disorders, hence collectively termed seronegative spondyloarthropathies (SpA). They include psoriatic arthritis, ankylosing spondylitis, reactive arthritis, ulcerative colitis, and Crohn’s disease. “Enthesitis”, the term used to describe inflammation at tendon, ligament, or joint capsule insertions, is considered a common feature in this domain and was included in the European Spondyloarthropathy Study Group criteria for the classification of SpA. Evaluation of entheseal-related changes at different joints by MRI became an important item on the research agenda in both differentiated and undifferentiated arthritis. Most of the research focused on MRI findings in the hand and wrist joints among patients with RA and SpA and support two patterns of inflammation “RA” phenotype where synovial involvement is the primary target of inflammation and “SpA” pattern where enthesitis comes first followed by synovitis. In this review, we summarize the literature on enthesitis in SpA and focus on MRI findings in the knee joint in the SpA group of disorders and subclinical synovitis among patients with skin psoriasis.     

Osteopoikilosis coexistent with ankylosing spondylitis and familial Mediterranean fever

Osteopoikilosis (OPK) is a rare benign sclerosing disease of the skeleton and inherited as an autosomal dominant trait. OPK is associated with inflammatory rheumatic disorders, such as rheumatoid arthritis, scleroderma, reactive arthritis and familial Mediterranean fever (FMF). We report a rare case of OPK coexistent with ankylosing spondylitis and FMF. The patient presented multiple sclerotic lesions within and around the sacroiliac joints and a series of radiological diagnostic challenges.     

EFFICACY AND SAFETY OF RADIATION SYNOVECTOMY WITH YTTRIUM-90: A RETROSPECTIVE LONG-TERM ANALYSIS OF 164 APPLICATIONS IN 82 PATIENTS

In this long term retrospective study of radiation synovectomywith Yttrium-90 (Y90), we evaluated the results of 164 applicationsin 82 patients with RA, OA with synovitis, ankylosing spondylitisand psoriatic arthritis. Radiation synovectomy with Y90 hasan overall success rate of approximately 50% and is thereforean effective alternative to surgical synovectomy in chronicsynovitis which fails to respond to conservative treatment.Elbow and knee responded significantly better than shoulderand ankle joints. Patients with radiological stages from 0 to2 showed a significantly better success rate than those withstage 3 changes. In responders, repeat therapy for recurrenceof symptoms or treatment of a symptomatic corresponding symmetricaljoint is advisable. Repeat therapy in a previous non-responderis associated with an unacceptably high failure rate. Therefore,when a joint fails to respond after 6 months, arthroscopy shouldbe performed to evaluate further treatment procedures. A successfulresult was found in only 11 of 25 joints treated with arthroscopicsynovectomy followed by radiation synovectomy within 2 weeks,indicating no benefit of this combination. KEY WORDS: Radiosynoviorthesis, Rheumatoid arthritis, Osteoarthritis, Psoriatic arthritis, Ankylosing spondylitis     

Cervical involvement in juvenile-onset ankylosing spondylitis with bone scintigraphy

Juvenile-onset ankylosing spondylitis is an unusual disorder which can present with either peripheral arthritis or more classic hip girdle and back symptoms. A 12-year-old child with this disease was admitted with walking disorder, cervical pain, restricted cervical motion, and right ankle swelling. Diffusely increased accumulation of radioactivity in the cervical spine, focally increased accumulation in bilateral sacroiliac joints, and diminished irregular uptake in thoracal spine were detected on technetium 99m methylene diphosphonate bone scintigraphy. As a result, this imaging technique may give important information for diagnosis and differential diagnosis in juvenile chronic arthritis.     

Echocardiographic abnormalities in ankylosing spondylitis.

Twenty four patients with ankylosing spondylitis of 10 or more years' duration were assessed for evidence of cardiac disease. Seven patients (29%) had evidence of cardiac disease, including one patient with a pericardial effusion, three with conduction abnormalities, and two with aortic incompetence. Aortic incompetence in one patient was clinically silent and was detected only with Doppler echocardiography. This patient had, in addition, thickening of the posterior aortic wall, an echocardiographic feature not previously described in ankylosing spondylitis. There was no evidence of aortic valve disease in a control group matched for age and sex. Patients with ankylosing spondylitis and cardiac abnormalities were older, had a longer disease duration, and more peripheral joint disease than those without cardiac abnormalities. Doppler echocardiography is a useful technique in the assessment of cardiac disease in ankylosing spondylitis and may detect aortic valve disease at an early preclinical stage.     

A longitudinal study on an autoimmune murine model of ankylosing spondylitis

BACKGROUND: Proteoglycan aggrecan (PG)-induced arthritis (PGIA) is the only systemic autoimmune murine model which affects the axial skeleton, but no studies have been performed characterising the progression of spine involvement. OBJECTIVES: To follow pathological events in experimental spondylitis, and underline its clinical, radiographic, and histological similarities to human ankylosing spondylitis (AS); and to determine whether the spondyloarthropathy is a shared phenomenon with PGIA, or an "independent" disease. METHODS: Arthritis/spondylitis susceptible BALB/c and resistant DBA/2 mice, and their F1 and F2 hybrids were immunised with cartilage PG, and radiographic and histological studies were performed before onset and weekly during the progression of spondylitis. RESULTS: About 70% of the PG immunised BALB/c mice develop spondyloarthropathy (proteoglycan-induced spondylitis (PGISp), and the progression of the disease is very similar to human AS. It begins with inflammation in the sacroiliac joints and with enthesitis, and then progresses upwards, affecting multiple intervertebral disks. In F2 hybrids of arthritis/spondylitis susceptible BALB/c and resistant DBA/2 mice the incidence of arthritis was 43.5%, whereas the incidence of spondylitis was >60%. Some arthritic F2 hybrid mice had no spondylitis, whereas others developed spondylitis in the absence of peripheral arthritis. CONCLUSIONS: The PGISp model provides a valuable tool for studying autoimmune reactions in spondylitis, and identifying genetic loci associated with spondyloarthropathy.     

Biologic therapies in the spondyloarthritis: new opportunities,new challenges

Therapeutic options for patients suffering from the more severe forms of spondyloarthritis have been rather limited in the last decades. There is now accumulating evidence that antitumor necrosis factor therapy is highly effective in spondyloarthritis, especially in ankylosing spondylitis and psoriatic arthritis. Based on the data recently published on more than 500 patients with ankylosing spondylitis and psoriatic arthritis, this treatment seems to be even more effective than in rheumatoid arthritis. The antitumor necrosis factor-alpha agents currently available, infliximab (Remicade), etanercept (Enbrel), and adalimumab (Humira), are approved for the treatment of rheumatoid arthritis in the United States and partly in Europe. The situation in spondyloarthritis is different from that of rheumatoid arthritis because there is an unmet medical need, especially in ankylosing spondylitis: no therapies with disease-modifying antirheumatic drugs are available for severely affected patients, especially with spinal disease. Thus, tumor necrosis factor blockers may even be considered a first-line treatment in a patient with active ankylosing spondylitis and psoriatic arthritis whose condition is not sufficiently controlled with nonsteroidal antiinflammatory drugs in the case of axial disease, and sulfasalazine or methotrexate in the case of peripheral arthritis. For infliximab, a dose of 5 mg/kg was required, and intervals between 6 and 12 weeks were necessary to suppress disease activity constantly-also a major aim for long-term treatment. The standard dosage of etanercept is 2 x 25 mg subcutaneously per week. There are no studies yet on adalimumab (standard rheumatoid arthritis dose, 20-40 mg subcutaneously every 1-2 weeks) in spondyloarthritis. Infliximab was very recently approved for AS in Europe. The efficacy of etanercept was first demonstrated in psoriatic arthritis, and it is now approved for this indication. A double-blind study has also been performed in ankylosing spondylitis, with similarly clear efficacy. There is preliminary evidence that both agents do also work in other spondyloarthritis, such as undifferentiated spondyloarthritis. Ideally, both agents will be approved soon for the short-term treatment of severe, uncontrolled spondyloarthritis. In parallel, studies should be performed to document the long-term efficacy of this treatment. There is hope that ankylosis may be preventable, but it remains to be shown whether patients benefit from long-term antitumor necrosis factor therapy and whether radiologic progression and ankylosis can be stopped. Severe adverse events have remained rare. Complicated infections including tuberculosis have been reported. These can be largely prevented by appropriate screening. At it stands now, the benefits of antitumor necrosis factor therapy in ankylosing spondylitis seem to outweigh these shortcomings.     

New therapeutic approaches for spondyloarthritis

PURPOSE OF REVIEW: Tumor necrosis factor alpha antagonists are effective for signs and symptoms of ankylosing spondylitis. Recent studies have evaluated the efficacy of these agents for structural disease modification. We critically review recent radiographic data suggesting that tumor necrosis factor alpha inhibition may have structure-modifying effects in ankylosing spondylitis, and may thereby alter the disease course. RECENT FINDINGS: Recent studies employing MRI suggest that therapy with tumor necrosis factor alpha antagonists significantly reduces spinal inflammation in active ankylosing spondylitis when compared to placebo; there was no comparable improvement in the severity of chronic stigmata, such as syndesmyophytes and vertebral bridging. These studies were of relatively short duration and small size. SUMMARY: Despite insufficient evidence to conclude definitively that tumor necrosis factor alpha-antagonist therapy provides durable and effective structure modification in ankylosing spondylitis, the data strongly suggest a benefit, at least in the short term. In the future, MRI data coupled with clinical outcomes in larger cohorts followed for longer durations may result in a paradigm shift for ankylosing spondylitis treatment similar to that undergone for rheumatoid arthritis, where patients with ankylosing spondylitis are offered therapy early in the disease course to arrest and prevent structural disease progression.     

Effects of 1-year anti-TNF-α therapies on bone mineral density and bone biomarkers in rheumatoid arthritis and ankylosing spondylitis

Clinical Rheumatology - Rheumatoid arthritis (RA) and ankylosing spondylitis (AS) have been associated with generalized and localized bone loss. We conducted a comprehensive study using imaging...     

Autoimmune joint diseases in Late Medieval skeletal sample from Croatia

Analysis of 25 skeletons from Late Medieval cemetery Uzdolje-Grablje near Knin, Croatia, revealed three cases of systematic pathological changes to joints. Observed pathological lesions were examined macroscopically and radiologically and compared to the available paleopathological standards in order to formulate a differential diagnosis. In all three cases observed changes were most consistent with autoimmune joint diseases including ankylosing spondylitis, juvenile idiopathic arthritis and psoriatic arthritis. Based on published clinical studies, we suggest that the high prevalence of autoimmune diseases in our skeletal sample stems from the genetic basis of the autoimmunity, and that three individuals describe here are possibly closely related.     

AXIAL SKELETAL CHANGES IN PARALYSED PATIENTS MAY MIMIC ANKYLOSING SPONDYLITIS

Patients with paralysis may develop radiographic changes inthe axial skeleton and sacroiliac joints that resemble thoseseen in ankylosing spondylitis. These similarities can resultin confusion when evaluating paralysed patients with back pain.We report on a patient with paralysis secondary to amyotrophiclateral sclerosis who developed back pain, apparent sacroiliacjoint fusion, and a ‘bamboo spine’, leading to themisdiagnosis of ankylosing spondylitis. Serial radiographs ofthe bony changes in our patient are presented, along with abrief review of the literature on axial skeletal abnormalitiesin paralysis and a discussion of the subtle changes that distinguishimmobilization spondyloarthropathy from ankylosing spondylitis. KEY WORDS: Paralysis, Ankylosing spondylitis, Sacroiliitis