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Spondyloarthropathies: Editorial overview
Abstract
: Current Opinion in Rheumatology 1993, 5:405-407
(C) Lippincott-Raven Publishers.
... Spondyloarthritis (SpA) is a group of diseases characterized by chronic inflammation in the axial joints (the sacroiliac and spinal joints), as well as the peripheral joints, associated with human leukocyte antigen (HLA)-B27 and lack of autoantibodies such as rheumatoid factor (RF) and antinuclear antibody (ANA) [1,2]. The prevalence of HLA-B27 largely differs among racial and ethnic populations [3] and is extremely low (0.3%) in the Japanese population [4], although a weaker association with HLA-B39 and other major histocompatibility complex regions may also be involved [5,6]. Accordingly, the prevalence of SpA, especially that of AS, which shows the strongest association with HLA-B27 (70-90%) [7,8], is much lower in Japan than in most other countries [9, 10•]. ...
... The prevalence of AS and nr-axSpA has been estimated to be 2.6/100,000 (0.0026%) and 0.6/100,000 (0.0006%), respectively [17••], in Japan, where HLA-B27 positivity in the general population is extremely low (0.3%) [3]. AS and IBD, but not PsA and nr-axSpA, are designated intractable diseases in Japan for which patient medical expenses are reduced by grants under the Act on Medical Care for Patients with Intractable Diseases. ...
Purpose of Review
The differences in the epidemiology and management of patients with axial spondyloarthritis (axSpA) among regions and countries largely depend on the positivity of human leukocyte antigen (HLA)-B27 and the health care system. This review article focused on axSpA in Japan, where the prevalence of HLA-B27 is extremely low (0.3%) and the universal health insurance system typically provides a 70% or more copayment by the government.
Recent Findings
A nationwide survey was conducted in Japan in 2018, which estimated that there were 3200 patients (95% confidence interval [CI]: 2400–3900) with ankylosing spondylitis (AS), a term interchangeable with radiographic axSpA (r-axSpA), and 800 patients (95% CI: 530–1100) had non-radiographic (nr)-axSpA. These data indicate a prevalence of 2.6/100,000 or 0.0026% for AS and 0.6/100,000 or 0.0006% for nr-axSpA. Patients with AS, but not those with nr-axSpA, are designated as suffering from intractable diseases in Japan; thus, their medical expenses are reduced by grant under the Act on Medical Care for Patients with Intractable Diseases. As of February 2022, infliximab, adalimumab, secukinumab, ixekizumab, and brodalumab have been approved for AS, and secukinumab, ixekizumab, and brodalumab have been approved for nr-axSpA. An algorithm for nr-axSpA in Japan has been developed for the proper diagnosis and use of these therapeutic agents.
Summary
A low prevalence of axSpA, especially that of nr-axSpA, was found in Japan. Early referral and the resultant diagnosis and appropriate treatment of these patients by rheumatologists are crucial issues in Japan, as in other countries.
Objective. To investigate the characteristics of HLA-B27 that render susceptibility to seronegative spondylarthropathies.
Methods. Serologic HLA class I typing of Japanese patients with ankylosing spondylitis (AS), juvenile rheumatoid arthritis (JRA), and healthy controls, was performed. HLA-B39 subtypes were determined by polymerase chain reaction-sequence-specific oligohy-bridization.
Results. HLA-B27 was present in 40 of 48 patients with AS (83%), and in only 1 of 210 healthy controls (0.5%). Three of 8 patients (37.5%) who were negative for HLA-B27 were positive for HLA-B39, which was significantly higher compared with the HLA-B27-negative controls (6.2% P = 0.01). Significant association with HLA-B39 was also noted in the JRA patients (16.7%; P < 0.01), especially in those patients with pauciarticular-onset disease (33.3%; P < 0.01). Ten of 13 HLA-B39-positive patients had subtype B*3901 and 3 had B*3902.
Conclusion. Because HLA-B27 and HLA-B39 share Glu at position 45 and Cys at position 67, both of which constitute components of the peptide-anchoring B pocket, and because they possess similar peptide-ligand motifs, our results may support either the role of the peptides presented by class I antigens or the importance of Cys at position 67, in the development of spondylarthropathies and pauciarticular-onset JRA.
Sacroiliitis is an indispensable condition for the diagnosis of ankylosing spondylitis according to the present criteria and is usually diagnosed on standard anteroposterior radiographs of the pelvis. In cases with suspicious abnormalities (grade 1 of the New York criteria) CT permits the diagnosis since it shows a higher degree of sacroiliitis. MRI is superior to CT in the early diagnosis as it can detect the cartilage abnormalities which precede bony changes. 'Romanus lesions' with 'shiny corners', 'squaring' of the vertebral bodies, syndesmophytes, spondylodiscitis and osteoporosis are the radiological findings of ankylosing spondylitis. The nonmarginal, asymmetric, coarse and broad syndesmophytes of psoriatic spondylitis and spondylitis associated with Reiter's syndrome resemble the flowing bone outgrowths of diffuse idiopathic skeletal hyperostosis (DISH). The ossification of the posterior longitudinal ligament and of the flavum ligament are rare manifestations of ankylosing spondylitis. Peripheral extra-articular enthesitis is a clinical hallmark of seronegative spondylarthropathies. Plain film radiography shows erosions and spurs but only in advanced phases. US shows the swelling of the entheses and the peritendinous soft tissues and the distension of adjacent bursae by fluid collection. MRI shows the inflammation of the bone adjacent to the insertion as well as the soft tissue changes. Dactylitis is another typical manifestation of seronegative spondylarthropathies. In the past it was thought to be due to concomitant tenosynovitis and arthritis. Recently, however, we have demonstrated with US and MRI that the 'sausage-like' appearance is due to the flexor tenosynovitis and that joint capsule distension is not indispensable.
To investigate features of chronic urogenic arthritis (CUA) and its differences with psoriatic arthritis (PA) and ankylosing spondylarthritis (AS) with joint lesion.
CUA, AS, PA were diagnosed according to S. M. Sidelnikova et al., S. van der Linden and Agababova, respectively, in 94 patients. The disease ran for more than 3 years. Articular syndrome was examined in CUA, AS and PA.
Articular syndrome in CUA remains for the most part monoarticular with affection of the joints of the low extremities. AS has clinical and x-ray signs of sacroileitis, affection of the spinal column and hip joints. PA runs with multiple arthritic lesions of the hand and foot joints.
Chronic infection persists in all the CUA and PA patients. Its exacerbation coincides with arthritis aggravations. Only in CUA there is a chronological connection between acute or aggravated chronic urogenital infection and initial symptoms of joint disease.
To investigate the relative contribution of MHC loci in their susceptibility to primary ankylosing spondylitis (AS) in HLA-B27 negative patients and to compare the clinical features and genetic factors with those of HLA-B27 positive AS.
DNA from patients with B27 negative primary AS (n = 28), B27 positive primary AS (n = 77), and matched healthy controls (B27-, n = 100; B27+, n = 70) were analyzed to investigate whether HLA genes determine the disease susceptibility, or whether other closely linked loci might play a role in disease development. HLA typing was carried out by serology and PCR/SSP (HLA-B, -DR), MICA-TM polymorphism in the transmembrane region by radioactive PCR, and tumor necrosis factor-alpha (TNF-alpha) promoter polymorphism at positions -238 and -308 by PCR-RFLP.
Subtle clinical differences were found for primary AS, the B27 negative patients being less frequently complicated by acute anterior uveitis and more associated with peripheral arthritis than B27 positive. Differences were found in the distribution of TNF-alpha -238 genotypes among patients with primary AS (B27- vs B27+). The TNF-alpha -238(A) polymorphism was present in 50% of the B27 negative patients carrying the -238 G/A and A/A genotypes and was significantly increased compared with B27 positive AS (odds ratio 4.3) and with the B27 negative control group (OR 5.9). The TNF-alpha genotypes were equally prevalent in B27 positive AS and healthy matched B27 positive controls. No significant HLA and MICA typing differences were found between the populations under study.
Our results indicate that the polymorphism variation in the TNF-alpha promoter -238.2(A) influences disease susceptibility in B27 negative primary AS but had no effect in our B27 positive AS population.
Psoriatic arthritis was described as a distinct rheumatic disease in the 1960s, and subsequently grouped among the spondyloarthropathies. Recently, other rheumatic manifestations of psoriasis, such as enthesopathy and osteoperiostitis, were recognized. This study attempts to examine the rheumatological and radiological manifestations of Psoriasis and their association with skin and nail disease. Eighty-one psoriatic outpatients were interviewed consecutively during 6 months. Questionnaires and indices were carried out to assess the extent and severity of skin and nail involvement, as well as the activity and severity of peripheral and axial rheumatic manifestations. Radiological examination of the hands, feet, spine and pelvis was also done for all patients. Fifty-nine psoriatic outpatients (73%) had rheumatic manifestations clinically and/or radiologically (Psoriatic arthropathy “PsA”). Clinical peripheral arthritis was found in 14 (23.7%) of the patients with PsA, being oligoarticular in 11, polyarticular in two, and exclusively of the distal interphalangeal (DIP) joints in one patient. Sacroiliitis and/or spondylitis were found in 38 (64.4%), enthesopathy in 36 (61%), dactylitis in two (3.3%), radiological DIP involvement in 24 (40.6%), and radiological osteoperiostitis in 49 (83%) of patients with PsA. Most PsA patients had more than one rheumatic manifestation, while four patients (6.7%) had isolated enthesopathy without any other rheumatic manifestations. Subungual hyperkeratosis of the nails was significantly correlated with PsA (pp
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