OBJECTIVE: We examined the clinical relationship between human leukocyte antigen B27 (HLA-B27) and juvenile idiopathic arthritis (JIA). Additionally, we assessed the usefulness of the Assessment of SpondyloArthritis International Society (ASAS) criteria for diagnosing juvenile spondyloarthropathies (SpA). METHODS: We retrospectively reviewed medical records of 239 patients with JIA classified according to the International League of Associations for Rheumatology (ILAR) classification to analyze the features of the joint involvement site. Results were correlated with the presence of HLA-B27. After that, we classified the 239 JIA patients according to the ASAS criteria to diagnose juvenile SpA. The relationship between the ASAS criteria and a diagnosis of juvenile SpA was analyzed by a chi-squared test. RESULTS: Back pain was associated with HLA-B27 in boys (p=0.002) but not in girls (p=0.616). In both sexes, involvement of the small joints in the lower extremities was highly associated with HLA-B27 (p=0.001 for boys, p=0.021 for girls). In addition, HLA-B27 was associated with enthesitis (p=0.004 for boys, p=0.021 for girls). Eighty-seven (36.4%) patients with JIA fulfilled the ASAS criteria; 2 (0.8%) had axial SpA and 85 (35.6%) had peripheral SpA. HLA-B27 was the most significant factor for diagnosing juvenile SpA (sensitivity 80%, specificity 99.31%, positive likelihood ratio, 116). CONCLUSION: The ILAR criteria have some weaknesses for diagnosing HLA-B27-positive JIA patients in early stages. The use of the ASAS criteria for juvenile patients will enable pediatric rheumatologists to diagnose juvenile SpA patients earlier.
Arthritis, Juvenile* ; Back Pain ; Classification* ; Diagnosis ; Female ; HLA-B27 Antigen ; Humans* ; Joints ; Leukocytes* ; Lower Extremity ; Medical Records ; Retrospective Studies ; Rheumatology ; Sensitivity and Specificity ; Spondylarthropathies* ; Spondylitis, Ankylosing

SAPHO syndrome, characterized by synovitis, acne, pustulosis, hyperostosis, and osteitis is rare compared to other spondyloarthropathies. It is also difficult to diagnose, and treatment methods have not yet been fully identified. Approximately 72% of patients are diagnosed with at least one other disease before a final diagnosis of SAPHO syndrome. In addition, SAPHO syndrome is subject to a delayed diagnosis period of 4.5 to 9.1 years. Medications such as non-steroidal anti-inflammatory drugs, disease-modifying anti-rheumatic drugs, and tumor necrosis factor inhibitors are used in treatment of SAPHO syndrome. Bisphosphonate is also used for refractory SAPHO syndrome; however, most reports on this relate to intravenous injection of medication. The authors experienced and subsequently reported on a case involving a patient with SAPHO syndrome accompanied by fracture and infection of the left second finger who was treated with the oral biphosphonate, alendronate.
Acne Vulgaris ; Acquired Hyperostosis Syndrome* ; Alendronate* ; Antirheumatic Agents ; Delayed Diagnosis ; Diagnosis ; Fingers ; Humans ; Hyperostosis ; Injections, Intravenous ; Osteitis ; Spondylarthropathies ; Synovitis ; Tumor Necrosis Factor-alpha

BACKGROUND: Flow cytometry (FC) HLA-B27 typing is still used extensively for the diagnosis of spondyloarthropathies. If patient blood samples are stored for a prolonged duration, this testing can be performed in a batch manner, and in-house cellular controls could easily be procured. In this study, we investigated various methods of storing patient blood samples. METHODS: We compared four storage methods: three methods of analyzing lymphocytes (whole blood stored at room temperature, frozen mononuclear cells, and frozen white blood cells [WBCs] after lysing red blood cells [RBCs]), and one method using frozen platelets (FPLT). We used three ratios associated with mean fluorescence intensities (MFI) for HLAB27 assignment: the B27 MFI ratio (sample/control) for HLA-B27 fluorescein-5-isothiocyanate (FITC); the B7 MFI ratio for HLA-B7 phycoerythrin (PE); and the ratio of these two ratios, B7/B27 ratio. RESULTS: Comparing the B27 MFI ratios of each storage method for the HLA-B27+ samples and the B7/B27 ratios for the HLA-B7+ samples revealed that FPLT was the best of the four methods. FPLT had a sensitivity of 100% and a specificity of 99.3% for HLA-B27 assignment in DNA-typed samples (N=164) when the two criteria, namely, B27 MFI ratio >4.0 and B7/B27 ratio <1.5, were used. CONCLUSIONS: The FPLT method was found to offer a simple, economical, and accurate method of FC HLA-B27 typing by using stored patient samples. If stored samples are used, this method has the potential to replace the standard FC typing method when used in combination with a complementary DNA-based method.
Blood Platelets/metabolism ; Erythrocytes/metabolism ; *Flow Cytometry ; Freezing ; HLA-B27 Antigen/*blood ; HLA-B7 Antigen/blood ; Histocompatibility Testing ; Humans ; Leukocytes, Mononuclear/metabolism ; Real-Time Polymerase Chain Reaction ; Spondylarthropathies/diagnosis ; Temperature

BACKGROUND: Flow cytometry (FC) HLA-B27 typing is still used extensively for the diagnosis of spondyloarthropathies. If patient blood samples are stored for a prolonged duration, this testing can be performed in a batch manner, and in-house cellular controls could easily be procured. In this study, we investigated various methods of storing patient blood samples. METHODS: We compared four storage methods: three methods of analyzing lymphocytes (whole blood stored at room temperature, frozen mononuclear cells, and frozen white blood cells [WBCs] after lysing red blood cells [RBCs]), and one method using frozen platelets (FPLT). We used three ratios associated with mean fluorescence intensities (MFI) for HLAB27 assignment: the B27 MFI ratio (sample/control) for HLA-B27 fluorescein-5-isothiocyanate (FITC); the B7 MFI ratio for HLA-B7 phycoerythrin (PE); and the ratio of these two ratios, B7/B27 ratio. RESULTS: Comparing the B27 MFI ratios of each storage method for the HLA-B27+ samples and the B7/B27 ratios for the HLA-B7+ samples revealed that FPLT was the best of the four methods. FPLT had a sensitivity of 100% and a specificity of 99.3% for HLA-B27 assignment in DNA-typed samples (N=164) when the two criteria, namely, B27 MFI ratio >4.0 and B7/B27 ratio <1.5, were used. CONCLUSIONS: The FPLT method was found to offer a simple, economical, and accurate method of FC HLA-B27 typing by using stored patient samples. If stored samples are used, this method has the potential to replace the standard FC typing method when used in combination with a complementary DNA-based method.
Blood Platelets/metabolism ; Erythrocytes/metabolism ; *Flow Cytometry ; Freezing ; HLA-B27 Antigen/*blood ; HLA-B7 Antigen/blood ; Histocompatibility Testing ; Humans ; Leukocytes, Mononuclear/metabolism ; Real-Time Polymerase Chain Reaction ; Spondylarthropathies/diagnosis ; Temperature

Psoriatic arthritis (PsA) is an autoimmune arthritis related to psoriasis and one of seronegative spondyloarthropathies. PsA provokes joint pain and morning stiffness more than 30 minutes, which is relieved by exercise. PsA usually affects distal small joints and exhibits asymmetry, which is one of the typical characteristics of PsA and gives clues to make a differential diagnosis between PsA and rheumatoid arthritis. Thirty to forty patients with PsA experience arthritis in one large joint or asymmetric multiple joints. Arthritis in distal joints and arthritis mutilans often develop concurrently and patterns of PsA change along with disease progression. Spondylitis is observed in 20-30% of PsA patients. In contrast to ankylosing spondylitis, spondylitis in PsA present with mild clinical symptoms despite radiological progression, inflammation limited to one spinal tract, cervical spine dominance, non-marginal syndesmophytosis. Enthesitis is also one of the typical characteristics of PsA and it frequently affects Achilles tendon, plantar fascia and tendons inserting pelvic bones. Tenosynovitis can develop accompanied by enthesitis. Typical dactylitis (sausage digit), pitting edema and nail lesions, including nail pits, onycholysis, hyperkeratosis and splinter hemorrhage, also contribute to a differential diagnosis of PsA. Anterior uveitis, SAPHO syndrome, amyloidosis and IgA nephropathy are well-known extra-articular manifestation of PsA. In 2006, a new classification-criterion for PsA was suggested by the CASPAR study. The CASPAR criteria included 5 categories with a certain number of points; 1) skin psoriasis, 2) nail lesions, 3) dactylitis, 4) negative RF and 5) bone formation around joints. The CASPAR criteria should be applied to PsA patients having at least one of three (peripheral arthritis, spondylitis and enthesitis).
Achilles Tendon ; Acquired Hyperostosis Syndrome ; Amyloidosis ; Arthralgia ; Arthritis ; Arthritis, Psoriatic ; Arthritis, Rheumatoid ; Diagnosis, Differential ; Disease Progression ; Edema ; Fascia ; Glomerulonephritis, IGA ; Hemorrhage ; Humans ; Inflammation ; Joints ; Nails ; Onycholysis ; Osteogenesis ; Pelvic Bones ; Psoriasis ; Skin ; Spine ; Spondylarthropathies ; Spondylitis ; Spondylitis, Ankylosing ; Tendons ; Tenosynovitis ; Uveitis, Anterior

Reiter's syndrome belongs to the family of spondyloarthropathies that usually present with a triad of arthritis, urethritis, and uveitis. The diagnostic criteria include clinical, radiological, and genetic findings, and the response to treatment. Nervous system involvement in Reiter's syndrome is extremely rare. We report here on a 36-year-old man who initially presented with progressive cervical myelopathy and was diagnosed as Reiter's syndrome 2 years later. The myelopathy was stable after treatment with methotrexate and sulfasalazine. This case suggests that Reiter's syndrome can present as progressive myelopathy and should be considered in the differential diagnosis of treatable myelopathies.
Adult ; Arthritis ; Arthritis, Reactive ; Diagnosis, Differential ; HLA-B27 Antigen ; Humans ; Methotrexate ; Nervous System ; Spinal Cord Diseases* ; Spondylarthropathies ; Sulfasalazine ; Urethritis ; Uveitis

OBJECTIVE: To investigate the diagnostic value of magnetic resonance imaging (MRI) in early detection of sacroiliitis, to identify risk factors of early sacroiliitis, and to propose a diagnostic algorithm for early ankylosing spondylitis (AS). METHOD: Twenty-nine consecutive patients with inflammatory back pain (IBP) and unclear sacroiliitis (unilateral grade 2> or=sacroiliitis in plain radiography (PR) based on modified New York criteria) were studied. Clinical features of spondyloarthropathy, HLA B27 positivity, and MR image set of the sacroiliac (SI) joints were obtained. Two radiologists interpreted MR images independently to diagnose definite sacroiliitis. An association between sacroiliitis in MRI and each clinical and laboratory feature was assessed with linear logistic regression analysis. Post-test probability was determined with sensitivity/specificity of clinical and laboratory features. RESULTS: MRI showed definite sacroiliitis in sixteen patients. The most frequently noted finding was erosion and high signal intensity lesion within the joint cavity in gadolinium enhanced T1-weighted images. Unilateral grade 2> or =sacroiliitis in PR was the only significant risk factor of definite sacroiliitis in MRI. When unclear sacroiliitis in PR, more than one clinical feature of spondyloarthropathy, and HLA B27 were found, probability of AS was 83% in a proposed diagnostic algorithm. CONCLUSION: MRI of the SI joints can detect sacroiliitis in more than half of patients with IBP and unclear sacroiliitis in PR. Unilateral grade 2> or =sacroiliitis in PR was a risk factor of definite sacroiliitis in MRI. A diagnostic algorithm for early detection of AS is proposed.
Back Pain ; Early Diagnosis* ; Gadolinium ; Humans ; Joints ; Logistic Models ; Magnetic Resonance Imaging* ; Radiography ; Risk Factors ; Sacroiliitis* ; Spondylarthropathies ; Spondylitis, Ankylosing

A 55-year-old-male patient had acute lower back pain and radiculopathy with high spiking fever, which indicated pyogenic discitis. Intravenous antibiotics were administered but a high spiking fever and elevated laboratory findings indicated an infection. Therefore, surgery was performed. However, histological diagnosis revealed tophaceous gout. A diagnosis of spinal gout should be considered when there are clinical presentations of acute back pain and fever, especially in patients with acute back pain and a prior history of hyperuricemia or gout.
Anti-Bacterial Agents ; Back Pain ; Diagnosis ; Discitis* ; Fever ; Gout ; Humans ; Hyperuricemia ; Low Back Pain ; Radiculopathy ; Spine* ; Spondylarthropathies*

We describe two cases of SAPHO syndrome with history of palmoplantar pustulosis and pain on the anterior chest wall and lower back area. The acronym SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome includes a group of disorders characterized by bony lesions commonly involving the anterior chest wall and associated with skin manifestations. The skeletal manifestation is characterized by the association of inflammation and hyperostotic change, in the form of sternocostoclavicular hyperostosis, spondyloarthropathy and chronic recurrent multifocal osteomyelitis. Common cutaneous lesions include palmoplantar pustulosis, pustulotic psoriasis, and severe forms of acne. The pathogenesis remains elusive, but a link with seronegative spondyloarthropathy is probable. To date, the treatment is empirical. Nonsteroidal anti-inflammatory drugs are the first choice, and other drugs including corticosteroid, disease modifying antirheumatic drugs, pamidronate, and infliximab have been tried with some therapeutic benefit. SAPHO syndrome is a condition in the differential diagnosis of infectious or tumorous conditions of the bone. Early and proper diagnosis is important to avoid unnecessary investigations or treatments.
Acne Vulgaris ; Acquired Hyperostosis Syndrome* ; Antirheumatic Agents ; Diagnosis ; Diagnosis, Differential ; Hyperostosis ; Hyperostosis, Sternocostoclavicular ; Inflammation ; Osteomyelitis ; Psoriasis ; Skin Manifestations ; Spine ; Spondylarthropathies ; Thoracic Wall ; Infliximab

We describe two cases of SAPHO syndrome with history of palmoplantar pustulosis and pain on the anterior chest wall and lower back area. The acronym SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome includes a group of disorders characterized by bony lesions commonly involving the anterior chest wall and associated with skin manifestations. The skeletal manifestation is characterized by the association of inflammation and hyperostotic change, in the form of sternocostoclavicular hyperostosis, spondyloarthropathy and chronic recurrent multifocal osteomyelitis. Common cutaneous lesions include palmoplantar pustulosis, pustulotic psoriasis, and severe forms of acne. The pathogenesis remains elusive, but a link with seronegative spondyloarthropathy is probable. To date, the treatment is empirical. Nonsteroidal anti-inflammatory drugs are the first choice, and other drugs including corticosteroid, disease modifying antirheumatic drugs, pamidronate, and infliximab have been tried with some therapeutic benefit. SAPHO syndrome is a condition in the differential diagnosis of infectious or tumorous conditions of the bone. Early and proper diagnosis is important to avoid unnecessary investigations or treatments.
Acne Vulgaris ; Acquired Hyperostosis Syndrome* ; Antirheumatic Agents ; Diagnosis ; Diagnosis, Differential ; Hyperostosis ; Hyperostosis, Sternocostoclavicular ; Inflammation ; Osteomyelitis ; Psoriasis ; Skin Manifestations ; Spine ; Spondylarthropathies ; Thoracic Wall ; Infliximab