Kyong-Hee Jung

Digital thermography of the fingers and toes in Raynaud's phenomenon.

The aim of this study was to determine whether skin temperature measurement by digital thermography on hands and feet is useful for diagnosis of Raynauds phenomenon (RP). Fifty-seven patients with RP (primary RP, n = 33; secondary RP, n = 24) and 146 healthy volunteers were recruited. After acclimation to room temperature for 30 min, thermal imaging of palmar aspect of hands and dorsal aspect of feet were taken. Temperature differences between palm (center) and the coolest finger and temperature differences between foot dorsum (center) and first toe significantly differed between patients and controls. The area under curve analysis showed that temperature difference of the coolest finger (cutoff value: 2.2℃) differentiated RP patients from controls (sensitivity/specificity: 67/60%, respectively). Temperature differences of first toe (cutoff value: 3.11℃) also discriminated RP patients (sensitivity/specificity: about 73/66%, respectively). A combination of thermographic assessment of the coolest finger and first toe was highly effective in men (sensitivity/specificity : about 88/60%, respectively) while thermographic assessment of first toe was solely sufficient for women (sensitivity/specificity: about 74/68%, respectively). Thermographic assessment of the coolest finger and first toe is useful for diagnosing RP. In women, thermography of first toe is highly recommended. Graphical Abstract

Associations of Vitamin D Binding Protein Gene Polymorphisms with the Development of Peripheral Arthritis and Uveitis in Ankylosing Spondylitis

Objective. Genetic factors account for more than 90% of overall susceptibility to ankylosing spondylitis (AS), and recent studies have focused on non-major histocompatibility complex genes. Vitamin D binding protein (DBP) is a highly polymorphic protein that transports vitamin D and its metabolites. In addition to its sterol binding capacity, DBP has many other roles in the inflammatory and immune systems, and has been reported to be associated with autoimmune diseases. We investigated the association between DBP polymorphisms and susceptibility to AS. Methods. This case-control study was conducted in 223 patients with AS and 239 ethnically matched controls who were genotyped for 8 single-nucleotide polymorphisms (SNP) in the DBP and its promoter. Genomic DNA was isolated from peripheral blood leukocytes using the standard phenolchloroform method, and the GoldenGate assay was used for genotyping. Results. No significant association was found between the susceptibility to AS and DBP polymorphisms. In a subgroup analysis of patients with AS, G alleles at rs222016 and rs222020 (OR 0.63, 95% CI 0.42–0.95, p = 0.03; OR 0.63, 95% CI 0.42–0.95, p = 0.03, respectively) and A allele at rs3733359 (OR 0.59, 95% CI 0.39–0.90, p = 0.01) showed the decreased risk of peripheral arthritis. G allele at rs4752 showed increased risk of uveitis (OR 2.04, 95% CI 1.12–3.72, p = 0.02). On the haplotype analyses, haplotype 2 (AGGA) protected against the development of peripheral arthritis (p = 0.01) and haplotype 3 (GAAG) was associated with an increased likelihood of uveitis (p = 0.02). Conclusion. DBP gene polymorphisms are associated with the development of peripheral arthritis and uveitis in Korean patients with AS. Given the influence of different DBP variants on the immune system, larger-scale studies are warranted to elucidate the role of DBP in the pathogenesis of AS.

Natural Killer Cell Cytolytic Function in Korean Patients with Adult-onset Still’s Disease

Objective. To investigate natural killer (NK) cell proportions, NK cell cytotoxicity, and interleukin 18 (IL-18) expression, in patients with adult-onset Still’s disease (AOSD). Methods. Forty-five patients with AOSD (active = 22, inactive = 23) and 32 healthy controls were included. The proportions of NK cells among peripheral blood mononuclear cells were assessed by flow cytometry. IL-18 and IL-18-binding protein (IL-18BP) concentrations were measured by ELISA. Twenty-four patients with AOSD and 18 controls were examined for cytotoxic activity of NK cells by co-incubating NK cells with NK-sensitive K562 cells. The association of NK cell function with clinical and laboratory measures was investigated. Results. The proportions of NK cells were significantly lower in patients with active AOSD than in patients with inactive disease and controls. NK cell cytotoxic function was significantly lower in patients with AOSD than in controls. NK cell proportions and cytotoxic functions were reexamined in 11 and 6 patients, respectively, after treatment. Low NK cell proportion and cytotoxic dysfunction were improved with clinical improvements of the patients. IL-18 and IL-18BP levels were much higher in patients with active AOSD than in controls. NK cell cytotoxic functions were consistently low and IL-18 and IL-18BP levels were constantly high in patients with AOSD, regardless of disease activity. Conclusion. Low NK cell proportion, defective cytotoxic function, and elevated IL-18 levels may be significant features of AOSD. After resolution of the acute phase, low NK cell proportion was recovered and NK cell cytolytic function was restored along with clinical improvement. These findings possibly contribute to immunologic abnormalities in AOSD.

Safety of Resuming Tumor Necrosis Factor Inhibitors in Ankylosing Spondylitis Patients Concomitant with the Treatment of Active Tuberculosis: A Retrospective Nationwide Registry of the Korean Society of Spondyloarthritis Research.

Backgrounds Patients who develop an active tuberculosis infection during tumor necrosis factor (TNF) inhibitor treatment typically discontinue TNF inhibitor and receive standard anti-tuberculosis treatment. However, there is currently insufficient information on patient outcomes following resumption of TNF inhibitor treatment during ongoing anti- tuberculosis treatment. Our study was designed to investigate the safety of resuming TNF inhibitors in ankylosing spondylitis (AS) patients who developed tuberculosis as a complication of the use of TNF inhibitors. Methods Through the nationwide registry of the Korean Society of Spondyloarthritis Research, 3929 AS patients who were prescribed TNF inhibitors were recruited between June 2003 and June 2014 at fourteen referral hospitals. Clinical information was analyzed about the patients who experienced tuberculosis after exposure to TNF inhibitors. The clinical features of resumers and non-resumers of TNF inhibitors were compared and the outcomes of tuberculosis were surveyed individually. Findings Fifty-six AS patients were treated for tuberculosis associated with TNF inhibitors. Among them, 23 patients resumed TNF inhibitors, and these patients were found to be exposed to TNF inhibitors for a longer period of time and experienced more frequent disease flare-up after discontinuation of TNF inhibitors compared with those who did not resume. Fifteen patients resumed TNF inhibitors during anti-tuberculosis treatment (early resumers) and 8 after completion of anti-tuberculosis treatment (late resumers). Median time to resuming TNF inhibitor from tuberculosis was 3.3 and 9.0 months in the early and late resumers, respectively. Tuberculosis was treated successfully in all resumers and did not relapse in any of them during follow-up (median 33.8 [IQR; 20.8–66.7] months). Conclusions Instances of tuberculosis were treated successfully in our AS patients, even when given concomitantly with TNF inhibitors. We suggest that early resumption of TNF inhibitors in AS patients could be safe under effective coverage of tuberculosis.

Improvement of vitiligo in a patient with rheumatoid arthritis after hydroxychloroquine treatment.

Dear Editor, Antimalarial agents are one of the most commonly prescribed drugs for the treatment of autoimmune diseases, including rheumatoid arthritis (RA). Hydroxychloroquine (HCQ) has long-term treatment benefits in RA. Recent data suggest that HCQ may have more wide-reaching medical implications, including diabetes mellitus, dyslipidemias, infectious diseases, coagulopathies and malignancies, but HCQ has also been reported to cause pigmentary changes in approximately 10–25% of patients. Here, we present a case of vitiligo in a patient with RA that improved following HCQ treatment. A 39-year-old woman presented in December 2010 with a 3-months history of multiple joint pain. Physical examination showed tenderness and swelling in several joints. Laboratory findings were positive for anti-cyclic citrullinated peptide antibody and anti-Ro antibody, but negative for rheumatoid factor and anti-nuclear antibody. Erythrocyte sedimentation rate and C-reactive protein level were elevated to 35 mm/h and 0.64 mg/ dL, respectively. Serum thyroid-stimulating hormone and free T4 levels were normal. Bone scintigraphy showed increased uptake in both the wrists and small joints of the right hand. Chest computed tomography showed mild ground-glass opacity in the peribronchovascular and subpleural areas. She was diagnosed with seropositive RA and interstitial lung disease. The patient had a 20-year history of vitiligo on the face, trunk and extremities. Before RA diagnosis, she was treated with ultraviolet light (UV) for 6 months and experienced improvement of facial vitiligo. She reported no change in other areas of her body. In May 2011, HCQ (200 mg daily) and methotrexate (MTX) (7.5 mg weekly) were prescribed for the treatment of her RA. Glucocorticoids were not prescribed. HCQ was discontinued from September 2011 to November 2011 because of the patient’s wish due to improvement in her arthritis. The patient revisited the rheumatology clinic in December 2011 with worsening

Angioimmunoblastic T cell lymphoma in an ankylosing spondylitis patient treated with etanercept.

Angioimmunoblastic T cell lymphoma (AITL) is a rare non-Hodgkin lymphoma that presents with profound immune dysfunction and immunodeficiency. The clinical and laboratory findings associated with AITL are similar to those of rheumatic disease, and AITL has been reported to be concurrent in patients with several rheumatic diseases. We present one case of AITL occurring in a patient with ankylosing spondylitis (AS) after treatment with etanercept. Constitutional symptoms and aggravation of peripheral arthritis in elderly AS patients may be due not only to flare-ups of AS but also to other complicating diseases, such as lymphoma. Although the occurrence of lymphoma in AS patients treated with etanercept has only rarely been reported, clinicians should keep in mind that instances of aggravation of peripheral arthritis in elderly AS patients occurring after immunosuppressant treatment may be due to other complicating systemic diseases such as AITL, rather than the rheumatic disease itself. Further study is needed in order to investigate whether or not using a TNF-α blocker such as etanercept increases the risk of lymphoma, especially for cases associated with Epstein–Barr virus.

Palisaded Neutrophilic Granulomatous Dermatitis in a Patient with Systemic Sclerosis-Rheumatoid Arthritis Overlap Syndrome

804 Ann Dermatol Received September 9, 2016, Revised October 22, 2016, Accepted for publication October 31, 2016 Corresponding author: Won Park, Division of Rheumatology, Department of Internal Medicine, Inha University, 366 Seohae-daero, Jung-gu, Incheon 22332, Korea. Tel: 82-32-890-3483, Fax: 82-32-8902237, E-mail: parkwon@inha.ac.kr This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Copyright

A case of migrating bone marrow edema syndrome in a patient with Behcet's disease.

Dear Editor, Bone marrow edema syndrome (BMES) refers to a transient clinical condition of unknown pathogenicity. In general, the course is self-limiting and symptoms resolve spontaneously over 6–12 months. Because of the lack of a specific diagnostic test and similar symptoms and imaging findings to those of other entities, differentiation of BMES from avascular necrosis (AVN), neoplasm and osteomyelitis is difficult. Early diagnosis is important for preventing overtreatment of benign conditions and for starting proper management in serious cases. Here, we present the first case of migrating BMES within the same foot in a patient with Behcet’s disease (BD). A 38-year-old woman with BD visited our clinic in February 2013 with a 10-day history of left ankle pain. She reported severe, progressive pain and tenderness in the left ankle. She had no history of a traumatic event. In 2009, she had been diagnosed with BD on the basis of findings of recurrent oral aphthous ulcers, genital aphthous ulcers, erythema nodosum and arthritis. She had been prescribed colchicine 0.6 mg daily, sulfasalazine 500 mg daily, and acetaminophen 1300 mg daily for 4 years. However, her drug compliance was poor, and she complained constantly of recurrent oral and genital ulcers. She took steroids only when the oral and genital ulcers were aggravated. She had a history of thyroid papillary carcinoma and had undergone a total thyroidectomy and radioiodine ablation 6 years before admission. She reported that she had been hospitalized for similar left ankle pain in November 2003 and July 2004. The magnetic resonance imaging (MRI) images obtained in 2003 demonstrated diffuse high signal intensity on T2-weighted images of the lateral portion of the calcaneus (Fig. 1a,b). Under consideration of a diagnosis of osteomyelitis, she had been treated with antibiotics for 50 days. The pain had subsided after 1 month. In 2004, the left foot and ankle pain developed again, and MRI revealed newly developed bone marrow edema at the talus and distal tibia, and the size of the previous lesion in the calcaneus had markedly decreased (Fig. 1c,d). Her doctor had suspected a recurrence of osteomyelitis and recommended a surgical (a) (b)

SAT0176 Smoking increases systemic lupus erythematosus susceptibility in women carrying the HLA-DRB1 risk alleles

Background While smoking exposure has been causally linked to rheumatoid arthritis, the relationship between smoking and the development of systemic lupus erythematosus (SLE) is less clear. The major studies in patients with SLE included did not have statistically significant associations and one outlying study increased the risk for SLE in a predominately Hispanic population. Objectives We investigated whether HLA-DRB1 risk alleles, smoking, or the combination contribute to the development of SLE and whether smoking influences the production of autoantibodies in a Korean population. Methods A total of 877 women from BAE SLE cohort, who fulfilled the American College of Rheumatology 1997 revised criteria for SLE, and 976 healthy women were recruited in a Korean population. Four-digit HLA-DRB1 typing was performed by a conventional PCR-SBT method. Information about smoking history was obtained through a questionnaire by face-to-face interviews. Odds ratios (OR) with a 95% confidence interval (CI) were calculated. Results The DRB1*1501 and *0803 alleles were associated with SLE susceptibility, using the relative predispositional effects according to controlling the Bonferroni method [OR 1.82 (95% CI: 1.44-2.03), p=4.45

A case of tracheo-bronchial amyloidosis.

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