Clinical phenotypes of Korean patients with Behcet disease according to gender, age at onset, and HLA-B51

BACKGROUND/AIMS: The clinical manifestations of Behcet disease (BD) have been reported to differ according to country, region, and race. Gender, onset age, and human leukocyte antigen (HLA)-B51 have also been known as the factors that influence the clinical features of BD. The aim of this study is to investigate the clinical phenotypes of Korean patients who visited the rheumatology clinic with BD with respect to gender, onset age, and HLA-B51. METHODS: Total 193 Korean patients (129 females and 64 males) fulfilling the international criteria for BD were retrospectively assessed. RESULTS: The mean age at disease onset and disease duration of the BD patients were 32.2 ± 11.1 and 14.2 ± 9.3 years, retrospectively. Females suffered more frequently from genital ulcers (90.7% vs. 75.0%, p 40 years) suffered from neurologic involvement (15.9% vs. 4.2%, p = 0.007) more frequently than those with early onset of BD. The patients with HLA-B51 showed earlier onset of disease than without HLA-B51 (28.3 ± 11.4 years vs. 33.8±11.6 years, p = 0.02) and the neurologic and gastrointestinal involvements were more frequent in the patients without HLA-B51 than with HLA-B51 (17.2% vs. 2.5%, p = 0.02 and 20.7% vs. 2.5%, p = 0.01, respectively). CONCLUSIONS: The clinical phenotypes in Korean patients with BD may be influenced by gender, onset age and HLA-B51.

Validation and reliability of a Behcet's Syndrome Activity Scale in Korea

BACKGROUND/AIMS: We prepared a cross-cultural adaptation of the Behcet's Syndrome Activity Scale (BSAS) and evaluated its reliability and validity in Korea. METHODS: Fifty patients with Behcet's disease (BD) who attended the Rheumatology Clinic of Gachon University Gil Medical Center were included in this study. The first BSAS questionnaire was administered at each clinic visit, and the second questionnaire was completed at home within 24 hours of the visit. A Behcet's Disease Current Activity Form (BDCAF) and a Behcet's Disease Quality of Life (BDQOL) form were also given to patients. The test-retest reliability was analyzed by intraclass correlation coefficients (ICC). To assess the validity, the total BSAS score was compared with the BDCAF score, the patient/physician global assessment, and the BDQOL by Spearman rank correlation. RESULTS: Twelve males and 38 females were enrolled. The mean age was 48.5 years and the mean disease duration was 6.7 years. Thirty-eight patients (76.0%) returned the questionnaire by mail. For the test-retest reliability, the two assessments were significantly correlated on all 10 items of the BSAS questionnaire (p < 0.05) and the total BSAS score (ICC, 0.925; p < 0.001). The total BSAS score was statistically correlated with the BDQOL, BDCAF, and patient/physician global assessment (p < 0.01). CONCLUSIONS: The Korean version of BSAS is a reliable and valid instrument to measure BD activity.

Behcet's Disease Current Activity Form as a Patient's Derived Measure

OBJECTIVE: This study measured the reliability of the Behcet's Disease Current Activity Form (BDCAF) questionnaire used as a patient self-report form. METHODS: A study was conducted among 63 patients with Behcet's disease who attended our rheumatology clinic. First, a physician administered a BDCAF questionnaire. Second, the patient completed a self-administered questionnaire at home within 24 hours of the visit. The test-retest reliability was analyzed using kappa tests. Kappa scores of >0.6 indicated good agreement. The BDCAF score was compared with the patient's/clinician's perception of disease activity and the Korean version of Behcet's Disease Quality of Life (BDQOL). RESULTS: The study included 17 males and 46 females. The mean age of participants was 47.7 years and the mean disease duration was 5.3 years at the first assessment. Fifty-three patients (84.1%) returned the questionnaires to us by mail. For test-retest reliability, good agreement was achieved with the items including headache, oral/genital ulceration, erythema, arthritis, and diarrhea with altered/frank blood per rectum; moderate agreement with skin pustules, arthralgia, and eye involvement; fair agreement with nausea/vomiting/abdominal pain, nervous system, and major vessel involvement. Significant associations were observed between BDCAF scores with the patient's/clinician's perception of disease activity and BDQOL (p<0.05). CONCLUSION: The BDCAF appears useful as a patient self-report instrument for assessment of disease status.

Chronic Tophaceous Gout in Multiple Spines: A Case Report and Literature Review

Gout typically affects the 1st metatarsophalangeal joint. Spinal gout is rarely reported. Moreover, involvement of extensive spines is remarkably unusual. We describe a case of a 76-year-old woman with gout involving cervical, thoracic, and lumbar spines and sacroiliac joint. She presented with fever, severe back pain, and polyarthralgia and had multiple tophi on both elbows and the left 2nd and 5th proximal interphalangeal (PIP) joints. Monosodium urate crystals were confirmed from tophi on the left 5th PIP joint by polarized optical microscopy. Magnetic resonance imaging and computed tomography showed joint space narrowing and bony erosions on cervical, thoracic, and lumbar spines, and sacroiliac joint. Fever, back pain, and polyarthralgia improved significantly with oral steroid therapy. Spinal gout can involve multiple spines and other joints and it can be improved by medical treatment only. It should be considered in patients with uncontrolled gout who have acute severe back pain.

Cross-cultural adaptation and validation of the Behcet's Disease Current Activity Form in Korea

BACKGROUND/AIMS: This study was undertaken to perform a cross-cultural adaptation of the Behcet's Disease Current Activity Form (BDCAF, version 2006) questionnaire to the Korean language and to evaluate its reliability and validity in a population of Korean patients with Behcet's disease (BD). METHODS: A cross-cultural study was conducted among patients with BD who attended our rheumatology clinic between November 2012 and March 2013. There were 11 males and 35 females in the group. The mean age of the participants was 48.5 years and the mean disease duration was 6.4 years. The first BDCAF questionnaire was completed on arrival and the second assessment was performed 20 minutes later by a different physician. The test-retest reliability was analyzed by computing kappa statistics. Kappa scores of > 0.6 indicated a good agreement. To assess the validity, we compared the total BDCAF score with the patient's/clinician's perception of disease activity and the Korean version of the Behcet's Disease Quality of Life (BDQOL). RESULTS: For the test-retest reliability, good agreements were achieved on items such as headache, oral/genital ulceration, erythema, skin pustules, arthralgia, nausea/vomiting/abdominal pain, and diarrhea with altered/frank blood per rectum. Moderate agreement was observed for eye and nervous system involvement. We achieved a fair agreement for arthritis and major vessel involvement. Significant correlations were obtained between the total BDCAF score with the BDQOL and the patient's/clinician's perception of disease activity p < 0.05). CONCLUSIONS: The Korean version of the BDCAF is a reliable and valid instrument for measuring current disease activity in Korean BD patients.

A Case of Enterocolic Lymphocytic Phlebitis Mimicking Surgical Abdomen

Vasculitis that involves the gastrointestinal (GI) tract often occurs as part of a systemic inflammatory process. It is a well-recognized manifestation of the small and medium sized vessel vasculitides. Vasculitis of the GI tract may occur in isolation; although it can progress to a systemic illness. It usually involves the arterioles, venules, and capillaries; however, it is very rare for only the venules to be affected. Enterocolic lymphocytic phlebitis is a localized vasculitis, typically affecting the small and medium-sized intramural and mesenteric veins of the intestines. We report a case of enterocolic lymphocytic phlebitis of the colon. A 38-year-old woman was presented with hematochezia and severe abdominal pain on the day of admission. She had no history of intestinal disease or systemic disease. Computed tomography showed an extremely thickened wall of the colon, along with several air bubbles in the colon with diffuse subcutaneous emphysema in the abdominal wall. An emergency exploration laparotomy and extended right hemicolectomy was performed. The patient recovered completely after surgery and remains well without further therapy.

Duration of Prophylactic Therapy Affects the Frequency of Gout Flare

OBJECTIVE: To evaluate the effect of prophylactic therapy on gout flare during urate lowering treatment. METHODS: We retrospectively examined the data derived from 59 patients who had been treated with allopurinol for more than six months after stopping prophylactic medication at our rheumatology clinic. Demographic data (age, sex, disease duration, tophi and comorbidity), clinical and laboratory features, including presence of gout flare during urate lowering treatment, dose of allopurinol, serum uric acid level and creatinine clearance at initiation and six months later, were collected. For the subgroup analysis, the same data were collected in 46 patients who had been followed up at one year after stopping prophylactic medication. RESULTS: Twenty-eight patients among 59 (47.4%) had experienced at least 1 gouty attack during urate lowering therapy. The mean duration of prophylactic medication was not different between the flare group (3.8 months) and the non-flare group (5.9 months, p=0.617). Six months later, the mean serum uric acid level was 6.3 mg/dL (6.1 mg/dL vs. 6.5 mg/dL). According to the duration of prophylactic treatment ( or =6 months), there were more frequent flares in the or =6 months group (51.2% vs. 38.9% in the six month follow-up group, 70.6% vs. 50% in the one year follow-up group). CONCLUSION: Prophylactic medication for more than six months could be a favorable factor for the prevention of recurrent gout flare during urate lowering treatment.

Duration of Prophylactic Therapy Affects the Frequency of Gout Flare

OBJECTIVE: To evaluate the effect of prophylactic therapy on gout flare during urate lowering treatment. METHODS: We retrospectively examined the data derived from 59 patients who had been treated with allopurinol for more than six months after stopping prophylactic medication at our rheumatology clinic. Demographic data (age, sex, disease duration, tophi and comorbidity), clinical and laboratory features, including presence of gout flare during urate lowering treatment, dose of allopurinol, serum uric acid level and creatinine clearance at initiation and six months later, were collected. For the subgroup analysis, the same data were collected in 46 patients who had been followed up at one year after stopping prophylactic medication. RESULTS: Twenty-eight patients among 59 (47.4%) had experienced at least 1 gouty attack during urate lowering therapy. The mean duration of prophylactic medication was not different between the flare group (3.8 months) and the non-flare group (5.9 months, p=0.617). Six months later, the mean serum uric acid level was 6.3 mg/dL (6.1 mg/dL vs. 6.5 mg/dL). According to the duration of prophylactic treatment ( or =6 months), there were more frequent flares in the or =6 months group (51.2% vs. 38.9% in the six month follow-up group, 70.6% vs. 50% in the one year follow-up group). CONCLUSION: Prophylactic medication for more than six months could be a favorable factor for the prevention of recurrent gout flare during urate lowering treatment.

Clinical and Radiographic Features of Adult-onset Ankylosing Spondylitis in Korean Patients: Comparisons between Males and Females

The objective of this study was to investigate clinical and radiographic features and gender differences in Korean patients with adult-onset ankylosing spondylitis. Multicenter cross-sectional studies were conducted in the rheumatology clinics of 13 Korean tertiary referral hospitals. All patients had a confirmed diagnosis of ankylosing spondylitis according to the modified New York criteria. Clinical, laboratory, and radiographic features were evaluated and disease activities were assessed using the Bath ankylosing spondylitis disease activity index. Five hundred and five patients were recruited. The male to female ratio was 6.1:1. Average age at symptom onset was 25.4+/-8.9 yr and average disease duration was 9.6+/-6.8 yr. Males manifested symptoms at a significantly earlier age. HLA-B27 was more frequently positive in males. Hips were more commonly affected in males, and knees in females. When spinal mobility was measured using tragus-to-wall distance and the modified Schober's test, females had significantly better results. Radiographic spinal changes, including bamboo spine and syndesmophytes, were more common in males after adjustment of confounding factors. In conclusion, we observed significant gender differences in radiographic spinal involvement as well as other clinical manifestations among Korea patients with adult-onset ankylosing spondylitis. These findings may influence the timing of the diagnosis and the choice of treatment.

A Case of Idiopathic Sclerosing Peritonitis / 대한류마티스학회지

Idiopathic sclerosing peritonitis is a rare disease characterized by fibrosis and adhesion of the peritoneum to loops of the small intestine. It may be the cause of an unusual surgical emergency such as small bowel ileus. It is diagnosed predominantly in female adolescents. We report the case of an idiopathic sclerosing peritonitis in Korea. A 38-year-old man visited emergency room for recurrent small bowel ileus and migrating mass like lesion. Computed tomography (CT) of abdomen showed acute peritonitis with a diffuse wall thickening of terminal ileum and extraluminal fluid collectionaround the terminal ileum. He underwent laparotomy. The ileocolectomy with adhesiolysis was performed and its pathological examination revealed the characteristic findings of idiopathic sclerosing peritonitis. Symptoms recurred 2 months after surgery, but improved with steroid treatment.

A Case of Ankylosing Spondylitis Accompanying Sarcoidosis / 대한류마티스학회지

Sacroiliitis is a rare manifestation in patients with sarcoidosis and there have been several case reports of ankylosing spondylitis (AS) accompanying sarcoidosis. Recently, the development of sarcoidosis has been reported in AS patients treated with tumor necrosis factor blocker. We described a 27-year-old male patient with AS and histologically proven sarcoidosis who was treated with etanercept without aggravation of sarcoidosis.

A Case of Systemic Lupus Erythematosus with Degos' Disease / 대한류마티스학회지

Degos' disease, malignant atrophic papulosis, is a rare disease characterized by a pathognomonic appearance of porcelain-white, atrophic papules with peripheral erythema and multiple occlusive thrombotic vascular pathology. It sometimes involves internal viscera such as the gastrointestinal tract, the central nervous system and kidney which may bring to death. Although several reports showed the associations between Degos' disease and antiphospholipid antibody or systemic lupus erythematosus, its pathogenetic significance remains elusive. The previous 4 Korean cases of Degos's disease involved only skin and/or the gastrointestinal tract. We report a case of systemic lupus erythematosus with Degos' disease involving skin, kidney and small intestine.