BACKGROUND/AIMS: We investigated whether transthoracic echocardiography-suspected pulmonary hypertension (PH) affects survival in systemic lupus erythematosus (SLE) patients and examined factors associated with PH occurrence and survival. METHODS: This retrospective single-center study included 154 Korean SLE patients fulfilling the American College of Rheumatology criteria (January 1995 to June 2013). Student t test, Mann-Whitney U test, Kaplan-Meier curves, and log-rank tests were used for comparisons. RESULTS: A total of 35 SLE patients with PH (SLE/PH+) and 119 without PH (SLE/PH-) were analyzed. Higher percentages of interstitial lung disease, Raynaud's phenomenon (RP), World Health Organization functional classification III/IV, and cardiomegaly were found in SLE/PH+ compared to SLE/PH-. Furthermore, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index was significantly higher in SLE/PH+ (2.46 +/- 1.245 vs. 1.00 +/- 1.235), whereas survival rates were significantly higher in SLE/PH- in log-rank tests (p = 0.001). In multivariate analysis, the adjusted mortality hazard ratio (HR) for SLE/PH+ patients was 3.10. Subgroup analysis demonstrated a higher percentage of lupus nephritis in the SLE/PH+ patients who died (p = 0.039) and low complement-3 levels (p = 0.007). In univariate analysis, the mortality HR for SLE/PH+ patients with lupus nephritis was 4.62, whereas the presence of RP decreased the mortality risk in multivariate analysis; adjusted HR, 0.10. CONCLUSIONS: PH is an independent factor predicting survival in SLE patients. The presence of lupus nephritis resulted in an increased trend for mortality, whereas coexistence of RP was associated with a better survival prognosis in SLE/PH+ patients.
Adolescent ; Adult ; Cardiomegaly/diagnosis/epidemiology ; Chi-Square Distribution ; Female ; Humans ; Hypertension, Pulmonary/diagnosis/*mortality ; Kaplan-Meier Estimate ; Lung Diseases, Interstitial/diagnosis/mortality ; Lupus Erythematosus, Systemic/diagnosis/*mortality ; Lupus Nephritis/diagnosis/mortality ; Male ; Multivariate Analysis ; Prognosis ; Proportional Hazards Models ; Raynaud Disease/diagnosis/epidemiology ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Young Adult

The effects of several antihypertensive drugs on bone mineral density (BMD) and micro-architectural changes in ovariectomized (OVX) mice were investigated. Eight-week-old female C57/BL6 mice were used for this study. Three days after ovariectomy, mice were treated intraperitoneally with nifedipine (15 mg/kg), telmisartan (5 mg/kg), enalapril (20 mg/kg), propranolol (1 mg/kg) or hydrochlorothiazide (12.5 mg/kg) for 35 consecutive days. Uterine atrophy of all mice was confirmed to evaluate estrogen deficiency state. BMD and micro-architectural analyses were performed on tibial proximal ends by micro-computed tomography (micro-CT). When OVX mice with uterine atrophy were compared with mice without atrophy, BMD decreased (P < 0.001). There were significant differences in BMD loss between different antihypertensive drugs (P = 0.005). Enalapril and propranolol increased BMD loss in mice with atrophied uteri compared with control mice. By contrast, thiazide increased BMD in mice with uterine atrophy compared with vehicle-treated mice (P = 0.048). Thiazide (P = 0.032) and telmisartan (P = 0.051) reduced bone loss and bone fraction in mice with uterine atrophy compared with the control. Thiazide affects BMD in OVX mice positively. The reduction in bone loss by thiazide and telmisartan suggest that these drugs may benefit menopausal women with hypertension and osteoporosis.
Animals ; Antihypertensive Agents/*pharmacology ; Atrophy ; Benzimidazoles/pharmacology ; Benzoates/pharmacology ; Bone Density/*drug effects ; Enalapril/pharmacology ; Female ; Mice ; Mice, Inbred C57BL ; Ovariectomy ; Propranolol/pharmacology ; Thiazides/pharmacology ; Tibia/radiography ; Tomography, X-Ray Computed ; Uterus/anatomy & histology/pathology

The effects of several antihypertensive drugs on bone mineral density (BMD) and micro-architectural changes in ovariectomized (OVX) mice were investigated. Eight-week-old female C57/BL6 mice were used for this study. Three days after ovariectomy, mice were treated intraperitoneally with nifedipine (15 mg/kg), telmisartan (5 mg/kg), enalapril (20 mg/kg), propranolol (1 mg/kg) or hydrochlorothiazide (12.5 mg/kg) for 35 consecutive days. Uterine atrophy of all mice was confirmed to evaluate estrogen deficiency state. BMD and micro-architectural analyses were performed on tibial proximal ends by micro-computed tomography (micro-CT). When OVX mice with uterine atrophy were compared with mice without atrophy, BMD decreased (P < 0.001). There were significant differences in BMD loss between different antihypertensive drugs (P = 0.005). Enalapril and propranolol increased BMD loss in mice with atrophied uteri compared with control mice. By contrast, thiazide increased BMD in mice with uterine atrophy compared with vehicle-treated mice (P = 0.048). Thiazide (P = 0.032) and telmisartan (P = 0.051) reduced bone loss and bone fraction in mice with uterine atrophy compared with the control. Thiazide affects BMD in OVX mice positively. The reduction in bone loss by thiazide and telmisartan suggest that these drugs may benefit menopausal women with hypertension and osteoporosis.
Animals ; Antihypertensive Agents/*pharmacology ; Atrophy ; Benzimidazoles/pharmacology ; Benzoates/pharmacology ; Bone Density/*drug effects ; Enalapril/pharmacology ; Female ; Mice ; Mice, Inbred C57BL ; Ovariectomy ; Propranolol/pharmacology ; Thiazides/pharmacology ; Tibia/radiography ; Tomography, X-Ray Computed ; Uterus/anatomy & histology/pathology

Polyarteritis nodosa (PAN) is a necrotizing vasculitis of the medium-sized arteries. The symptoms and signs of PAN include purpuric skin lesions, mononeuritis multiplex, symptoms of mesenteric ischemia, and renal involvement. We report the first case of PAN manifesting as a neuropathy after influenza infection in Korea. A 68-year-old woman had fever, myalgia, hyperesthesia of both hand and foot, and lower extremity weakness. EMG findings showed severe sensorimotor polyneuropathy, such as multiple mononeuritis. A sural nerve biopsy showed vasculitis and Influenza A (H1N1) were positive. Our patient was treated by glucocorticoid and oral cyclophosphamide, thereafter, symptoms and signs improved. No recurrence has been observed for five months.
Aged ; Arteries ; Biopsy ; Cyclophosphamide ; Female ; Fever ; Foot ; Hand ; Humans ; Hyperesthesia ; Influenza, Human ; Ischemia ; Korea ; Lower Extremity ; Mononeuropathies ; Organic Chemicals ; Polyarteritis Nodosa ; Polyneuropathies ; Recurrence ; Skin ; Sural Nerve ; Vasculitis

OBJECTIVE: The aim of this study was to assess the gender differences in the clinical presentation and treatment patterns between Korean women and men with ankylosing spondylitis (AS). METHODS: We retrospectively analyzed the data from extensive clinical assessments of 721 patients (162 women and 559 men) with AS, who were diagnosed at Seoul St. Mary's Hospital, between January 2000 and September 2009. Clinical data, regarding the disease onset, disease duration, clinical presentations, status of human leukocyte antigen (HLA)-B27, and bone mineral density, were determined using a dual-energy X-ray absorptiometry (DEXA). Finally, we analyzed the medical treatments prescribed for these patients. RESULTS: The ratio of men to women was 3.45:1. Compared to men, women were older at the time of diagnosis, had shorter disease durations, and were diagnosed in earlier stages of the disease. More women had a history of uveitis at diagnosis than men. Back pain was the main presenting symptom, and its prevalence was the same in both genders. Fewer women showed cervical and thoracic axial involvement than men. Initially, more women had wrist and hand pain than men; however, at some point, peripheral arthritis development was equally likely in both genders. Women experienced shoulder pain, during the disease course, more often thanmen. On the other hand, men presented with knee and hip pain more often than women. Sulfasalazine and anti-TNF agents were more often prescribed to women. CONCLUSION: The presentation and progression of AS showed a difference between women and men. Because of these differences, AS should be considered when a women presents with peripheral arthritis or uveitis in the early stage of the disease.
Absorptiometry, Photon ; Arthritis ; Back Pain ; Bone Density ; Female ; Hand ; Hip ; Humans ; Knee ; Leukocytes ; Male ; Prevalence ; Retrospective Studies ; Shoulder Pain ; Spondylitis, Ankylosing ; Sulfasalazine ; Uveitis ; Wrist

OBJECTIVE: The 2010 New American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for rheumatoid arthritis (RA) was raised to identify patients with early RA and replaced the 1987 ACR classification criteria. The aims of this study are to assess the availability of new classification criteria and to evaluate its potential limitation. METHODS: A total of 408 patients with newly diagnosed RA were included from 13 secondary or tertiary hospitals in South Korea. The symptom duration was less than 12 months before the diagnosis of RA. RA was defined as either 1987 ACR classification criteria or new 2010 ACR/EULAR criteria. We compared the full details of both classification criteria. RESULTS: The mean symptom duration was 5.1 months. The majority (76.2%) of the patients were female. Two hundred and seventy three patients (66.9%) fulfilled both of the 2010 and 1987 classification criteria. Forty-seven (14.7%) of the 320 patients fulfilling the 1987 criteria did not fulfill the new classification criteria. On the other hand, eighty-eight (24.4%) of the 361 patients fulfilling the 2010 ACR/EULAR classification criteria did not fulfill the 1987 ACR criteria. Thirty-six (55.4%) of the 65 patient with seronegative RA failed to meet the 2010 classification criteria. In case of seropositive RA (n=343), 85 additional patients (24.8%) could be diagnosed as RA using new classification criteria. CONCLUSION: The new 2010 ACR/EULAR classification criteria enable physicians to diagnose more patients with early RA via the help of serology. However, the sensitivity for the diagnosis of seronegative RA is projected to decrease.
Arthritis, Rheumatoid ; Female ; Hand ; Humans ; Republic of Korea ; Rheumatic Diseases ; Tertiary Care Centers

OBJECTIVE: We wanted to investigate the incidence of serious infections among the rheumatoid arthritis (RA) patients who were treated with tumor necrosis factor alpha (TNF-alpha) antagonists. METHODS: We enrolled the 175 RA patients who were treated with TNF-alpha antagonists for at least 3 months during February 2003 to July 2008, and these patients were in the SMART-b cohort of Kangnam St. Mary's hospital. Patients were prescribed infliximab, etanercept or adalimumab. The data was retrospectively collected. RESULTS: The incidence of serious infections among the RA patients treated with TNF-alpha was significantly increased according to the survival analysis, as compared with that of those patient treated with conventional DMARDs (p<0.01). The most common serious infection was pneumonia. There was no significant difference in the incidence of serious infections among the three TNF-alpha antagonists used in this study (p=0.96). But the serious infections occurred more often in the patients who received more than 10 mg methotrexate (MTX) per week (p=0.02). CONCLUSION: RA patients treated with TNF-alpha antagonists had a higher incidence of serious infection. Therefore, close monitoring for serious infection is needed for RA patients who are receiving TNF-alpha antagonists.
Antibodies, Monoclonal ; Antibodies, Monoclonal, Humanized ; Antirheumatic Agents ; Arthritis, Rheumatoid ; Cohort Studies ; Humans ; Immunoglobulin G ; Incidence ; Methotrexate ; Pneumonia ; Receptors, Tumor Necrosis Factor ; Retrospective Studies ; Tumor Necrosis Factor-alpha

OBJECTIVE: We wanted to investigate the incidence of serious infections among the rheumatoid arthritis (RA) patients who were treated with tumor necrosis factor alpha (TNF-alpha) antagonists. METHODS: We enrolled the 175 RA patients who were treated with TNF-alpha antagonists for at least 3 months during February 2003 to July 2008, and these patients were in the SMART-b cohort of Kangnam St. Mary's hospital. Patients were prescribed infliximab, etanercept or adalimumab. The data was retrospectively collected. RESULTS: The incidence of serious infections among the RA patients treated with TNF-alpha was significantly increased according to the survival analysis, as compared with that of those patient treated with conventional DMARDs (p<0.01). The most common serious infection was pneumonia. There was no significant difference in the incidence of serious infections among the three TNF-alpha antagonists used in this study (p=0.96). But the serious infections occurred more often in the patients who received more than 10 mg methotrexate (MTX) per week (p=0.02). CONCLUSION: RA patients treated with TNF-alpha antagonists had a higher incidence of serious infection. Therefore, close monitoring for serious infection is needed for RA patients who are receiving TNF-alpha antagonists.
Antibodies, Monoclonal ; Antibodies, Monoclonal, Humanized ; Antirheumatic Agents ; Arthritis, Rheumatoid ; Cohort Studies ; Humans ; Immunoglobulin G ; Incidence ; Methotrexate ; Pneumonia ; Receptors, Tumor Necrosis Factor ; Retrospective Studies ; Tumor Necrosis Factor-alpha

POEMS syndrome is multisystemic disorder characterized polyneuropathy, organomegaly, endocrinopathies, monoclonal gammapathy. A 38 year-old man visited our hospital because of progressive general weakness, hyperpigmentation. We diagnosed him as idiopathic primary adrenal insufficiency. The symptoms of general weakness were improved after steroid replacement therapy. But after 6 months, he revisited ER with chief complaints of abdominal distension and edema in lower extremities. He had also polyneuropathy, hepatosplenomegaly, IgA light chain monoclonal gammopathy, hyperpigmentation and thickening of the skin. Bone marrow biopsy showed that the plasma cells, mainly mature forms, are about 40% of all nucleated elements and on tissue section. We think this is the first case of POEMS syndrome presenting as primary adrenocortical insufficiency in Korea, so we report the case with reviews of other literatures. And we recommend performing a rapid ACTH stimulation test in all POEMS syndrome in order to detect adrenal insufficiency.
Addison Disease* ; Adrenal Insufficiency ; Adrenocorticotropic Hormone ; Adult ; Biopsy ; Bone Marrow ; Edema ; Humans ; Hyperpigmentation ; Immunoglobulin A ; Korea ; Lower Extremity ; Multiple Myeloma* ; Paraproteinemias ; Plasma Cells* ; Plasma* ; POEMS Syndrome* ; Polyneuropathies ; Skin

A xanthoma is an abnormal localized collection of histiocytic tissue containing lipid. It is not a true tumor, but is a reactive histiocytic proliferation often secondary to alternations in serum lipids. Xanthoma lesions are seen in the skin and tendons, and may occur at other sites, such as the stomach and arytenoepiglottic fold. However, a xanthoma is rare in the bladder, with only 9 reported cases. Here, a case of a xanthoma of the bladder detected incidentally during cystoscopic examination for a gross hematuria is reported.
Hematuria ; Skin ; Stomach ; Tendons ; Urinary Bladder* ; Xanthomatosis*