Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology that is characterized by high-spiking fever, arthralgia, sore throat, and skin rash. The typical rash of AOSD is an evanescent, salmon-colored erythema, which is considered to be the major diagnostic criterion. Recently, other cutaneous manifestations of AOSD, such as persistent plaque and urticaria, have been reported. Here, we report a rare case of AOSD presenting with periorbital swelling and erythema. A 47-year-old woman was presented with periorbital swelling, erythema, high fever, arthritis, and a sore throat. One year prior to admission, she was diagnosed with AOSD based on the diagnostic criteria of Yamaguchi. The patient's periorbital swelling and erythema may not have been associated with periorbital cellulitis because they did not respond to antibiotics but did improve after treatment with steroids. Considering all of her signs and symptoms with a history of AOSD, periorbital lesion was suspected as atypical cutaneous manifestation of AOSD.
Arthralgia ; Arthritis ; Cellulitis ; Edema ; Erythema ; Female ; Fever ; Humans ; Middle Aged ; Pharyngitis ; Steroids ; Still's Disease, Adult-Onset ; Urticaria

Methotrexate is often used in patients with systemic lupus erythematosus for effective disease controlsand steroid-sparing, and has been known to involve the development of lymphoproliferative disorders for patients with autoimmune diseases. We report a case of spontaneous regression of Epstein-Barr virus-positive methotrexate-associated Hodgkin's lymphoma in a 24-year-old woman with systemic lupus erythematosus. Following 6 months of treatment with low-dose methotrexate, the patient developed a neck mass in the right submandibular area. A computed tomography scan of the neck, chest and abdomen revealed multiple enlarged lymph nodes. Excisional biopsy of the neck masses confirmed infiltrations of malignant lymphoid cells that were positive for CD15, CD30, and Epstein-Barr virus-encoded RNA. Reduction of the mass was observed 3 weeks after withdrawing from the methotrexate treatment. At 7 months after initial presentation, computed tomography revealed near-complete regression of lymphadenopathy. After 30 months, the patient was still in complete clinical remission.
Abdomen ; Autoimmune Diseases ; Biopsy ; Female ; Hodgkin Disease ; Humans ; Lupus Erythematosus, Systemic ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Lymphoproliferative Disorders ; Methotrexate ; Neck ; Remission, Spontaneous ; Thorax ; Young Adult

OBJECTIVE: Although a series of trials support that rheumatoid arthritis (RA) is associated with increased atherosclerosis, the link between microvascular structural changes and the disease activity of RA has not been clarified. We measured changes in the retinal microvasculature using fundus fluorescein angiography (FAG) and investigated the association between the retinal vasculature and clinical parameters of RA. METHODS: Seventy-five RA patients and sixty healthy control were included. Morphometric and quantitative features in the capillary images including retinal vascular signs and vessel diameters were measured with fundus photography and FAG. RA activity was assessed based on high sensitivity C-reactive protein (hsCRP), disease activity score with 28 joints (DAS 28), and health assessment questionnaire (HAQ). RESULTS: Central retinal arteriolar equivalents (CRAE) was 118.1+/-31.3 microm in RA patients and 123.8+/-19.9 microm in control subjects, showing the tendency of retinal arteriolar narrowing in patients with RA but without statistical significance. The mean central retinal venular equivalents (CRVE) was 162.4+/-26.4 microm which was significantly higher than that of control group (144.1+/-23.1 microm, p<0.001). The prevalence of AVN was 34.7%, and significantly higher in RA group. Among retinal findings, the presence of early pinpoint hyperfluorescence and areas of delayed choroidal perfusion correlated with hsCRP. Age, disease duration, DAS 28, HAQ, and rheumatoid factor (RF) had no effect on CRAE and CRVE. In multivariate analysis, only hsCRP was found to be associated with wider venular caliber. CONCLUSION: Retinal venular widening was more common in RA patients. Retinal venular diameter had significant correlation with disease activity of RA. Retinal imaging is a comparative method for the assessment of microvascular findings of RA patients.
Arthritis, Rheumatoid ; Atherosclerosis ; C-Reactive Protein ; Capillaries ; Choroid ; Fluorescein ; Fluorescein Angiography ; Humans ; Joints ; Microvessels ; Multivariate Analysis ; Perfusion ; Photography ; Prevalence ; Rheumatoid Factor ; Surveys and Questionnaires

OBJECTIVE: To compare the urate-lowering efficacy and the safety of febuxostat, allopurinol and placebo in Korean patients with gout for 4 weeks. METHODS: Subjects (n=182) with gout were randomized to febuxostat (40, 80, 120 mg), allopurinol 300 mg, or placebo group. The primary end point was the proportion of subjects whose serum urate concentration fell to less than 6.0 mg/dL after the 4-week treatment. RESULTS: The primary end point was reached at 25.7%, 80.0% and 83.3% of patients receiving 40, 80 and 120 mg of febuxostat, respectively, 58.3% of those receiving 300 mg of allopurinol and none of the placebo (p<0.001: each febuxostat dose or allopurinol group versus placebo group, p=0.0484 and p=0.0196: febuxostat 80 and 120 mg compared with allopurinol, respectively). The number and proportion of subjects who developed adverse events (AEs) were 13 subjects (37%), 14 (39%) and 18 (50%) in the febuxostat of 40, 80 and 120 mg group, respectively, 21 (57%) in the allopurinol 300 mg group and 17 (46%) in the placebo group. No statistically significant differences in the incidence rates of adverse events were observed between the groups. There was no significant difference in gout flare-up incidence. CONCLUSION: Febuxostat, 80 mg or 120 mg, was more effective than allopurinol (300 mg) or placebo, when lowering the serum urate. The safety of febuxostat and allopurinol was comparable.
Allopurinol ; Gout ; Humans ; Incidence ; Thiazoles ; Uric Acid ; Febuxostat

Infection is a critical and challenging situation encountered in treatment of inflammatory rheumatic diseases (IRDs). It is associated with the disease activity itself and/or the immunosuppressant treatments. Vaccine preventable infections could be controlled by use of a timely vaccination protocol. Immunosuppressed situations observed in IRDs require some modification of the recommendation for the general population in each national society. Live attenuated vaccines are generally contraindicated in IRDs, except for varicella-zoster vaccination, which is solely permitted live vaccine and could be given on a case by case basis in autoimmune IRDs. Influenza and pneumococcal vaccines are strongly recommended due to increased mortality in patients with IRDs. The vaccination protocol reflects the current national medical environment and requirements; therefore, it could change with time. The Korean Rheumatology Society now requires that vaccination be recommended for patients with IRDs, with the possibility of both an adult and child version.
Adult ; Child ; Humans ; Influenza, Human ; Pneumococcal Vaccines ; Rheumatic Diseases ; Rheumatology ; Vaccination ; Vaccines, Attenuated

Many rheumatic diseases may involve any organ system of the body including the skin. Proper understanding of rheumatic skin disease is necessary for making a diagnosis. In addition, there are important relationships existing between the cutaneous and systemic manifestations of rheumatic diseases. In management of these diseases, the interdisciplinary approach could generate better results. Among rheumatic diseases, the cutaneous manifestations of three major rheumatic diseases such as lupus erythematosus (LE), dermatomyositis (DM)/polymyositis and scleroderma/systemic sclerosis (SSc) will all be reviewed.
Dermatomyositis ; Lupus Erythematosus, Systemic ; Rheumatic Diseases ; Polymyositis ; Scleroderma, Systemic ; Skin ; Skin Diseases

No abstract available.
Lupus Nephritis

Systemic sclerosis as a connective tissue disease could affect all internal organs of the body and could also manifest as a cutaneous lesion. Cardiac involvement leading to cardiac manifestations in systemic sclerosis patients is not rare. However, cardiac amyloidosis combined with systemic sclerosis is extremely rare. Although there were no definite treatment options in this case, symptomatic treatment is the cornerstone of the management plan. In this case report, we described a correct diagnosis and symptomatic medical care of early reactive cardiac amyloidosis with systemic sclerosis and summarize the current state of the relevant literature.
Amyloidosis* ; Cardiomyopathy, Restrictive ; Connective Tissue Diseases ; Diagnosis* ; Humans ; Scleroderma, Systemic*

OBJECTIVE: The purpose of this study is to evaluate the clinical and hematological effects of tocilizumab in active rheumatoid arthritis (RA) patients. METHODS: Fourteen patients with active RA were enrolled in this study. The patients received tocilizumab 8 mg/kg intravenously every four weeks for 6 months. Disease activity, anemia-related factors including serum hepcidin-25, and hematological parameters were monitored at baseline and at 1, 3, and 6 months after the initiation of treatment. RESULTS: Significant reductions in tender joint count, swollen joint count, visual analogue scale, erythrocyte sedimentation rate (ESR), and C-reactive (CRP) protein plus reductions in a 28-joint disease activity score were observed within one month after the first tocilizumab treatment. These effects lasted throughout the six-month study period. In addition, significant improvements in anemia-related factors such as hepcidin-25, ferritin, iron, hemoglobin, red blood cell counts and mean corpuscular volume were observed during the treatment period. Hematological parameters were improved with reductions in counts for leukocytes, monocytes, neutrophils, and platelets. The lymphocyte counts and their subset numbers were unchanged. Changes in hepcidin levels showed significant correlation with changes in CRP, ESR, ferritin, hemoglobin and counts for red blood cells, leukocytes, and neutrophils during the treatment period. CONCLUSION: This study demonstrates that tocilizumab significantly and meaningfully reduces disease burden in patients with active RA. In addition, tocilizumab diminishes the levels of inflammatory anemia by inhibiting hepcidin production. These clinical data provide evidence of a favorable outcome from tocilizumab in RA.
Anemia* ; Arthritis, Rheumatoid* ; Blood Sedimentation ; Erythrocyte Count ; Erythrocyte Indices ; Erythrocytes ; Ferritins ; Hepcidins* ; Humans ; Iron ; Joints ; Leukocytes ; Lymphocyte Count ; Monocytes ; Neutrophils

OBJECTIVE: Gout is known to be associated with cardiovascular disease (CVD), and hyperhomocysteinemia is one of the risk factors for CVD. We investigated the associations between the change of serum homocysteine (Hcy) level and those of the other parameters including serum uric acid level, renal function, and cholesterol profiles in chronic gout patients with longitudinal follow-up data. METHODS: Ninety-one male patients with chronic gout and 97 age-matched healthy male control subjects were included in the previous study. Among them, 33 patients with gout and 39 healthy control subjects underwent follow-up tests for Hcy levels with an average of 24.00+/-9.12 months in this study. RESULTS: The follow-up data showed that gout patients had significantly higher levels of Hcy in serum than control subjects (16.75+/-5.43 micromol/L vs. 13.17+/-3.83 micromol/L, p=0.002). In gout patients, the change of serum Hcy level after follow up showed positive correlation with the change of creatinine (gamma=0.442, p=0.009) and negative correlation with estimated glomerular filtration rate (eGFR; gamma=-0.528, p<0.001). However, the change of serum Hcy level did not show correlation with the changes of uric acid level or the lipid profiles. CONCLUSION: Serum Hcy level was elevated in gout patients compared with control subjects. The change of serum Hcy level showed negative correlation with the change of eGFR. Hyperhomocysteinemia in gout patients was associated with decreased renal function, but not with serum uric acid or lipid profiles.
Cardiovascular Diseases ; Cholesterol ; Creatinine ; Follow-Up Studies* ; Glomerular Filtration Rate ; Gout* ; Homocysteine* ; Humans ; Hyperhomocysteinemia ; Male ; Risk Factors ; Uric Acid*