ABSTRACT
High-dose intravenous immunoglobulins alter the disease activity of adult-onset Still's disease (AOSD). Because activation status of FcgammaR is possibly dependent on their genetic polymorphisms, we investigated whether the polymorphisms of FcgammaR IIa and IIIa are risk factors, and affect the clinical features of AOSD. Genomic DNA was extracted from 36 patients and from 197 healthy controls. Polymerase chain reaction for FcgammaR IIa and IIIa using the allele-specific primers and direct sequencing of FcgammaR IIIa polymorphic site were performed. The frequencies of FcgammaR IIa/IIIa genotype between patients with AOSD and controls were not different. The allelic frequencies of FcgammaR IIa/IIIa between patients with AOSD and controls were not different, either. However, the FcgammaR IIa-R/R131 genotype was associated with a higher concentration of hemoglobin (p=0.04) and stable liver function (p=0.009) than the other genotypes. The FcgammaR IIIa-F/F176 genotype was associated with significantly lower titers of serum ferritin (p=0.025), and higher serum albumin (p=0.037) and cholesterol (p=0.014) concentrations than the other genotypes. This study suggest that the FcgammaR IIa and IIIa polymorphisms might not be genetic risk factors for AOSD in Korean, but contribute to the activity of disease. FcgammaR IIa-R/R131 and IIIa-F/F176 genotypes, low-binding genotypes for IgG2a and G1, may have more protective effects in acute stage of the disease than the other genotypes.
Subject(s)
Adult , Female , Humans , Male , Antigens, CD/genetics , Genotype , Korea , Polymorphism, Genetic , Receptors, IgG/genetics , Still's Disease, Adult-Onset/geneticsABSTRACT
We report a 25-year-old Korean woman with Adult onset Still's disease (AOSD) presented with renal amyloidosis, which had developed four years after disease onset. We successfully treated her with prednisolone, colchicine and cyclophosphamide. A review of the literature uncovered about 10 cases, most of which were treated by various regimens that resulted in poor outcomes. Renal amyloidosis should be suspected in patients with AOSD who have unexplained proteinuria. Although the mechanism of renal amyloid deposition is not well known, earlier histopathologic diagnosis and choice of regimen may affect prognosis.
Subject(s)
Adult , Female , Humans , Amyloidosis , Kidney Diseases , Still's Disease, Adult-OnsetABSTRACT
The antisynthetase syndrome is characterized by inflammatory myositis associated with intersititial lung disease (ILD), polyarthritis, mechanic's hand, and Raynaud's phenomenon, and usually with fever. The patients with these conditions have autoantibodies to aminoacy1-tRNA synthetases (histidy1-, threony1-, alany1-, isoleucy1-, and glycy1-tRNA synthetase) ; the most common is anti-histidy1-tRNA sythetase (anti Jo-1), present in 20% of myositis patients in Western countries. However, the mechanisms of production of autoantibodies to aminoacy1-tRNA synthetases are not understood. Several hypotheses about the relationship of anti-aminoacy1-tRNA synthetases with inflammatory myositis have been reported. The poor prognostic outcome for the antisynthetase patients are related to ILD. We experienced two cases of antisynthetase syndrome which have characterized by the clinical manifestations, and report these cases with a review of the literatures.
Subject(s)
Humans , Arthritis , Autoantibodies , Fever , Hand , Ligases , Lung Diseases , MyositisABSTRACT
Hypothyroidism may be accompanied by different rheumatic syndromes such as neuropathy. Arthropathy and myopathy. Sebere myopathy with high elevations of creatine kinase (CK) is rarely seen in hypothyroidism. Authors experinced a case of myopathy with hypothyroidism. Similar to rheumatoid arthritis or polymyositis like syndrome. We have treated with thyroid hormone replacement (Levothyroxine sedium 100ug/day) for 10 months. The initial serology resulted in : free T4 0.51ng/dl, TSH 110ulU/ml, LDH 465 units, and CK 4385 units. After tratment, we gained free T4 1.60ng/dl, TSH 0.14uIU/ml, LDH 110 units, and CK 80 utits and musculoskeletal symptoms are much improved We reprort this patient with a review of literatures.
Subject(s)
Humans , Arthritis, Rheumatoid , Creatine Kinase , Hypothyroidism , Muscular Diseases , Polymyositis , Thyroid GlandABSTRACT
Lymphangioleiomyomatosis(LAM) is rare and essentially limited to women in the reproductive ages. A 39-year-old female was admitted due to progressive exerUional dyspnea and intermittent productive cough. Chest PA showed marked hyperinflation of the lung associated with a diffuse reticulo-nodular pattern. High resolution CT scan of the thorax demonstrated that diffusely scattered thin-walled cysts were distributed throughout the bilateral lung fields. Abdominal CT scan showed variable sized multiple angiomyolipoma of both kidney. By open lung biopsy, she was diagnosed as pulmonary LAM associated with Tuberous sclerosis and renal Angiomyolipoma. We present the case and discuss the connection between pulmonary LAM and Tuberous sclerosis.
Subject(s)
Adult , Female , Humans , Angiomyolipoma , Biopsy , Cough , Dyspnea , Kidney , Lung , Sclerosis , Thorax , Tomography, X-Ray Computed , Tuberous SclerosisABSTRACT
We studied sputum eosinophil count of patients with chronic cough. Differential count of sputum cells(simple direct smear with Wright stain) were successfully examined in 44(73%) among 60 patients with chronic cough. The patients were divided according to PC2O value; 20(45%) patients had increased airway hyper responsiveness(AHR) (PC2O 5%) was found in 66% of patients with increased AHR. In patients without increased AHR, increased sputum eosinophil ratio was found in three patients(12%) and increased peripheral blood eosinophil ratio was found in 24%. This discrepancy between two groups was statisticalla significant(p<0.01). In regarding to sputum eosinophilia, increased sputum eosinophil ratio was in 14(32%) of 44 patients with chronic cough. Eleven patients(78%) of them had increased AHR. Only nine(30%) among 30 patients without sputum eosinophilia had increased AHR. These discrepancies between two groups were statistically significant(p<0.01). To predict AHR, sputum eosinophilia has 55% of sensitivity and 88% of specificity. Significant positive correlation between peripheral blood eosinophil ratio and sputum eosinophil ratio was found in patients with cough-variant asthma (r=0.76, p<0.01), We suggest that simple direct smear for sputum eosinophil count could be an useful test to diagnose the patients with chronic cough.
Subject(s)
Humans , Asthma , Cough , Eosinophilia , Eosinophils , Immunoglobulin E , Sensitivity and Specificity , Skin Tests , SputumABSTRACT
Primary Pulmonary leiomyosarcomas are rare tumors. These tumors may arise at any level of the lung in which smooth muscle fibers are found. The highest incidence of sarcoma is during the fourth decade whereas for bronchogenic carcinoma the maximum incidence is during the sixth and seventh decades. Leiomyosarcomas are aggressive and progressive. Treatment is mainly surgical. The chemotherapy is ineffective and the effectiveness of radiotherapy depends on the total dose of irradiation. Prognosis and significant survival rate are related to the size of the lesion. We report one patient with primary pulmonary leiomyosarcoma involving the right lower lung.
Subject(s)
Humans , Carcinoma, Bronchogenic , Drug Therapy , Incidence , Leiomyosarcoma , Lung , Muscle, Smooth , Prognosis , Radiotherapy , Sarcoma , Survival RateABSTRACT
In the treatment of aortoiliac disease, two methods could be considered. One is percutaneous transluminal angioplasty, and the other is bypass graft. However sometimes two methods could be combined in high risk patients with a lengthy unilateral occlusion of one iliac artery and less extensive obstruction of the contralateral one, since higher risk aortofemoral bypass surgery may be obviated by femoro-femoral bypass graft after percutaneous treatment of the less diseased iliac artery. We report two cases of arortoiliac disease treated with Strecker stent followed by femoro-femoral bypass graft.
Subject(s)
Humans , Angioplasty , Iliac Artery , Stents , TransplantsABSTRACT
In the treatment of aortoiliac disease, two methods could be considered. One is percutaneous transluminal angioplasty, and the other is bypass graft. However sometimes two methods could be combined in high risk patients with a lengthy unilateral occlusion of one iliac artery and less extensive obstruction of the contralateral one, since higher risk aortofemoral bypass surgery may be obviated by femoro-femoral bypass graft after percutaneous treatment of the less diseased iliac artery. We report two cases of arortoiliac disease treated with Strecker stent followed by femoro-femoral bypass graft.