A Case of Behcet's Disease with Pericarditis, Thrombotic Thrombocytopenic Purpura, Deep Vein Thrombosis and Coronary Artery Pseudo Aneurysm

Behcet's disease with concomitant thrombotic thrombocytopenic purpura (TTP), coronary artery stenosis and coronary artery pseudo aneurysm is rare. Here we report a case of Behcet's disease with several cardiovascular complications, namely: pericarditis, deep vein thrombosis (DVT), TTP, coronary artery stenosis, and a coronary artery pseudo aneurysm. A 37-year-old female presented with sudden dyspnea and syncope at our emergency room and underwent pericardiectomy and pericardial window formation for the diagnosis of cardiac tamponade with acute hemorrhagic pericarditis. Thereafter, TTP and DVT complicated her illness. After confirmation of Behcet's disease on the basis of a history of recurrent oral and genital ulcers and erythema nodosum, remission was achieved after treatment with methylprednisolone pulse therapy, colchicine, catheter directed thrombolysis and thrombectomy. However, whilst maintaining anticoagulation therapy, a newly developed pericardial aneurysmal dilatation was noted on follow-up radiologic evaluation. Further evaluation revealed right coronary artery stenosis and a left coronary artery pseudo aneurysm; these additional problems were treated with the nonsurgical insertion of an endovascular graft stent . At the time of writing three months later after stent insertion, the aneurysm has continued to regress and no additional complications have intervened with combined immunosuppressive therapy.

Usefulness of Carotid Intima Media Thickness as Cardiovascular Risk Factor in Patient with SLE / 대한류마티스학회지

OBJECTIVE: The purpose of this study was to investigate the complication using carotid intima media thickness (CIMT) as an indicator for atherosclerosis and to verify the risk factors in systemic lupus erythematosus (SLE) patients compared with those of control group. METHODS: The study recruited 48 asymptomatic SLE and 32 healthy control group. These are examined by B-mode ultrasound (Acuson(TM) SequoiaC256 8 MHz linear transducer) to measure the CIMT at the far wall of the common carotid artery. A wide range of contribution factors to CIMT including age, body mass index, lipid profile, total cumulative steroid dose, SLEDAI, and proteinuria were considered. RESULTS: Although the results of CIMT in both groups were within normal limit (In healthy adult 0.25~1.5 mm, values > 1.0 mm often regarded as abnormal), the mean CIMT in patient group was 0.721+/-0.162 mm, which was thicker than that of control group with 0.5219+/-0.096 mm (p<0.001). In multiple regression analysis, only age showed significant contribution to CIMT (p<0.01). Disease duration, SLEDAI and serum lipids were of no statistical significance with CIMT. CONCLUSION: We were to elucidate the advance of atherosclerosis with measuring CIMT and assess factors associated with SLE. Even if the results of CIMT in both groups were within normal range, it is significant that the arterial thickening is more advanced in SLE than that of healthy control. Further studies based on CIMT are needed to screen and prevent cardiovascular complications from disease progression.

A Case of Protein-losing Enteropathy Treated with High Dose Intravenous Glucocorticoid Therapy in Systemic Lupus Erythematosus / 영남의대학술지

Generalized edema and hypoalbuminemia are relatively common presenting manifestations in many clinical situations. The differential diagnosis of hypoalbuminemia include: Kwashiorkor, synthetic dysfunction of the liver, and excessive protein loss as in nephrotic syndrome. In systemic lupus erythematosus (SLE), hypoalbuminemia and generalized edema are most commonly due to protein loss associated with lupus nephritis; gastrointestinal involvement is uncommon, and therefore protein loss through the gastrointestinal tract is quite rare. We report a case of a protein losing enteropathy (PLE) associated with SLE. The patient was referred to our hospital for generalized edema, arthralgia and facial rash. After clinical evaluation, the patient met the criteria for the SLE diagnosis; hypoalbuminemia with general edema was consistent with a protein losing enteropathy. After two weeks of therapy with parenteral high dose glucocorticoid, the patients was improved in laboratory findings as well as clinical symptoms.

TNF-alpha in the Pleural Fluid for the Differential Diagnosis of Tuberculous and Malignant Effusion / 결핵및호흡기질환

BACKGROUND: Determining the cause of an exudative pleural effusion is sometimes quite difficult, especially between malignant and tuberculous effusions. Twenty percent of effusions remain undiagnosed even after a complete diagnostic evaluation, including pleural biopsy. The activity of tumor necrosis factor-alpha (TNF-alpha), which is the one of proinflammatory cytokines, is increased in both infectious and malignant effusions. The aim of this study was to investigate the diagnostic efficiency of TNF-alpha activity in distinguishing tuberculous from malignant effusions. METHODS: 46 patients (13 with malignant pleural effusion, 33 with tuberculous pleural effusion) with exudative pleurisy were included. TNF-alpha concentrations were measured in the pleural fluid and serum samples using an enzyme- linked immunosorbent assay (ELISA). In addition, TNF-alpha ratio (pleural fluid TNF-alpha : serum TNF-alpha) was calculated. RESULTS: TNF-alpha concentration and TNF-alpha ratio in the pleural fluid were significantly higher in the tuberculous effusions than in the malignant effusions (p0.05). The cut off points for the pleural fluid TNF-alpha level and TNF-alpha ratio were found to be 136.4 pg/mL and 6.4, respectively. The sensitivity, specificity and area under the curve were 81%, 80% and 0.82 for the pleural fluid TNF-alpha level (p<0.005) and 76%, 70% and 0.72 for the TNF-alpha ratio (p<0.05). CONCLUSION: We conclude that pleural fluid TNF-alpha level and TNF-alpha ratio can distinguish a malignant pleural effusion from a tuberculous effusion, and can be additional markers in a differential diagnosis of tuberculous and malignant pleural effusion. The level of TNF-alpha in the pleural fluid could be a more efficient marker than the TNF-alpha ratio.

A Case of Imatinib-mesylate associated Hypersensitivity Pneumonitis / 결핵및호흡기질환

Imatinib-mesylate (Gleevec, Glivec) is a protein-tyrosine kinase inhibitor that inhibits the Bcr-Abl tyrosine kinase created by the Philadelphia chromosome abnormality in CML. Imatinib is also used to treat patients with c-kit (CD 117)-positive unresectable tumors, or metastatic malignant gastrointestinal stromal tumors, or both. Imatinib is a well- tolerated drug with few side effects. However, it has been associated with gastrointestinal irritation, fluid retention and edema, skin rashes, depigmentation, hepatotoxicity, hemorrhage, and hematological toxicity (anemia, neutropenia, and thrombocytopenia). In addition, imatinib has been associated with dyspnea and cough, which are mainly secondary to the pleural effusion and pulmonary edema, which represent local or general fluid retention. These events appear to be dose related and are more common encountered in the elderly. However, there has been no report of hypersensitivity pneumonitis associated with imatinib-mesylate in Korea. We report a case of 51-year old woman who developed hypersensitivity pneumonitis that might have been induced by imatinib-mesylate during the treatment of a gastrointestinal stromal tumor.

Impaired Osteophyte formation in Patients with Type II Diabetes Mellitus and Knee Osteoarthritis / 대한류마티스학회지

OBJECTIVE: Insulin has been known as a potent growth factor for connective tissues including cartilage and bone.The pathogenesis of type 2 diabetes mellitus is the insulin resistance due to insulin receptor or postreceptor abnormalities.In the present study,we investigate whether radiographic features of knee osteoarthritis in patients with type 2 DM differ from those in nondiabetogenic controls with knee osteoarthritis. METHODS: Radiographs (knee)from 59 female patients with diabetes and knee osteoarthritis were compared with those from 74 female controls who were similar with respect to age,weight,and duration of OA symptoms.Based on the Kellgren and Lawrence criteria,an overall OA severity grade for the index knee was assigned and was rated for the severity of subchondral sclerosis,osteophytes, geodes and joint space narrowing.Patients with inflammatory arthritis such as RA,severe peripheral neuropathy,or diabetic nephropathy were excluded. RESULTS: Although the 2 groups were similar with respect to the severity of joint space narrowing,geode formation,and subchondral sclerosis,osteophytes formation was less common in patients with type 2 diabetes (P=0.041)than controls.In the patients with symptomatic knee OA at least for 11 years,marked osteophyte formation was noted only in 7 of the 17 with DM (41%),but in 15 of the 27 controls (55%). CONCLUSION: Our results suggest that diminished availability of insulin at the cellular level or diabetic microvascular disease may attenuate the chondro- and osteogenesis required for osteophyte formation in the joint of patients with OA.The clinical significance of osteophyte and the pathologic mechanisms of diminished osteophyte formation in patients of knee osteoarthritis with type 2 diabetes is to be determined.

Evaluation of Lung Permeability in Patients with Connective Tissue Disease using 99mTc-DTPA Aerosol Scintigraphy / 대한류마티스학회지

OBJECTIVE: The association between the connective tissue diseases and lung diseases is well established. DLCO and 99mTc-DTPA aerosol scintigraphy are used for evaluation of the alvelolar-capillary permeability. This study evaluated the changes in permeability of alveolar-capillary membrane and the utility of the 99mTc-DTPA aerosol clearance to detect lung involvement in patients with connective tissue diseases. METHODS: The patient group consisted of the patients with any proven connective tissue diseases (27 rheumatoid arthritis, 17 systemic lupus erythematosus, 7 other connective tissue diseases) and the control group consisted of healthy 12 persons. The patients and controls were non-smokers and had no concomitant diseases that could affect the result (diabetes, any lung diseases etc). Chest X-ray, spirometric measurements of lung volumes, flow idices, diffusing capacities and 99mTc-DTPA aerosol scintigraphy were performed in the patient group and control group. 99mTc-DTPA aerosol (1110 MBq) was used with the aero-vent jet nebulizer as a lung delivery system. Patients in sitting position inhaled for 5 minutes at normal tidal oral breathing, Scintigraphic data were recorded using the Picker Prism 2000 gamma cammera, 15 frames of the lung were obtained as the area of interest anteriorly and posteriorly (120 msec at each frame, for 30 minutes). 99mTc-DTPA clearance rate was calculated as the time to half clearance (T1/2). RESULTS: The mean clearance rates of 99mTc-DTPA were 64.0+/-24.1 min (RA 70.7+/-26.2 min, SLE 61.6+/-14.0 min, Others 43.9+/-24.7 min), and 47.0+/-10.3 min in the patient group and the control group respectively. Significant correlation was not found between the pulmonary clearance rate of 99mTc-DTPA and other parameters (disease duration, ESR, CRP, DLCO and FEV1/FVC). CONCLUSION: 99mTc-DTPA clearance in the patient group (RA, SLE, others) was significantly decreased than that in control group (p<0.05). In the patient group with normal chest X-ray, 99mTc-DTPA clearance in the connective tissue disorders was significantly decreased than control group (p<0.05). We suggest that 99mTc-DTPA aerosol scintigraphy may be one of useful technique for early detection of the lung involvement in the connective tissue disorders.

Evaluation of Lung Permeability in Patients with Connective Tissue Disease using 99mTc-DTPA Aerosol Scintigraphy / 대한류마티스학회지

OBJECTIVE: The association between the connective tissue diseases and lung diseases is well established. DLCO and 99mTc-DTPA aerosol scintigraphy are used for evaluation of the alvelolar-capillary permeability. This study evaluated the changes in permeability of alveolar-capillary membrane and the utility of the 99mTc-DTPA aerosol clearance to detect lung involvement in patients with connective tissue diseases. METHODS: The patient group consisted of the patients with any proven connective tissue diseases (27 rheumatoid arthritis, 17 systemic lupus erythematosus, 7 other connective tissue diseases) and the control group consisted of healthy 12 persons. The patients and controls were non-smokers and had no concomitant diseases that could affect the result (diabetes, any lung diseases etc). Chest X-ray, spirometric measurements of lung volumes, flow idices, diffusing capacities and 99mTc-DTPA aerosol scintigraphy were performed in the patient group and control group. 99mTc-DTPA aerosol (1110 MBq) was used with the aero-vent jet nebulizer as a lung delivery system. Patients in sitting position inhaled for 5 minutes at normal tidal oral breathing, Scintigraphic data were recorded using the Picker Prism 2000 gamma cammera, 15 frames of the lung were obtained as the area of interest anteriorly and posteriorly (120 msec at each frame, for 30 minutes). 99mTc-DTPA clearance rate was calculated as the time to half clearance (T1/2). RESULTS: The mean clearance rates of 99mTc-DTPA were 64.0+/-24.1 min (RA 70.7+/-26.2 min, SLE 61.6+/-14.0 min, Others 43.9+/-24.7 min), and 47.0+/-10.3 min in the patient group and the control group respectively. Significant correlation was not found between the pulmonary clearance rate of 99mTc-DTPA and other parameters (disease duration, ESR, CRP, DLCO and FEV1/FVC). CONCLUSION: 99mTc-DTPA clearance in the patient group (RA, SLE, others) was significantly decreased than that in control group (p<0.05). In the patient group with normal chest X-ray, 99mTc-DTPA clearance in the connective tissue disorders was significantly decreased than control group (p<0.05). We suggest that 99mTc-DTPA aerosol scintigraphy may be one of useful technique for early detection of the lung involvement in the connective tissue disorders.