A dextran sulphate gel column removed anti-double-stranded DNA antibodies (anti-dsDNA) selectively, efficiently, and safely from the circulation of a patient with systemic lupus erythematosus (SLE). The mechanism of the removal is thought to be due to cross reactivity of anti-dsDNA with dextran sulphate, which has negatively charged units. Selective removal of anti-dsDNA is expected to contribute not only to the treatment but also to elucidation of the pathogenesis of SLE.
A 28 year-old female patient who has been diagnosed as having systemic lupus erythematosus (SLE) developed an acute dissecting aneurysm of the aorta (DeBakey type I). The long-term, large dose corticosteroid therapy (i.e., accumulative dose of about 60 g) administered for the treatment of lupus nephritis (WHO class III----IV) was considered to be responsible for a hypercholesterolemia (300-560 mg/dl) and a steroid-dependent hypertension (WHO class III) in this patient. The autopsy findings for the aorta were compatible with atherosclerotic changes but not with lupus arteritis. While atherosclerotic cardiovascular complications have been considered to be rare in patients with SLE, a growing body of evidence suggests that the incidence of such a complication may be increasing along with a dramatic improvement in the longevity of patients with SLE after an introduction of a large dose, long-term corticosteroid therapy.
Based on analysis of data obtained from multicenter patients with collagen diseases (83 with pulmonary hypertension(PH) and 472 without PH), preliminary criteria for the diagnosis of PH in mixed connective tissue disease (MCTD) were proposed by the Research Committee for MCTD of the Ministry of Health and Welfare of Japan. The diagnosis of PH requires four or more out of six clinical and laboratory findings, including exertional dyspnea, systolic pulsation on the left sternum, increased 2nd pulmonary sound, dilatation of the pulmonary artery on chest X-ray, right ventricular hypertrophy on the electrocardiogram, and right ventricular enlargement on the echocardiogram. Alternatively, either an increase of mean pulmonary artery pressure over 25 mmHg measured by right ventricle catheterization, or the corresponding finding on Doppler echocardiography is also valid for the diagnosis of PH. When these criteria were applied to the patients in this study, the sensitivity was 92% and the specificity 100%, showing that PH may be adequately diagnosed using non-invasive methods. The number of criteria satisfied by patients with PH was well correlated with their mean pulmonary artery pressure measured by heart catheterization.
Tree criteria for the classification of pulmonary hypertension (PH) in mixed connective tissue disease (MCTD) were developed by stratifying patients into groups according to the physician’s diagnosis, mean pulmonary artery pressure (mPA) and prognosis, respectively. A classification tree for PH diagnosed by the physician was constructed with two criteria: dilatation of the pulmonary artery segment evident on chest roentgenography (or an accentuated pulmonic sound as a surrogate) and shortness of breath on exertion, which demonstrated a sensitivity of 96% and a specificity of 99%. A classification tree for PH diagnosed by mPA was also constructed with almost similar criteria: an accentuated pulmonic sound (or dilatation of the pulmonary artery segment evident on chest roentgenography as a surrogate) and shortness of breath on exertion, which demonstrated a sensitivity of 100% and a specificity of 100%. The prognostic classification tree was constructed with four criteria: an accentuated pulmonic sound, systolic pulsation at the left sternal border, shortness of breath on exertion and retro-sternal pain on exertion, which demonstrated a sensitivity of 62% and a specificity of 98%. The classification tree criteria for the diagnosis and prognosis of PH in MCTD were found to be accurate and useful for the screening of PH.
Adherent synovial cells from both 13 patients without rheumatoid arthritis (RA) (gout, osteoarthritis and meniscal lesion) and 8 patients with RA consisted of dendritic cells, macrophage-like cells and fibroblast-like cells after cloning in a similar fashion as reported in our previous paper. All the adherent synovial cells from patients without RA did not release interleukin 1 (IL-1) beta and prostaglandin E2 (PGE2) spontaneously, while those cells released comparable amounts of IL-1 beta, but not PGE2 to RA cells after type II collagen stimulation. Only the synovial cells from RA, irrespective of morphology and cloning, released IL-1 beta and PGE2 without stimulation. Nonrheumatoid synovial cells may differ functionally from RA cells.
