[Subacute meningococcal septicemia and temporary deficiency in complement factor C4 in lupus disease in an outbreak].
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Adult↗
Biomedical subjects
A Kahan
Publications and source records attributed to A Kahan.
Nifedipine and thallium-201 myocardial perfusion in progressive systemic sclerosis.
Heart disease in patients with progressive systemic sclerosis may be due in part to myocardial ischemia caused by a disturbance of the coronary microcirculation. To determine whether abnormalities of myocardial perfusion in this disorder are potentially reversible, we evaluated the effect of the coronary vasodilator nifedipine on myocardial perfusion assessed by thallium-201 scanning in 20 patients. Thallium-201 single-photon-emission computerized tomography was performed under control conditions and 90 minutes after 20 mg of oral nifedipine. The mean (+/- SD) number of left ventricular segments with perfusion defects decreased from 5.3 +/- 2.0 to 3.3 +/- 2.2 after nifedipine (P = 0.0003). Perfusion abnormalities were quantified by a perfusion score (0 to 2.0) assigned to each left ventricular segment and by a global perfusion score (0 to 18) for the entire left ventricle. The mean perfusion score in segments with resting defects increased from 0.97 +/- 0.24 to 1.26 +/- 0.44 after nifedipine (P less than 0.00001). The mean global perfusion score increased from 11.2 +/- 1.7 to 12.8 +/- 2.4 after nifedipine (P = 0.003). The global perfusion score increased by at least 2.0 in 10 patients and decreased by at least 2.0 in only 1. These observations reveal short-term improvement in thallium-201 myocardial perfusion with nifedipine in patients with progressive systemic sclerosis. The results are consistent with a potentially reversible abnormality of coronary vasomotion in this disorder, but the long-term therapeutic effects of nifedipine remain to be determined.
Adult↗
Autoantibodies against gp140, the Epstein-Barr virus and C3d receptor in sera from rheumatoid arthritis patients.
gp140 is the Epstein-Barr virus receptor and the C3d receptor (EBVR/C3dR) of human B lymphocytes. Recently, we have shown that cross-linking of EBVR/C3dR on cell surface by polyclonal anti-gp140 induced B cell activation, in presence of T cell factors. Immunoregulatory abnormalities of EBV-induced B cell activation have been demonstrated in rheumatoid arthritis (RA) patients. These data prompted us to analyze the putative presence of anti-EBVR/C3dR autoantibodies in human sera. The IgG fractions from eleven RA and 10 normal sera were tested for their ability to: (a) bind to Raji cell surface; (b) inhibit the binding to cell surface of 3 anti-EBVR/C3dR monoclonal antibodies (mAb), which recognized different epitopes on gp140; (c) inhibit the binding of particle-bound C3d and (d) react with 1% Nonidet-P40-solubilized gp140 from Raji cell membranes, in immunoblotting assays. Three RA sera carry anti-EBVR/C3dR autoantibodies which react with gp140 expressed on Raji cell surface or its solubilized form. The purification of monomeric IgG fraction of selected RA sera ruled out involvement of immune complexes carrying C3 molecules, which could interfere in these assays. One of these 3 RA sera was able to inhibit the binding to cell surface of anti-EBVR/C3dR mAb and particle-bound C3d. However, the 2 other RA sera, found positive by immunoblotting, did not inhibit particle-bound C3d and presented differences in their inhibitory effect on anti-EBVR/C3dR mAb binding to Raji cell surface. These data allow us to demonstrate differences which exist in the properties of anti-EBVR/C3dR autoantibodies. These autoantibodies were not detected in all the normal and other RA sera. Anti-EBVR/C3dR autoantibodies could play a role "in vivo" in B lymphocyte activation of RA patients.
Arthritis, Rheumatoid↗
Reduced coronary flow and resistance reserve in primary scleroderma myocardial disease.
The maximum coronary vasodilator capacity after intravenous dipyridamole (0.14 mg X kg-1 X min-1 X 4 minutes) was studied in seven patients with primary scleroderma myocardial disease and compared to that of seven control subjects. Hemodynamic data and left ventricular angiographic data were not different in the two groups. The coronary flow reserve was evaluated by the dipyridamole/basal coronary sinus blood flow ratio (D/B CSBF) and the coronary resistance reserve by the dipyridamole/basal coronary resistance ratio (D/B CR). Coronary reserve was greatly impaired in the group with primary scleroderma myocardial disease: D/B CSBF was lower than in the control group (2.54 +/- 1.37 vs 4.01 +/- 0.56, respectively; p less than 0.05) and D/B CR was higher than in the control group (0.47 +/- 0.25 vs 0.23 +/- 0.04, respectively; p less than 0.05). Such a decreased coronary flow and resistance reserve in patients with primary scleroderma myocardial disease was not explained by an alteration of left ventricular function. It may be an important contributing factor in the pathogenesis of primary scleroderma myocardial disease.
Adult↗
Defective Epstein-Barr virus specific suppressor T cell function in progressive systemic sclerosis.
Several immunoregulatory defects of Epstein-Barr virus (EBV) induced B cell activation have been described in patients with rheumatoid arthritis (RA), suggesting that EBV may have a role in the pathogenesis of RA. We assessed EBV specific T cell regulation in 20 patients with progressive systemic sclerosis (PSS) and immune to EBV and in 10 control subjects also immune to EBV by comparing the secretion of IgM into supernatants of 16 day cultures of B cells alone and cocultures of B and autologous T cells. In control subjects autologous T cells mediated a significant decrease in the secretion of IgM by B cells at 12 and 16 days of culture. Analysis of individual responses showed the existence of two subgroups of patients with PSS: group I (10 patients) had a suppressor T cell function similar to that of controls; group II (10 patients) had a defective T cell function. Differences in the duration or severity of the disease, the slow acting therapeutic agents, and anti-inflammatory drugs could not account for these subdivisions. These results suggest that several immunoregulatory defects of EBV induced B cell activation exist in different connective tissue diseases.
B-Lymphocytes↗
Pharmacodynamic effect of dipyridamole on thallium-201 myocardial perfusion in progressive systemic sclerosis with diffuse scleroderma.
We evaluated the effect of dipyridamole on thallium-201 myocardial perfusion in 23 patients with progressive systemic sclerosis (PSS) with diffuse scleroderma. Thallium-201 single photon emission computed tomography (SPECT) was performed at rest and after coronary artery vasodilatation with intravenous dipyridamole (0.14 mg/kg/min for four minutes). The left myocardium was divided into nine segments; each segment was graded as 2.0, 1.5, 1.0, 0.5, 0 (zero represents no activity). Dipyridamole significantly improved resting thallium-201 myocardial perfusion: the mean (SD) number of segments with thallium defects decreased from 6.0 (2.1) at rest to 4.1 (2.5) after dipyridamole (p less than 0.0001); the mean (SD) score in segments with resting defects increased from 0.92 (0.24) at rest to 1.13 (0.38) after dipyridamole (p less than 0.0001); the mean (SD) global score per patient increased from 10.2 (1.8) at rest to 11.4 (2.1) after dipyridamole (p less than 0.02); the global score increased by at least 2.0 in 12 patients and worsened by at least 2.0 in three patients only (p = 0.05). The results of this acute study suggest that some drugs with potent vasodilator activity on small coronary arteries may be beneficial in the treatment of PSS patients with thallium-201 myocardial perfusion abnormalities.
Adult↗
Experimental autoimmune spondylodiscitis in rats.
Immunization of Lewis rats with nucleus pulposus in incomplete Freund's adjuvant, followed by in vivo injury of nucleus pulposus, induced spondylodiscitis characterized by mononuclear cell infiltration affecting the vertebral discs in 100% of experimental animals. Injury of the nucleus pulposus without prior immunization, or immunization without injury failed to induce spondylodiscitis. The nucleus pulposus, that is not antigenic normally, can lose its immunologic tolerance by the injection of syngeneic nucleus pulposus. When immunocompetent cells come into contact with the nucleus pulposus after tissue injury, an autoimmune response may appear.
Animals↗
Increased expression of Epstein-Barr virus receptor on lymphoblastoid cell lines from subsets of patients with rheumatoid arthritis.
We studied the expression of Epstein-Barr virus receptor (EBVR/CR2) on lymphoblastoid cell (LCL) lines established from 23 patients with rheumatoid arthritis and the T cell suppression of IgM secretion by EBV activated B cells. Ten patients had normal T cell suppression of IgM secretion, whereas 13 patients had defective suppressor T cell function. EBVR expression on LCL was assessed using a rabbit anti-gp 140 IgG; Raji cells, used as reference cell line expressed 50,000 EBVR, a 140 k glycoprotein (gp 140). Patients with defective T cell suppression of IgM secretion by EBV activated B cells had a significantly higher EBVR expression on LCL than patients with normal T cell suppression (mean +/- SD; 50.8 +/- 23.8 vs 29.5 +/- 13.2, respectively; p less than 0.05). These data suggest a relationship between the T cell suppression defect and an increased EBVR expression on LCL from patients with RA.
Arthritis, Rheumatoid↗
[The coronary hyperemic reaction in scleroderma].
Functional, organic and vascular anomalies could play an important role in systemic scleroderma. We have studied the coronary hyperemic reaction in subjects with primitive sclerodermatous cardiomyopathy, in order to specify the vascular anomalies. The coronary hyperemic reaction consists of a transient rise in coronary flow after selective injection of a contrast agent into the left coronary artery. In sclerodermatous subjects this coronary hyperemic reaction is significantly smaller than in control subjects. This limitation of the hyperemic reaction reflects a decrease in the ability of small arteries or coronary arterioles to dilate in sclerodermatous subjects. This anomaly could play an important role in primitive sclerodermatous cardiomyopathy.
Cardiac Catheterization↗
[Myasthenia induced by D-penicillamine during the treatment of rheumatoid polyarthritis].
Seven cases of myasthenia induced by D-penicillamine treatment of rheumatoid arthritis are presented. The 86 cases published so far are reviewed. The clinical symptoms do not differ from those of the idiopathic form. Progress is rapid and, generally, good. The duration and dosage of the treatment are not involved. The presence of acetylcholine-antireceptor antibodies confirms the autoimmune origin of the condition. Possible mechanisms by which this disorder could be induced are discussed. Investigation of HLA phenotypes suggests that the genetic basis of induced myasthenia differs from that of the idiopathic form (increased prevalence of DR1).
Adult↗
Meningoradiculitis associated with infections by Borrelia burgdorferi.
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Adult↗
Nifedipine and esophageal dysfunction in progressive systemic sclerosis. A controlled manometric study.
We evaluated the effect of the calcium channel blocking agent, nifedipine, on esophageal dysfunction in 15 patients with progressive systemic sclerosis, using a double-blind, randomized, crossover, placebo-controlled manometric study. Nifedipine significantly decreased lower esophageal sphincter pressure in these patients; this reduced lower esophageal sphincter pressure may cause gastroesophageal reflux. Thus, nifedipine may have detrimental effects on progressive systemic sclerosis patients.
Adolescent↗
Decreased coronary reserve in primary scleroderma myocardial disease.
We assessed coronary reserve, by measuring the increase in coronary sinus blood flow (CSBF) after intravenous administration of dipyridamole (0.14 mg/kg/minute for 4 minutes), in 7 patients with primary scleroderma myocardial disease (PSMD) and in 7 control subjects. Coronary reserve was greatly impaired in PSMD: before administration of dipyridamole, CSBF was similar in patients with PSMD (89 +/- 32 ml/minute/100 gm, mean +/- SD) and in controls (100 +/- 15 ml/minute/100 gm); after dipyridamole infusion, CSBF was significantly lower in patients with PSMD (191 +/- 45 ml/minute/100 gm) than in controls (399 +/- 58 ml/minute/100 gm) (P less than 0.01). Six of the 7 patients with PSMD had angiographically normal epicardial coronary arteries and normal left ventricular function. Decreased coronary reserve may be an important contributor to the pathogenesis of primary scleroderma myocardial disease.
Adult↗
Different defects of T cell regulation of Epstein-Barr virus-induced B cell activation in rheumatoid arthritis.
Several reports have shown a defective Epstein-Barr virus (EBV)-specific suppressor T cell function in rheumatoid arthritis (RA), suggesting that EBV may have a role in the pathogenesis of RA. EBV-specific T cell regulation was studied in 47 EBV-immune RA patients and in 14 EBV-immune control subjects by comparing the secretion of IgM into supernatants of 28-day cultures of B cells alone and cocultures of B and autologous T cells. In control subjects, autologous T cells mediated a significant decrease in the secretion of IgM by B cells at 12 and 16 days of culture. Analysis of individual responses demonstrated the existence of 3 subgroups of RA patients: group I (18 patients) had a suppressor T cell function similar to that of controls; group II (21 patients) had a defective T cell function; group III (8 patients) was characterized by a "late help phenomenon." Moreover, in RA group III, IgM secretion in cultures of B cells alone was lower than that seen in controls, RA group I, or RA group II. Differences in the duration or severity of the disease, or in the use of slow-acting therapeutic agents, corticosteroids, and nonsteroidal antiinflammatory drugs could not account for these subdivisions. Thus, our study demonstrates that several immunoregulatory defects exist in subgroups of RA patients.
Adult↗
Nifedipine and alpha 1-adrenergic blockade in Raynaud's phenomenon.
The efficacy of nifedipine and prazosin in the treatment of Raynaud's phenomenon was assessed in a prospective double-blind randomized cross-over trial in 15 patients. Each patient received one week of nifedipine 20 mg TID, one week of prazosin 1 mg TID, and 2 weeks of placebo. Nifedipine was shown to be effective in reducing both the frequency and the severity of Raynaud's phenomenon, whereas prazosin was ineffective. Before initiation of therapy in the 15 patients, pressor responses to the intravenous alpha 1-agonist phenylephrine were assessed in the basal state, 30 min after 20 mg oral nifedipine, and 30 min after 1 mg oral prazosin; the shift to the right of the log dose-vasopressor response curves to phenylephrine was similar with nifedipine and prazosin.
Adult↗
Libman-Sacks endocarditis: the diagnostic importance of two-dimensional echocardiography.
In 1980, a 44-year-old woman with the diagnosis of systemic lupus erythematosus presented with fever and mitral and aortic valvular insufficiency. Blood cultures were sterile. M-mode echocardiograms of the mitral valve revealed an image resembling vegetations of infective endocarditis. The diagnosis was rejected after two-dimensional echocardiography which failed to demonstrate vegetation. Two-dimensional echocardiography should therefore be systematically utilized in the diagnosis of valvular lesions in patients with systemic lupus erythematosus.
Adult↗
A randomised double-blind trial of diltiazem in the treatment of Raynaud's phenomenon.
The efficacy of diltiazem in the treatment of Raynaud's phenomenon was assessed in a prospective double-blind randomised cross-over trial in 16 patients (7 progressive systemic sclerosis, 2 rheumatoid arthritis, 1 systemic lupus erythematosus, and 6 idiopathic Raynaud's phenomenon). Each patient received diltiazem 120 mg and placebo three times a day for two weeks. The attack frequency of Raynaud's phenomenon was measured by a diary count; its severity was assessed by means of a 10 cm visual analogue scale with 0 representing minimum and 10 representing maximum severity. Diltiazem significantly decreased the frequency and severity of Raynaud's phenomenon as compared with placebo. This improvement was striking in patients with idiopathic Raynaud's phenomenon but did not reach statistical significance in patients with associated systemic disease. We conclude that diltiazem is effective in the treatment of Raynaud's phenomenon, especially in patients with idiopathic vasospastic disease.
Adolescent↗
Prevention with molsidomine of coronary artery spasm caused by alkalosis.
A provocative test of coronary artery spasm caused by alkalosis was used to evaluate a possible anti-coronary artery spasm effect of molsidomine. The rapid infusion of an alkaline buffer followed by maximal voluntary hyperventilation in 10 patients with angina at rest led to the appearance of anginal pain and significant, transient ischemic changes of the ST segment resulting from alkalosis-induced coronary spasm. A second provocative test was performed under the same conditions, 24 hours later, after the administration of 4 mg of molsidomine. Molsidomine prevented the development of coronary artery spasm in 8 of the 10 patients in the study group. These preliminary results justify further clinical evaluation of molsidomine in the treatment of vasospastic angina.
Alkalosis↗