[Dermatomyositis: clinical study of 34 patients]. / Dermatomyositis: klinikai megfigyelések 34 betegen.

Clinical data of 34 patients with DM, who have been treated during the years 1971 and 1998 were evaluated. 79% of the patients (27 patients) were female, 21% of them (7 patients) were male. 59% of the patients (20 pts) were between the ages of 41 and 50 years. The characteristic heliotrop rash were observed in 26 patients, Gottron's papules in 20 patients, poikiloderma in 2 patients, calcification, ulcers, Raynaud syndrome in 1 patient. 3 of the 34 patients presented with strongly itching erythematopapulosus symptoms, most prominently on the scalp. Cardiac involvement were present in 10 patients (29%), lung involvement in 8 patients (23%), gastrointestinal complaints in 11 patients, dysphagia, dysphonia in 4 patients, joint pain in 5 patients. Overlap syndrome--scleroderma-dermatomyositis, SLE-dermatomyositis--was present in 2 patients. 9 of the 34 patients (26%) suffered from malignant tumours: gastric, breast, lung, epipharynx carcinoma, malignant melanoma. 13 of the 34 patients have been treated with corticosteroids together with immunosuppressor agents, in most cases azathioprin was administered. Cyclosporin was given in 7 cases, chloroquine in 2 cases.

Effect of p40tax trans-activator of human T cell lymphotropic virus type I on expression of autoantigens.

The possibility of a retroviral etiology has long been raised in a number of autoimmune disorders. More recently, Sjögren's syndrome and rheumatoid arthritis were noted in transgenic mice carrying the tax gene of human T cell leukemia virus type I (HTLV-I). To evaluate the involvement of HTLV-I Tax in autoimmunity, its effect on expression of autoantigens was investigated. A metallothionein promoter-driven p40tax expression plasmid, pMAXRHneo-1, was stably transfected into Molt4 and Jurkat cells and the p40tax protein was induced with CdCl2. trans-Activation or trans-repression of autoantigens by HTLV-I Tax was studied by Western blot analysis utilizing autoantigen-specific murine monoclonal and rabbit polyvalent antibodies as well as sera from 161 autoimmune patients. Induction of p40tax of HTLV-I had no significant effect on levels of expression of common autoantigens U1 snRNP, Sm, Ro, La, HSP-70, topoisomerase I/Scl70, PCNA, and HRES-1. Expression of two potentially novel autoantigens, 44 and 46 kDa, was induced by p40tax as detected by sera of progressive systemic sclerosis patients, BAK and VAR. By contrast, expression of 24- and 34-kDa proteins was suppressed in response to induction of p40tax as detected by sera of systemic lupus erythematosus patients PUS and HOR. Because none of these patients were infected by HTLV-I, a protein functionally similar to p40tax may be involved in eliciting autoantigen expression and a subsequent autoantibody response in a minority of patients with PSS and SLE. Sera of autoimmune patients may also be utilized to detect novel proteins trans-activated or trans-repressed by p40tax of HTLV-I.

Human T-cell lymphotropic virus (HTLV)-related endogenous sequence, HRES-1, encodes a 28-kDa protein: a possible autoantigen for HTLV-I gag-reactive autoantibodies.

The presence of a human T-cell lymphotropic virus (HTLV)-related endogenous sequence, HRES-1, in the human genome has been documented. The HRES-1 genomic locus is transcriptionally active and contains open reading frames. Antibodies 232 and 233, specific for synthetic peptides pep14-24 and pep117-127, corresponding to two nonoverlapping HTLV-related regions in the longer open reading frame of HRES-1, recognize an identical 28-kDa protein in H9 human T cells. Thus, HRES-1 is a human endogenous retroviral sequence capable of protein expression. HRES-1/p28 is localized to the cytoplasm and nuclear bodies. While HTLV-I-specific antibodies react with HRES-1 peptides, antibody 233 cross-reacts with HTLV-I gag p24 protein. Three consecutive highly charged amino acid residues, Arg-Arg-Glu, present in both HRES-1 pep117-127 and HTLV-I gag p24 are likely to be the core of cross-reactive epitopes. The prevalence of antibodies to HRES-1 peptides pep14-24 and pep117-127 was determined in 65 normal blood donors and 146 patients with immunological disorders. Sera of patients with multiple sclerosis (19 out of 65, 29%), progressive systemic sclerosis (4 out of 17, 23%), systemic lupus erythematosus (4 out of 19, 21%), and Sjogren syndrome (2 out of 19, 10%) contained significantly higher HRES-1 peptide binding activity than sera of normal donors. Sera of patients with AIDS showed no specific binding to HRES-1 peptides. Nine of 30 HRES-1-seropositive patients showed immunoreactivity to HTLV-I gag p24. The data indicate that HRES-1/p28 may serve as an autoantigen eliciting autoantibodies cross-reactive with HTLV-I gag antigens.

Clinical evaluation and pathomechanism of urticaria-like skin eruption in systemic lupus erythematosus.

Clinical observations of 10 SLE patients having urticaria-like skin symptoms showed that the clinical process of urticaria found in patients suffering from SLE differs from the classic one. On the basis of histological and immunohistological studies in 5 cases, a characteristic histological manifestation of leukocytoclastic vasculitis of small veins in the upper third of the cutis was found. The various urticaria-like skin symptoms in SLE are caused by immunocomplexes of various components. The continuous presence of urticaria is caused by C-activating formation of anaphylatoxin. The appearance of urticaria in SLE patients is an unfavourable precursor which has been confirmed by statistical analysis.

[Electron microscopic changes in the liver in systemic lupus erythematosus]. / Szisztémás lupus erythematosusban jelentkezo májelváltozások elektronmikroszkópos vizsgálata.

Liver biopsy specimen of a patient with systemic lupus erythematosus was studied by light- and electron microscopy. On the background of the nonspecific hepatitis of a mild degree ultrastructural changes of the liver were revealed. Diffuse damage to the mitochondria and the vascular pole of the cells have been found. In the vascular endothelial cells of the liver presence of the so called tubuloreticular inclusions was observed. This can be regarded as the manifestation of the systematic disease in the liver.

Isomorphic skin reaction with DNCB in SLE and DLE.

DNCB sensitization was studied in 76 cases of systemic and 44 of discoid lupus erythematosus and in 101 immunosuppressed and tumor patients as controls, as well as in 40 contact-sensitized eczematous and 115 unselected patients. Sensitization of patients with discoid LE and that of mixed patients are of the same order, while it is lower in the systemic LE group. The highest incidence was found in the polysensitized positive control group; the lowest in the immunosuppressed and tumor patients. DNCB sensitization showed clear age dependency in the different groups. At the site of the DNCB exposure, isomorphic reactions were found in 43% of discoid and 25% of systemic LE patients. Histological and immunofluorescence studies supported the findings of the clinical picture, i. e. the appearance of isomorphic reactions at the site of the exposure cannot be related either to the intensity of the early reaction or with the developing intensity.

Immune-complex phagocytosis by human polymorphonuclear granulocytes.

A new two-step centrifugation technique is described for the rapid preparation of viable pure polymorphonuclear granulocytes from 9.5 ml of venous blood. The phagocytosis of these cells was stimulated by soluble DNA-anti-DNA complexes, produced in vitro by incubating high mol. wt. DNA with sera obtained from patients with severe SLE. The measure of phagocytosis was the amount of nitroblue-tetrazolium (NBT), reduced by the active microphages. The reduced NBT was estimated by counting the formazane positive cells or by extracting and measuring it spectrophotometrically. The immune-complex phagocytosis of control human granulocytes showed a linear relationship with the cell count per reaction mixture and displayed a bell-shaped dose-response curve as function of the immune-complex. The immune-complex phagocytosis by SLE granulocytes was decreased by about 40 per cent as compared to normal granulocytes.