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PubMed1989

[Measurement of anticardiolipin antibodies and its significance in systemic lupus erythematosus].

A method for detection of anticardiolipin (ACL) antibodies with enzyme-linked immunosorbent assay was developed. Microtitre plates were coated with cardiolipin at a concentration of 20 micrograms/ml by evaporation under 4 degree centigrade overnight. Non-specific binding of diluted sera was eliminated by blocking of plates with 10% fetal calf serum in phosphate buffered saline (FCS/PBS) for 2 hours at room temperature. Sera (50 microliters/well) at a dilution of 1:100 were incubated for 2 hours at room temperature. Horseradish peroxidase conjugated rabbit anti-human IgG, IgM, IgA at a dilution of 1:2000, 1:1000, 1:500 respectively was added to the wells, and incubated for one and half hours at room temperature. The results were read at 490nm after incubation with substrates at 37 degree centigrade. 85 patients with systemic lupus erythematosus (SLE), 45 with rheumatoid arthritis (RA), 25 with progressive systemic scleroderma (PSS), and 18 primary Sjogren's syndrome were tested. The frequency of ACL antibody in SLE (48%) was much higher than that in RA (11%), PSS (12), SS (5.5). Three isotypes of ACL (IgG, IgM, IgA) were detected in the study with predominance of IgG isotype. ACL antibody was significantly associated with thrombosis, cutaneous vasculopathy, thrombocytopenia, and spontaneous abortion in patients with SLE. Strong relationship between ACL antibody and lupus anticoagulant was found. There was no correlation between ACL and anti-DNA antibodies, nor was ACL and VDRL test. The level of ACL antibody could be reduced by use of corticosteroids.

Arthritis, Rheumatoid↗
PubMed1991

Prevalence and characteristics of anti-single-stranded DNA antibodies in localized scleroderma. Comparison with systemic lupus erythematosus.

Prevalence, levels, and immunoglobulin classes of anti-single-stranded DNA antibodies were determined by an enzyme-linked immunosorbent assay in 52 patients with localized scleroderma (33 with morphea, four with generalized morphea, and 15 with linear scleroderma), in 60 healthy controls, and, for comparison, in 31 patients with systemic lupus erythematosus. Localized scleroderma revealed a significant prevalence of anti-single-stranded DNA antibodies, mainly characterized by high levels and IgM and IgA isotypes. Comparison of antibody characteristics in different clinical forms of localized scleroderma showed some significant differences (levels and immunoglobulin isotypes). Comparison with systemic lupus erythematosus showed that frequency, high levels, and IgG isotype of anti-single-stranded DNA antibodies significantly prevailed in systemic lupus erythematosus, while the IgM isotype significantly prevailed in localized scleroderma. However, generalized morphea and linear scleroderma did not significantly differ from systemic lupus erythematosus as regards antibody frequency and prevalence of high antibody levels.

Adult↗
PubMed1999

Increased in vitro production of interleukin 6 in response to trimethoprim among persons with trimethoprim induced systemic adverse reactions.

OBJECTIVE: Trimethoprim occasionally triggers a systemic adverse reaction including fever, malaise, head and backache, and even overt meningeal irritation, particularly in women with an autoimmune rheumatic disease. To study the unknown pathogenesis of the reaction we measured the effect of trimethoprim upon the cytokine [interleukin (IL) 2, 6, 10, and tumor necrosis factor-alpha] production of trimethoprim reactive and tolerant persons' peripheral blood mononuclear cells in vitro. METHODS: Peripheral blood mononuclear cells from 12 women reactive to trimethoprim (3 with primary Sjögren's syndrome, 3 with systemic lupus erythematosus, 1 with systemic scleroderma, 5 with no rheumatic disease) were cultured in the presence of trimethoprim, and the cytokine production was measured. Eleven women who tolerated trimethoprim (6 with Sjögren's syndrome and 5 with no rheumatic disease) served as controls. RESULTS: Therapeutic trimethoprim concentration induced in the mononuclear cells of the trimethoprim reactive patients significantly higher IL-6 production [mean +/- SD (median), 2034+/-2965 (572) pg/ml] versus cells of the trimethoprim tolerant subjects [954+/-2552 (89) pg/ml; p = 0.036]. No significant differences in the production of other cytokines were detected. CONCLUSION: Trimethoprim induces IL-6 production in the peripheral blood mononuclear cells of trimethoprim reactive persons. We suggest that IL-6 production is the probable trigger leading to the clinical reaction.

Adult↗
PubMed1995

Limited scleroderma is associated with increased prevalence of macrovascular disease.

Microvascular involvement in scleroderma is well recognized. Macrovascular disease is not. OBJECTIVE. To test the hypothesis that the prevalence of macrovascular disease is increased in patients with limited scleroderma (systemic sclerosis, lSSc). METHODS. A retrospective cohort study design was employed in which the prevalence of macrovascular disease in all female patients from specified hospitals (1974-90) with lSSc of at least 5 years' duration was compared and contrasted with that in a comparable group of controls. Each control was matched to one lSSc case by sex; age (+/- 5 yrs); number of inpatient admissions (+/- 2); year of last hospital admission (+/- 2 yrs), history of hypertension, cigarette smoking and diabetes mellitus, and medical record number most closely approximating the case. The distribution of vascular disease was assessed in the peripheral, coronary, and cerebral arterial territories. RESULTS. Peripheral macrovascular disease (PVD) occurred in 18 (58%) of the lSSC patients and only 3 (9.6%) of the controls (RR = 6.0; 95% CI 2.0-18). Of the 18 lSSc cases, 8 had PVD documented angiographically, 4 by arterial Doppler ultrasound, and 6 had absent peripheral pulses. Five of these required subsequent partial limb amputation. Two of the 3 controls with PVD had absent peripheral pulses, and 1 had an angiographically documented abdominal aortic aneurysm. No control required limb amputation. There was no significant difference in the prevalence of coronary artery or cerebrovascular disease between the groups. CONCLUSION. The prevalence of peripheral large vessel occlusive disease is increased in lSSC and associated with severe morbidity.

Adult↗
PubMed2002

[A case of amyopathic dermatomyositis with systemic sclerosis sine scleroderma presenting abnormal vermiculation].

A 24-year-old Japanese woman was admitted to our hospital in January 2000 with daily diarrhea, abdominal distention, and abdominal pain. Raynaud's phenomenon with erythroderma desquamativum eruptions had been found in 1992. In 1999, small intestinal transillumination showed dilation of the second and third portion of the duodenum. She was diagnosed as amyopathic dermatomyositis because of Gottron's sign despite not having symptoms of myositis, normal serum CPK levels and no histological abnormality by muscle biopsy. In addition, the patient was diagnosed as systemic sclerosis sine scleroderma because Raynaud's phenomenon, leukoma and gastrointestinal dilation were present. Also, antinuclear antibody and anti centromere antibody were positive and anti Scl-70 antibody was dull-positive, despite the absence of scleroderma, extreme edema, and bone resorption. Careful, long term observation will be required because of the splanchnopathy in youth and the complications of systemic sclerosis.

Adult↗
PubMed1996

Epidemiological analysis of prognosis of 496 Japanese patients with progressive systemic sclerosis (SSc). Scleroderma Research Committee Japan.

For the first time, we performed an epidemiological study of SSc in Japan to study the factors influencing prognosis, survival rate and cause of death of Japanese SSc patients and to compare our data with those from foreign countries. Prognosis of 496 Japanese patients with progressive systemic sclerosis (SSc) was analyzed based on clinical data described in case cards provided by the members of the Scleroderma Research Committee of the Japanese Ministry of Health and Welfare. The essential observation period was from 5 to 20 years, at ending in 1994. Ninety patients died (males 11, females 79). The age of onset of the deceased patients was significantly higher than that of surviving patients (deceased, 45.6 yrs, surviving 41.3 yrs). Statistically significant factors for a poor prognosis were as follows: Barnett type III > type II > type I, positive for anti-Scl-70 antibody, negative for anti-centromere antibody. The survival rate at 5 years after the onset of the disease was 0.937, followed by 0.82 at 10 years, 0.567 at 20 years and 0.40 at 30 years after the onset. Sex was not a predictor for prognosis, although male patients died at an earlier stage of the disease. The most common causes of death were heart failure, pulmonary insufficiency, lung fibrosis, and renal failure. Twenty-four patients had cancer of which 13 were lung cancers. The current status of the survival rate and prognostic factors of 496 Japanese SSc patients is summarized. In future, more well-controlled studies using the same criteria should be performed for the better understanding and management of SSc.

Adult↗
PubMed1998

Oral iloprost treatment in patients with Raynaud's phenomenon secondary to systemic sclerosis: a multicenter, placebo-controlled, double-blind study.

OBJECTIVE: To evaluate the efficacy and tolerability of an oral preparation of iloprost, a prostacyclin analog, in patients with Raynaud's phenomenon (RP) secondary to systemic sclerosis (scleroderma). METHODS: A multicenter, randomized, parallel-group, placebo-controlled double-blind study was performed at university and community-based medical centers. Patients were randomly assigned to receive either 50 microg of iloprost orally twice daily or an identical gelatin-coated capsule containing placebo for 6 weeks. Outcome measures included average total daily duration of RP attacks, average number of RP attacks, and RP condition scored via a standardized daily diary. RESULTS: Three hundred eight patients with scleroderma (272 women, 36 men, mean age 49 years [range 18-80]) were enrolled. One hundred fifty seven were assigned to receive iloprost and 151 to receive placebo. One hundred forty-three patients in the iloprost group (91.1%) and 144 in the placebo group (95.4%) completed the 6-week treatment phase. Fifteen of these treated patients (8 iloprost, 7 placebo) failed to complete all of the followup visits. The mean reduction in the average duration of attacks from baseline to week 5-6 was 24.32 minutes in the iloprost group and 34.34 minutes in the placebo group (P = 0.569). Likewise, the mean reduction from baseline to week 5-6 in the daily frequency of attacks was 1.02 in the iloprost group and 0.83 in the placebo group (P = 0.459). The Raynaud's condition score, a patient-completed assessment of the severity of RP attacks, was reduced by 1.32 in the iloprost group and 1.00 in the placebo group (P = 0.323). The lack of significant difference between treatment groups did not change when a variety of factors, including use of other vasodilators, duration of disease, classification of scleroderma (limited versus diffuse), or number of baseline digital ulcers were taken into account. Premature withdrawal from the study due to adverse events occurred in 10 patients (6.4%) in the iloprost group and 3 (2.0%) in the placebo group (P = 0.058). CONCLUSION: Oral iloprost at a dosage of 50 microg twice daily is no better than placebo for management of RP secondary to scleroderma, either during 6 weeks of treatment or during 6 weeks of posttreatment followup.

Administration, Oral↗
PubMed1978

Fibrinogen turnover in progressive systemic sclerosis.

Large amounts of fibrin are seen in the intima of the renal arterioles in progressive systemic sclerosis (scleroderma). The half-life or half disappearance time of plasma fibrinogen in 15 patients with scleroderma was studied using 125I fibrinogen to find whether there is an increased turnover of plasma fibrinogen paralleling this morphologic abnormality. Patients had a more rapid fibrinogen turnover than normal controls (60.7 versus 90.6 hours); the subgroup of patients with "progressive" scleroderma had a more rapid fibrinogen half-life than those with "stable" scleroderma (56.5 versus 73.2 hours). The mean fibrinogen half-life of 8 patients given intravenous heparin increased to within one standard deviation of normal, a finding that suggested that the fibrinogen molecule in these patients was capable of normal survival. There was a considerable variation of normal survival. There was a considerable variation of fibrinogen half-lives in individual scleroderma patients over time (not seen in the normal controls) which may be the result of intermittently increased fibrinogen consumption.

Adult↗
PubMed1983

Capillary microscopy in eosinophilic fasciitis. A comparison with systemic sclerosis.

Capillary microscopy was performed on 19 patients with eosinophilic fasciitis. These patients were compared with 13 individuals with progressive systemic sclerosis (scleroderma). Capillary patterns were normal in 16 of 19 (84%) eosinophilic fasciitis patients; 3 exhibited either borderline or nonspecific changes, and none showed a definite scleroderma pattern. In contrast, characteristic nailfold capillary changes, consisting of both dilatation and loss of capillaries, were present in 11 of 13 (85%) scleroderma patients; the remaining 2 showed scleroderma-type abnormalities of only 1 finger and were, therefore, classified as borderline. These results suggest that capillary microscopy may help to distinguish these 2 disorders.

Adolescent↗
PubMed1983

67Gallium lung scans in progressive systemic sclerosis.

67Gallium lung scans were performed in 19 patients with progressive systemic sclerosis (scleroderma). Results were expressed quantitatively as the 67Gallium Uptake Index. The mean total pulmonary 67Gallium Uptake Index in patients was significantly higher than that in controls (41 versus 25), and 4 patients (21%) fell outside the normal range. There were no clinical or laboratory variables that correlated with the 56gallium uptake. Increased pulmonary 67gallium uptake in scleroderma may prove useful as an index of pulmonary disease activity.

Adolescent↗
PubMed1978

Atypical esophageal diverticula associated with progressive systemic sclerosis.

Five cases of unusual esophageal diverticula associated with progressive systemic sclerosis (scleroderma) or collagen vascular disease are presented. These wide-mouthed saccular diverticula were infrequently seen in a group of PSS patients with the typical motility disturbance of esophageal involvement and are reminiscent of the diverticula of the colon involved by PSS.

Adult↗
PubMed1998

[Extracorporeal photopheresis in therapy-refractory disseminated discoid lupus erythematosus].

Lupus erythematosus (LE) is a disease with a wide spectrum of cutaneous and systemic manifestations. Discoid LE (DLE) is the most common form of cutaneous LE; the disseminated form of DLE is rare. We report an encouraging response to treatment with extracorporeal photopheresis (ECP) in a single patient with disseminated DLE who did not respond to conventional therapy. To the best of our knowledge this is the first successful use of ECP in the management of such a patient. Extracorporeal photopheresis is a therapeutic modality that has been under investigation for more than 12 years. Although originally developed for the treatment of cutaneous T-cell lymphoma, ECP has recently been used for the management of autoimmune diseases including systemic scleroderma, pemphigus vulgaris and SLE, as well as prevent organ rejection in patients with cardiac or kidney transplants and graft versus host disease after bone marrow transplantation.

Dose-Response Relationship, Drug↗
PubMed2002

Rheumatoid syndrome associated with lung interstitial disorder in a dental technician exposed to ceramic silica dust. A case report and critical literature review.

Exposure to silica minerals is associated with silicosis and autoimmune disorders, especially systemic scleroderma. Evidence of this association has been increasingly reported in the last decade. The aim of this paper is to discuss, on the basis of a literature review, the case of a 28-year-old female dental technician who suffered from episodes of weakness, arthralgia, pain, swelling and stiffness of the fingers, dyspnoea with cough, a positive Waaler-Rose reaction, increased rheumatoid factor and normal ESR. She was a non-smoker. A rheumatoid syndrome with lung interstitial disorder, associated with silica exposure from dental ceramic products, was diagnosed. The patient had the HLA-A2-A31, HLA-B51-B18 and HLA-DR3-DR11 haplotypes, some of which are associated with autoimmune disease susceptibility. A 6-month follow-up, with adequate protection and without treatment, showed disappearance of the symptomatology and negative tests for Waaler-Rose reaction and rheumatoid factor. Exposure to silica should, therefore, be sought in the history of any patient with autoimmune or lupus-like syndrome and pulmonary changes. Symptoms associated with silica dust exposure from dental ceramic products should be recognised as being due potentially to an occupational disease, and dental technicians should be protected as workers at risk.

Adult↗
PubMed2005

Microscopic polyangiitis after silicone breast implantation.

We describe the case of a patient who developed microscopic polyangiitis (MPA) after silicone breast implantation. A 60-year-old woman who had undergone silicone breast implantation was admitted to our hospital with complaints of general malaise and hematoproteinuria. She was diagnosed as having MPA with evidence of acute progressive renal failure, pulmonary hemorrhage, and positivity for myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA). A renal biopsy showed severe necrotizing and crescentic glomerulonephritis with arteriolitis. The patient received high-dose steroids and plasma exchange treatment, but died of progressive pulmonary hemorrhage and multiple cerebral hemorrhage. Silicone implantation is associated with scleroderma, systemic lupus erythematosus, and rheumatoid arthritis. This case report indicates the possibility of the development of MPA after silicone breast implantation.

Acute Kidney Injury↗