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PubMedDate not supplied

Clinical subsets of scleroderma: relevance of fluorescent and precipitating antinuclear antibodies.

Sera from 7 patients with localized and 35 with systemic scleroderma were studied for the presence of fluorescent antinuclear antibodies (FANA) (by indirect immunofluorescence on HEp-2 cells) and antibodies to extractable nuclear antigens (anti-ENA) (by immunodiffusion - ID - and counterimmunoelectrophoresis - CIE). In localized disease, antinuclear autoimmunity was limited to 1 FANA positive serum (14%); in systemic disease, the prevalence of FANA was 94% and that of anti-ENA ranged from 29% to 49% (by ID and CIE, respectively). The commonest ENA system, Scl-70, could be easily detected by CIE, in spite of the reported basic nature of the antigen. The anticentromere antibody occurred only in patients with acrosclerosis (7/26-27%), whereas the association of nucleolar + homogeneous FANA, as well as the anti-Scl-70, were found more frequently in diffuse scleroderma (9/9-100% and 6/9-67%, respectively). The presence of the anticentromere antibody excluded that of any anti-ENA, while a close association was found between nucleolar + homogeneous FANA and the anti-Scl-70. Pulmonary involvement was significantly more frequent in nucleolar + homogeneous FANA positive patients; moreover, in two cases the same pattern proved to predict the development of diffuse scleroderma.

Antibodies, Antinuclear↗

PubMed1988

The diagnosis and classification of scleroderma (systemic sclerosis).

Difficulty in the diagnosis of the disease scleroderma may occur at the early stage prior to the development of obvious skin sclerosis. A presumptive diagnosis may be made if Raynaud's phenomenon is accompanied by a positive 'neck test', 'scleroderma' capillary changes in the nailfolds or antinuclear antibodies. Definitive diagnosis may have to be delayed for several years from the onset of Raynaud's phenomenon until definite characteristic skin changes are seen. Ten cases in which an earlier diagnosis of scleroderma was not substantiated are listed. The earlier incorrect diagnosis would have been avoided by use of the methods described in this paper. Various terms have been used to denote subdivisions of scleroderma. These include acrosclerosis, diffuse scleroderma and CREST. We have used the terms Type 1, Type 2 and Type 3 based on the early extent of the skin sclerosis where Type 1 (limited extent) indicates sclerodactyly only, Type 2 (moderate extent) indicates sclerosis proximal to the metacarpophalangeal joints but excluding the trunk and Type 3 (extensive) indicates diffuse skin sclerosis including the trunk. The clinical value of this simple classification is reviewed and contrasted to other classifications which appear to be poorly defined and of limited use.

Adult↗

PubMed2002

Prostacyclin for pulmonary hypertension.

BACKGROUND: Primary pulmonary hypertension (PPH) is progressive, resulting in right ventricular failure. Survival seldom exceeds five years. Pulmonary hypertension can be idiopathic or associated with other conditions. It is common in patients with diffuse scleroderma and the CREST syndrome where it is clinically, haemodynamically and prognostically indistinguishable from idiopathic primary pulmonary hypertension. Prostacyclin is a potent vasodilator and inhibitor of platelet aggregation. Iloprost is a chemically stable derivative of prostacyclin with similar biologic properties and can be given orally, by infusion or nebulised. OBJECTIVES: To determine the efficacy of prostacyclin or one of its analogues in idiopathic primary pulmonary hypertension. SEARCH STRATEGY: A search was carried out using the Cochrane controlled clinical trial register. SELECTION CRITERIA: Randomised controlled trials (RCTs) involving patients with primary pulmonary hypertension were selected by two reviewers. DATA COLLECTION AND ANALYSIS: Study quality was assessed and data extracted independently by two reviewers. Outcomes were analysed as continuous and dichotomous outcomes, using standard statistical techniques. MAIN RESULTS: Four RCTs were included, all of short duration (8-12 weeks). Three compared intravenous epoprostenol with conventional therapy, two were in PPH (n = 81 and n = 21) and one with pulmonary hypertension in scleroderma (n = 111). One compared intravenous Iloprost with placebo in 14 patients with scleroderma. Exercise capacity improved in patients treated with intravenous epoprostenol (Standardised Mean Difference) 0.69, 95% Confidence Intervals (CI) 0.40, 0.97. Several haemodynamic variables improved in the group treated with epoprostenol; e.g. mean pulmonary artery pressure fell: Weighted Mean Difference -6.3 mmHg (95% CI -3.9, -8.7). Mortality was improved Peto Odds Ratio 0.32 (95% CI 0.13, 0.77), although this effect was not significant when analysed using a more conservative random effects model: Odds Ratio 0.32 (95% CI 0.06, 1.58). Side effects and adverse events related to the indwelling catheter (sepsis and thrombosis) were common. REVIEWER'S CONCLUSIONS: Intravenous prostacyclin over 12 weeks improves exercise capacity, NYHA functional class and several cardiopulmonary haemodynamic variables. It may improve mortality.

Antihypertensive Agents↗

PubMed1996

A multicenter trial of recombinant human interferon gamma in patients with systemic sclerosis: effects on cutaneous fibrosis and interleukin 2 receptor levels.

OBJECTIVE: To evaluate the acute toxicity, potential efficacy, and effects on the soluble interleukin 2 receptor (sIL-2R) of recombinant human interferon gamma (rIFN-gamma) in patients with systemic sclerosis (SSc). METHODS: A multicentered, pilot clinical trial of rIFN-gamma was performed on 20 patients (15 women, 5 men, mean age 45 years) with active cutaneous SSc (mean disease duration 36 months) to evaluate it potential as a novel therapy for this untreatable disorder. After one week of rIFN-gamma 0.01 mg/m2/day, subjects self-administered rIFN-gamma 0.1 mg/m2/day intramuscularly for a total of 18 weeks. The major outcome variable was a modified skin score (0 = normal skin, 3 = hidebound skin) measured and summed from 15 anatomic areas of the body. sIL-2R levels were measured by ELISA at entry and exit from the study. RESULTS: The clinical results were modest at best. Nine of 20 patients achieved at least a 20% reduction in skin score, with one patient showing almost total remission of all skin abnormalities. The mean skin score at entry for all subjects was 22.8 +/- 8.9 and over the course of the trial improved marginally compared to baseline (mean change -4.72 +/- 6.62; p = 0.008). However, 8 subjects did not change appreciably while in the trial. Antibodies to Scl-70 were observed in only 5 patients (all with diffuse scleroderma) and were not associated with either response to or complications from therapy. The adverse reactions were frequent and occasionally severe. Ten subjects were withdrawn because of exacerbation of Raynaud's symptoms (n = 5), constitutional symptoms (n = 2), development of renal crises (n = 2), and mild pancytopenia (n = 1). Minor laboratory abnormalities were common and included elevation of cholesterol, triglycerides, hepatic transaminases, and reduction in white blood cell count. Compared to controls, mean sIL-2R was markedly elevated at entry (1309 +/- 495 U/ml; p = 0.0001) and did not change appreciably at exit. Spearman correlation analysis showed a trend but no statistically significant association of skin score with sIL-2R (R = 0.408; p = 0.074). However, sIL-2R was significantly correlated with erythrocyte sedimentation rate (R = 0.542; p = 0.0165). A subset analysis revealed that skin score (p = 0.0001) and sIL-2R (p = 0.00170) were significantly higher at baseline for patients with diffuse scleroderma compared to patients with limited disease. CONCLUSION: rIFN-gamma may be beneficial for some patients with SSc, but the benefit appears marginal for most individuals and the side effects frequent. Although sIL-2R was elevated in many of the patients with SSc, it did not appear to be correlated with activity of cutaneous disease or response to therapy.

Adult↗

PubMed1996

Clinical and laboratory features of patients with scleroderma and silicone implants.

We reviewed the available clinical and laboratory data from 56 patients with scleroderma and silicone implants from the English medical literature and 19 cases which have not been previously reported. The average age of onset of scleroderma was 43.6 +/- 10 years (range 20-73). Patients had silicone implants for an average of 9 +/- 4 years prior to the development of scleroderma (range 1-32). Most patients had limited scleroderma (41%). Twenty three percent had intermediate scleroderma and 36% had diffuse scleroderma. Clinical findings included: Raynaud's phenomenon in 77%, esophageal dysfunction in 53%, and pulmonary involvement in 47%. Cardiac and renal involvement were uncommon. Antinuclear antibodies by immunofluorescence were found in 83 percent of patients. The immunofluorescence pattern was speckled in 53%, centromere in 31% and nucleolar in 9%. Other antibodies (Scl-70, RNP, SSA/Ro, PM-Scl) were found in only a small proportion of patients. A clinical, serologic and immunogenetic comparison of patients with silicone implants and scleroderma and patients with idiopathic scleroderma is needed to better understand the pathogenesis of this disorder.

Adult↗

PubMed1972