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Results for “Scleroderma, Systemic”

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PubMed1999

Localized subcutaneous Nocardia farcinica abscess in a woman with overlap syndrome between systemic scleroderma and polymyositis.

Nocardia farcinica is an increasingly common cause of human infection in Europe. We observed an isolated nodular subcutaneous abscess due to N. farcinica in the forearm of a young patient with overlap syndrome between systemic sclerosis and polymyositis. Despite in vitro resistance to trimethoprim-sulfamethoxazole, this oral combination completely resolved the lesion within 3 weeks and was maintained for 6 months. The spectrum of cutaneous nocardiosis in the immunocompromised host is discussed.

Abscess↗
PubMed1993

Antinuclear antibodies in patients with scleroderma (systemic sclerosis) and in their blood relatives and spouses.

OBJECTIVE: To test the postulate that there is a higher prevalence of antinuclear antibodies (ANAs) in serum samples from blood relatives and from spouses of patients with scleroderma than in control samples, and that this provides evidence for both genetic and environmental factors influencing autoimmunity in scleroderma. METHOD: Testing for ANAs was performed on 58 patients with scleroderma, 30 of their spouses, 74 first degree relatives, and 66 control subjects broadly age matched to the patients, their spouses, and about half of the relatives (siblings and parents). RESULTS: On the basis of an ANA titre of > 40 as positive, 12 (18%) of the controls, 55 (95%) of the patients, one (3%) of the spouses and five (7%) of the relatives would be classified as positive. Thirty seven (64%) of the patients had defined specificities (ACA, Scl 70, U1 (RNP)) but none of the controls, spouses, or relatives had antibodies of these specificities. CONCLUSION: These findings give no support to the postulate that environmental or genetic factors contribute to the ANAs in scleroderma.

Adult↗
PubMed1998

[Finger tip necroses after dihydroergotamine medication in limited systemic scleroderma].

A 78 year old women developed acute fingertip necrosis just a few days after starting dihydroergotamine. The lesions healed in 3 weeks after the medication was stopped. The patient had suffered from Raynaud syndrome for 5 years and limited systemic sclerosis was diagnosed during the necrotic episode. Advanced age and microangiopathies are contraindications to the use of ergot-containing preparations.

Aged↗
PubMed1985

[Esophageal motility in Raynaud's disease, systemic scleroderma and presclerodermal Raynaud's syndrome].

Fifty-five consecutive subjects with Raynaud's phenomenon were submitted to esophageal manometry. According to the results of clinical examination and periungual capillaroscopy, they were divided into three groups: sixteen patients presented presclerodermal Raynaud's phenomenon, and nineteen, systemic sclerosis; twenty others had Raynaud's disease. Twenty volunteers formed the control group. Esophageal motor disorders were observed in the group with presclerodermal Raynaud's phenomenon, affecting the esophageal lower sphincter and the peristalsis of the body of the esophagus. In the group with systemic sclerosis, these motor disorders were associated with alterations in the amplitude and the duration of contractions. Esophageal motility was normal in subjects with Raynaud's disease. We can conclude that: 1) esophageal motor disorders can be seen in presclerodermal Raynaud's phenomenon. The kind of abnormalities observed at this stage of the disease are in favor of an early alteration of the neurogenic component of esophageal motility; 2) there is no relationship between esophageal motor disorders and Raynaud's phenomenon in scleroderma.

Adult↗
PubMed1999

[Extracapillary glomerulonephritis with anti-myeloperoxidase antibodies in 2 patients with systemic scleroderma treated with penicillamine D].

BACKGROUND: Side-effects, including autoimmune disorders, are frequent with D-penicillamine therapy. Proteinuria is observed in 10% of the patients, often secondary to extramembranous glomerulopathy. Necrotizing extracapillary glomerulonephritis is however exceptional. CASE REPORTS: Two patients with systemic sclerodermia were treated with D-penicillamine for 7 and 14 years. Both developed necrotizing extracapillary glomerulonephritis with anti-myeloperoxidase antibodies (anti-MPO), associated with hemorrhagic alveolitis in one case. Partial regression of the renal failure was obtained after withdrawal of D-penicillamine and combination treatment with prednisone and cyclophosphamide. DISCUSSION: Extracapillary glomerulonephritis or a lung-kidney syndrome are frequently associated with anti-MPO antineutrophil cytoplasm antibodies (ANCA). In systemic sclerodermia, the presence of anti-MPO appears to define a group of patients at risk of pauci-immune extracapillary glomerulonephritis or a lung-kidney syndrome. In addition, the presence of ANCA in patients with renal failure would suggest extracapillary glomerulonephritis rather than sclerodermic microangiopathy. Development of extracapillary glomerulonephritis with anti-MPO in patients who are taking D-penicillamine suggests that inductor mechanisms other than D-penicillamine are involved in the pathogenesis of these glomerulopathies.

Aged↗
PubMed2003

[Tumor necrosis factor alpha in systemic scleroderma].

The authors present the review of the literature on the role of tumor necrosis factor alpha (TNF alpha) in scleroderma systematica (SS). It is shown that TNF alpha participates in activation of vascular endothelium, regulation of immune response and metabolism of the connective tissue by modulation of fibroblastic function. SS patients exhibit a systemic and local rise of TNF alpha content. This rise contributes to SS progression, development of fibrosing alveolitis and skin fibrous alterations in Raynaud's syndrome.

Disease Progression↗
PubMed1989

[Von Willebrand factor multimers in systemic scleroderma].

In scleroderma endothelial cell damage plays an early and pivotal role in the pathogenesis of sclerotic lesions. In our study we determined whether or not plasma from 11 consecutive patients with scleroderma contained a subset of larger than normal (supranormal) multimers of von Willebrand factor which are potent inducers of platelet aggregation and adhesion. Supranormal multimers were found in all patients, but in none of the normal controls. Supranormal multimers may contribute to the pathogenesis of systemic sclerosis by inducing platelet aggregation and enhancing adhesion to subendothelium.

Adult↗
PubMedDate not supplied

[Distribution of HBs-(Australia) antigen in progressive systemic scleroderma and other connective tissue disorders].

Of 173 patients, mostly suffering from collagen diseases and rheumatoid disease, the HBs antigen (HBsAg) was present in 1.73%. This figure is no higher than that observed among blood donors from the same area (Naples and surroundings). One HBsAg positive patient suffered from chronic active hepatitis and rheumatoid arthritis; another from chronic active hepatitis and a secondary type of reticuloendotheliosis; the third was one of seven patients with psoriatic arthritis. HBsAg was not found in any of 48 patients with other collagen diseases, including 22 patients with Progressive Systemic Sclerosis. These data do not support the hypothesis based on observations of polyarteritis nodosa that HBsAg plays an ethiopathogenetic role in collagen diseases with serious vasculitis.

Arthritis, Rheumatoid↗
PubMedDate not supplied

The role of vascular endothelium in fibroblast activation and tissue fibrosis, particularly in scleroderma (systemic sclerosis) and pachydermoperiostosis (primary hypertrophic osteoarthropathy).

The vascular endothelium is an organ that is distinguished by its versatility and ability to modulate its surroundings. Endothelial cells (EC) interact with a variety of cell types including fibroblasts (FB), smooth muscle cells, circulating mononuclear cells, platelets and other cell types. This paper will focus on the interaction between endothelial cells and fibroblasts in the process of fibrosis. Vascular changes are described in most human fibrotic models, i.e. radiation, bleomycin, wound healing and particularly in scleroderma. FB migration to the perivascular spaces and proliferation is seen in the early settings of tissue fibrosis. The role of EC in this process is not precisely known; however, the EC contribution to fibrosis is likely to be multifactorial and may involve a spectrum of delicate mechanisms and an array of chemical signals. The induction of FB chemotaxis and FB proliferation by the EC's release of chemotactic factors and mitogens is one possible pathway of FB activation. Another major mechanism involves EC mobilization, guidance and regulation of mononuclear cell infiltration in the perivascular spaces and the subsequent impact of that process on FB activation. Histologic studies of human fibrotic disorders have emphasized early FB proliferation. The role of EC in the induction of FB proliferation should be evaluated carefully in order to understand human fibrosis.

Endothelium, Vascular↗
PubMed1984

[Hemodynamic response of the digital artery to heat and cold in normal subjects, in systemic scleroderma and in Raynaud's disease].

Measurement of digital systolic pressure under hot and cold conditions provides objective data (in mm Hg) of the humero-digital gradient and of spasm induced by cold. Normal values are: digital systolic pressure at 30 degrees C: 94 +/- 12% of humeral systolic pressure; at 15 degrees C: 94.46 +/- 9.32%, and at 10 degrees C: 93.6 +/- 12% of digital systolic pressure at 30 degrees C. The significance of these two parameters resides in the fact that the humero-digital gradient is of etiological value in organic arterial disease, the specificity of the test being 94.4% in allowing distinction between Raynaud's disease and systemic sclerosis. The cold-induced spasm can provide quantitative evaluation of a clinical Raynaud's phenomenon with which it is correlated (specificity: 96%).

Adolescent↗
PubMed2004

Impaired rectoanal inhibitory response in scleroderma (systemic sclerosis): an association with fecal incontinence.

Gastrointestinal abnormalities in systemic sclerosis (SSc) involve both myogenic and neural mechanisms. The aims of this study were to evaluate the rectoanal inhibitory response (RAIR) in SSc patients and to correlate RAIR with duration and subtype of disease, antibody status, and lower gastrointestinal symptoms. Thirty-five patients with SSc completed a questionnaire and underwent anorectal manometry (ARM). Forty-five patients without SSc served as controls. In the 35 SSc patients, 62.3% reported diarrhea, 57.1% reported constipation, and 37.1% reported fecal incontinence. Twenty-five of the 35 scleroderma patients (71.4%) demonstrated an impaired or absent RAIR, compared with none of the 45 controls (P < 0.001). Eleven of 13 incontinent SSc patients (84%) had an impaired RAIR. No correlation was found between RAIR and duration or subtype of SSc, antibody status, or presence of diarrhea or constipation. Impaired RAIR was closely correlated with fecal incontinence, suggesting a possible neural mechanism for maintenance of continence.

Adult↗
PubMed2000

Systemic scleroderma in Greece: low mortality and strong linkage with HLA-DRB1*1104 allele.

OBJECTIVE: Description of Greek patients with scleroderma with reference to (a) major organ disease, (b) autoantibodies, (c) survival rate, and (d) HLA associations. METHODS: The clinical files of 254 patients were analysed retrospectively and a standardised clinical chart was completed with age at disease onset, sex, date of first and last visit, clinical and serological findings, organs affected, reasons for death, and HLA class II alleles. HLA class II alleles (DRB1, DQA1, DQB1, DPB1) were determined by polymerase chain reaction amplification using oligopeptide probes. DNA was extracted from 98 patients and 130 Greek controls. RESULTS: 124 patients (49%) had limited systemic sclerosis (lSSc), 114 (45%) had diffuse systemic sclerosis (dSSc), and 16 (6%) had overlap syndromes. Patients with dSSc, compared with lSSc, were characterised by a higher prevalence of lung disease (p=0.0011), oesophageal, heart, and peripheral vessel disease (p=0.027, p=0.0025, and p=0.012, respectively). Anticentromere antibodies (ACA) occurred exclusively in lSSc (34%), whereas antibodies to topoisomerase I (anti-topo I) were associated with dSSc (p<0.0001). Anti-topo I were associated with interstitial pulmonary fibrosis, oesophageal and peripheral vessel disease (p=0.028, p=0.012, and p=0.01, respectively). The HLA-DRB1*1104 allele was associated with the disease (p<0.0001) and anti-topo I (p<0.001), whereas it was not associated with ACA serum reactivity (p<0.001). Renal disease occurred in 4% of patients with SSc. The estimated survival probability for this cohort of patients with SSc, four years after the first visit, is 94.8%. CONCLUSION: SSc among Greek subjects has the same pattern of organ disease as in other white populations. However, the prevalence of kidney disease is low. The HLA class II DRB1*1104 allele is associated with the disease, with anti-topo I, and not associated with ACA serum reactivity.

Adult↗