Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Scleroderma, Diffuse”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,189 records · Page 66Linked to original sources
PubMed1996

Pregnancy outcome in systemic sclerosis.

The outcome of pregnancy in systemic scleroderma (SSc) has been a subject of much interest. In a retrospective analysis of 59 pregnancies of 17 mothers with SSc, the effect of disease on pregnancy outcome was variable. There was no adverse effect on fertility. While there was an apparent decrease in fertility in the limited cutaneous disease, this was not seen in the diffuse variety. There was an decreased incidence of live birth after the onset of disease as compared prior to the onset of disease. The foetal wastage after the onset of disease was contributed equally by spontaneous abortions as well as induced terminations of pregnancies. The presence of anticardiolipin antibodies was not related with adverse outcome. The effect of pregnancy on disease was variable with the disease being stable in five patients, regressing in five and progressing in three. The increased terminations of pregnancy reflects the uncertainities of positive outcome both by parent and attending physicians.

Adolescent↗
PubMed1987

An autoimmune antibody from scleroderma patients recognizes a component of the plant cell nucleolus.

An auto-antibody from human serum of patients with the autoimmune disease scleroderma was used to localize the nucleolus in meristematic cells of onion and soybean roots using indirect immunofluorescence microscopy. Similar lots of antiserum recognized a single 34 kD, nucleolar protein, fibrillarin, in a variety of animal cells (Ochs et al. 1984, 1985). In both plants, antibody linked fluorescence is associated with the one to several nucleoli present in the interphase nucleus. The fluorescence becomes diffuse around condensing prophase chromosomes and becomes more diffused at metaphase with slightly more intense fluorescence surrounding the chromosomes. At anaphase-telophase the fluorescence is localized in dense areas within the chromosomes, presumably representing prenucleolar bodies which will form the interphase nucleoli of the daughter nuclei. This antiserum provides a new, valuable tool for the study of the nucleolus and the highly conversed nucleolar antigen(s) that it recognizes.

Antibodies, Antinuclear↗
PubMed1994

Localization of endothelin-1 and its binding sites in scleroderma skin.

OBJECTIVE: Endothelin-1 (ET-1) has been implicated in the pathogenesis of systemic sclerosis (SSc) as it is both a potent vasoconstrictor and fibroblast mitogen and is raised in the circulation of patients with SSc and primary Raynaud's phenomenon. METHODS: We examined the localization and level of expression of ET-1 and its putative receptors in clinically "uninvolved" (i.e., prescleroderma skin) and involved skin from patients with diffuse cutaneous systemic sclerosis (dcSSc), using the alkaline phosphatase antialkaline phosphatase technique while ET-1 binding sites were examined using in vitro autoradiography. RESULTS: There was an increase in dermal ET-1 staining in clinically uninvolved and involved skin from patients with early active dcSSc compared with late stage fibrotic SSc skin and normal skin from healthy volunteers. Increased ET-1 staining was associated predominantly with the superficial vessels in the SSc skin sections. In addition, there was a significant increase in [125I]ET-1 binding to superficial vessels and the dermal/epidermal junction in SSc skin compared with the binding to similar structures in normal tissue. There was no change in [125I]ET-1 binding to the deep dermal vessels in both SSc and normal skin. This increase in [125I]ET-1 binding in SSc skin was not maintained with increasing tissue fibrosis. CONCLUSION: The presence of increased ET-1 levels as well as its binding sites in both the prescleroderma and involved skin of patients with dcSSc compared to controls suggests that ET-1 may play a role in the pathology of dermal fibrosis and vasoconstriction in SSc.

Adult↗
PubMed1978

Chronic cutaneous graft-versus-host disease in man.

This clinicopathologic study of patients with chronic graft-versus-host disease (GVHD) after allogeneic marrow transplantation emphasizes the most prominent feature of the syndrome, the cutaneous aspects, and describes the ophthalmic-oral sicca syndrome with sialoadenitis and the neurologic findings. Chronic cutaneous GVHD affected 19 of 92 recipients surviving 150 days or more. In 6 patients chronic GVHD presented as a continuation of acute GVHD; in 8 it occurred after the resolution of acute GVHD; and in 5 it arose without preceding acute GVHD, ie, de novo late onset. Two cutaneous types were distinguished. The generalized type affected 16 patients and ran a progressive course resulting in late complications of poikiloderma, diffuse dermal and subcutaneous fibrosis, and contractures. Microscopically, it resembled generalized morphea and lupus erythermatosus hypertrophicus et profundus. The local type affected 3 patients with a more variable picture of poikiloderma, dermal sclerosis, and contractures. Microscopically, it resembled lupus of erythematosus profundus and scleroderma. Guidelines for defining and subclassifying chronic cutaneous GVHD are proposed.

Adolescent↗
PubMed1988

Main manifestations of carditis in diffuse connective tissue diseases in children.

238 children with diffuse connective tissue diseases were clinically examined with the use of echography. The presence was documented of characteristic morphofunctional changes in the heart in collagen diseases. Characteristic haemodynamic changes (predominantly contraction disturbances in systemic lupus erythematodes and dermatomyositis, and relaxation disturbances in systemic scleroderma) were identified.

Child↗
PubMed1987

Immunoglobulin G subclass distribution of autoantibodies in systemic sclerosis, primary biliary cirrhosis, and overlap syndromes.

The IgG subclass reactivities of six anticellular antibodies were measured by indirect immunofluorescence on HEp2 cells using murine monoclonal antibodies to the four human IgG subclasses. Patients with scleroderma, primary biliary cirrhosis (PBC), systemic lupus erythematosus, and mixed connective tissue disease were studied. Anticentromere antibody (ACA) was virtually all IgG1 and 3; antibody to multiple nuclear dots (NSpI) was IgG1, 2, and 3; antimitochondrial antibody was mainly IgG2 and 3; nucleolar staining was varied in subclass reactivity but most often IgG4; the diffusely grainy staining associated with Scl-70 antibody was chiefly IgG1; and the speckled pattern associated with anti-RNP antibody was always IgG1 and 4, with IgG2 and 3 in some cases. These data fail to support the hypothesis that the various patterns of autoimmune disease reflect differences in the biological properties of the associated antibodies. The prominence of IgG2 in antibodies associated with PBC suggests the possibility of an immune response independent of T cells in that condition. Differential subclass staining showed an unexpectedly high frequency of antibody to multiple nuclear dots in ACA positive sera, and such patients (all with CREST syndrome) could be at increased risk of developing PBC later.

Antibodies, Antinuclear↗
PubMed1990

[Pulmonary manifestation of progressive systemic scleroderma: prognostic value of centromere antibodies and antibodies to Scl-70 nucleoprotein].

We examined 74 patients with systemic sclerosis. 21 of them (= 28%) had Scl-70 antibodies in their blood serum and 12 (= 16%) were anticentromere antibody positive, whereas in 41 cases (= 56%) none of these antibodies could be detected. In patients with Scl-70 antibodies lung function tests showed a decrease in vital capacity (p less than 0.05), total capacity (p less than 0.01), and diffusing capacity (p greater than 0.05/n.s.) as compared to ACA-positive patients. A limitation of pulmonary function was seen in 76% of Scl-70-positive patients, in 44% of antibody-negative patients, and in only 33% in the ACA-positive group. A disabling respiratory limitation was found in 14% of Scl-70-positive patients, and in 7% of antibody-negative patients, whereas none of the ACA-positive patients had a higher degree of respiratory insufficiency. We conclude that pulmonary involvement in systemic sclerosis is most frequent and severe in patients with Scl-70 antibodies; it is relatively rare and of minor severity in ACA-positive patients. Antibody-negative patients are in between these extremes.

Adolescent↗
PubMed2001

[Autoantibodies to vasoactive peptides and angiotensin converting enzyme in patients with systemic diseases of the connective tissue].

AIM: To estimate the level of natural autoantibodies (NAAb) to angiotensin-converting enzyme (ACE) and endogenic mediators affecting vascular tone (bradykinin--BK, angiotensin II--AII, vasopressin--VP) as well as the activity of serum ACE in patients with systemic diseases of the connective tissue. MATERIAL AND METHODS: Levels of NAAb were measured by enzyme immunoassay in sera from 30 patients with SLE, 19 patients with rheumatoid arthritis (RA) and 36 patients with scleroderma systematica (SS). Serum from donors served control. IgM NAAb to ACE were measured by a new technique. Serum ACE activity was determined by the initial velocity of hydrolysis reaction using spectrofluometry. RESULTS: IgM NAAb were detected in the sera of both patients and donors. SS patients had the level of NAAb to ACE in diffuse form significantly higher than in limited (p < 0.05). In SLE and SS patients ACE activity was significantly lower (p < 0.05) than in healthy subjects and RA patients. Levels of NAAb to BK was significantly elevated (p < 0.01) in patients with SLE and RA vs donors while to AII in SS patients it was lowered (p < 0.001). Patients with diffuse SS had NAAb to BK higher than patients with SS limited form (p < 0.01). In SLE the lowest levels of NAAb to all the mediators studied were observed in patients with nephritis, for NAAb to VP the differences were significant (p < 0.05). In patients with urinary syndrome concentration of NAAb to BK was significantly higher (p < 0.01), differences between their levels in patients with nephritis and urinary syndrome were also significant (p < 0.05). CONCLUSION: Further studies are needed for specification of physiological or pathological role of NAAb to endogenic mediators.

Angiotensin II↗
PubMed1991

[Pulmonary arterial hypertension and systemic diseases].

Certain system diseases which include vascular involvement may be accompanied by pulmonary artery hypertension (PAHT). This chiefly concerns connective tissue disorders, notably scleroderma, CREST syndrome and mixed connective tissue disease and, to a lesser extent, systemic lupus erythematosus. The onset of PAHT in relation to vasculitis of the great vessels or necrosing angeitis is rare. It may result from pulmonary artery vasoconstriction of hypoxic origin during diffuse interstitial pulmonary fibrosis, a thrombo-embolic mechanism, a vasomotor phenomenon equivalent at pulmonary level to Raynaud's syndrome, and above all from pulmonary vasculitis, the commonest mechanism. The incidence of PAHT is such connective tissue disease is probably underestimated since it is a complication often unrecognised clinically. It is a major cause of death in such conditions, certain of which nevertheless enjoy a benign reputation. With the exception of rare cases of favourable outcomes under the influence of corticosteroids and immunosuppressants in cases of mixed connective tissue disease, no treatment has in fact been shown to be effective in terms of survival.

Connective Tissue Diseases↗
PubMed1996

Lung involvement in systemic sclerosis (scleroderma): relation to classification based on extent of skin involvement or autoantibody status.

Lung involvement accounts for significant morbidity and is a leading cause of mortality in patients with systemic sclerosis (SSc). It has been shown that different patterns of pulmonary involvement are seen in different subtypes of SSc. This paper reports a retrospective review of 72 patients with SSc to determine whether disease classification according to the extent of skin involvement alone (diffuse vs. limited) or autoantibody status was predictive of pulmonary parenchymal involvement. The diagnosis of interstitial lung disease was based on pulmonary function tests and chest radiographs. Restrictive lung disease was common in both limited SSc (lSSc) and diffuse SSc (dSSc), occurring in 30% and 50% of these patients respectively (P = 0.16). Radiographic evidence of significant interstitial disease was also comparable between the groups [nine of 32 lSSc patients (28%) vs. six of 17 dSSc patients (32%), P = n.s.]. No significant difference in mean lung function was found between patients with anti-Scl 70 antibody (n = 12) compared to those without (n = 60) (TLC 79.0 +/- SE 5.1% predicted vs. 82.8 +/- 2.2, P = n.s.; DLCO 63.0 +/- 5.1 vs. 59.7 +/- 2.5, P = n.s.). By contrast, statistically significant differences in mean lung function were found between patients with anticentromere antibody (ACA) (n = 24) and those without ACA (n = 48) (TLC 98.6 +/- SE 3.9% predicted vs. 79.7 +/- 3.1%, P < 0.001); and less frequent radiographic evidence of severe interstitial disease (0 of 17 with significant interstitial changes on chest radiograph vs. 15 of 32 (47%), P = 0.002). It is concluded that classification of SSc patients on the basis of the distribution of skin involvement poorly predicts the occurrence of interstitial lung disease. On the other hand, ACA is highly associated with the absence of interstitial lung disease.

Adult↗
PubMed1993

Lung disease in systemic sclerosis (scleroderma).

Scleroderma (SSc) is a disease characterized by skin fibrosis but it is the end-organ effect of microvascular injury and fibrosis that is important prognostically. Pulmonary involvement in SSc patients, either of parenchymal fibrosis and/or pulmonary hypertension, is a major cause of morbidity and mortality. Interstitial lung disease occurs more commonly in patients with diffuse SSc and is associated with a loss of lung volume, as well as a defect of gas exchange. Parenchymal fibrosis may also cause pulmonary hypertension. Isolated pulmonary hypertension occurs exclusively in patients with limited SSc and is detectable by a reduced DCO. The early identification of either manifestation is difficult. Patients may have minimal symptoms, unremarkable physical findings, normal chest radiographs and/or minimally abnormal pulmonary function tests at a time when significant lung pathology is present. It is essential to attempt to identify pulmonary disease early, at a potentially reversible stage. Multiple therapeutic endeavours have yielded only short-term or minimal benefits in symptoms and pulmonary function, and thus a major alteration in SSc pulmonary prognosis has not been achieved. Further study of the pathogenesis of this disease manifestation will be helpful in its earlier identification and intervention.

Humans↗
PubMed1982

Pericapillary fibrin in the ulcer-bearing skin of the leg: the cause of lipodermatosclerosis and venous ulceration.

Forty-one biopsy specimens, taken from the ulcer-bearing skin of 41 legs of 21 patients attending the varicose vein clinic, were selectively stained for fibrin with phosphotungstic acid haemotoxylin before being blindly assessed,. Layers of fibrin were found surrounding the dermal capillaries in all 26 legs with lipodermatosclerosis. None of the specimens from the 15 legs with clinically normal skin contained fibrin. There was also an increased number of dermal capillaries cut in cross section per high powered field in 24 of the 26 legs with lipodermatosclerosis compared with two of the 15 legs with normal skin (p less than 0.001). The mean reduction in foot vein pressure during exercise was significantly less in the 26 limbs with pericapillary fibrin than in the other 15 limbs (p less than 10(-6). Lipodermatosclerosis is synonymous with pericapillary fibrin deposition and is associated with, and probably secondary to, both a persistently raised venous pressure and an increase in the size of the dermal capillary bed. This extravascular deposition of fibrin probably stimulates tissue fibrosis and blocks the diffusion of oxygen to the overlying epidermis, producing cellular death and venous ulceration.

Capillaries↗
PubMed1985

B cell malignant lymphoma in a patient with progressive systemic sclerosis and Sjögren's syndrome. Report of a case and review of the literature.

We report a patient with progressive systemic sclerosis (PSS) and Sjogren's syndrome (SS) who developed B cell malignant lymphoma 11 and 10 years after the onset of signs of PSS and SS, respectively. As her lymphoma developed, autoantibodies such as rheumatoid factor and antinuclear antibody disappeared from the serum, the lupus erythematosus test yielded negative results and her immunoglobulin levels declined. A good response to chemotherapy was obtained but she died of acute interstitial pneumonitis. Postmortem examination revealed marked diffuse interstitial fibrosis of the lungs.

B-Lymphocytes↗
PubMed1984

HLA antigens, autoantibodies and clinical subsets in scleroderma.

The HLA antigens were determined in 54 Caucasoid patients with scleroderma (ARA criteria). All were assessed for extent of skin and organ involvement, anticentromere (ACA), and scleroderma 70 (Scl 70) antibodies. The antigens DR1, DR3 and DR5 were raised in the patient group, and DR2 was lowered. Of these only the increase in DR5 was significant (chi 2 = 5.2; p = 0.02). The increased frequency of DR3 was attributable to a rise in the A1, B8, DR3 haplotype (chi 2 = 3.9; p = 0.05). Patients with non-diffuse disease showed a significant increase in the DR1 and DR5 antigens (chi 2 = 3.7, p = 0.05; chi 2 = 8.0, p = 0.01). No association was found between the HLA antigens and specific organ involvement. Anticentromere antibodies were present in 15 of the HLA-DR typed patients. Thirteen of these 15 patients were either DR1 or DR5. No relationship was found between the frequencies of HLA antigens and anti-Scl 70 antibodies.

Adult↗