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PubMed1998

Progressive systemic sclerosis: rare localization of the maxillofacial district.

The authors focus on a case of scleroderma, or progressive systemic sclerosis. Scleroderma is a disease of unknown cause, characterized by an abnormal synthesis of connective tissues that causes a sclerosis of the skin involving organs and systems at various levels. It may appear in different forms and may involve, although not frequently, the maxillofacial district. The authors examined a 21-year-old woman who had been diagnosed with systemic progressive sclerosis in 1994. The patient showed a strong limitation of oral aperture, evidenced by an electrognathographic test, and osseous changes. Panoramic and full-mouth intraoral radiographs showed a complete agenesis of coronoid processes on both sides, whereas the angles and the rising branches of the mandibular and periodontal ligaments appeared normal. The patient was diagnosed as having maxillofacial localization of scleroderma with involvement of coronoid processes bilaterally.

Adult↗
PubMedDate not supplied

[Scleroderma and occupational risk factors: a case-control study].

In a hospital-based case-referent study of systemic sclerosis (SSc) and occupational risk factors (55 cases and 171 referents), an excess risk for SSc was observed in men with previous occupational exposure to solvents and certain chemicals (vinyl chloride, formaldehyde). SSc was associated, even though not significantly, with occupational exposure to solvents and silica among women. The possible role of occupational exposures in the etiology of SSc, as well as the methodological limitations of the present study, are discussed.

Case-Control Studies↗
PubMed1989

[Comparison of humeral blood flow during rewarming and recooling of the hand in normal subjects or presenting Raynaud's phenomenon].

Vascular reactivity to heat and cold was studied in 11 normal subjects without vascular disease and in 23 patients with Raynaud's phenomenon (etiologies: Raynaud's disease, scleroderma, thoracic outlet syndrome). The study of hand and digital temperatures and brachial artery blood flow was performed in ambiant conditions (room temperature 23.5 +/- 1 degree C) and after thermal (cold or warm exposure: 10, 33 and 40 degrees C), mechanical and metabolic modifications (with a wrist tourniquet). In these conditions, blood flow was studied at each temperature, before, during and after 3 minutes ischemia of the hand. Analysis of results showed that vasomotricity possibilities were preserved but that responses were not identical. Patients with primary Raynaud's phenomenon, and even more those with scleroderma as well, had reduced brachial artery blood flow after cooling (10 degrees C). After ischemia, maximal blood flow was also reduced. The microcirculatory disease existing in Raynaud's phenomenon limits the vasodilator capacity of hand vessels, but probably more in tissues with vascular lesions. Vasodilation seems to be limited during exposure to low well as high temperatures, but vasoconstriction capacity is not disturbed.

Adult↗
PubMed2006

Macrophage migration inhibitory factor promoter polymorphisms and the clinical expression of scleroderma.

OBJECTIVE: To investigate the potential association between functional polymorphisms in the gene for the innate mediator, macrophage migration inhibitory factor (MIF), and the clinical expression of systemic sclerosis (SSc). METHODS: Genomic DNA samples and clinical data were collected from the Scleroderma Family Registry and DNA Repository at the University of Texas Health Science Center at Houston. A total of 740 subjects were studied; 203 of them had diffuse cutaneous SSc (dcSSc), 283 had limited cutaneous SSc (lcSSc), and the remaining 254 healthy subjects served as controls. Association analyses were performed on the whole data set and on patient and sex subsets. Significant relationships were determined between clinical variables and MIF polymorphisms for each disease subtype in the studied groups. RESULTS: The frequency of the -173*C MIF allele, which was previously reported to be associated with high production of MIF, was lower in the lcSSc group (12.6%) than in the dcSSc (19.2%) or control (18.5%) groups (P = 0.010 and P = 0.011, respectively). Haplotype analysis for 2 closely linked polymorphisms in the MIF promoter showed that in white subjects with lcSSc or dcSSc, the lcSSc population had a significantly lower representation of the high-expression MIF haplotype defined by -173*C and -794 with 7 CATT repeats (C7) (P = 0.015, odds ratio 1.94 [95% confidence interval 1.14-3.32]). Fibroblasts encoding the C7 MIF haplotype were observed to produce more MIF upon in vitro stimulation than those with a non-C7 haplotype. CONCLUSION: Functional promoter polymorphisms in the MIF gene affect the clinical presentation of SSc. The proinflammatory haplotype defined by C7 is underrepresented in patients with lcSSc.

Female↗
PubMed1999

TNF-alpha and TNF-beta gene polymorphisms in systemic sclerosis.

Tumor necrosis factor (TNF)-alpha and TNF-beta, mediators of inflammatory responses, have been implicated in the pathogenesis of autoimmune diseases. The aim of this investigation was to determine whether two promoter region polymorphisms of the TNF-alpha gene (TNF-alpha -308 and TNF-alpha -238) and a determinant in the first intron of the TNF-beta gene (TNF-beta +252) affect susceptibility to systemic sclerosis (scleroderma) (SSc). Fifty patients and 60 healthy blood donors from Japan were genotyped for these markers by polymerase chain reaction-based methods. Fisher's exact test was used to test for significant associations. Because of very limited variation at the TNF-alpha -308 and TNF-alpha -238 loci in the Japanese people, statistical analyses with sufficient power could not be done for these genotypes. However, the two homozygous genotypes of the TNF-beta +252 locus were found to be significantly associated with SSc. Compared to controls, the frequency of the TNF-1 genotype was decreased, whereas that of TNF-2 was increased in SSc patients. The former implies an association with resistance, while the latter suggests an association with susceptibility to the disease. These results show that the TNF-beta +252 locus plays an important role in the etiopathogenesis of SSc.

Case-Control Studies↗
PubMed1992

[Evaluation of cardiac functions in patients with progressive systemic sclerosis (PSS)].

Cardiac functions in 20 female patients with PSS were measured by ultrasound cardiography and compared with those of 10 healthy age-matched controls. The following results were obtained: 1) The patients with PSS showed the increases in left ventricular mass (LVmass), interventricular septal thickness (IVS) and left ventricular posterior wall thickness (LVPW) more than those of controls. 2) Diastolic descent rate (DDR) of patients decreased significantly in comparison with the healthy controls. 3) Right ventricular dimension (RVD) of patients were demonstrated to dilate more than those of controls. Conclusively ultrasound cardiography (UCG) is an available method for detecting the cardiac lesion of PSS. Abnormal signs were recognized even though their clinical features were limited to the extremities.

Adult↗
PubMed1992

A human monoclonal autoantibody to a nucleolar structure.

Peripheral blood lymphocytes from a scleroderma patient (CDC) were isolated, transformed with Epstein-Barr virus and fused to the heteromyeloma SHM-D33. Supernatants from cultures were screened for autoantibody production against nucleoprotamine by ELISA. Positive wells were cloned by limiting dilution. After cloning, supernatants from two wells were positive for the nucleoprotamine assay. One named CDC-1 has been studied in our laboratory. CDC-1 recognized a nucleolar antigen by indirect immunofluorescence. By using an ELISA with purified recombinant antigens, CDC-1 reacted against Ro/SS-A, U1 (RNP) and Sm. By immunoblotting using a lysate of MOLT-4 cell line, CDC-1 was able to react against a structure of 60 kD. When the antigen recognized by CDC-1 was purified, SDS-PAGE under reducing conditions with purified antigen and subsequent silver staining of the gel allowed us to detect three bands at 60, 55 and 39 kD, respectively. A screening by ELISA with previously characterized antisera against our purified antigen demonstrated reactivity of the CDC-1 antigen with those antisera able to recognize Ro/SS-A.

Antibodies, Antinuclear↗
PubMed2003

Evaluation and management of alveolitis and interstitial lung disease in scleroderma.

PURPOSE OF REVIEW: In the fibrosing alveolitis of systemic sclerosis, treatment decisions depend on prognostic evaluation, which continues to excite considerable interest and debate. Advances in the staging of fibrosing alveolitis of systemic sclerosis and recent therapeutic studies are discussed in this review. RECENT FINDINGS: The decision about whether to start treatment is often the most difficult clinical challenge, because many patients have limited pulmonary fibrosis that will not necessarily progress. The estimation of disease extent (using high-resolution CT) and disease severity (using pulmonary function tests) is pivotal. Factors reducing the threshold for treatment, in addition to severe disease, include evidence of recent deterioration, a short duration of systemic disease, antitopoisomerase antibody positivity, and, in some cases, bronchoalveolar lavage findings (although the role of bronchoalveolar lavage remains contentious). Histologic appearances at surgical biopsy have little prognostic value, with the great majority of patients having nonspecific interstitial pneumonia. Best current initial treatment consists of either oral or intravenous cyclophosphamide, usually administered with low-dose corticosteroid therapy, although the risk of scleroderma renal crisis with low-dose steroid therapy requires further evaluation. SUMMARY: Careful prognostic evaluation, including the staging of disease severity and the definition of longitudinal disease behavior (by serial imaging and pulmonary function tests), is central to the formulation of a logical management plan in fibrosing alveolitis of systemic sclerosis. Cyclophosphamide, the best initial treatment currently, is associated with significant toxicity, justifying therapeutic studies of other immunosuppressive agents and a wide range of anticytokine and antifibrotic agents.

Adrenal Cortex Hormones↗
PubMed1990

Autoantibody to Th ribonucleoprotein (nucleolar 7-2 RNA protein particle) in patients with systemic sclerosis.

We studied sera of 371 consecutive new patients with systemic sclerosis (SSc; scleroderma) who were first evaluated during 1984-1988. All sera were tested for antinuclear antibodies by immunofluorescence staining using HEp-2 cells as substrate. We excluded 219 sera showing dark nucleoli and screened for antibodies to Th in the remaining 152 sera by immunoprecipitation of a 32P-labeled HeLa cell extract. Fifteen (4.0%) of 371 sera were anti-Th+. Anti-Th antibodies were present in 14 (8.4%) of 167 SSc patients with limited cutaneous involvement, in 1 of 167 with diffuse cutaneous involvement, and in 0 of 37 with SSc overlap syndrome. Among 244 controls with other connective tissue diseases, anti-Th was detected in only 3 patients, all having primary Raynaud's phenomenon of less than 2 years duration. In the subgroup with SSc with limited cutaneous involvement, the 14 anti-Th+ patients had a significantly greater frequency of puffy fingers, small bowel involvement, and hypothyroidism, and a significantly lower frequency of arthralgia and/or arthritis. Their cumulative survival rate from the time of onset of symptoms was lower than that for anti-Th- patients (78% versus 91% at 10 years), primarily due to 3 deaths from pulmonary arterial hypertension (2 from primary pulmonary hypertension and 1 from pulmonary hypertension secondary to pulmonary interstitial fibrosis). Serum anti-Th antibodies are present almost exclusively in patients with SSc with limited cutaneous involvement or in those with primary Raynaud's phenomenon whose disease may evolve to SSc with limited cutaneous involvement, and these antibodies may identify those patients who are at greater risk for reduced survival.

Autoantibodies↗
PubMed2007

Cutaneous vascular deposition of C5b-9 and its role as a diagnostic adjunct in the setting of diabetes mellitus and porphyria cutanea tarda.

BACKGROUND: The cutaneous lesions of diabetes mellitus (DM) and porphyria cutanea tarda (PCT) exhibit distinctive microvascular changes including basement membrane zone thickening and lamellation, morphologically appearing as hyaline-like alterations of the vessel wall. Immunofluorescence demonstrates homogeneous mantles of immunoglobulin in the microvasculature. The staining intensity is variable and in some cases can closely approximate those immunofluorescent changes seen in photoaged skin. OBJECTIVE: The purpose of this study was to establish an association between the microvascular changes seen in the skin from patients with DM and PCT and the presence of C5b-9 deposition, potentially defining the C5b-9 assay as an additional diagnostic adjunct. METHODS: Routine light microscopy and immunofluorescence studies were conducted on skin biopsy specimens from 14 patients with cutaneous manifestations of DM and 17 patients with PCT. The immunofluorescence profile included IgG, IgM, IgA, C3, C3d, C4d, and C5b-9. RESULTS: Fourteen of 14 DM and 17 of 17 PCT skin biopsy specimens revealed extensive granular and homogeneous vascular deposition of C5b-9; a similar pattern was observed for C3d and C4d. Control specimens from patients without DM and PCT, where C5b-9 was not an expected immunoreactant, were negative. Positive controls were cases of vasculitis, scleroderma, and dermatomyositis without DM and PCT where C5b-9 deposition was expected. C5b-9 deposition was observed and was of lesser magnitude than that encountered in patients with PCT or DM. LIMITATIONS: We were unable to obtain detailed clinical information on some of the diabetic patients in regards to significant extracutaneous vascular complications. In addition, a correlation between hemoglobin 1 Ac levels and the extent of C5b-9 deposition could not be ascertained as the serum levels for hemoglobin 1 Ac were unknown. CONCLUSION: Granular and homogeneous deposits of C5b-9 in vessels, along with homogeneous deposits of immunoglobulin within the blood vessels, are characteristic immunofluorescence findings in patients with DM and PCT. In regards to potential mechanisms of C5b-9 deposition, decreased metabolism of C5b-9 due to glycosylation of CD59 in the setting of DM and activation of complement by irradiated porphyrins in PCT are proposed. The extent of C5b-9 deposition suggests that this complex may play a pathogenetic role in the evolution of microvascular injury in patients with DM and PCT.

Adult↗
PubMed1998

Health status measurement in Toxic Oil Syndrome.

Toxic Oil Syndrome (TOS) is a previously unreported condition which affected more than 20,000 people in Spain in 1981 and whose natural history is unknown. In 1993-94, a stratified random sample of 1400 survivors was drawn to measure their health status through clinical examination and their self-perception of well-being through the Nottingham Health Profile Questionnaire (NHPQ). Two-thirds of the sample population responded; indirect estimates suggest that selection bias was limited. Clear and intermediate signs of neuropathy were found in one-fifth and one-half of the patients, respectively. One-fourth and one-sixth showed some degree of scleroderma and contractures. All conditions were more frequent in women than in men and in age >50 than in younger ages. Although no concurrent control group was included in the study, prevalences of these conditions are well above expectations and are largely attributable to TOS. NHPQ scores increased with age in both sexes up to age 50, after which they reached a plateau (with values around 48 in men and 62 in women). Scores were associated to the occurrence of peripheral neurological changes, contractures, and scleroderma-like conditions. A multivariate analysis indicated age, sex, and severity of neurological conditions as major determinants of the NHPQ scores. This overall pattern of findings is peculiar to TOS and differs from the typical post-disaster nonspecific syndrome.

Adult↗
PubMed1981

Scleroderma: oral manifestations.

An oral clinical and radiologic study of twenty-one patients suffering from scleroderma revealed that in one half of the patients the blood sedimentation rate and the antinuclear factor were elevated. When both these laboratory findings were present at the same time, they proved to be well correlated. The majority of the patients had limited mouth opening. Gross jawbone changes affecting the mandibular condyle, coronoid process, and posterior part of the ascending ramus occurred in four patients. There was no correlation in these four patients with regard to the bone changes and clinical and laboratory findings or to age and duration of illness. Widening of the periodontal ligament space, as measured in a meticulous manner, was a feature in all twenty-one patients and in all teeth.

Adult↗
PubMed1983

The effect of percutaneous dimethyl sulfoxide on cutaneous manifestations of systemic sclerosis.

DMSO exerts a palliative, therapeutic effect on healing of cutaneous ulcers in systemic sclerosis. The therapeutic response was variable and, therefore, the concentration of DMSO, as well as frequency and duration of treatments, should be individualized to obtain maximum healing effect with a minimum of adverse reactions. There was no evidence of ocular toxicity or other serious toxicity manifestations in this group of patients treated with topical DMSO for one year or longer. Delayed improvement was observed in the untreated extremity in the majority of patients studied. In no instance did improvement in the untreated extremities exceed improvement in the treated, bilateral counterpart. It is believed this resulted from a systemic, carry-over effect of DMSO rather than spontaneous improvement in the disease course. DMSO is a worthwhile, supplemental, therapeutic agent providing the limitations of therapy are understood.

Administration, Topical↗
PubMedDate not supplied

Physical injury as a provoking factor in three patients with scleroderma.

A 51-year-old female developed linear-like scleroderma in the left thigh following a linear wound caused by a car accident. 27 years later she also developed a typical diffuse cutaneous systemic sclerosis with extensive skin involvement and bibasilar pulmonary fibrosis. The second case is a 39-year-old female who had a history of Raynaud's phenomenon since early childhood. She developed a morphea following a burning injury of the left thigh. 17 years later she also developed a typical limited cutaneous systemic sclerosis with sclerodactyly, skin ulcers and subcutaneous calcinosis. The third case is a 43-year-old female who developed a typical morphea of the right elbow around the site of a previous local corticosteroid injection. The two remarkable points of these 3 cases are the possible role of physical injury in the provocation of localized scleroderma and in the first 2 cases the unusual later development of a systemic form of scleroderma.

Adult↗
PubMed1976

Ganglion cells in achalasia of the cardia.

The histopathology of 40 cases of achalasia of the cardia, 6 cases of oesophageal spasm-incoordination and 4 cases of scleroderma was examined. Three cases of carcinoma and 6 cases of reflux oesophagitis were used as a control group. A nearly complete loss of myenteric ganglion cells was found in the upper thickened segment in achalasia. Some surviving ganglion cells were found in the lower segments in half the cases of achalasia; in two cases counts were normal in this segment. The occurrence of neuronal chromatolysis in 9 biopsies of achalasia supports the view that an active disease process was involved. The preganglionic parasympathetic fibres in two cases of achalasia were normal in appearance and number; this somewhat limited evidence tends to count against a primary disorder of the preganglionic neurone in this condition. The 6 cases of oesophageal spasm-incoordination showed similar neuronal loss to that in the lower segment in achalasia. Possibly "oesophageal spasm" represent an early stage or incomplete expression of achalasia. One cases of scleroderma showed loss of ganglion cells, but the myenteric plexus was here involved by the disease process. None of the 9 cases in the control group showed any loss of ganglion cells or chromatolysis. Acute and chronic inflammation was not convincingly associated with loss of ganglion cells in either achalasia or oesophageal spasm.

Autonomic Fibers, Preganglionic↗