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Results for “Scleroderma, Systemic”

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PubMed2002

Vascular and connective tissue histopathologic alterations of the female lower genital tract in scleroderma.

OBJECTIVE: It has been suggested that women with scleroderma (systemic sclerosis, SSc) have cervical changes that may lead to obstetrical or postoperative complications. We evaluate histopathologic features characteristic of SSc in cervicovaginal tissue from women with SSc and compare them to age matched controls. METHODS: Records from the Scleroderma Registry at Wayne State University were matched with surgical specimens in the anatomic pathology files at Hutzel Hospital. Five women with SSc (2 with limited SSc, 3 with diffuse SSc; mean age 49 yrs) were identified who had cervical or vaginal tissue specimens available for evaluation. Small arterioles and surrounding connective tissue in these specimens and those from 26 age matched controls (15 normotensive, 11 hypertensive) were evaluated in blinded fashion. RESULTS: The following specific histopathological features were evaluated in the SSc patients: duplication or disruption of the internal elastica in 5 (100%), medial hypertrophy in 5 (100%), adventitial changes in 3 (60%), connective tissue fibrosis in 1 (20%), and vasculitis in 1 (20%). There was no significant difference in the frequency of the histopathologic changes between SSc and control patients when evaluated independently. However, the presence of 3 or more features was significantly more frequent in the SSc patients (100%) than in the controls (38%) (p = 0.018). CONCLUSION: The histopathological features evaluated were collectively more frequent in SSc patients; however, many of the control patients also exhibited similar abnormalities. In the female lower genital tract these changes, previously attributed solely to SSc, may be related to other factors.

Adult↗
PubMed1977

[Successive connective tissue diseases (scleroderma, then lupus). Clinical and biological study of 4 cases].

The authors present 4 patients who had suffered for several years from isolated systemic scleroderma and in whom, after 3, 10, 19 and 22 years, clinical, biological and histological signs of DLE appeared. There was no current further progression of the scleroderma in two cases, at the time of onset of DLE. In the oter two cases, it had disappeared. All four patients had a serum cryoglobulin. In all cases the kidneys were involved histologically : extra membranous glomerulitis in one, segmental glomerulitis in a second, and diffuse progressive glomerulitis in the last two. The authors analyse the biological changes which accompanied the clinical transformation. One of the patients had anti-ribonucleoprotein antibodies. She died from severe renal involvement which would be against the good prognosis usually associated with the presence of this type of antibody.

Adult↗
PubMed2005

Osteoporosis in scleroderma.

OBJECTIVE: To review the literature describing the association of osteoporosis (OP) with scleroderma (SSc). METHODS: A Medline (PubMed) search identified all studies from 1966 to 2004 that investigated the association between OP and SSc. Search terms included "scleroderma," "systemic sclerosis," "osteoporosis, " "bone mineral density," "bone densitometry," and "prevalence." RESULTS: Eight case control studies and 1 retrospective study (comparing OP status to a reference standard) were identified. There is no clear association between bone mineral density (BMD) scores and scleroderma. Two of 4 studies have reported lower BMD scores in SSc, but appear not to have considered possible confounding risk factors. Earlier age of menopause has been reported in 2 of 3 studies, and thus, may be a confounder in some samples of women with SSc. Studies of bone metabolism markers have not provided any consistent explanatory mechanism for increased OP in SSc, and such markers may be unreliable in SSc as these are affected by the altered collagen turnover and fibrosis characteristic of SSc. CONCLUSIONS: It is unknown whether OP is truly increased in SSc or whether this association has been observed in some studies as a result of other confounding risk factors for OP. Clinical heterogeneity of SSc study samples and small sample sizes have contributed to the difficulty in obtaining valid estimates of the risk for the development of OP. There is no strong evidence in the literature for consistently lower BMD scores in SSc, or for altered biomarkers of bone resorption. Earlier menopause, corticosteroid use in some patients, and other factors secondary to SSc (such as malabsorption and inflammation), may be causal factors or may be confounders in studies of OP in SSc.

Biomarkers↗
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↗
PubMed1989

[Current methods of research in early diagnosis of cor pulmonale in children with diffuse connective tissue diseases].

Twenty children with systemic lupus erythematosus (SLE) and 13 children with systemic scleroderma were examined by electrocardiography, tetrapolar chest rheography, rheopulmonography and echocardiography. Half of the cases manifested pulmonary hypertension, which was more appreciable in SLE patients. The results of rheopulmonographic and echocardiographic research methods turned out highly comparable.

Adolescent↗
PubMed1979

[Changes in the pulmonary artery in collagen diseases].

Morphological changes in the pulmonary artery and its major branches were studied histologically and histochemically on autopsy cases of polyarteritis nodosa (23), systemic lupus erythematosus (10), systemic scleroderma (12), and rheumatoid arthritis (5). The ages of the fatalities ranged from 9 months to 77 years. An identical type of lesions was revealed: disorders of the connective tissue, destruction of elastic fibers, alterations of vasa vasorum, with cellular reactions typical of each nosological form reflecting the peculiarities of the immunological processes. The initial stage in the genesis of lesions in the vascular walls of the major pulmonary arteries is the involvement of vasa vasorum as a regular disorder of the microcirculatory ways in all collagen diseases.

Adolescent↗
PubMedDate not supplied

Pityriasis versicolor rubra.

We report six typical cases with pityriasis versicolor (PV) rubra, with a background of collagen diseases in five cases and none in one case. Two cases of systemic lupus erythematosus (SLE) and one case of systemic scleroderma (SSc) had both PV rubra and nigra on the trunk. Diagnosis of superficial infections of PV was made by microscopic examination of skin scrapings following KOH, and many small whitish colonies were obtained in Sabouraud's slant agar medium culture containing cycloheximide with olive oil in all cases. Malassezia sympodialis was isolated from the scales of two different lesions at a 6 week-interval in a same person (a 32 year-old male without SLE nor SSc) by the method of Makimura et al. [5], although the other five cases were not examined for the isolation. Histopathological features of the lesion on the dorsum of the trunk showed no epidermal hyperplasia without elongation of rete ridges and no inflammatory cell infiltration in the dermis, however there was only dilatation of small blood vessels in the dermis, which was reconfirmed capillaroscopically. In the horny layers, several yeastlike and fine filamentous structures were seen which were positive with PAS and Grocott stains. Both clinical and histological features led us to speculate PV rubra. All the patients were treated with anti-fungal ointment, and the lesions diminished in less than 2 weeks. No recurrence has been seen in any of the cases. This PV rubra may be independent from PV nigra, although Horiuchi [2] suggested the earlier lesion occurs in advance of PV alba or nigra.

Adult↗
PubMed1998

Carbon dioxide laser resurfacing of peri-oral rhytids in scleroderma patients.

BACKGROUND: The short pulse duration high-energy carbon dioxide (CO2) lasers have been used in the past for treating cutaneous pathology and more recently for cosmetic improvements of rhytids, photo-aging, and acne. OBJECTIVE: This study was undertaken to determine the benefits of CO2 laser resurfacing on the severe peri-oral rhytidosis commonly seen in patients with systemic scleroderma. METHODS: Three patients were studied and evaluated for satisfactory wound healing, improved cosmetic result, and lack of complications. RESULTS: All three patients experienced significant improvement following laser resurfacing. CONCLUSION: The study demonstrated the safe and effective treatment for peri-oral rhytidosis in patients with generalized scleroderma utilizing CO2 laser resurfacing.

Aged↗
PubMed1978

The management of renal scleroderma: experience with dialysis, nephrectomy and transplantation.

In 25 of 100 patients with scleroderma seen over a five year period irreversible renal failure developed; renal support was instituted in 17. Ten of 17 received peritoneal or hemodialysis, one survived. The remaining seven received hemodialysis plus nephrectomy; three survived. Two of these three underwent renal transplantation; one survived. This experience is presented to encourage improvement of these and other technics to increase the survival rate in the otherwise uniformly fatal renal failure associated with scleroderma (systemic sclerosis).

Adult↗
PubMed1983

Anticentromere and anticentriole antibodies in the scleroderma spectrum.

We studied serum samples from 106 patients, including 80 in the scleroderma spectrum, by indirect immunofluorescent microscopy, using PtK1 rat kangaroo tissue culture cells as substrate. Anticentromere (Kinetochore) antibodies were present in 28 patients, and anticentriole antibodies were present in four patients. Anticentromere antibodies were usually present in patients with a benign, chronic form of systemic scleroderma, which has been termed the CREST (Calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, and telangiectasia) syndrome. The four patients with a previously undescribed anticentriole antibody were all in the scleroderma spectrum. Possibly, these antibodies may have diagnostic and prognostic importance. Further, they will be useful in studying the structure and function of these cellular organelles.

Antibodies↗
PubMedDate not supplied

Implant-supported overdenture rehabilitation and progressive systemic sclerosis.

Progressive systemic sclerosis, or systemic scleroderma, is an autoimmune multisystemic disease associated with vascular abnormalities, connective tissue sclerosis, atrophy, and autoimmune changes. The complex oral problems that may develop in patients with the disease and the difficult oral access necessitate extreme care in preventing dental disease. The oral management of a patient with an edentulous mandible and progressive systemic sclerosis, using an overdenture supported by osseointegrated implants, is presented and discussed.

Aged↗
PubMed1988

[Raynaud's phenomenon (a clinico-physiologic analysis)].

On the basis of examination of three groups of patients with Raynaud's phenomenon (RP) (Group I was composed of patients with an idiopathic forms of RP; Group II, of those with neurovascular syndromes of cervical osteochondrosis; Group III, of those with systemic scleroderma) which involved a series of vegetative tests (measurement of the heart rate, the cold test, Aschner's test, determination of autonomic provision of intellectual and other types of activity), authors draw a conclusion about the presence of a close correlation between RP and the psychovegetative syndrome and dysfunction of nonspecific systems of the brain. On the other hand there are differences in the psychophysiological organization of the idiopathic form of RP vs. Raynaud's syndrome, which should be taken into account in the treatment of angiotrophopathic disturbances.

Adult↗
PubMed2005

Risk of malignancy in scleroderma: a population-based cohort study.

OBJECTIVE: To determine the incidence of cancer in patients with scleroderma (systemic sclerosis) and to compare those rates with cancer rates in the local population. METHODS: Cancer risk in scleroderma patients in the Detroit metropolitan area was assessed by linking patient identification codes of the Michigan Scleroderma Registry to the Metropolitan Detroit Cancer Surveillance System database. Patients were screened between the years 1973 and 2002, with additional followup to 2004. Standardized incidence ratios (SIRs) were calculated for selected malignancies (lung, liver, colon, breast, cervical, and prostate cancers, and non-Hodgkin's lymphomas), with stratification by sex and race. RESULTS: Of 934 patients in the Scleroderma Registry, 538 were included in the study based on tri-county residency (436 females and 102 males). Of these, 45 first malignancies were noted (37 females and 8 males). Lung cancer (10 cases) was found to be the most common cancer in scleroderma patients. However, its incidence was not significantly different from that in the general population of metropolitan Detroit (SIR 1.23). Other types of cancer were examined, and no significant differences were found as compared with the rates in the local population, with 1 exception: black females with scleroderma had significantly higher rates of liver cancer (SIR 45.8). CONCLUSION: Contrary to previous studies, this study did not find statistical evidence of an increased incidence of cancer in scleroderma patients, except for liver cancer. One possible reason is the high background rates of certain cancers in the metropolitan Detroit area. It may be necessary to consider local cancer rates when comparing different scleroderma cohorts.

Adult↗
PubMed2002

Increased prevalence of scleroderma in southwestern Ontario: a cluster analysis.

OBJECTIVE: To estimate the prevalence of scleroderma (systemic sclerosis, SSc) in 3 cities, Windsor, Sarnia, and Woodstock, Ontario, within our referral area, which has a referral base population of 1 million. METHODS: To compare the addresses and exposures of referrals with SSc, we performed a case control study using our patients with scleroderma and 2 age and sex matched controls from the same rheumatologist's practice. RESULTS: Sixty-seven of 91 patients with SSc and 87 of 154 controls responded. The mean age of patients with SSc was 53.2 years versus 52.8 years in controls. There was no statistically significant increase in the number of SSc patients from Windsor (population 197,694): 14 patients (15.4%) with SSc versus 18 controls (11.6%) (p < 0.41); or Sarnia (population 72,738): 7 patients (7.7%) with SSc versus 7 controls (4.5%) (p < 0.31). However, there were 9 cases (9.9%) from Woodstock (population 32,086) versus one control (0.64%) (p < 0.0004). The point prevalence of scleroderma was at least 0.71/10,000 in Windsor, 0.96/10,000 in Sarnia, and 2.8/10,000 in Woodstock. There were no significant between-group differences in exposure to industrial toxins or chemicals including vinyl chloride, silica, and benzene, but exposure rates in both groups were low. Occupations and proportion of those who were work disabled were not different. Patients with SSc were not more likely to have smoked cigarettes (p < 0.43); however, they were more likely to drink at least 6 drinks of alcohol per week (p < 0.04) and had more dental fillings (p < 0.05). Patients with SSc knew on average 3.2 others with this disease, and controls knew only 0.25 others with scleroderma (p < 0.00001). Two patients with SSc knew someone with SSc in their workplace versus none of the controls. CONCLUSION: Our a priori expected higher prevalence of scleroderma in Windsor and Sarnia did not reach significance, but the cluster in Woodstock seems statistically validated, and the exact reason for this cluster remains unclear. It is unlikely that all patients with SSc in Woodstock were seen by us, so the prevalence of scleroderma is at least 2.8/10,000, which is a medium to high prevalence compared to other studies. Associations with alcohol and dental fillings require further study.

Adult↗
PubMed2003

Autoantibodies to the epidermal growth factor receptor in systemic sclerosis, lupus, and autoimmune mice.

Autoantibodies to the recombinant extracellular domain of epidermal growth factor receptor (exEGFR) were detected by ELISA in the serum of Fas-defective old MRL/MpJ/lpr and C3H/HeJ/gld mice, but not young mice from these strains, or nonautoimmune young and old BALB/c, MRL/MpJ/++, and C3H/HeJ/MMTV mice. Compared with control human subjects without autoimmune disease, the frequency of exEGFR-binding autoantibodies was increased in scleroderma (systemic sclerosis) patients and to a lesser extent in lupus patients. Phage autoantibodies (Fv fragments) isolated from a lupus library by selection on a linear epitope of EGFR (residues 294-310) displayed the ability to bind exEGFR. Treatment of EGFR-expressing A431 cells with autoantibodies purified by affinity chromatography on immobilized exEGFR resulted in specific staining of the cells. Short-lived but strong inhibition of cellular DNA synthesis was observed in the presence of the autoantibodies. We concluded that autoantibody responses to EGFR hold the potential of fulfilling a pathogenic role in autoimmune disease.

Animals↗
PubMed1992

[The importance of osteoscintigraphy for the early detection of rheumatic joint lesions].

The scintigraphic semeiotics was investigated of involvement of osteoarticular system in rheumatoid polyarthritis in 186 patients (79 of them suffered of rheumatoid arthritis, 62 of systemic lupus erythematosus, 34 of systemic scleroderma, 11 of dermatomyositis). Roentgenological examination was supplemented by consecutive local scintigraphy of axial skeleton and peripheral joint by of gamma rays with labeled 99mTc phosphate complexes. Isoactive zones with subsequent quantitative evaluation of the information were obtained as a result of computerized processing of the scintigraphic images of the joints. The results were compared with those obtained in patients with rheumatoid arthritis. It was established that inspite of the similarity of the scintigraphic manifestations, each of them has its regularities due to differences of the pathomorphological processes in the joints.

Adolescent↗
PubMed2005

New therapeutic strategies for systemic sclerosis--a critical analysis of the literature.

Systemic sclerosis (SSc) is a multi-system disease characterized by skin fibrosis and visceral disease. Therapy is organ and pathogenesis targeted. In this review, we describe novel strategies in the treatment of SSc. Utilizing the MEDLINE and the COCHRANE REGISTRY, we identified open trials, controlled trials, for treatment of SSc from 1999 to April 2005. We used the terms scleroderma, systemic sclerosis, Raynaud's phenomenon, pulmonary hypertension, methotrexate, cyclosporin, tacrolimus, relaxin, low-dose penicillamine, IVIg, calcium channel blockers, losartan, prazocin, iloprost, N-acetylcysteine, bosentan, cyclophosphamide, lung transplantation, ACE inhibitors, anti-thymocyte globulin, and stem cell transplantation. Anecdotal reports were omitted. Methotrexate, cyclosporin, tacrolimus, relaxin, low-dose penicillamine, and IVIg may be beneficial in improving the skin tightness in SSc. Calcium channel blockers, the angiotensin II receptor type 1 antagonist losartan, prazocin, the prostacyclin analogue iloprost, N-acetylcysteine and the dual endothelin-receptor antagonist bosentan may be beneficial for Raynaud's phenomenon. Epoprostenol and bosentan are approved for therapy of pulmonary hypertension (PAH). Other options under investigation include intravenous or aerolized iloprost. Cyclophosphamide (CYC) pulse therapy is effective in suppressing active alveolitis. Stem cell and lung transplantation is a viable option for carefully selected patients. Renal crisis can be effectively managed when hypertension is aggressively controlled with angiotensin converting enzyme (ACE) inhibitors. Patients should continue taking ACE inhibitors even after beginning dialysis in hope of discontinuing dialysis. Antithymocyte globulin and mycophenolate mofetil appear safe in SSc. The improvement in skin score and the apparent stability of systemic disease during the treatment period suggest that controlled studies of these agents are justified. Stem cell transplantation is under investigation for severe disease. Novel therapies are currently being tested in the treatment of SSc and have the potential of modifying the disease process and overall clinical outcome. The evaluation of these studies is still a difficult process.

Clinical Trials as Topic↗