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Results for “Scleroderma, Limited”

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PubMed2001

[Ventilatory failure due to the limitation of chest movement in a case of FPS].

A 72-year-old woman presented with cervicothoracal skin lesions mimicked to scleroderma and muscular atrophy in 1996. Because of the elevation of serum creatinine kinase (CK), muscular biopsy was performed at another institution. Under the diagnosis of polymyositis, she was treated with corticosteroid. Despite of the decrease in serum CK levels by corticosteroid therapy, skin lesions and mascular dystrophy gradually worsened to extend to the regions of major pectoral, paravertebral, and femoral muscles. In 1997, she was admitted to our hospital because of dyspnea. On admission, the limitation of the chest movement was obvious and she developed respiratory arrest due to CO2 narcosis. The femoral magnetic resonance image (MRI) showed increased signal intensity of subcutaneous tissues and fascia on T2-weighted image. The block biopsy specimens obtained from the cervical lesion revealed fibrotic thickness and chronic inflammation of subcutaneous septa, fascia, and perimysium. She was treated by mechanical ventilation and cimetidine and weekly methotrexate were added to the corticosteroid therapy because of the diagnosis of FPS. Thereafter, the skin and muscular lesions as well as the MRI findings were improved. The concept of FPS was proposed by Naschitz et al. This condition is pathologically characterized by cicatrizing fascitis, septal and lobular panniculitis, and perimysial fibrosis and peripheral blood and tissue eosinophilia is not important for diagnosis. FPS includes classical eosinophilic fascitis but is also associated with several disorders such as malignancy. This case is suggestive of the therapeutic consideration of FPS in terms of the response to cimetidine and MTX.

Aged↗

PubMed2004

Childbearing and the risk of scleroderma: a population-based study in Sweden.

This study examined associations between childbearing and risk of scleroderma by using national population-based registry data from Sweden. Women with a discharge diagnosis of scleroderma from 1964 to 1999 (n = 2,149) were identified in the Swedish Inpatient Register. These cases were matched by year and month of birth and region of residence to as many as five controls obtained from the Multi-Generation Register. Pregnancy history (number of births, age at each birth) was restricted to births before the first scleroderma-related hospitalization for cases and the corresponding age for their matched controls. Risk estimates, measured by the odds ratio and 95% confidence interval, were obtained by using conditional logistic regression. Nulliparity was associated with an increased risk of scleroderma (odds ratio = 1.37, 95% confidence interval: 1.22, 1.55). Risk decreased with increasing number of births. Similar results were found when analyses were limited to births up to 2 years or up to 5 years before hospitalization. Among parous women, younger age at first birth was associated with an increased risk of scleroderma. The association between lower parity and increased risk of scleroderma could reflect subfecundity caused by scleroderma before disease became clinically evident, possible common causes of infertility and scleroderma, or a protective effect of pregnancy through an unknown mechanism.

Adolescent↗

PubMedDate not supplied

Eosinophilic fasciitis occurring four weeks after the onset of dialysis in a renal failure patient.

BACKGROUND: Eosinophilic fasciitis is a rare, scleroderma-like disease that usually affects the extremities of young to middle-aged males. The disease may cause flexion contractures and limit joint mobility and is associated with peripheral eosinophilia. The fascia, by definition, is infiltrated with mononuclear cells and typically with eosinophils. Eosinophilic fasciitis may be separated from another sclerodermatous disorder, linear scleroderma, by its response to systemic corticosteroids. The etiology is unclear but eosinophilic fasciitis has numerous disease associations. However, it has not previously been associated with renal failure and hemodialysis. OBJECTIVE: This article reports a case of eosinophilic fasciitis occurring four weeks following the onset of hemodialysis. METHODS: The clinical and histologic features confirmed the diagnosis of eosinophilic fasciitis. He was treated with systemic corticosteroids with good response. CONCLUSION: This is the first reported patient who developed eosinophilic fasciitis in close temporal relationship with the start of hemodialysis. While eosinophilic fasciitis may be coincidental with a common disorder, namely, renal failure, it is interesting to note that hemodialysis patients often have immune-regulation abnormalities and peripheral eosinophilia.

Aged↗

PubMed1982

The articular manifestations of progressive systemic sclerosis (scleroderma).

The articular manifestations of progressive systemic sclerosis (PSS) were studied in 38 patients. Of these, 66% experienced joint pain and 61% had signs of joint inflammation. Limitation of joint movement was seen in 45%. Radiological abnormalities included periarticular osteoporosis (42%), joint space narrowing (34%), and erosions (40%). Erosive disease did not correlate with disease duration, presence of rheumatoid factor, antinuclear antibodies, distal tuft resorption, or the extent of the scleroderma skin changes. Calcinosis was seen more frequently in those patients with articular erosions (67%). Erosive osteoarthritis of the distal interphalangeal joints (7 patients) was associated with impaired finger flexion. Joint involvement in PSS occurs frequently and may resemble rheumatoid arthritis in the early stages but is less destructive. The occurrence of unrelated arthropathy, such as primary osteoarthritis, is not uncommon, and its differentiation from true PSS joint disease can be difficult.

Adolescent↗

PubMed1996

Childhood-onset scleroderma: is it different from adult-onset disease.

OBJECTIVE: To distinguish childhood-onset scleroderma from adult-onset disease. METHODS: The clinical and serologic features of 58 patients with childhood-onset scleroderma (11 patients with diffuse cutaneous systemic sclerosis [SSc], 16 with linear SSc, 14 with linear morphea, and 17 with morphea) were examined in the largest cohort of such patients studied to date. These parameters were compared with data obtained from patients with adult-onset disease. RESULTS: Childhood-onset scleroderma resembled adult-onset disease with regard to the heterogeneity of clinical expression and subsets of disease, but it also differed from adult-onset disease in a number of clinical and laboratory parameters. The predominant childhood-onset disease presentation was the localized form of the disease, with limited and diffuse SSc being less notable. There was a significant association of trauma with childhood-onset scleroderma (P < 0.0001), which was not noted in adult-onset disease. Furthermore, in contrast to adult disease, patients with childhood-onset disease had normal levels of parameters of vascular activation (von Willebrand factor, angiotensin-converting enzyme, E-selectin, and endothelin-1), T cell activation (soluble interleukin-2 receptors), and collagen synthesis (carboxy-terminal type I and amino-terminal type III), a notable lack of anticentromere antibodies, and abnormal coagulation indices. CONCLUSION: A number of features distinguish childhood-onset scleroderma from adult-onset disease.

Adolescent↗

PubMed1980

Serum immunoreactive prolyl hydroxylase in inflammatory rheumatic diseases.

Serum immunoreactive prolyl hydroxylase protein (S-IRPH) was measured in 56 patients with inflammatory rheumatic diseases, and the values were compared with those in 32 control subjects. S-IRPH was above the 95% confidence limit of the controls in about 70% of the patients with active systemic lupus erythematosus, rheumatoid arthritis, scleroderma, Reiter's syndrome, Sjögren's syndrome, polyarteritis nodosa, or polymyositis. Raised values were observed in about half of the patients with an erythrocyte sedimentation rate (ESR) of 21-50 and in about 90% of those with ESR of over 50, whereas only about 10% of the patients with an inactive disease had an S-IRPH concentration exceeding this limit. Only 1 out of 8 patients with active ankylosing spondylitis had a raised S-IRPH value. The results support previous data indicating that significant changes in collagen metabolism occur in active connective tissue diseases. Assays of S-IRPH might be of some value in assessing the activity of these diseases and in monitoring the treatment provided.

Adult↗

PubMed1993

Juvenile-onset mixed connective tissue disease: longitudinal follow-up.

To establish the symptoms and clinical course of juvenile-onset mixed connective tissue disease, we studied 14 patients, classified according the criteria of Kasukawa et al. The patient records were studied retrospectively and all patients were examined in a 1-day follow-up program. Systemic lupus erythematosus and polymyositis/dermatomyositis-like symptoms disappeared in time, whereas scleroderma-like symptoms (such as in the Raynaud phenomenon) and joint abnormalities persisted. Extensive limitation of joint function was found in four patients. At the time of follow-up, no active renal disease was found. Thrombocytopenia was still present in one of the three patients who had had this feature. All patients had abnormalities of esophageal motility. Long-term corticosteroid treatment was associated with aseptic bone necrosis in three patients and growth retardation in one. We conclude that the Kasukawa criteria are easy to apply to children, and that juvenile-onset mixed connective tissue disease has many similarities to the adult form of the disease.

Adolescent↗

PubMedDate not supplied

Scleroderma in association with the use of docetaxel (taxotere) for breast cancer.

The taxanes, paclitaxel (Taxol) and docetaxal (Taxotere), are a new class of anti-microtubule agents which have shown cytotoxic activity in a number of solid tumours. Phase I and II trials confirm that docetaxal is highly active in the treatment of metastatic breast cancer. Reported toxicities of docetaxel include, dose limiting neutropenia, alopecia, skin reactions and fluid retention. Here we report the first case of rapid onset, diffuse scleroderma-like illness, which occurred in a 59-year-old female receiving treatment with docetaxel for locally invasive and advanced metastatic breast cancer.

Antineoplastic Agents, Phytogenic↗

PubMed1976

Peritoneal clearances in scleroderma and diabetes mellitus: effects of intraperitioneal isoproterenol.

Reduced peritoneal clearances of creatinine and urate were demonstrated repeatedly in a patient with scleroderma and a patient with diabetes mellitus. Urea clearances were not significantly different from usual values. The findings suggest decreased peritoneal membrane permeability and/or area (if urea clearance is flow limited). Clearances increased to usual values with intraperitioneal isoproterenol in the patient with diabetes. There was no effect of isoproterenol in the patient with scleroderma.

Adult↗

PubMed1997

Anticentromere antibodies in rheumatologic practice are not consistently associated with scleroderma.

Anticentromere antibodies identified by indirect immunofluorescence are a valuable aid to the diagnosis and prognosis of patients with systemic sclerosis since they are associated in 50% to 80% of cases with limited cutaneous systemic sclerosis, a pattern usually associated with a good prognosis. We studied clinical presentations in rheumatology patients with anticentromere antibodies by indirect immunofluoresence and by ELISA and/or Western blot, but without scleroderma or Raynaud's phenomenon. Eight of 34 (23.5%) rheumatology clinic patients with centromere antibodies met these criteria, seven women and one man, with a median symptom duration of six years (range 1-20 years). Four had Sjögren's syndrome, one had isolated xerostomia, one systemic lupus erythematosus, one seronegative symmetric polyarthritis and one primary biliary cirrhosis with arthralgia. The mean anticentromere antibody titer in these eight patients was similar to that in the patients who had at least Raynaud's phenomenon. Given the low incidence of scleroderma, these data illustrate the poor predictive value of anticentromere antibodies for the diagnosis of scleroderma in rheumatology clinic patients.

Adult↗

PubMed1987

Frequency, levels, and significance of blood eosinophilia in systemic sclerosis, localized scleroderma, and eosinophilic fasciitis.

Blood eosinophilia is a common feature of eosinophilic fasciitis and is variably reported in systemic sclerosis and localized scleroderma. Since these diseases share cutaneous fibrosis as the final outcome and have other clinical and pathologic features that are difficult to differentiate, the presence of blood eosinophilia may be a further source of confusion. In this study, we examined the frequency and level of blood eosinophilia in 715 patients with systemic sclerosis, 72 patients with localized scleroderma, and 22 patients with clinically active eosinophilic fasciitis. When defined as greater than 400 cells/mm3, eosinophilia was present in 7% of patients with systemic sclerosis, 31% of patients with localized scleroderma, and 83% of patients with eosinophilic fasciitis. Greater than 1000 eosinophils/mm3 were present less frequently in systemic sclerosis (1%) and localized scleroderma (8%) than in eosinophilic fasciitis (61%). No difference in the frequency of eosinophilia was present in patients with the limited cutaneous CREST syndrome or the diffuse cutaneous variety of systemic sclerosis, and in these patients the presence of eosinophilia did not correlate with the extent of cutaneous or internal organ involvement or with other laboratory abnormalities. Among patients with localized scleroderma, eosinophilia was more common in those with linear scleroderma and generalized morphea than in those with morphea, and both the frequency and level of eosinophilia were greater in individuals with clinically active disease (p less than 0.02). Eosinophilia was a persistent feature in untreated patients with active eosinophilic fasciitis, even up to 30 months of disease duration.

Eosinophilia↗

PubMed1993

Patients with anticentromere antibodies, clinical features, diagnoses and evolution.

The sera of 1373 patients derived from a random population of 2627 individuals examined in a rheumatology outpatient clinic were found to be positive for antinuclear antibodies (ANA). Forty-seven of 1373 ANA positive patients (3.42%) presented a pattern of anticentromere antibodies (ACA), and of these 41 were followed-up. The predominant clinical features among ACA positive patients were Raynaud's phenomenon, arthralgias, scleroderma affecting the hands, feet, face and forearms, telangiectases and pulmonary involvement. There were 26 patients (64%) with limited cutaneous systemic sclerosis (lSSc), seven with primary Sjögren's syndrome (Ss) (17%), five with idiopathic Raynaud's phenomenon (IRPh) (12%) and three (7%) with other diseases. The lSSc subset was characterized by a significantly younger age for disease onset than for the IRPh patients. The Raynaud's phenomenon of lSSc patients lasted for many years before the onset of scleroderma, while their pulmonary involvement was mild and subclinical. The Ss patients with ACA were characterized by a lower incidence of parotid gland enlargement and anti-La (SSB) antibodies than described in the literature. Finally, the presence of ACA correlates with the clinical criteria proposed for the diagnosis of lSSc.

Adult↗

PubMed1976

Cutaneous antigen-stimulating lymphokine production by lymphocytes of patients with progressive systemic sclerosis (scleroderma).

Cell-mediated immunity to skin extracts was studied by the macrophage migration inhibition test, lymphocyte transformation, and direct cytotoxicity to skin fibroblasts, in normal individuals and patients with progressive systemic sclerosis. The latter included 18 individuals with diffuse scleroderma and 12 with the CREST syndrome, a variant form of systemic sclerosis in which there is more limited involvement of the skin. Controls consisted of 13 patients with other connective tissue diseases and 16 normal individuals. Phosphate-buffered saline and 3 M KCl extracts of both normal and sclerodermatous skin were used as antigens. No evidence of lymphocyte reactivity was found by the lymphocyte transformation and direct cytotoxicity test procedures. However, the lymphocytes of patients with diffuse scleroderma did respond to extracts of both normal and sclerodermatous skin in the migration inhibition assay. 10 of 16 patients (62.5%) had migration indices below 2 SD of the normal range, 1 of 10 CREST patients and 1 of 13 patients with other connective tissue diseases showed similar reactivity. Antisera specific for immunoglobulin-bearing lymphocytes (B lymphocytes) and T lymphocytes were used to characterize the lymphocytes found in skin biopsies of patients with diffuse scleroderma. T lymphocytes made up the majority of lymphocytes in the skin infiltrates. These findings suggest that lymphocytes sensitized to skin extracts are present in patients with diffuse scleroderma. The cell-mediated immune reaction to skin antigens may be a factor in the pathogenesis of diffuse scleroderma.

Adolescent↗

PubMed1979

Chronic graft versus host disease: a syndrome of disordered immunity.

A chronic, debilitating syndrome related to graft-versus-host disease (GVHD) has been recognized in long-term survivors following allogeneic bone marrow transplantation. In six of 20 marrow graft recipients who survived for more than one year after receiving a transplant, this complication developed; they were studied to better define the syndrome. There was no association between the sex of either donor or recipient, HLA type, blood group, conditioning regimen or marrow cell dose and subsequent development of chronic GVHD. All six patients had mild to moderate manifestations of acute GVHD following prompt engraftment. Chronic GVHD was characterized in each patient by progression to scleroderma-like skin involvement with hyperkeratosis, reticular hyperpigmentation, atrophy with ulceration and fibrosis with limitation of joint movement. A sicca syndrome was prominent in five patients. Four patients had idiopathic interstitial pneumonitis. Infectious complications were frequent, and DNA viral infections were prominent. Autoimmune hemolytic anemia was present in three patients, and one patient had antinuclear antibody (ANA). A spectrum of immune abnormalities was observed including hypergammaglobulinemia, immunoglobulin M (IgM) paraprotein, elevated circulating immune complexes, plasma cell hyperplasia, lymphocytotoxic antibodies and autoantibodies to autologous or donor lymphocytes. Despite clinical similarity to collagen vascular diseases, none of these patients had anti-DNA antibodies or antibodies to smooth muscle, thyroid or extractable nuclear antigens. In one patient, a skin graft from the marrow donor remained healthy despite progressive involvement in recipient skin, whereas unrelated skin grafts were rejected. Immunosuppressive therapy and plasmapheresis have not been effective. Four patients have died (median survival 458 days from transplantation). Chronic GVHD appears to be a syndrome of disordered immune regulation features of immunodeficiency and autoimmunity.

Adolescent↗

PubMed2007

Calcareous lesions of the distal extremities resembling tumoral calcinosis (tumoral calcinosislike lesions): clinicopathologic study of 43 cases emphasizing a pathogenesis-based approach to classification.

Herein, we analyze the clinicopathologic features of 46 distal extremity lesions that have histologic features similar to conventional tumoral calcinosis (tumoral calcinosislike; TC-L). The study included 31 females and 12 males (whites:non-whites>3:1) ranging in age from 1 to 91 (mean, 39; median, 42) years. The lesions presented in fingers (n=20), feet (n=10), wrist (n=6), hands (5), toes (n=4), and ankle (n=1) were solitary in all but 5 patients and ranged in size from 0.3 to 4.5 (mean, 1.6; median, 1.4) cm. Chief initial complaints included presence of a painful (n=16) or asymptomatic (n=7) mass, and limitation of joint mobility (n=3). Pertinent clinical associations included antecedent trauma (n=7), scleroderma (n=3), long-standing osteoarthritis (n=3), bony deformities (n=5), including 2 infants with congenital hand malformations, and chronic renal failure (n=2). Patients were stratified into one of the 3 recognized clinical settings of TC: primary normophosphatemic (n=17), secondary (n=5), or primary hyperphosphatemic TC (n=1). The 20 remaining patients were placed in an "indeterminate TC" category. Most lesions were located in tenosynovial/fascial tissue, but 13 lesions involved dermis and 1 was intra-articular. Histologically, the process consisted of multiple cystic or cleftlike spaces bordered by histiocytes, osteoclastlike giant cells, and a variable inflammatory infiltrate and containing fibrin, granular calcific debris, and calcospherites. Pools of calcific debris bordered by sclerotic collagen and a sparse cellular element predominated in 4 cases. Cartilaginous metaplasia was identified in 10 lesions and evidence of hemorrhage or specific injury was observed in 12 examples. Follow-up data for 22 patients (interval range, 1 to 30 y; median, 6 y) revealed 17 individuals with no evidence of recurrent disease or the development of additional lesions after simple (local) excision. One patient (indeterminate TC) required reexcision of a thumb mass 1 year after surgery. All 3 scleroderma patients developed additional TC-L lesions. Acral TC-L lesions are histologically similar to conventional TC, but present as smaller size lesions. Most TC-L lesions are closely aligned with primary normophosphatemic or secondary TC. Acral TC-L lesions may be the first manifestation of scleroderma, where the process has the potential to follow an unrelenting course.

Adolescent↗

PubMed2005

Pulmonary hypertension and chronic obstructive pulmonary disease: a case for treatment.

Current pharmacotherapy for chronic obstructive pulmonary disease (COPD) relieves symptoms and reduces exacerbation through improving airflow limitation. Such drugs do not effectively improve exercise tolerance due in part to pulmonary hypertension associated with severe COPD, nor impact on its increased morbidity and mortality. Exercise intolerance is often improved (temporarily) by lung volume reduction surgery and pulmonary rehabilitation. Ambulatory oxygen is the most effective treatment of exercise limitation. Chronic cigarette smoking is the principal cause of COPD. An early change in smokers' lungs is pulmonary artery intimal thickening and vessel narrowing, which, as COPD develops, is correlated with both the severity of emphysema and bronchiolitis. This may be the consequence of combined smoking-induced apoptosis, inflammation, and imperfect repair. End-stage bronchiolitis and emphysema are likely to limit the effectiveness of bronchodilators and corticosteroids. There are effective treatments for idiopathic and scleroderma pulmonary arterial hypertension, which increase exercise tolerance and improve survival. Because idiopathic and COPD pulmonary hypertension share a common vascular intimal thickening, excess endothelin receptor expression, and plasma endothelin-1, an important therapeutic question to address is whether an oral endothelin-1 antagonist can improve exercise tolerance in severe COPD.

Adaptation, Physiological↗

PubMed2002

Lung and heart-lung transplantation at the University of Pittsburgh: 1982-2002.

The University of Pittsburgh lung transplantation program began in 1982 and through 2002 we have performed 576 lung and 101 heart-lung transplants. One-, 3- and 5-year survival rates over the past decade have been 77%, 62% and 55%, respectively, comparing favorably to ISHLT registry outcomes of 73%, 57% and 46%. We continue to utilize a very thorough evaluation process but have been flexible and aggressive with potential recipients with regard to age, coronary artery disease and disease state (eg., scleroderma). Despite worldwide progress in the field of lung transplantation, many difficulties remain. The limited number of lungs deemed acceptable for transplantation continues to hinder application to a greater number of patients. Our efforts in this regard have focused on cooperation with our OPO in education and detailed donor management protocols. Chronic rejection also remains a major difficulty frequently leading to death. Recent work utilizing aerosol cyclosporine in patients with established chronic rejection suggests that this therapy may prolong life. We are also hopeful that recently initiated therapies utilizing T-cell induction strategies may contribute to further improvement in outcomes.

Brain Death↗

PubMedDate not supplied

The 72-kilodalton IE-1 protein of human cytomegalovirus (HCMV) is a potent inducer of connective tissue growth factor (CTGF) in human dermal fibroblasts.

Latent human cytomegalovirus (HCMV) infection has been implicated in diseases characterized by tissue remodeling. Because of recent evidence indicating the possibility of a partial HCMV reactivation, the purpose of this study was to examine the role of the HCMV immediate early (IE) genes in the regulation of extracellular matrix (ECM) related host genes. Adenoviral vector expressing IE1 was generated to allow efficient gene delivery into human fibroblasts. IE1 stimulated the prolonged expression of connective tissue growth factor (CTGF) and TIMP1. IE1-dependent stimulation of CTGF was partially mediated by TGF-beta. Moreover, whereas collagenous proteins and collagen type 1 mRNA were only transiently induced by IE1 in the majority of fibroblasts, in selected fibroblast strains IE1 induced persistent ECM upregulation for up to 120 hours. This study suggests that transient or limited HCMV reactivation may play a direct role in abnormal matrix remodeling in GVHD, scleroderma, atherosclerosis and other HCMV-linked diseases.

Cell Culture Techniques↗