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At least 811 records · Page 45Linked to original sources
PubMed1993

Overlapping syndromes, undifferentiated connective tissue disease, and other fibrosing conditions.

Connective tissue diseases (CTDs) frequently present as undifferentiated disorders that may or may not develop into one of the well defined CTDs. The natural evolution of the early undifferentiated CTDs is not well known. Some years ago a cooperative study was started on 410 patients with early CTD; within this group, 48 patients had early scleroderma. Raynaud's phenomenon has been recognized as an early sign of CTD. Several studies on Raynaud's phenomenon patients have shown that abnormal nailfold capillary patterns have prognostic significance for evolution to a CTD, particularly scleroderma. Mixed connective tissue disease (MCTD) is the prototype of an overlap syndrome. The relevance of defining MCTD as a separate disease entity has been challenged. Follow-up studies show that many patients originally diagnosed with MCTD develop a definite CTD, particularly scleroderma or systemic lupus erythematosus, within a few years. In patients with MCTD, primary pulmonary hypertension is an important cause of death. This condition seems to be associated with anticardiolipin antibodies. A number of case reports suggest that immunosuppressive treatment is of benefit in this almost always fatal condition. The main reason for describing MCTD as a separate entity is its association with antibodies to U1-RNP. Recent studies have suggested that the production of anti-U1-RNP is an antigen-driven process, but the nature of the trigger for its production has not been elucidated. Retroviral proteins do not appear to be involved. Antibodies to the protein part of the U1-RNP complex and antibodies to the RNA component have been described. Levels of anti-RNA antibodies seem to parallel disease activity in MCTD patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Overlapping syndromes, undifferentiated connective tissue disease, and other fibrosing conditions.

Mixed connective disease is the prototype of an overlapping connective tissue disease. Based on the presence of a specific autoantibody directed against U1-snRNP (U1 small ribonucleoprotein), the syndrome has been considered a distinct disease entity. Patients with anti-U1-snRNP antibodies, however, do not present with a homogeneous clinical syndrome, and the majority develop a classified connective tissue disease within 5 years of presentation. Signs of scleroderma predominate in most patients. The immune response resulting in anti-U1-snRNP seems antigen driven and may result from molecular mimicry between U1-snRNP and certain viral proteins. Fibrotic conditions related to extrinsic factors have been described as human adjuvant disease. Case reports suggest that Swanson silastic joint prostheses can induce granulomatous reactions at a distance from the joints due to leaking of silicone from the implants. Much attention recently has been given to the possible induction of scleroderma-like syndromes in relation to silicone gel breast implants. However, the presently available data do not show convincing evidence that women with silicone gel breast implants are significantly at risk for developing a connective tissue disease.

Breast Implants↗
PubMed1995

Overlapping syndromes, undifferentiated connective tissue disease, and other fibrosing conditions.

Connective tissue diseases (CTDs) frequently present with one or only a few symptoms, which does not allow prompt diagnosis. Raynaud's phenomenon is one of those symptoms. However, only a minority of patients who present with Raynaud's phenomenon develop a CTD. Prognostic factors for the future development of CTD in such patients are older age at presentation, more severe Raynaud's phenomenon, the presence of antinuclear antibodies, and abnormal patterns on nailfold capillary microscopy. Some patients have overlapping symptoms of various CTDs. Mixed connective tissue disease (MCTD) is the prototype of such an overlapping syndrome. However, during follow-up, most patients with MCTD develop a specific CTD, either scleroderma, systemic lupus erythematosus, rheumatoid arthritis, or combinations of those illnesses. Primary pulmonary hypertension is one of the leading causes of death in MCTD. Its treatment is insufficient, although continuous prostacyclin infusion may provide some relief. New therapies such as nitric oxide and combined heart-lung transplantation in an early stage should be explored. The autoimmune response to small nuclear ribonucleoproteins, which is highly characteristic for MCTD, interestingly shows cross-reactivity with retroviral antigens, and the cooccurrence of human T cell lymphotropic virus type I and HIV infection with MCTD has been reported. This suggests that those viruses, possibly by molecular mimicry, play a role in the induction of the disease. Fibrotic conditions related to silicone exposure still evoke much interest. However, most recent data do not substantiate a role for silicone gel breast implants in the development of autoimmune CTDs.

Connective Tissue Diseases↗
PubMed2004

Two patients with abnormal skeletal muscle uptake of Tc-99m hydroxymethylene diphosphonate following liver transplant: nephrogenic fibrosing dermopathy and graft vs host disease.

Tc-99m hydroxymethylene disphosphonate administered for skeletal scintigraphy may be seen in the extraosseous tissues for various etiologies. This report presents similar abnormal lower extremity skeletal muscle uptake in 2 patients status postorthotopic liver transplant. Although the history and scintigraphic appearance of the 2 were similar, subtle differences in cutaneous lesions and histology led to disparate diagnoses.

Fibrosis↗
PubMed1993

Increased serum concentrations of procollagen type III peptide in severely injured patients: an indicator of fibrosing activity?

OBJECTIVES: To determine the serum concentrations of procollagen type III peptide in severely injured patients with different outcomes and to evaluate the relationship between serum procollagen type III peptide concentrations, sources of increased posttraumatic fibrotic activity (wounds, lung, liver, kidney), and decreased elimination of procollagen type III peptide (liver). DESIGN: Prospective study. SETTING: Surgical ICU, university hospital. PATIENTS: Fifty-seven patients (mean injury severity score: 38.5 points, range 13 to 75 points), between 16 and 70 yrs of age, treated in our institution within 6 hrs after the accident. MEASUREMENTS: Serial measurements were started on admission and continued on a 6-hr basis. After 48 hrs, the monitoring interval was extended to 24 hrs until recovery (but at least until day 14) or death. At each point of evaluation, pulmonary and circulatory function parameters and chest radiographs (once a day) were evaluated, the results were recorded, and blood samples were drawn to determine procollagen type III peptide, total bilirubin, creatinine, gamma-glutamyl transferase, polymorphonuclear elastase, and other parameters. Statistic evaluation was done with the Wilcoxon test, Spearman rank correlation, and a multiple regression model. RESULTS: Mean procollagen type III peptide serum concentrations (+/- SD) were significantly different in patients who died (8.0 +/- 3.8 U/mL) compared with those patients who survived with organ failure (2.7 +/- 1.3 U/mL) or without complications (1.4 +/- 0.5 U/mL), respectively. Significant correlations of procollagen type III peptide concentrations with the serum bilirubin concentrations (r = .7), days with need of mechanical ventilation (r = .64), PaO2/FIO2 ratio (r = -.6), polymorphonuclear elastase (r = .6), serum creatinine concentrations (r = .55), and injury severity score (r = .33) were observed. There was a tendency toward higher serum procollagen type III peptide concentrations in patients with severe skeletal injuries. CONCLUSIONS: Serum procollagen type III peptide concentrations in severely injured patients may be considerably increased in correlation with injury severity and outcome. Procollagen type III peptide serum concentrations seem to reflect the sum of increased collagen formation from wound healing and fibrogenesis of mediator-related organ damage (especially lung) and decreased procollagen type III peptide excretion due to impaired liver function. Further data are necessary to evaluate the role of hepatic elimination in these patients.

Adolescent↗
PubMed1997

Fibrosing inflammatory pseudotumors of the central skull base.

The most important step in the differential diagnosis of mass lesions of the central skull base is to rule out malignant neoplasms. However, nonneoplastic lesions, such as infections or nonspecific inflammatory lesions of the skull base, can mimic malignant processes. In this study, the authors analyzed seven cases of nonneoplastic noninfectious mass-forming lesions involving the central skull base. In most cases, malignant processes were suspected at the initial phase of diagnostic work-up, but subsequent histologic examinations revealed that these lesions consisted of inflammatory cells and fibrosis without neoplastic cells. Common manifestations were pain and other neurological symptoms related to the involved anatomical sites. A variety of neurological dysfunctions of the cranial nerves not including the olfactory and spinal accessory nerves were observed. No patient developed separate lesions outside the head and neck region. After the pathologic diagnosis, most of the patients were treated with oral steroid therapy, with initial doses of prednisolone, 60 to 100 mg/d. It was difficult to relate responsiveness to steroid therapy with the histologic degree of sclerosis, fibrosis, or chronicity of the disease in these cases. Otolaryngologists should be aware of this disease when making treatment decisions for their patients with skull base lesions.

Adult↗