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PubMed2005

[An up date on pulmonary hypertension related to systemic sclerosis].

Systemic sclerosis (scleroderma) is a chronic, progressive connective tissue disease characterized by typical skin changes and internal organ involvement. Arterial pulmonary hypertension is found in approximately 10-15% of patients with systemic sclerosis, and is considered as one of the most fatal complications of the disease. When pulmonary hypertension secondary to scleroderma related interstitial lung disease, left-sided cardiac insufficiency and thrombo-embolic disease is also taken into account, the frequency of pulmonary hypertension in systemic sclerosis may be as high as.50%. Recent investigations brought new information considering the pathogenesis of pulmoanry hypertension and allowed new therapeutic approches to be introduced. Early diagnosis of pulmonary hypertension is a key issue in clinical practice, but it is a challange in patients with systemic disease such as systemic sclerosis. Current review presents up to date information on the pathogenesis, early diagnosis and treatment of pulmonary hypertension related to systemic sclerosis.

Humans↗
PubMed1978

The Shulman syndrome.

The Shulman syndrome is a symptom complex recently described in the rheumatology literature that is characterized by eosinophilia, hypergammaglobulinemia, and a diffuse scleroderma-like process of the extremities. The onset of illness has been associated with a period of unusual physical exertion. The condition seems to respond to corticosteroid therapy for systemic effect.

Adult↗
PubMed1994

[Systemic sclerosis].

Systemic sclerosis is a generalised connective tissue disorder characterised clinically by thickening and fibrosis of the skin and by the involvement of such internal organs as the kidneys, lungs, heart and gastro-intestinal tract. Although the aetiology of systemic sclerosis is unknown, its prevalence has been reported to be increased in workers exposed to silica. Most pathogenic findings in patients with systemic sclerosis involve changes in the vascular system, immunological anomalies and disturbances in the regulation of fibroblast function. Two subsets are recognised according to the degree of skin involvement: the limited cutaneous form, usually confined to acral features; and diffuse cutaneous scleroderma, manifesting involvement both of the trunk and extremities. The latter subset is characterised by more rapid progression of skin and visceral involvement, poorer prognosis and manifestly reduced survival. No adequate therapy has been found, and optimal use of the available therapies necessitates accurate subset assignment of the patient in order to be able to assess the stage of disease in relation to vascular, immunological and fibroblast function changes.

Adjuvants, Immunologic↗
PubMed2002

Predictors and outcomes of scleroderma renal crisis: the high-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis trial.

OBJECTIVE: The reported frequency of scleroderma M01-R renal crisis (SRC) in diffuse systemic sclerosis (SSc; scleroderma) is 15-20%. Early use of angiotensin-converting enzyme (ACE) inhibitors has markedly improved outcome. The present analysis reexamines the prognostic factors for and outcome of SRC in a prospective cohort of patients with early diffuse SSc. METHODS: We retrospectively evaluated the cohort of SSc patients who participated in the High-Dose Versus Low-Dose D-Penicillamine in Early Diffuse SSc trial. Patients with diffuse cutaneous scleroderma were enrolled if their disease duration was <18 months. Because the trial failed to show a difference between treatment groups, the data were pooled. RESULTS: One hundred thirty-four SSc patients entered the observation period a mean +/- SD of 0.8 +/- 0.3 years after onset of SSc. SRC occurred in 18 patients a mean +/- SD of 0.9 +/- 1.1 years after entry. During a mean +/- SD 4.0 +/- 1.1 years of followup after entry, 9 of the 18 patients died (mean +/- SD 0.6 +/- 0.9 years after SRC onset). Baseline characteristics that predicted SRC included a modified Rodnan skin thickness score of >or=20 (P < 0.01), enlarged cardiac silhouette on radiograph (P = 0.04), large joint contractures (wrist, elbow, knee) (P = 0.008), and prednisone use at entry (P = 0.01). Baseline characteristics that did not predict SRC included age, sex, race, Health Assessment Questionnaire score, fist closure, handspread, lung involvement, muscle weakness, erythrocyte sedimentation rate, and platelet count. In 5 of 10 subjects for whom at least 2 sequential skin scores were available, skin scores increased significantly (P = 0.012) in the 6 months before onset of SRC. CONCLUSION: SRC occurred in 13% of patients soon (mean 11 months) after entry into the cohort. Predictors of SRC identified in this study included higher than average skin score, prednisone use at study entry, large joint contractures, and heart enlargement. Our data suggest, however, that low-dose prednisone alone was not associated with the onset of SRC, except in the appropriate clinical setting. Although ACE inhibitors and dialysis are now readily available, SRC continues to be associated with poor survival (in this study, 50% of patients with SRC died).

Adult↗
PubMed1993

Treatment of systemic sclerosis.

Although there have been no major breakthroughs in scleroderma therapy, new treatments have been tested in patients with systemic sclerosis, including both interferon alfa and interferon gamma. These biologic agents can reduce collagen synthesis, which is a rational target for scleroderma therapy. Debate about the use of photopheresis continues, and it was suggested in a recent editorial that photopheresis is no better than D-penicillamine in the treatment of scleroderma and is more expensive. Cyclosporine appears to have frequent renal toxicity when used to treat scleroderma. Outcome measurements have been concentrated on in scleroderma trials. Several types of scleroderma classifications were compared, and the classification of diffuse and limited scleroderma was strongly related to disease severity. Skin score was systematically compared with mapping the surface area of involved skin, and the skin score was found to be more reliable. A possible prognostic indicator in scleroderma is high-resolution pulmonary computed tomography, which is sensitive in early detection of scleroderma-associated interstitial lung disease. Classification of Raynaud's phenomenon into primary and secondary forms has been proposed, and further testing of the criteria and long-term follow-up is necessary to validate this classification. Over the past year, treatment of vasospasm with prostacyclin analogues has been efficacious with iloprost but not with low-dose oral cicaprost. Tissue plasminogen activator is not beneficial in the treatment of Raynaud's phenomenon. A report of radical microarteriolysis for the treatment of refractory Raynaud's phenomenon seems promising, warranting further investigation.

Clinical Trials as Topic↗
PubMedDate not supplied

CREST syndrome: a variant of systemic sclerosis.

A variant of systemic sclerosis (scleroderma), CREST syndrome affects a relatively small number of patients. CREST (also called limited cutaneous scleroderma) has a more favorable prognosis than the diffuse form of scleroderma. However, as with most of the connective tissue diseases, the daily issues faced by patients are frequently difficult to manage and require diligent support from the health care team. This article summarizes the clinical manifestations and potential management plan to help the patient cope with this little-understood syndrome. The nursing care model follows a problem-oriented approach for nursing management.

Adaptation, Psychological↗
PubMedDate not supplied

Increased levels of circulating intercellular adhesion molecule-1 in patients with systemic sclerosis.

OBJECTIVE: To determine the value of the circulating intercellular adhesion molecule (cICAM-1) as a marker for the inflammatory and fibrotic processes in systemic sclerosis (SSc). METHODS: We determined serum levels of cICAM-1 and of the soluble interleukin-2 receptor (sIL-2R) by an enzyme-linked immunosorbent assay in 33 patients with SSc. These values were compared to the concentrations of acute phase reactants and to the extent of skin involvement in diffuse and limited scleroderma. RESULTS: cICAM-1 was elevated in patients with diffuse SSc (498 +/- 134 ng/ml) as compared with 82 healthy controls (312 +/- 71 ng/ml) (mean +/- SD, p < 0.0001). The elevation of cICAM-1 did not correlate with the duration of disease, the pattern of organ manifestations or the type of treatment. While the concentrations of acute phase proteins were not elevated in SSc, a significant correlation between increased serum sIL-2R and cICAM-1 was observed. CONCLUSION: Increased levels of cICAM-1 indicate an activation of immune processes in SSc. The clinical value of the cICAM-1 determination in SSc can only be judged in longitudinal studies.

Adult↗
PubMed1990

[The fibrillarin (Scl-34) autoantibody in systemic scleroderma].

Reexamination of ANA positive sera with nucleolare fluorescence staining on HEp-2 cells and hamster liver imprints revealed 3 sera with an uniform characteristic pattern. It is characterized by: 1. Strong clumpy fluorescence of nucleoli in the interphase. 2. Missing nucleoplasma staining (pure nucleolar). 3. Characteristic granular staining of the cytoplasma from the condensed chromosoma in mitosis. 4. Additive fluorescence of a nucleolus-like body. 5. Extremely high antibody titers. 6. Negative immunodiffusion. This nucleolar staining pattern was described by Bernstein et al. as a clumpy nucleolar pattern. It is produced through an antibody to the dense fibrillar component of the nucleolus (fibrillarin). This antigen is a protein of the U3 RNP with a molecular weight 34 kDa. Clinically is the fibrillarin antibody highly specific for systemic scleroderma especially with diffuse skin involvement. We found it in 1% of scleroderma patients. Its pathogenetic importance is not known.

Antibodies, Antinuclear↗
PubMed1991

Defects of the retinal pigment epithelium in scleroderma.

We completed ocular examination, including retinal fluoroangiography, in 19 unselected patients with diffuse systemic sclerosis (scleroderma) and compared the findings with those made in 50 consecutive patients with systemic lupus erythematosus, 18 with primary Sjögren's syndrome, 20 with mixed connective tissue disease, and 20 healthy women. Five of 19 scleroderma patients had atrophy of the retinal pigment epithelium (26.3%) while none of the controls and only four of the 88 (4.5%) patients with other connective tissue diseases (P less than 0.01) had this anomaly. Atrophy of the pigmented epithelium of the retina may occur in scleroderma as a result of damage of the choroidal plexus.

Adult↗
PubMed1991

[Study of the capillary microscopy changes in scleroderma and their association with organ disease, clinical manifestations and disease progression].

BACKGROUND: The changes of nailfold capillaries detected by capillary microscopy is a characteristic frequently observed in systemic sclerosis. The relationship between the different capillary microscopic changes and clinical manifestation, organic disease and disease progression were studied. METHODS: Sixty-three patients were studied in whom capillary microscopy was performed and organic disease was determined by analytical and morphological examination. The presence of association between the capillary microscopic signs and clinical manifestation and disease progression were investigated by the chi-square and Fisher tests. RESULTS: Capillary microscopic alterations were found in 95% of the patients. The extense capillary loss was associated as statistically significant to the diffuse form of scleroderma. Statistically significant associations were not found between the different capillary microscopic signs and disease progression and organic disease (global and individual per each organ). CONCLUSIONS: Although capillary microscopic changes are present in most scleroderma patients they are not useful in the evaluation of organ involvement of the disease. The presence of extense capillary loss is linked to worse prognosis in its association to the diffuse form of scleroderma.

Adolescent↗
PubMed2003

Human scleroderma sera contain autoantibodies to protein components specific to the U3 small nucleolar RNP complex.

OBJECTIVE: To determine whether antifibrillarin autoantibodies in scleroderma patients are associated with autoantibodies to protein components specific for U3 small nucleolar RNP (U3 snoRNP). METHODS: Sera from 220 scleroderma patients were examined for antinucleolar autoantibodies (ANoA) and for antibodies to fibrillarin and the U3 snoRNP-specific proteins Mpp10 and hU3-55K. Clinical correlates were determined for the different autoantibody specificities. RESULTS: Fifty-nine of the 220 patients were positive for ANoA, and 31 of these patients were antifibrillarin positive. Anti-hU3-55K was found in 10 patients, all of whom were antifibrillarin positive. Twenty-nine patients had anti-Mpp10 antibodies; 23 of these were antifibrillarin positive and 6 were antifibrillarin negative. ANoA, including antifibrillarin, anti-hU3-55K, and anti-Mpp10, were associated with diffuse, rather than limited, systemic or localized scleroderma. Esophageal and lung involvement were more common in patients with antifibrillarin and anti-Mpp10 antibodies, and the highest frequency was in patients with anti-Mpp10 alone. CONCLUSION: Antifibrillarin autoantibodies are associated with autoantibodies to protein components specific to U3 snoRNP, particularly Mpp10. The prevalence of anti-Mpp10 antibodies in antifibrillarin-positive patients suggests that the U3 snoRNP particle is a source of immunogenic/antigenic material for the anti-snoRNP response in scleroderma. Autoantibodies to snoRNP components were more frequent in patients with diffuse scleroderma than in those with either the limited systemic or localized forms. The increased expression of these antibodies in patients with the more severe form of scleroderma, coupled with the observations that fibrillarin expression is positively linked to collagen expression in fibroblasts and that fibrillarin is overexpressed in scleroderma fibroblasts, suggests a source of snoRNP to initiate and maintain these autoantibody responses.

Adult↗
PubMed1998

Iloprost as cyclic five-day infusions in the treatment of scleroderma. An open pilot study in 20 patients treated for one year.

OBJECTIVE: To evaluate the safety and efficacy of cyclic intravenous iloprost therapy in diffuse or limited scleroderma. METHODS: Twenty patients, 14 women and 6 men with a mean age of 47.8 +/- 8.2 years, were given iloprost infusions for six hours a day during five consecutive days, at a rate of 0.5 to 2 ng/kg/min. The course was repeated every three months for one year. Efficacy was evaluated based on a scleroderma skin lesion score, an ischemic lesion score, a well-being self-assessment score, and lung function tests including measurement of the diffusing capacity of the lung for carbon monoxide. Safety was assessed based on adverse event collection. RESULTS: The scleroderma skin lesion and ischemic lesion scores decreased significantly over the one-year treatment period, from 37.1 +/- 16.5 to 10.2 +/- 6.9 (P < 0.001) and from 31.8 +/- 19.1 to 2.2 +/- 2.0 (P < 0.05), respectively. The well-being self-assessment score also showed a significant improvement, from 71.4 +/- 16.5 to 15.0 +/- 6.6 (P < 0.001). The diffusing capacity for carbon monoxide was decreased in 11 patients at baseline and showed a slight, non significant increase in these patients after the treatment period. No serious or persistent side effects were recorded. CONCLUSION: Cyclic intravenous iloprost therapy was associated with improvements in skin changes and in general health, as well as with a slight increase in the diffusing capacity of the lung for carbon monoxide. Our data suggest that iloprost may act on some of the pathogenetic mechanisms of scleroderma in a way that improves the course of the disease.

Female↗
PubMed1993

Nonspecific motor disorder of the esophagus: a real disorder or a manometric curiosity?

BACKGROUND: Nonspecific esophageal motility disorder (NEMD) has become a catchall term to describe abnormal esophageal manometric findings that do not meet strict criteria for established esophageal motility disorders. The aim of this study was to determine whether NEMD is a real esophageal motility disorder characterized by impairment of its motor function or simply a manometric disturbance with no clinical consequences. METHODS: Esophageal transit of liquid and semisolids was studied using radioscintigraphic techniques in 10 symptomatic patients with manometrically diagnosed NEMD, 26 healthy control subjects, and 40 disease control subjects. The disease controls included 24 patients with achalasia, 9 with scleroderma, and 7 with diffuse esophageal spasm. RESULTS: Patients with NEMD had no impairment of liquid emptying compared with healthy controls. Liquid emptying was markedly delayed in patients with achalasia and scleroderma. However, semisolid emptying was markedly delayed in patients with NEMD compared with healthy controls (P < 0.001), and the extent of its delayed emptying was similar to that seen in patients with achalasia, scleroderma, and diffuse esophageal spasm. CONCLUSIONS: NEMD is not a manometric curiosity but a disorder characterized by selective impairment of semisolid emptying.

Adult↗
PubMed2002

[Scleroderma and pregnancy].

PURPOSE: - The frequency of concurrent scleroderma and pregnancy is low because scleroderma is a rare connective-tissue disease and the mean age of symptom onset is in the early 40s. The reciprocal influence of pregnancy and scleroderma and management of pregnancy are the purpose of this review. Current knowledge and key points. - There is no increase in infertility in women with scleroderma. The effect of pregnancy on scleroderma is a greater risk of renal crisis, especially when scleroderma is evolutive, diffuse and recent. Conversely, pregnancy is characterized by prematurity and small full-term infants because scleroderma induce placentar vascular abnormalities. There is no increase in miscarriages and spontaneous abortions. The pregnant scleroderma patient is a potential anaesthetic challenge because of physical difficulties and visceral involvement. Future and projects. - Planning of pregnancy and follow-up by a multidisciplinary experienced team in high risk pregnancies are guarantees for successful pregnancies. Future research will try to determine the role of microchimerism in scleroderma physiopathology and specify placental findings in order to ameliorate the obstetrical prognosis.

Acute Kidney Injury↗
PubMed2004

A modified model of graft-versus-host-induced systemic sclerosis (scleroderma) exhibits all major aspects of the human disease.

OBJECTIVE: Diffuse systemic sclerosis (SSc; scleroderma) is a debilitating disease characterized by excessive dermal fibrosis with later progression to internal organs. In addition to the fibrotic component, major aspects of the disease include vascular or circulatory involvement and immune dysregulation evidenced by inflammatory cells in affected tissues and production of autoantibodies. Many animal models resembling this disease have been studied, including genetic models in mice and chickens, challenge with chemicals such as bleomycin or vinyl chloride to induce fibrosis, and models of graft-versus-host (GVH)-induced disease using certain strains of mice with differences in minor histocompatibility loci. The present studies were undertaken to determine if alteration of the induction of GVH-induced scleroderma could result in a model that more fully represented the human condition. METHODS: Disease was induced by injection of spleen cells from B10.D2 mice into BALB/c mice deficient in mature T and B cells (recombination-activating gene 2 targeted). Dermal thickening, collagen deposition, vasoconstriction, and parameters of immunity were analyzed. RESULTS: Similar to the human disease, this modified GVH model of SSc demonstrated evidence of dermal thickening, particularly in the extremities, progressive fibrosis of internal organs, vasoconstriction and altered expression of vascularity markers in skin and internal organs, early immune activation, inflammation in skin and internal organs, and autoantibody generation. CONCLUSION: This modified model of GVH-induced SSc exhibits all major components of human disease and is likely to contribute to better understanding of the disease mechanisms and, ultimately, improved treatments for patients.

Animals↗