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PubMed1981

[Incapacitating pansclerotic morphea in childhood].

An 8-year old boy with generalized morphea involving all levels of the skin and soft tissues with disabling course, policlonal elevation of gammaglobulins and peripheral eosinophilia is examined. Neither Raynaud's disease nor sings of systemic scleroderma were present. The biopsy specimen showed thickening and hyalinization of collagenous tissue with moderate lymphocytic and plasma cell infiltration, mainly in the subcutaneous region and fascia. A diagnosis of Disabling Panscleroti morphea of children (Díaz-Pérez et al., 1980) was done. With this case and others reported in the literature, the authors compare this peculiar picture with the usual type of morphea, systemic scleroderma and eosinophilic fasciitis.

Antibodies, Antinuclear↗
PubMed1985

[Minimal etiological evaluation of Raynaud's phenomenon. 102 cases].

The efficacy of a minimal aetiological investigation program was tested in a population with Raynaud's phenomenon. The program had been devised to fulfill the following criteria: non invasive, simple and relatively cheap methods capable of detecting systemic scleroderma at an early stage. These methods turned out to be physical examination, screening for antinuclear antibodies, capillaroscopy and radiography of the hands and chest. After investigations which, in addition to the minimal program, included exploration of the digestive and respiratory tracts, heart, kidneys, articulations and eyes, 102 consecutive cases of Raynaud's phenomenon were studied and divided into 3 groups: idiopathic (n = 15); secondary (n = 50, including 36 cases of collagen disease, 29 of which were systemic scleroderma), and suspected idiopathic or pre-collagen disease (37 cases of undetermined origin but not considered idiopathic in view of clinical and/or paraclinical abnormalities). In non idiopathic Raynaud's phenomenon, physical examination, capillaroscopy and screening for antinuclear antibodies gave abnormal results in 96.5% of the cases. This figure was raised to 98.8% when radiography of the hands and chest was added to these 3 examinations, but the difference was not significant. Since 96.5% of non idiopathic Raynaud's phenomena could be diagnosed by physical examination, capillaroscopy and screening for antinuclear antibodies, it is suggested that all patients presenting with the phenomenon should be subjected to these three simple examinations; negative results then dispense from supplementary investigations.

Adolescent↗
PubMed1988

Familial clustering of scleroderma spectrum disease.

This is the second case report of familial scleroderma (systemic sclerosis) in South Carolina. The family includes two cases of scleroderma meeting American Rheumatism Association criteria, one of systemic sclerosis sine scleroderma, and two other cases of undifferentiated connective tissue disease with features of scleroderma spectrum disorders; there are also two cases of Raynaud's phenomenon (one associated with rheumatoid arthritis), for a total of seven affected relatives. Evidence of scleroderma spectrum disorders was sought in six siblings of the two co-index cases and in 23 of the 35 offspring. Laboratory studies included antinuclear antibody determinations and typing for the following genetic markers: HLA (A, B, C, DR), complotypes, Gm and Km allotypes, and alpha-1 antitrypsin phenotypes. No common genetic markers restricted to affected members of this family were found, and no environmental exposures were detected that could explain this familial clustering of cases. This report should, however, add to the slowly accumulating information on the genetic characteristics of families at unusually high risk for scleroderma spectrum disorders. Positive antinuclear antibody tests at a titer of 1/40 or higher were present in 57 percent of the first-degree relatives of the affected cases.

Adult↗
PubMed1999

Thrombotic thrombocytopenic purpura as an initial presentation of limited systemic sclerosis.

Thrombotic thrombocytopenic purpura (TTP) is a rare complication of scleroderma (systemic sclerosis, SSc). In the 5 reports documenting the association of TTP and SSc, the TTP syndrome developed on a background of well established SSc. We describe a 51-year-old woman with a 5 month history of an evolving connective tissue disease syndrome who presented initially with TTP, followed 4 months later by limited cutaneous SSc and Raynaud's phenomenon.

Brain↗
PubMed2002

Antibody-mediated gastrointestinal dysmotility in scleroderma.

BACKGROUND & AIMS: Defects in enteric excitatory neurotransmission have been proposed to underlie the gastrointestinal dysmotility associated with scleroderma (systemic sclerosis). This study investigated whether patients with scleroderma produce antibodies that inhibit M3-muscarinic or neurokinin receptor-mediated intestinal contractions, either directly or via an effect on L-type voltage-gated calcium channels (VGCCs). METHODS: Responses of mouse colon longitudinal muscle to stimulation by the muscarinic agonist carbachol (1-300 micromol/L) and neurokinin-1 and -2 receptor agonists were measured in the absence and presence of serum (2%) or immunoglobulin G (IgG) (0.3-1.0 mg/mL) from patients with scleroderma, those with other autoimmune disorders, and healthy controls. The role of L-type VGCCs in carbachol- and tachykinin-evoked contractions was assessed using nicardipine. RESULTS: M3-muscarinic receptor-mediated contractions were inhibited by Ig fractions from 7 of 9 patients with scleroderma (limited and diffuse forms), 4 of 4 patients with primary Sjögren's syndrome, and 3 of 3 patients with secondary Sjögren's syndrome. Ig fractions from healthy controls did not inhibit the M3-muscarinic receptor-mediated contractions. Inhibition by Ig was concentration-dependent; a maximum inhibition of approximately 40% occurred at 0.6 mg/mL IgG. Both M3-muscarinic and neurokinin receptor-mediated contractions were L-type VGCC dependent. Patient sera had no effect on responses to neurokinin receptor stimulation, demonstrating the lack of antibodies inhibiting L-type VGCCs. CONCLUSIONS: Functional antibodies specifically inhibiting M3-muscarinic receptor-mediated enteric cholinergic neurotransmission may provide a pathogenic mechanism for the gastrointestinal dysfunction seen in patients with scleroderma.

Aged↗
PubMed1996

VCAM-1 expression on endothelium in lesions from cutaneous lupus erythematosus is increased compared with systemic and localized scleroderma.

The cutaneous lesions in systemic sclerosis (SSc) and lupus erythematosus (LE) are pathologically distinct and may display separate cell adhesion receptors. We have scored lesional skin for the presence of cell adhesion molecules that may influence inflammatory and fibrotic processes in five patients with LE, six patients with diffuse scleroderma and four patients with morphoea. The immunohistological distribution, and the number and intensity of cells staining, were recorded for VCAM-1, ICAM-1, E-selectin, alpha 2 to alpha 6 and beta 2 integrins and HLA-DR. VCAM-1 staining intensity was increased on endothelium from lesions in LE compared with SSc (P = 0.05). Low-level VCAM-1 and E-selectin expression was present on endothelium from uninvolved skin including that from patients with morphoea. HLA-DR expression was increased on infiltrating mononuclear cells (P < 0.05) and keratinocytes in LE (P < 0.05) and the number of fibroblasts staining for ICAM-1 was increased in lesions from patients with SSc, although this did not reach statistical significance. Overall, with respect to endothelial adhesion events, our findings support an important role for VCAM-1 in sustaining chronic inflammation in cutaneous LE.

Adult↗
PubMed1998

Interstitial lung disease in systemic sclerosis: comparison of BALF lymphocyte phenotype and DLCO impairment.

Patients with scleroderma (systemic sclerosis-SSc) frequently develop an interstitial lung disease. The role of lymphocytes in fibrosing alveolitis preceding lung fibrosis has been established. The purpose of this work was to evaluate cell profiles and lymphocyte phenotypes in the bronchoalveolar lavage (BAL) fluid and to correlate them with depression in lung function tests detected by depletion of diffusing capacity (DLCO). BAL was carried out in 25 untreated, non-smoking patients suffering from diffuse scleroderma and in 12 healthy non-smoking volunteers. For the analysis of lymphocyte sub-sets flow cytometry and monoclonal antibodies were used. The following cell sub-types were counted: T lymphocytes, B lymphocytes, helper lymphocytes, suppressor/cytotoxic lymphocytes, natural killer cells, cytotoxic T lymphocytes and activated T lymphocytes. The total cell count was higher in the group of patients with mild and moderate impairment in DLCO. The percentage of lymphocytes was greater in patients with DLCO lower than 65% of the predicted value since neutrophilia was found in patients with severe DLCO depletion, i.e. significant when compared with healthy subjects. The proportions of suppressor/cytotoxic lymphocytes and of activated T lymphocytes were higher in patients than in controls. The statistical analysis revealed significant differences between patients with moderate and mild changes in DLCO and the healthy volunteers. A decreased helper/suppressor ratio was noticed in these patients. We concluded that the BALF lymphocyte phenotype analysis may reflect the features of alveolitis in patients with SSc.

Adult↗
PubMedDate not supplied

[Heart involvement in progressive systemic sclerosis (generalized scleroderma)].

17 patients with progressive systemic sclerosis were examined for cardiac involvement using standard ECG, chest X-ray, 24-h Holter ECG, and echocardiography. The control groups consisted of 7 patients with mixed connective tissue disease, 10 patients with systemic lupus erythematosus, and 10 patients with rheumatoid arthritis. The results disclosed a cardiac involvement of 70% using all 4 methods, which is more than is reported in the literature. The data also show that all 4 methods are necessary to diagnose the cardiac involvement. Conduction and rhythm disorders especially are more frequent than in other collagen vascular diseases. The use of calcium channel blockers is discussed as a new therapeutic possibility.

Electrocardiography↗
PubMed1994

Increased frequency of in vivo hprt gene-mutated T cells in the peripheral blood of patients with systemic sclerosis.

OBJECTIVE: Activated T lymphocytes are involved in the pathogenesis of scleroderma (systemic sclerosis, SSc); such cells rapidly divide in vivo and are thus theoretically subject to random mutation more frequently than resting cells. To study whether SSc is associated with rapidly expanding T cell clones the frequency was determined of in vivo mutated T cells (MF) at the hypoxanthine guanine phosphoribosyl transferase (hprt) gene in the peripheral blood from patients with SSc. Specific clinical or serological associations were also investigated. METHODS: Peripheral blood lymphocytes from 16 healthy individuals and 20 patients with SSc were cultured using an hprt clonal assay; mutated and wild T cell clones were established to assess individual values of T cell MF. T cell clones were further expanded in vitro and their phenotype was determined by standard immunofluorescence technique. Enzyme-linked immunosorbent assays were used for simultaneous measurements of plasma levels of soluble Interleukin-2 receptors (s-IL-2R) and Intercellular adhesion molecule-1 (s-ICAM-1). RESULT: Mean (SD) value of T cell MF in patients with SSc was 2.5-fold higher than the normal mean (SD) value [10.6 (6.6) x 10(-6) v [4.4 (2.8) x 10(-6), p = 0.0007]. Eleven of 20 patients with SSc (55%) had T cell MF values greater than two SD above the normal mean value. The majority (84%) of mutated T cells had a helper/inducer, memory phenotype while 12% were cytotoxic/suppressor T cells. There was no association between T cell MF and the extent of skin involvement or the duration of Raynaud's phenomenon. High individual T cell MF values were not related to a possible concurrent immune overactivity as assessed by plasma levels of s-IL-2R and s-ICAM-1. Patients with long standing skin disease, however, had almost double T cell MF values than patients with early skin disease [(13.6 (7.4)) x 10(-6) v (7.5 (4.3)) x 10(-6), p = 0.03], suggesting that increased T cell MF in SSc may reflect an ongoing process of chronic in vivo T cell proliferation and/or prolonged survival. CONCLUSION: Increased in vivo T cell mutation in patients with SSc suggests that excessive division and/or survival of T cell clones contribute to the pathology in SSc; this approach can be used in further investigations to identify the stimulus that is triggering T cell activation in this disease.

Adolescent↗
PubMed1986

[The coronary hyperemic reaction in scleroderma].

Functional, organic and vascular anomalies could play an important role in systemic scleroderma. We have studied the coronary hyperemic reaction in subjects with primitive sclerodermatous cardiomyopathy, in order to specify the vascular anomalies. The coronary hyperemic reaction consists of a transient rise in coronary flow after selective injection of a contrast agent into the left coronary artery. In sclerodermatous subjects this coronary hyperemic reaction is significantly smaller than in control subjects. This limitation of the hyperemic reaction reflects a decrease in the ability of small arteries or coronary arterioles to dilate in sclerodermatous subjects. This anomaly could play an important role in primitive sclerodermatous cardiomyopathy.

Cardiac Catheterization↗
PubMed1997

Scleroderma renal crisis complicated by hemolytic uremic syndrome in a case of elderly onset systemic sclerosis.

We report a case of renal crisis in a patient with elderly onset systemic scleroderma (SSc). A sixty-one-year-old woman was diagnosed as having SSc with rapidly advancing generalized skin sclerosis. After experiencing an upper respiratory infection, she suddenly developed renal failure, hemolytic anemia and malignant hypertension. Laboratory examination revealed uremia with a significantly high plasma renin level. Ophthalmologic study revealed Keith-Wagner's retinopathy Grade IV. Combination therapy including captopril, systemic corticosteroid and prostaglandin E1 venous infusion for the hemolytic uremic syndrome was effective and saved her from the renal crisis. However, her renal function deteriorated and she needs permanent hemodialysis. Rapidly progressive skin sclerosis in SSc, especially in elderly onset cases, suggests a high risk for renal crisis and indicates the need for careful consideration of hypertension and renal function.

Acute Disease↗
PubMed2003

Homocysteine plasma concentration is related to severity of lung impairment in scleroderma.

OBJECTIVE: To investigate the correlation between plasma concentration of total homocysteine and pulmonary involvement in patients with limited or diffuse scleroderma (systemic sclerosis, SSc). METHODS: Seventy-one patients with scleroderma were divided into 3 groups based on pulmonary involvement: Group A comprised patients without lung involvement (9 cases); Group B patients with lung involvement of mild and moderate stages (44 cases); and Group C patients with lung involvement of severe stage and endstage (18 cases). At the time of evaluation of lung involvement all patients underwent determination of plasma homocysteine concentration. Homocysteine concentration was also measured in 30 healthy controls homogeneous for sex and age. RESULTS: In patients with scleroderma the homocysteine concentration was significantly higher than in controls (11.1 and 6.9 micromol/l, respectively; p < 0.001). We found a significant association between plasma homocysteine concentration and severity of lung involvement that was not modified by correction for age, time from the diagnosis, type of scleroderma pattern, and serum creatinine and folate levels. Homocysteine concentration progressively increases in scleroderma patients with more severe pulmonary involvement. Subjects with high homocysteine concentration (i.e., > or = 75th percentile of homocysteine concentration in patients with scleroderma without lung involvement) were mostly present in the group with the greatest lung involvement. CONCLUSION: High level of homocysteinemia is associated with an increased risk of pulmonary disease in patients with scleroderma. We hypothesize that hyperhomocysteinemia may worsen injury of the endothelium, a key lesion in scleroderma disease, favoring the development of lung involvement. Our data support the hypothesis that homocysteine could be involved in the pathogenetic process of scleroderma pulmonary involvement.

Adult↗
PubMed1995

Interstitial lung disease of systemic sclerosis.

Fibrosis of the pulmonary parenchyma is a frequent and serious complication of scleroderma (systemic sclerosis, SSc), resulting in significant morbidity and mortality. During the past decade data have accumulated in support of an inflammatory process affecting the alveoli and distal airways that culminates in irreversible fibrosis in many SSc patients. Recent findings indicate the presence of lung fibroblasts with altered phenotype and biologic activity (myofibroblasts), perhaps arising from the influence of cytokines on resident lung fibroblasts. Acute-phase inflammatory cytokines such as IL-1 alpha, TNF-alpha, MIP-1 alpha, IL-8 and RANTES are increased in SSc bronchoalveolar lavage (BAL) fluid, as is thrombin, a potent mitogen for lung fibroblasts. Chronic-phase inflammatory and fibrogenic cytokines such as PDGF and TGF-beta are also present in increased amounts in SSc BAL fluid. The inciting event(s) and the process(es) leading to the perpetuation of fibrosis in SSc are unknown. Treatment of SSc lung disease has been empiric and generally disappointing, and it is likely that effective treatment awaits a better understanding of the biological events that regulate collagen and other extracellular matrix synthesis.

Bronchoalveolar Lavage Fluid↗
PubMed2002

[Treatment of scleroderma].

No single therapeutic agent has been shown to increase survival of patients with systemic scleroderma in a prospective randomized trial. Drugs as colchicine and D-penicillamine are disappointing. Conversely, disease modifying agents are emerging such as cyclophosphamide in interstitial pulmonary disease, and stem cell autograft after high dose cyclophosphamide therapy in patients who develop visceral involvement in the three first years of evolution of the disease is under study. Organ specific therapy may show significant benefit, such as angiotensin converting enzyme inhibitors in renal crisis and epoprostenol in primary pulmonary hypertension.

Angiotensin-Converting Enzyme Inhibitors↗