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PubMed1996

Anti-topoisomerase-I and clinical findings in systemic sclerosis (scleroderma).

The relationship between anti-topoisomerase-I antibodies and clinical findings was studied in 191 patients with definite systemic sclerosis. This was done by performing ELISA to detect antibodies to recombinant topoisomerase-I. Antibodies to topoisomerase-I were found in 72 patients (37%) with systemic sclerosis, which is a higher percentage than reported in most previous reports on a large unselected population. In 43 patients the presence of antibodies to recombinant topoisomerase-I was confirmed using both the immunodiffusion method and ELISA, with similar results. When classified into diffuse versus limited disease, a significant difference in antibody prevalence was demonstrated (P < 0.005), thus indicating that anti-topoisomerase-I antibody detection with ELISA may assist in early identification of systemic sclerosis subtypes.

Autoantibodies↗
PubMed1998

Clinical approach to scleroderma.

Systemic sclerosis (SSc) is a heterogenous disease with a morbidity and mortality that varies widely. Nonetheless, the future clinical course of an individual patient can be estimated based on the severity of skin and internal organ involvement within the first several years of the disease. Patients with limited cutaneous SSc (ISSc) have skin thickening below the elbows or knees and may have face and neck involvement. Patients with this subtype of SSc have Raynaud's phenomenon, digital ulcers, and esophageal dysfunction. Significant morbidity and mortality arises in those patients with ISSc who develop interstitial lung disease or pulmonary artery hypertension. Patients with diffuse cutaneous SSc (dSSc) have skin thickening above the elbows and knees or on the trunk. These patients have a more abrupt onset of disease, often with constitutional symptoms and arthalgias. Severe heart, lung, gut, and renal involvement, if it occurs, tends to develop within the first 5 years of disease, especially within the first several years. Patients with significant internal organ involvement have a poorer prognosis than patients who do not. The goals of the initial history and physical and laboratory examinations are to classify the type of scleroderma as ISSc or dSSc, to estimate disease duration, and to define the extent and severity of organ involvement. Treatment of SSc is organ based. Treatment may reduce morbidity associated with Raynaud's phenomenon, digital ulcers, esophageal dysmotility, esophageal reflux, gut dysmotility, arthralgias, myositis, and pulmonary artery hypertension. Therapy may stabilize lung function in patients with interstitial lung disease with alveolitis and stabilize renal function in patients with renal crisis. The overall prognosis for patients with SSc appears to be improving. Patients with early dSSc should be considered for enrollment onto protocol testing of potential disease-modifying therapies.

Arthralgia↗
PubMed1986

Association of bronchiolitis with connective tissue disorders.

Among 173 consecutive open lung biopsies, nine gave a histopathological diagnosis of bronchiolitis. Seven of these patients had some connective tissue disorder (CTD), six of whom are presented in this report; two had classical and one possible rheumatoid arthritis (RA), one ankylosing spondylitis, one scleroderma, and one developed classical RA four years after biopsy. Four of the patients were smokers, most suffered from breathlessness and cough. In terms of lung function three patients had obstruction, one both restriction and obstruction and three a decreased diffusion capacity. For control purposes peripheral lung tissue was studied histologically from 24 consecutive smoking patients without CTD who underwent a lobectomy for cancer. Intraluminal plugs and mucosal lymphoplasmocytic infiltration of the bronchiolar walls were more prevalent and abundant in the CTD patients than in the controls (p less than 0.02 and p less than 0.001 respectively). Two CTD patients also showed some obliterative bronchiolitis. Corticosteroids were effective in one out of four patients treated. One patient improved and the others did not show any progression during the follow up. The results suggest that smoking alone does not explain the lesions of the small airways found in CTD patients, and that bronchiolitis may be specifically associated with the basic disorder in such cases.

Bronchitis↗
PubMed1978

Cryoablation of drug-resistant ventricular tachycardia in a patient with a variant of scleroderma.

A 37-year-old man with a benign variant of scleroderma (CRST syndrome: calcinosis circumscripta, Raynaud's phenomenon, sclerodactyly, and telangiectasia) presented with recurrent ventricular tachycardia. Preoperative electrophysiologic study suggested that the mechanism of tachycardia was an ectopic pacemaker focus in the right ventricle. Right ventricular dilatation, tricuspid insufficiency, normal pulmonary pressures, and normal coronary arteries were also demonstrated. At surgery, epicardial mapping localized the site of origin of ventricular tachycardia to the anterior right ventricle near the crista supraventricular. Intramural recordings of the site of tachycardia demonstrated autonomous activity unreflected on the peripheral ECG during brief periods of sinus rhythm. Local epicardial cooling of this area with a cryoprobe promptly terminated ventricular tachycardia with resumption of tachycardia on warming. The focus was ablated by freezing the area at -60 degrees C. The patient remained free of dysrhythmia on no anti-arrhythmic agents for eight months at which time he had a single recurrence of ventricular tachycardia from a different site in the right ventricle. This technique offers a method for ablating sites of dysrhythmia arising in diffusely diseased myocardium.

Adult↗
PubMed2000

Pulmonary hypertension with limited cutaneous scleroderma (CREST syndrome).

A patient is described with a typical manifestation of pulmonary hypertension associated with limited cutaneous scleroderma, also known as CREST syndrome. The patient was treated with a calcium antagonist, oral anticoagulation and, because of evidence for parenchymal inflammation of the lung, with low-dose prednisone and cyclophosphamide. This treatment resulted in initial improvement of diffusion capacity and exercise tolerance, however, 1 year after diagnosis the patient died of progressive pulmonary hypertension.

Angiography↗
PubMed1996

Increased pigmentation in scleroderma.

OBJECTIVE: Increased pigmentation is found in patients with scleroderma (systemic sclerosis, SSc) even in areas that have never clinically been involved with skin thickening. We wanted to determine if the pigmentation is due to subclinical sclerodermatous changes, or a systemic factor such as an increase in adrenocorticotropic hormone (ACTH). METHODS: To determine if clinically uninvolved skin that is pigmented differs from nonpigmented skin, skin biopsies were taken from patients with scleroderma from 2 different sites: (1) from pigmented but otherwise clinically normal skin, and (2) from nonpigmented clinically normal skin adjacent to the first site. Biopsies were examined by a dermatopathologist for evidence of dermal and epidermal changes. Cosyntropin stimulation tests were performed after baseline cortisol and ACTH samples were obtained. RESULTS: Six patients with diffuse SSc (dSSc) and 4 with limited SSc (ISSc) had skin biopsies. Patients with dSSc were more likely than those with ISSc to have increased pigmentation in uninvolved skin. Pigmented skin specimens had either a higher content of epidermal melanin and/or a more severe degree of pigmentary incontinence with a higher number of dermal melanophages in the superficial dermis. The cosyntropin stimulation tests were normal and there were no differences between subjects with diffuse and limited SSc or between those with and without increases in pigmentation. CONCLUSION: Clinically uninvolved skin in many of these patients with SSc was abnormal, and subtle changes of SSc were present, especially in the pigmented biopsies. There was no evidence of adrenal deficiency in these patients. It is difficult pathologically to differentiate the changes in pigmented compared to unpigmented skin in patients with SSc, except for changes of increased melanin and pigmentary incontinence.

Adrenocorticotropic Hormone↗
PubMed1989

[Two cases of scleroderma associated with vibration syndrome].

Case 1, a 49-year-old male who had been engaged in repair and reclamation of automobile tires, developed symptoms of vibration syndrome (Raynaud's phenomenon, numbness of both hands, tinnitus and impaired hearing) after some 30 years' use of a grinder and impact wrench. Two years thereafter, multiple sclerodermic lesions appeared over the trunk, upper extremities, and thighs; these disappeared about 2 years later. Histologically, hyperplasia and nodular swelling of collagen bundles were present in the dermis. An immunological study showed the serum to be positive for anti-centromere antibody, but no visceral lesions were demonstrable. This case corresponded to generalized morphea. Case 2, a 53-year-old male, developed symptoms of vibration syndrome (Raynaud's phenomenon, numbness of both hands, impaired hearing and arthralgia) after 25 years' use of a jack hammer in a quarry. Thereafter, sclerodermic changes of the forearms, lower legs, face and abdomen occurred with an associated sclerodactyly. Histological examination of involved skin revealed diffuse hyperplasia and homogenization of collagen bundles throughout the entire thickness of the dermis. These findings, together with serum positivity for anti-RNP antibody and dilation of the lower portion of the esophagus, led us to a diagnosis of progressive systemic sclerosis. We inferred that the vibration syndrome in the present cases might be related etiologically to these forms of scleroderma.

Autoantibodies↗
PubMed2001

Systemic sclerosis with renal crisis and pulmonary hypertension: a report of eleven cases.

OBJECTIVE: To describe a series of systemic sclerosis (SSc) patients with the unusual combination of scleroderma renal crisis (SRC) and pulmonary hypertension (PHT) without interstitial lung disease. METHODS: The medical records of 2,459 SSc patients in the University of Pittsburgh Scleroderma Databank first evaluated between 1972 and 1999 were reviewed. RESULTS: Eleven patients (0.45%) had both SRC and PHT. All had been evaluated since 1979, when angiotensin-converting enzyme (ACE) inhibitor therapy for SRC became available. Seven had SSc with limited cutaneous involvement, and 4 had SSc with diffuse cutaneous involvement. SRC occurred first in all patients except 1, in whom the onsets of SRC and PHT were simultaneous. SRC preceded PHT by a mean of 4.3 years. Four patients had anti-Th/To antibody, 5 had anti-RNA polymerase III antibody, 2 had anti-U3 RNP antibody, and none had anticentromere or antitopoisomerase I antibody. Ten of the 11 patients died, 8 from PHT. Ten patients were being treated with ACE inhibitor drugs when PHT developed. CONCLUSION: In SSc, SRC and PHT are not mutually exclusive complications. SSc patients surviving SRC who have serum antibodies to Th/To, RNA polymerase III, or U3 RNP are at increased risk to develop PHT. ACE inhibitor therapy did not prevent the development of PHT.

Acute Kidney Injury↗
PubMed1996

Measurement of skin elastic properties with a new suction device (II): systemic sclerosis.

We previously reported the skin elastic properties of normal subjects as measured by a newly developed suction device (6). In the present report, we examined the skin elastic properties of 62 patients with systemic sclerosis (SSc), clinically diagnosed as limited or diffuse type. We measured the skin elastic properties on the forearm and chest and investigated correlations with disease type and clinical skin score. Patients with the diffuse type SSc showed significantly lower values than those with the limited type SSc or normal subjects in distention and retraction ability at the chest and forearm. This suction device is clinically useful for the objective evaluation of skin elastic properties in diffuse systemic sclerosis.

Adult↗
PubMed1991

Progressive systemic sclerosis with mental disorder.

We present a clinical case of a 43-year-old female with progressive systemic sclerosis, Basedow's disease, atrial fibrillation and diabetes mellitus. She twice developed exacerbations of a psychotic state. She showed a depressive state followed by a paranoic hallucinatory state and stupor. Electroencephalograms revealed slow abnormal records both in psychotic and remitting periods and 123I IMP SPECT of the brain showed a low blood flow in the psychotic stage. A case of progressive diffuse sclerosis with a paranoic hallucinatory state is rarely reported. As her Basedow's disease had been well controlled, the psychotic symptoms of the present case are regarded as organic hallucinosis and organic delusional disorder due to progressive diffuse sclerosis.

Adult↗
PubMed1993

Autoantibody reactive with RNA polymerase III in systemic sclerosis.

OBJECTIVE: To determine the clinical significance of anti-RNA polymerase III antibody in systemic sclerosis (SSc). DESIGN: A point prevalence study of autoantibody to RNA polymerase III and longitudinal examination of its clinical significance in patients with SSc and in controls. SETTING: University medical center rheumatology practice. PATIENTS: Two hundred fifty-two consecutive new patients with SSc and 170 controls (150 patients with other connective tissue diseases and 20 normal volunteers). MEASUREMENTS: The presence of anti-RNA polymerase III antibody was determined by immunoprecipitation, immunoblotting, and immunodepletion studies. MAIN RESULTS: Serum specimens from 57 of the 252 patients with SSc (23%; 95% CI, 18% to 28%) reacted with RNA polymerase III, compared with none of the specimens from 170 controls (0%; 95% CI, 0% to 2%). In 40 of these 57 specimens, immunoprecipitation studies also showed the presence of RNA polymerase I or II, or both. Anti-RNA polymerase III antibody was detected in sera from 50 of the 111 patients (45%) who had SSc with diffuse cutaneous involvement (dcSSc), 7 of 114 patients (6%) who had SSc with limited cutaneous involvement, and none of 27 patients with an SSc overlap syndrome (P < 0.001). Among patients with dcSSc, anti-RNA polymerase III antibody was more common than antitopoisomerase I antibody (45% compared with 27%; P = 0.008). Patients with anti-RNA polymerase III antibody had a statistically significant higher mean maximum skin thickness score but statistically significant lower frequencies of telangiectasias, inflammatory myopathy, restrictive lung disease, and serious cardiac abnormalities than did patients with antitopoisomerase I antibody. CONCLUSION: Anti-RNA polymerase III antibody is a new marker autoantibody for many patients who have SSc with diffuse or extensive cutaneous involvement.

Antibodies, Antinuclear↗
PubMed1977

Scleroderma in children.

Fifteen children with scleroderma have been presented. All had characteristic cutaneous abnormalities at onset and during the course of disease. All were girls. All had visceral involvement, primarily of the gastrointestinal tract, heart, and lungs. The presence of visceral disease might have been missed without specific and extensive diagnostic procedures, including gastrointestinal barium studies, esophageal motility, pulmonary function and carbon monoxide diffusing capacity, and plethysmography. Raynaud's phenomenon was frequent and accompanied by evidence of occlusive vascular disease. Contractures around joints were readily evident and arthralgias were common, but evidence of objective arthritis was absent. Sixty percent of the patients in this series had ANA. Overlap syndromes with myositis and SLE were present in 7 patients. Three of 15 children died 6-10 years after onset of disease.

Adolescent↗
PubMed1998

QT interval and QT dispersion in systemic sclerosis (scleroderma).

OBJECTIVES: To measure QT interval and QT dispersion, and to evaluate possible relationships between these measurements, heart rate variability parameters, and early myocardial involvement in patients with systemic sclerosis (SSc). DESIGN: Prospective study. SETTING: Tertiary care centre, University 'La Sapienza', Rome, Italy. SUBJECTS: Thirty-eight patients with SSc (35 females and three males, mean age 47 +/- 11 years), 19 patients with the diffuse form of disease and 19 with the limited form, and 17 healthy controls (11 females and six males, mean age 43 +/- 10 years) were studied. INTERVENTIONS: Both patients and control subjects underwent resting 12-lead electrocardiogram and 24-hour Holter monitoring. Moreover, resting myocardial scintigraphy with 99 m Tc-sestamibi was performed in all SSc patients. MAIN OUTCOME MEASURES: Bazett's formula was used to obtain rate corrected value of QT interval (QTc). QT and QTc dispersion were defined as the difference between maximum and minimum QT or QTc interval, respectively. Twenty-four-hour heart rate variability was analysed both in the frequency and in the time domain. RESULTS: Twenty-three SSc patients (60.5%) had myocardial resting perfusion defects (group A) and 15 (39.5%) did not (group B). Maximum QTc interval, QT and QTc dispersion were significantly increased in SSc patients compared to the control subjects. No significant differences between group A and group B were observed for all QT measurements. Furthermore, maximum QTc interval, QT dispersion and QTc dispersion were significantly increased in group A patients compared to the control group. Total power, low-frequency, and high-frequency values were significantly lower in all SSc patients, whether in group A or group B, than in control subjects. On the other hand, low-frequency/high-frequency ratio was similar in all groups. Heart rate variability in time domain analysis showed no statistically significant differences between groups. CONCLUSIONS: Patients with SSc have increased QTc interval, QT dispersion, and QTc dispersion. The role of autonomic nervous system and myocardial involvement on ventricular repolarization in patients with SSc needs further investigation.

Adult↗
PubMed2004

Clinical and laboratory features of scleroderma patients developing skeletal myopathy.

Skeletal muscle involvement, or myopathy, has been a recognized feature of systemic sclerosis (SSc). We studied retrospectively 302 Japanese patients with SSc to elucidate the clinical and laboratory features in scleroderma patients developing skeletal myopathy during their clinical course. Forty-three patients (14%) developed skeletal myopathy during their course of the disease. The mean age of the patients who developed skeletal myopathy was significantly lower than that of those who did not. The ratio of male to female was significantly higher in the myopathic patients. The patients with diffuse cutaneous SSc were more likely to develop myopathy than those with limited cutaneous SSc. The prevalences of heart involvement, pulmonary fibrosis, diffuse pigmentation of the skin, and contracture of phalanges were significantly greater in those with skeletal myopathy than in those without. None of the patients with skeletal myopathy had anticentromere antibody. These findings suggested that the SSc patients with severe internal organ involvement, such as pulmonary fibrosis and heart disease, and some other complications were prone to develop skeletal myopathy during their clinical course of the disease.

Adult↗
PubMed2006

Prognostic markers for systemic sclerosis.

The prognosis of systemic sclerosis depends chiefly on the extent of the skin lesions, which correlates with the severity of the cardiovascular, pulmonary, and renal manifestations. An erythrocyte sedimentation rate greater than 15-25 mm/h or a hemoglobin level lower than 12.5-11 g/dl is associated with a 2.5- to 3-fold increase in mortality. Anticentromere antibodies are associated with delayed pulmonary hypertension, anti-topoisomerase I antibodies (Scl 70) with interstitial lung disease, and anti-RNA polymerase III antibodies with renovascular hypertension. The risk of death is directly related to the autoantibody pattern. For instance, in a study of 1432 cases from the Pittsburgh Scleroderma Databank, 10-year survival among patients with limited cutaneous disease was 88% in the group with anti-U1-RNP, 75% in the group with anticentromere antibodies, 72% in the group with anti-PmScl, and 65% in the group with anti-Th/To. Ten-year survival in patients with diffuse cutaneous disease was 64% with anti-topoisomerase antibodies, 61% with anti-U3-RNP, and 75% with anti-RNA polymerase III. Several prognostic markers are also available for predicting the risk of organ involvement. For instance, serum levels of KL-6, surfactant proteins SP-A and SP-D, the collagen peptide PIIINP, and homocysteine are associated with the risk of fibrosing alveolitis. Serum levels of CD40L and NP-ProBNP, circulating endothelial cells, and serum anticardiolipin titers correlate with the risk of arterial hypertension. Serum VCAM1 and markers for oxidative stress such as carboxyl terminus residues predict the risk of vascular disease. Other serum markers for organ involvement are under study, although their predictive performance remains to be evaluated.

Autoantibodies↗
PubMed1998

Antiendothelial cell antibodies in scleroderma correlate with severe digital ischemia and pulmonary arterial hypertension.

OBJECTIVE: To determine the prevalence of IgG antiendothelial cell antibodies (AECA) in patients with scleroderma (systemic sclerosis, SSc) and to correlate it with clinical spectrum and autoantibody profile. METHODS: Seventy-six patients with SSc and 50 matched healthy controls were studied. Immunological variables were antinuclear antibody (ANA), rheumatoid factor (RF), and Scl-70. IgG-AECA was measured by cellular ELISA. RESULTS: The prevalence of IgG-AECA was 27.6% in patients with SSc compared to 6% in controls (p < 0.01). Forty percent of patients with diffuse disease had this antibody, versus 13.5% of those with limited cutaneous involvement (p < 0.05). Patients with AECA had significantly higher incidence of digital infarcts and gangrene (p < 0.01) and pulmonary arterial hypertension (p < 0.001) than those without. In the AECA positive group, mean IgG-AECA levels (measured by absorbance values) were significantly higher in patients with digital infarcts (0.91+/-0.31 vs 0.60+/-0.05; p < 0.01) and pulmonary arterial hypertension (1.14+/-0.37 vs 0.68+/-0.13; p < 0.001) compared to those without these features. CONCLUSION: IgG-AECA appears to be an important marker for disease severity in scleroderma.

Adolescent↗