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PubMed1992

Antibodies to mycobacterial 65 kDa heat shock protein in systemic sclerosis (scleroderma).

It has been reported that immunity to the 65 kDa heat shock protein of Mycobacterium tuberculosis (MT-hsp65) not only accompanies rheumatoid arthritis (RA), but may also be characteristic of chronic inflammation. We now report serum antibodies to MT-hsp65 in 47% of systemic sclerosis (SSc), 38% of primary Raynaud's phenomenon (PRP) and 5% of systemic lupus erythematosus (SLE). Antibody levels were higher in patients with active or progressive SSc and correlated with the degree of skin fibrosis. Thus, immunity to MT-hsp65 appears in SSc and is not limited to RA. However, it does show some degree of specificity beyond chronic inflammation: PRP patients have a higher reactivity than do SLE patients.

Adolescent↗
PubMed1999

The association of anti-Ro52 autoantibodies with myositis and scleroderma autoantibodies.

The presence of autoantibodies to the Ro52 protein in sera from patients with idiopathic inflammatory myopathies has recently been reported. These antibodies were found predominately in sera with the myositis-specific autoantibody anti-histidyl-tRNA synthetase (anti-Jo-1). In this report, we analysed sera from 216 patients to determine whether anti-Ro52 antibodies are associated with myositis autoantibodies other than anti-Jo-1. These included sera containing antibodies that recognize threonyl- or alanyl-tRNA synthetases, Mi-2, PM-Scl, signal recognition particle (SRP), as well as the systemic sclerosis-related antibodies anti-topoisomerase I (Scl-70) and anti-centromere. A high proportion of sera that contain anti-aminoacyl-tRNA synthetase antibodies, anti-SRP, or anti-PM-Scl antibodies were found to contain antibodies to the Ro52 protein. In contrast, in sera containing anti-Mi-2, anti-Scl-70 or anti-centromere antibodies, anti-Ro52 antibodies were absent or occurred infrequently. In addition, only one serum from 41 rheumatoid arthritis patients was positive for anti-Ro52 autoantibodies. These data indicate that anti-Ro52 antibodies are produced in particular subsets of myositis patients, and are not limited to sera with anti-Jo-1 antibodies.

Amino Acyl-tRNA Synthetases↗
PubMed1981

Human skin fibroblasts in culture: procollagen synthesis in the presence of sera from normal human subjects and from patients with dermal fibroses.

Various dermal fibrotic conditions, such as progressive systemic sclerosis, localized morphea and familial cutaneous collagenoma, are characterized by excessive deposition of collagen in the skin. In the present study, we examined the possibility that a circulating serum factor(s) is responsible for increased collagen production in these diseases. The effects of human serum on the synthesis of procollagen were examined by incubating normal human dermal fibroblasts with [3H]proline and varying concentrations of dialyzed heat-inactivated serum. The synthesis of procollagen was measured as formation of nondialyzable [3H]hydroxyproline and collagenase-digestible 3H]polypeptides. In the absence of serum little procollagen was formed but the synthesis was markedly stimulated by the addition of normal serum in a concentration-dependent manner. THe ratio of genetically distinct 3H-procollagens of type I and type III, assayed by DEAE-cellulose chromatography and SDS-polyacrylamide gel electrophoresis after limited pepsin proteolysis, was unaffected by the addition of serum. Thus, normal human serum contains a nondialyzable factor(s) which stimulates the synthesis of procollagens type I and type III equally. Sera from 5 patients with progressive systemic sclerosis, 3 with localized scleroderma, and 2 with familial cutaneous collagenoma were also tested. Sera from these patients failed to stimulate 3H-procollagen production more than sera from healthy age-matched controls. Therefore, no increased quantities or qualitatively aberrant factors were shown to be present in the sera of these patients.

Blood Physiological Phenomena↗
PubMed1999

Clinical and laboratory manifestations of systemic sclerosis (scleroderma) in Black South Africans.

A retrospective study of systemic sclerosis (SSc) in Blacks attending a tertiary hospital on the Witwatersrand, South Africa, was undertaken. The female:male ratio of the 63 patients was 4.6:1 and the mean age of onset of SSc was 36.1 yr. Four of the 11 males were ex-goldminers and nine females resided close to goldmines. Forty-one patients had diffuse cutaneous SSc (dcSSc), 18 had limited cutaneous SSc (lcSSc) and four were unclassified. Overall, 56% had pulmonary fibrosis, 37% had myositis and 98% were antinuclear antibody (ANA) positive, with a notable absence of anti-centromere antibodies. Subset comparisons showed myositis and a reduced forced vital capacity to be significantly more common with dcSSc than lcSSc. The only significant sex differences were that arthralgia/arthritis was more common in women, while calcinosis occurred more frequently in men. Seven of the eight known deaths occurred in patients with dcSSc. These findings, particularly the age of disease onset, predominance of the dcSSc subset, inflammatory features of myositis and a raised erythrocyte sedimentation rate, and absence of anti-centromere antibodies, are similar to those reported previously in African-Americans.

Adolescent↗
PubMed2000

Cyclosporin and tacrolimus: their use in a routine clinical setting for scleroderma.

BACKGROUND: Cyclosporin and tacrolimus are immunomodulatory drugs which act predominantly on T cells. Improvements in certain manifestations, particularly skin tightness, have been observed in a number of patients with scleroderma treated with these drugs. However, to date there have been no reports of their use in a routine clinical setting. METHODS: Patients attending clinical immunology clinics who had progressive systemic sclerosis and related syndromes and who had received cyclosporin and/or tacrolimus were identified. Details of their treatment, including drug dosage, duration of and response to treatment, side-effects and reasons for withdrawal, were recorded. RESULTS: Sixteen patients had been given cyclosporin and 13 of these had been treated for skin tightness. Half noticed significant softening of their skin whilst on treatment, and resolution was observed in all four of the patients treated for digital vasculitis. Side-effects were common and dose-limiting, and contributed to withdrawal in 12 out of 13 patients. Eight patients had been treated with tacrolimus; two of these had stopped the drug because of progression of their disease, one developed diarrhoea, prompting withdrawal, one stopped tacrolimus following improvement, and four remained on the drug. Side-effects had occurred in three patients. CONCLUSION: Improvements in skin occur in approximately half of all cases of scleroderma treated with either cyclosporin or tacrolimus, suggesting a beneficial effect. Side-effects, especially hypertension, are common with cyclosporin and often necessitate withdrawal. Adverse effects are also observed with tacrolimus, but in the small cohort so far treated only one patient had stopped the drug for this reason.

Adult↗
PubMed1999

[Eye involvement in circumscript scleroderma--manifestation of borreliosis?].

BACKGROUND: The circumscript cutaneous sclerosis is an inflammato-edematous erythema of the skin, usually leading to a plaque-like sclerosis and cutaneous atrophy. The etiology of this disease remains unknown, but some authors presume a systemic infection with Borrelia burgdorferi as the underlying pathological condition. PATIENT: We present a case of a 66-year-old male patient who suffered from episcleritis of his left eye. Funduscopy showed an unilateral papillary edema. Visual acuity was 1.0. The patient had multiple erythemata of the skin of the body-trunk and the arms. On histological examination the lesions were compatible with circumscript sclerosis. Neurological examination was normal. Brain imaging (MRI), CSF examination and serology showed no pathological findings, although the patient presented with raised antibodies against Borrelia burgdorferi (IgG positive, IGM negative). Intravenous antibiotic treatment and topical steroids for three weeks resulted in a complete recovery of the scleritis, but had no effect on the papillary edema. CONCLUSION: Inflammatory pathological findings of the eye can be associated with circumscript sclerosis, a disease, that is normally limited to the skin. The pathological mechanism remains unclear. The presumption of an underlying borreliosis could not be confirmed in this case.

Aged↗
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↗
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↗
PubMed2004

IgG antibodies to human cytomegalovirus late protein UL94 in patients with systemic sclerosis.

Human cytomegalovirus (HCMV) has been proposed as an amplifying agent for at least some of the spectrum of systemic sclerosis (SSc; scleroderma). In support of this hypothesis, antibodies to the HCMV late protein UL94 have been detected in the majority of SSc patients in a study involving Caucasian subjects from Italy. The aim of this investigation was to determine whether elevated levels of anti-UL94 antibodies are present in African American and Caucasian SSc patients from the U.S. We further wished to determine whether there was a significant difference in the levels of anti-UL94 antibodies between the diffuse and the limited forms of the disease. IgG antibodies to a UL94 peptide were measured in 254 Caucasian and 90 African American subjects by an enzyme-linked immunosorbent assay (ELISA). In both Caucasian and African American subjects, the mean antibody level in the diffuse form of SSc was significantly higher than that in the respective control subjects (714 vs. 466 ng/ml, p = 0.005; 1226 vs. 512 ng/ml, p < 0.0001). Also, among Caucasian SSc patients, the mean antibody level in the diffuse form of SSc was significantly higher than that in the limited form of the disease (714 vs. 465 ng/ml, p = 0.02). These results show that increased levels of antibodies to the HCMV late protein UL94 are associated with SSc and they may be a marker for the severity of the disease.

Black or African American↗
PubMed2000

Watermelon stomach following and preceding systemic sclerosis.

OBJECTIVES: To report two patients with watermelon stomach associated with systemic sclerosis (SSc) and review the literature on that subject. METHODS: We describe the clinical presentation, course and outcome of our two patients and reviewed the medical literature registered in the MedLine PubMed database from 1966 to 1999 by using the key words watermelon stomach, gastric antral vascular ectasia, systemic sclerosis, scleroderma. RESULTS: The two patients presented with microcytic hypochromic anemia. Esophagogastroscopy showed multiple linear vascular malformations in the antrum compatible with watermelon stomach. They responded to Nd-Yag laser therapy with resolution of the lesions and improvement of the anemia. Patient 1 had a history of diffuse SSc while patient 2 developed limited SSc 2 years after the diagnosis of watermelon stomach. A literature review disclosed 16 documented case reports of watermelon stomach associated with SSc, 14 of whom were women. In most cases, watermelon stomach occurred in patients with established SSc but in some it antedated it by several years. In many cases, other autoimmune syndromes such as hypothyroidism, primary biliary cirrhosis, and Sjögren's syndrome were present. The presenting symptom was iron deficiency anemia, which in 11 cases was severe enough to require blood transfusions. Nine patients were successfully treated with several transendoscopic treatments, four required surgical intervention, and in three treatment was not specified. CONCLUSION: Although watermelon stomach is a rare syndrome, it is recognized as a cause of persistent bleeding in patients with SSc. Awareness of this condition may increase its recognition and treatment.

Anemia, Hypochromic↗
PubMed1983

Subclavian vein catheterization for apheresis access.

We have inserted Shiley Vas-Caths into the subclavian veins of 20 patients. These indwelling catheters are used for apheresis and some were maintained up to 3 months. Of the 430 apheresis procedures performed, 30 (6.9%) had good peripheral vein access while 400 (93.1%) required subclavian vein catheterization. No complications, including infection and blood clotting, have been recorded. The only contamination noted was normal skin flora cultured on the outside of the exposed portion of the cannula. We attribute our success to limited catheter manipulation, frequent dressing changes, and adhering to strict, sterile procedures.

Blood Component Removal↗
PubMed1996

[Clinical aspects, pathophysiology, diagnosis and therapy of gastrointestinal manifestations of progressive systemic scleroderma].

About 50% of patients with progressive systemic sclerosis develop significant clinical involvement of the alimentary tract. In decreasing order of frequency esophagus (75%), anorectum (50-70%), small bowel (50%), colon (40%) and stomach (40%) can exhibit characteristic morphological or functional features. Typical symptoms of reluxesophagitis and severe constipation are often reported. Beside this, diarrhea, steatorrhea and malnutrition are common complaints. Manometric and electrophysiological studies brought evidence of a neuropathy of the enteric nervous system in the early stages of the disease, resulting in disturbances of the digestive and interdigestive peristalsis and therefore e.g. leading to gastroparesis, bacterial overgrowth of the small intestine or constipation. In late PSS collagen deposition and atrophy of the smooth muscle layer of the bowel wall cause loss of function of sphincters as the lower esophageal sphincter or the anal sphincter and marked atony of parts of the intestine. The diagnostic procedures consist of esophageal manometry, 24-h pH-metry, esophageal and gastric radionuclide transit studies, H2-breath tests, barium enemas, anorectal manometry and endoscopy. Therapeutic options include H2-antagonists, proton-pump inhibitors, prokinetic drugs, octreotides and antibiotics. Nutritional supplementation and surgical interventions are often of limited therapeutic value. Finally in some cases long-term total parenteral nutrition is warranted.

Digestive System↗
PubMed1995

Arthritis in scleroderma.

Thirty-four patients (24 females and 10 males) selected from 300 consecutive patients with established systemic sclerosis (SSc), with a current or past history of articular symptoms, were clinically documented and further studied using thermography and bone scan to define the pattern of arthritis. Clinical evidence of synovitis was observed in 30 (88%) and joint inflammation was detected in 31 (91%) by the above-mentioned imaging techniques. A distinctive subset of 10 patients with deforming arthritis was characterized in which seven (70%) patients fulfilled criteria for both rheumatoid arthritis and SSC; three of these satisfied the criteria for diagnosis of CREST, but none met the criteria of mixed connective tissue disease. These patients, as a group, when compared with the rest showed limited skin involvement (skin score of 19 +/- 11 vs 33 +/- 14; P < 0.05) and were positive for rheumatoid factor (80 vs 13%; P < 0.05) and anticentromere antibodies (37 vs 4%; P < 0.05).

Adult↗
PubMed1999

Chronic Intestinal Pseudo-obstruction.

For many patients, nutritional support and relief of symptoms remain the primary management goal of pseudo-obstruction. Specific pharmacological agents for this disorder are, in general, lacking. Given that the efficacy of many of the individual available agents is far from excellent, several centers have turned to combination therapy. Though there is at present no evidence from controlled studies to support this strategy, it is, at the very least, theoretically attractive as these agents act through a number of separate mechanisms. The combination of a prokinetic and an emetic may prove especially useful. As the pseudo-obstruction syndromes are, individually, rare, and experience with any given prokinetic agent in these disorders limited, it is difficult to develop strict guidelines for their use in this context. It stands to reason that a response to a prokinetic agent would seem unlikely in a patient with an advanced myopathic process; anecdotal evidence suggests, however, that some patients with severe scleroderma may derive some symptomatic improvement. Where oral therapy is tolerated, cisapride would appear the best choice among available agents. When this fails, subcutaneous octreotide may be added or substituted. In the acute situation, intravenous erythromycin may alleviate gastroparesis, but probably exerts little beneficial effect beyond the pylorus; parenteral metoclopramide may be tried, but, here again, convincing evidence of efficacy is lacking. The roles of endoscopy and surgery are largely confined to facilitating nutrition and providing decompression.

Journal Article↗
PubMed2005

Raynaud's phenomenon in mixed connective tissue disease.

Raynaud's phenomenon affects most patients who have mixed connective tissue disease (MCTD) and frequently represents the initial manifestation of the disease. It is the cutaneous symptom of a systemic vasculopathy that is characterized by intimal fibrosis and blood vessel obliteration that frequently leads to visceral involvement, particularly pulmonary hypertension. An association between Raynaud's phenomenon and the characteristic autoantibody in MCTD, anti-U1-RNP (ribonucleoprotein), is found across the spectrum of rheumatic diseases, including undifferentiated connective tissue disease, scleroderma, and systemic lupus erythematosus. Capillary nailfold examination represents a valuable tool to identify patients who are at risk for MCTD. The goal in the therapy of Raynaud's phenomenon in MCTD is to decrease the frequency of attacks, to prevent digital ulceration, and to limit progressive vascular damage. Therapeutic regimens include the traditional use of calcium channel blockers and novel vascular therapies.

Autoantibodies↗
PubMed1993

Carboxyterminal type I procollagen peptide concentrations in systemic sclerosis: higher levels in early diffuse disease.

Systemic sclerosis (SSc) is characterized by increased collagen deposition in skin and internal organs, and increased serum concentrations of different connective tissue metabolites have been reported. In this study serum concentrations of carboxyterminal type I (PICP) and aminoterminal type III procollagen peptide (PIIINP) were higher in 24 patients with diffuse cutaneous systemic sclerosis (dSSc), than in 30 patients with limited cutaneous systemic sclerosis (lSSc). Thirty patients with advanced and progressing disease had higher serum concentrations of both peptides than 24 patients with milder disease. In patients with advanced disease, both peptides showed a negative correlation to disease duration. Despite being relatively higher both in early, widespread and advanced disease, serum PICP concentrations varied only within the normal range for healthy controls. Thus PICP is of limited value as a marker of disease activity in SSc.

Adult↗
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↗