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PubMed1996

[Raynaud phenomenon and scleroderma].

The Raynaud phenomenon occurs in 90-100% of patients with systemic scleroderma. It is caused by dysregulation of the vascular tone between vasoconstrictive and vasodilatory influences. Early damage to the vascular endothelium and the activation of platelets lead to the release of vasoactive substances. Structural and function impairment of the perivascular neural fibers contributes to the vasospasm. New vasodilatory treatment strategies, e.g. infusions with calcitonin gene-related peptides, could be useful in the control of vasospasm in the peripheral extremities as well as internal organs in systemic scleroderma.

Calcitonin Gene-Related Peptide↗
PubMed1981

Characterization of a spontaneous disease of white leghorn chickens resembling progressive systemic sclerosis (scleroderma).

University of California, Davis (UCD) line 200 White Leghorn Chickens spontaneously develop a syndrome that has many analogous features to human progressive systemic sclerosis. This syndrome is characterized by progressive involution of comb, dermal fibrosis, and distal polyarthritis. These three features occur within 6 wk after hatching, and are accompanied by a 40% mortality as a result of vaso-occlusive disease, with development of secondary infection of peripheral gangrenous lesions. Birds that survive greater than 2 mo after hatching progressively develop fibrosis of the esophagous and mononuclear infiltration of heart and kidney, with prominent occlusion of small and medium sized blood vessels. In addition, line 200 chickens develop rheumatoid factors, antinuclear antibodies, and antibodies to collagen, but do not have antibodies to thymocytes, DNA, or extractable nuclear antigens. Moreover, antinuclear antibodies when studied using HEp-2 cells as substrate demonstrate predominantly a speckled pattern. This syndrome of line 200 chickens is not detectable in F1 crosses to several UCD inbred lines. F1 X parental line BC1 backcrosses have an approximately 50% incidence of disease, suggesting that this syndrome is inherited as autosomal recessive. However, only 4% of F2 generation birds show abnormal symptoms, suggesting the presence of modifying genes. There is no appearance of IgG deposition, as determined by immunofluorescence, in either skin, blood vessels, esophagus, or heart. However, approximately 20% of chickens have a glomerulonephritis; this feature appears to be a terminal event and does not appear clinically significant. Although this syndrome of line 200 chickens has several features that are in sharp distinction to human scleroderma, the presence of common immunologic and pathologic denominators suggest that this spontaneous disease may be an appropriate model to develop a better understanding of autoimmune connective tissue diseases.

Age Factors↗
PubMed1976

Scleroderma (progressive systemic sclerosis) with severe bowel involvement. Treatment by extensive resection of the small intestine.

A 48-year-old man with previously diagnosed scleroderma with bowel involvement was admitted to hospital with severe malnutrition attributed to malabsorption. Shortly after this, he developed features of intestinal obstruction followed by paralytic ileus. Due to failure to respond to medical treatment, operation was carried out. On two occasions adhesions were divided, but the bowel failed to function. At a third operation the proximal half of the small bowel below the duodeno-jejunal flexure was excised. Following this the patient made a good recovery. Small bowel involvement in scleroderma is discussed. Malabsorption is probably related to bacterial proliferaiton in the small bowel secondary to stasis and may be helped by antibiotic drugs. Other disturbances resulting in inability of the bowel to propel its contents may comprise syndromes of obstruction and paralytic ileus. Although management of scleroderma bowel involvement is usually medical, surgical treatment may be indicated under certain circumstances. It may be life-saving.

Humans↗
PubMed2003

[Cyclophosphamide pulse therapy for pediatric systemic sclerosis].

We encountered three patients with pediatric systemic sclerosis. Patient 1 had systemic scleroderma, pigmentation and interstitial pneumonia at the age of 10 years. Nine months after disease onset, she was treated with intravenous cyclophosphamide pulse therapy as induction therapy. After the initial treatment, the following clinical manifestations were dramatically improved: interstitial pneumonia, scleroderma and total skin score. Patient 2 was a 7-year-old girl, who complained of systemic scleroderma and pigmentation, and was found to have pulmonary hypertension. Six months after disease onset, she was administrated intravenous cyclophosphamide pulse therapy. Her scleroderma and total skin score were improved and the pulmonary hypertension did not deteriorate. Patient 3 was a 15-year-old girl. Her initial treatment was vitamin E alone. She was admitted to our hospital two and half years after disease onset. Although she was given immunosuppressive therapy including cyclophosphamide, the severe condition of persisted, and she died after five months. It became possible for patient 1 and 2 to achieve and maintain a marked improvement of the clinical manifestations as a result of cyclophosphamide pulse therapy early in the course of the disease. We further observed that their total skin score was decreasing while the clinical manifestations improved.

Adolescent↗
PubMed1990

Porphyria cutanea tarda and scleroderma--chance association or related disease: a case report.

The concurrence of systemic scleroderma and porphyria cutanea tarda (PCT) seems very rare. Only six cases have been reported to our knowledge. On the other hand, cutaneous sclerodermatous lesions in PCT occur more frequently and can affect 1.8-18% of cases. These observations raise the problem of a possible physiopathological relation between scleroderma and PCT. In fact, Grossman and colleagues have found anti-nuclear antibodies (ANA) in 38% of patients so tested. In the present report, the association of systemic scleroderma and PCT with Sjögren's syndrome and anti-RNP antibodies raises further questions concerning the relationship between PCT and auto-immune diseases.

Aged↗
PubMed1984

A controlled trial of antihypertensive therapy in systemic sclerosis (scleroderma).

Antihypertensive treatment may be life saving in scleroderma renal crisis. Patients surviving such crises frequently have had dramatic improvement in the dermal manifestations of their scleroderma. To investigate the potential role of antihypertensive treatment in nonhypertensive patients we randomly assigned 28 patients with systemic sclerosis into drug (14) and placebo (14) groups, using blocked randomisation , and followed them up in a prospective, double-blind clinical trial for 24 months. Overall, both groups improved slightly, with both subjective and objective markers. There were no statistically significant differences and no clinically meaningful trends between the 2 groups, except that the blood pressure was reduced in the group on the active drug.

Blood Pressure↗
PubMed1992

Serum adenosine deaminase activity in systemic sclerosis (scleroderma) and related disorders.

BACKGROUND: Adenosine deaminase (ADA) activity in serum is mainly derived from T lymphocytes. OBJECTIVE: Our purpose was to clarify the significance of ADA activity in systemic sclerosis (PSS) and related disorders. METHODS: ADA activity was determined with an enzymatic method in 34 patients with PSS, 4 with mixed connective tissue disease (MCTD), 6 with dermatomyositis (DM), 11 with localized scleroderma (LS), and 13 with other collagen diseases. RESULTS: Serum ADA activity was elevated over the mean (+2 standard deviations) of the control in 85% of the patients with PSS, all with MCTD, 83% of those with DM, and 82% of those with LS. The mean values in 10 PSS patients with anti-topoisomerase I antibodies, 14 patients with anti-RNP antibodies, 12 patients with anticentromere antibodies (ACAs), and 5 patients without antinuclear antibodies (ANAs) were 26.1, 24.9, 22.6, and 16.8 IU/L, respectively. In most cases, except for ACA-positive patients, serum ADA activity changed almost in parallel with ANA titers. CONCLUSION: These results support the notion that T cells are involved in the pathogenesis of PSS and related disorders.

Adenosine Deaminase↗
PubMed1996

Autoantibodies to fibrillarin in systemic sclerosis (scleroderma). An immunogenetic, serologic, and clinical analysis.

OBJECTIVE: To determine the frequency, clinical associations, and any major histocompatibility complex correlations of antifibrillarin antibodies in patients with systemic sclerosis (SSc). METHODS: Antifibrillarin antibodies were determined by indirect immunofluorescence, immunoblotting, and immunoprecipitation, and HLA class II alleles by DNA oligotyping, in a large cohort of SSc patients. RESULTS: Antifibrillarin was found in 8% of 335 SSc sera and was significantly more common in blacks (16%) than whites (5%), in males (33%) than females (14%), and in patients with cardiac, renal, or gut involvement. The HLA class II haplotype DRB1*1302, DQB1*0604 was found significantly more frequently in SSc patients with antifibrillarin compared with race-matched normal controls and 260 SSc patients without antifibrillarin. In addition, 1 or more of the HLA-DQB1 alleles *0604, *0301, *0602, and/or *0302 was found in all antifibrillarin-positive patients, and 62% of the antifibrillarin-positive patients had 2 of these HLA-DQB1 alleles, a highly significant difference from both race-matched normal controls and antifibrillarin-negative SSc patients. CONCLUSION: Antifibrillarin, although an infrequent nucleolar autoantibody, is a marker for severe SSc, especially in blacks and males, and is strongly associated with a unique HLA haplotype, as well as with combinations of certain HLA-DQB1 alleles.

Alleles↗
PubMed2001

Abnormalities in fibrillin 1-containing microfibrils in dermal fibroblast cultures from patients with systemic sclerosis (scleroderma).

OBJECTIVE: To determine if there are abnormalities in fibrillin 1-containing microfibrils in the extracellular matrix (ECM) of primary dermal fibroblasts explanted from patients with systemic sclerosis (SSc). METHODS: Explanted fibroblasts from unaffected skin of 12 SSc patients were used to examine fibrillin 1-containing microfibrils by immunofluorescence (IF) using a monoclonal antibody (mAb) to fibrillin 1. Metabolic labeling of the fibroblast cultures was used to study the synthesis, secretion, and processing of fibrillin 1, as well as to observe microfibril formation and stability. Microfibrils elaborated by the SSc cells were analyzed by electron microscopy for ultrastructural abnormalities, and the results were confirmed by immunoblotting. RESULTS: Control and SSc fibroblasts displayed a prominent meshwork of fibrillin 1-containing microfibrils when visualized by IF using a fibrillin 1 mAb. Paradoxically, metabolic studies indicated a paucity of fibrillin 1 in the ECM in the majority of the SSc fibroblast strains. Subsequent rotary-shadowed electron microscopy revealed reduced amounts of and ultrastructural abnormalities in the microfibrils elaborated by all strains of SSc cells. Immunoblots confirmed the lack of the high molecular weight form of fibrillin 1 in the SSc fibroblasts of Choctaw American Indians. Finally, in vitro studies indicated that the amount of fibrillin 1 in the ECM of SSc cells diminished at a faster rate than the amount of fibrillin 1 in the ECM of control cells with time. CONCLUSION: Although SSc fibroblasts assemble microfibrils, these microfibrils are unstable, suggesting that an inherent defect of fibrillin 1-containing microfibrils may play a role in the pathogenesis of SSc.

Adult↗
PubMed2002

Sleep disruption in systemic sclerosis (scleroderma) patients: clinical and polysomnographic findings.

BACKGROUND: Progressive systemic sclerosis (SSc) is a connective tissue disease characterized by endothelial lesions and fibrosis of the skin and other organs. Patients' quality of life and life expectancy are determined by the intensity of pulmonary, esophageal, cardiac, and renal involvement. Neurological involvement was considered to be rare, but increasing evidence indicates that peripheral and autonomic neuropathies commonly occur. Conditions seen with SSc, such as pulmonary fibrosis and gastro-esophageal reflux, have the potential to affect sleep. No systematic assessment of sleep-related problems associated with this disorder has been performed. MATERIAL AND METHODS: An all-night polysomnogram and a clinical interview blinded to sleep status were obtained for 27 consecutive SSc patients. Sleep data were compared to age adjusted published normative values. Correlations were determined for sleep and clinical data. RESULTS: Patients with SSc had a reduced sleep efficiency (SE) (mean+/-SD: 82+/-12.3%) and rapid eye movement sleep (13.1+/-5.6%) and increased arousal index (26.1+/-13.0) and slow wave sleep (25.7+/-9.7%). The periodic leg movement index (PLMI) exceeded 5/h in 13 patients (48%) and 25/h in seven patients. Six patients had restless legs syndrome (RLS). Significant disordered breathing was absent. The RLS patients showed a greater arousal index than all other patients. PLMI was correlated with SE for RLS but not for non-RLS patients. Esophageal dyskinesia, dyspnea, and RLS were significantly associated with poor sleep. CONCLUSION: Patients with SSc have significant disturbance of their sleep. Esophageal dyskinesia and dyspnea, which are common complications of SSc, were commonly associated with indices of sleep disruption. RLS but not sleep apnea appears to have an increased prevalence in SSc.

Journal Article↗
PubMed1977

Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma).

Severe pulmonary hypertension without pulmonary fibrosis occurred in 10 patients with the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia), reputedly a benign variant of progressive systemic sclerosis. Time from the initial symptom, Raynaud's phenomenon, to the recognition of pulmonary hypertension was as long as 40 years. Pulmonary hypertension and increased pulmonary vascular resistance was shown in all patients. Autopsy examination in three of six deaths attributable to pulmonary hypertension showed intimal proliferation with myxomatous change in the small- and medium-sized pulmonary arteries similar to changes in the digital arteries of patients with scleroderma and Raynaud's phenomenon, and interlobular renal arteries of those with "scleroderma kidney." It is concluded that the CREST syndrome is not entirely benign but may be complicated, after a long clinical course, by progressive pulmonary vascular obliteration, pulmonary hypertension, and death in the absence of significant pulmonary fibrosis.

Adult↗
PubMed1981

Primary heart disease in systemic sclerosis (scleroderma): advances in clinical and pathologic features, pathogenesis, and new therapeutic approaches.

Heart disease in SSCL may be primary or secondary. Primary involvement includes pericarditis, myocardial fibrosis, and contraction band necrosis with congestive cardiomyopathy, conduction system fibrosis, intramural coronary artery lesions and, rarely, valvular disease. Symptoms include those of left and right ventricular failure, chest pain, palpitations, syncope, and sudden death. Pathogenesis may be related to functional Raynaud's phenomenon of the heart and/or structural small vessel disease. Therapy at present is symptomatic; however, new therapeutic approaches are warranted.

Coronary Vessels↗