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PubMed1992

Alpha interferon-2a (Roferon-A) in the treatment of diffuse cutaneous systemic sclerosis: a pilot study. UK Systemic Sclerosis Study Group.

Intramuscular alpha-interferon improved or stabilized skin score in 10/14 (71%) patients with diffuse cutaneous systemic sclerosis. In 64% of patients, their treating physicians rated it as having stabilized or improved the disease. However, it had no significant effect on grip strength, digital contractures, respiratory function or visceral involvement. Type I collagen synthesis was significantly reduced in fibroblasts cultured from clinically 'uninvolved' skin but not in those from lesional skin. Amino-terminal procollagen III peptides in the interferon treated group were not significantly reduced after 6 months of therapy but showed a trend towards stabilization and reduction compared to disease matched controls on no therapy.

Adult↗
PubMed2005

Increased plasma soluble CD40 ligand concentrations in systemic sclerosis and association with pulmonary arterial hypertension and digital ulcers.

BACKGROUND: Increased expression of CD40L has been reported on activated CD4+ T lymphocytes in systemic sclerosis. CD40L can be expressed in soluble form (sCD40L). OBJECTIVE: To compare sCD40L concentrations in patients with systemic sclerosis and healthy controls. METHODS: Quantitative sandwich ELISA was used to measure plasma sCD40L in systemic sclerosis (n = 50) and matched healthy controls (n = 20). Patients with systemic sclerosis had limited cutaneous disease (29), digital ulcers (14), pulmonary arterial hypertension (PAH) (10), pulmonary fibrosis on CT (23), positive anti-Scl70 (14), and anti-centromere antibodies (10). Calcium channel blockers were discontinued 72 hours before measurements. RESULTS: Median (range) sCD40L concentration (pg/ml) was higher in systemic sclerosis than in controls (495 (10 to 7720) v 79 (50 to 118); p = 0.003), in limited cutaneous disease v diffuse disease (620 (20 to 7720) v 250 (10 to 2690); p = 0.005), in patients with digital ulcers v those without (1430 (36 to 7720) v 370 (10 to 2320); p = 0.002), and in those with PAH v those without (995 (15 to 3850) v 400 (10 to 7720); p = 0.048). sCD40L correlated with pulmonary arterial pressure estimated by Doppler echocardiography (r = 0.41; p = 0.005). CONCLUSIONS: The soluble form of CD40L is increased in plasma in systemic sclerosis and may be associated with vascular complications of the disease.

Adult↗
PubMed1997

Malignant lymphoma in patients with rheumatic diseases other than Sjögren's syndrome: a clinicopathologic study of five cases and a review of the Japanese literature.

We conducted clinicopathologic and immunohistochemical analysis of five patients with malignant lymphoma complicating rheumatic diseases other than Sjögren's syndrome, and reviewed 26 cases of similar lesions reported in the Japanese literature over a 17-year period. All five patients were women ranging in age from 31 to 74 years (mean 55 years). Two of them fulfilled the diagnostic criteria for systemic lupus erythematosus, two for dermatomyositis and one for progressive systemic sclerosis. The use of immunosuppressive drugs before the onset of malignant lymphoma was recorded in four patients. All the biopsied or resected specimens showed non-Hodgkin's lymphoma of B-cell phenotype. Three were nodal in origin (one diffuse mixed, one diffuse large cell and one immunoblastic) and two were extranodal (one low-grade B-cell lymphoma of mucosa-associated lymphoid tissue and one diffuse large cell). In three of four cases examined, Epstein-Barr virus-encoded small RNAs were identified in a small to large number of the lymphoma cells by in situ hybridization. Our study showed that the clinicopathological features of malignant lymphomas complicating rheumatic disease in Japan were similar to those in England and the USA. Furthermore, our findings suggested no evidence for a causative association between iatrogenic immunosuppression due to methotrexate therapy and the development of EBV-related lymphoid neoplasms.

Adult↗
PubMed1991

[Esophageal manometry and pH-monitoring in surgical indications].

In the context of esophageal disease, the study of motility and reflux by means of manometry and pH-metry has proved rewarding in terms of prospects thanks to the physiopathologic information it is capable of providing and to the clinical applications which, in many cases, are of considerable importance. In the area of surgery, in our experience, indications for manometry include: the physiopathologic definition of a variety of diseases such as achalasia, diffuse esophageal spasm, hypertonic conditions responsible even for the formation of diverticula, esophageal reflux in the presence of a hypotonic L.E.S.. Further indications are intra- and post-operative, to verify the extension and the validity of the surgery. Surgical indications for pH-metry must include all those conditions in which esophageal reflux is not underscored by means of other investigative tools. However the omnipresence of a surgical indication in these cases is debatable. In any event this objective will be within reach only after an adequate refinement of investigative techniques and the optimal utilization of the findings obtained in the course of diagnostic exploration.

Adult↗
PubMed2002

Autoantibodies against small nucleolar ribonucleoprotein complexes and their clinical associations.

Sera from patients suffering from systemic autoimmune diseases such as systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) have been shown to contain reactivities to nuclear components. Autoantibodies specifically targeting nucleolar antigens are found most frequently in patients suffering from SSc or SSc overlap syndromes. We determined the prevalence and clinical significance of autoantibodies directed to nucleolar RNA-protein complexes, the so-called small nucleolar ribonucleoprotein complexes (snoRNPs). A total of 172 patient sera with antinucleolar antibodies were analysed by immunoprecipitation. From 100 of these patients clinical information was obtained by chart review. Autoantibodies directed to snoRNPs were detected not only in patients suffering from SSc and primary Raynaud's phenomenon (RP), but also in patients suffering from SLE, rheumatoid arthritis (RA) and myositis (PM/DM). Antibodies against box C/D small snoRNPs can be subdivided in antifibrillarin positive and antifibrillarin negative reactivity. Antifibrillarin-positive patient sera were associated with a poor prognosis in comparison with antifibrillarin negative (reactivity with U3 or U8 snoRNP only) patient sera. Anti-Th/To autoantibodies were associated with SSc, primary RP and SLE and were found predominantly in patients suffering from decreased co-diffusion and oesophagus motility and xerophthalmia. For the first time autoantibodies that recognize box H/ACA snoRNPs are described, identifying this class of snoRNPs as a novel autoantigenic activity. Taken together, our data show that antinucleolar patient sera directed to small nucleolar ribonucleoprotein complexes are found frequently in other diseases than SSc and that categorization of diagnoses and clinical manifestations based on autoantibody profiles seems particularly informative in patient sera recognizing box C/D snoRNPs.

Autoantibodies↗
PubMed1997

Circulating intercellular adhesion molecule-1 in the sera of patients with systemic sclerosis: enhancement by inflammatory cytokines.

We measured serum levels of circulating intercellular adhesion molecule-1 (cICAM-1) in patients with systemic sclerosis (SSc) and normal controls. The levels of cICAM-1 were determined by sandwich enzyme-linked immunosorbent assay in sera from 88 patients with SSc and in 20 healthy controls. In addition, these levels were examined in the supernatants of cultured peripheral blood mononuclear cells (PBMC) and dermal fibroblasts from 10 patients with SSc and 10 healthy control subjects. Serum levels of cICAM-1 were significantly higher in patients with SSc than in healthy controls. Serum cICAM-1 levels were significantly higher in patients with diffuse cutaneous SSc (dcSSc) than in patients with limited cutaneous SSc (lcSSc). These serum levels were correlated with the presence of contracture of phalanges, pulmonary fibrosis, joint involvement and increased erythrocyte sedimentation rate. The release of cICAM-1 was significantly increased in the supernatants of cultured PBMC from patients with SSc. Moreover, inflammatory cytokines (interferon-gamma, interleukin-1 and tumor necrosis factor-alpha) enhanced the release of cICAM-1 in vitro in SSc cells. These findings suggest that cICAM-1 may be involved in immune reactions in this disease.

Cells, Cultured↗
PubMed2006

Lack of association between three vascular endothelial growth factor gene polymorphisms and systemic sclerosis: results from a multicenter EUSTAR study of European Caucasian patients.

INTRODUCTION: Systemic sclerosis (SSc) is characterised by disturbed vessel morphology and an overproduction of vascular endothelial growth factor (VEGF). The VEGF gene located on chromosome 6p21.3 has several polymorphisms. OBJECTIVE: To test the hypothesis that disturbed angiogenesis may be related to the genetic background of the VEGF gene. MATERIALS AND METHODS: EUSTAR centres included European Caucasian patients with SSc and matched controls with osteoarthritis. The VEGF gene was genotyped by polymerase chain reaction, followed by restriction enzyme analysis. The 634 C/T and 936 C/G mutations and an 18-base pair insertion/deletion at -2549 of the VEGF promoter region were tested. RESULTS: 416 patients with SSc and 249 controls were included in the study population. Of the patients with SSc, 42% had a diffuse cutaneous subtype, 16% had increased pulmonary arterial pressure and 61% had decreased carbon monoxide diffusion capacity. The genotype frequencies in the patients with SSc and in controls were in Hardy-Weinberg equilibrium. The allele and genotype frequencies of the polymorphisms did not differ between patients with SSc and controls. No association was found between these polymorphisms and disease phenotypes. CONCLUSION: This study shows that there is no association between the three selected functional VEGF polymorphisms and SSc.

Adult↗
PubMed2002

Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings.

Collagen vascular diseases that demonstrate features of interstitial lung disease include systemic lupus erythematosus, rheumatoid arthritis, progressive systemic sclerosis, dermatomyositis and polymyositis, ankylosing spondylitis, Sjögren syndrome, and mixed connective tissue disease. At histopathologic analysis, interstitial lung diseases associated with collagen vascular diseases are diverse and include nonspecific interstitial pneumonia, usual interstitial pneumonia, bronchiolitis obliterans organizing pneumonia (BOOP), apical fibrosis, diffuse alveolar damage, and lymphocytic interstitial pneumonia. Although proportions of interstitial pneumonias vary, nonspecific interstitial pneumonia accounts for a large proportion, especially in progressive systemic sclerosis, dermatomyositis and polymyositis, and mixed connective tissue disease. The more favorable prognosis in interstitial pneumonia associated with collagen vascular diseases than in idiopathic interstitial pneumonias may be explained by the larger proportion of nonspecific interstitial pneumonia than of usual interstitial pneumonia. High-resolution computed tomography seems to help characterize and determine the extent of interstitial lung disease in collagen vascular diseases.

Arthritis, Rheumatoid↗
PubMed1996

The relationship of thoracic lymphadenopathy to pulmonary interstitial disease in diffuse and limited systemic sclerosis: CT findings.

OBJECTIVE: Mediastinal lymphadenopathy is frequently associated with interstitial lung disease (ILD) in patients with systemic sclerosis (SSc). This study compared the extent of lymphadenopathy in diffuse and limited SSc with the presence and type of ILD found on CT scans. SUBJECTS AND METHODS: Seventy-three patients with diffuse and limited SSc underwent thoracic CT. The presence of lymphadenopathy was correlated with the frequency and type of ILD. RESULTS: Fifty-eight percent (18/31) of patients with diffuse cutaneous SSc and 40% (17/42) of patients with limited cutaneous SSc had lymphadenopathy. A significant relationship was found between lymphadenopathy and the presence of interstitial disease (p = .0002). Seventy-two percent (18/25) of patients with a ground-glass parenchymal pattern of interstitial disease and 74% (14/19) of patients with a honeycomb pattern had lymphadenopathy. CONCLUSION: Lymphadenopathy is prevalent in patients with SSc and ILD regardless of clinical subtype or interstitial pattern. Lymphadenopathy appears to increase as a function of the profusion rather than the morphology of ILD.

Female↗
PubMed2003

[Diffuse interstitial lung disorders in systemic diseases].

Diffuse parenchymal lung disorders (DPLD) can develop in a variety of systemic disorders. Schematically grouped, these include connective tissue disorders, vasculitis, neoplastic disorders, sarcoidosis and a group of inherited or other rare miscellaneous disorders. This overview focuses on sarcoidosis, systemic sclerosis and Churg Strauss vasculitis. Pulmonary involvement occurs in more than 90% of all patients with sarcodosis. Grading into 4 stages is based on the chest radiograph. Forms characterised by an acute clinical onset or a low grade lung involvement have the highest spontaneous remission rate. The cause of sarcoidosis remains unknown. The diagnosis therefore is descriptive, based on the combination of clinical observations, chest X ray, and the histological documentation of non-caseating epitheloid granulomas in tissue biopsies. Treatment with steroids is only indicated if organ involvement leads to functional impairment. Lung fibrosis is the most important complication of both the "limited" and "diffuse cutaneous form" of systemic sclerosis, involving 90% of all patients. The histological pattern is that of "Usual Interstitial Pneumonia" (UIP) or "Non-specific Interstitial Pneumonia" (NSIP). The pathogenesis of the disorder is thought to consist of an abnormal, excessive regenerative response to an auto-immune mediated lung injury. Churg Strauss vasculitis is characterised by asthma, blood eosinophilia and vasculitis of the small vessels. The affected vessels wall shows signs of fibrinoid necrosis and are infiltrated by eosinophils. pANCA (anti-myeloperoxidase) is considered to play a role in the pathogenesis of the disease. Concern has risen that CysLT1 receptor antagonists might induce production of pANCA. To date, this has not been substantiated.

Churg-Strauss Syndrome↗
PubMedDate not supplied

Clinical evaluation of systemic sclerosis: a comprehensive panel of diagnostic tests to assess skin, microvascular and visceral involvement. The Genoese Group for the Study of Systemic Sclerosis.

The work proposes a set of diagnostic tests and relative clinical scores for the staging and follow-up of patients with systemic sclerosis. Thirty-one consecutive patients (30 females, 1 male, age range 17-65 years) affected by systemic sclerosis, according to the American College of Rheumatology criteria for classification of this disease, were enrolled in the study at the time of their first admission to the Department of Internal Medicine in Genoa. The following battery of tests was utilized: nailfold capillary videomicroscopy, esophageal manometry and pH-metry, lung vital capacity and diffusing capacity for carbon monoxide, intrarenal duplex Doppler sonography, echocardiography and skin plicometry. Good compliance by the patients was obtained for the tests we used. We found that nailfold capillary videomicroscopy and plicometry scores were statistically associated each other as well as with the scores measuring esophagus and/or lung function. The use of wide range of diagnostic procedures, such as those proposed in this paper, allows appropriate definition of the stage of patients with systemic sclerosis. The use of nailfold capillaroscopy and skin plicometry as the simplest tests for monitoring patients is suggested: the scores derived from these procedures are statistically associated with some internal organ status and could be useful in the evaluation of therapeutical results.

Adolescent↗
PubMed1993

[A case of progressive systemic sclerosis with Sjögren's syndrome presenting with coma, convulsion and bilateral thalamic hypodensity on computed tomography].

A 35-year-old female with progressive systemic sclerosis (PSS) and Sjögren's syndrome developed consciousness disturbance and generalized seizure after the episode of fever and erythema lasting for 3 weeks. Neurological examination disclosed deep coma and spastic tetraplegia with pathological reflexes. Laboratory data showed mild anemia, severe hypoproteinemia, hypoalbuminemia and increase of protein content in the cerebrospinal fluid. Cranial CT scans obtained after convulsion revealed diffuse brain swelling and bilateral symmetrical hypodensity involving the thalami and posterior limbs of the internal capsule. High-dose corticosteroid therapy and osmotherapy with correction of hypovolemia were started, because severe dehydration, hypoalbuminemia and cerebral vasculitis were suspected to change the vascular permeabilities. Neurological symptoms and CT findings were rapidly improved. Cerebral angiography 4 weeks after convulsion showed definite angitis. The patient was discharged 9 weeks after convulsion. In our case, a possible cause of the characteristic CT findings may be the disturbance of cerebral venous return due to hypovolemia and vasculitis. High-dose corticosteroid therapy may be recommended in patients with collagen disease, who show the bilateral symmetrical thalamic hypodensity on CT scans.

Adult↗
PubMed2002

Presence of microchimerism in labial salivary glands in systemic sclerosis but not in Sjögren's syndrome.

OBJECTIVE: To determine whether microchimerism can be implicated in Sjögren's syndrome (SS) by studying minor salivary glands, one of the targets of the disease. METHODS: Labial salivary gland (LSG) biopsy specimens from 16 female patients with primary SS and 11 with systemic sclerosis (SSc) (a disease in which microchimerism is frequently detected) were analyzed. All 27 women had a history of pregnancy with a male baby. Specimens were microdissected, and polymerase chain reaction (PCR) was performed using the unique sex-determining region Y gene probe. RESULTS: The sensitivity of PCR for detecting male cells in LSG was high; the presence of 3 male cells was consistently detected in DNA extracted from a normal female LSG specimen to which male DNA had been added, and 1 male cell was detected in 50% of specimens analyzed. Male DNA was not found in any of the specimens from the 16 SS patients but was detected in 5 (45%) of 11 SSc specimens (P = 0.006). No differences in the rate of detection were found between patients with diffuse and limited SSc (male DNA detected in 2 of 3 and 3 of 8, respectively; P = 0.55) or between patients with and those without secondary SS (1 of 6 and 4 of 5, respectively; P = 0.08). CONCLUSION: The results of our study strengthen the possibility that microchimerism is implicated in SSc. This is the first study to demonstrate the presence of chimeric cells in LSG from 45% of SSc patients, independent of the presence of secondary SS. However, microchimerism was not detected in LSG from patients with primary SS, suggesting that the pathogenesis of the 2 diseases is different.

Adult↗
PubMed2005

Epidemiology of systemic sclerosis in northwest Greece 1981 to 2002.

OBJECTIVES: To investigate the incidence and prevalence, as well as the mortality and survival rates, of systemic sclerosis (SSc) in a defined area of northwest Greece with a population of about 500,000 inhabitants. MATERIALS AND METHODS: Cases have been recorded from the following sources: (1) inpatients and outpatients referred to the Rheumatology Clinics of the Ioannina University Hospital and the Ioannina General Hospital; (2) patients referred to the private rheumatologists practicing in the study area. All patients recorded between 1/1/1981 and 31/12/2002, resident in the study area, were included in the study. Diagnosis was based on the American College of Rheumatology classification criteria for SSc. Incidence and prevalence rates were calculated as number of cases per 10(5) inhabitants. Population data were based on the National Census of 1981, 1991, and 2001. RESULTS: The age-adjusted prevalence of SSc was 15.40 cases/10(5) inhabitants on 31/12/2002. A total of 109 new cases were diagnosed during the study period, giving a mean annual age-adjusted incidence rate of 1.10 cases/10 5 inhabitants. There were 98 women and 11 men, giving a ratio of 8.9/1. Limited SSc was diagnosed in 75% and diffuse in 25% of the patients. Esophageal involvement was found in 59%, lung involvement in 56%, and renal disease in 5%. Thirty-six deaths were recorded during the study period in this incidence cohort. The 5-year survival rate was 83% and the 10-year survival rate was 70%. CONCLUSIONS: The incidence and prevalence of SSc in northwest Greece were found to be lower than those of the USA and Australia, and higher than those of northern European countries and Japan. The survival rates were similar to those reported by other studies.

Adolescent↗
PubMed2001

Ankle brachial pressure index in systemic sclerosis: influence of disease subtype and anticentromere antibody.

OBJECTIVE: To test the hypothesis that patients with limited cutaneous systemic sclerosis (SSc) have a higher incidence of lower limb large vessel disease than patients with diffuse cutaneous disease, and that anticentromere antibody is a risk factor for lower limb large vessel disease. METHODS: Clinical and laboratory data from 119 patients with SSc (91 patients with limited cutaneous disease, 28 patients with diffuse cutaneous disease) who had bilateral ankle brachial pressure indices (ABPI) measured between March 1997 and January 2000 were reviewed retrospectively. RESULTS: There was no evidence of reduced ABPI in limited cutaneous disease (P=0.65), average reduction 0.01 [95% confidence interval (CI) -0.04 to +0.07]. There was some suggestion of reduced ABPI in anticentromere-positive patients (P=0.12), average reduction 0.04 (95% CI -0.01 to +0.09). CONCLUSIONS: The severity of large vessel macrovascular disease, as assessed by ABPI, is not dependent on disease subtype. Anticentromere antibody may be weakly associated with a reduction in ABPI.

Adult↗
PubMed2006

Malignant lymphoma in patients with systemic rheumatic disease (rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, and dermatomyositis): a clinicopathologic study of 24 Japanese cases.

We conducted clinicopathologic and immunohistochemical analyses of the prevalence of Epstein-Barr virus (EBV) among 24 patients with malignant lymphoma complicating systemic rheumatic diseases. (SRD) These 24 patients included 17 with rheumatoid arthritis (RA), 3 with systemic lupus erythematosus (SLE), 2 with systemic sclerosis (SS), and 2 with dermatomyositis (DM). There were 2 men and 22 women ranging in age from 30 to 86 years (mean: 64 years). The interval between the onset of rheumatic disease and that of malignant lymphomas ranged from 3 months to 35 years (mean: 142 months). The use of immunosuppressive drugs before the onset of malignant lymphoma was recorded in 15 patients. Among them, 5 patients received methotrexate (MTX) therapy. Malignant lymphomas were found at extranodal sites in 9 patients, and the disease was in the advanced stage in 17 patients. Histologic and immunohistochemical studies demonstrated that 18 cases (75%) were B-cell lymphoma (RA=12, SLE=2, SS=2, DM=2), 3 (12.5%) were peripheral T-cell lymphoma (RA=3), and 3 (12.5%) were classical Hodgkin lymphoma (RA=2, SLE=1). As in previous reports, there was an increased frequency of diffuse large B-cell lymphoma (50%) in the present series. Moreover, a majority of the diffuse large B-cell lymphomas exhibited activated B-cell phenotype. EBV-encoded small RNAs (Epstein-Barr early region [EBER]-) and/or LMP-1+tumor cells were identified in only 3 cases of classical Hodgkin lymphomas. Our findings suggested EBV-associated lymphoma comprised only a small fraction of all non-Hodgkin's lymphomas in the general SRD patient population.

Adult↗
PubMed1991

Pulmonary functions in children with progressive systemic sclerosis.

The patterns of pulmonary involvement in 13 children with progressive systemic sclerosis were investigated. Eight patients (61%) had respiratory symptoms or signs and 7 patients (55%) had abnormalities on chest roentgenogram. Twelve patients (92%) had abnormal pulmonary function tests: 7 had restrictive disease, 2 had obstructive disease, 2 had small airway disease, and 1 had an isolated reduction in the diffusing capacity of carbon monoxide. Nine patients had the test performed during the first year of illness, 3 during the second year, and 1 at 5 years. All patients had abnormal pulmonary function tests when first studied. Subsequent pulmonary function tests over a period of 3 to 10 years (mean 6.2 years) showed substantial changes in only 2 patients (1 patient had initial worsening of diffusing capacity of carbon monoxide followed by normalization and another patient showed improvement of obstructive disease). Two patients died during follow-up, 1 of pulmonary hypertension, the other of severe restrictive lung disease and myocardial fibrosis. The major findings of this study were (1) high frequency of pulmonary disease in children with progressive systemic sclerosis, (2) early involvement of the lungs, (3) relatively indolent progression of lung disease, and (4) the prognostic importance of the severity of pulmonary disease. Pulmonary manifestations of progressive systemic sclerosis in children appear to be similar to those of affected adults.

Adolescent↗
PubMed2005

Digital ulcers in patients with systemic sclerosis.

Digital ulcers (DU), defined as necrotic lesions located at distal digits or overlying bony prominences, occur in up to 50% of patients with limited or diffuse systemic sclerosis (SSc). These lesions are extremely painful and lead to substantial functional disability. The pathogenesis of DU differs depending on their location. DU located at distal aspects of digits are thought to be related to tissue ischemia from several processes, including vasospasm secondary to Raynaud's phenomenon, intimal fibro-proliferation, and thrombosis of digital arteries. DU located over bony prominences, such as the phalangeal joints and elbows, are thought to be due to repetitive microtrauma and difficulty healing due to atrophic, avascular tissue overlying the joints. Management of DU include non-pharmacologic and pharmacologic modalities. This review summarizes the current available and investigational therapies for the treatment and prevention of DU in patients with SSc.

Anticoagulants↗