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PubMed1976

Pseudoobstruction in ceroidosis.

Diffuse deposition of ceroid pigment in the muscularis propria of the gastrointestinal tract in a patient with a long history of malabsorption of unknown origin is reported. The depostion of this waste pigment is not reversible and is related to prolonged depletion of vitamin E. Progressive dilatation and hypomotility of the entire gastrointestinal tract are demonstrated by radiographic studies and possibly related to infiltrate of ceroid pigment in the smooth muscle cell with resulting functional impairment. In the differential diagnosis of ceroidosis with other disease, scleroderma has the closest roentgenographic similarity. Pseudoobstruction of the small bowel which can develop must be treated conservatively to avoid unnecessary bowel resection.

Adult↗
PubMed1994

Association of autoantibodies to topoisomerase I and the phosphorylated (IIO) form of RNA polymerase II in Japanese scleroderma patients.

Autoantibodies to RNA polymerases (RNAP) I and III are highly specific for scleroderma (SSc), whereas autoantibodies to RNAP II are associated with systemic lupus erythematosus (SLE) and overlap syndromes, as well as SSc. The specificities of autoantibodies to RNAP I, II, and III in 129 SSc sera were investigated in the present study. Immunoprecipitation and pulse-chase analysis demonstrated several patterns of autoantibody recognition of RNAPs. Some sera immunoprecipitated RNAP II only after its largest subunit was phosphorylated, suggesting that they contained autoantibodies that recognized an epitope carrying a phosphoamino acid. Autoantibody recognition of all three classes of RNAPs was influenced strongly by race. Although in SLE, autoantibodies to the phosphorylated form of RNAP II (RNAP IIO) were identified in all races, in SSc, these autoantibodies were seen in 21% of Japanese and 5% of Black patients, but never in Caucasians. A striking association of anti-RNAP IIO with anti-topoisomerase I (topo I) autoantibodies was found in Japanese and Black SSc, but not SLE, patients. However, anti-topo I Abs were not associated with anti-RNAP IIO in Caucasians. Japanese SSc patients who were positive for both anti-RNAP IIO and anti-topo I Abs had a significantly higher frequency of diffuse disease, pigmentation changes, flexion contractures, and acro-osteolysis than patients having autoantibodies to topo I alone, and were diagnosed at a younger age (p < 0.05). These data suggest that genetic factors (possibly HLA-linked) influence autoantibody specificity, and that different autoantibody fine specificities may either cause, or be predictive of, different clinical outcomes.

Adult↗
PubMed2005

Early impairment of myocardial function in systemic sclerosis: non-invasive assessment by Doppler myocardial and strain rate imaging.

BACKGROUND: Aim of the present study was to analyze both left (LV) and right ventricular (RV) myocardial function in patients with Systemic Sclerosis (SSc), and their relation to other instrumental features of the disease. METHODS AND RESULTS: Twenty-five healthy subjects and 23 age- and sex-comparable asymptomatic patients classified as having either diffuse (11 patients) or limited form (12 patients) of SSc underwent clinical examination, serological tests, high-resolution chest-CT, standard Doppler echo, pulsed Doppler myocardial imaging (DMI) and strain rate imaging (SRI) of both LV and RV lateral walls. By chest-CT, 11 patients showed interstitial pulmonary fibrosis. Serological antibodies analysis detected anti-centromere pattern in 8 patients, and anti Scl-70 in 15 patients. LV diameters and ejection fraction were comparable between the two groups, while RV end-diastolic diameter was increased in SSc (p<0.01). Tricuspid inflow E/A ratio was slightly decreased in SSc (p<0.01), while systolic pulmonary pressure was increased (p<0.001). Pulsed DMI detected in SSc impaired myocardial RV early-diastolic (Em) peak velocity (p<0.0001), and prolonged myocardial time intervals at tricuspid annulus level. In SSc, peak systolic RV SR and strain were both reduced in basal, middle and apical RV lateral free walls, and in basal and middle LV lateral walls. By multivariate analysis, independent inverse association of RV peak Em velocity with both Rodnan Skin Score (p<0.0005) and pulmonary systolic pressure (p<0.0001), as well as independent inverse correlation of the same RV peak Em velocity with pulmonary fibrosis (<0.0005) in SSc patients were observed. In addition, RV Em was an independent predictor of the anti Scl-70 antibody pattern (p<0.001). CONCLUSIONS: Pulsed DMI and SRI are valuable non-invasive and easy-repeatable tools for detecting RV and LV myocardial involvement caused by SSc, and may therefore be useful to early identify patients with more diffused and severe form of SSc.

Autoantibodies↗
PubMed1999

Quantitative analysis of nailfold capillary abnormalities in patients with connective tissue diseases.

BACKGROUND: Nailfold capillary microscopy has been shown to reflect microvascular disturbances mainly in connective tissue diseases including systemic sclerosis (SSc). METHODS: Nailfold capillary abnormalities were analyzed with a light microscope under immersion oil at magnifications of x60 and x400. RESULTS: Abnormal nailfold capillary pattern in SSc was different from that of systemic lupus erythematosus (SLE) and normal controls, but not from dermatomyositis (DM). Seventy-two per cent of patients with Raynaud's phenomenon showed an abnormal nailfold capillary pattern. In primary Raynaud's phenomenon, 12% of subjects developed undifferentiated connective tissue disease. In undifferentiated connective tissue disease, 23% developed SSc. The apical limb width, capillary width and capillary length of subjects who developed SSc were significantly larger than in those who did not. With regard to the clinicolaboratory findings, the occurrence rate of an abnormal apical limb width, abnormal capillary width, abnormal capillary length, and antinuclear antibody in patients who developed SSc was significantly higher than in those who did not. An abnormal capillary pattern correlated with an elevated pulmonary artery resistance. All the patients with pulmonary arterial hypertension showed an abnormal capillary pattern, decreased diffusion capacity for carbon monoxide, and elevated pulmonary vascular resistance. Nailfold capillary abnormalities show a close relation to pulmonary arterial hypertension. CONCLUSIONS: Nailfold capillary abnormalities are useful for detecting vascular abnormality in clinical practice. These facts stress the importance of nailfold capillary abnormalities in SSc.

Capillaries↗
PubMed2001

Non-specific interstitial pneumonia as pulmonary involvement of systemic sclerosis.

The pathological features of lung disease in nine patients with systemic sclerosis (SSc) were evaluated. The patients comprised one man and eight women, with a median age of 58 years. SSc was diagnosed according to the criteria of the American Rheumatism Association. In all patients, high resolution computed radiographic scanning of the lungs (HRCT) was performed, and apparent honeycomb formation was seen in four patients. Pathologically, four patients were diagnosed with usual interstitial pneumonia (UIP), three with non-specific interstitial pneumonia (NSIP) group II, one NSIP group II-III, and one NSIP group II with diffuse alveolar damage. HRCT showed no apparent honeycomb formations in patients diagnosed with NSIP. This is the first report describing NSIP as a pulmonary complication of SSc.

Adult↗
PubMed2001

[Lung fibrosis quantified by HRCT in scleroderma patients with different disease forms and ANA specificities]

OBJECTIVE: To define the prevalence of interstitial lung fibrosis in systemic sclerosis (SSc) and its relationship with the different clinical forms of disease and ANA specificities. METHODS: Fifty patients with SSc were submitted to pulmonary high resolution computed tomography (HRCT). Lung abnormalities were evaluated according to Warrick's score that considers both the severity and the extent of fibrotic lesions. RESULTS: Pulmonary HRCT abnormalities were observed in 84% of SSc patients. Ground glass aspects (60%), irregular pleural margins (56%) and septal/subpleural lines (68%) were the most common lesions. The distribution of these abnormalities favoured the posterior basilar segments of both lungs. HRCT findings were significantly more frequent in males and in patients with the cutaneous diffuse form of SSc and with the specific antibody anti-Scl70. CONCLUSIONS: HRCT is a very useful method for the diagnosis of interstitial lung fibrosis in SSc. Warrick's score permits to quantify the HRCT findings and to evaluate their relationship with the disease clinical forms and ANA specificities.

Journal Article↗
PubMed1992

Studies of the microvascular endothelium in uninvolved skin of patients with systemic sclerosis: direct evidence for a generalized microangiopathy.

Various parameters for assessing endothelial cell (EC) metabolism, including immunohistochemistry and adenosine uptake, have been compared in the clinically uninvolved skin of patients with diffuse systemic sclerosis (DSS), CREST, incomplete CREST syndrome (ICREST), primary Raynaud's disease (1 degree RD) and normal controls. Evidence of platelet adhesion to EC, decreased EC storage of factor VII-related antigen, changes in EC morphology and decreased adenosine uptake by EC, were found in the dermal microvasculature of normal skin of patients with DSS, CREST and ICREST, but not in 1 degree RD. These data indicate that a generalized microvascular endothelial dysfunction is present in the skin of patients with the systemic forms of scleroderma.

Adult↗
PubMed1990

Clinical significance of antinuclear antibodies in Japanese patients with systemic sclerosis.

Clinical significance of specific antinuclear antibodies (ANA) in Japanese patients with systemic sclerosis was studied. The patients with systemic sclerosis were classified into four groups according to ANA: (1) anticentromere antibody-positive; (2) anti-Scl-70 antibody-positive; (3) anti-nRNP antibody-positive, and (4) others. The mean score (the number of positive signs in six selected specific signs) of the patients with anti-Scl-70 antibody was significantly higher than those of the other three groups. More frequent contracture of phalanges in the patients with anti-Scl-70 antibody, less diffuse pigmentation in the patients with anti-nRNP antibody, and less pulmonary fibrosis in the patients with anticentromere antibody were revealed. As shown above, the detection of specific ANA in systemic sclerosis is clinically important.

Adult↗
PubMed2004

Nonmyeloablative stem cell transplant in a patient with advanced systemic sclerosis and systemic lupus erythematosus.

Systemic sclerosis (SSc) is an uncommon connective tissue disease characterized by excessive collagen deposition within the skin and internal organs. Most patients with diffuse severe SSc are treated with immunosuppressive agents, but patients with advanced disease have very high 5-year mortality rates despite adequate therapy. We describe a patient with both diffuse cutaneous SSc and systemic lupus erythematosus who showed mixed chimerism 29 months after undergoing nonmyeloablative stem cell transplant. She experienced remission of both diseases.

Adult↗
PubMed1985

Endomyocardial biopsy.

Endomyocardial biopsy is an accepted, useful invasive tool for the analysis of human endomyocardium at the cellular and subcellular levels. It is applicable in the evaluation of specific diseases including cardiac allograft rejection, myocarditis, anthracycline cardiotoxicity, and infiltrative cardiomyopathies. The procedure can be performed in a cardiac catheterization room on an outpatient basis. The technique is quite safe when performed by trained cardiologists. Left ventricular biopsies are also safe but require systemic heparinization to prevent thromboembolization. The clinical indications for performing an endomyocardial biopsy include routine followup and suspected rejection of cardiac allograft, suspected myocarditis, monitoring or diagnosis of suspected anthracycline cardiotoxicity, and suspected secondary cardiomyopathies. Left ventricular endomyocardial biopsy is indicated for diseases that predominantly involve the left side of the heart, including left heart irradiation, cardiac fibroelastosis in infants, endomyocardial fibrosis, and scleroderma heart disease, and when right ventricular biopsy is unsuccessful. Endomyocardial biopsy is increasingly being used for research in the areas of tissue biochemistry, primary and valvular cardiomyopathies, immunology, beta receptor enzymology, drug interactions, and myocardial fibrosis. Endomyocardial biopsy has not been shown to be clinically useful in the evaluation of primary, dilated, hypertrophic, or alcoholic cardiomyopathies. These disease processes all lack pathognomomic microscopic abnormalities, and subclassification has neither been successful nor therapeutically useful. In addition, this technique is limited in diagnosing any cardiac abnormality that is not diffuse, inasmuch as only a few samples of the endomyocardial layer are obtained for evaluation. Therefore, a negative biopsy result is not 100 percent specific in excluding certain diseases. A further limitation of this technique is the need for an experienced cardiac pathologist who is well versed in interpretation of biopsy specimens. Finally, there should be a sufficiently large case load to train and to maintain skilled practitioners so that the procedure can be performed with little risk. The role of endomyocardial biopsy will continue to expand as research continues to find more uses for the technique and as more clinicians become skilled in its use.

Antibiotics, Antineoplastic↗
PubMed2006

Coronary angiographic findings in asymptomatic systemic sclerosis.

The objective of this study was to assess coronary arterial involvement in asymptomatic systemic sclerosis (SSc) patients. Fourteen female patients with SSc (five limited and nine diffuse) were recruited for this study. All patients fulfilled the following 1980 American College of Rheumatology criteria for classification of SSc Masi et al (Arthritis Rheum 23:581-590 1980). None of them had chest pain nor electrocardiogram (ECG) changes suggestive of myocardial ischemia. All patients underwent thorough history taking, full clinical examination, routine laboratory investigations, and basic screening for conventional atherosclerotic disease risk factors. ECG and coronary catheterization were done for all patients. We detected 19 coronary angiographic abnormalities in our cohort. Three out of nine diffuse SSc patients (33.33%) had ectasia of the coronary arteries, and all of them had slow flow but none in the limited type. One patient with limited SSc showed spasm. Three out of five patients with limited type (60%) had stenosis, one of them had uncontrolled hypertension, while none of the diffuse type had. Five patients (55.55%) of the diffuse type had tortuosity, while it was found in only two patients (40%) of the limited type. Three patients (33.3%) of the diffuse type had calcification of the coronaries, while it was seen in two patients (40%) of the limited type. Pathological involvement of coronary arteries in asymptomatic SSc patients is not uncommon but not paralleled by clinical symptomatology.

Cardiac Catheterization↗
PubMed2006

Pneumatosis cystoides intestinalis with systemic sclerosis, limited type resulting in a poor prognosis.

Pneumatosis cystoides intestinalis (PCI) is a rare disease characterized by the presence of multilocular intramural clusters of gas in the alimentary tract and has been considered to have a favorable response to conservative treatment. We describe the first case of limited type of systemic sclerosis (SSc) with PCI. A 74-year-old Japanese woman presented with a 4-month history of an unhealed cutaneous ulcer on the right third finger, along with sclerodactyly of bilateral hands. Proximal skin sclerosis was absent. The patient reported acute abdominal pain, and a diagnosis of PCI was established on plain radiography. The patient died of multiple organ failure 5 months after the development of PCI. PCI is rarely complicated with SSc, and all cases previously reported were associated with diffuse SSc. Because PCI is one of the poor prognostic factors of SSc, we should recognize the presence of this condition even in patients with limited cutaneous involvement.

Aged↗
PubMed1996

Diffuse alveolar hemorrhage in limited cutaneous systemic sclerosis with positive perinuclear antineutrophil cytoplasmic antibodies.

A 54-year-old woman with limited cutaneous systemic sclerosis (SSc) developed repeated diffuse alveolar hemorrhage with positive perinuclear antineutrophil cytoplasmic antibodies (pANCA). Reports of diffuse alveolar hemorrhage in SSc have been rarely described only in the diffuse clinical variant. To our knowledge, this is the first report of pANCA positive diffuse alveolar hemorrhage in the limited form of SSc. Diffuse alveolar hemorrhage is a rare, but potentially life threatening cause of respiratory distress in the limited form of SSc that should be recognized and treated effectively.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2005

Juvenile systemic sclerosis: a follow-up study of eight patients.

Juvenile systemic sclerosis (jSSc) is a rare form of systemic sclerosis (also known as scleroderma). Fewer than 10% of SSc cases have their onset before age 20 and fewer than 2% before the age of 10. Few case reports and cohort studies on jSSc have been published. Our objective was to assess the clinical and laboratory characteristics of eight cases of juvenile-onset SSc followed up at our institution. Clinical manifestations of SSc were recorded, and immunologic laboratory tests including antinuclear antibodies (ANAs), anti-centromere antibodies (ACAs), and anti-Scl-70 antibodies were assessed. The female-to-male ratio was 7:1, and age at onset ranged from 3-17 years. At the time of analysis, 2 of 8 patients were still under 18 years of age, and 6 of 8 patients were adults. The mean follow-up period from the onset of disease was 19.1 years. Raynaud's phenomenon was present in all cases. Only 2 of 8 patients had diffuse SSc. During follow-up, pulmonary fibrosis developed in only 2 of 8 patients and cardiovascular manifestations in 3 of 8 patients. Secondary sicca syndrome was present in 2 of 8 cases. Regarding immunologic laboratory markers, 7 of 8 patients were ANA positive. However, none of these patients ever carried anti-Scl-70 antibodies, and only 2 of 8 patients had ever had ACA seropositivity. Our results suggest that in jSSc, Raynaud's phenomenon is more severe, whereas internal organ manifestations and the frequency of autoantibodies are far less pronounced than in adult-onset SSc. Also, the survival rate and final outcome of patients with jSSc appear to be better than those in patients with adult-onset SSc.

Adolescent↗
PubMed1995

Small bowel bacterial overgrowth in systemic sclerosis: detection using direct and indirect methods and treatment outcome.

Twenty-four patients with proven systemic sclerosis and with symptoms suggestive of malabsorption (i.e. chronic diarrhoea and weight loss) were investigated for small bowel bacterial overgrowth. Of the patients selected, six were suffering from the diffuse form of the disease. Jejunal aspiration was performed in all patients, and in nine normal volunteers. A specially designed double-lumen sterile catheter was used for this purpose and was introduced via a gastroscope. Twenty of these patients underwent a glucose hydrogen breath test. Eight patients (33%) had significant bacterial counts: > 10(5) colony forming units per ml (cfu/ml) of jejunal fluid. Less than 10(2) cfu/ml were found in the jejunal fluid from the nine control subjects. Glucose hydrogen breath testing was positive in seven patients, all of whom had significant jejunal bacterial growth. Diarrhoea rather than weight loss was shown to be the symptom which correlated best with the presence of bacterial overgrowth. Ciprofloxacin was used in six patients whose diarrhoeal symptoms ceased dramatically within 48 h of commencing the antibiotic. Trimethoprim produced a partial response despite bacterial sensitivity. A disadvantage of the hydrogen breath test is that subsequent antibacterial therapy cannot be specific, as bacterial species, antibiotic sensitivity and resistance are unknown. Systemic sclerosis involving the small intestine in the past has been said to more prevalent in patients with diffuse disease, whereas this study showed a preponderance of patients with long-standing limited cutaneous systemic sclerosis and small bowel involvement.

Adult↗
PubMedDate not supplied

Vesical telangiectasias as a cause of macroscopic hematuria in systemic sclerosis.

A 36-year-old female with diffuse cutaneous systemic sclerosis (dcSSc) developed macrohematuria due to vesical telangiectasias that was responsive to diathermocoagulation of the vasal lesions. This is the first report of a patient with dcSSc and vesical telangiectasias leading to severe macrohematuria that was successfully treated with diathermocoagulation.

Adult↗
PubMed1991

[Occupational exposure to silicon and dermatopolymyositis. 3 cases].

After 5, 16 and 21 years of occupational exposure to silica, 2 men and 1 woman, respectively, were diagnosed as having dermatopolymyositis. Pulmonary impairment was always present and took the form of diffuse interstitial fibrosis in 2 patients, whose lung biopsies revealed high particulate silica contents. The role of silica in the induction of dermatopolymyositis is discussed in light of published information on silica-induced connective tissue diseases.

Adult↗
PubMed1990

[Pulmonary changes in progressive systemic sclerosis].

The pulmonary changes of progressive systemic sclerosis in 8 cases were reported. The major clinical complaints were unproductive cough and exertional dyspnea. Pulmonary function tests showed restrictive ventilatory defects and impaired diffusing capacity in most of the cases examined. On chest X-rays, diffuse mottling and linear densities were seen in lungs, predominately in the basal regions. On pathological examinations, pulmonary interstitial fibrosis occurred in early stage of the disease. The results showed that pulmonary involvement may be an early event in progressive systemic sclerosis.

Adolescent↗