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Three patients with diffuse variety of systemic scleroderma were treated with 2-chloro-2'-deoxyadenosine. In two of them a moderate improvement of skin involvement was observed. No significant effects of the drug on the immunological parameters have been found.
A number of reports of potential etiologic agents of localized and systemic scleroderma appeared in the past year, including alterations in tryptophan metabolism, use of appetite suppressants, and exposure to silicone. An infectious agent, Borrelia burgdorferi, was found not to be implicated in localized scleroderma. The improvement in outcome of systemic scleroderma complicated by renovascular hypertension was highlighted in several papers, as was the emerging importance of cardiac and pulmonary involvement. Recent advances in the early detection and evaluation of cardiac and pulmonary complications of scleroderma are discussed.
BACKGROUND: Patients with systemic scleroderma exhibit a noticeable slowing of blood cell velocities or even stasis in the capillaries of the skin. In this study the effects of transdermally applied prostaglandin PGE1 ethyl ester on nutritive cutaneous perfusion and on Raynaud's symptoms were investigated. PATIENTS AND METHODS: 24 patients with systemic scleroderma were treated transdermally over a period of 14 days with prostaglandin E1 ethyl ester patches. The response of blood cell velocity in the nailfold capillaries to cold exposure was tested in 20 patients, and all of the patients recorded the number of Raynaud's episodes in a journal over a period of two weeks. RESULTS: After the transdermal application of prostaglandin E1 ethyl ester there was an increase in blood cell velocity in the nutritive capillaries of systemic scleroderma patients (increase from 0.35 +/- 0.14 mm/s to 0.47 +/- 0.11 mm/s, (p < 0.05)). At the same time there was a decrease in the number of Raynaud's episodes (2.9 +/- 2.4 per day to 2.6 +/- 2.0 per day (p < 0.05)). CONCLUSION: The transdermal application of prostaglandin E1 ethyl ester was shown to have a favourable effect on nutritive blood flow in the capillaries of the skin in systemic scleroderma patients.
There is growing evidence that pathologic changes in the vascular system are implicated in the pathogenesis of systemic scleroderma. It has been suggested that immune complex deposition may be responsible for such changes. We measured circulating immune complexes in 10 patients with severe systemic scleroderma, 1 of whom had clinical evidence of renal disease, and in 3 patients with generalized morphea. None of the patients had significantly elevated levels. Our findings suggest that although circulating immune complexes are of diagnostic and prognostic value in other collagen vascular diseases, they do not play a major role in the pathogenesis of systemic scleroderma in patients who lack clinical evidence of renal disease.
We describe a case of systemic scleroderma associated with Graves' disease and review six previously described cases associating the two diseases. Our case seems to be unique in that Graves' disease preceded the occurrence of systemic scleroderma.
Antibodies against nuclear ribonucleoprotein (RNP) were found in 5 of 63 cases of systemic scleroderma, whereas they were present in all but one case of mixed connective tissue disease and in 15 of 67 cases of systemic lupus erythematosus. In all RNP positive cases of systemic scleroderma there were some features of other collagen diseases, and their course was relatively more benign. Studies of RNP antibodies in systemic scleroderma may be of importance for treatment and prognosis.
In recent years a possible aetiological connection between skin sclerosis and an infection with Borrelia burgdorferi has been discussed, but this association has not yet been reported for systemic scleroderma. Several treatment modalities are suggested for systemic scleroderma, but no treatment has yet been found to alter the overall course of the disease. This report describes a 61-year-old woman with Raynaud's phenomenon, nail-fold changes and circulating anticentromere antibodies, who showed an abrupt onset of erythemas and doughy swellings involving the face and upper trunk, followed by thickening and induration of the skin mimicking diffuse systemic scleroderma. Laboratory tests including enzyme-linked immunosorbent assay (ELISA), immunoblot and urine polymerase chain reaction (PCR) showed an infection with B. burgdorferi sensu lato that was successfully treated with intravenous ceftriaxone, an antibiotic recommended for Lyme borreliosis. Fourteen days after the end of treatment the skin was no longer stiff and indurated and had returned to its normal predisease state. This case suggests that Lyme disease should be considered in atypical cases of skin sclerosis in patients predisposed to the development of systemic scleroderma.
The data of instrumental studies in 43 patients with systemic scleroderma were compared to the clinical picture, which made it possible to specify the character and to reveal new regularities of heart lesions in patients with the above disease. The instrumental research methods, echo- and polycardiography in particular, allow an objective control of heart lesions in systemic scleroderma which should be specified in making the diagnosis and in the course of the follow-up of patients.
Hyperbaric oxygenation treatment of systemic scleroderma has a favourable effect on microcirculatory changes whose positive dynamics can be demonstrated by conjunctival biomicroscopy. These changes include accelerated blood flow and decrease in the degree of erythrocyte aggregation. The method can be used for the objective assessment and for prognosis of the effectiveness of hyperbaric oxygenation treatment in patients with systemic scleroderma.
Lung lesion is a most common organ changes in systemic scleroderma (SSD) detectable in approximately 70% of the patients at autopsy. Echocardiography is now one of the main non-invasive diagnostic methods for pulmonary hypertension (PH), in patients with scleroderma in particular. The study was undertaken to examine the incidence of PH and the specific features of its development in patients with SSD. Thirty-one patients with SSD (30 females and 1 males) treated at the rheumatological unit of the regional clinical hospital in 2000 to 2002 were examined. The patients' age ranged from 33 to 75 years (mean 47.7 +/- 1.7 years). Pulmonary systolic blood pressure (PSBP) (higher than 30 mm Hg) was recorded in 51.6% of the patients. Hypertrophy of the right ventricle (RV) (more than 0.5-cm increases in the thickness of its anterior wall) was found in 20 (64.5%) patients with SSD, 4 cases had hypertrophy of the RV anterior wall without resting elevated PSBP. More than 2.5-cm RV dilation was observed in 6 (19.4%) patients. This study has provided evidence for that echocardiography is of high informative value in detecting PH and right cardiac changes in patients with systemic scleroderma, which shows this technique to be a screening in these cases.
In 135 patients with systemic scleroderma, we compared three different methods to determine antinuclear autoantibody (ANA) specificity: indirect immunofluorescence, double immunodiffusion, and, employing recombinant antigens, immunoblotting using both marker autoantigens of this disease. A characteristic Scl-70 antibody pattern was found on HEp-2 cells in 83.8% of the patients, double immunodiffusion was positive for the Scl-70 antibodies in 81.9%, and immunoblot with the recombinant topoisomerase I (Topo I) was positive in 71% of the patients. For the centromere autoantibodies we found a high concordance between the anticentromere antibody (ACA) pattern on HEp-2 cells (27 patients positive) and the detection of recombinant kinetochore in immunoblotting (26 patients positive). The three testing techniques gave comparable results, except that the Topo I recombinant antigen used in immunoblotting reacted strongly with fewer than expected of the known Scl-70-positive sera. However, a method using recombinant antigens expressing all epitopes (rather than one of the epitopes of Topo I) will undoubtedly become the method of choice for detecting antibodies in systemic scleroderma. Using the immunoblotting technique with the recombinant antigens we detected in four patients antibodies against both Topo I and kinetochore. More severe symptoms of systemic scleroderma were found in patients who had both antibodies. The combined presence of both marker autoantibodies is therefore not as rare as previously reported and may predict severe disease.
Prognosis of systemic sclerosis (scleroderma, Ssc) is largely depending on involvement of internal organs. Abnormalities of the gastrointestinal tract are found most frequently (85%), especially decreased motility of the oesophagus, which has little impact on the longterm clinical course of Ssc. Pulmonary manifestations can be demonstrated in 40-90% of patients; one must distinguish between pulmonary hypertension or fibrotic lung disease. The heart is affected in 50% of cases. Patchy or diffuse myocardial fibrosis, as well as pericarditis and pericardial effusions can induce symptoms of arrhythmia or congestive heart failure. Renal involvement is associated with increased mortality and occurs in 45% of Ssc, producing proteinuria, hypertension, scleroderma renal crisis and renal failure. In conclusion, involvement of the lungs, heart and kidneys are determining factors for the longterm course of systemic sclerosis.
Liver electron microscopic studies were performed in 14 patients with systemic scleroderma. In 13 of these patients, giant mitochondria were demonstrated in the hepatocytes. This ultrastructal abnormality was present whatever the type and duration of the disease and was also present even when the liver was histologically normal. The mechanism of formation of giant mitochondria in systemic scleroderma is unknown.
There is increasing evidence that endothelial damage occurs at a very early stage during the course of systemic scleroderma. Endothelial damage is accompanied by impaired microvascular function, which has clearly failed in patients with systemic scleroderma, as evidenced by necrosis of the fingertips in severe cases. We investigated two important determinants of microvascular function, namely capillary blood pressure and capillary red blood cell velocity, simultaneously in the same capillary. In patients with systemic scleroderma and in healthy volunteers matched for age and sex, capillary blood pressure was measured by direct cannulation and capillary red blood cell velocity by video microscopy. Capillary blood pressure and capillary red blood cell velocity were significantly lower in patients (14.27 +/- 4.34 mmHg, 230 +/- 310 microm per s) than in healthy controls (19.06 +/- 3.69 mmHg, p < 0.008, and 910 +/- 240 microm per s, p < 0.003) at an ambient temperature of 22 degrees C, whereas no significant difference in skin temperature was observed (23.7 +/- 0.9 degrees C vs 24.7 +/- 1.9 degrees C) and no occlusion of finger arteries was detected. Capillary blood pressure in enlarged capillaries did not differ from that in normal-shaped capillaries in the patients (correlation of diameter and capillary blood pressure, R2 = 0.04), which was also the case with capillary red blood cell velocity (R2 = 0.13). Capillary pulse pressure amplitude and capillary red blood cell velocity showed a strong correlation (R2 = 0.81), suggesting that the pressure gradient across the capillary loop, which is the driving force for capillary red blood cell velocity, was mainly dependent on precapillary resistance. These observations reflect the inadequate microvascular function in systemic scleroderma, which may be due mainly to a pathophysiologic functional increase in precapillary resistance, even at comfortable ambient temperatures.
This study was performed on seven children with systemic scleroderma, three with the diffuse and four with the limited type. All three patients with diffuse scleroderma had high titers of clumpy pattern antinucleolar antibody on HEp-2 cells. The course of the disease was severe, and two children died. Four children with limited scleroderma had mild disease, and Scl-70 antibody, an immunologic marker that in adults is associated mostly with diffuse scleroderma. In one child Scl-70 antibody and anticentromere antibody coexisted, although previously the two were believed to be mutually exclusive. This study shows that limited scleroderma of childhood with slight cutaneous involvement may be associated with the Scl-70 marker. The findings in 10 adults in whom Raynaud's phenomenon developed in childhood and indurations appeared some years later, point to the significance of careful observation of these children, with repeated testing for immunologic markers of SSc. An important new finding is the association of different types of systemic sclerodermas with specific immunologic markers.
Different subsets of peripheral blood mononuclear cells (MNC) from 15 patients with systemic scleroderma were tested for their ability to evoke angiogenesis in a xenogenic system. The angiogenic capability of total MNC from patients with systemic scleroderma was lower than that of normal human cells, irrespective of the form of the disease. However, the capability of a monocyte-enriched subset of MNC from patients with scleroderma was found to be increased, as compared with their total MNC and with that of the corresponding subset from healthy individuals. This might be due to the activation of monocytes in the disease.
The purpose of this study is to evaluate the cardiovascular function in patients with incomplete type of SSc (SSSD; Scleroderma Sjögren syndrome associated Spectrum Disorders), SSc (Systemic Scleroderma) and SLE (Systemic Lupus Erythematosus) by using ECG and digital plethysmograph. We also preformed ambulatory ECG monitoring for cases of SSSD and SSc with QT interval prolongation. 1) In ECG findings, ischemic myocardial damage, left ventricular hypertrophy or QT interval prolongation was observed in 33% cases of SSSD and 32% cases of SSc, respectively. In addition, parasympathetic disorders were observed in approximately 20% cases of SSSD, SSc and SLE. 2) Digital plethysmogram findings suggested that the peripheral vascular damage was present in both cases of SSSD and SSc, and its severity was lower in SSSD than SSc. The incidence of abnormal cardiovascular function tests in SSSD was relatively less than that in SSc. The cardiovascular function tests are useful to find cardiovascular abnormalities in these collagen diseases.
A 49-year-old patient with systemic scleroderma was referred to the Department of Dental prostheses for the Degree in Dentistry and Dental Prostheses at Florence University because he was unable to wear his existing complete dentures. The upper denture was not correct owing to the position of the artificial teeth and the short base, and the lower denture caused pain when used. A complete new dental prosthesis was therefore required to overcome the difficulties caused by this systemic disease. Scleroderma is a progressive disease that causes the anelasticity of the mesenchymal tissues owing to post-inflammatory fibrotic and degenerative alterations of unknown etiology. Important changes also occur in the mouth: difficulty opening the mouth, hypo- or non-extendibility of the soft perioral tissues. The construction of the complete upper and lower dentures posed a series of major technical problems compared to a patient not suffering from this pathology. The first core, the rimming of the individual core holder, calculating the vertical dimension and the assembly of the front teeth were all phases that required technical modifications and special care, so that the techniques of our School could be adapted to this particular case. The cosmetic and functional result was undoubtedly good. The patient has been wearing these dental prostheses for five years with satisfaction.