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PubMedDate not supplied

[Scintigraphic diagnosis of esophageal involvement in systemic sclerosis (scleroderma)].

Motor function disturbances (MFD) of the esophagus occur in the majority (90%) of patients with systemic sclerosis. We have tested the diagnostic value of esophagus scintigraphy in 20 patients with systemic sclerosis (12 CREST and 8 with diffuse form) and 18 controls (11 normals and 7 with pyrosis and/or regurgitation), in order to establish sensitivity, specificity, effects of body posture, and use of a liquid or solid meal in the evaluation of MFD of the esophagus. The patients drank 4 ml of fruit juice marked with 500 microCi of 99mTc-sulfur-colloid and afterwards 10 g of an equally marked and scrambled egg. Pictures were made with a Gamma-Camera until 180 s. Radioactivity was plotted against time and separately analysed for the upper, middle and distal esophagus. 65% of the systemic sclerosis patients had dysphagia and 70% had abnormal barium meal transit in the esophagus. The scintigrams were altered in all the patients with systemic sclerosis (p less than 0.001). Sensitivity using fluids was 95%, and specificity was 89%; with solid food 100% and 50%, respectively. Tests done in the upright position showed a better clearance of the esophagus in patients with systemic sclerosis and control patients, with loss of sensitivity. The qualitative analysis yielded little benefit to the reported results. The quantitative analysis under use of 2 indices (total transit time and clearance rate) permitted a clear distinction among patients and control individuals with and without upper digestive symptoms in the first 15 s of the test. The esophagus scintigraphy is a simple, quantitative and very sensitive method for the diagnosis of MFD of the esophagus in patients with systemic sclerosis.

Adult↗
PubMed2005

[Syndromes of peripheral nervous system lesions and mechanisms of their formation in disorders of connective tissue].

Systemic rheumatoid diseases are often concomitant with the development of central and peripheral systems pathologies. Presented are the results revealing high frequency of peripheral nervous system lesions (lupus erythematosus and systemic scleroderma), which characterized by polyneuropathy and tunnel syndromes. Based on the results of literature and own studies, pathological mechanisms of peripheral nervous system lesions in diffusion disorders of connective tissue were singled out as follows: ischemic, immunological and metabolic. Taking these mechanisms into account will permit to conduct pathogenetically valid therapy and to improve its results.

Connective Tissue Diseases↗
PubMed1991

[Clinical report of a case of CREST syndrome].

A case of CREST syndrome presenting with marked diffuse calcinosis and prevailing joint involvement is presented by the authors. Diffractometry revealed the presence of calcified hydroxy-apatite crystals within subcutaneous nodules. Scleroderma-like microcirculatory changes with blood flow alterations typical of Raynaud's syndrome were present on capillaroscopy. The authors feel the case to be of interest not only in view of the rare occurrence of the condition but also of the particular extent and severity of the signs and symptoms presented by this patient.

Female↗
PubMed2006

Macrophage migration inhibitory factor promoter polymorphisms and the clinical expression of scleroderma.

OBJECTIVE: To investigate the potential association between functional polymorphisms in the gene for the innate mediator, macrophage migration inhibitory factor (MIF), and the clinical expression of systemic sclerosis (SSc). METHODS: Genomic DNA samples and clinical data were collected from the Scleroderma Family Registry and DNA Repository at the University of Texas Health Science Center at Houston. A total of 740 subjects were studied; 203 of them had diffuse cutaneous SSc (dcSSc), 283 had limited cutaneous SSc (lcSSc), and the remaining 254 healthy subjects served as controls. Association analyses were performed on the whole data set and on patient and sex subsets. Significant relationships were determined between clinical variables and MIF polymorphisms for each disease subtype in the studied groups. RESULTS: The frequency of the -173*C MIF allele, which was previously reported to be associated with high production of MIF, was lower in the lcSSc group (12.6%) than in the dcSSc (19.2%) or control (18.5%) groups (P = 0.010 and P = 0.011, respectively). Haplotype analysis for 2 closely linked polymorphisms in the MIF promoter showed that in white subjects with lcSSc or dcSSc, the lcSSc population had a significantly lower representation of the high-expression MIF haplotype defined by -173*C and -794 with 7 CATT repeats (C7) (P = 0.015, odds ratio 1.94 [95% confidence interval 1.14-3.32]). Fibroblasts encoding the C7 MIF haplotype were observed to produce more MIF upon in vitro stimulation than those with a non-C7 haplotype. CONCLUSION: Functional promoter polymorphisms in the MIF gene affect the clinical presentation of SSc. The proinflammatory haplotype defined by C7 is underrepresented in patients with lcSSc.

Female↗
PubMed1999

[Scleroderma--systemic sclerosis. Serology, lung function and survival].

Patients with systemic sclerosis (SSc) were studied with regard to the presence of antinuclear antibodies (ANA) and their clinical correlates (n = 230), pulmonary function (n = 176), and mortality and causes of death (n = 344). ANA were found in 85%. Anti-centromere antibodies were found in 34%, anti-Scl-70 antibodies in 13% and anti-U1-RNP antibodies in 6.5%. These serological groups were associated with limited SSc, diffuse SSc, and myositis/arthritis, respectively. The most prevalent finding at first lung function test was isolated reduction of diffusion capacity (47%). Further deterioration of diffusion capacity was related to the presence of anti-centromere antibodies and increased sedimentation rate. The standardized mortality rate (SMR) was 2.9, higher in young patients (SMR = 13) and patients with diffuse SSc (SMR = 4.5).

Adolescent↗
PubMed1992

Acute myocarditis in fulminant systemic sclerosis.

Myositis and myocarditis have been reported in progressive systemic sclerosis, and these patients have had favorable therapeutic responses to intravenous pulse methylprednisolone. Thus far, premortem biopsy documentation of myocarditis and myocardial fibrosis has not been reported in such patients. We report the case of a patient with subacute congestive heart failure six months after she developed Raynaud's phenomenon. Clinical examination was typical of scleroderma but there was no proximal muscle weakness. She had elevated creatine kinase and MB-creatine kinase and laboratory evidence of hypothyroidism. Echocardiogram demonstrated four-chamber dilatation and severe left ventricular dysfunction. Cardiac catheterization revealed normal epicardial coronary arteries and severely decreased cardiac index. A skin biopsy specimen of the forearm was consistent with diffuse systemic sclerosis, and an endomyocardial biopsy specimen demonstrated mild fibrosis and lymphocytic infiltrate. Her heart failure initially improved with digoxin, furosemide, and enalapril. She also received L-thyroxine and intravenous methylprednisolone. The heart failure progressed over the next six weeks and she died. Patients with scleroderma and new-onset heart failure may have acute myocarditis.

Acute Disease↗
PubMed1988

A survival study of patients with scleroderma diagnosed over 30 years (1953-1983): the value of a simple cutaneous classification in the early stages of the disease.

A longitudinal study was carried out on 177 patients with scleroderma between 1953 and 1983. Patients were classified into 3 types according to the extent of skin sclerosis within one year of presentation: Type 1 with sclerodactyly only; Type 2, skin stiffness proximal to the metacarpophalangeal joints but sparing the trunk; and Type 3, diffuse skin stiffness including the trunk. Ten year survival was 71% in Type 1, 58% in Type 2 and 21% in Type 3. Quality of life was best in Type 1, intermediate in Type 2 and worst in Type 3. Nailfold capillary abnormalities did not discriminate between the types, but anticentromere antibody correlated strongly with Type 1. The simple subdivision of scleroderma based on the early extent of clinical skin involvement has been found to be useful in predicting longterm outcome in this highly variable disease.

Adult↗
PubMed1996

Role of Borrelia burgdorferi in the pathogenesis of morphea/scleroderma and lichen sclerosus et atrophicus: a PCR study of thirty-five cases.

Morphea (localized scleroderma), and lichen sclerosus et atrophicus (LSA) share common features with acrodermatitis chronica atrophicans (ACA), a known chronic form of borreliosis. These include similar histologic findings such as diffuse dermal fibrosis. These observations have led several investigators to consider the possibility of Borrelia burgdorferi (Bb) as a common etiologic factor among all of these diseases. The aim of this study is to investigate the role of Bb in the pathogenesis of morphea and LSA, by assaying for its presence in lesional skin biopsies from patients with these diseases. We utilized the nested polymerase chain reaction (PCR) technique to selectively amplify a longer segment of a Bb-specific somatic gene, on DNA from paraffin-embedded, formalin-fixed tissues. The results revealed no Bb-specific DNA sequence in 28 specimens of morphea/scleroderma and 7 of LSA with varying stages of disease. Furthermore, confirmatory Southern blot of the PCR product, resulted in similar findings. These data seriously question the role played by this spirochete in the pathogenesis of morphea and LSA, at least in the southeastern part of the USA.

Adult↗
PubMed1990

Development of diffuse fasciitis with eosinophilia during L-tryptophan treatment: demonstration of elevated type I collagen gene expression in affected tissues. A clinicopathologic study of four patients.

We describe the cases of four women who developed a scleroderma-like syndrome during L-tryptophan treatment for insomnia or tinnitus. The illness was characterized by swelling of the extremities, skin rash, myalgia, and elevation of the peripheral blood eosinophil count, followed by rapidly progressive cutaneous and subcutaneous induration. The histopathologic examination of affected skin showed thickening of the fascia, deep dermal fibrosis, and accumulation of mononuclear cells and abundant eosinophils. The expression of the type I procollagen gene was examined by in-situ hybridizations of affected skin with a human sequence-specific complementary DNA (cDNA). Increased hybridization signals were detected in the deep dermis and fascia, indicating enhanced expression of the collagen gene. The temporal association of L-tryptophan use and the development of a scleroderma-like illness in these four patients suggests a causal relation between L-tryptophan or its metabolites and the stimulation of fibroblast collagen gene expression that results in dermal and fascial fibrosis.

Aged↗
PubMed1989

Esophageal electromyography in scleroderma patients with functional dysphagia.

Simultaneous recordings of myoelectric and manometric activities of the esophagus were made in two groups of patients with scleroderma. Group A consisted of patients who suffered from functional dysphagia that had appeared recently, and who had a normal size esophagus when examined radiographically. Patients in group B had complained of functional dysphagia for several years and showed a dilated hypotonic esophagus on x-ray. A control group of five normal subjects was also examined in the same manner. The study was carried out by means of a peroral probe with two pairs of suction-needle electrodes and two manometric side-hole catheters positioned at the same levels as the electrodes. Standard manometric examination of the esophagus was carried out in all patients. Normal subjects after each deglutition showed a propagated burst of spikes in correspondence with the ascending phase of the peristaltic wave, whereas, in the period between deglutitions, rare spikes and no pressure waves were recorded. Group A patients were characterized by the frequent appearance during the interdeglutitive period of spontaneous rhythmic sequences or bursts of spikes associated with pressure waves. In these patients, repetitive nonpropagated spike bursts with a higher than normal amplitude and duration were observed after deglutitions in association with high-amplitude nonpropagated repetitive pressure waves similar to those observed in diffuse esophageal spasm (DES). Five of the seven group B patients showed spike bursts and pressure waves with both amplitude and duration markedly lower than normal. The remaining two patients from group B presented no spike bursts or pressure waves at all. Standard manometry demonstrated findings equivalent to those obtained via electromyography (EMG) in all patients of group B and in only two patients of group A. In conclusion, the functional dysphagia of patients with scleroderma can be attributed to two different motor disorders. The first one is characterized by disorganized myoelectric hyperactivity and may have a manometric appearance similar to that of diffuse spasm. The second one is characterized by a marked decrease in myoelectric activity and corresponds to the classic manometric finding of scleroderma involvement of the esophagus. Both these myoelectric patterns seem to be related to subsequent stages of esophageal scleroderma involvement.

Adult↗
PubMed1996

Cardiorespiratory responses to incremental exercise in patients with systemic sclerosis.

Patients with systemic sclerosis are known to have histologic pulmonary abnormalities despite normal chest radiograph or conventional pulmonary function or both. In an attempt to detect early features of lung involvement in progressive systemic sclerosis, we investigated patients with systemic sclerosis using cardiopulmonary exercise testing. We have studied 78 patients who fulfilled the American Rheumatism Association criteria for the classification of systemic sclerosis, and according to the classification of LeRoy, 44 had limited cutaneous systemic sclerosis and 34 had diffuse cutaneous systemic sclerosis. A significantly decreased diffusing capacity (65 +/- 3% of that predicted) was present only in the group with diffuse cutaneous systemic sclerosis. The patients with lung involvement showed a significant reduction in exercise capacity (54 +/- 3% of that predicted) and in oxygen uptake (70 +/- 3% of that predicted). Additionally, we could demonstrate an increased functional dead space ventilation (0.34 +/- 0.02) and widened alveolar-arterial oxygen difference during exercise (44 +/- 3 mm Hg). By cardiopulmonary exercise testing, 12 of the 78 patients (15%) with normal single-breath diffusing capacity for carbon monoxide had increased dead space to tidal volume ratio. Our results suggest that occult pulmonary impairment may be present in patients with normal pulmonary function and that cardiopulmonary exercise testing enables detection of such impairment. Our study results show the limitations of resting data in predicting abnormalities during exercise in patients with systemic sclerosis.

Adult↗
PubMed1986

The microvascular localization of laminin and fibronectin in scleroderma muscle by immune electron microscopy.

Immune electron microscopy using a peroxidase-antiperoxidase (PAP) technique was done on muscle biopsies from patients with scleroderma (PSS), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and normal controls, in order to localize laminin and fibronectin in the muscle capillary basement membrane (BM). Three patterns of staining were noted for the 2 antigens: in/out, diffuse and negative. Staining patterns in PSS biopsies were markedly different from both the normal and disease controls. For laminin the expected in/out pattern was rare in PSS, with virtually all biopsies being either diffuse or negative. In the case of fibronectin, the staining patterns were of equal frequency in PSS, whereas in the control groups diffuse staining was rare. These findings suggest that in addition to the morphologic abnormalities previously noted in PSS muscle capillary BM, there is also an alteration in the distribution of specific BM components in PSS.

Arthritis, Rheumatoid↗
PubMed1982

D-Penicillamine therapy in progressive systemic sclerosis (scleroderma): a retrospective analysis.

In a retrospective study on progressive systemic sclerosis, we compared 73 patients who had received D-penicillamine therapy for a minimum of 6 consecutive months with 45 patients who had not received this drug. All patients had diffuse sclerodermatous skin changes and early disease (less than 3-years duration). D-Penicillamine was prescribed for an average of 24 months (range, 6 to 68 months) with a maximum daily dose of 500 to 1500 mg (median, 750 mg). During a mean follow-up interval of 38 months, the degree and extent of skin thickness, determined on physical examination, decreased considerably more in the patients treated with D-penicillamine than in patients in the comparison group (p = 0.07). The rate of new visceral organ involvement was reduced in patients treated with D-penicillamine, especially for the kidney (p = 0.01). Patients treated with D-penicillamine had a greater 5-year cumulative survival rate (88% versus 66%, p less than 0.05). Therapy with colchicine (23 patients) or immunosuppressive agents (26 patients) was not associated with these improvements.

Biopsy↗
PubMed2002

Vascular and connective tissue histopathologic alterations of the female lower genital tract in scleroderma.

OBJECTIVE: It has been suggested that women with scleroderma (systemic sclerosis, SSc) have cervical changes that may lead to obstetrical or postoperative complications. We evaluate histopathologic features characteristic of SSc in cervicovaginal tissue from women with SSc and compare them to age matched controls. METHODS: Records from the Scleroderma Registry at Wayne State University were matched with surgical specimens in the anatomic pathology files at Hutzel Hospital. Five women with SSc (2 with limited SSc, 3 with diffuse SSc; mean age 49 yrs) were identified who had cervical or vaginal tissue specimens available for evaluation. Small arterioles and surrounding connective tissue in these specimens and those from 26 age matched controls (15 normotensive, 11 hypertensive) were evaluated in blinded fashion. RESULTS: The following specific histopathological features were evaluated in the SSc patients: duplication or disruption of the internal elastica in 5 (100%), medial hypertrophy in 5 (100%), adventitial changes in 3 (60%), connective tissue fibrosis in 1 (20%), and vasculitis in 1 (20%). There was no significant difference in the frequency of the histopathologic changes between SSc and control patients when evaluated independently. However, the presence of 3 or more features was significantly more frequent in the SSc patients (100%) than in the controls (38%) (p = 0.018). CONCLUSION: The histopathological features evaluated were collectively more frequent in SSc patients; however, many of the control patients also exhibited similar abnormalities. In the female lower genital tract these changes, previously attributed solely to SSc, may be related to other factors.

Adult↗
PubMed2002

Life-threatening complications of systemic sclerosis.

Pulmonary arterial hypertension is common in patients with SSc. Fig. 1 shows the diagnostic and therapeutic approach to PAH in SSc. Doppler echocardiography may suggest the diagnosis, but RHC is necessary to confirm PAH and to measure vasoreactivity. Therapy is directed at the underlying connective tissue disease. Vasoreactive patients often benefit from therapy with high-dose calcium-channel [figure: see text] blockers, but most patients are not vasoreactive. Intravenous epoprostenol and oral endothelin-1 receptor antagonists improve hemodynamic measurements and symptoms in SSc-associated PAH. The therapy of right ventricular failure is focused on vasodilators, inotropes, and diuretics with careful attention to avoiding systemic hypotension. The scleroderma pulmonary-renal syndrome and the scleroderma renal crisis are distinct syndromes with different clinical presentations, histopathologic manifestations, treatments, and outcomes. The scleroderma pulmonary renal syndrome is an autoimmune vasculitis of kidney and lung associated with normal blood pressure. Treatment is supportive, and prognosis is dismal. In contrast, scleroderma renal crisis is associated with systemic hypertension, onion skinning of afferent arterioles, and response to ACE inhibition and renal replacement therapy. Pericardial effusions are common but only occasionally lead to tamponade. Esophageal dysmotility is often associated with aspiration, leading to pulmonary fibrosis, pneumonia, or ARDS. Diffuse bowel involvement may result in pseudo-obstruction, bacterial overgrowth, or malabsorption. Prokinetic agents, antibiotics, and parenteral nutrition may be required.

Catheterization, Swan-Ganz↗
PubMed1995

[Phenotype of lymphocytes in bronchoalveolar lavage fluid of patients with lung fibrosis in the course of scleroderma].

Patients with systemic sclerosis frequently develop interstitial lung disease. One of the major sites of pathogenic activity are lymphocytes from lung interstitium. The purpose of this work was phenotype analysis of lymphocytes in bronchoalveolar lavage fluid (BALF) with flow cytofluorometry. We found an increase number of CD8+ lymphocytes and CD8+/T ratio, and an reduced CD4+/CD8+ ratio in patients when compared to healthy volunteers. The diffusing capacity (DLCO) correlated positive with percentage of neutrophils and HLA-DR+ lymphocytes. There are the features of mixed type alveolitis in patients with short disease duration. We found increase number of lymphocytes, neutrophils, CD8+ lymphocytes, HLA-DR+ lymphocytes and CD25+ lymphocytes in this patients. We concluded that fluorocytometric BALF analysis may be useful for the diagnosis and management of lung involvement in patients with scleroderma before the treatment.

Adult↗