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PubMed1989

Progressive systemic sclerosis sine scleroderma presenting as pulmonary interstitial fibrosis.

PURPOSE: The purpose of this work was to report the clinical features of 10 patients with systemic sclerosis presenting with lung disease in the absence of cutaneous involvement (systemic sclerosis sine scleroderma). PATIENTS AND METHODS: The study involved a retrospective review of patients with systemic sclerosis at a tertiary care facility presenting with clinical and/or radiographic evidence of interstitial lung disease in the absence of cutaneous manifestations of scleroderma, and a MEDLINE, computer search for patients who presented with lung disease and later developed taut skin (scleroderma). RESULTS: Ten patients with systemic sclerosis sine scleroderma presenting with pulmonary signs or symptoms were discovered. A positive antinuclear antibody (seven of eight), esophageal dysmotility (eight of nine), restrictive lung disease (six of eight), Raynaud's phenomenon (five of eight), and abnormal nailfold capillaries (six of six) were present in these patients. Six of these 10 patients developed typical scleroderma from four months to seven years after presentation. Lung pathology was examined in six patients and all had changes consistent with interstitial pneumonitis and fibrosis. Four of the 10 patients had occupations that could have exposed them to potential pulmonary toxins. In contrast to patients with typical scleroderma, all 10 patients we discovered were men. conclusions: Systemic sclerosis should be considered in patients with idiopathic interstitial lung disease even in the absence of classic sclerodermatous cutaneous involvement. An esophagram, antinuclear antibody, and widefield nailfold examination may facilitate the diagnosis.

Diagnosis, Differential↗
PubMed1992

Capillary abnormalities, Raynaud's phenomenon, and systemic sclerosis in patients with localized scleroderma.

OBJECTIVES AND DESIGN: In vivo capillaroscopic examination was performed on patients with localized scleroderma to determine whether nailfold capillary abnormalities seen in systemic scleroderma (systemic sclerosis) were also present in the localized form. Twenty-seven patients (24 women, three men) were examined by this technique. RESULTS: Only two patients exhibited scleroderma-type nailfold capillary abnormalities similar to those seen in systemic sclerosis. Both patients also suffered from Raynaud's phenomenon and showed evidence of coexisting systemic sclerosis, one on first examination, the other 1.5 years later. Our results are compared with earlier studies reporting such rare coexistence of the two forms of scleroderma. Earlier capillaroscopic work in this disorder is also reviewed. CONCLUSIONS: These results suggest that the presence, in a patient with localized scleroderma, of nailfold capillary abnormalities similar to those seen in systemic sclerosis should alert the physician to a possible association with systemic sclerosis.

Adolescent↗
PubMed1994

Progression of childhood linear scleroderma to fatal systemic sclerosis.

An 8-year-old girl presented with linear scleroderma, no evidence of systemic disease, and a negative antinuclear antibody (ANA) test. Over the next 12 months, she functioned normally. However, over the subsequent 5 months, she developed dyspnea, progressive pulmonary hypertension, a positive ANA test, and died 17 months after presentation. At autopsy, diffuse pulmonary interstitial fibrosis, small pulmonary arterial fibroplasia, tricuspid and mitral valve subendocardial fibrosis, and distal esophageal fibrosis were seen. Contrary to suggestions in the rheumatology literature, childhood linear scleroderma, even when ANA negative at presentation, may progress to fatal systemic sclerosis.

Antibodies, Antinuclear↗
PubMed1984

[Long-term development of eosinophilic fasciitis. Study of 11 cases].

The long-term outcome of eosinophilic fasciitis (EF) described for the first time by Shulman in 1974 remains unclear. Its exact pathological classification is still contested. We reviewed 11 cases selected exclusively on their initial symptoms being compatible with EF. The outcome of the cutaneous lesions, the investigations of possible visceral involvement were analysed with a follow-up of 1 to 9.5 years (average 4.8 years). Regression of the cutaneous infiltration was complete in 5 cases and partial in 3 cases. No regression was observed in 3 cases. Steroid therapy was given in 10 patients: objective improvement was observed in 5 patients. There was no improvement in the other 5 cases. Ungual capillaroscopy was performed in 9 patients and showed none of the characteristic capillary changes of scleroderma. These results suggest that the prognosis and outcome of EF justify its distinction from systemic scleroderma. The effects of steroid therapy on the cutaneous lesions are very variable.

Adult↗
PubMed1995

Clinical aspects of systemic and localized scleroderma.

After the skin, the gastrointestinal tract is the most frequently affected organ in systemic sclerosis. Gastrointestinal symptoms already may be present early in the course of the disease and do not necessarily correlate with objective findings. Esophageal dysmotility is not specific for systemic sclerosis but occurs in other connective tissue diseases as well. Peripheral macrovascular disease was shown to be increased in patients with limited cutaneous sclerosis; signs of autonomic dysfunction were found in patients with the CREST (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) variant. Pulmonary involvement was shown to be moderately or severely decreased in 40% of a large cohort of scleroderma patients. In one study, no support was found for the association between pulmonary involvement and gastroesophageal reflux. Peripheral nerve involvement is often subclinical and might be associated with anti-U1-RNP and anti-topoisomerase I antibodies. Internal organs are seldomly affected in localized scleroderma. When occurring in childhood and involving an extremity, localized scleroderma can cause growth failure, resulting in long-term functional disability.

Humans↗
PubMed2000

Systemic sclerosis sine scleroderma: demographic, clinical, and serologic features and survival in forty-eight patients.

OBJECTIVE: To describe the demographic, clinical, and laboratory features and natural history of patients with systemic sclerosis sine scleroderma (ssSSc), and to compare them with those of patients with SSc and limited cutaneous involvement (IcSSc). METHODS: The University of Pittsburgh Scleroderma Databank served as the data source. Patients were divided into those who had no skin thickening (ssSSc) and those who had skin thickening only distal to elbows or knees and/or of the face (IcSSc) during their disease course. These two groups were compared with regard to demographic characteristics, clinical, laboratory, and serologic features, and survival rates. Chi-square and Student's t-test analyses were performed, and Fisher's exact test was used as appropriate. RESULTS: Of 555 consecutive patients without diffuse cutaneous SSc, 48 (9%) had ssSSc and 507 (91%) had IcSSc. The ssSSc patients had a mean total disease duration of 18.6 years (15.1 years before study entry and 3.5 years of followup after study entry), and had not developed IcSSc or another connective tissue disease. Other than the absence of skin thickening, the ssSSc group had no significant differences in individual internal organ involvements, laboratory features, serum autoantibody type, or survival rate compared with patients with IcSSc. Within the category of lung involvement, patients with ssSSc had a significantly greater frequency of dyspnea with mild exertion or at rest, and a tendency toward reduced carbon monoxide diffusing capacity (<70% of predicted normal) and primary pulmonary arterial hypertension. Patients with IcSSc had significantly more frequent individual manifestations of digital pitting scars, digital-tip ulcers, telangiectasia, and calcinosis than those with ssSSc, in part related to increased time of observation. Puffy fingers and finger joint contractures were detected significantly more often in IcSSc patients. CONCLUSION: Systemic sclerosis sine scleroderma should be included in the spectrum of SSc with limited cutaneous involvement and should not be considered a distinct or separate disorder.

Adolescent↗
PubMed1994

Neurotropic ulceration in systemic sclerosis.

Scleroderma is primarily a cutaneous disorder but systemic involvement is common and neurological complications are increasingly recognized but previously poorly documented. Few cases of peripheral nervous system involvement in scleroderma have been reported. The peripheral nervous system, however, is rich in connective tissue and sclerodermal involvement may not be as unusual as the literature suggests. A case in which there was a mixed sensorimotor peripheral neuropathy with neurotropic ulceration in a patient with systemic sclerosis is reported.

Foot Ulcer↗
PubMed2006

A case of early onset normotensive scleroderma renal crisis in a patient with diffuse cutaneous systemic sclerosis.

Scleroderma renal crisis (SRC) has classically been defined as a new onset of accelerated arterial hypertension associated with a rapid increase in serum creatinine concentration and/or microangiopathic hemolytic anemia. SRC occurs in approximately 20% of patients with systemic sclerosis who have diffuse cutaneous manifestations. In addition, 10% of reported cases of SRC with diffuse cutaneous involvement have normal blood pressures; in the majority of these cases, SRC occurs after treatment with corticosteroids. We describe a patient who presented with an early onset SRC in the setting of diffuse cutaneous systemic sclerosis in evolution without prior accelerated arterial hypertension or corticosteroid use.

Biopsy↗
PubMed1995

Anti-vascular endothelial cell antibodies (AECA): comparison of two assay methods and clinical applications.

Vascular endothelial cells may be a target for autoantibodies (AECAs) against membrane antigens that are constitutively expressed, induced or bound to their surface. To test this hypothesis, we used an enzyme-linked immunosorbent assay (ELISA) with two types of human endothelial cells as the substrate, i.e., human umbilical cord vein endothelial cells (HUVECs) or the hybrid cell line EAhy-926 obtained by fusion of HUVECs with the bronchial carcinoma cell line A549. A comparative functional study of these two cell types demonstrated that EAhy-926 cells produced only small amounts of VIII von Willebrand factor and tissular factor, did not contain Weibel Palade bodies visible under the electron microscope, and expressed ICAM-1 and selectin E in levels of no more than 15% of those expressed by human umbilical cord vein endothelial cells both after stimulation by bacterial lipopolysaccharide and under basal conditions. However, the two assay methods yielded similar IgG AECA titers when used on sera from patients with rheumatoid vasculitis or antiphospholipid syndrome. These antibodies did not exhibit cytotoxicity for cord vein or EAhy-926 cells. They were not specific for endothelium, since their activity decreased by a mean of 40% after incubation of sera with the epithelial cell line A549. A cross-sectional study of 565 sera demonstrated that anti-vascular IgG and IgM AECAs reactive with EAhy-926 cells occurred mainly in patients with dermatomyositis (IgG, 58%; IgM, 22%), systemic scleroderma (IgG, 48%; IgM, 18%), primary Sjögren's syndrome (IgG, 44%; IgM, 12%) and secondary and primary systemic vasculitides (IgG, 38%; IgM, 18%) including Wegener's granulomatosis. A longitudinal study in patients with Wegener's granulomatosis showed that AECAS were predictive of disease activity.

Antibodies↗
PubMed2001

Localized scleroderma or morphea?

Morphea is a frequently mild, benign, and self-limiting skin disease with a less than 1% reported chance of progressing to systemic scleroderma. Morphea is a sufficient and less terrifying name for these disorders than localized scleroderma.

Humans↗
PubMed2004

[Wegener's disease and exposure to silica. Study of the physiopathological mechanisms].

CRYSTALLINE SILICA: Crystalline silica is an abundant mineral found in multiple occupational exposures. It is a recognised occupational risk factor for some systemic auto-immune diseases such as progressive systemic scleroderma and Caplan-Colinet's syndrome, or just suspected in Goujerot-Sjögren's syndrome, periarteritis nodosa and systemic lupus erythematosus. WEGENER'S GRANULOMATOSIS: A rare disease of unknown of unknown aetiology, the disease is characterised by granulomatous vasculitis affecting the upper respiratory tracks, lungs and kidneys, associated with glomerulonephropathy. THE ROLE OF SILICA PARTICLES: Studies have shown that crystalline silica may be implied in Wegener's disease. Associated with other factors, notably with a genetic predisposal, silica particles might, through the release of reactive oxygen radicals and inflammatory cytokines, trigger-off immunopathological mechanisms. The latter would be manifested by exocytosis of the proteinase 3 and myeloperoxidase antigens on the surface of the polynuclear neutrophils.

Cytokines↗
PubMed1996

Specificity of antinuclear antibodies in scleroderma-like chronic graft-versus-host disease: clinical correlation and histocompatibility locus antigen association.

Chronic graft-versus-host disease after bone marrow transplantation presents, in a few cases, as mild to severe scleroderma-like changes. Patients with chronic graft-versus-host disease with and without sclerodermatous skin changes were analysed for antinuclear autoantibodies (ANA) and antinucleolar autoantibodies (ANoA) and the results correlated with disease symptoms and histocompatibility locus antigen (HLA) pattern. Nineteen patients with chronic graft-versus-host disease and scleroderma-like skin changes, 18 with chronic graft-versus-host disease without scleroderma, and 17 controls on immunosuppressive treatment were screened for ANA and ANoA using enzyme-linked immunosorbent assay, immunodiffusion and immunoblot techniques. Four patients with severe scleroderma had antibodies to topoisomerase I, two had antibodies against PM-Scl, both characteristic serological findings in idiopathic systemic scleroderma. One patient had La/SSB antibodies and, in three cases, antibodies to the nucleolar antigen C23 (nucleolin) could be identified. A possible correlation between antinucleolin antibodies and disease activity was observed. HLA-A1, -B1, and -B2 were found significantly more often in patients with scleroderma-like symptoms in comparison to patients without scleroderma-like symptoms. Chronic graft-versus-host disease with scleroderma-like manifestations can be associated with the occurrence of ANA specific for idiopathic scleroderma. The development of scleroderma after bone marrow transplantation might have a HLA-linked genetic background.

Antibodies, Antinuclear↗
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↗
PubMed1989

[Growth factors in the pathogenesis of progressive systemic sclerosis].

Scleroderma is a connective tissue disorder characterized by vascular lesions, fibrosis and inflammation. The pathogenesis of this disease is not clear. A vascular lesion, possibly caused by deposition of immune complexes or by release of cytotoxic factors, seems to be at the origin of the disease. As a consequence, platelet adhesion and activation might occur in sclerodermic patients. The observation that platelet might release, upon aggregation, a potent mitogenic factor, named Platelet Derived Growth Factor (PDGF) has focused interest on platelets as the potential mediators of the fibrotic process, characteristic of systemic scleroderma. We found an increased mitogenic activity in plasma derived serum (PDS) of a group of patients with progressive systemic sclerosis (PSS), as compared to control subjects. The activity was inhibited by incubation with anti-PDGF IgG's, suggesting that abnormal PDGF levels might indeed be present in plasma of PSS patients.

Adult↗
PubMed1976

Lymphocyte-epithelial cell interactions in oral mucosal inflammatory diseases.

Lymphocyte cytotoxicity for target cells is a method for evaluating specific lymphocyte stimulation. In vitro lymphocytotoxicity has been demonstrated in chronic ulcerative colitis and granulomatous colitis, polymyositis and dermatomyositis, systemic scleroderma, recurrent aphthous stomatitis, and periodontal disease. We have investigated lymphocytotoxicity in 36 patients with various oral inflammatory diseases using an automated cell-counting system. This investigation demonstrated in vitro lymphocytotoxicity for gingival epithelial target cells by aggressor lymphocytes harvested from patients with recurrent aphthous stomatitis and periodontal disease. This effect was not seen when the lymphocytes were harvested from normal subjects or from patients with other oral mucosal inflammatory diseases. The automated cell-counting system corresponded very well with the assay technique of exclusion of supravital dye.

Antigen-Antibody Reactions↗