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PubMed2006

Pseudoscleroderma associated with cancer.

In the period 1994-2002, we saw 71 patients with sclerotic skin changes; 66 were diagnosed with systemic sclerosis (SSc) while five (7%) were diagnosed with pseudoscleroderma associated with various malignancies. The mean duration of disease in these five patients was significantly shorter than that of patients with SSc. The incidence of positive antinuclear antibodies, Raynaud's phenomenon or oesophageal involvement in patients with pseudoscleroderma was significantly lower than that in patients with SSc. The distributions of skin sclerosis varied in each case. Serum basic fibroblast growth factor (bFGF) levels in the five patients with pseudoscleroderma were very elevated compared with levels in controls. Elevated expression of bFGF was detected on fibroblasts of affected skin and in one lung-cancer tissue sample obtained by excision.

Adenocarcinoma↗
PubMed1990

Autoantibodies to mitochondrial and centromere antigens in primary biliary cirrhosis and systemic sclerosis.

High levels of IgG antibodies to the multi-enzyme complex bovine pyruvate dehydrogenase (PDHC) were detected by a sensitive ELISA in all patients with histologically proven primary biliary cirrhosis (PBC), in a minority of patients with systemic sclerosis without overt PBC or with chronic liver diseases of another cause and in no rheumatoid arthritis or normal controls. Most (15/21) but not all of these positive patients recognized a 70-kD (E2) component of PDHC on immunoblotting. Additional recognition of a centromere-associated 140-kD polypeptide was associated with the presence of scleroderma-like features (Raynaud's phenomenon and sclerodactyly) in PBC, further emphasizing the serological and clinical overlap between these two conditions.

Autoantibodies↗
PubMed1991

Fc gamma RII restriction fragment length polymorphism (RFLP): analysis in systemic lupus erythematosus and scleroderma and evidence of an alpha gene duplication.

The characteristic finding of high levels of circulating immune complexes in patients with the autoimmune connective tissue diseases systemic lupus erythematosus (SLE) or scleroderma has raised the possibility that these patients may have a primary defect in immune complex clearance. The Fc receptor for IgG (Fc gamma R) plays a central role in the phagocytosis of antibody complexes. We have analysed Fc gamma R (type II) RFLPs identified in TaqI- and MspI-restricted genomic DNA and found that their distribution in SLE and scleroderma did not differ significantly from controls. Hybridization with specific regions of the Fc gamma RII cDNA clone indicate that part of the Fc gamma RII alpha locus is duplicated in some individuals. A further Fc gamma RII gene has recently been identified (Fc gamma RII alpha'). This gene shows greater than 95% homology with Fc gamma RII alpha and may thus be the candidate gene for the apparent alpha duplication seen in some individuals. It is possible that an individual may possess one, two, three or four TaqI Fc gamma RII alpha/alpha' alleles, correlating with incidence and numerical heterogeneity in Fc gamma RII alpha and alpha'. The physiological effects of this numerical heterogeneity remain to be investigated.

Antigens, Differentiation↗
PubMed1989

Association of HLA antigens with progressive systemic sclerosis and morphea.

The HLA phenotype frequencies of 40 patients with progressive systemic sclerosis (PSS) and 42 patients with morphea (sclerodermia circumscripta) indicate a weak, HLA-linked genetic predisposition of the affected individuals (70 female, 12 male). An "opposite" HLA A1, B8 (high immune response) vs A3, B7, DR2 (low immune response) constellation in PSS and morphea supports the clinical subdivision of scleroderma into these different nosological entities, and may serve as a differential diagnostic tool and prognostic marker.

Female↗
PubMed2006

Studies of scleroderma at the Alfred Hospital, Melbourne.

Scleroderma had been virtually unrecognized in this country before this study. Our interest in this condition was raised by the discovery that certain patients being investigated for ischaemic disease of the hand had scleroderma. Although uncommon, it is not excessively rare and we have been able to study an increasingly large number of patients, eventually resulting in 177 patients over a period of 35 years. The clinical features in these patients have been delineated. At first, the patients were subdivided into types: type 1, skin changes obvious only in the hands; type 2, skin changes extending beyond the hands but excluding the trunk; type 3, skin changes diffuse and involving the trunk. All types have similar visceral changes, but these are more severe and there is a worse prognosis in type 3 patients. Types 1 and 2 can conveniently be combined as acrosclerosis. Types 1 and 2 have a similar and good prognosis with survival at 30 years of 40%. Type 3 patients have a much worse prognosis, with no type 3 patients living more than 20 years. All types have a high incidence of autoantibodies, but these are generally not related to the severity of the disease and do not occur in relatives or spouses, this being the evidence of the absence of hereditary and environmental factors in their presence. Although patients may receive much relief from symptomatic measures, no treatment had lessened the skin stiffness and there is no specific treatment for the visceral lesions. The cause of the condition remains unknown.

Female↗
PubMedDate not supplied

Breast cancer and scleroderma.

INTRODUCTION: Epidemiologic studies have shown that patients with scleroderma have an increased risk of cancer. METHODS: The authors report eight cases and retrospectively review the published data on the possible association between breast cancer and scleroderma of the cutaneous and systemic forms. The records of all patients with scleroderma and breast cancer who were hospitalized during 1980-2002 in the Chaim Sheba Medical Center were surveyed. A literature search for breast cancer and scleroderma was performed. RESULTS: The clinical data of 65 women patients with breast cancer and scleroderma, eight of whom were hospitalized in the Chaim Sheba Medical Center and 57 who were reported in the literature, are summarized. In 29 patients (44%), scleroderma appeared before or simultaneously with the diagnosis of breast cancer. While a higher frequency of systemic scleroderma was described in this group, a higher rate of cutaneous scleroderma appeared in the group where scleroderma followed breast cancer diagnosis (p<0.001). CONCLUSIONS: Large-scale case-control studies are needed to substantiate the possible association between scleroderma-both cutaneous and systemic-and breast cancer.

Adult↗
PubMed1998

Gastrointestinal hemorrhage in patients with systemic sclerosis and CREST syndrome.

OBJECTIVES: Systemic sclerosis (SSc) and calcinosis, Raynaud's phenomenon, esophageal disease, sclerodactyly, telangiectasia (CREST) syndrome present distinctive microvasculature lesions that are thought to be responsible for tissue damage and disease progression. Involvement of the gastrointestinal tract may lead to the occurrence of profuse hemorrhage. We performed a study to assess the incidence and characteristics of gastrointestinal hemorrhage in a large group of patients with SSc and CREST syndrome. METHODS: We reviewed the medical records of 144 patients with SSc/CREST seen at our institution during the period 1985-1996. Endoscopic findings and clinical data were correlated. Data are expressed as means +/- SD. RESULTS: Twenty-two of 144 (15.2%) patients had at least one episode of gastrointestinal hemorrhage (16 women, 6 men; mean age, 59.4 +/- 17.6 yr). Eight patients (8/22; 36%) had multiple episodes and four (4/22; 18%) required chronic transfusion therapy. Mucosal telangiectasias were the most common cause of bleeding (9/22; 40.9%), followed by peptic ulcer disease (7/22; 31.8%) and erosive gastritis (3/22; 13.6%). Bleeding telangiectasias occurred in the entire gastrointestinal tract, including oral cavity (n = 1), esophagus (n = 1), stomach (n = 3), duodenum (n = 1), ileum (n = 1), cecum (n = 2), and colon (n = 2). Mortality was 22.7% in patients with gastrointestinal bleeding, compared with 7.3% in patients without bleeding. CONCLUSIONS: Patients with SSc/CREST syndrome are at risk of developing severe gastrointestinal hemorrhage. This complication is associated with frequent hospitalization, blood transfusions, and increased mortality. Mucosal telangiectasias are the most common source of bleeding. Appropriate endoscopic intervention is recommended in evaluating and preventing bleeding in patients with SSc/CREST.

Adolescent↗
PubMed1993

Central nervous system involvement and psychiatric manifestations in systemic sclerosis (scleroderma): clinical and neurophysiological evaluation.

We evaluated central nervous system and psychiatric involvement in a clinical sample of 32 patients with systemic sclerosis (SSc) (scleroderma). All patients underwent clinical neurological examination. Electroencephalography (EEG) and visual evoked potentials (VEPs) were also recorded. Prominent central nervous system (CNS) or psychiatric symptoms were present in 5 patients (16%), including encephalopathy, psychosis, anxiety disorder, grand mal seizures and transient ischemic attack. In addition, abnormal VEPs were recorded from 5/32 patients (16%), suggesting optic neuropathy. EEGs were mainly normal or showed only slight, nonspecific changes. Primary CNS involvement in scleroderma, however, could not be shown in any of the 5 cases with neuropsychiatric symptoms. Our results suggest that neuropsychiatric symptoms in SSc are, if not coincidental, indirectly caused by internal organ involvement of SSc or by possible overlapping connective tissue diseases. On the other hand, optic neuropathy might be a primary complication of SSc.

Adult↗