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PubMed2003

Life with a rare chronic disease: the scleroderma experience.

BACKGROUND: Little is known about the experience of living with a rare disease and how people with rare diseases cope with not only the disease but also the reactions of others. Scleroderma is a rare chronic connective tissue disease that results in fibrotic changes involving all or some organs of the body. The two types of scleroderma are systemic scleroderma, which involves the skin and internal organs and is the more serious type, and local scleroderma, which attacks the skin and surrounding tissues. Some people with scleroderma have signs that are visible to outsiders, while others have invisible signs. Having this chronic condition and being different from the general population may subject people with scleroderma to stigmatization by others. AIM: The aim of this study was to understand, from the individual's perspective, the experience of living with scleroderma. METHOD: Focus group interviews were conducted with two groups of individuals with scleroderma. Because of the rarity of the disease and the illness of the participants, only two groups were held. The same questions were asked of both groups. A moderator and assistant guided the groups. FINDINGS: Data analysis revealed five themes: physical manifestations, disclosure/non-disclosure to others, living, being normal and facing the future. The data are discussed in light of participants' having visible signs, invisible signs and the rarity of their condition. For those with visible signs, disclosure was automatic. They were conscious of being different from others without scleroderma. Those with invisible signs managed their disease information in such a way as to minimize the stigma of being different. The rarity of the disease added the problem of others not understanding their difficulties. Those who disclosed their disease not only had to deal with the reactions of others, but faced the additional burden of having to explain their condition. CONCLUSION: Nurses may have little knowledge about scleroderma. It is possible that they, through their ignorance of such rare conditions, may stigmatize individuals. Through understanding about rare diseases will they be able to teach patients the skills necessary to help them cope with their symptoms, as well as the reactions of others to their diagnosis and appearance.

Adaptation, Psychological↗
PubMed1993

The development of systemic sclerosis (scleroderma) following augmentation mammoplasty.

We describe 3 patients developing systemic sclerosis (scleroderma) following breast augmentation with paraffin injections, and saline and silicone gel filled implants. Although previously considered inert, silicone appears capable of inducing human adjuvant disease including scleroderma in the susceptible individual. The occurrence of systemic sclerosis following the use of silicone implants however, appears to be uncommon.

Adult↗
PubMed1982

The articular manifestations of progressive systemic sclerosis (scleroderma).

The articular manifestations of progressive systemic sclerosis (PSS) were studied in 38 patients. Of these, 66% experienced joint pain and 61% had signs of joint inflammation. Limitation of joint movement was seen in 45%. Radiological abnormalities included periarticular osteoporosis (42%), joint space narrowing (34%), and erosions (40%). Erosive disease did not correlate with disease duration, presence of rheumatoid factor, antinuclear antibodies, distal tuft resorption, or the extent of the scleroderma skin changes. Calcinosis was seen more frequently in those patients with articular erosions (67%). Erosive osteoarthritis of the distal interphalangeal joints (7 patients) was associated with impaired finger flexion. Joint involvement in PSS occurs frequently and may resemble rheumatoid arthritis in the early stages but is less destructive. The occurrence of unrelated arthropathy, such as primary osteoarthritis, is not uncommon, and its differentiation from true PSS joint disease can be difficult.

Adolescent↗
PubMed2002

[Pathogenesis of scleroderma].

Systemic sclerosis is a systemic inflammatory disorder, affecting the skin and other organs, especially the lungs and the digestive tract. The pathogenic mechanisms of systemic sclerosis still remain unknown, although there is evidence that many parameters may play a role in the pathogenesis of the disease, including: vascular involvement, immunity impairment, and both environmental and genetic factors. To date, many authors support the hypothesis that, in patients with genetic predisposition, environmental stimuli (occupational factors and/or virus) may lead to vascular dysfunction, particularly, endothelial damage and immune activation, resulting in tissue fibrosis at late stages of the disorder.

Cardiovascular System↗
PubMed1980

Pulmonary cysts in progressive systemic sclerosis (scleroderma).

A young man with progressive systemic sclerosis (PSS) and pulmonary complaints exhibited enlarging lung cysts on chest x-ray. These rather uncommon findings in PSS result from alveolar dissolution and obstructive emphysema. Diffuse pulmonary fibrosis is basic to this process as well as to the more familiar pulmonary roentgen changes in PSS.

Adult↗
PubMed1997

Symptomatic spinal calcinosis in systemic sclerosis (scleroderma).

Two patients with diffuse cutaneous systemic sclerosis and spinal calcification, involving the lumbar spine in one and the cervical spine in the other, are described. Computed tomography-guided aspiration of the calcific masses was performed, and material aspirated from one patient was shown to be apatite, Ca5(PO4)3OH. One patient showed improvement following lumbar laminotomy, hemilaminectomy, and diskectomy.

Calcinosis↗
PubMed2001

Von Willebrand factor propeptide as a marker of disease activity in systemic sclerosis (scleroderma).

In 44 consecutive patients with systemic sclerosis (SSc), plasma concentrations of von Willebrand factor (vWf) were higher than those of the vWf propeptide, but the propeptide showed less variability within patient subgroups. Higher values of the propeptide were observed in patients with early pulmonary involvement. A closer correlation of the propeptide than of vWf to biochemical markers of activity was also evident. Our results suggest that the propeptide, despite a shorter circulating half-time and lower plasma concentrations than vWf, is more useful in the assessment of disease activity in SSc.

Adult↗
PubMed1997

Systemic sclerosis (scleroderma): an integrated challenge in rehabilitation.

Systemic sclerosis (SSc), a multisystem disease involving the microvascular system and the connective tissue, is considered one of the most difficult rheumatic diseases to treat. The natural history of the disease evolves from an edematous to a scleroatrophic phase following two different temporal patterns: acute or chronic. The former leads to early death, and the latter evolves slowly toward severe disability that deserves rehabilitative intervention. Despite the poor prognosis, recent improvements in diagnosis and treatment have led to longer patient survival, thus increasing the need to intervene against the development of tissue fibrosis and contractures by using appropriate integrated rehabilitation programs. This article does not review the medico-pharmacological management of visceral manifestations of the disease. Rather, it is divided into six parts, which include analyses of the changes in skin, joints and tendons, and muscle induced by SSc; examination of the existing literature on rehabilitation strategies and treatments; discussions of the pain and peripheral sensory-motor system involvement that are present to a greater or lesser extent in almost all patients and influence not only the duration and outcome of rehabilitation but also the patient's family, social life, and working ability; and consideration of ergonomic and occupational interventions. No controlled studies have been done on the few rehabilitation guidelines and specific protocols identified, so it must be emphasized that this article is a summary of opinions expressed in the literature and the authors' own findings. Particularly lacking are studies on such aspects as ergonomics, work intervention, or the management of sexual dysfunction. Experience gained in the rehabilitation of skin burns and other rheumatic diseases forms the basis for a logical approach to SSc patients.

Acute Disease↗
PubMed1996

Silica and progressive systemic sclerosis (scleroderma): evidence for workers' compensation policy.

The occurrence of several confirmed cases of progressive systemic sclerosis (scleroderma) among male miners prompted a request by a member of the provincial parliament (MPP) of Ontario that the Industrial Disease Standards Panel (IDSP) evaluate the evidence for an occupational connection. A number of publications in reputable peer-reviewed medical journals offer case-control evidence gathered over four decades on three continents showing a rather clear-cut relationship between occupational exposure to crystalline silica and scleroderma. This article summarizes the evidence for a causal relationship and describes the process by which the members of the panel, using the criteria developed by Sir Austin Bradford Hill as a guide, made a finding of probable connection, the term mandated by the Workers' Compensation Act of Ontario. It provides insight into the difficulties encountered by those setting occupational disease policy when scientific certainty is unobtainable.

Canada↗
PubMedDate not supplied

Ultrasound examination of skin thickness in patients with progressive systemic sclerosis (scleroderma).

A new ultrasound technique for measuring skin thickness in 40 patients with progressive systemic sclerosis (scleroderma) and in 10 healthy control subjects has been evaluated. High frequency (10 MHz) dynamic ultrasound examination was performed at the proximal and middle phalanges of both second fingers. Measurements of the control subjects on two separate occasions demonstrated a good reproducibility of the technique with a standard deviation of 0.06 mm. The skin was thicker in patients with scleroderma 3.3 +/- 0.7 mm (mean +/- SD) compared with control subjects 2.5 +/- 0.2 mm (p less than 0.001). No relation between skin thickness and duration of disease was observed. This method can be used to monitor changes in skin thickness over time.

Adult↗
PubMed1993

Features of systemic sclerosis (scleroderma) in an identical twin pair.

The aetiology of systemic sclerosis (scleroderma) is unknown but it is thought to have both genetic and environmental components. The familial incidence of the disease is very low and we have been able to find only one report of scleroderma in identical twins which was in the Russian literature. We report here on a set of identical twins and their mother who all had features of systemic sclerosis.

Adolescent↗
PubMed2002

Ulnar artery involvement in systemic sclerosis (scleroderma).

OBJECTIVE: Microvascular disease is one of the hallmarks of systemic sclerosis (SSc, scleroderma), but macrovascular involvement also exists in some patients. Patients with SSc may have severe Raynaud's phenomenon (RP) characterized by refractory digital ulcerations. We investigated if large artery involvement, that is, ulnar artery occlusion, has a role in the development of refractory digital ulcerations, and if both screening for this involvement and revascularization of the ulnar artery occlusive disease may improve digital ulcer healing. METHODS: A retrospective chart review was performed of 15 patients with SSc, all of whom had severe RP and digital ulceration, together with a positive Allen test and ulnar artery occlusive disease documented by angiography. RESULTS: Women outnumbered men 2:1, with limited disease predominating (7), 5 patients having diffuse cutaneous disease and 3 overlap syndromes. All patients had positive antinuclear antibody and capillary microscopy findings consistent with SSc. Antiphospholipid antibodies were present in 4 of 6 patients tested. Tobacco use was seen in 5 patients, only 2 of whom were current smokers. All patients failed conventional medical therapy (nitrates, calcium channel blockers, antiplatelet agents) for RP and digital ulceration. Only 1/8 patients improved with stellate ganglion block, and one patient had no improvement following digital sympathectomy. Eight patients underwent ulnar artery revascularization combined with digital sympathectomy, and 8 experienced dramatic improvement in RP and healing of digital ulcers. CONCLUSION: An Allen test should be performed routinely on all SSc patients with severe RP and refractory digital ulceration to investigate the possibility of ulnar artery occlusive disease. If suspected ulnar artery occlusion is confirmed by angiography or ultrasonography, ulnar artery revascularization with or without digital sympathectomy should be considered in patients who fail conventional medical therapy.

Antibodies, Antinuclear↗
PubMed2004

Bleomycin-induced scleroderma.

Systemic sclerosis is a connective tissue disease, which can be triggered by environmental factors. We report one such case of bleomycin-induced scleroderma.

Antibiotics, Antineoplastic↗
PubMed1989

Anti-Ku antibodies in connective tissue diseases. Report of three cases.

Three patients are described with clinical features of connective tissue diseases, namely, dermatomyositis, progressive systemic scleroderma, and systemic lupus erythematosus. In two patients the symptoms of disease overlapped. The rare anti-Ku antibody was found in all of them. Anti-Ku antibody characterizes those patients, with polymyositis-scleroderma overlap syndromes who have a good prognosis. One of our patients, who also had severe anti-Sm-positive systemic lupus erythematosus, seems to be an exception.

Adult↗
PubMed2006

A case-control study of the pathology of oesophageal disease in systemic sclerosis (scleroderma).

BACKGROUND: Atrophy of the smooth muscle layers of the muscularis propria characterises oesophageal involvement in systemic sclerosis (scleroderma). The aetiology of this atrophy and of the resultant oesophageal dysfunction is unknown. OBJECTIVES: To examine oesophageal tissue for evidence of fibrosis, vascular disease, inflammatory reactions and neural abnormalities to determine the possible causes of this disease process. METHODS: A case-control survey was conducted using oesophageal tissue from 74 scleroderma cases and 74 age, race and sex-matched controls from our autopsy files. Histological evidence of oesophageal muscle atrophy was correlated with the degree of vascular changes, inflammatory infiltration, fibrosis, abnormalities of the myenteric plexus and reduction of interstitial cells of Cajal (ICC) using a predesigned semiquantitative descriptive method. RESULTS: Smooth-muscle atrophy was found in 94% of scleroderma cases, and in 5% of controls (p<0.001). Atrophy was evident in the circular smooth muscle in 93% of cases, and in the longitudinal smooth muscle in 66% of cases. Intimal proliferation of arterioles was found in 38% of cases and in 5% of controls (p<0.001), but was not associated with smooth-muscle atrophy (p = 0.29). Despite these vascular changes, there was no evidence of compromised perfusion, such as findings suggestive of acute ischaemic necroses. Minimal cellular infiltrates were seen in the myenteric plexus in 82% of cases and in 92% of controls (p = 0.091). ICC were found in fewer numbers in areas of atrophic smooth muscle compared with adjacent normal smooth muscle in selected scleroderma cases. CONCLUSION: The pathological findings of oesophageal lesions in scleroderma seem inconsistent with either an ischaemic or an inflammatory process. The loss of circular and longitudinal smooth muscle in the distal scleroderma oesophagus may represent loss of normal neural function followed by secondary tissue atrophy, or may be a primary smooth muscle lesion.

Adolescent↗