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PubMed1991

HLA in systemic scleroderma (PSS) and familial scleroderma.

HLA in systemic scleroderma (PSS), including three familial cases, is reported. Three families in which one sister developed PSS and another sister suffered from either PSS (family 1), mixed connective tissue disease (MCTD) (family 2), or Sjögren's syndrome (SjS) (family 3) were described. The elder sister in family 1 died of respiratory insufficiency caused by scleroderma lung. The sisters in family 2 both had SjS, anti SS-A antibodies, and HLA A2-Bw55-Cw1-DRw8 haplotype in common. The elder sister with PSS in family 3 also had SjS and Hashimoto's thyroiditis. HLA in 28 PSS patients including these 3 familial cases were analyzed with 4 MCTD and 4 generalized morphea patients. HLA A2, Bw46, DR2, DRw8, DRw6 and DQw1 antigens were more frequently found in the PSS patients than in the controls. HLA DRw6 was the only antigen that was positive in common in the 3 familial cases. In those patients with anti topoisomerase I antibodies, HLA DR2 antigen was found more frequently than in the controls. Some, but not all, of these results were similar to the previous reports on HLA in PSS. Further investigations on more patients and the other members of these families would be necessary to clarify the significance of these results.

Adult↗
PubMed2006

[Mandibular resorption, an underdiagnosed manifestation of systemic scleroderma].

INTRODUCTION: Systemic sclerosis (SSc) is sometimes associated with bone resorption that can reach the mandible. CASES: We report here the cases of two women (aged 47 and 57 years) with SSc diagnosed 13 and 26 years earlier, respectively. Both presented marked mandibular bone resorption. The first had prominent interstitial lung disease, and the second, who died within a few months, had severe left ventricular dysfunction due to SSc. DISCUSSION: Mandibular resorption is a rare but probably underdiagnosed manifestation of SSc. In addition to its esthetic effects, it can cause severe disability.

Bone Resorption↗
PubMed1994

[Initial visceral involvement with diffuse systemic scleroderma].

Progressive systemic sclerosis (PSS) is characterized in most patients by initially unspecific symptoms like acrocyanosis. Raynaud's phenomenon, general malaise or acral paresthesia. Clinical manifestations appear with an edematous-inflammatory stage of disease leading to a chronic-progressive phase with increasing fibrosclerosis of the skin and internal organs. Dermal involvement and the demonstration of immunopathological abnormalities in the peripheral blood precede visceral fibrosis in most cases. We report on an unusual case of diffuse PSS with preceding involvement of internal organs. Early visceral fibrosis is demonstrated by histomorphological results and is correlated with laboratory findings. Serum antinuclear antibodies could not be obtained until 4 months ante finem and the liver was also involved in the fibrotic process.

Antibodies, Antinuclear↗
PubMed1992

[An analysis of the hormonal response during the performance of stress tests in patients with systemic lupus erythematosus and systemic scleroderma].

Patients with systemic lupus erythematosus (SLE), systemic scleroderma (SSD) and donors were examined for the blood levels of adrenocorticotropic hormone, hydrocortisone, follicle-stimulating hormone, luteinizing hormone, prolactin, estradiol, testosterone, progesterone, thyroid-stimulating hormone, triiodothyronine, thyroxin, and insulin. The corticotropin load test was carried out in 38 SLE patients, 32 SSD patients and 24 donors. The prednisolone test was made in 15 SSD patients and 27 donors. The studies were made with the aid of RIA. The patients with SLE manifested a decline of the basal level of hydrocortisone as well as a reduction of the reserve potentialities of the pituitary-adrenal system. The patients with SSD demonstrated a negligible decrease of the basal level of hydrocortisone with an evident lowering of the reserves of the same system. The treatment of SLE and SSD patients with glucocorticoids was followed by marked hyperinsulinemia.

Adrenocorticotropic Hormone↗
PubMed1988

Systemic scleroderma. Clinical and pathophysiologic aspects.

Systemic scleroderma is a generalized disease of connective tissue involving mainly the skin, the gastrointestinal tract, the lungs, the heart, and the kidneys. It can be present in different forms, of which acroscleroderma, with limited cutaneous and extracutaneous involvement, and diffuse scleroderma within a more rapid progression are most characteristic. Circulating antibodies against antinucleolar antigens are present in most patients with systemic scleroderma. They are helpful for establishing a classification and for determining the prognosis of the disease; their involvement in the pathogenesis, however, is still unclear. Alterations of the blood vessels and induction of fibroblasts by potent mediators are thought to play an important role in the early phase of scleroderma. Therefore early diagnosis is required, which then can initiate vasoactive therapy. In patients with systemic scleroderma, who also suffer from additional myositis, interstitial lung diseases, or arthritis, anti-inflammatory treatment with prednisolone and azathioprine is suggested. Development and progression of fibrosis cannot yet be influenced sufficiently. Only D-penicillamine affecting cross-linking of collagen has been widely used in scleroderma and has some beneficial effect.

Anti-Inflammatory Agents↗
PubMed1995

Lichen planus presenting with erythema-multiforme-like bullous lesions in a patient with systemic scleroderma.

A patient with systemic scleroderma is described who developed a cutaneous eruption of papules and vesicobullae. Over time, the latter turned into papules. The histopathological and immunofluorescence features of the papular lesions were unequivocally those of lichen planus, while those of the bullous lesions reminded of erythema multiforme. Histologically, erythema multiforme shares common features with lichenoid reactions, such as necrotic keratinocytes. Our case suggests that erythema multiforme and lichen planus may coexist or succeed each other as different stereotype immune reactions against the same antigen(s) within the epidermis.

Blister↗
PubMed1979

Morphaea-like cutaneous changes in a patient with systemic scleroderma.

A case of systemic scleroderma with peculiar morphaea-like lesions is reported. The patient at first started with typical acrosclerosis, some parts of which later became softened and apparently resumed the appearance of normal skin, so that morphaea-like cutaneous plaques which remained hard, were soft. These changes were surrounded by erythema resembling the 'lilac ring' as noted in characteristic active lesions of morphaea.

Female↗
PubMed1984

[Systemic scleroderma].

The etiology of systemic scleroderma is still unknown. This disease is well individualized by its usual forms, i.e. acrosclerosis and diffuse form, whose features and prognosis differ. The description of new entities has generated further interest, particularly through a different diagnostic and therapeutic approach. No specific biologic or immunologic criteria of the disease have as yet been discovered. Prognosis depends mainly upon the severity of visceral involvement. There have been little improvements in therapy which rests upon presumptive physiopathological hypotheses.

Bone Diseases↗
PubMed1985

Clinical significance of lymphocyte responsiveness to scleroderma-inducing glycosaminoglycan-associated antigen in systemic scleroderma.

Twenty-nine patients with systemic scleroderma (SSD), whose lymphocytes significantly responded to a scleroderma-inducing glycosaminoglycan-associated antigen (stimulation index greater than or equal to 1.7), were separated into the two groups of marked and moderate responses, according to the mitogenic response of their peripheral lymphocytes. A marked response, with a [3H]-thymidine uptake of +3 SD over the mean of unstimulated SSD lymphocytes, was seen in severely affected patients with a high OKT4/OKT8 ratio. In contrast, the lymphocytes of patients with benign disease responded moderately to the antigen, showing lower thymidine uptake by both unstimulated and stimulated lymphocytes. This latter group had a high frequency of elevated anti-nRNP antibody.

Adolescent↗
PubMed1998

Histopathologic differentiation between localized and systemic scleroderma.

It is widely accepted that a histopathologic differentiation between localized scleroderma (LS) and systemic scleroderma (SS) is not always possible. With the objective of identifying differentiating histopathological features between them, 32 cases of LS and 19 cases of SS were reviewed. Histological features such as the distribution, composition, and density of the inflammatory cell infiltrate, thickness of the dermis, alterations of dermal collagen, and the presence of calcification were evaluated in each case. On the basis of our observations, LS and SS can be differentiated usually by the distribution and density of the inflammatory infiltrate and by the involvement of the papillary dermis. Inflammatory changes are more prominent in LS than in SS. Sclerosis of the papillary dermis is frequently seen in LS but is absent in SS.

Adolescent↗
PubMed1996

[Pregnancy and systemic scleroderma. A case report].

The systemic scleroderma is an auto-immune affection characterised by diffuse lesions of the connective tissues with inflammatory predominance. As less frequent as this association with pregnancy, it usually occurs to the woman at the end of genital life activity. The obstetrical past history of a G5P2 of 40 years old affected with systemic scleroderma, is marked by four tardive abortions and two premature deliveries. The evolution of the disease was progressively unfavourable with visceral, digestive and pulmonary blows them sclerodermatomyositis. A review of literature reveals the interactions between this affection and pregnancy. The aggravation of the disease during pregnancy is possible: nephopatic scleroderma is then the most frequent complication. The gynaecological and obstetrical consequences are sterility, habitual abortions, intra-uterine growth retardation, intra-uterine foetal death, premature delivery and expulsion difficulties. The infant prognostic is specially related to the risk prematurity. The corticotherapy is the only acceptable treatment during pregnancy.

Adult↗
PubMed2001

[Radiofrequency ablation of recurrent monomorphic ventricular tachycardia in a patient with severe systemic scleroderma].

A case of progressive systemic scleroderma in a 33 year-old woman who was referred to our Arrhythmia Unit due to daily palpitations and dizziness is presented. The 24-hour Holter recording showed monomorphic ventricular tachycardia which lasted several minutes. Hemodynamic study showed dilated right chambers and right ventricular dysfunction, without pulmonary hypertension. Left ventricular angiography and coronary arteries were normal. During programmed electrical stimulation, two different ventricular tachycardia were induced and ablated with radiofrequency on the right ventricle. The patient remains free of recurrence of tachycardia after (10 months of follow up). Patients with progressive systemic scleroderma may present several different cardiac arrhythmias. Involvement of the right ventricle is particularly frequent as is the origin of ventricular tachycardia in this ventricle. Radiofrequency catheter ablation is safe and effective in the management of these patients.

Adult↗
PubMed1991

Unilateral enophthalmos in systemic scleroderma.

Atrophy of orbital fat is a reported complication of localized scleroderma but not of systemic scleroderma. Here we present a case of systemic scleroderma with unilateral enophthalmos. Orbital fat atrophy was the presumed cause on computerized tomography.

Adipose Tissue↗
PubMed1975

An approach to experimental scleroderma, using urinary glycosaminoglycans from patients with systemic scleroderma.

As a result of intraperitoneal injections in mice of crude glycosaminoglycans obtained from the urine of patients with systemic scleroderma, a fibrotic process similar to the intial changes of human scleroderma was frequently observed, especially in the skin and esophagus when the glycosaminoglycans contained a probable variant of heparan sulfate. Furthermore, the scleroderma-like change was also observed, to some extent, in the ultrastructure of collagen fibers and glycosaminoglycans of the skin.

Animals↗
PubMed1983

Vitiligolike macules in systemic scleroderma.

Changes of hyperpigmentation and depigmentation in systemic scleroderma have been known since the last century, but their relationship to the pathogenesis of the condition is poorly understood. Clinical, histologic, histochemical, immunopathologic, and electron microscopic studies of the depigmented lesions in seven patients with systemic scleroderma demonstrated changes similar to those of vitiligo, but with subtle differences. Immunologic mechanisms seem to be involved in the induction of depigmentation in both vitiligo and systemic scleroderma.

Adult↗
PubMed1996

Antiubiquitin antibody in localised and systemic scleroderma.

OBJECTIVE: To determine the presence of antiubiquitin antibody (AUbA) in localised scleroderma and systemic sclerosis, as it is frequently found in the sera of patients with systemic lupus erythematosus (SLE) and has also been shown to have a close relationship with antihistone antibodies that have an important role in scleroderma. METHODS: Serum samples from patients with localised scleroderma (n = 48) and systemic sclerosis (n = 52) were examined by enzyme linked immunosorbent assay. Twenty samples from patients with SLE, 20 from patients with dermatomyositis, and 30 samples from healthy individuals were used as controls. RESULTS: AUbA was demonstrated in 44% of patients with localised scleroderma and in 42% of those with systemic sclerosis. The presence of AUbA correlated with the presence of antihistone antibodies in both localised scleroderma and systemic sclerosis. CONCLUSIONS: AUbA is frequently present in patients with localised scleroderma and systemic sclerosis. Induction of AUbA is closely associated with that of antihistone antibodies, suggesting that ubiquitinated histone may be the target in autoimmune responses of these disorders.

Adolescent↗