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PubMed2003

[A case of pseudoscleroderma as paraneoplastic syndrome due to carcinoma of cervical uteri].

A 65-year-old female began experiencing arthralgia, morning stiffness and psychroesthesia in April 2000. She visited a rheumatologist and was suspected of having early-stage rheumatoid arthritis. She was referred to our hospital, and was admitted in December 2000. At that time, sclerosis of the skin was advanced, and Raynaud's phenomenon was confirmed. The patient also exhibited nailfold bleeding, and was diagnosed as having systemic scleroderma. She visited a gynecologist for screening and cervical uteri carcinoma was confirmed. She underwent surgery in March 2001, and subsequently, sclerosis in her skin was almost stopped. We believe that these clinical symptoms were related to paraneoplastic syndrome. We therefore immunochemically investigated the pathogenesis of paraneoplastic syndrome and found that connective tissue growth factor (CTGF) might be involved and transforming growth factor-beta (TGF-beta) might not be involved in this case.

Aged↗
PubMed1994

Spinal cord stimulation for the treatment of progressive systemic sclerosis and Raynaud's syndrome.

Progressive Systemic Scleroderma (PSS) is a generalized disease of connective tissue involving the skin, as well as other internal organs. The cutaneous signs are characterized by a progressive sclerosis and loss of function or dexterity in the hands. Between 1987 and 1992, 15 patients affected by scleroderma were treated by means of spinal cord stimulation (SCS) in order to reduce signs and symptoms related to vascular damage. The follow-up ranged from 12 months to 6 years. The study confirms that SCS is an effective therapy in patients with PSS and Raynaud's phenomenon because of its beneficial effects on the Raynaud episodes, ulcers, pain, vascular sclerosis and hand function. This method may have a primary role in the treatment of this chronic disorder because of the high probability of failure of other medical or surgical therapy.

Adult↗
PubMed1999

De novo systemic sclerosis after radiotherapy: a report of 3 cases.

We describe 3 patients in whom the onset of systemic scleroderma occurred shortly after ionizing irradiation for nasopharyngeal or breast carcinoma. This relationship has been described rarely as has the exacerbation of a preexisting scleroderma after irradiation. This gives indications for direction of studies.

Adult↗
PubMed2000

[Evaluation of early cardiovascular involvement in patients with systemic sclerosis].

UNLABELLED: The aim of the study was to evaluate cardiac function in patients with systemic sclerosis by means of noninvasive methods in order to detect early dysfunction of cardiovascular system. MATERIAL AND METHODS: We studied a group of 19 patients (15 women, 4 men, aged 17-74 yrs, av. 51 +/- 11) with systemic sclerosis comparing the results with a group of 23 healthy volunteers (17 women, 6 men aged 21-69 yrs, av 53 +/- 15). All the patients with SSc were taking corticosteroids, immunosuppressants or vasodilators at the time of the study. In all the patients we performed 24-hour Holter monitoring for the evaluation of arrhythmias, conduction disturbances, ischaemia, heart rate variability (HRV) and late potentials (LP). The following parameters of HRV in time domain were analyzed: SDNN, SDANN, SDNNI, rMSSD, pNN50. Standard ECG was performed to assess QT interval (QT, QTc, QTd). In all the patients the echocardiography examination was performed (M-Mode, 2-D, Doppler echocardiography). The morphology of heart structures and haemodynamic function were analyzed. RESULTS: In patients with SSc Holter monitoring revealed tendency to tachycardia. The mean heart rate was 81 +/- 11 vs. 71 +/- 9 in controls. Conduction disturbances were observed in 3 pts. In 6 pts we found significant ventricular arrhythmia. Silent ischaemia episodes were detected in 6 pts. Concerning HRV analysis the significantly lower values were detected in pts with SSc vs. controls: SDNN 123 vs 170; SDNNI 51 vs 76; SDANN 110 vs 152; rMSSD 29.6 vs 54; pNN50 6.1 vs 21. Late potentials were present in one patient with SSc vs none in the control group. The mean values QT-371, QTc-419, QTd-40- did not exceed the ranges of normal values. No signs of systolic cardiac dysfunction were detected, while in 6 pts we recognized left ventricle diastolic dysfunction. Valvular lesions were observed in 8 pts, but only in 2 pts they were hemodynamically important. CONCLUSIONS: 24-hour Holter monitoring and ECHO examination are valuable methods, which allow to detect early dysfunction of cardiovascular system in patients with systemic scleroderma presenting no apparent cardiac impairment symptoms.

Adolescent↗
PubMed2001

[A patient with dermatomyositis and systemic sclerosis with preferential facioscapulohumeral muscle involvement and fatal cardiomyopathy].

We report a 23-year-old man suffering from an overlap syndrome of systemic scleroderma and dermatomyositis who died from severe dilated cardiomyopathy. Because his weakness involved predominantly muscles in the facio-scapulo-humeral regions, he was initially thought to have facioscapulohumeral muscular dystrophy (FSHD) at other hospitals. However, he had also Raynaud phenomenon and low voltages on electrocardiogram. His apparent facial weakness was mainly due to atrophic skin changes. Unlike FSHD, the deltoid and levator scapulae muscles were also atrophic. Deltoid muscle biopsy performed one year earlier at another hospital showed mild myopathic changes without inflammation, but there were scattered thick-walled endomysial capillaries, suggesting inflammatory myopathy. Biceps brachii muscle biopsy in our hospital showed marked inflammation with perifascicular atrophy. In this patient, the cardiac muscle involvement progressed together with the skeletal muscle inflammation before scleroderma became apparent.

Adult↗
PubMed1992

[Infection of ascitic fluid by perforation of a sclerodermic colon].

In progressive systemic scleroderma, a disease of protean manifestations, involvement of the gastrointestinal tract is noted infrequently in patients who are asymptomatic, and is rarely fatal. We report the case of a 65-year-old woman who was admitted for ascitis related to a non A, non B cirrhosis. The patient presented with bacterial peritonitis due to colonic perforation by fecal impaction associated with sclerodermic involvement. Pathologic study of the resected colon showed that the true muscularis was very atrophic and fibrotic, characteristic of scleroderma. To our knowledge, this cause of ascitic fluid infection has not been previously reported.

Aged↗
PubMed1992

Immunologic abnormalities associated with primary anetoderma.

BACKGROUND AND DESIGN: Primary anetoderma is a rare cutaneous elastolytic disorder, the etiopathogenesis of which has not yet been established. Six patients with primary anetoderma were studied in an attempt to assess the role of the immunologic system in the elastolytic process. The investigation included the medical history, physical examination, routine blood tests, specific tests for collagen diseases, prothrombin time, activated partial thromboplastin time, thyroxine, indirect immunofluorescence test, and skin biopsies for histopathologic study and direct immunofluorescence. RESULTS: Two of the patients presented with autoimmune disorders: the first had Graves' disease, lupus anticoagulant, and autoimmune hemolysis, and the second had systemic scleroderma. There were positive direct immunofluorescence findings in most of the patients. Furthermore, all of them were found to have serologic immunologic abnormalities, of which the most common was a positive antinuclear factor. CONCLUSIONS: These findings indicate that there is an immunologic involvement in primary anetoderma.

Adult↗
PubMed1991

Neurogenic inflammation induced by capsaicin in patients with psoriasis.

Increasing doses of capsaicin were applied topically to the forearm skin of 30 patients with psoriasis, 16 patients with systemic scleroderma and 16 healthy volunteers. Only one-third of the patients with psoriasis responded with neurogenic inflammation to capsaicin doses of 0.125 and 0.25 microgram/cm2 in contrast to 81% of scleroderma patients and all the normal controls, who showed a positive cutaneous reaction. Higher doses of capsaicin (0.5-4 micrograms/cm2) were required to induce erythema and flare in patients with late-onset psoriasis (after 21 years of age) as well as in patients with more than 40% of skin surface involved with psoriatic lesion.

Administration, Cutaneous↗
PubMed1985

[Changes in the functional state of the cardiovascular system as an indicator of the general reaction of the body to the application of therapeutic mud].

Multimodality clinical and physiological studies on the CVS in patients with rheumatoid arthritis (56), deforming osteoarthrosis (97) and systemic scleroderma (29) before and after various mud application procedures during the entire course of treatment showed that the CVS sensitively responded to the mud effect. As to the nature and expression of reactions peloid applications produced a general effect on the body; the most informative reactions were hemodynamic effects, changes of indices of intervalograms and the capillary blood flow. The hemodynamic effect was of a hypodynamic type of the CVS adaptation response to mud therapy. The author proposed that these indices should be used as tests for working out methods of control of the process of mud therapy and for other practical problems of pelotherapy.

Adolescent↗
PubMedDate not supplied

Microvascular abnormalities as possible predictors of disease subsets in Raynaud phenomenon and early connective tissue disease.

In vivo capillary examination was performed on 80 patients included in a prospective study of Raynaud phenomenon (RP): 40 RP only (RPO), 20 undifferentiated connective tissue disease (UCTD) and 20 systemic scleroderma (SD) of less than 5 years' duration. On initial examination, SD-pattern capillary abnormalities were found in 7/40 patients with RP alone, 18/20 with UCTD and 19/20 with SD. On follow-up a change of diagnosis into a definite connective tissue disease occurred in 4 patients with UCTD; one patient with RP alone died with symptoms of myositis and fulminant pulmonary vasculitis. All five showed SD-pattern capillary abnormalities of the "active" type on initial examination. A significant correlation between the "slow" capillary pattern and the presence of anticentromere antibodies was also observed. Only one patient of 25 RPO with normal capillaries available for follow-up showed any change in clinical status.

Antibodies, Antinuclear↗
PubMed1984

Paroxysmal dysarthria and Raynaud's phenomenon in the tongue.

Three patients suffering from systemic scleroderma and Raynaud's phenomenon in the digits as well as the tongue are reported. Following exposure to cold, a vasospasm was observed in the digits and the tongue accompanied by severe dysarthria. These striking oral symptoms had been overlooked for years in the medical ward. It is recommended to question all patients with Raynaud's phenomenon about visceral manifestations during the digital attacks.

Aged↗
PubMed1983

[Morphology and pathogenesis of penicillamine-induced nephropathy].

Histological, immunohistological and electron microscopical investigations on the glomerular renal lesions in penicillamine-induced nephropathy were carried out by studying biopsy material of eight patients with rheumatoid arthritis (4 cases), allergic alveolitis (3 cases) and systemic scleroderma (1 case) under the treatment with D-penicillamine. Summarizing all the findings, the characteristic pattern of immune complex nephritis as membranous type glomerulonephritis on the one hand and proliferative type glomerulonephritis (mesangioproliferative GN, focal proliferative GN or minimal proliferative GN) on the other hand could be demonstrated. The different types of glomerular alterations in penicillamine nephropathy obviously depend on the kind and duration of the primary main disease and on the individual immune reactivity of the body was a whole by this determined. The possible pathogenesis related to definite effects of the D-penicillamine is discussed and compared with the communications in the literature.

Adult↗
PubMedDate not supplied

Surgical aspects of multifocal involvement of the gastrointestinal tract in progressive systemic sclerosis.

An operative approach to the problem of progressive systemic scleroderma, affecting both the esophagus and duodenum, is demonstrated in the case of a 38-year-old woman severely incapacitated by protracted vomiting. Nissen's fundoplication, antrectomy and Billroth II gastroenterostomy were performed. The need for a comprehensive balanced surgical therapeutic approach in patients suffering from scleroderma is emphasized.

Adult↗
PubMed1996

Pregnancy outcome in systemic sclerosis.

The outcome of pregnancy in systemic scleroderma (SSc) has been a subject of much interest. In a retrospective analysis of 59 pregnancies of 17 mothers with SSc, the effect of disease on pregnancy outcome was variable. There was no adverse effect on fertility. While there was an apparent decrease in fertility in the limited cutaneous disease, this was not seen in the diffuse variety. There was an decreased incidence of live birth after the onset of disease as compared prior to the onset of disease. The foetal wastage after the onset of disease was contributed equally by spontaneous abortions as well as induced terminations of pregnancies. The presence of anticardiolipin antibodies was not related with adverse outcome. The effect of pregnancy on disease was variable with the disease being stable in five patients, regressing in five and progressing in three. The increased terminations of pregnancy reflects the uncertainities of positive outcome both by parent and attending physicians.

Adolescent↗
PubMed1983

D-penicillamine treatment of progressive systemic sclerosis (scleroderma): a comparison of clinical and in vitro effects.

Punch biopsies of skin were obtained from the forearms of 3 patients with progressive systemic sclerosis with diffuse scleroderma before and after treatment for 1 year or more with D-penicillamine. While on therapy, each patient studied had demonstrated a marked reduction in skin thickening. Collagenase-sensitive protein and glycosaminoglycan accumulation were measured in fibroblast cultures derived from these biopsies. No differences were observed pre- and post-treatment. We conclude that although D-penicillamine may exert its effect in vivo on connective tissue synthesis, maturation and/or turnover, fibroblasts remaining in the thinned dermis retain their potential for increased synthesis of connective tissue.

Adult↗
PubMed1977

Oral radiographic changes in patients with progressive systemic sclerosis (scleroderma).

Intraoral and panoramic radiographs were made of 35 patients with progressive systemic sclerosis. Of those patients, 13 (37%) were found to have abnormally thickened periodontal ligament spaces. Six of the 35 demonstrated mild to significant resorption of the posterior mandibular angle. Three patients, including two showing angle resorption also had some degree of coronoid process destruction. All patients with this disease should receive initial and follow-up panoramic radiographic examinations to determine any osseous changes.

Adult↗
PubMed1995

Altered collagen and glycosaminoglycan syntheses in familial scleroderma skin fibroblasts.

BACKGROUND: The main clinical symptoms of systemic scleroderma are caused by an accumulation of collagen in the tissues. Familial scleroderma is a very rare disorder in which systemic scleroderma occurs in the members of the same family. OBJECTIVE: To know whether or not the biosynthetic properties of familial scleroderma skin fibroblasts are similar to those of nonfamilial scleroderma fibroblasts, syntheses of extracellular matrix components in the two fibroblast strains obtained from familial scleroderma patients were studied. METHODS: Expression of collagen and glycosaminoglycans by cultured familial scleroderma fibroblasts was determined. RESULTS: Collagen synthesis by patient fibroblasts was elevated 1.4-fold compared with control fibroblasts. Translational activity of type I procollagen mRNA and the level of alpha 1 (I) mRNA in the patient fibroblasts was increased 1.7- and 1.6-fold, respectively. The ratio of hyaluronic acid/dermatan sulfate in the patient fibroblasts was lower than that in control fibroblasts. CONCLUSION: The results indicate that the biosynthetic phenotypes of familial scleroderma fibroblasts are similar to those of nonfamilial scleroderma fibroblasts which have already been reported.

Adult↗