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PubMed1991

Predictors of survival in systemic sclerosis (scleroderma).

We conducted followup of 264 patients with definite systemic sclerosis (SSc) who were entered into the multicenter Scleroderma Criteria Cooperative Study (SCCS) during 1973-1977. At the end of the study (average 5.2 years of followup), 38% were known to be alive, 50% were dead (68% of these deaths definitely related to SSc), and 12% were lost to followup. Survival analyses of 484 demographic, clinical, and laboratory items recorded at entry into the SCCS (within 2 years of physician diagnosis of SSc) were performed. Survival declined linearly, and the cumulative survival rate was less than 80% at 2 years, 50% at 8.5 years, and 30% at 12 years after entry. Analysis using combinations of entry variables identifying organ system involvement confirmed that renal, cardiac, pulmonary, and gastrointestinal involvement in SSc predicted reduced survival; however, data on organ system involvement at study entry could not be used to consistently predict which organ system would ultimately be involved as the primary cause of death. By survival tree analysis, the individual entry variables best predicting reduced survival included older age (greater than 64 years), reduced renal function (blood urea nitrogen greater than 16 mg/dl), anemia (hemoglobin less than or equal to 11 gm/dl), reduced pulmonary diffusing capacity for carbon monoxide (less than or equal to 50% of predicted), reduced total serum protein level (less than or equal to 6 gm/dl), and reduced pulmonary reserve (forced vital capacity less than 80% with hemoglobin greater than 14 gm/dl or forced vital capacity less than 65% with hemoglobin less than or equal to 14 gm/dl). Cox proportional hazards model analysis confirmed these results. Different combinations of variables led to markedly different survival rates. The poorest prospects for survival were in patients with SSc who were less than or equal to 64 years old with a hemoglobin level less than or equal to 11 gm/dl, and in those greater than 64 years old with a blood urea nitrogen level greater than 16 mg/dl. These results may be useful in predicting individual patients at risk for shortened survival.

Age Factors↗
PubMed1978

Some observations on the immunological status in scleroderma (progressive systemic sclerosis).

A variety of tests were performed to determine the immune status of a group of patients with scleroderma (progressive systemic sclerosis). Sera of 11 of 35 patients showed autoantibodies by an immunofluorescent technique. There was no significant difference between the C3 and C4 components of complement in 12 patients tested and laboratory controls. IgM levels were raised in 24 of 34 sera. Four of ten patients showed some deficiency of phagocytic function in the leucocyte iodination test. B cell and T cell numbers were normal in the eight patients tested. Reactivity to mitogens was usually normal in ten patients tested with a deficient response to one of the three mitogens in two patients. In skin reactivity tests in 23 patients, only one was anergic to all of the four antigens tested. It is concluded that although some immune abnormalities are common in scleroderma there is no consistent abnormality in any of the tests used. This does not exclude the possibility that immune factors may be involved in systemic manifestations of the disease, or that there may be some subtle specific abnormality not revealed by the tests performed.

Adult↗
PubMed1975

Peripheral blood lymphocyte distribution in scleroderma.

Determination in peripheral blood T and B lymphocytes performed in progressive systemic scleroderma (8 patients); dermatomyositis (5 patients); and inflammatory or mesenchymal scleroderma ('mixed' connective tissue disease) (5 patients) demonstrated a marked increase in peripheral B cells with T cell depression in patients with mesenchymal scleroderma. Patients with progressive systemic scleroderma and dermatomyositis demonstrated also a peripheral T depression, but no consistent changes in B cell population were found. The clear relationship between a B lymphocyte increase and elevated immunoglobulins, antinuclear and antiribonucleoprotein antibody and positive direct immunofluorescence of skin (basal membrane and blood vessels) defines inflammatory or mesenchymal scleroderma in which a disturbance in humoral immunity is involved. B lymphocyte determination can help to measure these changes in immunoreactivity.

Adult↗
PubMed2000

Systemic sclerosis (scleroderma) and related disorders: clinical aspects.

To adequately evaluate and manage patients with systemic sclerosis, practitioners and patients alike must have a working knowledge of the natural history of the disease. What we know generally about the natural history should be coupled with four pieces of patient specific clinical information. (i) Does the patient have systemic sclerosis? (ii) How long has the patient had systemic sclerosis? (iii) How extensive was/is the sclerodermatous skin induration/ thickening at its worst? (iv) Is there presently evidence of visceral involvement (heart, lung, kidney, gastrointestinal, musculoskeletal)? This chapter will show you how to use this information to construct (for you and the patient) a picture of where that patient is presently in the natural history, where the patient is likely to be in the future (in general terms), what visceral involvements are likely or unlikely to occur, what evaluations are needed now and in the future, and what treatments are recommended and why.

Gastrointestinal Diseases↗
PubMed1983

Selective atrophy of the type IIb muscle fibers in rheumatoid arthritis and progressive systemic sclerosis (scleroderma). A biopsy histochemical study.

We have examined biopsy material from the left m. vastus lateralis of eight patients suffering from rheumatoid arthritis and four patients with progressive systemic sclerosis (scleroderma). All were chosen according to the duration and the severity of disease, so that the broadest possible spectrum of signs and symptoms could be considered. Muscular specimens showed a selective and constant atrophy of the type IIB fibers, as revealed by the myofibrillar ATPase histochemical reaction (both at a pH of 9.4 and with pre-incubation at pH 4.35 and 4.63). Atrophy of the type I fibers was seen only occasionally. Neither structural abnormalities, such as 'motheaten' fibers, nor inflammatory reactions were observed. We think that (1) changes in skeletal muscles of patients with rheumatoid arthritis and progressive systemic sclerosis may be quite selective, and (2) the myopathy associated with rheumatoid arthritis can be differentiated from inflammatory myopathies, even in muscle biopsy specimens, on the basis of histoenzymologic investigations.

Adult↗
PubMed1986

[Autoimmune changes in various rheumatic diseases].

A combined clinicoimmunologic investigation of 895 patients was conducted, of them 518 had rheumatic fever, 68 rheumatoid arthritis, 35 systemic scleroderma and 12 systemic lupus erythematosus. The immune status of the patients was evaluated by the results of the rosette-formation method, the reaction of lymphocyte blasttransformation, the reaction of leucocyte migration inhibition using purified cardial and other tissue antigens. The weakening of rosette-forming function of lymphocytes, a decrease in a mitogenic response to PHA, dysimmunoglobulinemia, imbalance in antibody production, particularly hyperproduction of cardial antibodies in rheumatic fever were observed as was marked delayed-type hypersensitivity to tissue antigens, more frequently to purified cardial antigens--to myocardial cell membranes and myosin. It indicates the involvement of the autoimmune mechanisms in the development of the disease.

Arthritis, Rheumatoid↗
PubMed2004

High plasminogen activator inhibitor type 2 expression is a hallmark of scleroderma fibroblasts in vitro.

Systemic scleroderma is a chronic disease, which leads to fibrosis of the skin and internal organs. Fibroblasts obtained from patients with this disease demonstrate an activated state in culture. We, in this study, report strong, constitutive overexpression of plasminogen activator inhibitor type-2 (PAI-2) in scleroderma fibroblasts and demonstrate that this induction observed at the mRNA and protein level is dependent on serum addition. Induced PAI-2 protein levels were restricted to the non-glycosylated 47-kDa form, which is located intracellularly. Induction was stable for at least 12 passages. No modulation by fibrogenic cytokines--for example, transforming growth factor-beta1 or connective tissue growth factor--or by antagonizing IL-1 receptors was observed. The data indicate that scleroderma fibroblasts are more sensitive to the induction of PAI-2 expression than control fibroblasts by a presently unknown factor in serum.

Adult↗
PubMed1977

Failure of dimethyl sulfoxide in the treatment of scleroderma.

Nineteen patients with systemic scleroderma and five with localized scleroderma were treated with topical dimethyl sulfoxide by painting and immersion techniques. Partial control was obtained by using a very low concentration (5%) on one side when involvement was symmetrical. Duration of treatment ranged from 3 to 15 months. Topical dimethyl sulfoxide did not improve the skin induration, range of motion, or Raynaud's phenomenon in the scleroderma patients. No substantial beneficial effect was noted on the healing of ischemic ulcers, and the continuous application of dimethyl sulfoxide did not prevent new ulceratins from developing. Relief of pain was noted in ten of 16 patients, probably due to the local analgesic effect of dimethyl sulfoxide.

Adult↗
PubMed1991

Fibrin and fibrinogen-related antigens in systemic sclerosis (scleroderma)

Abnormalities in fibrin deposition are implicated in the pathogenesis of vascular occlusion in systemic sclerosis. We have used a technique that involves electrophoresis and densitometric analysis of captured fibrin- and fibrinogen-related antigens to measure the concentration of the individual fibrin and fibrinogen degradation products in 13 patients with systemic sclerosis and in 15 healthy control subjects. As a group, patients with systemic sclerosis had markedly elevated levels of total fibrin-related antigen (p = 0.0007) and D-dimer (p = 0.0004), the terminal degradation product of cross-linked fibrin. The levels of fibrin monomer, an intermediate product in the conversion of fibrinogen to cross-linked fibrin, and of D-monomer, a terminal breakdown fragment of fibrinogen and fibrin monomer, were also elevated (p less than 0.005). We conclude that patients with systemic sclerosis have evidence of enhanced fibrin formation and degradation.

Adult↗
PubMed1991

Antibodies to mouse laminin in patients with systemic sclerosis (scleroderma) recognize galactosyl (alpha 1-3)-galactose epitopes.

Employing radioimmunoinhibition assays with distinct oligosaccharides as inhibitors, this study demonstrates that the epitope recognized on mouse laminin by sera from patients with systemic sclerosis (scleroderma) is a terminal galactosyl (alpha 1-3)-galactose disaccharide. The reaction with this alpha-digalactose was further confirmed when the sera were tested in radioimmunoassay (RIA) binding assay and in ELISA with synthetic galactose alpha 1-3 galactose coupled to human serum albumin. The circulating antibody appeared restricted to the IgG class and mostly to the subclass IgG3 and IgG4. Antibodies with the same specificity can be found in patients with American cutaneous leishmaniasis and Chagas disease; however, whilst in these diseases the antibody production is triggered by antigenic determinants present on the surface of the parasites, the events eliciting their appearance in systemic sclerosis are unknown.

Disaccharides↗
PubMedDate not supplied

[Differential diagnosis of diffuse eosinophilic fasciitis].

The authors studied clinical course, morphological findings, condition of microcirculation in 22 patients with diffuse fasciitis++ in comparison with 20 patients with systemic scleroderma and 20 patients with other similar diseases. Comparing clinico-laboratory data of 62 patients sharp differences were observed between diffuse fasciitis++ and systemic scleroderma. In diffuse fasciitis++ microcirculation disorders were less marked than in systemic scleroderma. According to the above research diffuse eosinophilic fasciitis++ is considered to be an independent nosologic form.

Diagnosis, Differential↗
PubMed1993

Systemic sclerosis (scleroderma) associated with discoid lupus erythematosus.

Six patients with systemic sclerosis (SS) and discoid lupus erythematosus (DLE) were studied to determine whether such cases have some common clinical and laboratory findings. DLE preceded SS in all cases. Three patients had diffuse scleroderma with lung and esophagus involvements and the others limited scleroderma. Three patients had anti-topoisomerase-I and antiribonucleoprotein antibodies, 2 had either of them and the remaining anticentromere antibodies. Four had DLE located on the scalp, leading to alopecia. The other 2 had DLE on the face and extremities. No case fulfilled criteria for systemic lupus erythematosus (SLE). The present cases with SS and DLE, but without SLE, indicate that this type of systemic-cutaneous collagen disease overlap does exist and may be not so rare.

Adult↗
PubMed1978

Scleroderma (progressive systemic sclerosis): progress and course based on a personal series of 118 cases.

A follow-up study has been made of a personal series of 118 cases of patients with scleroderma (progressive systemic sclerosis) followed for periods up to 25 years. These were classified according to the early distribution of skin changes as Type 1 (skin changes in fingers only), 48 cases; Type 2 (skin changes beyond the fingers but mainly in extremities), 47 cases; and Type 3 (diffuse), 19 cases, and atypical, four cases. Eighty per cent of the total series have survived five years, and 68% have survived 10 years. Survival is related to the type of scleroderma, being much better for Types 1 and 2 (acrosclerotic) than for Type 3 (diffuse). Actual compared with expected survival rate was better for younger than for older patients and for females than for males. Although the disease type gives an indication of survival in general, the caution must be used in the individual cases, as the course of the disease is very variable even in patients of the same type, and is subject to remission and relapse.

Adolescent↗
PubMed1996

[HRCT of the lung in collagenoses].

Collagen vascular diseases, representing systemic soft tissue disorders, may cause a broad spectrum of pathologic changes of the respiratory tract. The type and extent of manifestations can vary considerably among individuals and entities. This survey describes the chest radiographic and, in particular, high-resolution computed tomographic (HRCT) findings of individual lesions of the respiratory tract. It includes fibrosing alveolitis (alveolitis, interstitial pneumonia, pulmonary fibrosis) and bronchial (bronchitis/bronchiolitis, bronchiectasis), pleural and vascular manifestations, as well as lymphadenopathy and abnormalities related to therapy. We present typical patterns of changes in progressive systemic sclerosis (PSS, scleroderma), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD, Sharp syndrome), Sjögren syndrome, overlap syndrome and rheumatoid arthritis (RA). Furthermore, we describe findings which are specific for individual entities such as esophageal involvement in PSS, acute pneumonitis and pulmonary hemorrhage in SLE, lymphoproliferative disease in Sjögren syndrome and necrobiotic nodules in RA.

Arthritis, Rheumatoid↗
PubMed1984

Serum amyloid A protein concentration in progressive systemic sclerosis (scleroderma).

Serum amyloid A protein (SAA) concentrations were determined in 62 patients with progressive systemic sclerosis (PSS). Forty-seven patients had normal or slightly elevated SAA levels (less than 1000 ng/ml = micrograms/l), while 15 patients had moderately to markedly elevated SAA levels, similar to those observed in active rheumatoid arthritis (RA) (greater than or equal to 1000 ng/ml = micrograms/l). Five patients with PSS had SAA levels corresponding to those observed in amyloidosis secondary to RA. High SAA was associated with more severe skin thickening and diminished cumulative survival at five years. The rarity of amyloidosis secondary to PSS is unlikely to be related to an intrinsic defect in SAA production.

Amyloid↗
PubMed1995

Electrophysiological evidence of "nerve entrapment syndromes" and subclinical peripheral neuropathy in progressive systemic sclerosis (scleroderma).

We report the electrophysiological findings and the management of 5 subjects with progressive systemic sclerosis (PSS) and clinical evidence of nerve entrapment. Three had carpal tunnel syndrome (CTS), 1 bilateral CTS and right tarsal tunnel syndrome (TTS) and 1 Guyon's canal syndrome. Only 1 patient (with CTS) showed significant clinical improvement after surgical decompression; the other 4 demonstrated a slight recovery of conduction without lasting clinical relief after conventional treatment. To explain these failures we hypothesized that these entrapment syndromes were the clinical expression of underlying diffuse damage to the peripheral nervous system (PNS). The conduction values of nerves unaffected by entrapment syndromes were within normal limits, but almost all distal velocities were below the mean of controls. Such subclinical distal peripheral neuropathy was also verified in a selected sample of 17 patients with PSS, without clinical symptoms or signs of PNS involvement. In these 17 cases the mean distal sensory and motor conduction findings of the median, ulnar, sural and tibial nerves were significantly lower than those of a control group, while no significant differences were found in the more proximal tracts of the same nerves. Furthermore, 3 of the 17 patients showed classical electrophysiological evidence of TCS and TTS without any clinical symptoms. We conclude that the subjects with PSS had subclinical polyneuropathy which may become plain polyneuropathy or nerve entrapment syndromes perhaps induced by other risk factors.

Adult↗