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PubMed1991

Non-invasive evaluation of long-term cardiac effects of captopril in systemic sclerosis.

Impairment of left ventricular (LV) function has previously been reported in patients with systemic sclerosis (SScl). An intermittent vasospastic process in the myocardium may contribute to the development of myocardial dysfunction. Vasodilators may therefore be potentially useful in the treatment of cardiac dysfunction in patients with SScl. This study was designed to evaluate the long-term effects of captopril on the myocardial function of patients with SScl. Twenty-two patients with SScl (15 patients with diffuse scleroderma and 7 patients with CREST syndrome, i.e. calcinosis. Raynaud's phenomenon, oesophageal hypomotility, sclerodactyly, telangiectasia) were investigated by means of Doppler and echophonocardiography before and after treatment with captopril (1.3 mg kg-1 body weight d-1) for 11-15 months. There were no significant differences in heart rate, systolic and diastolic blood pressure, end-systolic blood pressure, total peripheral resistance or LV diameters before or after treatment. However, captopril treatment exerted significant effects on LV function: the pre-ejection period (PEP) and the ratio of pre-ejection period to LV ejection time decreased significantly (P less than 0.05). Mitral E-point septal separation decreased significantly (P less than 0.01), even after adjustment for LV end-diastolic diameter (P less than 0.01). The ejection fraction increased significantly (P less than 0.05), and the isovolumic relaxation time decreased (P less than 0.01). The left atrial emptying index increased (P less than 0.01). The Doppler peak late to early ventricular filling velocity decreased (P less than 0.05), and the isovolumic index was also reduced (P less than 0.05). We conclude that both systolic and diastolic LV function indices improved in patients with SScl after captopril treatment for a mean period of 1 year. The effects of captopril might be due to vasodilation of the myocardial vessels and/or a direct effect on the renin-angiotensin system of the heart.

Adult↗

PubMed1993

Anticentromere antibodies in subjects with no apparent connective tissue disease.

OBJECTIVES: To study the association of anticentromere antibodies (ACA) in various diseases. METHODS: A total of 4800 consecutive serum samples were tested for ACA by indirect immunofluorescence using HEp-2 cells as substrates and by immunoblotting of Molt-4 cell mitotic chromosomal antigens and recombinant CENP-B protein. RESULTS: Anticentromere antibodies were identified in the serum samples of 24 subjects, including eight without apparent connective tissue diseases, six with primary biliary cirrhosis, two with diffuse scleroderma, one with pulmonary hypertension, one with primary Raynaud's phenomenon, one with CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia), and five with other connective tissue diseases. By immunoblotting using Molt-4 cells mitotic chromosomal antigens three centromere antigens were recognised by these serum samples. These were: CENP-A (17 kilodalton recognised by 22 of 24 ACA positive serum samples); CENP-B (80 kilodalton recognised by 22 of 24 ACA positive serum samples); and CENP-C (140 kilodalton recognised by 19 of 24 ACA positive serum samples). There was no specific pattern for serum samples from patients with different groups of diseases on immunoblotting. Recombinant CENP-B proteins were all recognised by these samples. Patients without apparent connective tissue disease often had a lower ACA titre than patients with primary biliary cirrhosis. CONCLUSIONS: These data suggest that a positive result for ACA does not always indicate the presence of a connective tissue disease.

Adult↗

PubMed1994

Endothelin and collagen vascular disease: a review with special reference to Raynaud's phenomenon and systemic sclerosis.

Endothelin-1 (ET-1), endothelin-2, and endothelin-3, a family of 21-aminoacid peptides, are potent, long-acting vasoconstrictors of mammalian blood vessels. Elevated levels of ET-1 have been found in the circulation of patients with various diseases, all of which have some degree of vascular involvement that is associated with endothelial cell activation. These diseases include systemic sclerosis, primary and secondary Raynaud's phenomenon, pulmonary hypertension, Buerger's disease, and Takayasu's arteritis. The role of circulating ET-1 in the pathogenesis of the vascular and fibrotic lesions in these conditions remains to be elucidated. This review focuses on the recent findings of the clinical significance and pathogenetic role of endothelin in collagen vascular diseases, especially in Raynaud's phenomenon and systemic sclerosis. Although the etiology of systemic sclerosis is unknown, a vascular pathogenesis is supported by a variety of clinical and laboratory observations. The fact that plasma levels of ET-1 are elevated in patients with systemic sclerosis and this elevation is more remarkable in patients with diffuse scleroderma compared with limited scleroderma patients might further support the vascular pathogenesis of systemic sclerosis.

Collagen Diseases↗

PubMed1986

Long-term griseofulvin treatment for progressive systemic sclerosis.

Thirty-three patients with progressive systemic sclerosis (PSS) (24 women and 9 men, 27 with diffuse scleroderma and 6 with CREST syndrome) were treated with griseofulvin alone (375-500 mg/day) for 12-84 months (mean 33). Clinical and functional assessment of the results included: a self-evaluation (general status, skin toughness, cold sensitivity, dysphagia), a physical examination (fingerprint areas, chest expansion, mouth widening, grip strength) routine laboratory tests, electrocardiogram, glomerular filtration rate (GFR), esophagus and chest X-rays. After griseofulvin, a significant improvement was noted in 28/33 patients (85%) regarding subjective condition and skin thickening and elasticity, particularly in the trunk and proximal limbs (p less than 0.05 for chest expansion), and of GFR (p less than 0.01). Lung and esophageal involvement, on the whole, remained unchanged during the treatment, which does not appear to modify the progression of PSS myocardiopathy. No patient stopped using griseofulvin because of side effects. The present results show that griseofulvin is a safe drug for PSS treatment and that it can influence the skin and renal involvement.

Adolescent↗

PubMed1988

The use of immunoblotting to detect antibodies to nuclear and cytoplasmic antigens. Clinical and serological associations in rheumatic diseases.

Using the immunoblotting technique, sera from 433 patients with rheumatic diseases were screened for the presence of antibodies against several nuclear and cytoplasmic antigens, such as RNP, Sm, Ro(SSA), La(SSB), CR-19 (centromeric antigen), Topo-1 (Scl-70), Jo-1, histone and 56 kD. At the same time clinical data from these patients were collected without prior knowledge of the immunoblotting results. Syndrome-specific autoantibodies were found for mixed connective tissue disease (antibodies against the RNP related 70 kD antigen), for CREST (anti-CR-19 antibodies), for diffuse scleroderma (anti-Topo-1 antibodies) and for polymyositis (anti-Jo-1 antibodies). Almost all specific autoantibodies were present exclusively in patients with a connective tissue disease. Controls were only in a few cases positive for antihistone and anti-56 kD antibodies. Associations of specific autoantibodies with clinical and laboratory features of the patients were mostly as expected. However, some unexpected associations were found, for example polymyositis and calcinosis with anti-Sm antibodies, sicca symptoms with anti-centromere antibodies and leucopenia with Ro(SSA) and La(SSB).

Antibodies↗

PubMedDate not supplied

Association of CTLA-4 with systemic sclerosis in Japanese patients.

OBJECTIVE: The contribution of CTLA-4 alleles to the pathogenesis of systemic sclerosis (SSc) was studied in Japanese patients. METHODS: CTLA-4 typing in 2 dimorphic sites, +49 A/G and -308 C/T, was carried out in 62 SSc patients and 107 normal subjects by the PCR-RFLP (restriction fragment length polymorphism) method. HLA-DRB1*15 and *08 genotyping were carried out by the PCR-SSCP (simple-stranded DNA conformation polymorphism) method. RESULTS: In SSc the frequency of the +49A allele increased slightly (40.3%), but was not significant. In SSc with diffuse scleroderma and SSc with anti-topoisomerase I antibody, the +49A also increased (43.8%, and 48.0%, respectively) but again was not significant. A significant increase in the +49A was not observed in SSc with HLA-DRB1*1502 or ORB1*0802. In contrast, the +49A had significantly increased in SSc with the anti-RNP antibody [52.9%, p = 0.0337, Odds ratio (OR) = 2.27 (95% confidential interval (CI) = 1.09-4.71)]. HLA-DRB1*1502 and *0802 had no influence on the association of anti-RNP antibody with the +49A. The +49AA genotype increased significantly in SSc without lung fibrosis [31.8%, p = 0.0456, OR = 3.37 (CI = 1.16-9.87)], especially in limited SSc without lung fibrosis [33.3%, p = 0.0319, OR = 3.62 (CI = 1.16-11.29)]. The dimorphism at -308 did not associate with SSc. CONCLUSION: In Japanese scleroderma, the +49A allele of CTLA-4 increased in the presence of SSc with the anti-RNP antibody.

Abatacept↗

PubMedDate not supplied

[L-tryptophan-associated chronic eosinophilia-myalgia syndrome treated with cyclosporin].

After 2 weeks of ingestion of 130 g L-Tryptophan a 52 year old female develops an Eosinophilia Myalgia Syndrome with acute onset of deep venous thrombosis of forearm and possible initial cardiac manifestation featuring intermittent sinustachykardia. This is followed by a severe chronic disease (follow-up 15 months) with diffuse scleroderma and sensomotoric polyneuropathia. The deep muscle biopsy-specimen shows mononuclear infiltration of fascia and interstitial myositis with rare eosinophils. A blood eosinophilia (900/ul) occurs only in the initial acute onset of the illness. Plasma level of Kynurenine is significantly high (4000 pmol/ml), collagenneosynthesis is activated (Procollagen type III peptid 0.927 U/ml). No significant clinical improvement was seen with Acathioprine (100 mg/d) and Prednisolon (40-60 mg/d), after treatment with Ciclosporin scleroderma regresses completely, polyneuropathy is persisting.

Biopsy↗

PubMedDate not supplied

[Clinical usefulness of determining the antibodies to soluble nuclear antigens in various collagen diseases].

The purpose of the study was evaluation of the clinical usefulness of determination of antibodies to soluble nuclear antigens. The study was carried out in 71 cases of various collagen diseases. Antibodies dsDNA (IIF method with Crithidium luciliae as substrate) were found only in patients with SLE and renal involvement. RNP antibodies (double immunodiffusion method) were demonstrated in 83.3% of cases of mixed connective tissue disease, and Sm antibodies in 8% of SLE patients. It is worth stressing that in the presented material Sm antibodies were present only in association with RNP antibodies. Antibodies Ro and/or La were present most often in the sera of patients with SCLE, while Scl 70 antibodies were a marker of systemic sclerosis, more frequent in patients with diffuse scleroderma, while their demonstration in acroscleroderma suggested a more severe course of the disease. The study showed a high diagnostic and prognostic value of antibodies to soluble nuclear antigens in collagen diseases.

Antibodies, Antinuclear↗

PubMed1990

[Anticardiolipin antibodies in diffuse connective tissue diseases with IgG, IgM and IgA isotypes].

Anticardiolipin (aCL) antibodies were assessed in isotypes IgG, IgM and IgA by the enzyme immunochemical technique in serum of 86 subjects with diffuse connective tissue affections and in 75 subjects of three control groups (syphilis, syndrome of common variable immunodeficiency and blood donors). In systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and syphilis the mean values of the three isotypes of aCL antibodies were significantly higher than in blood donors (p = 0.05 to 0.001); in diffuse scleroderma and primary polymyositis/dermatomyositis in isotype IgG (p = 0.01-0.001). Positive findings of aCL antibodies (isolated or in combinations of Ig isotypes (were found most frequently in SLE (34.4%), RA (33.3%) and syphilis (66.6%); sera of blood donors were positive in 8.7%. Venous thrombosis was recorded in the case-records of 28% patients with SLE but only in 5.4% of those with RA. Spontaneous abortion terminated 8/66 pregnancies in 28 women with SLE. In one female patient with SLE the aCL syndrome was detected. On account of frequent positivity of aCL antibodies in syphilis, the authors consider it essential to rule out the coincidence of this disease. Examination of aCL-IgA antibodies extends the detection of positive cases (isolated or in combinations of Ig) in SLE and RA.

Adult↗

PubMed1990

[A case of progressive systemic sclerosis associated with mutilans-type arthropathy and suspected Felty's syndrome].

A patient who developed mutilans-type arthropathy, splenomegaly, leukopenia, leg ulcer and massive hydroxyapatite accumulation during the course of progressive systemic sclerosis (PSS) was reported. A 56-years-old female had suffered Raynaud's phenomenon since the beginning of her third decade. She developed multiple symmetrical arthritis and morning stiffness at the age of 29, and was treated with NSAIDs and low dose corticosteroids under the diagnosis of rheumatoid arthritis (RA) Because of dysphagia and diarrhea, she was admitted in Niigata-Kenritsu Senami Hospital in September, 1987. Physical and roentgenographic examinations revealed diffuse scleroderma, mutilans-type arthropathy, lung fibrosis, splenomegaly and right leg ulcer. Laboratory examinations showed leukopenia, high titer of anti-DNA antibody, positive anti-Scl-70 antibody and mild hypocomplementemia. These findings suggested that she had PSS and Felty's syndrome. Furthermore, massive subcutaneous and intraarticular hydroxyapatite accumulation were noticed. The leg ulcer and laboratory data gradually improved with the combination therapy of corticosteroids, D-penicillamine and plasmapheresis. Although it has been well recognized that PSS patients reveal frequently the articular lesions similar to these of RA, severe mutilans-type arthropathy seen in this case is extremely rare. The joint contracture might be induced by hydroxyapatite accumulation, of which the early diagnosis seems to be very important in long-standing PSS patients.

Antibodies, Antinuclear↗

PubMedDate not supplied

[Angiopathy caused by vibration and CREST syndrome: beware of hidden pathologies!].

The etiologic definition of an occupational disease should be achieved via an accurate differential diagnosis. A careful assessment of any clinical features that are atypical or unusual for the disease in question can sometimes lead to the identification of other morbid states that are either hidden or show few symptoms, and may or may not interfere with the occupational picture. The case described is of a man who, after working for 10 years with vibrating tools, developed a vibration disease with typical circulatory alterations in the hands and osteoarticular and neurological alterations. However, the appearance of a necrotic ischaemic lesion at the tip of the second finger of the right hand and signs of sclerodactylia in the second finger of both hands led us to suspect the presence of another concomitant disease. The finding of anticentromere antibodies suggested the presence of a CREST syndrome, which is a serologic variant of diffuse scleroderma. More in-depth investigations and examination of the state of other organs and apparatuses confirmed the diagnostic suspicion, with evidence of impairment also of the cardio-pulmonary system. The presence of the CREST syndrome was interpreted as a state of hypersusceptibility to the traumatic action of vibrating tools on the vascular system.

Arm↗

PubMed1989

[Peritoneal dialysis, the method of treatment for end-stage renal insufficiency: development of indications during the past 10 years in relation to the initial renal disease and extra-renal pathology].

A decade after its first introduction, the advantages and drawbacks of continuous ambulatory peritoneal dialysis over hemodialysis remain controversial. This present paper is a review of the literature, focused on the indications of this dialysis modality in different circumstances: extra-renal pathology, systemic diseases (lupus erythematosus--diffuse scleroderma--plasma cell disorders--amyloidosis--HIV infected patients) and complications related to hemodialysis.

Humans↗

PubMedDate not supplied

Cineradiography identifies esophageal candidiasis in progressive systemic sclerosis.

Cineradiography of the esophagus showed signs of esophageal candidiasis in 11 out of 71 patients with progressive systemic sclerosis (PSS) - both in diffuse scleroderma and the CREST syndrome. Culture of esophageal brushings confirmed the presence of Candida albicans in eight of these 11 patients. Antimycotic treatment decreased the cineradiographic signs of candidiasis and the degree of dysphagia. Since impaired esophageal motility and treatment with immunosuppressive drugs may predispose to candida esophagitis, and since dysphagia will decrease after antimycotic treatment esophageal mycosis should always be sought in patients with PSS.

Candidiasis↗

PubMed1989

[Radiological changes in feet of patients with progressive systemic sclerosis].

Forty six patients (41 female and 5 male) with progressive systemic sclerosis were analysed through X-ray photographs of feet. Age ranged from 33 to 74 (mean: 48 +/- 13) years old, and durations of the disease were one to 28 (mean: 9.8 +/- 8) years. Abnormal radiological findings of feet were noted as following; bone absorption of the terminal tufts of the first phalanx (6 out of 46 cases: 13%), bone absorption of metatarsals and/or mid, basal phalanges (2 cases: 4.3%), bone erosion (6 cases: 13.0%), calcification (7 cases: 15.2%), diffuse osteoporosis (2 cases: 4.3%) and cystic lesion (one case: 2.2%). All patients with abnormal findings on X-ray photographs of feet simultaneously showed radiological changes of hands. It took 13.3 +/- 7 years until the radiological findings appeared in feet. On the other hand, it took much shorter 8.8 +/- 6 years in hands. Patients with radiological changes in feet tended simultaneously to have pulmonary and digestive tract involvements, and widespread skin lesions such identified as diffuse scleroderma. Anti-Scl 70 antibody were positive in a half of these patients.

Adult↗

PubMed1987

[Motility disorders of the esophagus].

The paper describes the physiology of swallowing, the methods for the assessment of esophageal motility, and the motility disorders of the tubular part and the lower sphincter of the esophagus, except for gastroesophageal reflux disease. Primary esophageal motility disorders are achalasia (incomplete relaxation of the lower sphincter in response to swallowing), diffuse esophagospasm (simultaneous repetitive contractions), and the nutcracker esophagus (propulsive peristalsis with abnormally high amplitude). Besides, there are non-specific as yet unclassified contraction abnormalities. Since hypermotile contraction abnormalities can mimic chest pain of cardiac origin, differential diagnosis of anginal chest pain should include esophageal motility disorders. Contraction abnormalities of the esophagus may occur in diffuse scleroderma, after therapeutic radiation of the mediastinum, and possibly after sclerotherapy of esophageal varices.

Esophageal Achalasia↗

PubMedDate not supplied

The value of echocardiography in the early diagnosis of myocardial impairment due to connective tissue diseases.

The study comprised 14 patients with systemic lupus erythematosus (SLE) and 12 patients with diffuse scleroderma. Echocardiography ascertained 5 cases of congestive and 1 case of restrictive cardiomyopathy among the patients with SLE, as well as 6 cases with restrictive cardiomyopathy among those with scleroderma. The authors specify two important echocardiographic variables: the E-septum distance and the septal and wall motion after amyl-nitrite test, which reveal the intrinsic contractile properties of the myocardium, allowing an early diagnosis of myocardial impairment, as well as the selection of patients for an efficient treatment with vasodilator drugs.

Cardiomyopathy, Dilated↗

PubMedDate not supplied

[Streptococcus antibodies in a rural population and in patients with inflammatory rheumatic diseases].

An account is given of assessed titres of anti-streptolysin O (ASO) and antihyaluronidase (AH) in rheumatic fever, in rheumatic patients of inactive phase and in tonsillitis. The ASO titre was examined in 1739 members of the general public and in 360 patients with chronic rheumatic diseases. The author analyses 6235 examinations of ASO titres and 1210 AH examinations. In rheumatic fever the mean maximum ASO titre was 397 u, while the mean maximum AH titre was 3583 u. In inactive phase rheumatic patients the mean ASO titre was 187 u, the mean AH titre 630 u. The mean ASO titre at the onset of the disease in tonsillitis was 172 u. During the second examination after 3-4 weeks, the mean rise of the ASO titre was 205 u. A increased AH titre above 1280 u was recorded during the first examination in 35.2%, during the second examination in 52.3%. The mean ASO titre in the population group comprising 1739 subjects was 148 u. A titre increased above 200 u was recorded in 20.7%. The mean ASO titre in rheumatoid arthritis was 139 u, in ankylosing spondylitis 199 u, in systemic lupus erythematosus and diffuse scleroderma 128 u. In all groups the ASO and AH levels were inversely proportional to the age of the examined subject. Attention is drawn to the correct evaluation of the assessed antibody titres.

Adolescent↗

PubMed1977

[An case of acute diffuse seleroderma in an infant].

The authors report a case of diffuse scleroderma in a 15 months old infant. Dermatologic (clinical and pathological) findings are quite typical of the disease. On the other hand, in this case some particularities were observed: the age of the infant (second published case beginning before the age of two); the presence of a durable eosinophilia, the absence of visceral lesions and of biological abnormaliteis (of auto-immune nature specially), the evolution towards athrepsica and death within one year. Thus, because of these particularities, the diagnosis of scleroderma remains questionable and the diagnosis of progeria has been considered. The affection appeared in the course of a hepatitis leaving a hepatic fibrosis without inflammatory signs; no conclusion can be drawn about the relations between the hepatic affection and the fatal dermatologic disease.

Acute Disease↗