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PubMed1988

Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis.

Of 397 systemic sclerosis (scleroderma) patients from the University of Pittsburgh, who had serum determinations of both anti-Scl-70 and anticentromere antibody (ACA), 26% had anti-Scl-70 and 22% had ACA. No patient had both autoantibodies. Weak associations with HLA-DR5 and HLA-DR1 were detected with anti-Scl-70 and ACA, respectively. ACA was found almost exclusively (96%) in patients with limited cutaneous scleroderma (the CREST syndrome variant), but the majority (57%) of patients with limited scleroderma did not have this antibody. Among patients with limited scleroderma, those with ACA more often had calcinosis and telangiectasias and less often had pulmonary interstitial fibrosis and restrictive lung disease. However, the frequency of pulmonary hypertension and the survival rates were similar in the ACA+ and ACA- limited scleroderma patients. Two-thirds of patients with anti-Scl-70 had diffuse scleroderma, but only 33% of all diffuse scleroderma patients had this antibody. Within the subset of diffuse scleroderma, anti-Scl-70 was associated with peripheral vascular disease (digital pitting scars) and pulmonary interstitial fibrosis, but was not predictive of cardiac or renal involvement or survival. ACA and anti-Scl-70 are useful in diagnosing and classifying systemic sclerosis variants and in predicting the natural course of the disease. Their mutually exclusive occurrence suggests either 2 separate clinical entities or important host factors determining their production.

Adult↗
PubMed1999

Pregnancy in women with systemic sclerosis.

OBJECTIVE: To determine pregnancy outcomes in women with systemic sclerosis. METHODS: Women of childbearing age with systemic sclerosis seen at the University of Pittsburgh between 1987 and 1996 were observed prospectively. Pregnancy outcomes included abortion, miscarriage, preterm and term birth, and perinatal death. Complications of pregnancy and scleroderma were determined during and after pregnancy. RESULTS: Fifty-nine women with systemic sclerosis had 91 pregnancies during the 10-year period. No increase in the frequency of miscarriage was found, except in those with long-standing diffuse scleroderma. Preterm births occurred in 29% of pregnancies, and all but one of the infants survived. Symptoms related to scleroderma, particularly Raynaud phenomenon, improved during pregnancy, but esophageal reflux became worse. After pregnancy, some women with diffuse scleroderma had increased skin thickening. There were three cases of renal crisis during pregnancy, all in women with early diffuse scleroderma. Four women had five healthy infants while taking angiotensin-converting-enzyme inhibitors. CONCLUSION: Women with systemic sclerosis can safely have healthy pregnancies. Those with early diffuse scleroderma should wait until their disease stabilizes before becoming pregnant to decrease the risk of renal crisis. High-risk pregnancy management should be standard for all scleroderma pregnancies because of the high frequency of premature births.

Adult↗
PubMed1996

Monoclonal antibody therapy of diffuse cutaneous scleroderma with CAMPATH-1H.

A worker in a polyvinyl chloride factory with rapidly progressive diffuse cutaneous scleroderma was treated with the humanized monoclonal antibody (Mab) CAMPATH-1H. Treatment was followed by rapid and substantial improvement in skin score, which was maintained during followup without additional therapy. The mode of action of CAMPATH-1H and its relationship to clinical improvement are discussed.

Adult↗
PubMedDate not supplied

Association of TAP1 and TAP2 with systemic sclerosis in Japanese.

OBJECTIVE: The contribution of polymorphism of transporter associated with antigen processing 1 and 2 (TAP1 and 2) alleles to the pathogenesis of Japanese SSc was studied. METHODS: TAP1 and TAP2 typing was carried out in 55 Japanese SSc patients and 95 normal Japanese subjects by the PCR-RFLP (restriction fragment length polymorphism) method. HLA-DR typing and HLA DRB1*15, *16 and *08 genotyping were carried out by the PCR and the PCR-SSCP (single-stranded DNA conformation polymorphism) methods, respectively. RESULTS: The frequencies of the TAP1A and TAP2A alleles were significantly increased in SSc with diffuse scleroderma (100%, p < 0.005; 80.0%, p < 0.001, respectively) and in SSc with antitopoisomerase 1 antibody (a-Scl-70), (93.2%, p = not significant (NS); 63.6%, p < 0.05). In contrast, the TAP1B allele was significantly decreased in diffuse scleroderma (0%, p < 0.005) and SSc with a-Scl-70 (4.5%, p < 0.05), and TAP2B was decreased in diffuse scleroderma (12.5%, p < 0.01). CONCLUSION: Association analysis among TAP1A, TAP2A and DRB1*1502 indicated that increases in TAP1A and TAP2A were not primary, but were reflective of an increase in HLA DRB1*1502 in Japanese SSc patients with diffuse scleroderma and SSc with a-Scl-70.

ATP-Binding Cassette Transporters↗
PubMed1997

The palpable tendon friction rub: an important physical examination finding in patients with systemic sclerosis.

OBJECTIVE: To evaluate the clinical and prognostic significance of palpable tendon friction rubs in patients with systemic sclerosis (SSc). METHODS: SSc patients evaluated prospectively at the University of Pittsburgh were examined serially for the presence of tendon friction rubs on physical examination. Demographic, clinical, and laboratory features of disease were obtained by patient examination, annual patient questionnaire, and medical record review. Patients were classified as having limited or diffuse scleroderma according to standard definitions. The prognostic significance of the presence of tendon friction rubs was determined using this comprehensive database. RESULTS: The SSc patients (n = 1,305) were first evaluated during 1972 through 1991 and were followed up for a mean of 6.3 years. Tendon friction rubs were detected most frequently in patients who had or who developed diffuse cutaneous involvement. There were strong correlations between the presence of tendon friction rubs and symptoms and signs typical of diffuse scleroderma, including more severe skin thickening, more frequent heart and kidney involvement, and decreased survival. In multiple regression analyses, the presence of 1 or more tendon friction rubs was one of the best predictors of both evolution to diffuse scleroderma and reduced survival. CONCLUSION: The palpable tendon friction rub is an easily detected, inexpensively obtained physical examination finding which is highly associated with diffuse cutaneous scleroderma and decreased survival. This observation should lead to the early diagnosis of diffuse scleroderma and should identify patients at high risk for serious visceral involvement who are thus candidates for potential disease-modifying therapy.

Ankle Joint↗
PubMedDate not supplied

[Systemic connective tissue diseases--prognostic conclusions of a 30-year study].

In the course of the last three decades a gradually supplemented group of 627 patients was subjected to prognostic analysis, incl. 388 with systemic lupus erythematosus, 160 with diffuse scleroderma, 51 with dermato- and polymyositis and 28 with a mixed connective tissue syndrome. Taking into account the onset of the disease, sex, extent of organ affection and therapy as well as the survival period and mortality, the conclusions of the statistical evaluation revealed that more than 90% of patients survived for more than 5 years: 92.2% with systemic lupus erythematosus, 94.3% with diffuse scleroderma 93.3% with dermato- and polymyositis and 100% with mixed connective tissue syndrome; a ten-year survival was recorded in cca 80% patients (77.6% with systemic lupus erythematosus, 81.1% with diffuse scleroderma, 89.7% with dermato- and polymyositis); a 15-year survival was recorded still by more than 50% patients (53.4% with systemic lupus erythematosus, 58.2% with diffuse scleroderma and as many as 80.0% with dermato- and polymyositis). The above data indicate considerable improvement of the prospects of these diseases, whereby the more than treble prolongation of survival of patients as compared with the previous period is due to effective, although not causal immunotherapy, in particular in systemic lupus erythematosus and dermato- and polymyositis.

Adult↗
PubMedDate not supplied

Scleroderma renal crisis: poor outcome despite aggressive antihypertensive treatment.

BACKGROUND: Scleroderma renal crisis (SRC) is a rare but feared complication of scleroderma. Angiotensin--converting enzyme (ACE) inhibition has significantly improved survival, but it is unknown whether prophylactic ACE inhibitors will prevent this complication. AIMS: To determine: (i) the frequency of SRC in our cohort of well-characterized scleroderma patients resident in South Australia, (ii) any predisposing clinical and serological features, (iii) median disease duration at which SRC occurs, (iv) possible precipitants, (v) disease outcome, and (vi) whether patients were taking ACE inhibitors prior to onset of SRC. METHODS: Systematic review of the clinical course of all patients registered on the South Australian Scleroderma Register. RESULTS: SRC occurred in 16 patients. This constituted 2.8% of a total scleroderma cohort and 15% of the diffuse scleroderma cohort identified in South Australia. All 16 patients had diffuse cutaneous scleroderma. SRC occurred at a median disease duration of 15 months (range 1 week-11 years). Disease outcome was poor (five deaths, three requiring long-term dialysis and only two patients regaining a normal creatinine) despite aggressive antihypertensive treatment (including ACE inhibitors) in an intensive care or specialized renal unit. Two patients were later able to discontinue dialysis. Only two patients were taking small doses of ACE inhibitors prior to the onset of their SRC. The frequency of Scl-70 was decreased in the SRC group (P = 0.003). CONCLUSION: SRC is a rare event occurring in a small proportion of patients with diffuse scleroderma. The outcome of SRC was poor despite aggressive antihypertensive treatment. It is hypothesized that prophylactic ACE inhibition in susceptible patients might prevent or ameliorate this complication.

Adult↗
PubMed1988

Renal vascular histology and morphometry in systemic sclerosis. A case-control autopsy study.

An autopsy case-control study of renal vascular histology and morphometry in systemic sclerosis (scleroderma) was performed. Thirty-five of 70 systemic sclerosis cases had renal tissue available for study: 26 had diffuse cutaneous involvement (9 with "renal crisis" and 17 without) and 9 had limited cutaneous disease (CREST syndrome [calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias]). Age-matched (within 10 years) and sex-matched controls with renal specimens were obtained. New sections were cut from tissue blocks, and morphometry was completed using a Zeiss Image Analyzer. Using analysis of variance, the intimal area (Ai) was significantly increased (intimal thickening) in small and medium-sized arteries of patients with diffuse scleroderma and in small arteries of CREST patients, compared with those in controls, while a decreased medial area (Am) was seen consistently in all groups. The proportion of the vessel wall occupied by intima (Ai:[Ai + Am]) was significantly greater in all vessel size groups in patients with diffuse scleroderma compared with that in controls. The percentage of luminal occlusion was greatest in patients with diffuse disease with renal crisis. These same patients had severe edematous and mucinous intimal thickening in small and medium vessels, often in association with fibrinoid necrosis. We conclude that renal vascular structural changes are an integral part of systemic sclerosis. However, the significant differences between diffuse scleroderma patients and CREST syndrome patients, for both intimal thickening and percentage of luminal occlusion, suggest that the arterial disease in these 2 patient subsets is distinctive.

Calcinosis↗
PubMed1985

Anticentromere antibody: an immunological marker of a subset of systemic sclerosis.

Our clinical and immunological studies of 114 cases of systemic sclerosis, 54 of Raynaud's disease and 46 of other connective tissue diseases, centered on the diagnostic and prognostic significance of anticentromere antibodies (ACA). The ACA occurred in 21 of 84 patients with acrosclerosis, in four of 54 patients with Raynaud's disease but in none of 30 patients with diffuse scleroderma or transitional form, acrosclerosis-diffuse scleroderma, or 46 cases of other connective tissue diseases. The ACA-positive patients had no contracture or immobilization of the fingers, the indurations and/or indurative oedema were confined to fingers and usually no other types of ANA were detected. However, systemic involvement and the course of the disease were comparable in ACA-negative and ACA-positive acrosclerosis patients. The studies indicate that there is a subset of acrosclerosis with minimal indurations confined to the fingers, and ACA appears to be its serological marker. We propose to use the term CREST for this subset, which to date has not been exactly defined and is regarded by some authors as synonymous with acrosclerosis.

Adult↗
PubMed1985

Anticentromere antibody and immunoglobulin allotypes in scleroderma.

Fifty-five unrelated whites with disorders in the scleroderma spectrum who had both antinuclear antibodies and Raynaud's phenomenon (RP) were studied. Of the 22 patients with anticentromere antibody (ACA), three had diffuse scleroderma; 16 had the complete or incomplete syndrome of calcinosis, RP, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST syndrome); and three had RP only. Thirty-three patients with other nuclear patterns all had systemic scleroderma (28 diffuse scleroderma, five CREST syndrome). Patients with ACA had less organ system involvement, and lower frequencies of anemia and elevation of sedimentation rate than ACA-negative patients, but these differences were not statistically significant. They also had fewer manifestations of CREST syndrome. All 55 patients were studied for the Gm and Km allotypic markers. No association was found between Gm or Km allotypic markers and scleroderma or between the allotypic markers and the presence of ACA.

Adolescent↗
PubMed1989

Marker antibodies in scleroderma and polymyositis: clinical associations.

Sera of 34 patients with progressive systemic sclerosis and of 11 patients with polymyositis/dermatomyositis (PM/DM) were analyzed by the immunoblotting technique for the presence of marker antibodies. The presence of anti-centromere, anti-Topoisomerase-I (anti-Topo-I) and anti-Jo-1 antibodies was found to be highly specific for the CREST syndrome, diffuse scleroderma and PM/DM, respectively, but only of limited sensitivity (78, 44 and 45%, respectively). Anti-Topo-I positive diffuse scleroderma patients had a more severe disease (digital pitting scars and renal insufficiency) than anti-Topo-I negative diffuse scleroderma patients. Anti-Jo-1 was associated with interstitial lung disease. Longitudinal studies showed a constant antibody pattern. Our results confirm the clinical usefulness of these marker antibodies.

Antibody Specificity↗
PubMed2000

Brachial plexopathy associated with diffuse edematous scleroderma.

Peripheral nervous system (PNS) involvement is rare in systemic sclerosis (SSc), usually restricted to peripheral nerve entrapment. To our knowledge, only one case of scleroderma with brachial plexus involvement has been reported previously. We report here on a 61-year-old woman with past history of limited cutaneous SSc who developed motor deficiency in the left arm concomitant with diffuse edematous scleroderma without evidence for trauma or compression of the brachial plexus. After six months intravenous pulse cyclophosphamide therapy, dramatic improvement of skin and neurological involvement was observed.

Anti-Inflammatory Agents↗
PubMed1985

Assessment of esophageal abnormalities in progressive systemic sclerosis using radionuclide transit.

Radionuclide transit (RT) is a noninvasive test of esophageal function with a sensitivity equivalent to manometry. Using RT, 34 patients with progressive systemic sclerosis (PSS), 15 with diffuse scleroderma and 18 with the CRST variant, were studied and compared to 22 patients with other connective tissue diseases and 20 normal volunteers. Abnormalities were present in 87% of patients with diffuse scleroderma, 72% with CRST, 38% with other connective tissue diseases and in none of the controls. The most frequent abnormality in PSS was that of adynamic transit. Abnormalities correlated with disease duration in the diffuse scleroderma group but not the CRST group. There was a positive association between abnormal RT and the presence of esophagitis in the PSS patients (p = 0.003).

Adolescent↗
PubMed1994

Association of HLA-DR with progressive systemic sclerosis in Japanese.

OBJECTIVE: To clarify the contribution of HLA-DR genes to the susceptibility to progressive systemic sclerosis (PSS). METHODS: HLA-DR typing was carried out in 36 Japanese patients with PSS, 42 with systemic lupus erythematosus and 104 healthy subjects by polymerase chain reaction (PCR) method using specific primers and by PCR-SSCP (single-standard DNA conformation polymorphism) method. RESULTS: A haplotype DRB1*1502-DRB5*0102 was significantly increased in PSS (50.0%, p < 0.00004, pc < 0.001), especially in antitopoisomerase I antibody (a-Scl-70) positive patients (62.5%, p < 0.00003, pc < 0.001) and PSS with diffuse scleroderma (75.0%, p < 0.00001, pc < 0.0001). In addition, DRB1*0802 was also increased in DRB1*1502 negative patients with a-Scl-70, (50.0%, p = 0.033, pc = not significant) and in DRB1*1502 negative patients with diffuse scleroderma (75.0%, p = 0.008, pc = not significant). Thus, 81.3% of a-Scl-70 positive patients, and 93.8% of patients with PSS with diffuse scleroderma showed either HLA-DRB1*1502 or 0802. CONCLUSIONS: Our observations show the extreme difference of genetic background of a-Scl-70 positive PSS, with regard to HLA-DR, between Japanese and other ethnic groups including Caucasian and American black persons. The increase in DRB1*1502-DRB5*0102 haplotype supported the hypothesis of Reveille, et al that uncharged polar amino acid residue at position 30 of HLA-DQB1 allele was important for a-Scl-70 positive PSS because close association of the haplotype with DQB1*0601 was well established in Japanese; listed as a hypothetical candidate of PSS susceptible DQB1 allele. DRB1*0802 were also associated with hypothetical candidates of DQ alleles. Furthermore, the sharing of the particular amino acid sequence: valine38 and phenylalanine67-lysine68-glutamic acid69-asparic acid70-arginine71, by DRB5*0102, DRB1*0802 and DR11 (associated with Caucasian PSS) also suggests a contribution of the sequence in HLA-DR molecules to the pathogenesis of PSS according to the shared epitope hypothesis.

Adult↗
PubMed1986

Scl 70 antibody--a specific marker of systemic sclerosis.

Scl 70 antibodies were tested for in 107 patients with systemic sclerosis: 68 with acrosclerosis and 39 with diffuse scleroderma. Anticentromere antibodies (ACA) and other antinuclear antibodies (ANA) were tested for by indirect immunofluorescence on HEp-2 cells. Positive results for Scl 70 antibodies were obtained in 77% of cases of diffuse scleroderma and 44% of acrosclerosis. ACA and Scl 70 antibodies were found to be mutually exclusive. If acrosclerosis cases positive for anticentromere antibodies are excluded, the percentage of acrosclerosis cases positive for Scl 70 was 63%. ACA were found to be a marker of a benign, abortive subset of acrosclerosis with almost no cutaneous involvement (CREST), whereas Scl 70 did not discriminate between acrosclerosis and diffuse scleroderma. On HEp-2 cells Scl 70 positive sera gave a characteristic, fine speckled, almost homogeneous nuclear staining pattern.

Antibodies, Antinuclear↗