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Virginia D Steen

Publications and source records attributed to Virginia D Steen.

9 recordsLinked to original sources
PubMed2006

Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease.

OBJECTIVE: To describe the differences between patients with systemic sclerosis (SSc) having childhood versus adult onset evaluated at a single medical center. METHODS: Patients were divided into those with childhood onset (first SSc symptom or finding before age 16 yrs) and those with early adult and late adult onset. The 3 groups were compared with respect to disease classification, clinical, laboratory and serologic data, and survival. RESULTS: One hundred eleven childhood onset SSc cases seen between 1960 and 2003 were compared with 2559 adult onset SSc cases (1087 with onset age 16-40 and 1472 with onset after age 40 yrs) first evaluated between 1972 and 2001. Age distribution at onset was unimodal, suggesting that childhood disease is part of the spectrum of adult onset SSc. A significantly greater proportion of childhood onset patients had overlap syndromes, most frequently with polymyositis-dermatomyositis (PM-DM), and skeletal muscle involvement. Children with diffuse cutaneous (dc) SSc had significantly lower maximum mean total skin thickness scores than adult patients with dcSSc. Renal involvement was uncommon in childhood onset cases, and the frequency increased with age of onset. Serum anti-PM-Scl and anti-U1RNP antibodies were detected significantly more frequently in childhood than in adult onset cases. In contrast, anti-RNA polymerase III and anticentromere antibodies were found significantly more frequently in adults. Survival was significantly better among childhood than all adult onset cases combined, but similar to survival in young adult onset SSc cases. Scleroderma heart disease was a frequent cause of death among children with SSc. CONCLUSION: Patients with juvenile onset SSc more frequently have an overlap syndrome with PM-DM, higher frequency of skeletal muscle involvement, serum anti-PM-Scl and anti-U1RNP antibody, fatal cardiac disease, and improved survival compared with adult onset SSc cases.

Adult↗
PubMed2006

Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension.

OBJECTIVE: Lung transplantation is a viable, life-saving intervention for several primary pulmonary disorders complicated by severe lung dysfunction. This study was undertaken to evaluate whether patients with systemic sclerosis (scleroderma), a systemic autoimmune rheumatic disorder, would receive similar benefit from this intervention. METHODS: Survival following lung transplantation was examined at 2 university medical centers among 29 patients with scleroderma as compared with 70 patients with idiopathic pulmonary fibrosis (IPF) and 38 with idiopathic pulmonary arterial hypertension (IPAH), the latter groups representing pathologically related primary pulmonary disorders. The end point was death from any cause. Risk of mortality in patients with scleroderma was compared with that in patients with IPF or IPAH, with adjustment for demographic and clinical parameters. RESULTS: During 2 years of followup, 11 patients with scleroderma (38%), 23 with IPF (33%), and 14 with IPAH (37%) died. Cumulative survival at 6 months posttransplantation was 69% in the scleroderma group compared with 80% in the IPF group (log-rank P = 0.21) and 79% in the IPAH group (P = 0.38). The estimated risk of mortality at 6 months was increased in patients with scleroderma compared with those with IPF (relative risk [RR] 1.70, 95% confidence interval [95% CI] 0.74-3.93) and those with IPAH (RR 1.52, 95% CI 0.59-3.96), but the differences were not statistically significant. Over the following 18 months, there was convergence in the survival rates such that cumulative survival at 2 years was comparable, at approximately 64%, among all 3 groups. CONCLUSION: Patients with scleroderma who are recipients of lung transplantation experience similar rates of survival 2 years after the procedure compared with those with IPF or IPAH. Lung transplantation may represent a viable therapeutic option to consider for patients with end-stage lung disease due to scleroderma.

Adult↗
PubMed2005

Correlation of the degree of dyspnea with health-related quality of life, functional abilities, and diffusing capacity for carbon monoxide in patients with systemic sclerosis and active alveolitis: results from the Scleroderma Lung Study.

OBJECTIVE: To determine whether baseline self-assessment measures of health status and physiologic indices of disease severity in alveolitis-positive patients with systemic sclerosis (SSc) correlate with the severity of their dyspnea, and to quantify functional impairment in patients with scleroderma lung disease and compare it with that in patients with chronic obstructive pulmonary disease (COPD). METHODS: SSc patients (n = 138) with diffuse (n = 81) or limited (n = 57) cutaneous disease and active alveolitis (determined by bronchoalveolar lavage and/or high-resolution computed tomography) who participated in the National Heart, Lung, and Blood Institute-sponsored, multicenter, parallel-group, double-blind, randomized, placebo-controlled trial of oral cyclophosphamide for treatment of SSc-associated interstitial lung disease were evaluated. Pearson's univariate correlations were determined between the Short Form 36 (SF-36) physical component summary (PCS) and mental component summary (MCS) scales, functional questionnaires, and physiologic parameters of breathing (forced vital capacity [FVC] and single-breath diffusing capacity for carbon monoxide [DLCO]). Student's t-test was used to compare subgroups. Scores from 2 instruments for self-assessment of breathlessness, Mahler's baseline dyspnea index (BDI) and a visual analog scale (VAS) for breathing, were divided at the median. Values for the DLCO and FVC (% predicted) were divided based on the American Thoracic Society guidelines for mild (>70% of predicted), moderate (50-70% of predicted), and severe (<50% of predicted) physiologic impairment. RESULTS: Scores on the BDI and VAS for breathing were highly correlated (r = -0.61). The PCS and MCS were able to differentiate patients with more breathlessness (measured by BDI and VAS for breathing) and more abnormal physiologic measures (FVC and DLCO). In SSc patients with alveolitis, all 8 domains of the SF-36 were significantly impaired as compared with the healthy population and were similar to those reported by patients with COPD. CONCLUSION: The SF-36 was able to discriminate between scleroderma lung disease patients with more severe and less severe breathlessness, the primary symptom of active alveolitis. The SF-36 complements the BDI and VAS scores for breathing in scleroderma lung disease and is variably correlated with results of pulmonary function tests, suggesting that the SF-36 should be included as an outcome measure in intervention trials in this population.

Carbon Monoxide↗
PubMed2005

Autoantibodies in systemic sclerosis.

OBJECTIVES: To describe the clinical, laboratory, and prognostic features associated with the scleroderma-specific autoantibodies. METHODS: Using the Pittsburgh Scleroderma Databank, all consecutive patients seen between 1980 and 1995 who had autoantibody studies performed were studied. Anticentromere antibodies (ACA), antitopoisomerase (TOPO), anti-U1-RNP (U1-RNP), anti-RNA Polymerase III (Pol 3), anti-U3-RNP (U3-RNP), anti-Th/To (Th/To), and anti-Pm/Scl (Pm/Scl) were determined according to previously described methods. The frequency of clinical features, organ system outcomes, and survival within the patients with a specific antibody were cumulative over the course of the disease. The frequency of a specific feature was compared across groups to identify significant manifestations and outcomes in patients with a specific antibody. RESULTS: Some demographic, clinical, and organ system findings were associated with the specific antibody, and other features with the scleroderma subtype (limited cutaneous or diffuse cutaneous scleroderma). U3-RNP, U1-RNP, and TOPO were seen more commonly in African-American patients, and ACA was seen in older, female Caucasians. Muscle inflammation was seen in patients with U1-RNP and U3-RNP. Digital tip ulcers and digital tuft resorption were seen more frequently in those with ACA and TOPO. A vasculopathy causing pulmonary hypertension typically occurs with ACA and pulmonary fibrosis with TOPO; however, both types of lung disease were seen in patients with nucleolar antibodies, Th/To and U3-RNP. Importantly, severe interstitial fibrosis was rarely seen in cases with Pol 3. Renal crisis was strongly associated with Pol 3. Survival within limited scleroderma was decreased in the Th/To patients compared with ACA patients. Within the diffuse scleroderma group, patients with Pol 3 had the best survival. CONCLUSIONS: Scleroderma autoantibodies are associated with very specific demographic, clinical, organ system, and survival features. RELEVANCE: The determination of scleroderma autoantibodies may be helpful in assessing the prognosis, monitoring, and treatment of scleroderma patients.

Adult↗
PubMed2005

Kidney disease other than renal crisis in patients with diffuse scleroderma.

OBJECTIVE: To determine the frequency and severity of kidney abnormalities in patients with diffuse scleroderma. METHODS: All patients with diffuse scleroderma seen at the University of Pittsburgh between 1972 and 1993 were included in the study. Kidney function tests were routinely obtained as part of the Pittsburgh Scleroderma Outcome Study. Additional kidney tests were obtained as part of the 1992 biannual outcome assessment. Patients who had kidney abnormalities including a serum creatinine > 1.2 mg/dl or proteinuria prior to 1993 were identified. The clinical setting and longterm outcome of kidney disease were evaluated. RESULTS: Renal crisis occurred in 129/675 (19.5%) patients. Kidney function abnormalities or proteinuria were present in 173 (26%); 48% had no abnormalities. Most patients had other explanations for the abnormality. Only 12 (2%) of the 675 patients with diffuse scleroderma had no explanation for the elevated creatinine level. Most patients with proteinuria had toxicity from D-penicillamine. No explanations for proteinuria were found in 16 (2%) of this cohort. Thus, a total of only 28 (4%) of these 675 patients had an unknown cause for their kidney dysfunction or proteinuria. None of these patients, who were followed for a mean of 10 years after onset of scleroderma, have developed chronic renal insufficiency that progressed to dialysis. CONCLUSION: Patients with diffuse scleroderma without renal crisis rarely have significant increases in serum creatinine or proteinuria that cannot be explained by other etiologies. These patients with scleroderma should be carefully evaluated for non-scleroderma causes of kidney disease.

Adult↗
PubMed2005

Responsiveness of the SF-36 and the Health Assessment Questionnaire Disability Index in a systemic sclerosis clinical trial.

OBJECTIVE: This study compares the responsiveness to change of the Medical Outcomes Study Short Form Health Survey (SF-36), a measure of health related quality of life (HRQOL), and the Health Assessment Questionnaire Disability Index (HAQ-DI), a function instrument, in a randomized clinical trial for treatment of systemic sclerosis (SSc). METHODS: A phase 2/3, multicenter, prospective, placebo controlled trial was conducted to evaluate human recombinant relaxin treatment in patients with diffuse SSc over 24 weeks. At baseline, subjects had stable, moderately severe, diffuse SSc of disease duration < or = 5 years, modified Rodnan skin score > or = 20, serum creatinine < 2.0 mg/dl, percentage forced vital capacity (% FVC) predicted > or = 50%, and % DLCO predicted > or = 40% and were not receiving concomitant disease modifying therapies. Internal consistency reliability of multi-item scales was estimated using Cronbach's alpha. Responsiveness to change of the SF-36 and HAQ-DI was computed between Weeks 0 and 24. Subjects were classified as unchanged or having a meaningful change in 4 different external measures: Change in (1) skin score > or = 30%; (2) % FVC predicted of > or = 15%; (3) self-reported patient global assessment by visual analog scale (VAS) > or = 20%; and (4) physician global assessment by VAS of > or = 20%. Responsiveness indices were computed and Cohen's effect size criteria were used to assess the magnitude of change. RESULTS: A total of 239 patients participated in this trial, with 196 completing the 24 week trial. Cronbach's alpha for the SF-36 scales ranged from 0.76 to 0.93 and for the HAQ-DI ranged from 0.69 to 0.91 (good to excellent). The SF-36 had a larger magnitude of responsiveness in overall disease (patient and physician global assessment) compared to the HAQ-DI, while the HAQ-DI had a larger magnitude of responsiveness in clinical measures (i.e., change in skin score and % FVC predicted) than the SF-36. CONCLUSION: These data support inclusion of both the SF-36 and HAQ-DI as outcome measures in future clinical trials of diffuse SSc.

Adult↗
PubMed2003

A comparison between anti-Th/To- and anticentromere antibody-positive systemic sclerosis patients with limited cutaneous involvement.

OBJECTIVE: To compare anti-Th/To-positive and anticentromere antibody (ACA)-positive patients with limited cutaneous systemic sclerosis (lcSSc). METHODS: We reviewed the medical records of 107 anti-Th/To-positive patients and 365 ACA-positive patients who were first evaluated during 1985-2000. ACA was detected by indirect immunofluorescence on HEp-2 cell substrate, and anti-Th/To was detected by RNA immunoprecipitation with K562 cell extracts. Patients were included if they had a clinical diagnosis of lcSSc, and excluded if they had >1 SSc-associated serum autoantibody. RESULTS: The final study groups comprised 87 lcSSc patients with anti-Th/To antibodies and 306 with ACAs. Anti-Th/To-positive patients were younger (P < 0.04) and had a shorter disease duration at their first evaluation (P < 0.003). Patients with anti-Th/To antibodies had more subtle skin changes, less severe vascular involvement, and less frequent distal esophageal hypomotility. Both groups had a higher frequency of "intrinsic" pulmonary hypertension than has been previously reported in the literature (28% and 19% of anti-Th/To-positive and ACA-positive patients, respectively), perhaps due to referral bias. Patients in the anti-Th/To group more often had radiographic evidence of interstitial lung disease (48% versus 13% of the ACA group; P < 0.0001). Scleroderma renal crisis was uncommon (4 cases), but occurred exclusively in the anti-Th/To group. Survival among the anti-Th/To-positive patients was reduced compared with that in the ACA group (P < 0.02). CONCLUSION: Patients with anti-Th/To and those with ACA most often develop lcSSc and have a high frequency of "intrinsic" pulmonary hypertension. Compared with the ACA patients, anti-Th/To lcSSc patients have more subtle cutaneous, vascular, and gastrointestinal involvement, but more often have certain features typically seen in diffuse scleroderma, such as pulmonary fibrosis and scleroderma renal crisis, as well as reduced survival.

Adult↗
PubMed2003

Scleroderma renal crisis.

Renal crisis occurs in patients who have systemic sclerosis with rapidly progressive diffuse cutaneous thickening early in their disease. SRC is characterized by malignant hypertension, hyperreninemia, azotemia, microangiopathic hemolytic anemia, and renal failure. SRC was almost uniformly fatal, but in most cases it can now be successfully treated with ACE inhibitors. This therapy has improved survival, reduced the requirement for dialysis, and often allowed for the discontinuation of dialysis 6 to 18 months later. Prompt diagnosis and early, aggressive initiation of therapy with ACE inhibitors will result in the most optimal outcome.

Acute Kidney Injury↗