Tracy Frech

University of Utah, Salt Lake City, UT, United States

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Publications (25)59.36 Total impact

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    ABSTRACT: We sought to examine the relationship between measures of ILD severity and PH in patients with SSc.
    Clinical and experimental rheumatology 11/2014; 32 Suppl 86(6):109-14. · 2.66 Impact Factor
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    ABSTRACT: Objectives: The aim of this research was to examine parental influence on the development of Systemic Sclerosis (SSc). We designed three studies: (1) mitochondrial inheritance; (2) birth order (a possible surrogate marker for microchimerism); and (3) paternal age at conception (a possible surrogate for telomere erosion) to examine their association with development of SSc.Methods: SSc was defined by International Classification of Diseases Ninth and Tenth Revision codes (ICD-9 710.1 and ICD 10 M34.0, M34.1, and M34.9) and identified from statewide discharge data, University of Utah Health Science Center Enterprise Data Warehouse (UUHSC), and death certificates that were linked to the Utah Population Database (UPDB) for analysis. Mitochondrial inheritance was evaluated by Conditional Logistic Regression (CLR) and Population Attributable Risk (PAR) using familial standardized incidence ratio (FSIR) as the covariate. Chi square test and logistic regression was used to evaluate birth order and maternal/paternal age at conception of the SSc proband.Results: We found 1947 unique SSc patients from UUHSC and UPDB. We selected 5 controls per case, (n=9115) matched by birth year and sex. Mitochondrial inheritance analysis indicated no evidence to suggest SSc was associated with mitochondrial inheritance. Birth order and maternal/paternal age at conception analysis results show they too do not significantly affect SSc development.Conclusions: Results suggest that while heritable risk of SSc is observed, mitochondrial inheritance, birth order, and parental age are not likely responsible for pathogenesis. © 2014 American College of Rheumatology.
    Arthritis Care & Research. 04/2014;
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    ABSTRACT: Objective To assess cumulative survival rates and identify independent predictors of mortality in patients with incident systemic sclerosis (SSc)–associated pulmonary arterial hypertension (PAH) who had undergone routine screening for PAH at SSc centers in the US. Methods The Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma registry is a prospective registry of SSc patients at high risk for PAH or with definite pulmonary hypertension diagnosed by right-sided heart catheterization within 6 months of enrollment. Only patients with World Health Organization group I PAH (mean pulmonary artery pressure ≥25 mm Hg and pulmonary capillary wedge pressure ≤15 mm Hg without significant interstitial lung disease) were included in these analyses. ResultsIn total, 131 SSc patients with incident PAH were followed for a mean ± SD of 2.0 ± 1.4 years. The 1-, 2-, and 3-year cumulative survival rates were 93%, 88%, and 75%, respectively. On multivariate analysis, age >60 years (hazard ratio [HR] 3.0, 95% confidence interval [95% CI] 1.1–8.4), male sex (HR 3.9, 95% CI 1.1–13.9), functional class (FC) IV status (HR 6.5, 95% CI 1.8–22.8), and diffusing capacity for carbon monoxide (DLco) <39% predicted (HR 4.2, 95% CI 1.3–13.8) were significant predictors of mortality. Conclusion This is the largest study describing survival in patients with incident SSc-associated PAH followed up at multiple SSc centers in the US who had undergone routine screening for PAH. The survival rates were better than those reported in other recently described SSc-associated PAH cohorts. Severely reduced DLco and FC IV status at the time of PAH diagnosis portended a poor prognosis in these patients.
    Arthritis Care & Research. 03/2014; 66(3).
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    ABSTRACT: Type I interferons (IFNs) are implicated in the pathogenesis of systemic sclerosis (SSc). MEDI-546 is an investigational human monoclonal antibody directed against the type I IFN receptor. This Phase 1 study evaluated the safety/tolerability, pharmacokinetics (PK), immunogenicity, and pharmacodynamics (PD) of single and multiple intravenous doses of MEDI-546 in adults with SSc. Subjects (>=18 years) with SSc were enrolled in an open-label, dose-escalation study to receive single (0.1, 0.3, 1.0, 3.0, 10.0, or 20.0 mg/kg), or 4 weekly intravenous doses (0.3, 1.0, or 5.0 mg/kg/week) of MEDI-546. Subjects were followed for 12 weeks. Safety assessments included adverse events (AEs), laboratory results, and viral monitoring. Blood samples were collected from all subjects for determination of PK, presence of anti-drug antibodies (ADAs), and expression of type I IFN-inducible genes. Of 34 subjects (mean age 47.4 years), 32 completed treatment and 33 completed the study. Overall, 148 treatment-emergent AEs (TEAEs) were reported (68.9% mild, 27.7% moderate). TEAEs included one grade 1 infusion reaction (5.0 mg/kg/week multiple dose). Of 4 treatment-emergent serious AEs (skin ulcer, osteomyelitis, vertigo, and chronic myelogenous leukemia (CML)), only CML (1.0 mg/kg/week multiple dose) was considered possibly treatment-related. MEDI-546 exhibited non-linear PK at lower doses. ADAs were detected in 5 subjects; no apparent impact on PK was observed. Peak inhibition of the type I IFN signature in whole blood was achieved within 1 day and in skin after 7 days. The safety/tolerability, PK, and PD profiles observed in this study support further clinical development of MEDI-546.Trial Registration: ClinicalTrials.gov NCT00930683.
    Arthritis research & therapy 02/2014; 16(1):R57. · 4.27 Impact Factor
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    ABSTRACT: In this study, 1,833 systemic sclerosis (SSc) cases and 3,466 controls were genotyped with the Immunochip array. Classical alleles, amino acid residues, and SNPs across the human leukocyte antigen (HLA) region were imputed and tested. These analyses resulted in a model composed of six polymorphic amino acid positions and seven SNPs that explained the observed significant associations in the region. In addition, a replication step comprising 4,017 SSc cases and 5,935 controls was carried out for several selected non-HLA variants, reaching a total of 5,850 cases and 9,401 controls of European ancestry. Following this strategy, we identified and validated three SSc risk loci, including DNASE1L3 at 3p14, the SCHIP1-IL12A locus at 3q25, and ATG5 at 6q21, as well as a suggested association of the TREH-DDX6 locus at 11q23. The associations of several previously reported SSc risk loci were validated and further refined, and the observed peak of association in PXK was related to DNASE1L3. Our study has increased the number of known genetic associations with SSc, pro- vided further insight into the pleiotropic effects of shared autoimmune risk factors, and highlighted the power of dense mapping for detecting previously overlooked susceptibility loci.
    The American Journal of Human Genetics 01/2014; 94:47-61. · 11.20 Impact Factor
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    ABSTRACT: Background Electronic medical records (EMR) provide an ideal opportunity for the detection, diagnosis, and management of systemic sclerosis (SSc) patients within the Veterans Health Administration (VHA). The objective of this project was to use informatics to identify potential SSc patients in the VHA that were on prednisone, in order to inform an outreach project to prevent scleroderma renal crisis (SRC). Methods The electronic medical data for this study came from Veterans Informatics and Computing Infrastructure (VINCI). For natural language processing (NLP) analysis, a set of retrieval criteria was developed for documents expected to have a high correlation to SSc. The two annotators reviewed the ratings to assemble a single adjudicated set of ratings, from which a support vector machine (SVM) based document classifier was trained. Any patient having at least one document positively classified for SSc was considered positive for SSc and the use of prednisone>10 mg in the clinical document was reviewed to determine whether it was an active medication on the prescription list. Results In the VHA, there were 4,272 patients that have a diagnosis of SSc determined by the presence of an ICD-9 code. From these patients, 1,118 patients (21%) had the use of prednisone>10 mg. Of these patients, 26 had a concurrent diagnosis of hypertension, thus these patients should not be on prednisone. By the use of natural language processing (NLP) an additional 16,522 patients were identified as possible SSc, highlighting that cases of SSc in the VHA may exist that are unidentified by ICD-9. A 10-fold cross validation of the classifier resulted in a precision (positive predictive value) of 0.814, recall (sensitivity) of 0.973, and f-measure of 0.873. Conclusions Our study demonstrated that current clinical practice in the VHA includes the potentially dangerous use of prednisone for veterans with SSc. This present study also suggests there may be many undetected cases of SSc and NLP can successfully identify these patients.
    Computers in Biology and Medicine. 01/2014;
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    ABSTRACT: Gastrointestinal tract (GIT) involvement in systemic sclerosis (scleroderma, SSc) is the most common internal complication. This review discusses the outcome measures to capture GIT involvement in clinical care and trials. Patient-reported outcome measures have been validated (UCLA Scleroderma Clinical Trial Consortium GIT 2.0 and NIH PROMIS scales) in SSc-GIT. Multiple objective measures are available to assess mucosal involvement and motility in GIT. However, these need to be validated in SSc for trials. GIT is a common cause of morbidity and has negative impact on quality of life in SSc. Recommendations are given for trial design and evaluation of GIT involvement in SSc.
    Current opinion in rheumatology 09/2013; · 4.60 Impact Factor
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    ABSTRACT: Autophagosomes are formed during autophagy, which is activated by hypoxia and starvation. Autophagy is important for mast cell degranulation. We hypothesized that autophagy is a key feature in the pathogenesis of systemic sclerosis (SSc). We examined SSc clinical features and mast cell density across the presence and severity of autophagy. Skin punch biopsy was performed on 33 SSc patients and 6 healthy controls (HC). Autophagy was evaluated by immunofluorescence on paraffin sections using LC3-FITC staining on these patients. The intensity of staining and mast cell density was examined across clinical features in 19 of the SSc patients. Presence of autophagosome formation was assessed by EM in 17 of the SSc patients and 4 HC. In our SSc study population, 29 of subjects were female and 23 were limited cutaneous. Twenty-nine of 33 SSc patients had autophagy by LC3-FITC staining. Intensity of staining decreased with longer duration of SSc (p = 0.09) and RP (p = 0.10). Bloating and distention differed across level of intensity staining (Wilcoxon signed-rank test, p = 0.05), with the greatest levels among those with moderate intensity. On EM, autophagosome formation was present in 16 of 17 SSc patients and no HC. All SSc patients had perivascular mast cells. Autophagy was present in 29 of 33 SSc patients, and none of our HC suggesting importance in pathogenesis. Autophagy staining was greater among those with shorter duration of SSc. Bloating and distention were higher in patients with moderate autophagy staining. Perivascular mast cells were present in all SSc patients. The role of autophagy in vasculopathy and mast cell activation in SSc warrants further studies.
    Rheumatology International 08/2013; · 2.21 Impact Factor
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    ABSTRACT: We performed bedside testing for peripheral neuropathy in our systemic sclerosis (SSc) population to determine whether foot care guidelines should be developed for SSc. Twenty consecutive SSc patients and 20 healthy control (HC) patients were evaluated for peripheral neuropathy in both feet using the 10-g Semmes-Weinstein monofilament examination (SWME) and 128 Hz vibration sensation using the on-off method. Independent, blinded, vibratory sensation, and SWME evaluations were performed on each subject by two investigators who had completed a training session to standardize each exam. An additional consecutive 20 patients with type 2 diabetes mellitus (DM) were examined by a diabetologist to compare with peripheral neuropathy prevalence in SSc patients. We examined the inter-rater variability using Cohen's kappa. We compared SWME and vibratory sensation in SSc to HC using Fisher's exact. The t test was used to compare duration of disease and modified Rodnan skin score (mRSS) for those with abnormal SWME or vibratory sensation. Two of 20 SSc patients reported sensory foot symptoms consistent with peripheral neuropathy prior to the examination. Inter-rater agreement for both SWME and vibratory sensation was strong (kappa: 0.72 and 0.83, respectively). Two HC and 12 SSc patients demonstrated abnormal vibratory sense (one-sided Fishers' exact, p < 0.002). No HC and four SSc patients had abnormal monofilament exams (one-sided Fisher's exact, p = 0.053). Neither mRSS (p = 0.28) nor duration of non-Raynauds (p = 0.07) symptoms differed between those with peripheral neuropathy and those without. Duration of Raynaud's symptoms were clinically significantly associated with presence of peripheral neuropathy (p = 0.04). The prevalence of sensory loss to monofilament in SSc was identical to DM patients (4/20). SSc patients have a considerable prevalence of pedal peripheral neuropathy as detected by loss of vibratory sensation or inability to sense the 10-g SWME. Further studies are indicated to determine if routine screening for neuropathy and subsequent podiatric care for SSc patients with abnormalities can reduce pedal complications.
    Clinical Rheumatology 02/2013; · 2.04 Impact Factor
  • Maureen A Murtaugh, Tracy M Frech
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    ABSTRACT: BACKGROUND AND AIMS: Gastrointestinal manifestations in systemic sclerosis (SSc) can influence the nutritional status of patients. Our objective was to examine whether nutritional status was associated with symptoms captured by the University of California Los Angeles Scleroderma Clinical Trials Consortium Gastrointestinal Tract Questionnaire (GIT 2.0). METHODS: A series of 24 University of Utah SSc Center patients were assessed using the MUST, SGA, and GIT 2.0. A single evaluator administered the nutrition assessment and gastrointestinal symptom questionnaire. RESULTS: Nine patients were assessed at moderate to high risk of malnutrition using the Malnutrition Universal Screening Tool (MUST) and 12 patients with moderate to severe malnutrition using Subjective Global Assessment (SGA). Neither MUST nor SGA status was associated with duration of disease. Soilage, social function and emotional subscores were associated with SGA nutritional status. Clinically significant differences in Total GIT 2.0 score, reflux, distention/bloating, soilage, diarrhea, social function and emotional well-being were observed across levels of nutritional status. CONCLUSIONS: Clinically significant differences in gastrointestinal tract symptoms were observed across levels of nutritional status in patients with varying severity of SSc. These two clinically utilized tools, the SGA and the GIT 2.0, appear to be complementary in the evaluation of SSc patients.
    Clinical nutrition (Edinburgh, Scotland) 07/2012; · 3.27 Impact Factor
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    ABSTRACT: The main histopathological focus of systemic sclerosis (SSc) has concentrated on fibrotic changes. We investigated the microvasculature alterations in the skin of patients with SSc at various stages of disease duration with whole-field digital microscopy. Twenty consecutive patients with SSc, 1 with Raynaud's phenomenon (RP) without SSc, and 4 healthy controls underwent punch biopsy on the medial forearm. Eighteen patients were included in the primary analysis. Two with recent-onset diffuse cutaneous disease, 1 repeat SSc biopsy, and 1 patient with RP without SSc were also evaluated. All specimens were processed with histochemical stains and immunohistochemistry. We analyzed microvasculature abnormalities in an objective and systematic manner taking advantage of recent advances in whole-field digital microscopy. This analysis was coupled with ultrastructural evaluation performed with transmission electron microscopy (TEM). Whole-field digital microscopy and TEM of SSc skin biopsies revealed that endothelial abnormalities are a universal feature regardless of clinical features and/or duration of disease. These features were not seen in any healthy control specimens or in the single RP patient samples. Whole-field digital microscopy identified increased interstitial edema (31.0% ± 9.6% vs 17.6% ± 3.3% in controls; p = 0.009) and fibrosis (75.6% ± 5.7% vs 66.1% ± 9.8% in controls; p = 0.02) in all patients with SSc. Lower CD34 staining was seen in SSc compared to healthy controls (0.32% ± 0.22% vs 1.31% ± 0.34%; p < 0.0001) and within the SSc population with interstitial lung disease (0.55% ± 0.22% vs 0.15% ± 0.16%; p = 0.01). Perivascular and interstitial infiltrate of mast cells was present in all SSc specimens. Whole-field digital microscopy offers a means of rapidly carrying out objective, fully quantitative, and reproducible measurements of microscopic features of SSc microvascular change. The universal morphologically abnormal endothelial cells and interstitial edema in all patients with SSc biopsied suggests that SSc may be intrinsically a disease of the endothelium characterized by vascular leak.
    The Journal of Rheumatology 06/2012; 39(7):1385-91. · 3.26 Impact Factor
  • American Thoracic Society 2012 International Conference, May 18-23, 2012 • San Francisco, California; 05/2012
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    ABSTRACT: Patients with normal (mean pulmonary arterial pressure (mPAP) ≤20 mm Hg) and borderline mean pulmonary pressures (21-24 mm Hg) are "at risk" of developing pulmonary hypertension (PH). The objectives of this analysis were to examine the baseline characteristics in systemic sclerosis (SSc) with normal and borderline mPAP and to explore long-term outcomes in SSc patients with borderline mPAP versus normal haemodynamics. PHAROS is a multicentre prospective longitudinal cohort of patients with SSc "at risk" or recently diagnosed with resting PH on right heart catheterisation (RHC). Baseline clinical characteristics, pulmonary function tests, high-resolution CT, 2-dimensional echocardiogram and RHC results were analysed in normal and borderline mPAP groups. 206 patients underwent RHC (results showed 35 normal, 28 borderline mPAP, 143 resting PH). There were no differences in the baseline demographics. Patients in the borderline mPAP group were more likely to have restrictive lung disease (67% vs 30%), fibrosis on high-resolution CT and a higher estimated right ventricular systolic pressure on echocardiogram (46.3 vs 36.2 mm Hg; p<0.05) than patients with normal haemodynamics. RHC revealed higher pulmonary vascular resistance and more elevated mPAP on exercise (≥30; 88% vs 56%) in the borderline mPAP group (p<0.05 for both). Patients were followed for a mean of 25.7 months and 24 patients had a repeat RHC during this period. During follow-up, 55% of the borderline mPAP group and 32% of the normal group developed resting PH (p=NS). Patients with borderline mPAP have a greater prevalence of abnormal lung physiology, pulmonary fibrosis and the presence of exercise mPAP ≥30 mm Hg.
    Annals of the rheumatic diseases 02/2012; 71(8):1335-42. · 8.11 Impact Factor
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    ABSTRACT: Objective. Little is known about prevalence of osteoporosis risk factors among American Indians and Alaska Natives (AIAN). Methods. We included AIAN people (n=8,039) enrolled in the Education and Research Towards Health (EARTH) Study. Prevalence ratios were used to determine cross-sectional associations of risk factors with self-reported bone fractures. Results. There is a high prevalence of multiple risk factors for osteoporosis in AIAN, although the factors that are associated with past fracture vary by gender and geographical area. In general, women who reported a fracture reported more risk behaviors, more than two medical conditions, and low physical activity. Men with higher BMI were less likely to report a fracture. Smoking history was associated with fracture for both genders, though not significantly in all sub-groups. Conclusion. We prevent a high prevalence of risk factors for osteoporosis for AIAN. Future research for osteoporosis risk reduction and prevention in AIAN people is indicated.
    Journal of Health Care for the Poor and Underserved 01/2012; 23(3):1157-73. · 1.10 Impact Factor
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    ABSTRACT: Introduction. Accurate blood pressure (BP) measurement is essential to the diagnosis and management of hypertension in patients with systemic sclerosis (SSc) to help prevent renal and cardiovascular complications. The presence of an auscultatory gap during manual BP measurement-the temporary disappearance of the Korotkoff sounds during cuff deflation-leads to a potentially important underestimate of systolic BP if undetected. Objectives. Since the presence of an auscultatory gap is frequently associated with increased vascular stiffness, we investigated its presence and correlates in 50 consecutive SSc patients. Methods. For each patient, BP was measured sequentially using three different approaches performed in the same order. Results. Sixteen of 50 patients (32%) had an auscultatory gap which if undetected would have resulted in clinically important underestimates of systolic BP in 4 patients. The presence of an auscultatory gap was statistically associated with the presence of antibodies to RNA polymerase III (P<0.0068) and SSc diagnosis type (P<0.01). Conclusions. Our study demonstrates that auscultatory gaps are relatively common in SSc and correlate with markers for SSc vasculopathy. If undetected auscultatory gaps may result in clinically important underestimation of BP. Thus, electronic oscillometric BP may be preferred in SSc patients.
    International Journal of Rheumatology 01/2012; 2012:590845.
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    ABSTRACT: Objectives: There is a need for standardization in systemic sclerosis (SSc) management. Methods: SSc experts (n 117) were sent 3 surveys to gain consensus for SSc management. Results: First-line therapy for scleroderma renal crisis (SRC) was an angiotensin-converting en-zyme inhibitor (ACEi). For SRC there were not many differences between treating mild or severe SRC. In general, Second-line was to add either a calcium channel blocker (CCB) or angiotensin receptor blocker (ARB) and then an alpha-blocker (66% agreed). Endothelin receptor agonists (ERAs) were the first treatment in mild pulmonary arterial hypertension (PAH) (72%), followed by adding a phosphodiesterase-5 inhibitor (PDE5i) (77%) and then a prostanoid (73%). For severe PAH, initial treatment was 1 of the following: a prostanoid (49%), combination of a ERA and a PDE5i (18%), or combination of a ERA and a prostanoid (16%) (71% agreed). For mild Raynaud's phenomenon (RF), after a CCB and adding a PDE5i (35%), trying an ARB (32%) and finally a prostanoid (23%) was suggested. For more severe RF, 54% agreed on adding a PDE5i (45%) or prostanoid (32%) to a CCB. In the prevention of digital ulcers (DU), initial treatment was a CCB (73%), then adding a PDE5i, then use of a ERA, and then a prostanoid (44% agreed). In interstitial lung disease/pulmonary fibrosis, for induction, usually intravenous cyclophosph-amide and mycophenolate mofetil (MMF) or azathioprine were chosen. For maintenance, MMF was chosen by three-fourths (56% agreed). For gastroesophageal reflux disease, 50% would exceed the maximum recommended proton pump inhibitor dose if required (72% agreed). For skin involvement after methotrexate, MMF was usually chosen (37% agreement). For SSC-related inflammatory arthritis, methotrexate therapy (60%) was followed by adding corticosteroids (37%) or hydroxychloroquine (31%) (62% agreed). Conclusions: Discrepancies in drug choices occurred in treatment after first line in SSc. Not all algorithms had good agreement. This study provides some guidance for SSc management.
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    ABSTRACT: Pulmonary arterial hypertension (PAH) is a clinical condition characterised by the presence of precapillary pulmonary hypertension (PH). Included within the subcategorisation of PAH are heritable (HPAH) and PAH associated various conditions (APAH) including systemic sclerosis (SSc). The pathogenesis of HPAH and SSc has been linked to both a genetic predisposition and epigenetic factors. TGF-β superfamily signalling has also been implicated in the development of these conditions. In this review, we discuss the role of genetic predisposition, epigenetic factors along with dysregulation in TGF-β superfamily signalling in the pathogenesis of PAH and SSc.
    International journal of clinical practice. Supplement 08/2011;
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    ABSTRACT: Rheumatologic disorders are associated with sleep disturbances. This study examines sleep disturbance correlates in patients with SSc. Participants are 180 SSc patients in an observational study. At baseline, patients completed the Medical Outcomes Study Sleep measure (MOS-Sleep scale). In addition, patients were administered other patient-reported outcome (PRO) measures including the 36-item short form (SF-36), HAQ disability index (HAQ-DI), Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-Fatigue), Center for Epidemiologic Studies Depression (CESD) scale and a University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract Questionnaire (UCLA SCTC GIT 2.0). Descriptive statistics were assessed for six scales of MOS-Sleep and the 9-item sleep problem index (SLP-9; a composite index). We computed Spearman's rank-order correlations between the MOS-Sleep scales and the HAQ-DI, FACIT-Fatigue, CESD, SSc-SCTC GIT 2.0 and SF-36 scales. In addition, we developed a regression model to assess predictors of SLP-9 scores. Covariates included demographics, physician variables of disease severity and patient-reported variables of worsening symptoms and the PRO measures. SSc patients reported a mean (s.d.) of 7.1 (1.73) h of sleep a night. Patients reported worse scores on four of six scales (except for snoring and sleep quantity) compared with the US general population (P < 0.001). SLP-9 was correlated with worsening pain and dyspnoea over the past 1 month, reflux scale of the UCLA SCTC GIT 2.0, CESD and FACIT-Fatigue (ρ 0.26-0.56). In the stepwise multivariate regression model, the CESD, worsening dyspnoea and reflux scale were significantly associated with SLP-9 index. Sleep disturbances are common in SSc and are associated with worsening dyspnoea, depressed mood and severity of reflux symptoms.
    Rheumatology (Oxford, England) 02/2011; 50(7):1280-7. · 4.24 Impact Factor
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    ABSTRACT: Pruritus is a common symptom in systemic sclerosis (SSc), an autoimmune disease which causes fibrosis and vasculopathy in skin, lung, and gastrointestinal tract (GIT). Unfortunately, pruritus has limited treatment options in this disease. Pilot trials of low-dose naltrexone hydrochloride (LDN) for pruritus, pain, and quality of life (QOL) in other GIT diseases have been successful. In this case series we report three patients that had significant improvement in pruritus and total GIT symptoms as measured by the 10-point faces scale and the University of California Los Angeles Scleroderma Clinical Trials Consortium Gastrointestinal Tract 2.0 (UCLA SCTC GIT 2.0) questionnaire. This small case series suggests LDN may be an effective, highly tolerable, and inexpensive treatment for pruritus and GIT symptoms in SSc.
    International Journal of Rheumatology 01/2011; 2011:804296.
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    ABSTRACT: Treatment for gastrointestinal tract (GIT) disease in systemic sclerosis (SSc) is challenging as no immunosuppressive or anti-fibrotic therapy is available with clearly proven efficacy. Probiotics are viable, non-pathogenic microorganisms that are hypothesized to improve the composition of the intestinal microbiota from a potentially harmful composition to a composition that is beneficial to the host. Our hypothesis is that GIT symptoms in SSc patients with moderate bloating would improve with probiotic implementation. Ten patients with a moderate-to-severe distention/bloating score (1.25-3.00) on the University of California Los Angeles Scleroderma Clinical Trials Consortium Gastrointestinal Tract 2.0 (UCLA SCTC GIT 2.0), but otherwise stable organ disease not requiring any medication adjustment were recruited from the University of Utah Scleroderma Center. We compared the GIT 2.0 scores at baseline and after 2 months of use of Align (bifidobacterium infantis; 109 CFU per capsule) or Culturelle (lactobacillus GG; 109 CFU per capsule) using paired t-test and calculated effect size (ES). Significant improvement in total GIT 2.0 score (ES = 0.82), reflux (ES = 0.33), bloating/distention (ES = 1.76), and emotional scales (ES = 0.18) were reported after two months of daily probiotic use. This pilot study suggests probiotics significantly improve the reflux, distention/ bloating, and total GIT scales in SSc patients. As hypothesized, the largest effect was seen in distention/bloating scale. Probiotics may be useful for treatment of SSc-associated distention/ bloating.
    Clinical and experimental rheumatology 01/2011; 29(2 Suppl 65):S22-5. · 2.66 Impact Factor