The prevalence of organ complications in scleroderma (systemic sclerosis; SSc) varies by definition used. This study was done to determine the frequency of several features of SSc.
A search of ...Medline-Ovid/Embase, PubMed, and Scopus databases from 1980 to November 30, 2011, was conducted to identify relevant articles with at least 50 patients with SSc extracting prevalence of each organ complication. Study quality was assessed using the STROBE (Strengthening The Reporting of OBservational studies in Epidemiology) checklist. Pooled prevalence was calculated using the random effects method. Heterogeneity was quantified using I(2).
A total of 5916 articles were identified (913 from Medline-Ovid/Embase, 1009 from PubMed, and 3994 from Scopus); 5665 were excluded, leaving 251 articles for full-text review, with 69 included. Where available, frequencies were also included from the Canadian Scleroderma Research Group. Many severe complications in SSc occur about 15% of the time, including cardiac involvement (15%, 95% CI 6-24), diastolic dysfunction (16%, 95% CI 14-17), estimated pulmonary artery pressure > 40 mm Hg (18%, 95% CI 14-21), pulmonary arterial hypertension by right heart catheterization (15%, 95% CI 12-17), forced vital capacity (FVC) < 70% predicted (15%, 95% CI 12-17), FVC < 80% predicted (17%, 95% CI 12-21), myositis (13%, 95% CI 10-17), inflammatory arthritis (12%, 95% CI 9-16), Sjögren overlap (13%, 95% CI 10-16), and digital ulcers (DU; 15%, 95% CI 10-20); and 15% of DU have complications (amputations 12%, 95% CI 8-16, and hospitalizations 13%, 95% CI 6-21). Scleroderma renal crisis is uncommon but occurs in almost 15% (12%, 95% CI 5-19) of cases of disseminated cutaneous SSc. There is no 15% rule within skin and gastrointestinal tract for SSc.
The "15%" rule for frequency of significant organ involvement in SSc is helpful.
The purpose of this study was to review the potential importance of interleukin 6 (IL-6) in systemic sclerosis (SSc).
PubMed and Scopus databases and American College of Rheumatology (from 2009-10) ...and European League Against Rheumatism abstracts (2009-11) were searched using keywords "scleroderma; SSc; cytokines; interleukins; interleukin 6" and publications were excluded if not pertaining to IL-6 in SSc. Data were extracted from selected articles to construct a cell interaction model of the effects of IL-6 in SSc.
A total of 416 reports were found (PubMed, n = 82; Scopus, n = 331; 3 abstracts); 372 were excluded (irrelevant) leaving 41 publications and 3 abstracts (39 from PubMed, 18 from Scopus; but 16 were repeated from PubMed search), where 40 suggested IL-6 was important in SSc and 4 did not. Effects of IL-6 in SSc were summarized schematically.
Of the 44 publications, 40 suggested that IL-6 may be important in SSc, allowing for a conceptual framework within SSc including effects on macrophages, fibroblasts, plasma cells, monocytes, and extracellular matrix.
Treatment Algorithms in Systemic Lupus Erythematosus Muangchan, Chayawee; van Vollenhoven, Ronald F.; Bernatsky, Sasha R. ...
Arthritis Care and Research,
September 2015, Letnik:
67, Številka:
9
Journal Article
Recenzirano
Odprti dostop
Objective
To establish agreement on systemic lupus erythematosus (SLE) treatment.
Methods
SLE experts (n = 69) were e‐mailed scenarios and indicated preferred treatments. Algorithms were constructed ...and agreement determined (≥50% respondents indicating ≥70% agreement).
Results
Initially, 54% (n = 37) responded suggesting treatment for scenarios; 13 experts rated agreement with scenarios. Fourteen of 16 scenarios had agreement as follows: discoid lupus: first‐line therapy was topical agents and hydroxychloroquine and/or glucocorticoids then azathioprine and subsequently mycophenolate (mofetil); uncomplicated cutaneous vasculitis: initial treatment was glucocorticoids ± hydroxychloroquine ± methotrexate, followed by azathioprine or mycophenolate and then cyclophosphamide; arthritis: initial therapy was hydroxychloroquine and/or glucocorticoids, then methotrexate and subsequently rituximab; pericarditis: first‐line therapy was nonsteroidal antiinflammatory drugs, then glucocorticoids with/without hydroxychloroquine, then azathioprine, mycophenolate, or methotrexate and finally belimumab or rituximab, and/or a pericardial window; interstitial lung disease/alveolitis: induction was glucocorticoids and mycophenolate or cyclophosphamide, then rituximab or intravenous gamma globulin (IVIG), and maintenance followed with azathioprine or mycophenolate; pulmonary hypertension: glucocorticoids and mycophenolate or cyclophosphamide and an endothelin receptor antagonist were initial therapies, subsequent treatments were phosphodiesterase‐5 inhibitors and then prostanoids and rituximab; antiphospholipid antibody syndrome: standard anticoagulation with/without hydroxychloroquine, then a thrombin inhibitor for venous thrombosis, versus adding aspirin or platelet inhibition drugs for arterial events; mononeuritis multiplex and central nervous system vasculitis: first‐line therapy was glucocorticoids and cyclophosphamide followed by maintenance with azathioprine or mycophenolate, and then rituximab, IVIG, or plasmapheresis; and serious lupus nephritis: first‐line therapy was glucocorticoids and mycophenolate, then cyclophosphamide then rituximab.
Conclusion
We established variable agreement on treatment approaches. For some treatment decisions there was good agreement between experts even if no randomized controlled trial data were available.
The objective of this study is to investigate the impact of skin sclerosis burden on an internal organ involvement over a 1-year period, as measured by time-adjusted accrual-modified Rodnan skin ...score (TA-mRSS), and to evaluate association between TA-mRSS patterns and laboratory tests in patients with systemic sclerosis (SSc). This prospective study was conducted at Siriraj Hospital (Bangkok, Thailand) during the November 2013–November 2016. SSc patients by ACR/EULAR 2013 or ACR 1980 criteria were eligible. TA-mRSS was classified as low, intermediate, or high, and then compared between groups. Correlation between the arithmetic mean of laboratory tests and TA-mRSS was assessed by multiple linear regression analysis. A total of 118 patients, with 81.4% women, median (IQR) age 49.8 (43.8, 55.1) years, disease duration from onset of non-Raynaud symptoms to first visit of 3.3 (1, 6.8) years, 78% dcSSc, and 75.3% anti-Scl-70 positivity, were analyzed. TA-mRSS over 1 year ranged from 0 to 37.44. The high skin sclerosis burden group had a median TA-mRSS > 7.26 (> 67th percentile). Patients with high TA-mRSS were dcSSc, high initial and average mRSS, and had tendon friction rub, digital ischemic complications, usual interstitial pneumonia, diastolic dysfunction, gastrointestinal dysmotility, and low serum albumin. In multiple linear regression analysis, the arithmetic mean of hemoglobin (
B
= − 1.007, 95% CI − 1.779 to − 0.236), erythrocyte sedimentation rate (
B
= − 0.078, 95% CI − 0.126 to − 0.029), serum glutamic oxaloacetic transaminase (
B
= 0.073, 95% CI 0.026–0.12), creatine phosphokinase (
B
= 0.012, 95% CI 0.003–0.021), and albumin (
B
= − 4.117, 95% CI − 6.958 to − 1.276) were associated with TA-mRSS. This study found a higher cumulative course of mRSS over a 1-year period to be significantly associated with severe internal organ involvement.
Objective:
To investigate the prevalence of and independent predictors for digital ischemic complications in patients with systemic sclerosis.
Method:
Patients enrolled in the Siriraj Systemic ...Sclerosis Cohort registry during 2013–2019 were classified as having or not having digital ischemic complications at the baseline and 1-year timepoints.
Results:
A total of 171 patients with systemic sclerosis were included. The prevalence of digital pulp loss, digital pitting scar, digital ulcer, and digital amputation at baseline and 1 year was 41.5%, 39.8%, 3.5%, 7.6% and 37.4%, 43.9%, 14.1%, 6.4%, respectively. Over half (58.5%) of overall systemic sclerosis had developed new digital ischemic complications during the 1-year follow-up. Those with digital ischemic complications at baseline were at high risk for developing new digital ischemic complications (odds ratio: 15.9). Diffuse cutaneous systemic sclerosis is associated with digital ischemic complications (odds ratio: 6.0), digital pitting scar (odds ratio: 4.9), and digital pulp loss (odds ratio: 6.4). Tendon friction rub is associated with digital pitting scar (odds ratio: 5.0). Salt-and-pepper skin appearance is associated with digital pulp loss (odds ratio: 3.0) and digital ulcer (odds ratio: 6.9). Disease duration > 3 years is associated with digital ulcer (odds ratio: 4.4). Male gender is associated with digital ulcer (odds ratio: 5.4).
Conclusion:
Digital pulp loss, digital pitting scar, digital ulcer, and digital amputation were common manifestations of digital ischemic complications, and diffuse cutaneous systemic sclerosis was the strongest of the six independent predictors.
Objectives:
This study aims to investigate the prevalence of persistent eosinophilia and associated organ complications in Thai patients with systemic sclerosis (SSc).
Patients and methods:
This ...post-hoc study included 107 adult patients (23 males, 84 females; mean age: 50.4±11.6 years; range, 18 to 79 years) diagnosed with SSc between November 2013 and June 2017. Eosinophilia was defined as an absolute eosinophil count of >500/μL or a percentage count of >7%. Eosinophil levels collected at every visit over one year were categorized as persistently high (PH), persistently low (PL), high-to-low (HL), low-to-high (LH), or variable levels (VL). The study compared variables between PH and non-PH (PL+HL+LH+VL) groups. The patients with baseline eosinophilia were also identified and compared with the non-eosinophilia group.
Results:
The median disease duration was 3.2 years. Of the patients, 79.4% had diffuse cutaneous SSc and 76.7% had anti-Scl-70 positivity. A total of 11.2%, 66.4%, 1.9%, 8.4%, and 12.1% of the patients were categorized into the PH, PL, HL, LH, and VL groups, respectively. Compared to non-PH groups, the PH group had a higher prevalence of anti-centromere antibody (ACA), higher baseline percent predicted total lung capacity, and lower baseline C-reactive protein and creatine phosphokinase (p<0.05 for all). The ACA positivity (odds ratio OR: 18.5; 95% confidence interval CI: 1.64-208.46) was associated with PH. The patients with baseline eosinophilia (17.8%) had a higher prevalence of non-specific interstitial pneumonia with periodic eosinophilia at the time of diagnosis (100% vs. 6.5%, p<0.0001; OR: 4.667; 95% CI: 1.712-12.724).
Conclusion:
The PH was seldom (11%) in patients with SSc compared to periodic eosinophilia, which was more prevalent (18%). It may be related to ACA positivity and better pulmonary outcomes, whereas periodic eosinophilia may involve interstitial lung disease.
Objective
This study was performed to determine the prevalence of elevated C‐reactive protein (CRP) levels and the significance of CRP in clinical parameters in systemic sclerosis (SSc; scleroderma) ...patients.
Methods
Canadian Scleroderma Research Group data were used. Statistical comparisons were made for CRP levels ≤8 mg/liter versus >8 mg/liter, early (≤3 years from first non–Raynaud's phenomenon symptom) versus late SSc, and diffuse cutaneous SSc (dcSSc) versus limited cutaneous SSc (lcSSc). A survival analysis was analyzed between patients with normal versus elevated CRP levels.
Results
A total of 1,043 patients (mean ± SD age 55.4 ± 12.1 years, mean ± SD disease duration of 11.0 ± 9.5 years) were analyzed; elevation of CRP level and erythrocyte sedimentation rate (ESR; >20 mm/hour) occurred in 25.7% and 38.2%, respectively. Mean ± SD baseline CRP level in dcSSc (11.98 ± 25.41 mg/liter) was higher than in lcSSc (8.15 ± 16.09 mg/liter; P = 0.016). SSc patients with an early disease duration had a higher mean ± SD CRP level (12.89 ± 28.13 mg/liter) than those with a late disease duration (8.60 ± 17.06 mg/liter; P = 0.041). Although not consistent in all subsets, CRP was significantly associated (P < 0.01) with ESR, modified Rodnan skin score (MRSS), worse pulmonary function parameters, disease activity, damage, and Health Assessment Questionnaire. CRP level seemed to normalize in many SSc patients over time. Total lung capacity <80% predicted, MRSS, and serum creatinine were predictors of elevated CRP levels in SSc (odds ratio OR 2.76 95% confidence interval (95% CI) 1.73–4.40, P = 0.0001; OR 1.03 95% CI 1.01–1.05, P = 0.005; and OR 1.005 95% CI 1.001–1.010, P = 0.02, respectively). Survival for patients with elevated CRP levels was less than for patients with normal CRP levels (P = 0.001).
Conclusion
CRP level is elevated in one‐quarter of SSc patients, especially early disease. It is correlated with disease activity, severity, poor pulmonary function, and shorter survival.
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