Clinical course and factors associated with progressive acro-osteolysis in early systemic sclerosis: a retrospective cohort study
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SUBMITTER: Thammaroj P
PROVIDER: S-EPMC10907566 | biostudies-literature | 2024 Jan
REPOSITORIES: biostudies-literature
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| S-EPMC10184007 | biostudies-literature
Project description:BackgroundCalcinosis cutis of hands can progress and impair hand function in systemic sclerosis (SSc). Understanding the natural disease and comprehensive management is crucial.ObjectiveTo examine clinical course and identify risk factors associated with progressive calcinosis cutis in early SSc.MethodsDual time-point hand radiography was performed at initial and after diagnosis at median interval (range 2.9 ± 0.4 years) in 53 recruited patients with early SSc. Progressive calcinosis cutis defined as the worsening of severity according to simple soring scoring system (no, mild, moderate, severe) comparing to previous hand radiography. Odds ratio (OR) and their 95%CI were used to evaluate associated factors and calcinosis cutis progression.ResultsA total of 35 cases (155 per 100 person-year), showed progressive calcinosis cutis with the incidence of 22.6 per 100-person-years (95%CI 16.2-31.4). The most common area of progressive calcinosis cutis was at right distal phalanx, 12 of 35 (22.6%). Although statistically not significant by logistic regression analysis, elderly patients, Raynaud's phenomenon, ischemic ulcer, telangiectasia, and salt-pepper tended to be more frequent in progressive calcinosis cutis than those who had no progression. Around one-quarter of those who had no calcinosis cutis experienced worsening across more than one level of severity.ConclusionProgression of calcinosis cutis in early SSc increased over time, particularly within 3 years after the first evaluation. Elderly patients and those with vasculopathy were found more frequently. Further study with a larger cohort is needed to support these findings.
| S-EPMC11755737 | biostudies-literature
Project description:ObjectiveTo evaluate the relationship between early relapse recovery and onset of progressive multiple sclerosis (MS).MethodsWe studied a population-based cohort (105 patients with relapsing-remitting MS, 86 with bout-onset progressive MS) and a clinic-based cohort (415 patients with bout-onset progressive MS), excluding patients with primary progressive MS. Bout-onset progressive MS includes patients with single-attack progressive and secondary progressive MS. "Good recovery" (as opposed to "poor recovery") was assigned if the peak deficit of the relapse improved completely or almost completely (patient-reported and examination-confirmed outcome measured ≥6 months post relapse). Impact of initial relapse recovery and first 5-year average relapse recovery on cumulative incidence of progressive MS was studied accounting for patients yet to develop progressive MS in the population-based cohort (Kaplan-Meier analyses). Impact of initial relapse recovery on time to progressive MS onset was also studied in the clinic-based cohort with already-established progressive MS (t test).ResultsIn the population-based cohort, 153 patients (80.1%) had on average good recovery from first 5-year relapses, whereas 30 patients (15.7%) had on average poor recovery. Half of the good recoverers developed progressive MS by 30.2 years after MS onset, whereas half of the poor recoverers developed progressive MS by 8.3 years after MS onset (p = 0.001). In the clinic-based cohort, good recovery from the first relapse alone was also associated with a delay in progressive disease onset (p < 0.001). A brainstem, cerebellar, or spinal cord syndrome (p = 0.001) or a fulminant relapse (p < 0.0001) was associated with a poor recovery from the initial relapse.ConclusionsPatients with MS with poor recovery from early relapses will develop progressive disease course earlier than those with good recovery.
| S-EPMC4553030 | biostudies-literature
Project description:ObjectiveSystemic sclerosis (SSc) is a rare and clinically heterogeneous autoimmune disorder characterised by fibrosis and microvascular obliteration of the skin and internal organs. Organ involvement mostly manifests after a variable period of the onset of Raynaud's phenomenon (RP). We aimed to map the incidence and predictors of pulmonary, cardiac, gastrointestinal (GI) and renal involvement in the early course of SSc.MethodsIn the EUSTAR cohort, patients with early SSc were identified as those who had a visit within the first year after RP onset. Incident SSc organ manifestations and their risk factors were assessed using Kaplan-Meier methods and Cox regression analysis.ResultsOf the 695 SSc patients who had a baseline visit within 1 year after RP onset, the incident non-RP manifestations (in order of frequency) were: skin sclerosis (75%) GI symptoms (71%), impaired diffusing capacity for monoxide<80% predicted (65%), DU (34%), cardiac involvement (32%), FVC<80% predicted (31%), increased PAPsys>40mmHg (14%), and renal crisis (3%). In the heart, incidence rates were highest for diastolic dysfunction, followed by conduction blocks and pericardial effusion. While the main baseline risk factor for a short timespan to develop FVC impairment was diffuse skin involvement, for PAPsys>40mmHg it was higher patient age. The main risk factors for incident cardiac manifestations were anti-topoisomerase autoantibody positivity and older age. Male sex, anti-RNA-polymerase-III positivity, and older age were risk factors associated with incident renal crisis.ConclusionIn SSc patients presenting early after RP onset, approximately half of all incident organ manifestations occur within 2 years and have a simultaneous rather than a sequential onset. These findings have implications for the design of new diagnostic and therapeutic strategies aimed to 'widen' the still very narrow 'window of opportunity'. They may also enable physicians to counsel and manage patients presenting early in the course of SSc more accurately.
| S-EPMC5051961 | biostudies-literature
Project description:Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is usually detected in a patient known to have SSc but may be diagnosed prior to SSc. We probed an insurance database to investigate documentation of ILD prior to SSc. Using Optum's Clinformatics® Data Mart Database, we identified patients with an SSc index date between January 1, 2010, and September 30, 2015, based on International Classification of Diseases (ICD)-9-Clinical Modification (CM) codes, ≥2 medical claims associated with SSc on different dates within 1 year, and ≥3 years of continuous enrollment prior to SSc index date (ICD-9-CM cohort). We identified an ICD-10-CM cohort comprising patients with an SSc index date between October 1, 2017, and June 30, 2019, based on ICD-10-CM codes, ≥2 medical claims associated with SSc on different dates within 1 year, and ≥2 years of continuous enrollment prior to SSc index date. ILD was defined as ≥2 medical claims associated with ILD on different dates. The ICD-9-CM and ICD-10-CM cohorts comprised 1779 and 1032 patients, respectively. In these cohorts, respectively, 7.6% and 9.3% of patients had their second medical claim associated with ILD prior to their SSc index date, and 4.3% and 5.6% of patients had their second medical claim associated with ILD >1 year prior to the SSc index date. In this analysis, 4% to 6% of patients with SSc had claims for ILD >1 year prior to a claim for SSc. These data show that SSc can affect the lung early and demonstrate the importance of screening patients with SSc for ILD and patients with ILD for SSc.
| S-EPMC9371507 | biostudies-literature
Project description:Digital ulcers in patients with systemic sclerosis (SSc) can be complicated by SSc-related osteomyelitis (SRO). The microbiological data and optimal management of SRO remain unclear. This single-center retrospective study involved patients with SSc aged 18 or older from April 2005 to March 2022. Diagnosis of SRO was based on clinical presentation and MRI findings. The accuracy of the superficial swab culture results was estimated using the bone culture as a reference. Temporal changes in local signs for up to a year were collected, and their association with (1) duration of antimicrobial therapy (> 6 weeks) or (2) surgical interventions was assessed using univariable analyses. Among the 2,126 patients, 46 (2.2%) were diagnosed with SRO. In seven patients whose swab and bone cultures were both available, two (28.6%) had swab cultures identifying all the organisms detected in bone cultures. Resolution of local inflammatory signs consistently preceded wound closure. Three months after therapy initiation, prolonged antimicrobial therapy was not significantly associated with the resolution of local inflammatory signs (16/19 [84.2%] vs. 12/14 [85.7%]; P = 1.00), and surgical intervention was not significantly associated with wound dehiscence (6/9 [66.7%] vs. 20/24 [83.3%]; P = 0.36). Superficial swab cultures may not reliably reflect the true causative organism of SRO. Prolonging antimicrobial therapy beyond six weeks may be of little benefit for patients with SRO when local inflammatory signs improve. Surgical intervention may be a safe and effective option for selected patients with SRO.
| S-EPMC11903529 | biostudies-literature
Project description:ObjectiveTo conduct an exploratory cluster analysis of systemic sclerosis patients from the baseline data of the Indian systemic sclerosis registry.MethodsPatients satisfying American College of Rheumatology-European League Against Rheumatism classification criteria for systemic sclerosis were included. The clusters formed using clinical and immunological parameters were compared.ResultsOf the 564 systemic sclerosis registry participants, 404 patients were included. We derived four clusters of which three were anti-topoisomerase I predominant and one was anti-centromere antibody 2 dominant. Cluster 1 (n-82 (20.3%)) had diffuse cutaneous systemic sclerosis patients with the most severe skin disease, anti-topoisomerase I positivity, males, younger age of onset and high prevalence of musculoskeletal, vasculopathic and gastrointestinal features. Cluster 2 (n-141 (34.9%)) was also diffuse cutaneous systemic sclerosis and anti-topoisomerase I predominant but with less severe skin phenotype than cluster 1 and a lesser prevalence of musculoskeletal, vasculopathic and gastrointestinal features. Cluster 3 (n-119 (29.5%)) had limited cutaneous systemic sclerosis patients with anti-topoisomerase I positivity along with other antibodies. The proximal muscle weakness was higher and digital pitting scars were lower, while other organ involvement was similar between clusters 2 and 3. Cluster 4 (n-62 (15.30%)) was the least severe group with limited cutaneous systemic sclerosis and anti-centromere antibody predominance. Age of onset was higher with low musculoskeletal disease and a higher presence of upper gastrointestinal features. The prevalence of interstitial lung disease was similar in the three anti-topoisomerase I predominant clusters.ConclusionWith exploratory cluster analysis, we confirmed the possibility of subclassification of systemic sclerosis along a spectrum based on clinical and immunological characteristics. We also corroborated the presence of anti-topoisomerase I in limited cutaneous systemic sclerosis and the association of interstitial lung disease with anti-topoisomerase I.
| S-EPMC10848923 | biostudies-literature
Project description:IntroductionAlthough aspiration pneumonia is the most common complication of progressive supranuclear palsy (PSP), the clinical impact of aspiration pneumonia on disease course and survival has not been fully estimated. Thus, we retrospectively analyzed the prognostic factors and clinical consequences of pneumonia in PSP.MethodsThe clinical course of patients with aspiration pneumonia was surveyed. The association between baseline clinical features (2 years from disease onset) and latency to the initial development of pneumonia was investigated using survival time and Cox regression analyses.ResultsNinety patients with a clinical diagnosis of PSP were observed for 5.1±3.8 years (mean±SD), and 22 had aspiration pneumonia. Subsequently, 20 patients (91%) had to discontinue oral feeding entirely and 13 (59%) died, whereas, of 68 patients without pneumonia, only three patients (4%) died. Time to initial development of pneumonia was strongly correlated with survival time (Spearman R = 0.92, P<0.001), with a mean latency of 2.3 years to death. Among baseline clinical features, early fall episodes and cognitive decline were significant predictors of pneumonia (P = 0.001 and P<0.001, respectively, log rank test). Cox regression analysis demonstrated that early fall episodes (adjusted hazard ratio: 3.9, 95% confidence interval: 1.2-12.5, P = 0.03) and cognitive decline (adjusted hazard ratio: 5.2, 95% confidence interval: 1.4-19.3, P = 0.02) independently predicted pneumonia. By contrast, dysphagia was not associated with pneumonia (P = 0.2, log rank test).ConclusionInitial development of pneumonia indicates an unfavorable clinical course and predicts survival time (mean survival time 2.3 years). Patients with early falls and cognitive decline were at high risk of early development of pneumonia.
| S-EPMC4536232 | biostudies-literature
Project description:OBJECTIVES:To assess the incidence of head injury and predictors of complication across the care continuum. DESIGN:Retrospective cohort study using data from a research network. We calculated the incidence of overall head injury in a longitudinal cohort covering 1-year interval (31 369 patient-years), and the incidence of complicated head injury in a longitudinal cohort covering 10 years interval (220 352 patient-ears). Incidence rates were calculated per 1000 patient-years with 95% CI using the Mid-P exact test. We calculated ORs to assess potential risk factors for a complicated head injury. SETTING:A practice-based research network covering a population of >30 000 patients. PARTICIPANTS:All patients listed in practices within the research network during the years 2005-2014. MAIN OUTCOME MEASURES:Incidence of (complicated) head injury and predictors for clinical complications. RESULTS:The incidence of overall head injury was 22.1 per 1000 person-years and the incidence of a complicated course following head injury was 0.16 per 1000 person-years. The following determinants were risk factors for a complicated course: high energy trauma, bicycle accident, traffic accident in general, use of anticoagulants, alcohol intoxication, age above 60 years and low Glasgow Coma Scale at initial presentation. A complicated course was very unlikely when the patients' first encounter with a healthcare professional was in primary care (OR 0.03, 95% CI 0.01 to 0.07). CONCLUSIONS:Complication after head injury are rarely seen in general practice. Patients who do experience complications are often easily identifiable as requiring specialist care. A more reserved referral policy for general practice may be desirable, suggesting that current guidelines are too defensive.
| S-EPMC5988084 | biostudies-literature
Project description:ObjectiveTo develop a model that assesses the risk for progressive disease in patients with systemic sclerosis (SSc) over the short term, in order to guide clinical management.MethodsBaseline characteristics and 1 year follow-up results of 163 patients with SSc referred to a multidisciplinary healthcare programme were evaluated. Progressive disease was defined as: death, ≥10% decrease in forced vital capacity, ≥15% decrease in diffusing capacity for carbon monoxide, ≥10% decrease in body weight, ≥30% decrease in estimated-glomerular filtration rate, ≥30% increase in modified Rodnan Skin Score (with Δ≥5) or ≥0.25 increase in Scleroderma Health Assessment Questionnaire. The number of patients with progressive disease was determined. Univariable and multivariable logistic regression analyses were used to assess the probability of progressive disease for each individual patient. Performance of the prediction model was evaluated using a calibration plot and area under the receiver operating characteristic curve.Results63 patients had progressive disease, including 8 patients who died ≤18 months after first evaluation. Multivariable analysis showed that friction rubs, proximal muscular weakness and decreased maximum oxygen uptake as % predicted, adjusted for age, gender and use of immunosuppressive therapy at baseline, were significantly associated with progressive disease. Using the prediction model, the predicted chance for progressive disease increased from a pretest chance of 37% to 67-89%.ConclusionsUsing the prediction model, the chance for progressive disease for individual patients could be doubled. Friction rubs, proximal muscular weakness and maximum oxygen uptake as % predicted were identified as relevant parameters.
| S-EPMC4680735 | biostudies-literature