Characterizing Disease Manifestations and Treatment Patterns Among Adults with Systemic Sclerosis: A Retrospective Analysis of a US Healthcare Claims Population

Gale, Sara; Trinh, Huong; Mathew, Nitya; Jahreis, Angelika; Lin, Celia J F; Sarsour, Khaled

doi:10.1007/s40744-019-00181-8

Cited by 10 publications

(7 citation statements)

References 31 publications

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“…During the whole study period, 42.5% of the SSc cohort and 45.0% of the SSc-ILD cohorts started a stable immunomodulatory regimen [12]. In another US healthcare claims database-based study, 30.8% of patients with SSc received immunomodulatory therapy during the first year after diagnosis [29]. Comparatively in Japan, immunomodulatory therapy was used infrequently.…”

Section: Discussionmentioning

confidence: 99%

Incidence Rate and Prevalence of Systemic Sclerosis and Systemic Sclerosis-Associated Interstitial Lung Disease in Japan: Analysis Using Japanese Claims Databases

et al. 2022

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Introduction: Systemic sclerosis (SSc) is a complex autoimmune disease with increased mortality, and interstitial lung disease (ILD) is a major cause of death. There are no recent epidemiological data on SSc and SSc-associated ILD (SSc-ILD) in Japan and little is known about how patients with these diseases are treated. Methods: The incidence rate and prevalence of SSc and SSc-ILD in Japan were estimated using the Japanese Medical Data Centre (JMDC) database. The demographic and clinical characteristics of patients and the immunomodulatory medications they received were also assessed using JMDC and the Medical Data Vision (MDV) databases. All analyses were descriptive. Results: The overall incidence rates of SSc and SSc-ILD per 100,000 person-years were 6.6 (95% confidence interval [CI] 6.2-7.1) and 1.9 (95% CI 1.6-2.1), respectively, and the overall prevalence per 100,000 persons was 37.0 (95% CI 35.6-38.5) and 13.9 (95% CI 13.0-14.8), respectively. ILD was the most common comorbidity in patients with SSc present in approximately 30% of patients (JMDC, 29.3%; MDV, 30.1%). The immunomodulatory medications prescribed were similar in patients with SSc and SSc-ILD, and each of the medications in this analysis was prescribed in less than 15% of patients. Conclusion:We have demonstrated that estimates of prevalence and incidence rates of SSc and SSc-ILD in Japan are comparable to similar database studies conducted in the USA, using a medical claims database. Only a small proportion of patients were receiving immunomodulatory treatments, suggesting undertreatment in Japan.

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Section: Discussionmentioning

confidence: 99%

Incidence Rate and Prevalence of Systemic Sclerosis and Systemic Sclerosis-Associated Interstitial Lung Disease in Japan: Analysis Using Japanese Claims Databases

et al. 2022

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“…These have been effective in preventing the progression of skin thickness and ILD; therefore, they have been proposed as the main treatments for SSc [33,34]. In the United States, 30.8% of patients with SSc receive immunomodulatory therapy during the rst year after diagnosis [35]. In Korea, we found that only a small number of patients were treated with these drugs, presumably because they were not covered by national health insurance.…”

Section: Discussionmentioning

confidence: 87%

Characteristics, mortality, and risk factors in patients with systemic sclerosis-associated interstitial lung disease

Jang

Woo

Kim

et al. 2022

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Background Systemic sclerosis (SSc) is a heterogeneous autoimmune disease characterized by the dysregulation of fibroblast function, causing multi-organ damage, which often involves the lungs. Combined interstitial lung disease (SSc-ILD) is a major cause of death among patients with SSc. Our study aimed to identify the risk factors for mortality and compare the clinical characteristics of patients with SSc-ILD. Methods Patients were retrospectively enrolled between 2010 and 2018 in a tertiary hospital in Korea. Patients with SSc-ILD were classified into two groups depending on the first pulmonary function test or radiologic findings: extensive (n = 46, forced vital capacity [FVC] < 70% or > 20% disease extent on CT scan) or limited (n = 60, FVC ≥ 70% or < 20% disease extent on CT scan). Results Patients in the extensive group were younger (mean age ± SD 49.3 ± 11.5) than those in the limited group (53.9 ± 12.5, p = 0.067). The extensive group showed frequent pulmonary hypertension (43.5% vs. 16.7%, p = 0.009), higher erythrocyte sedimentation rate (61.3 ± 33.7 vs 42.1 ± 26.0, p = 0.003), and mortality (32.6% vs. 10.0%, p = 0.011). ILD was detected within five years from the first visit (mean number of years 3.2 ± 3.9 vs. 4.3 ± 5.5, survivors vs. non-survivors), and mortality occurred in 19.8% of all patients during a 15-year follow-up. Older age, lower FVC, and combined malignancy were associated with mortality, but FVC decline was similar in the limited and extensive groups, such as 15–20% in the first year and 8–10% in the next year, regardless of the initial extent of the disease. Conclusions Patients with SSc-ILD had a heterogeneous disease course. Approximately 10% of the patients in the limited group showed progression, which was similar to the proportion of patients in the extensive group. ILD was detected within the first five years of the total follow-up period; therefore, it is necessary to carefully monitor patients’ symptoms and signs from the early stage. Long-term surveillance is also required.

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“…These have been effective in preventing the progression of skin thickness and ILD; therefore, they have been proposed as the main treatments for SSc [ 43 , 44 ]. In the United States, 30.8% of patients with SSc receive immunomodulatory therapy during the first year after diagnosis [ 45 ]. In Korea, only a small number of patients were found to be treated with these drugs, presumably because they were not covered by national health insurance.…”

Section: Discussionmentioning

confidence: 99%

Characteristics and risk factors of mortality in patients with systemic sclerosis-associated interstitial lung disease

et al. 2023

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Background Systemic sclerosis (SSc) is a heterogeneous autoimmune disease characterized by dysregulation of fibroblast function, which often involves the lungs. Interstitial lung disease (ILD) associated with SSc (SSc-ILD) is a major cause of death among patients with SSc. Our study aimed to identify risk factors for mortality and compare the clinical characteristics of patients with SSc-ILD. Patients and methods Patients were retrospectively enrolled between 2010 and 2018 in a tertiary hospital in Korea. Patients with SSc-ILD were classified depending on the first pulmonary function test or radiologic findings: extensive ( n = 46, >20% disease extent on computed tomography (CT) or forced vital capacity [FVC] < 70% in indeterminate cases) and limited ( n = 60, <20% disease extent on CT or FVC ≥70% in indeterminate cases). Results Patients in the extensive group were younger (mean age ± SD 49.3 ± 11.5) than those in the limited group (53.9 ± 12.5, p = .067) at diagnosis. The extensive group showed frequent pulmonary hypertension (43.5% vs. 16.7%, p = .009) and higher erythrocyte sedimentation rate (61.3 ± 33.7 vs. 42.1 ± 26.0, p = .003) and mortality (32.6%, mean duration of follow-up, 100.0 ± 44.7 months vs. 10.0%, 86.0 ± 53.4 months, p = .011). ILD was detected within five years from the first visit (median years 3.5 (1.0, 6.0) vs. 4.5 (0.6, 9.0), survivors vs. non-survivors), and mortality occurred in 19.8% of all patients during a 15-year follow-up. Older age, lower FVC, and initial disease stage (limited or extensive) were associated with mortality, but FVC decline was similar in the limited and extensive groups, such as 15–20% in the first year and 8–10% in the next year, regardless of the initial extent of the disease. Conclusions Approximately 10% of patients with SSc-ILD in the limited and extensive group showed progression. ILD was detected at a median of less than five years from the first visit; therefore, it is necessary to carefully monitor patients’ symptoms and signs from an early stage. Long-term surveillance is also required. Key messages Patients with systemic sclerosis-interstitial lung disease manifested a heterogeneous disease course. Approximately 10% of the patients in the limited group showed progression, which was similar to the proportion of patients in the extensive group. Interstitial lung disease was detected at a median of less than five years from the first visit.

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Characterizing Disease Manifestations and Treatment Patterns Among Adults with Systemic Sclerosis: A Retrospective Analysis of a US Healthcare Claims Population

Cited by 10 publications

References 31 publications

Incidence Rate and Prevalence of Systemic Sclerosis and Systemic Sclerosis-Associated Interstitial Lung Disease in Japan: Analysis Using Japanese Claims Databases

Incidence Rate and Prevalence of Systemic Sclerosis and Systemic Sclerosis-Associated Interstitial Lung Disease in Japan: Analysis Using Japanese Claims Databases

Characteristics, mortality, and risk factors in patients with systemic sclerosis-associated interstitial lung disease

Characteristics and risk factors of mortality in patients with systemic sclerosis-associated interstitial lung disease

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