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

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. Incidence Rate and Prevalence of Systemic Sclerosis and Systemic Sclerosis-associated Interstitial Lung Disease in Japan: Analysis Using Japanese Claims Databases-A Video Abstract. (MP4 68892 KB).

Experience from Asian centers in a named-patient-use program for afatinib in patients with advanced non-small-cell lung cancer who had progressed following prior therapies, including patients with uncommon EGFR mutations.

BACKGROUND: This study evaluated outcomes among patients with advanced/metastatic non-small-cell lung cancer (NSCLC) treated at Asian centers participating in the global named-patient-use (NPU) program for afatinib. METHODS: Patients had progressed after initial benefit with erlotinib or gefitinib, and/or had an EGFR or HER2 mutation, had no other treatment options, and were ineligible for afatinib trials. The recommended starting dose of afatinib was 50 mg/day. Dose modifications were allowed, and afatinib was continued as long as deemed beneficial. Response and survival information was provided voluntarily. Safety reporting was mandatory. RESULTS: 2242 patients (26% aged ≥ 70 years, 96% with adenocarcinoma) received afatinib at centers in 10 Asian countries. Most were heavily pre-treated, including prior treatment with erlotinib or gefitinib. Of 1281 patients tested, 1240 had EGFR mutations (common: 1034/1101; uncommon: 117/1101). There were no new safety signals, the most common adverse events being rash and diarrhea. Objective response rate (ORR) was 24% overall (n = 431 with data available), 27% for patients with common EGFR mutations (n = 230) and 28% for those with uncommon mutations (n = 32); median time to treatment failure (TTF) in these groups was 7.6 months (n = 1550), 6.4 months (n = 692) and 8.4 months (n = 83), respectively. In patients with EGFR exon 20 insertions (n = 23) and HER2 mutations (n = 12), median TTF exceeded 12 months. CONCLUSIONS: Patient outcomes in this study were similar to those reported in the analysis of the global NPU. Afatinib achieved clinical benefits in patients with refractory NSCLC. ORR and TTF were similar between patients with tumors harboring uncommon and common EGFR mutations.

Disease frequency, patient characteristics, comorbidity outcomes and immunosuppressive therapy in systemic sclerosis and systemic sclerosis-associated interstitial lung disease: a US cohort study.

OBJECTIVES: To investigate prevalence estimates and incidence rates (IRs) for SSc and SSc-associated interstitial lung disease (SSc-ILD) cohorts and describe patient characteristics, immunosuppressive therapy (IST) and comorbid outcomes among incident SSc and SSc-ILD cohorts. METHODS: Data were obtained from the US IBM MarketScan (2008-2017) claims database using algorithms developed with expert consultation. For the SSc cohort, newly diagnosed patients (aged ≥18 years) had one or more diagnostic claim for SSc. For the SSc-ILD cohort, patients had an additional ILD claim. Sensitivity analyses using two or more claims or alternative ILD diagnostic codes were also conducted. RESULTS: When requiring one or more diagnostic claim, the prevalence of SSc and SSc-ILD per 100 000 persons was 72.1 and 19.0. The IR for SSc and SSc-ILD per 100 000 person-years was 18.3 and 4.3. Sensitivity analyses requiring two or more claims yielded much lower prevalence (SSc: 41.5; SSc-ILD: 13.3) and IR (SSc: 8.8; SSc-ILD: 1.6) estimates. Patients with SSc-ILD were older, with increased comorbidities and diagnostic procedures at baseline. MTX and MMF were the most common ISTs; 12.7% of the SSc-ILD cohort received therapy at baseline vs 8.2% for SSc. A total of 42.5% and 45.0% of the SSc and SSc-ILD cohorts, respectively, started a stable IST regimen and 21.7% and 19.4% of these had an escalation. Skin disorders were the most common comorbid outcome in both cohorts during follow-up. CONCLUSIONS: SSc, with or without associated ILD, is a rare disease in the US. Newly diagnosed patients with SSc-ILD had received more IST and had more comorbidities compared with newly diagnosed SSc.

Healthcare Resource Utilization Among Patients in England with Systemic Sclerosis-Associated Interstitial Lung Disease: A Retrospective Database Analysis.

INTRODUCTION: Systemic sclerosis-associated interstitial lung disease (SSc-ILD) places a substantial burden on patients and healthcare systems. The objectives of this study were to describe clinical characteristics and assess healthcare resource utilization and costs of patients with SSc-ILD in England, compared with patients with non-pulmonary organ involvement related to SSc (SSc-OOI). METHODS: This population-based retrospective study used data from the Clinical Practice Research Datalink linked to Hospital Episode Statistics. Data were extracted from medical records dated January 1, 2005 to March 31, 2016. Patients with SSc were identified and placed in subgroups based on organ involvement: SSc-ILD, SSc-OOI, and both (SSc-ILD-OOI). Patients with SSc-ILD-OOI were included in both the SSc-ILD and SSc-OOI subgroups. All-cause healthcare costs, excluding medication costs, were calculated to 2016 British pounds sterling (£). RESULTS: This study included 675 patients with SSc: 174 (26%) had neither ILD nor other organ involvement (OOI); 127 (19%) had SSc-ILD; 477 (71%) had SSc-OOI; 103 (15%) had SSc-ILD-OOI. Age-weighted median [interquartile range (IQR)] annual healthcare costs per patient were: £1496 (£664-£2817) in SSc only; £6375 (£3451-£15,041) in SSc-ILD; £4084 (£1454-£10,105) in SSc-OOI; £6632 (£4023-£17,009) in SSc-ILD-OOI. In multivariate analysis, older age at diagnosis, diagnosis of anemia, and number of comorbid diseases were associated with higher yearly healthcare costs. CONCLUSION: The annual healthcare cost for patients with SSc-ILD is substantial, and higher than that of patients with SSc-OOI or SSc only. These results quantify the economic burden of SSc-ILD in a real-world setting, and highlight the need for treatment of this disease.

SSc is a rare disease that causes fibrosis, or thickening, of the skin. In some patients, SSc can also affect the lungs ('SSc-ILD') or other organs, e.g., the heart ('SSc-OOI'). Patients with SSc-ILD typically have high healthcare costs; however, it is not clear how costs for SSc-ILD compare with those for SSc-OOI. To investigate this, we evaluated the costs associated with SSc-ILD and compared them with those for SSc only or SSc-OOI. In this England-based study, the annual healthcare costs for patients with SSc-ILD were approximately 50% higher than for those without lung disease (SSc only) or SSc-OOI. These results highlight the importance of promptly diagnosing and treating patients with lung fibrosis complicating SSc.

Incidence of lower extremity amputations among patients with type 1 and type 2 diabetes in the United States from 2010 to 2014.

AIM: To compare the incidence of lower extremity amputation (LEA) among patients with type 1 diabetes (T1D) and patients with type 2 diabetes (T2D) with those without diabetes using US commercial claims and to assess the presence of key co-morbidities and precipitating factors at the time of the LEA. METHODS: Cohorts were defined via IBM MarketScan research databases for beneficiaries with T1D and T2D during 2010-2014. For each T1D and T2D patient, one patient without a prior diabetic claim matched on calendar time, sex and age, was randomly selected. Multivariable Cox proportional hazards models were used to estimate adjusted hazard ratios (aHRs) and 95% confidence intervals. RESULTS: Among the matched cohorts of 120 129 T1D patients and 1.7 million T2D patients, the incidence of LEA was higher among patients with T1D than patients with T2D, with the most frequent cases being minor LEAs (4.85 and 1.53 per 1000 patient years [PY], respectively), largely toe amputations (4.49 and 1.43 per 1000 PY, respectively). Compared with non-diabetic patients matched on age, sex and calendar time, T1D and T2D patients had more co-morbidities and a higher incidence of LEA (6.02 vs. 0.14 per 1000 PY; aHR, 22.47 [16.42-30.73] and 1.90 vs. 0.23 per 1000 PY; aHR, 4.64 [4.32-4.98]). CONCLUSIONS: Our data showed a higher incidence of LEA, especially minor LEA, in patients with T1D and T2D compared with those without diabetes, with a greater risk among patients with T1D than patients with T2D. Accounting for known and measurable risk factors for LEA reduced the relative hazard by nearly 50%; the majority of LEA cases were minor LEAs and toe amputations.