PCV5 Clinical Characteristics of Patients with Transthyretin Amyloid Cardiomyopathy (ATTR-CM) in France: EPACT, a Study Based on the French Nationwide Claims Database Snds

Abstract Background Hereditary transthyretin amyloid cardiomyopathy (ATTR-CM) is an increasingly recognized progressive cardiomyopathy with heterogenous clinical manifestations that lead to its misdiagnosis and poor prognosis. This study was performed to describe the clinical characteristics and natural history of Chinese patients to improve clinical awareness of this condition. Methods In this study, we retrospectively investigated 23 patients with a confirmed diagnosis of hereditary ATTR-CM in Peking Union Medical College hospital from From January 1, 2000 to December 31, 2018. Results In all, 16 patients (69.6%) were males, the median age at disease onset was 45 (33,55) years old. The median duration from symptom onset to diagnosis was 30 (18,46) months. Phenotypes were classified as exclusively cardiac (n = 1, 4.3%) and mixed type (n = 22, 95.6%). The common mutations were Gly47Arg (7 patients [30.4%]) and Val30Ala (3 patients [13%]). Ventricular hypertrophy was observed in 23 (100%) patients, the mean thickness of the ventricular septum was 16.1 ± 3.9 mm, the mean thickness of the left ventricular posterior wall was 15.1 ± 2.8 mm. The mean left ventricle ejection fraction (LVEF) was 57.3 ± 11.9% and only 5 patients (21.7%) had LVEF < 50%. 18 (78.3%) patients had abnormal electrocardiography and the most common feature was pseudoinfarct pattern (56.5%). Overall survival at 12, 24, 36, 48, and 60 months after diagnosis was 77.8, 55.6, 38.9, 27.8, and 11.1%, respectively. Survival was better in patients with EF ≥50% than in those with EF < 50% [log Rank (Mantel-Cox), χ2 = 4.03, P = 0.045]. Conclusions The clinical characteristics of ATTR are heterogeneous: men are more likely to be affected and onset symptoms are not obvious in the heart and mainly include peripheral neuropathy and autonomic neuropathy; however, LV hypertrophy, especially a thick ventricular septum and posterior wall with preserved LVEF, are often detected on echocardiography. Abnormal ECG manifestations are common. The prognosis is poor, and patients with EF > 50% have better survival. Clinicians should be more aware of the complex clinical profile of ATTR amyloidosis to avoid misdiagnosis in practice.

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PCV67 Epidemiology of Transthyretin Amyloid Cardiomyopathy (ATTR-CM) in France: EPACT, a Study Based on the French Nationwide Claims Database Snds

Value in Health ◽

10.1016/j.jval.2020.08.558 ◽

2020 ◽

Vol 23 ◽

pp. S498-S499

Author(s):

T. Damy ◽

G. Bourel ◽

M. Slama ◽

V. Algalarrondo ◽

O. Lairez ◽

...

Keyword(s):

Claims Database ◽

Amyloid Cardiomyopathy

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Prevalence of Vaso-Occlusive Crises in Patients with Sickle Cell Disease: A Retrospective US Claims Database Analysis

Blood ◽

10.1182/blood-2020-140756 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 8-9

Author(s):

Abiola Oladapo ◽

Elyse Swallow ◽

Allison Briggs ◽

Miriam L. Zichlin ◽

Bjorn L Mellgard

Keyword(s):

Health Care ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Clinical Characteristics ◽

Cell Disease ◽

Publicly Traded ◽

Database Analysis ◽

Claims Database ◽

Blood Disorder ◽

The Mean

Introduction: Sickle cell disease (SCD) is an inherited blood disorder affecting ~100,000 individuals in the US. SCD is considered a chronic, lifelong condition that requires comprehensive management. Vaso-occlusive crises (VOCs) are the most common complications of SCD, resulting in intense pain and potential irreversible organ damage. The objective of this study was to characterize the demographic and clinical characteristics of patients with SCD. Methods: A retrospective database analysis was conducted using data from the IBM MarketScan Commercial Claims and Medicare-Supplemental Claims database (July 1, 2013 to June 30, 2018). Patients were included if they met the following criteria: ≥2 diagnoses of SCD on different claims between July 1, 2013 and January 1, 2017, ≥6 years of age on January 1, 2017, and continuous enrollment throughout the 1-year study period (January 1, 2017 to December 31, 2017). Descriptive statistics were used to assess patient demographics (age and sex) and clinical characteristics (Charlson Comorbidity Index [CCI] and other selected comorbidities). In addition, the following outcomes were assessed: the proportion of patients who experienced ≥1 VOC, the frequency of VOCs by care setting, the duration of inpatient VOCs, the monthly VOC risk, and the time between subsequent VOCs. Results: A total of 8174 patients met the inclusion criteria. The mean (± standard deviation [SD]) age was 40.8 (±19.5) years and 63.5% of the patients were female. The mean (±SD) CCI was 0.6 (±1.3), with chronic pulmonary disease, diabetes, renal disease, cerebrovascular disease, and stroke identified as the most common comorbidities. Approximately 20% (n=1659) of patients experienced ≥1 VOC and the mean monthly VOC risk was 0.07 (±0.19). Among patients with ≥1 VOC, the mean (±SD) number of VOCs was 5.2 (±7.7) and the median (interquartile range) time from first to second VOC was 2.4 (0.5-8.2) months. Approximately 18% (n=1461) of patients experienced ≥1 VOC managed in an outpatient setting and 10% (n=844) of patients experienced ≥1 VOC managed in an inpatient setting. Approximately 8% (n=646) of patients experienced ≥1 inpatient and ≥1 outpatient VOC during the study period. Among patients with ≥1 outpatient VOC, the mean (±SD) number of outpatient VOCs was 4.6 (±6.9); among patients with ≥1 inpatient VOC, the mean (±SD) number of inpatient VOCs was 2.2 (±2.3) and the mean (±SD) inpatient VOC duration was 6.6 (±6.4) days. Conclusions: VOCs are common complications of SCD, affecting a subset of patients who often experience recurrent VOC episodes requiring professional health care. VOCs are associated with a significant disease burden on the patient and, potentially, the health care system. Disclosures Oladapo: Takeda:Current Employment, Current equity holder in publicly-traded company.Swallow:Analysis Group, Inc.:Current Employment.Briggs:Analysis Group, Inc.:Current Employment.Zichlin:BMS:Other: Employee of Analysis Group Inc., which received consulting fees.Mellgard:Baxalta US Inc., a Takeda company:Current Employment, Current equity holder in publicly-traded company.

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Demographic and clinical characteristics of patients with metastatic colorectal cancer (mCRC) associated with RAS testing from 129 million covered lives.

Journal of Clinical Oncology ◽

10.1200/jco.2017.35.4_suppl.739 ◽

2017 ◽

Vol 35 (4_suppl) ◽

pp. 739-739

Author(s):

Bruce A. Bach ◽

Alexandra Christodoulopoulou ◽

Andrew Klink ◽

Guy Hechmati ◽

Urvi Mujumdar ◽

...

Keyword(s):

Colorectal Cancer ◽

Metastatic Colorectal Cancer ◽

Clinical Characteristics ◽

Patient Characteristics ◽

Health Claims ◽

Chi Square ◽

Claims Database ◽

Ras Mutations ◽

Commercial Insurance ◽

Patient Demographics

739 Background: Guidelines for the treatment of patients with metastatic colorectal cancer (mCRC) recommend that all tumors be tested for RAS mutations to identify patients who will most benefit from chemotherapy regimens with biologic agents. The aim of this study was to describe characteristics of patients captured by a large national health claims database who were tested vs those who were not tested for RAS mutations and to identify potential predictors of RAS testing. Methods: Patients with mCRC diagnosed from 2012–2014 (ICD-9 codes 153.x, 154.0x, or 154.1x and 197.x–198.x) were identified from a US healthcare claims database consisting of 129 million unique covered patient lives. Patient demographics and baseline clinical characteristics were compared between patients who were and were not tested for RAS mutations (identified by CPT codes) using chi-square and t-tests. Odds ratios (ORs) were estimated for potential predictors by fitting multivariate logistic regressions to adjust for patient characteristics. Results: Of the 4,527 mCRC patients identified (mean age at diagnosis, 61.2 years; 54% male); 39% (n = 1,787) had a claim for RAS testing during the study period. Patients tested had similar mean age and comorbidity index at diagnosis compared to those not tested (60.8 vs 61.5 years, P = 0.06; 3.98 vs 3.90, P = 0.47). A greater proportion of RAS-tested patients were commercially insured compared to those not tested (37% vs 26%, P < 0.01). Adjusted for other patient characteristics, patients with commercial insurance (OR: 1.62 [95% confidence interval–CI: 1.41–1.87]), metastases to viscera (OR: 1.61 [95% CI: 1.42–1.84]), or diagnosis of colon primary site compared to rectum (OR: 1.32 [95% CI: 1.16–1.51]) had increased odds of receiving RAS testing; all P < 0.01. Conclusions: These observational data show that mCRC patients with commercial insurance, metastases to viscera, and colon cancer have a higher probability of being tested for RAS mutations. Overall, the data suggest that RAS status is underdetermined, with certain subgroups appearing to be more likely to be tested than others.

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Clinical characteristics and natural history of wild‐type transthyretin amyloid cardiomyopathy in Japan

ESC Heart Failure ◽

10.1002/ehf2.12884 ◽

2020 ◽

Vol 7 (5) ◽

pp. 2829-2837

Author(s):

Toshihiro Yamada ◽

Seiji Takashio ◽

Yuichiro Arima ◽

Masato Nishi ◽

Mami Morioka ◽

...

Keyword(s):

Natural History ◽

Clinical Characteristics ◽

Wild Type ◽

History Of ◽

Amyloid Cardiomyopathy

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Clinical characteristics and comorbidities in adult-onset Still’s disease using a large US administrative claims database

Rheumatology ◽

10.1093/rheumatology/kez622 ◽

2020 ◽

Vol 59 (7) ◽

pp. 1725-1733 ◽

Cited By ~ 1

Author(s):

Aleksander Lenert ◽

GYeon Oh ◽

Michael J Ombrello ◽

Sujin Kim

Keyword(s):

Clinical Characteristics ◽

Adult Onset Still’S Disease ◽

Administrative Claims ◽

Adult Onset ◽

Still’S Disease ◽

Claims Database ◽

Still's Disease ◽

Icd 10 ◽

Yamaguchi Criteria ◽

Adult Onset Still's Disease

Abstract Objectives We aimed to describe clinical characteristics, treatment patterns and major comorbidities of a US-based adult-onset Still’s disease (AOSD) cohort. Methods Administrative claims data from Truven MarketScan were collected from 2009 to 2015. An AOSD case was defined as ≥1 M06.1 International Classification of Diseases 10th revision (ICD-10) medical claim code. We extracted data for the AOSD cohort (n = 106) and 1:5 matched controls (n = 530) without AOSD. Outcomes of interest and a novel claims-based set of Yamaguchi criteria were identified by relevant ICD 9th revision (ICD-9) and ICD-10 codes. Bivariate descriptive analyses were conducted on all variables. Comorbidity rates and rate ratios were calculated in AOSD cases and matched controls. Statistical significance of cohort differences was determined to compare AOSD cases and matched controls. Results The AOSD cohort, with a mean age of 43.08 (standard deviation, s.d. 13.9) years and with female predominance (68.9%) was observed over a mean of 750.12 (637.6) days. A total of 35.9% of AOSD patients fulfilled claims-based Yamaguchi criteria compared with 0.4% matched controls (P< 0.05). We identified severe AOSD-related complications, including macrophage activation syndrome (4.7%) and acute respiratory distress syndrome (12.3%). Treatment commonly involved systemic glucocorticoids (62.2%), MTX (51%) and anakinra (24.5%). Compared with matched controls, serious infections were significantly increased (rate ratio 2.58, 95% CI: 1.53, 4.37, P = 0.0004), while hyperlipidaemia (0.54, 95% CI: 0.35, 0.85; P = 0.008) and obesity (0.30, 95% CI: 0.15, 0.62; P = 0.001) were significantly decreased in AOSD patients. Conclusion We characterized a first US-based AOSD cohort using a large national administrative claims database, and identified key complications, treatments and comorbidities.

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