Citations

Objective To describe the methodology used to develop new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIMs) and their major subgroups. Methods An international, multidisciplinary group of myositis experts produced a set of 93 potentially relevant variables to be tested for inclusion in the criteria. Rheumatology, dermatology, neurology and paediatric clinics worldwide collected data on 976 IIM cases (74% adults, 26% children) and 624 non-IIM comparator cases with mimicking conditions (82% adults, 18% children). The participating clinicians classified each case as IIM or non-IIM. Generally, the classification of any given patient was based on few variables, leaving remaining variables unmeasured. We investigated the strength of the association between all variables and between these and the disease status as determined by the physician. We considered three approaches: (1) a probability-score approach, (2) a sum-of-items approach criteria and (3) a classification-tree approach. Results The approaches yielded several candidate models that were scrutinised with respect to statistical performance and clinical relevance. The probability-score approach showed superior statistical performance and clinical practicability and was therefore preferred over the others. We developed a classification tree for subclassification of patients with IIM. A calculator for electronic devices, such as computers and smartphones, facilitates the use of the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria. Conclusions The new EULAR/ACR classification criteria provide a patient’s probability of having IIM for use in clinical and research settings. The probability is based on a score obtained by summing the weights associated with a set of criteria items.

177 Background: Dignity Therapy (DT) is a psychosocial intervention used primarily at the end of life to improve quality of life and promote patient dignity, but many individuals are unable to complete it due to health decline/death. In addition, when individuals face potential mortality, they evaluate life plans to determine tasks and behaviors most important to them. Consistent with guidelines to provide palliative care interventions simultaneously with cancer treatment, the purpose of this study was to evaluate initial patient-reported outcomes of an intervention combination (Dignity Therapy + Life Plan [DT/LP]) for those with advanced pancreatic or lung cancer receiving treatment within a year of diagnosis. Methods: This was a single-arm, Phase I/II pilot study, conducted in the outpatient setting at an NCI-designated comprehensive cancer center. Dignity Therapy consisted of a focused life review intervention delivered during three outpatient oncology encounters for each patient. Interviews were audiotaped, transcribed, and edited to produce a legacy document. Participants scribed a list of 3-6 life goals for the LP intervention. Outcomes measured at baseline, immediately post DT/LP, and 3 months later were distress (Distress Thermometer), quality of life (FACT and LASA), spirituality (FaCIT-Sp-12), dignity (Patient Dignity Inventory), and purpose in life (Purpose in Life Test- Short Form). Data were analyzed with one-sample, two-sided t-tests (alternate: Wilcoxan). Results: Eighteen advanced pancreatic or lung cancer patients enrolled, mean age 64. There were no significant differences among all variables from baseline to post-DT/LP and 3 months later, with the exception of less distress experienced between baseline and 3 months after DT/LP ( p= 0.04). Conclusions: The DT/LP intervention did not show improvements in these psychosocial outcomes, but patients with advanced cancer typically experience worsening physical and psychosocial symptoms with active treatment. Therefore, maintaining these outcomes from baseline is a worthy goal, as we may be preventing further morbidity in an advanced cancer population. Further research is needed, however, given this small sample size. Clinical trial information: NCT02132325.

Objective Determinants of the increased risk of diffuse large B-cell lymphoma (DLBCL) in SLE are unclear. Using data from a recent lymphoma genome-wide association study (GWAS), we assessed whether certain lupus-related single nucleotide polymorphisms (SNPs) were also associated with DLBCL. Methods GWAS data on European Caucasians from the International Lymphoma Epidemiology Consortium (InterLymph) provided a total of 3857 DLBCL cases and 7666 general-population controls. Data were pooled in a random-effects meta-analysis. Results Among the 28 SLE-related SNPs investigated, the two most convincingly associated with risk of DLBCL included the CD40 SLE risk allele rs4810485 on chromosome 20q13 (OR per risk allele=1.09, 95% CI 1.02 to 1.16, p=0.0134), and the HLA SLE risk allele rs1270942 on chromosome 6p21.33 (OR per risk allele=1.17, 95% CI 1.01 to 1.36, p=0.0362). Of additional possible interest were rs2205960 and rs12537284. The rs2205960 SNP, related to a cytokine of the tumour necrosis factor superfamily TNFSF4, was associated with an OR per risk allele of 1.07, 95% CI 1.00 to 1.16, p=0.0549. The OR for the rs12537284 (chromosome 7q32, IRF5 gene) risk allele was 1.08, 95% CI 0.99 to 1.18, p=0.0765. Conclusions These data suggest several plausible genetic links between DLBCL and SLE.

Background Although most patients express a preference to die at home, many (over 30 percent) still die in hospital. This study's purpose was to explore the experience of hospital death from the perspective of patients’ family members. Methods Interviews were conducted with family members of patients who had died at hospitals affiliated with a large tertiary referral centre in the United States. Content analysis was used to analyze findings. Findings We interviewed 30 family members by phone. Themes were arranged by time frame: before death, time of death, and after death. Conclusion Families do not interpret clinical cues leading up to death in the same way healthcare providers do; families need clear and direct explanations from providers. Clinicians should assess patient and family understandings of prognosis and communicate clearly and directly. Family members value being with their loved one at the time of death, and they value spending time with the body after death; this should be facilitated in clinical practice.

Background: The quality of perimortem care received by patients who died at our hospitals was unknown. Objective: To describe the quality of hospital care experienced in the last week of life, as perceived by decedents’ families. Design: Telephone survey that included established measures and investigator-developed content. Setting: Large, tertiary care center known for high-quality, cost-effective care. Participants: Family members of 104 patients who died in-hospital (10% of annual deaths) over the course of 1 year. Intervention: None. Measurements: Participant perceptions of the decedent’s care, including symptom management, personal care, communication, and care coordination. Results: Decedents were mostly male (64%), white (96%), married (73%), and Christian (91%). Most survey participants were spouses of the decedent (68%); they were predominately white (98%), female (70%), and Christian (90%) and had a median age of 70 years (range, 35-91 years). Overall satisfaction was high. Pain, dyspnea, and anxiety or sadness were highly prevalent among decedents (73%, 73%, and 55%, respectively) but largely well managed. Most participants believed that decedents were treated respectfully and kindly by staff (87%) and that sufficient help was available to assist with medications and dressing changes (97%). Opportunities for improvement included management of decedents’ anxiety or sadness (29%) and personal care (25%), emotional support of the family (57%), communication regarding decedents’ illness (29%), and receiving contradictory or confusing information (33%). Conclusion: Despite high satisfaction with care overall, we identified important unmet needs. Addressing these gaps will improve the care of dying patients.

Background: Nonvascularized bone grafting (NVBG) and vascularized bone grafts (VBGs) have been used to treat scaphoid nonunions. A comparison of a single institution’ s experience using structural iliac crest bone graft (ICBG), 1,2-intercompartmental supraretinacular artery (1,2-ICSRA), and medial femoral condyle (MFC) grafts to treat scaphoid nonunions was undertaken. Methods: Thirty-one patients underwent ICBG, 33 patients underwent 1,2-ICSRA, and 45 patients underwent MFC grafting. Mean time to follow-up was 16 months (range, 2-153). Twenty-three percent used tobacco products. Mean age was 24 years (range, 11-66), with 86% male patients. Outcomes included union rate, time to healing, range of motion, complications, and reoperations. Results: Union rates and mean time to union were 71% and 19 weeks for the ICBG group, 79% and 26 weeks for the 1,2-ICSRA group, and 89% and 16 weeks for the MFC group, respectively. Failure of union was associated with tobacco use but not with age, gender, time from injury to surgery, or type of fixation. There was no significant difference between the groups regarding range of motion or strength. Conclusions: These findings demonstrate the need for careful patient selection. In patients with the most complicated scaphoid nonunions (avascular necrosis, carpal collapse, and previous surgeries), the MFC group had the highest union rate and fastest time to union. Patients with risk factors for failure should be counseled on the outcomes and possible need for salvage fusion surgery.

Objective To develop and validate new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIM) and their major subgroups. Methods Candidate variables were assembled from published criteria and expert opinion using consensus methodology. Data were collected from 47 rheumatology, dermatology, neurology, and pediatric clinics worldwide. Several statistical methods were utilized to derive the classification criteria. Results Based on data from 976 IIM patients (74% adults; 26% children) and 624 non‐IIM patients with mimicking conditions (82% adults; 18% children), new criteria were derived. Each item is assigned a weighted score. The total score corresponds to a probability of having IIM. Subclassification is performed using a classification tree. A probability cutoff of 55%, corresponding to a score of 5.5 (6.7 with muscle biopsy) “probable IIM,” had best sensitivity/specificity (87%/82% without biopsies, 93%/88% with biopsies) and is recommended as a minimum to classify a patient as having IIM. A probability of ≥90%, corresponding to a score of ≥7.5 (≥8.7 with muscle biopsy), corresponds to “definite IIM.” A probability of <50%, corresponding to a score of <5.3 (<6.5 with muscle biopsy), rules out IIM, leaving a probability of ≥50–<55% as “possible IIM.” Conclusion The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for IIM have been endorsed by international rheumatology, dermatology, neurology, and pediatric groups. They employ easily accessible and operationally defined elements, and have been partially validated. They allow classification of “definite,” “probable,” and “possible” IIM, in addition to the major subgroups of IIM, including juvenile IIM. They generally perform better than existing criteria.