Combination rituximab and intravenous immunoglobulin for treatment of refractory vasculitic neuropathy: a case series.

OBJECTIVE: Limited evidence exists to guide treatment of refractory vasculitic neuropathy. While rituximab (RTX) and IVIG have both been proposed as individual treatment options for these patients, combination therapy has never been reported. METHODS: Written informed consent was obtained from three patients with refractory vasculitic neuropathy who were treated with combination RTX and IVIG. Their electronic medical records were reviewed and clinical and functional outcomes were reported. RESULTS: Two male patients with non-systemic vasculitic neuropathy and one male patient with granulomatosis with polyangiitis were treated with combination RTX and IVIG therapy. All three patients demonstrated clinical improvement with at least partial functional recovery and a reduction in corticosteroid dose. This combination was generally well tolerated. CONCLUSIONS: Combination RTX and IVIG therapy may be a safe and effective treatment option for patients with refractory vasculitic neuropathy. Further studies are needed to better characterize the risks and benefits of this combination.

Kikuchi-Fujimoto disease presenting in a patient with SARS-CoV-2: a case report.

BACKGROUND: We present a yet to be described association of SARS-CoV-2 infection with Kikuchi-Fujimoto disease. CASE PRESENTATION: A 32-year-old physician with history of SARS-CoV-2 infection presented to the emergency department with 2 weeks of fever, chills, and right sided cervical lymphadenopathy. He was treated empirically for presumed folliculitis with worsening of symptoms leading to repeat presentation to the emergency department. Extensive workup was unrevealing of an infectious cause and needle biopsy of the lesion was unrevealing. An excisional lymph node biopsy revealed follicular hyperplasia with necrotic foci showing abundance of histiocytes at the edge of necrosis with CD8 predominance of T-cells. Final diagnosis was deemed to be Kikuchi-Fujimoto disease. Antibiotic therapy was discontinued, and the patient's symptoms resolved with steroid therapy and expectant management. CONCLUSIONS: This is the first report of a patient developing Kikuchi-Fujimoto disease following SARS-CoV-2 infection. Clinicians should be aware of Kikuchi-Fujimoto disease as a possibility when approaching patients with hyper-inflammatory states who present with cervical lymphadenopathy.

Association of the Systemic Lupus International Collaborating Clinics Frailty Index and Damage Accrual in Longstanding Systemic Lupus Erythematosus.

OBJECTIVE: To externally validate the Systemic Lupus International Collaborating Clinics Frailty Index (SLICC-FI) in a prevalent systemic lupus erythematosus (SLE) cohort and to assess the ability of the SLICC-FI to predict organ damage accrual among individuals with longstanding SLE. METHODS: This was a secondary analysis of data from the Study of Lupus Vascular and Bone Long-Term Endpoints (SOLVABLE) cohort, which consists of adult women from the Chicago Lupus Database who met the 1997 revised American College of Rheumatology (ACR) classification criteria for SLE. There were 185 patients with SLE enrolled, of whom 149 patients were included in a 5-year follow-up analysis. The SLICC-FI and SLICC/ACR Damage Index (SDI) scores were calculated at baseline and 5-year follow-up. Unadjusted and adjusted logistic regression models estimated the association of baseline SLICC-FI scores (per 0.05 increase) with damage accrual at 5-year follow-up. RESULTS: At enrollment the mean ± SD age of the 149 patients was 43.30 ± 10.15 years, the mean ± SD disease duration was 11.93 ± 8.46 years, and the mean ± SD SDI score was 1.64 ± 1.83. At baseline, the mean ± SD SLICC-FI score was 0.18 ± 0.08, and 36% of participants were categorized as frail (SLICC-FI score >0.21). In a model adjusted for age, race, and disease duration, each 0.05-unit increase in the baseline SLICC-FI score was associated with 28% higher odds of subsequent damage accrual (odds ratio 1.28, 95% confidence interval 1.01-1.63). CONCLUSION: In a prevalent cohort of women with established SLE, higher baseline SLICC-FI scores were associated with a higher risk of subsequent damage accrual at 5-year follow-up.

Telemedicine in Adult Rheumatology: In Practice and in Training.

Many rheumatology providers, including fellows-in-training, responded to the immediate need for maintaining patient access to care via telerheumatology during the COVID-19 pandemic. The rapidity of this transition did not permit an intentional approach to integrating fellow education and training into virtual patient care. Virtual patient care has since become an integrated, and perhaps, an embedded part of rheumatology practice that will likely endure beyond the COVID-19 pandemic. Thus, the development of best practices in telerheumatology, including those for fellow education and training as these new entrants prepare to enter our workforce, will benefit the entire specialty. In this work, we seek to describe current models for training learners in virtual patient care, characterize existing barriers to virtual care models, and offer strategies to integrate telerheumatology into curriculum development and training.

Factors Associated With Participation in Rheumatic Disease-Related Research Among Underrepresented Populations: A Qualitative Systematic Review.

OBJECTIVE: Nonwhite racial/ethnic groups remain underrepresented in rheumatic disease-related research, despite being disproportionately affected by these disorders. Our objective was to systematically review the literature regarding underrepresented patients' perceptions of participation in rheumatic disease research and to develop strategies to improve diversity. METHODS: A systematic search of Embase, PubMed/Medline, PsycINFO, and Cochrane was performed through October 2018. Two independent reviewers identified 642 unique studies; 7 met inclusion criteria (peer-reviewed articles, published in English in the last 20 years, adult population, and with a focus on underrepresented patients' participation in rheumatic research). Five coauthors provided final approval of included articles. Data abstraction was performed, and common themes and key differences were determined and adjudicated. RESULTS: The 7 articles included (n = 1,892 patients, range per article 20-961) evaluated factors associated with research participation of underrepresented populations. Five articles were related to lupus and 2 to rheumatoid arthritis, and 5 focused on African American patients and 1 on Hispanic patients. Five of the studies provided quantitative data through surveys (n = 3) and chart review (n = 2), while 2 used qualitative analyses. Key themes regarding underrepresented patients' perceptions of participating in research included: 1) the importance of trust in the patient- physician relationship, 2) the understanding of heterogeneity within and between ethnic groups, 3) the need for authentic academic-community partnerships, and 4) the implications of strict inclusion criteria on study participant diversity. CONCLUSION: Limited evidence exists regarding underrepresented patients' attitudes toward research participation in rheumatology, and further investigation is warranted. The themes identified provide a starting point for future interventions that promote increased diversity in rheumatic disease-related research studies.

A New Diagnosis of Left Ventricular Non-Compaction in a Patient Presenting with Acute Heart Failure.

Left ventricular non-compaction is an overall rare cardiomyopathy; however, it is increasingly being recognized with advances in imaging technology. We present the case of a 47-year-old man with new diagnosis of heart failure and left ventricular non-compaction. We review the literature regarding diagnostic imaging criteria and management of this condition.