A Review of Difficult-to-Treat Rheumatoid Arthritis: Definition, Clinical Presentation, and Management.

PURPOSE OF REVIEW: A subset of patients with rheumatoid arthritis (RA) who fail multiple biologic therapies are deemed to have "difficult-to-treat" (D2T) RA. In 2021, a European Alliance of Associations for Rheumatology (EULAR) task force proposed a clinical definition of D2T RA. Here we review RA phenotypes and clinical assessment of RA, propose a different definition of D2T RA, discuss possible D2T RA risk factors, and summarize existing literature on the management of D2T RA. RECENT FINDINGS: High disease activity at the time of diagnosis or prior to treatment with a biologic is associated with the development of D2T RA. Prolonged time from diagnosis to beginning treatment has been consistently associated with the development of D2T RA. Other clinical factors such as burden of disease, extraarticular disease, obesity, smoking, pain, fatigue, and psychological conditions have inconsistent associations with D2T RA according to current literature. D2T RA is a relatively new concept that represents an area of great need for research regarding the characterization of those with the disease as well as how best to treat the disease. With this gained knowledge, rheumatologists will be able to better identify patients at the time of diagnosis that are likely to develop D2T RA to help guide management.

Ocular manifestations of rheumatoid arthritis.

PURPOSE OF REVIEW: This article summarizes the pathophysiology of rheumatoid arthritis and common ocular manifestations that it is associated with: keratoconjunctivitis sicca, episcleritis, scleritis, and peripheral ulcerative keratitis. RECENT FINDINGS: Newer biologic agents are being used to effectively treat rheumatoid arthritis and its ocular manifestations. SUMMARY: The eye is a frequent extra-articular site of inflammation in patients with rheumatoid arthritis. Ocular involvement can range from more benign conditions such as keratoconjunctivitis sicca and episcleritis, to potentially vision and globe-threatening diseases like scleritis and peripheral ulcerative keratitis. Clinicians should be aware of these ophthalmic manifestations and the various treatment options that are available. Coordination between ophthalmology and rheumatology is helpful in the treatment of these patients.

Contraceptive Use in Women of Childbearing Ability With Rheumatoid Arthritis.

BACKGROUND/OBJECTIVE: Rheumatoid arthritis (RA) is a complex disease that may require treatment with one or several disease-modifying antirheumatic drugs (DMARDs). Many DMARDs have potential teratogenic effects or are newer agents with limited safety data in pregnancy. This study evaluated 20 common RA medications and the rate of contraceptive prescribing and counseling patterns in women with RA of childbearing ability. METHODS: This was an observational study of women with RA and childbearing ability aged 18 to 44 years who were seen at an academic rheumatology clinic from April 1, 2014, to March 31, 2016. Descriptive statistics and univariate logistic regression were used for analysis. RESULTS: One hundred fifty women were included in the analysis. The majority of patients were taking methotrexate (55.3%), followed by chronic prednisone (31.3%) and hydroxychloroquine (28.7%). A documented method of contraception was noted in 64/150 (42.7%). For women on contraception, most used combined oral contraceptives (31/64, 48.4%) or levonorgestrel intrauterine device (10/64, 15.6%). Of the 86 patients not on contraception, 19 (22.1%) received counseling regarding a pregnancy plan. CONCLUSIONS: Most women with RA of childbearing age and ability were not using contraception. Among these patients, only a minority prescribed DMARD therapy had documented pregnancy or contraceptive counseling. Women with RA who were prescribed with a DMARD should discuss the use of effective contraception with their provider if sexually active and not desiring pregnancy or wanting to avoid potential teratogenic effects. Potential strategies are discussed to improve healthcare delivery to this population in hopes of avoiding unintended pregnancy and potential teratogenic effects of RA medications.

Granulomatosis with polyangiitis in a patient treated with dabrafenib and trametinib for BRAF V600E positive lung adenocarcinoma.

BACKGROUND: Dabrafenib and trametinib combination therapy is approved for the treatment of patients with BRAF V600E positive tumors including melanoma and lung cancer. The effect of BRAF and MEK inhibitors on the immune system is not fully understood although a number of case reports indicate autoimmune side effects related to the use of these drugs. Here, we discuss a case of a patient diagnosed with granulomatosis with polyangiitis (GPA) shortly after starting treatment with dabrafenib and trametinib for BRAF V600E positive metastatic lung adenocarcinoma. CASE PRESENTATION: A 57 years old female patient was diagnosed with recurrent lung adenocarcinoma following initial lobectomy for early stage disease. A BRAF V600E mutation was identified at the time of recurrence and she received combination dabrafenib and trametinib therapy. Shortly after commencement of treatment, she developed persistent fevers necessitating withholding both drugs. Pyrexia continued and was followed by left vision loss and acute kidney injury. Further rheumatological workup led to the unifying diagnosis of GPA. The patient was then treated with rituximab for GPA to the present date while all antineoplastic drugs were held. Lung cancer oligoprogression was addressed with radiation therapy and has not required further systemic treatment whereas GPA has been controlled to-date with rituximab. CONCLUSIONS: This case report raises awareness among clinicians treating patients with lung cancer for the possibility of triggering a flare of autoimmune diseases like GPA in patients with BRAF V600E positive lung cancer receiving treatment with BRAF directed therapy.

Rheumatologists and Ophthalmologists Differ in Treatment Decisions for Ocular Behçet Disease.

BACKGROUND: Ocular involvement in patients with Behçet disease represents a significant clinical morbidity in this disease, and the prevention of visual impairment is an important treatment goal. There are no randomized controlled trials for the treatment of ocular Behçet disease; however, clinicians must still make treatment decisions. OBJECTIVES: The goals of this study were to describe the treatment preferences of rheumatologists and ophthalmologists for the treatment of ocular Behçet disease and to identify factors that influence these decisions. METHODS: Eight hundred fifty-two rheumatologists and 934 ophthalmologists were surveyed via e-mail regarding their choice of therapy for a hypothetical patient with ocular Behçet disease. Respondents were asked to select first- and second-choice therapies and then reselect first and second choices assuming there would be no issues with cost or insurance prior authorization. RESULTS: One hundred thirty two physicians (7.4%) who were willing to recommend treatment completed the survey: 68 rheumatologists and 64 ophthalmologists. The most common first-choice therapy for both specialties was a biologic agent. Significantly more rheumatologists than ophthalmologists chose methotrexate (P < 0.025) and azathioprine (P < 0.005) as their first-choice therapy. After assuming there were no concerns with cost or prior authorization, rheumatologists were still more likely to choose azathioprine compared with ophthalmologists (P < 0.02), and ophthalmologists were more likely to choose local steroid implants (P < 0.02). Both rheumatologists and ophthalmologists increased their choice of an anti-tumor necrosis factor agent when cost and prior authorization issues were removed (P < 0.0001 and 0.008, respectively). CONCLUSIONS: Physician decision making is influenced by medical specialty and concerns regarding cost and prior authorization.

Performance of anti-cyclic citrullinated Peptide assays differs in subjects at increased risk of rheumatoid arthritis and subjects with established disease.

OBJECTIVE: To compare the diagnostic accuracy and agreement of commonly available assays for anti-citrullinated protein antibodies in patients with established rheumatoid arthritis (RA) and subjects at increased risk of RA. METHODS: Tests for anti-cyclic citrullinated peptide (anti-CCP) antibodies were performed using CCP2 IgG and CCP3.1 IgA/IgG enzyme-linked immunosorbent assays in the following groups: probands with established RA (n = 340) from the Studies of the Etiology of Rheumatoid Arthritis (SERA) cohort and their first-degree relatives (FDRs) without inflammatory arthritis (n = 681), Department of Defense Serum Repository (DoDSR) RA cases with pre-RA diagnosis samples (n = 83; 47 cases also had post-RA diagnosis samples), and blood donor and DoDSR control subjects (n = 283). RESULTS: In patients with established RA, the CCP2 assay was more specific (99.2% versus 93.1%; P < 0.01) but less sensitive (58.7% versus 67.4%; P = 0.01) than the CCP3.1 assay; the specificity of the CCP3.1 assay increased to 97.2% when cutoff levels ≥3-fold the standard level were considered. In all subjects, CCP3.1 assay positivity (using standard cutoff levels) was more prevalent. Among DoDSR cases, the CCP2 assay was more specific than the CCP3.1 for predicting a future diagnosis of RA, and higher CCP levels trended toward increasing specificity for the development of RA within 2 years. At standard cutoff levels, assay agreement was good in patients with established RA (κ = 0.76) but poor in FDRs without inflammatory arthritis (κ = 0.25). CONCLUSION: Anti-CCP assays differ to an extent that may be meaningful for diagnosing RA in patients with inflammatory arthritis and evaluating the natural history of RA development in subjects at risk of RA. The mechanisms underlying these differences in test performance need further investigation.

Multiple cytokines and chemokines are associated with rheumatoid arthritis-related autoimmunity in first-degree relatives without rheumatoid arthritis: Studies of the Aetiology of Rheumatoid Arthritis (SERA).

OBJECTIVE: We investigated whether rheumatoid arthritis (RA)-related autoantibodies were associated with systemic inflammation in a prospective cohort of first-degree relatives (FDRs) of RA probands, a population without RA but at increased risk for its future development. METHODS: We studied 44 autoantibody positive FDRs, of whom 29 were rheumatoid factor (RF) positive, 25 were positive for the high risk autoantibody profile (HRP), that is, positive for anti-cyclic citrullinated peptide and/or for at least two RF IgM, IgG or IgA isotypes, and nine FDRs who were positive for both; and 62 FDRs who were never autoantibody positive. Twenty-five cytokines/chemokines were measured using a bead-based assay in serum. As a comprehensive measure of inflammation, we calculated a Cytokine Score by summing all cytokine/chemokine levels, weighted by their regression coefficients for RA-autoantibody association. We compared C-reactive protein, individual cytokines/chemokines and Cytokine Score to the outcomes: positivity for RF and for the HRP using logistic regression. RESULTS: Adjusting for age, sex, ethnicity and ever smoking, the Cytokine Score and levels of IL-6 and IL-9 were associated with both RF and HRP. IL-2, granulocyte macrophage-colony stimulating factor (GM-CSF), and interferon (IFN)-γ were associated with HRP only. Associations between the Cytokine Score and RF and HRP positivity were replicated in an independent military personnel cohort. CONCLUSIONS: In first-degree relatives of patients with RA, RA-related autoimmunity is associated with inflammation, as evidenced by associations with multiple cytokines and chemokines.

Brief report: airways abnormalities and rheumatoid arthritis-related autoantibodies in subjects without arthritis: early injury or initiating site of autoimmunity?

OBJECTIVE: To evaluate the presence of pulmonary abnormalities in rheumatoid arthritis (RA)-related autoantibody-positive subjects without inflammatory arthritis. METHODS: Forty-two subjects who did not have inflammatory arthritis but were positive for anti-cyclic citrullinated peptide antibodies and/or ≥2 rheumatoid factor isotypes (a profile that is 96% specific for RA), 15 autoantibody-negative controls, and 12 patients with established seropositive early RA (<1-year duration) underwent spirometry and high-resolution computed tomography (HRCT) lung imaging. RESULTS: The median age of autoantibody-positive subjects was 54 years, 52% were female, and 38% were ever-smokers; these characteristics were not significantly different from those of autoantibody-negative control subjects. No autoantibody-positive subject had inflammatory arthritis based on joint examination. HRCT revealed that 76% of autoantibody-positive subjects had airways abnormalities including bronchial wall thickening, bronchiectasis, centrilobular opacities, and air trapping, compared with 33% of autoantibody-negative controls (P = 0.005). The prevalence and type of lung abnormalities among autoantibody-positive subjects were similar to those among patients with early RA. In 2 autoantibody-positive subjects with airways disease, inflammatory arthritis classifiable as articular RA developed ∼13 months after the lung evaluation. CONCLUSION: Airways abnormalities that are consistent with inflammation are common in autoantibody-positive subjects without inflammatory arthritis and are similar to airways abnormalities seen in patients with early RA. These findings suggest that the lung may be an early site of autoimmune-related injury and potentially a site of generation of RA-related autoimmunity. Further studies are needed to define the mechanistic role of lung inflammation in the development of RA.

Virtually Speaking: The Impact of a Nationwide Synchronous and Asynchronous Learning Forum.

OBJECTIVE: To address significant disruptions in didactic education precipitated by the COVID-19 pandemic, a group of rheumatology program directors collaborated with the American College of Rheumatology to create a virtual fellows-in-training (V-FIT) program. METHODS: A working group was composed to develop the virtual didactic program comprising live virtual sessions of core curricular rheumatology topics that were recorded to permit asynchronous learning. Nationally recognized educators were invited to lead sessions to fill the void in didactic education occurring on a broad scale across US rheumatology fellowship training programs. Demographic information, live and asynchronous participation data, and feedback surveys were collected from participants in the program. RESULTS: There were 3 components to V-FIT: the Virtual Rheumatology Learning (ViRL) series, the Virtual Rheumatology Practicum (ViP), and the Virtual Rheumatology Teaching Lessons (ViTLs). The ViRL program had global impact with more than 2,000 learners from more than 55 countries. ViP provided a standardized curriculum of rheumatology topics for incoming first-year fellows. ViTLs addressed advanced and interdisciplinary rheumatic disease topics for learners at all stages. CONCLUSION: With collaboration, adaptation, and innovation, the V-FIT program not only maintained but also enhanced education for rheumatology trainees, was enriched by national and international participation, and provided standardized, broadly accessible content with interdisciplinary learning.

Cataract Surgery Outcomes in Patients with Non-ocular Autoimmune Disease.

INTRODUCTION: While phacoemulsification cataract extraction is generally highly effective and safe, patients with a history of uveitis are at higher risk for postoperative complications and often require a modified perioperative medication regimen. No data exists on risks of postoperative complications and persistent anterior uveitis (PAU) in patients with non-ocular autoimmune disease. METHODS: Medical records were reviewed of patients who underwent phacoemulsification cataract surgery with intraocular lens implantation between January 1, 2014 and December 31, 2019 at the University of Colorado Hospital (UCH) as part of a retrospective cohort study. Exclusion criteria included patient history of ocular inflammation and cataract surgery combined with another intraocular surgery. Patients were only included as having autoimmune disease if the diagnosis was confirmed by a relevant specialist at UCH. Patients with autoimmune disease were then stratified into systemic versus organ-specific autoimmune disease, and patients with systemic autoimmune disease were further stratified into immunosuppressed and not immunosuppressed at the time of cataract surgery. Patients with PAU were identified according to the Standardization of Uveitis Nomenclature Working Group. Data including sex, race/ethnicity, intraoperative cumulative dissipated energy (CDE), and postoperative best-corrected visual acuity (BCVA) and intraocular pressure (IOP) were obtained. RESULTS: A total of 422 eyes from 248 patients had confirmed autoimmune disease, compared to a control group of 10,201 eyes. The autoimmune and systemic autoimmune disease groups were not more likely to have postoperative complications or PAU compared to the control group. Immunosuppression status among the systemic autoimmune disease group was also not associated with postoperative complications or PAU. CONCLUSION: Patients with non-ocular autoimmune disease do not appear to be at higher risk for postoperative complications, including worse BCVA or increased rates of IOP elevation and PAU, following phacoemulsification cataract surgery. These patients do not appear to require modification of the typical perioperative medication regimen.

Addressing Competency in Rheumatology Telehealth Care Delivery.

OBJECTIVE: Telehealth is an essential facet of care delivery for patients with rheumatic diseases. The Association of American Medical College's (AAMC) telehealth competencies (TCs) define the skills required for delivering general telehealth care across the range of clinician experience. In this study, the American College of Rheumatology's (ACR) TCs working group aimed to adapt the AAMC TCs to rheumatology, outlining the skills acquisition unique to rheumatology with a focus on knowledge, skills, and behaviors expected of recent rheumatology fellowship graduates. METHODS: Through a collaborative process, the working group adapted the AAMC TCs to the training structure and practice of rheumatology. The rheumatology TCs underwent peer review among recipients of the Clinician Scholar Educator Award and attendees at the ACR 2021 Convergence conference. RESULTS: The rheumatology TCs define 24 essential skills required for synchronous telehealth care of patients with rheumatic diseases. The working group adapted the AAMC's 20 TCs organized within 6 domains, added 2 skills to the AAMC's domains of patient safety and appropriate use, and data collection and assessment, and created a novel domain of systems-based requirements with 2 competencies. The rheumatology TCs define expected skill levels for recent rheumatology fellowship graduates and experienced rheumatology clinicians. CONCLUSION: The rheumatology TCs represent the first adaptation of the AAMC TCs to subspecialty care, expanding the scope to include rheumatology fellowship graduates and additional domains of rheumatology practice. These competencies can guide curricular innovations and measurements of proficiency in telehealth care delivery among rheumatology trainees and experienced clinicians, enhancing the care provided to patients with rheumatic diseases.

Incorporating Telemedicine in Rheumatology Fellowship Training Programs: Needs Assessment, Curricular Intervention, and Evaluation.

OBJECTIVE: To increase the confidence of rheumatology fellows in training (FITs) in delivering virtual care (VC) and prepare them for independent practice, we developed educational materials addressing gaps in their skills. METHODS: We identified gaps in telemedicine skills based on FIT performance in a virtual rheumatology objective structured clinical examination (vROSCE) station on VC delivery using video teleconference technology and survey (survey 1) responses. We created educational materials including videos of "mediocre" and "excellent" VC examples, discussion/reflection questions, and a document summarizing key practices. We measured change in the confidence levels of FITs for delivering VC with a post-intervention survey (survey 2). RESULTS: Thirty-seven FITs (19 first-year, 18 second- plus third-year fellows) from 7 rheumatology fellowship training programs participated in a vROSCE and demonstrated gaps in skills mapping to several Rheumatology Telehealth Competency domains. Confidence levels of FITs improved significantly from survey 1 to survey 2 for 22 of 34 (65%) questions. All participating FITs found the educational materials helpful for learning and reflecting on their own VC practice; 18 FITs (64%) qualified usefulness as "moderately" or "a lot." Through surveying, 17 FITs (61%) reported implementing skills from the instructional videos into VC visits. CONCLUSION: Continually assessing our learners' needs and creating educational materials addressing gaps in training are requisite. Using a vROSCE station, needs assessments, and targeted learning with videos and discussion-guidance materials enhanced the confidence level of FITs in VC delivery. It is imperative to incorporate VC delivery into fellowship training program curricula to ensure breadth in skills, attitudes, and knowledge of new entrants into the rheumatology workforce.

Quality Improvement Intervention to Reduce Thirty-Day Hospital Readmission Rates Among Patients With Systemic Lupus Erythematosus.

OBJECTIVE: Systemic lupus erythematosus (SLE) has one of the highest 30-day hospital readmission rates among chronic diseases in the US. This quality improvement initiative developed and assessed the feasibility of a multidisciplinary postdischarge intervention to reduce 30-day readmission rates among SLE patients. METHODS: A retrospective study was performed using electronic health records of patients with SLE admitted to a university hospital prior to (nonintervention group) and after initiation of the study intervention (intervention group). The study population included patients with a diagnosis of SLE who were admitted to the hospital for any reason during an 8-month time period. The intervention involved sending a templated message at the time of discharge to the rheumatology clinic nurses, which prompted the nurses to call the patient to coordinate future visits and provide education. The primary outcome was the 30-day hospital readmission rate. Data were analyzed using a multivariate mixed binomial regression model. RESULTS: There were 59 hospitalizations in the nonintervention group and 73 hospitalizations in the intervention group during the 8-month study period. The 30-day readmission rate was 29% in the nonintervention group and 19% in the intervention group. The difference in readmission rates between the 2 groups was not statistically significant based on the multivariate model. CONCLUSION: Our study demonstrates the feasibility of implementing a multidisciplinary postdischarge intervention to reduce readmission rates for patients with SLE in a large academic medical center. Further investigation is warranted to determine if this approach reduces the unacceptably high hospital readmission rates among SLE patients.

PURTSCHER'S‒LIKE RETINOPATHY SECONDARY TO GRANULOMATOSIS WITH POLYANGIITIS FOLLOWING AUTOLOGOUS STEM CELL TRANSPLANTATION.

PURPOSE: The authors describe a man undergoing autologous stem cell transplant, who developed granulomatosis with polyangiitis and Purtscher's‒like retinopathy. METHODS: A 25-year-old man underwent fundus photography, optical coherence tomography, and fluorescein angiography to obtain a diagnosis and follow the treatment course. RESULTS: The initial ophthalmic presentation and imaging confirmed the findings of Purtscher's‒like retinopathy. Throughout his course, he had progressive neovascularization and vitreous hemorrhages in both eyes, requiring vitrectomy and endolaser, and bevacizumab injections. CONCLUSION: The authors describe a patient who underwent autologous stem cell transplantation, and subsequently developed granulomatosis with polyangiitis. The presentation of Purtscher's‒like retinopathy suggests that microvascular occlusion in the retina was likely the result of granulomatosis with polyangiitis-driven vasculitis of the precapillary arterioles.

Rheumatology Milestones 2.0: A Roadmap for Competency-Based Medical Training of Rheumatology Fellows in the 21st Century.

OBJECTIVE: Since 2014, rheumatology fellows have been assessed not only based on their ability to provide patient care and possession of medical knowledge but also on their skill in serving as patient advocates, navigators of health systems, and members of a health care team. Such assessments have been carried out through the use of competency-based milestones from the Accreditation Council of Graduate Medical Education (ACGME). However, a needs assessment has demonstrated interest in more context validity and subspecialty relevance since the development of the ACGME internal medicine (IM) subspecialty reporting milestones. The ACGME thus created a milestones working group, and the present study was undertaken to develop Rheumatology Milestones 2.0 as well as a supplemental guide to assist with implementation. METHODS: The working group, consisting of 7 rheumatology program directors, 2 division directors, a community practice rheumatologist, a rheumatology fellow in training, and a public member who is a rheumatology patient, was overseen by the ACGME vice president for milestones development and met through three 12-hour, in-person meetings to compose the rheumatology specialty milestones and supplemental guide within the ACGME Milestones 2.0 project. RESULTS: Informed by the needs assessment data and stakeholders, the working group revised and adapted the ACGME IM subspecialty reporting milestones to create a rheumatology-specific set of milestones and a supplemental guide for their implementation. CONCLUSION: The Rheumatology Milestones 2.0 provides a specialty-specific, competency-based evaluation tool that can be used by program directors, clinical competency committees, and others to assess the competencies of rheumatology fellows during training and help measure readiness for independent practice.

Autoimmunity to peptidyl arginine deiminase type 4 precedes clinical onset of rheumatoid arthritis.

OBJECTIVE: To determine whether antibodies against peptidyl arginine deiminase type 4 (PAD-4) are present in the preclinical phase of rheumatoid arthritis (RA) and to compare the timing and extent of their appearance with those of other preclinical autoantibodies. METHODS: Prediagnosis serum samples from 83 patients with RA were evaluated for the presence of anti-PAD-4 antibody, anti-cyclic citrullinated peptide (anti-CCP) antibody, and rheumatoid factor. In addition, a control cohort (n = 83) matched by age, sex, race, number of serum samples, and duration of serum storage was tested for the presence of anti-PAD-4 antibody to determine its sensitivity and specificity for the subsequent development of RA. RESULTS: Fifteen of 83 patients with RA (18.1%) had at least 1 prediagnosis sample positive for anti-PAD-4. One of 83 control subjects (1.2%) had at least 1 positive sample, resulting in a sensitivity and specificity of 18.1% and 98.8%, respectively, of anti-PAD-4 for the future development of RA. The mean duration of anti-PAD-4 positivity prior to clinical diagnosis was 4.67 years. Anti-PAD-4 positivity was associated with anti-CCP positivity (odds ratio 5.13 [95% confidence interval 1.07-24.5]). In subjects with prediagnosis samples that were positive for both antibodies, anti-CCP positivity predated anti-PAD-4 positivity in 9 of 13 cases (69%). CONCLUSION: Autoantibodies to PAD-4 are present during the preclinical phase of RA in a subset of patients and are associated with anti-CCP positivity. Further exploration is needed regarding the timing of appearance and disease-related effects of PAD-4 autoimmunity.

Applying Educational Theory to Optimize Trainee Education in the Ambulatory Virtual Care Environment.

Virtual care (VC) encounters have become an essential part of outpatient clinical care. The theory of situated learning and legitimate peripheral participation posits that medical trainees learn best when they participate in authentic patient care experiences and engage effectively with their preceptors, members of the health care team, and the clinical learning environment. This theory can provide a framework from which to approach teaching in the VC setting, whereby preceptors may capitalize on the unique learning and assessment opportunities provided during VC encounters and optimize educational experiences for trainees as well as clinical outcomes for patients. In this monograph, we propose an approach grounded in situated learning and legitimate peripheral participation for teaching in the VC environment, particularly during real-time video visits.

Retinal artery occlusion followed by contralateral amaurosis fugax in association with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

PURPOSE: To describe two cases of retinal artery occlusion followed by contralateral amaurosis fugax associated with eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome). OBSERVATIONS: Case 1 is a 57 year-old male who presented with transient vision loss in the right eye two weeks after a cilioretinal artery occlusion in the left eye. Evaluation eventually led to a diagnosis of EGPA. The patient was treated with high-dose steroids followed by systemic immunomodulatory therapy. Vision in the right eye recovered to 20/20 with no further episodes of vision loss. Case 2 is a 55 year-old male with a known diagnosis of EGPA who presented with transient vision loss in the right eye four weeks after a central retinal artery occlusion of the left eye. This patient also successfully recovered vision in the right eye after treatment with high-dose steroids following a change in his systemic immunomodulatory therapy. CONCLUSIONS AND IMPORTANCE: While ANCA-vasculitides are an uncommon cause of retinal artery occlusion and amaurosis fugax, it is important that they remain in the differential diagnosis, as good visual outcomes can be achieved with prompt initiation of appropriate therapies.

The Effect of Pre-Appointment Consultation Triage on Patient Selection and Revenue Generation in a University Rheumatology Practice.

OBJECTIVE: To evaluate the effectiveness of pre-appointment consult screening to identify patients with autoimmune and inflammatory rheumatic disease (AIRD) and to evaluate the revenue implications of routine outpatient care of patients with AIRD compared to that of non-AIRD patients. METHODS: Using data in the electronic medical records, we retrospectively analyzed all new outpatients who were referred for rheumatology consults during a 9-month period for a final diagnosis and revenue generation for routine outpatient care over 1 year following the consult review or initial evaluation. RESULTS: A total of 961 patients were referred to the outpatient rheumatology clinic and underwent pre-appointment triage. Overall, 673 patients were approved for evaluation of AIRD, and 288 patients were denied rheumatology consultation. Patients were seen an average of 13 days after the consult review. Among patients who were approved for consult, 597 came for evaluation, with 357 diagnosed as having an AIRD and 240 with a non-AIRD. Among patients who were denied a consult, 128 had 1-year follow-up data, with 6 patients eventually diagnosed as having an AIRD (consult triage sensitivity 98%, positive predictive value 60%). The consult triage system allowed more AIRD patients to be seen over a 1-year period. Revenue data for outpatient care was available for 318 of 357 patients with an AIRD and 192 of 240 non-AIRD patients and showed that care for patients with an AIRD generates 44 times more revenue compared to care for non-AIRD patients ($5,877 per AIRD patient versus $134 per non-AIRD patient; P < 0.001). CONCLUSION: Pre-appointment consult screening is an effective method to identify patients with an AIRD. This approach enables timely access to care for patients with the highest need for evaluation and results in significantly more revenue generation.