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Treatment of cardiac sarcoidosis is challenging, as the disease can be refractory to traditional treatment with steroids. Infliximab, a tumor necrosis factor-α inhibitor, has been reportedly used in cardiac sarcoidosis, but published evidence is limited. The potential cardiotoxicity of infliximab and the Food and Drug Administration black-box warning for patients with heart failure have hindered the use of this agent in cardiac sarcoidosis. Here, we report a case of refractory cardiac sarcoidosis successfully treated with infliximab and discuss the important role of fluorine-18–fluorodeoxyglucose positron emission tomography in prognostication and guidance of therapy. (Level of Difficulty: Intermediate.), Graphical abstract, Treatment of cardiac sarcoidosis treatment is challenging, and the disease can be refractory to traditional treatment with steroids. Infliximab…

Idiopathic aortitis is an inflammatory disease of the aorta that is diagnosed after the less frequent infectious and rheumatologic variants are excluded. The etiology and natural history of the disease are poorly understood, and its presentation is variable; the need for exclusion of infectious, malignant, and rheumatologic causes can make its evaluation and diagnosis challenging. Treatment is tailored to the diagnosis and may include observation, antimicrobial therapies, and immunosuppressive agents when appropriate. Operative therapy is rarely needed and reserved for symptomatic patients or instances in which infection cannot be excluded. We present a case of idiopathic aortitis that resolved spontaneously with expectant management and discuss the pitfalls in the diagnosis and care of the disease. Keywords: Aortitis, Large vessel vasculitis, Nonaneurysmal, Noninfectious, Idiopathic

Background/objective New classification criteria for SLE have recently been developed. How these criteria affect the classification of patients with the SLE-mimicking condition UCTD is poorly understood. This study investigated the reclassification of UCTD patients using newly derived SLE criteria. Methods Patients with UCTD were identified within a single academic medical center using ICD9/10 codes. Medical record review was performed to confirm UCTD diagnosis and identify disease features present at diagnosis. The SLICC and ACR/EULAR criteria were applied, after which we compared the proportion of patients reclassified as SLE and determined which disease features were associated with reclassification. Results A total of 129 patients were included in the study. When applying the SLICC and ACR/EULAR criteria, 18 (14.0%) and 26 patients (20.2%) were reclassified as SLE. Comparison with McNemar’s test trended toward statistical significance (p = 0.057). Cohen’s kappa coefficient was 0.62 (p Conclusions Both the SLICC and ACR/EULAR criteria exhibit increased SLE classification. These newer classification criteria could be used to increase the number of SLE patients in future clinical studies.

Objective To describe the character and composition of the 2015 US adult rheumatology workforce, evaluate workforce trends, and project supply and demand for clinical rheumatology care for 2015-2030. Methods The 2015 Workforce Study of Rheumatology Specialists in the US used primary and secondary data sources to estimate the baseline adult rheumatology workforce and determine demographic and geographic factors relevant to workforce modeling. Supply and demand was projected through 2030, utilizing data-driven estimations regarding the proportion and clinical full-time equivalent (FTE) of academic versus nonacademic practitioners. Results The 2015 adult workforce (physicians, nurse practitioners, and physician assistants) was estimated to be 6,013 providers (5,415 clinical FTE). At baseline, the estimated demand exceeded the supply of clinical FTE by 700 (12.9%). By 2030, the supply of rheumatology clinical providers is projected to fall to 4,882 providers, or 4,051 clinical FTE (a 25.2% decrease in supply from 2015 baseline levels). Demand in 2030 is projected to exceed supply by 4,133 clinical FTE (102%). Conclusion The adult rheumatology workforce projections reflect a major demographic and geographic shift that will significantly impact the supply of the future workforce by 2030. These shifts include baby-boomer retirements, a millennial predominance, and an increase of female and part-time providers, in parallel with an increased demand for adult rheumatology care due to the growing and aging US population. Regional and innovative strategies will be necessary to manage access to care and reduce barriers to care for rheumatology patients.

Objectives The aim was to estimate the impact of TNF inhibitor (TNFi) exposure on radiographic disease progression in US Veterans with RA during the first year after initiating therapy. Methods This historical longitudinal cohort design used clinical and claims data to evaluate radiographic progression after initiation of TNFi. US Veterans with RA initiating TNFi treatment (index date), ≥ 6 months pre-index and ≥ 12 months post-index VA enrolment/activity, and initial (6 months pre-index to 30 days post-index) and follow-up (10–18 months post-index) bilateral hand radiographs were eligible. The cumulative TNFi exposure and change in modified Sharp score (MSS) between initial and follow-up radiographs were calculated. The percentage of patients with clinically meaningful change in MSS (≥ 5) for each month of exposure was assessed using a longitudinal marginal structural model with inverse probability of treatment weights. Mean values and CIs were generated using 1000 bootstrapped samples. Results For 246 eligible patients, the mean (s.d.) age was 58 (11) years; 81% were male. The mean (s.d.) initial MSS was 19.6 (33.4) (range 0–214). The mean change (s.d.) in MSS was 0.3 (3.6) (median 0, range −19 to 22). Patients with the greatest exposure had the least radiographic progression for both crude and adjusted model analyses. Adjusted rates of MSS change ≥ 5 points (95% CI) were 10.6% (9.8%, 11.4%) for patients with 3 months of exposure compared with 5.4% (5.1%, 5.7%) for patients with 12 months of exposure. Conclusion One-year changes in radiographic progression were small. Patients with the greatest cumulative TNFi exposure experienced the least progression.

This study examines: 1) alveolar bone loss (ABL), a hallmark of periodontitis, in anti-citrullinated protein antibody (ACPA)-positive rheumatoid arthritis (RA) patients versus control patients with osteoarthritis (OA); and 2) the association of ABL with RA disease activity and ACPA concentrations, including multiple antigen-specific ACPA.This multicenter case-control study includes 617 patients diagnosed with RA (n = 287) or OA (n = 330). Panoramic radiographs were taken; patients were categorized into low, moderate, or high tertiles based on mean percentage ABL. Serum ACPA was measured using second-generation anticyclic citrullinated peptide enzyme-linked immunosorbent assay and a multiplex platform to assess distinct antigen-specific ACPA. A generalized linear mixed model for binary data was used to compare stratified ABL in RA versus OA patients. Associations of moderate and high ABL (versus low) with RA disease activity and severity measures were examined using multivariate regression. Antigen-specific ACPA responses were compared among ABL tertiles using significance analysis of microarrays.ACPA-positive patients with RA had a significantly higher mean percentage of sites with ABL20% compared with patients with OA (P = 0.03). After multivariate adjustment, greater ABL was significantly associated with higher serum ACPA concentration (P = 0.004), 28-joint Disease Activity Score (P = 0.023), health assessment questionnaire disability (P = 0.05), tender joint count (P = 0.02) and joint space narrowing scores (P = 0.05) among patients with RA. ACPAs targeting citrullinated vimentin and histone were significantly higher in moderate and high ABL groups versus low, regardless of smoking status (q0.1%).Greater ABL was associated with higher ACPA, consistent with findings at articular sites. ACPA targeting could provide novel insight into important linkages between RA and periodontitis.

Periodontitis (PD) has emerged as a risk factor in a number of health conditions including rheumatoid arthritis (RA) (1). Sharing both morphologic and histopathologic similarities with RA (2), PD is an inflammatory disease initiated by bacterial infection resulting in soft and hard tissue destruction and ultimately leading to tooth loss. In addition to shared inflammatory pathways, PD and RA share risk factors for susceptibility and progression, most notably cigarette smoking and, possibly, shared epitope-containing HLA-DRB1 alleles, the latter associated with localized aggressive periodontitis (3–10). Although a causal link between these conditions has not been established, several reports have demonstrated an increased PD prevalence in RA patients compared to controls (11–18). Growing evidence suggests that pathogens associated with PD could play a role in RA propagation. Chief among the organisms of interest is Porphyromonas gingivalis (P. gingivalis) (19). P. gingivalis is the only known pathogen expressing peptidylarginine deiminase (PPAD). Similar to its human counterpart, P. gingivalis-expressed PAD catalyzes the citrullination of arginine-containing peptides. This is noteworthy because citrullinated antigens are thought to drive adaptive immune responses that are nearly exclusive to RA. The potential role of P. gingivalis in RA pathogenesis has been borne out in epidemiologic investigations. Concentrations of circulating antibody to P. gingivalis have been demonstrated to be associated with the expression of anti-citrullinated peptide antibody (ACPA) (20–22). More recently, our group has shown that antibody to P. gingivalis is associated with the presence of RA-related autoantibody (a combination of rheumatoid factor [RF] and/or ACPA) among individuals at increased risk for disease but who have not yet developed RA symptoms (23), underscoring the potential role of this pathogen in RA development. As part of the present study, we conducted a large case-control investigation to examine the relationship of PD with established RA. We sought to examine the degree to which this relationship is impacted by shared genetic and/or environmental factors. We also sought to elucidate the degree to which the relationship of PD with RA may be related to infection and/or colonization with P. gingivalis. By using a rigorously selected control population, we attempted to mitigate issues of bias or unmeasured confounding that may have impacted other efforts often using healthy volunteers as comparators (16–18). Finally, using a multiplex approach, we examined the associations of PD and P. gingivalis with autoreactivity to several citrullinated autoantigens that have been implicated in RA disease pathogenesis.

Objective. To examine whether vascular calcifications on hand films in RA might aid in determining mortality risk. Methods. Hand radiographs from 906 RA patients were scored as positive or negative for vascular calcifications. Patient characteristics associated with vascular calcifications were assessed using multivariable logistic regression, and associations with mortality were examined using Cox proportional hazards regression. Cytokines and multiplex ACPA were measured in both groups. Results. A total of 99 patients (11%) demonstrated radiographic vascular calcifications. Factors independently associated with vascular calcifications included diabetes [odds ratio (OR) 2.85; 95% CI 1.43, 5.66], cardiovascular disease at enrolment (OR 2.48; 95% CI 1.01, 6.09), prednisone use (OR 1.90; 95% CI 1.25, 2.91), current smoking (OR 0.06; 95% CI 0.01, 0.23) and former smoking (OR 0.36; 95% CI 0.27, 0.48) vs never smoking. In cytokine and ACPA subtype analysis, IL-4 and anti-citrullinated apolipoprotein E were significantly increased in patients with vascular calcifications in fully adjusted multivariable models. After multivariable adjustment, vascular calcifications were associated with an increase in all-cause mortality (hazard ratio 1.41; 95% CI 1.12, 1.78; P = 0.004). Conclusion. Vascular calcifications on hand radiographs were independently associated with increased all-cause mortality in RA. Mechanisms underpinning the associations of IL-4 and select ACPA with vascular calcifications and their utility as biomarkers predictive of cardiovascular disease risk in RA merit further study.

Wide variability exists in the treatment of gout. We compared the treatment practices of rheumatologists with those of primary care physicians (PCPs) in the management of gout. Pharmacy records were reviewed to identify patients treated with colchicine, allopurinol, probenecid, or sulfinpyrazone. Forty PCP patients were compared with 33 patients followed by rheumatologists. Rheumatologists were three times more likely to confirm the diagnosis with joint aspiration and guide therapy with 24-h urine uric acid collections than were PCPs. Rheumatologists were more likely to use prophylaxis in acute gout before initiating uric acid-lowering therapy than were PCPs. All PCP patients were treated with allopurinol compared with 65% of rheumatology patients. Mean posttreatment uric acid levels were lower for rheumatology patients (5.0 mg/dL) compared with PCP patients (6.0 mg/dL). Previous studies have reported poor symptom control and increased toxicity in gouty patients with suboptimal treatment. With the vast majority of patients being treated by PCPs in a man-aged care setting, further studies will be necessary to determine whether treatment variability affects outcome between the two groups.

Objective. To test the usefulness and cost savings resulting from application of the new American College of Rheumatology (ACR) guidelines for assessing the risk for the development of clinically significant liver disease in rheumatoid arthritis (RA) patients treated with methotrexate (MTX). Methods. One-hundred twelve MTX-treated RA patients were prospectively followed up for MTX hepatotoxicity and underwent liver biopsies according to modified guidelines of the Psoriatic Task Force (PTF). All biopsies were graded according to the Roenigk classification. The new ACR recommendations were then retrospectively applied to test their usefulness and cost-effectiveness in this cohort. Results. Based on the PTF guidelines, 66 patients underwent liver biopsies; a total of 110 liver biopsies were performed. Two patients had biopsy-related complications. Five patients were found to have Roenigk grade IIIB or IV histologic abnormalities. The total cost for this group was $111,380. Applying the new ACR criteria, only 15 patients would have undergone liver biopsies; there would have been a total of 18 biopsies, with no complications. Four of the 5 patients with Roenigk grade IIIB or IV liver abnormalities would have been identified. One patient with insulin-dependent diabetes mellitus (IDDM) who was found to have cirrhosis (Roenigk grade IV) on liver biopsy as a result of use of the PTF guidelines would have been missed with use of the ACR guidelines. The total cost for the group receiving biopsies based on the ACR guidelines would have been $16,956. Overall, the new ACR guidelines had 80% sensitivity and 82% specificity and resulted in a cost savings of $1,430 per patient. Conclusion. The new ACR guidelines on MTX monitoring and biopsy surveillance appear to be clinically useful and result in considerable cost savings. However, 1 IDDM patient with significant liver histologic abnormalities would have been missed. We suggest that IDDM be added to the ACR guidelines as a risk factor for MTX hepatotoxicity.

Rheumatoid arthritis (RA) is a chronic, systemic inflammatory disorder primarily targeting the synovium. Unchecked disease activity is associated with significant morbidity and an increased mortality. Recent advances in the understanding of the pathogenesis of RA and the capability of biologically engineered treatments for RA have expanded the armamentarium of antirheumatic agents.A systematic literature review was conducted through PubMed.At present, a common strategy for the treatment of RA uses methotrexate either as monotherapy or in combination with a variety of conventional and/or biologic disease-modifying antirheumatic drugs (DMARDs), with the goal of inducing remission of active disease. The choice of which agent(s) to use is based upon patient-specific criteria (activity of disease, comorbidities, patient preferences, costs etc.). Emerging therapies that target specific cytokines and growth factors in the inflammatory cascade of RA offer a potent new means of modifying disease activity, but many questions regarding their use remain unanswered.

We describe a case of a young woman with a magnesium renal wasting syndrome leading to severe hypomagnesemia and a chronic pseudo-osteoaritis calcium pyrophosphate crystal deposition disease (CPDD) arthropathy. A chronic CPDD arthropathy secondary to hypomagnesemia has not been previously reported. The identification of CPDD, particularly in the young, can be a clue to the presence of a potentially treatable underlying metabolic disorder, such as hypomagnesemia.

The availability of biologic agents targeting tumor necrosis factor (TNF)-alpha represents a significant advance in the management of rheumatoid arthritis. Anti-TNF-alpha therapy has been associated with dramatic improvements in the clinical signs and symptoms of rheumatoid arthritis and has been shown to greatly retard the destructive process that too often characterizes this condition. Although effective and well-tolerated in a substantial proportion of patients, primary and secondary failures of anti-TNF-alpha strategies have been well described, affecting up to one-third to one-half of subjects treated with these agents. Switching from one anti-TNF-alpha agent to a second (or even third) anti-TNF-alpha therapy has emerged as a means of addressing treatment failures with this drug class. This review examines data addressing the practice of switching anti-TNF-alpha agents in the context of initial treatment failure, with a focus on data from peer-reviewed reports.

We present a 42-year-old woman with primary Sjogren's syndrome and a polyclonal gammopathy who presented with pretibial petechiae, purpura, and tender indurated plaques. The indurated plaques revealed a lobular plasma cell panniculitis, and thus Sjogren's syndrome should be added to the short list of collagen vascular diseases that can present as plasma cell panniculitis. Her biopsies also demonstrated intense perieccrine plasma cell infiltrates that may account for Sjogren's syndrome-associated hypohidrosis. We also observed occasional vascular occlusion of vessels with an amorphous, eosinophilic material possibly related to her hypergammaglobulinemic purpura.

To examine the potential relationship between gout and hypothyroidism.Fifty-four consecutive patients with a diagnosis of monosodium urate crystal-proven gouty arthritis on joint aspiration were prospectively evaluated for hypothyroidism with an ultrasensitive thyroid-stimulating hormone (TSH) assay. Twenty-five patients with a diagnosis of monosodium urate crystal-proven gout were retrospectively identified from a population of 137 patients receiving uric acid-lowering medications. These patients were also screened for hypothyroidism. Age, race, sex, and weight matched patients with noninflammatory rheumatic diseases and no history of gout served as controls. Hypothyroidism was diagnosed when a TSH was greater than 6.0 microU/mL or if a history of hypothyroidism requiring replacement therapy was documented.The prevalence of hypothyroidism in the prospective group was significantly increased compared to controls (P0.05). Overall 15% of these patients, 25% of women and 12% of the men, had hypothyroidism. These rates were 2.5 times greater in women and 6 times greater in men than found in the controls. The mean TSH of the prospective gouty patients was also significantly greater than those levels found in control patients (5.2 +/- 12 versus 1.8 +/- 1.1 microU/mL, P0.05, chi-square), even when all abnormally elevated TSH values were excluded from analysis. The prevalence of hypothyroidism in the retrospective group was even higher: 20% overall, 40% in women and 15% in men.The prevalence of hypothyroidism is significantly increased in patients with aspirate-proven gouty arthritis. Screening for hypothyroidism with an ultrasensitive thyroid-stimulating hormone assay should be considered in all patients presenting with gouty arthritis and those with a history of recurrent gouty flares.