Contextual factors influence work outcomes in employed patients with ankylosing spondylitis starting etanercept: 2-year results from AS@Work.

Objectives: The aim was to determine changes over time in work outcomes and investigate the predictive value of baseline personal and work-related factors on the evolution of work outcomes among employed patients with AS initiating etanercept. Methods: Employment status, absenteeism and presenteeism were assessed using the Work Productivity and Activity Impairment for AS questionnaire in a 24-month open-label, observational study (NCT01421303). The potential effect of baseline factors on work outcomes was analysed using predictive modelling (Cox regression and linear mixed models). Results: After 24 months, 11/75 (14.7%) patients had permanently withdrawn from employment (seven because of AS). Absenteeism and presenteeism decreased significantly within 6 months of etanercept treatment and remained stable thereafter. Predictive modelling indicated that male sex (hazard ratio = 0.18; 95% CI: 0.04, 0.85), (log) number of working hours per week (hazard ratio = 0.13; 95% CI: 0.03, 0.51) and the possibility of developing skills (hazard ratio = 0.42; 95% CI: 0.19, 0.91) positively influenced time in employment. Over time, lower absenteeism was significantly associated with the quality of contact with colleagues [coefficient (s.e.): -0.35 (0.10)] and importance of the job for quality of life [-0.49 (0.17)], and higher absenteeism with current smoking [1.66 (0.44)] and change in job because of illness [1.51 (0.66)]. Over time, lower presenteeism was associated with male sex [-14.5 (2.64)], the possibility of postponing work [-6.60 (2.73)], quality of contact with colleagues [-2.04 (0.96)] and >50 workers in the company [-7.65 (2.76)], and higher presenteeism with manual profession [8.41 (2.72)]. Conclusion: Contextual factors influence work outcomes over time and should not be ignored when aiming to improve work outcomes in patients with AS. Trial registration: ClinicalTrials.gov, http://clinicaltrials.gov, NCT01421303.

Degree of bone marrow oedema in sacroiliac joints of patients with axial spondyloarthritis is linked to gut inflammation and male sex: results from the GIANT cohort.

INTRODUCTION: Bone marrow oedema (BMO) of the sacroiliac joints (SIJs) is a hallmark of axial spondyloarthritis (SpA). However, the relationship between the extent of BMO and disease phenotype is poorly understood. OBJECTIVE: To assess the link between BMO of the SIJs and gut inflammation. We have also evaluated the correlation between BMO and established disease activity parameters. METHODS: Sixty-eight patients with axial SpA from the Gent Inflammatory Arthritis and spoNdylitis cohorT underwent ileocolonoscopy and MRI of the SIJs. Histopathological analysis and SPondyloArthritis Research Consortium of Canada (SPARCC) scores were performed. RESULTS: A significant higher SPARCC score (median (range)) was observed in axial SpA patients showing chronic gut inflammation (16.9 (3.8-68.3)) compared with axial SpA patients showing normal gut histology (9.8 (0.0-45.0); p<0.05). In a multiple linear regression model, we identified, besides chronic gut inflammation (effect size of 11.3, 95% CI (2.1 to 20.4)), male sex (effect size of 10.5, 95% CI (3.3 to 17.8)) to be independently associated to the extent of BMO. There was a low to moderate correlation between the degree of BMO and C-reactive protein(r=0.39, p=0.002) and Ankylosing Spondylitis Disease Activity Score (r=0.35, p=0.007). CONCLUSIONS: Higher degrees of BMO were observed in patients showing chronic gut inflammation. These data solidify a link between mucosal inflammation and progressive disease in axial SpA.

Microscopic gut inflammation in axial spondyloarthritis: a multiparametric predictive model.

OBJECTIVE: To assess the rates and explore predictors of microscopic gut inflammation in a cohort of patients with axial and peripheral spondyloarthritis (SpA). METHODS: Ileocolonoscopy was performed in 65 patients with axial and peripheral SpA from the Gent Inflammatory Arthritis and spoNdylitis cohorT. Histopathological analysis and scoring were performed by an experienced pathologist. RESULTS: Overall, 46.2% of the patients with SpA showed microscopic gut inflammation. In axial SpA, the following parameters were independently associated with gut involvement: male sex (OR=8.9, p=0.035); high disease activity measured by the Bath Ankylosing Spondylitis Disease Activity Index (OR=2.05, p=0.032); restricted spinal mobility measured by the Bath Ankylosing Spondylitis Metrology Index (OR=1.94, p=0.009); and younger age (OR=0.85, p=0.013). No clear association was found for human leucocyte antigen-B27 status, presence of peripheral arthritis, enthesitis, uveitis, psoriasis, intake of non-steroidal anti-inflammatory drugs and family history of SpA. The prevalence of gut inflammation in non-radiographic axial SpA and ankylosing spondylitis was comparable. CONCLUSIONS: The prevalence of microscopic gut inflammation in SpA remains unaltered over time. Younger age (shorter symptom duration), progressive disease, male sex and higher disease activity are independently associated with microscopic gut inflammation in axial SpA.

Comparison of two referral strategies for diagnosis of axial spondyloarthritis: the Recognising and Diagnosing Ankylosing Spondylitis Reliably (RADAR) study.

OBJECTIVE: To determine which of two referral strategies, when used by referring physicians for patients with chronic back pain (CBP), is superior for diagnosing axial spondyloarthritis (SpA) by rheumatologists across several countries. METHODS: Primary care referral sites in 16 countries were randomised (1 : 1) to refer patients with CBP lasting >3 months and onset before age 45 years to a rheumatologist using either strategy 1 (any of inflammatory back pain (IBP), HLA-B27 or sacroiliitis on imaging) or strategy 2 (two of the following: IBP, HLA-B27, sacroiliitis, family history of axial SpA, good response to non-steroidal anti-inflammatory drugs, extra-articular manifestations). The rheumatologist established the diagnosis. The primary analysis compared the proportion of patients diagnosed with definite axial SpA by referral strategy. RESULTS: Patients (N=1072) were referred by 278 sites to 64 rheumatologists: 504 patients by strategy 1 and 568 patients by strategy 2. Axial SpA was diagnosed in 35.6% and 39.8% of patients referred by these respective strategies (between-group difference 4.40%; 95% CI -7.09% to 15.89%; p=0.447). IBP was the most frequently used referral criterion (94.7% of cases), showing high concordance (85.4%) with rheumatologists' assessments, and having sensitivity and a negative predictive value of >85% but a positive predictive value and specificity of <50%. Combining IBP with other criteria (eg, sacroiliitis, HLA-B27) increased the likelihood for diagnosing axial SpA. CONCLUSIONS: A referral strategy based on three criteria leads to a diagnosis of axial SpA in approximately 35% of patients with CBP and is applicable across countries and geographical locales with presumably different levels of expertise in axial SpA.

Development of new syndesmophytes and bridges in ankylosing spondylitis and their predictors: a longitudinal study.

BACKGROUND: Structural damage of the spine in ankylosing spondylitis (AS) is associated with worse physical function and impaired spinal mobility. Knowledge about predictors of new syndesmophyte formation is limited. OBJECTIVES: To assess the development of new syndesmophytes at the level of individual vertebral bodies and to assess predictors for this development. METHODS: Clinical and radiological data from 132 patients from the Outcome in Ankylosing Spondylitis International Study for whom complete sets of radiographs were available at baseline and at 2- and 4-year follow-up were used. Univariable and multivariable logistic regression analyses were performed to identify predictors associated with development of new syndesmophytes within 4 years. RESULTS: At baseline, 81 (61%) patients had syndesmophytes. New syndesmophytes developed in 44 (33%) patients within 2 years and in 63 (48%) patients within 4 years. The RR of developing new syndesmophytes was 5.0 (95% CI 2.5 to 10.2) at 4 years in patients with existing syndesmophytes as compared with patients without. In the univariable analysis, older age, worse functional status, male gender, erythrocyte sedimentation rate and existing syndesmophytes were associated with development of new syndesmophytes at 4 years. In the multivariable logistic regression analysis, only the presence of existing syndesmophytes was a significant predictor (OR 18.72, 95% CI 6.44 to 54.42). When existing syndesmophytes were taken out from the model, age (OR 1.07, 95% CI 1.03 to 1.11) and male gender (OR 3.98, 95% CI 1.47 to 10.77) were statistically significant contributors. CONCLUSION: In AS, patients with existing syndesmophytes are prone to develop new syndesmophytes over time.

Are syndesmophytes most prevalent in the lumbar or in the cervical spine in patients with ankylosing spondylitis and do they develop in a specific direction?

OBJECTIVES: To describe the distribution of prevalent syndesmophytes and bridges, and the occurrence of new ones in a prevalence cohort of patients with AS. METHODS: Clinical and radiological data from 132 patients from the Outcome in Ankylosing Spondylitis International Study of which complete sets of radiographs were available at baseline and at 2- and 4-year follow-up were used. RESULTS: At baseline, 81 (61%) patients, of which 17 (45%) were females and 64 (65%) males (P = 0.03), had prevalent (bridging) syndesmophytes. Both syndesmophytes and bridges were found at all vertebral levels. Syndesmophytes were more frequently seen in the cervical spine compared with the lumbar spine (mean per vertebral level 17.5 vs 11.2%, respectively, P = 0.01). Bridges were more frequently seen in the lumbar spine compared with the cervical spine (mean per vertebral level 16.9% vs 12.1%, P = 0.02). With increasing disease duration more (bridging) syndesmophytes were found, occurring similarly at the lumbar and cervical spines. After 2- and 4-years of follow-up, new (bridging) syndesmophytes developed throughout the entire cervical and lumbar spines. CONCLUSION: In general, syndesmophytes occur more frequently in the cervical spine and bridges more frequently in the lumbar spine, but neither a specific predilection site nor any particular order for occurrence and development of syndesmophytes could be detected.

Mucosal inflammation in spondylarthritides: past, present, and future.

Spondylarthritides (SpA) and inflammatory bowel disease (IBD) are idiopathic, chronic inflammatory disorders. Although they are very distinct and well-defined entities, there is clinical and genetic evidence supporting some degree of overlap between the pathogenesis of the two. Subclinical gut inflammation is present in up to two thirds of all SpA patients and can evolve into IBD. This subclinical gut inflammation has been shown to be strongly associated with joint inflammation, providing a clue for a common pathophysiologic background. Despite extensive research progress in the field over the past few years, many questions remain unanswered. In this paper, we focus on the clinical, genetic, and pathophysiologic overlap of SpA and IBD. Furthermore, we discuss some of the targets that may influence therapeutic decision making.

Interactions between gut inflammation and arthritis/spondylitis.

PURPOSE OF REVIEW: The spectrum of spondyloarthritis is characterized by the intriguing co-occurrence of gut and joint inflammation, although no obvious anatomical link exists. RECENT FINDINGS: Data from animal models identify stromal cells as important players in pathogenesis, although signalling through TNFRI appeared to be sufficient for development of combined gut and joint inflammation. Interleukin-23 receptor was identified as a susceptibility locus for ankylosing spondylitis. SUMMARY: Human genome studies combined with animal model research provide us with new evidence in the fascinating field of the gut-joint axis. However, how these newly identified genetic associations can influence the immunological environment remains to be elucidated.

Systemic levels of IL-23 are strongly associated with disease activity in rheumatoid arthritis but not spondyloarthritis.

OBJECTIVES: Th17 cells are an effector T-cell population that plays a role in chronic inflammatory conditions and is dependent on IL-23 for their survival and expansion. More recently, a genetic association was discovered between polymorphisms in the gene coding for the IL-23 receptor and spondyloarthritis. This study aimed to evaluate the role of Th17-associated cytokines in spondyloarthritis pathogenesis by measuring their levels in the joints and circulation as well as correlating them with disease activity parameters. METHODS: Paired synovial fluid (SF), serum and synovial biopsies were obtained from 30 non-PsA (psoriatic arthritis) spondyloarthritis, 22 PsA and 22 rheumatoid arthritis (RA) patients. IL-17, IL-23 and CCL20 were measured by ELISA in the SF and serum of patients and correlated with systemic and local parameters of disease activity. RESULTS: Concentrations of CCL20, a major Th17-attracting chemokine, tended to be higher in the joints of RA than in spondyloarthritis patients. Interestingly, levels of CCL20 were markedly higher in SF as opposed to serum. In addition, there was a remarkable association between the expression of the Th17 cytokine system and the presence of intimal lining layer hyperplasia in RA. Also in the serum, there was a tendency for higher IL-23 levels in RA, which correlated strongly with disease activity parameters. CONCLUSIONS: Th17-related cytokines are expressed in joints of spondyloarthritis as well as RA patients. IL-23 levels, however, correlate with disease activity parameters in RA only. These results point towards a differential regulation of the Th17 cytokine system in spondyloarthritis compared with RA.

Clinical assessment in the spondyloarthropathies.

In order to measure disease activity, progression and response to therapy, it is important to use accurate, reliable and feasible outcome measures that can ideally be used in longitudinal cohorts, clinical trials and clinical practice. With emerging therapies, the focus on the methodology of outcome assessment has increased to ensure that discriminant and responsive instruments are used. This chapter reviews available measures of three major areas of disease impact in the spondyloarthropathies (disease activity, structural damage and functioning) and discusses the relevance for use in clinical practice. First, the outcome measures available for the assessment of different domains in ankylosing spondylitis, composite-indices and response criteria for use in clinical trials and clinical practice in ankylosing spondylitis are discussed. Secondly, the performance of these in psoriatic arthritis and more disease-specific instruments in psoriatic arthritis are discussed.

Spondyloarthropathies: progress and challenges.

The spondyloarthropathies are a group of human rheumatic disorders that are often associated with extra-articular features. Although a substantial number of studies is undertaken each year, many issues concerning the pathogenesis remain unanswered. There are several unresolved questions with regard to pathogenesis and treatment in spondyloarthropathies. First, the precise sites where inflammation originates within the joints have been a matter of controversy as enthesitis, synovitis and even bone marrow inflammation can occur during the course of spondyloarthropathies. In addition, the genetic predisposition involved in the origin of the close linkage between gut and joint inflammation, a prominent feature of SpA, has gathered much attention lately. Finally, whereas the effect of tumour necrosis factor (TNF) blockers in modulating inflammatory symptoms in SpA is well established, their ability to prevent new bone formation is much less certain. In addition, some marked differences appear to exist in the ability of the different TNF-blocking agents to modulate extra-articular disease manifestations.

Clinical and genetic factors associated with sacroiliitis in Crohn's disease.

BACKGROUND AND AIM: Radiographic sacroiliitis (SI), often asymptomatic, is considered the most frequent extra-intestinal manifestation (EIM) of Crohn's disease (CD). Data on the association of SI with other clinical features of CD are limited. Association of SI with CARD15 polymorphisms has recently been suggested. In a multicenter study, we investigated the association of SI in CD patients with clinical phenotypes, other EIM and CARD15 polymorphisms. METHODS: Radiographs of the sacroiliac joints were taken in 251 CD patients from three Belgian university hospitals and scored by two blinded rheumatologists. Clinical features were obtained from medical records. Forty-three percent of patients carried at least one CARD15 polymorphism. RESULTS: Sacroiliitis, defined as the presence of at least grade 2 unilateral changes, was diagnosed in 65 of the 244 scorable radiographs (27%). Only 16 of these patients were previously diagnosed with ankylosing spondylitis (AS). HLA-B27 positivity was observed in 53% of patients with AS and 7% of patients with radiographic SI. In univariate and multivariate analysis, associations between the presence of SI and peripheral arthritis (P = 0.005) and between AS and uveitis (P = 0.005) were found. No associations with other recorded clinical features or with CARD15 polymorphisms were observed. CONCLUSION: We confirm the high prevalence of radiographic sacroiliitis in a multicenter CD cohort. Uveitis is only associated with AS whereas all patients with SI are more prone to develop peripheral arthritis during their disease course, suggesting similar pathogenetic mechanisms in the development of these EIM. The previously reported association between SI and CARD15 polymorphisms was not confirmed.

Bilateral spontaneous pneumothorax in a patient with pulmonary rheumatoid nodules, secondary infected by Aspergillus.

This case report describes a 50-year-old woman with rheumatoid arthritis (RA) in whom nodular opacities were found on chest X-ray. She developed a bilateral spontaneous pneumothorax treated with surgical pleurodesis. Cultures remained negative. Histological examination of specimens confirmed the clinical diagnosis of rheumatoid granulomata. Therefore, corticosteroid therapy was started, after which the nodules decreased slightly in size and inflammatory parameters normalized. Three months later, she presented with respiratory insufficiency based on pulmonary fungus infection. Differential diagnosis between rheumatoid nodules and granulomas caused by Aspergillus is difficult in RA patients with pulmonary nodular lesions; in this case, both complications appeared subsequently.

To be or not to be rheumatologist: survey among Belgian medical students and internal medicine trainees: what do certified rheumatologists think about the current rheumatology training program?

In several countries, there have been increasing concerns over the years that fewer medical students or trainees choose rheumatology as a specialty. The aim of this three-step survey is to study the motivational factors for students and trainees in internal medicine to choose for rheumatology as a future career option and the idea among experienced rheumatologists about the needs for changes in the training program. An online survey was distributed among students in medical training (in the final 3 years) and trainees in internal medicine from the Ghent University and University Hospital. Questions concerned the level of clinical exposure to rheumatology and the motivation about becoming rheumatologist. Next, experienced rheumatologists were asked about the needs to change the current training. Descriptive data are shown and chi-squared tests were calculated to assess differences between groups (based on gender and exposure). Logistic regression was performed to study associations between demographic variables and choosing rheumatology as career. Only a minority of students (17%) and about half of trainees (45%) were ever exposed to rheumatology. Only 11% of students and 17% of trainees considered becoming rheumatologist. There was no difference in choice based on gender but previous exposure seemed to play an important role, and especially during the pre-specialty years. Univariate logistic regression identified the year of training and exposure as predictors for choosing rheumatology. Multivariate analysis only retained exposure as significantly associated (odds ratio (95% CI) = 2.88 (1.51-12.58)). Rheumatology is considered to be a fascinating discipline among Belgian students and trainees. Exposure during pre-specialty years is the strongest predictor for choosing rheumatology as future career option.

High Prevalence of Undiagnosed Axial Spondyloarthritis in Patients with Chronic Low Back Pain Consulting Non-Rheumatologist Specialists in Belgium: SUSPECT Study.

INTRODUCTION: Diagnosis of axial spondyloarthritis (SpA) can be delayed for several years mainly because of low awareness of axial SpA among non-rheumatologists who are the first interlocutors of potential SpA patients. One strategy to decrease the delay between appearance of first symptoms and diagnosis of axial SpA and to allow early management of the disease is to provide the non-rheumatologists with tools to identify patients requiring prompt referral to rheumatologists. This study was designed to evaluate in a real-world setting whether screening patients with chronic low back pain who consult physical medicine and rehabilitation (PMR) physicians, orthopedists, and ophthalmologists is useful in detecting axial SpA. METHODS: During this non-interventional cross-sectional study, data from 161 patients with chronic back pain, consulting an orthopedist, PMR physician, or ophthalmologist were collected during a single visit. Any patient who presented with at least four out of five symptoms of inflammatory back pain (IBP) and at least one additional SpA feature were to be referred to a rheumatologist. Analysis was purely descriptive. RESULTS: IBP was diagnosed in approximately half of the patients (89 patients) and 72 of them met the referral criteria. A total of 117 patients were finally referred to a rheumatologist and axial SpA was diagnosed for 37 of them. CONCLUSIONS: The high prevalence of undiagnosed axial SpA in patients with chronic back pain visiting PMR physicians, orthopedists, and ophthalmologists suggests that these healthcare professionals may play a key role in the strategy developed to shorten the delay observed in the formal diagnosis of SpA. FUNDING: Abbvie.

A Nationwide Survey on Patient's versus Physician´s Evaluation of Biological Therapy in Rheumatoid Arthritis in Relation to Disease Activity and Route of Administration: The Be-Raise Study.

OBJECTIVES: Biological treatment of rheumatoid arthritis (RA) is one of the cornerstones of current treatment strategies for the disease. Surprisingly little information exists on whether the route of administration affects patients' treatment satisfaction. It is equally unclear whether rheumatologists are able to accurately perceive their patients' appreciation. Thus, the Belgian Be-raise survey aimed to examine whether RA patient's experience of their current biological treatment coincided with the treating physician's perception. METHODS: A nationwide cross-sectional survey was conducted by 67 Belgian rheumatologists providing data obtained from 550 RA patients. Patients under stable dose of biologics for at least 6 months, were enrolled consecutively and all completed questionnaires. Separate questionnaires were completed by the treating rheumatologist which evaluated their patient's perception of the route of treatment administration. This study therefore evaluates whether a treating physician perceives the satisfaction with the route of administration to the same degree as the patient. RESULTS: Completed questionnaires were obtained from 293 and 257 patients who obtained treatment via the intravenous (IV) or subcutaneous (SC) route of administration, respectively. 58.4% of patients were in DAS28-CRP(3) remission. Patient satisfaction with disease control was higher (44% scored ≥ 9) than that of the treating physician (35%), regardless of the route of administration (p< 0.01). No differences were seen for the patients treated with an IV as opposed to a SC route of administration. The physician´s perception of patient's satisfaction with disease control was markedly lower for IV treated patients as opposed to SC treated patients (p< 0.001). CONCLUSIONS: Patients' satisfaction with biological treatment is high, but there is a considerable mismatch between patients´ and rheumatologists´ appreciation on the route of administration of biological therapy in RA. Physicians consistently consider IV biological therapy to be less satisfactory. Patient´s appreciation is largely dependent on disease control, irrespective of the route of administration. Therefore, and encouraging shared decision making, we suggest that physicians and patients discuss the route of administration of biologicals in an open way.

Rapidly growing gastric metastasis of Merkel cell carcinoma, an unusual cause of melena.

Merkel cell carcinoma (MCC) is an uncommon, highly aggressive neuroendocrine skin carcinoma that has a tendency for local recurrence and metastatic disease. We report a rare case of recurrent melena in a 77-year-old Caucasian male. Three years earlier, the patient had undergone a radical resection of a para-umbilical MCC. A repeat esophagogastroduodenoscopy proved necessary to identify rapidly proliferating gastric metastasis of MCC as the cause of bleeding.

Understanding limitations in at-work productivity in patients with active ankylosing spondylitis: the role of work-related contextual factors.

OBJECTIVE: To explore the effect of health-related and contextual factors on presenteeism, absenteeism, and overall work productivity loss in patients with active ankylosing spondylitis (AS). METHODS: Consecutive patients with AS starting their first tumor necrosis factor inhibitor and in paid employment were eligible. Patients completed the Work Productivity and Activity Impairment (WPAI) questionnaire for AS to assess presenteeism, absenteeism, and overall work productivity loss in the previous 7 days. In addition, they answered questions about work characteristics (type, characteristics of workplace, satisfaction of contacts with colleagues, and importance of work in life) and health status [Bath AS Functional Index (BASFI), AS Disease Activity Score-C-reactive protein (ASDAS-CRP)]. Physicians assessed the Bath Ankylosing Spondylitis Metrology Index, presence of articular and extraarticular manifestations, comorbidities, and laboratory indicators of inflammation. Stepwise regression models were computed to determine which work-related and health-related factors contributed to WPAI outcomes. RESULTS: The study included 80 patients. The WPAI presenteeism, absenteeism, and overall work productivity loss scores were 49.1%, 30.2%, and 53.1%, respectively. Presenteeism was associated with higher BASFI, female sex, and poor quality of contact with colleagues. Absenteeism was associated with increasing age, current smoking status, higher ASDAS-CRP, and low importance of work for life. Overall work productivity loss was associated with female sex, higher BASFI, past adaptation of job because of illness, number of working hours, and manual profession. CONCLUSION: Both health-related and contextual factors contribute to work limitations in patients with AS and suggest additional opportunities for improvement by addressing the working environment.

Sustained response to tocilizumab in a patient with relapsing polychondritis with aortic involvement: a case based review.

This paper presents a case with refractory relapsing polychondritis (RPC), complicated with severe aortic involvement, which is successfully treated with tocilizumab. Previous treatments consisted of methotrexate, corticosteroids, cyclosporine, cyclophosphamide, infliximab, and etanercept. With these treatments, the patient had recurrent episodes of fever, polyarthritis, tenosynovitis, subcutaneous nodules, and progressive cardiac disease. One year after the start of treatment with tocilizumab, there is resolution of all symptoms, normalization of C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), and the dose of prednisolone is tapered down to 2 mg/day. We have reviewed the English literature for reports of patients with refractory RPC, successfully treated with tocilizumab. We found five additional case reports. In one case report, a patient with refractory RPC complicated with aortitis was successfully treated with tocilizumab. In three case reports, patients with refractory RPC complicated with laryngotracheal involvement were successfully treated with tocilizumab. All cases had, like our patient, failed conventional treatment. We also reviewed the literature for reports of the effect of biologicals on cardiac involvement in RPC. Current literature is presented and discussed.