Amelia Spinella

Large Vessel Vasculitis Occurring in Rheumatoid Arthritis Patient under Anti-TNF Therapy.

Vasculitis is a heterogeneous group of disorders characterized by the presence of necrotic inflammatory phenomena and destruction of blood vessels. Vasculitis is classified as primary (idiopathic) or secondary to infections, connective tissue diseases and drugs but can also be considered as a paraneoplastic phenomenon. Evidence shows that the increasing use of biological agents results in a growing number of reports of autoimmune diseases induced by these therapies. An inflammatory articular chronic disease such as rheumatoid arthritis may be complicated by extra-articular manifestations, such as cutaneous or systemic vasculitis. Herewith, we describe the case of a great vessels arteritis in a patient affected by rheumatoid arthritis in therapy with an anti-TNF agent (etanercept).

Beyond Cat Scratch Disease: A Case Report of Bartonella Infection Mimicking Vasculitic Disorder

Cat scratch disease (CSD) is a bacterial disease caused by Bartonella henselae and it is mainly characterized by self-limiting lymphadenopathy in the draining site of a cat scratch or bite. We report a patient with history of fever, swelling lymph nodes, vasculitic-like skin lesions, and positivity of Bartonella serology initially considered as expression of a disimmune disease.

Erratum to “From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution”

[This corrects the article DOI: 10.1155/2018/1284687.].

Elevated Troponin Serum Levels in Adult Onset Still's Disease

Adult onset Stills disease (AOSD) is a rare inflammatory systemic disease that occasionally may affect myocardium. Diagnosis is based on typical AOSD symptoms after the exclusion of well-known infectious, neoplastic, or autoimmune/autoinflammatory disorders. In the case of abrupt, recent onset AOSD, it could be particularly difficult to make the differential diagnosis and in particular to early detect the possible heart involvement. This latter event is suggested by the clinical history of the four patients described here, incidentally observed at our emergency room. All cases were referred because of acute illness (high fever, malaise, polyarthralgias, skin rash, and sore throat), successively classified as AOSD, and they presented abnormally high levels of serum troponin without overt symptoms of cardiac involvement. The timely treatment with steroids (3 cases) or ibuprofen (1 case) leads to the remission of clinicoserological manifestations within few weeks. These observations suggest that early myocardial injury might be underestimated or entirely overlooked in patients with AOSD; routine cardiac assessment including troponin evaluation should be mandatory in all patients with suspected AOSD.

SAT0621 Validation Study of Predictive Value of Capillaroscopic Skin Ulcer Risk Index (CSURI) in Scleroderma Patients Treated with Bosentan

Background CSURI is a capillaroscopic index, able to identify scleroderma (SSc) patients at high risk for new or non-healing digital ulcers (DU) in the next three months. CSURI has been validated, only in patients not treated with bosentan, in a large multicenter study in 2011. Objectives To evaluate the predictive value of CSURI in SSc patients assuming bosentan for the prevention of DU. Methods Seventy-six consecutive SSc patients treated with bosentan were enrolled in a multicenter study (F/M 4.4/1; mean age 56.4±13.6 years; diffuse/limited cutaneous subset 30/44). All patients undergone to NVC and CSURI was calculated according to published studies. At baseline all patients had a history of at least one DU in the last year, and 26 patients (30.3%) showed a current DU. At the time of the study 76.3% of patients were also treated with intravenous prostanoids, while no patients was assuming bosentan for pulmonary arterial hypertension. Results After 3 months from NVC 18/26 patients showed non healing ulcers and 18/76 patients developed new DU. Receiver operator characteristic curve, performed to analyze the prognostic accuracy of CSURI in regard to DU development, is reported in the figure. The area under the curve (AUC) was 0.69 (95% CI 0.57-0.79, p=0.0019) and the higher sensitivity and specificity were observed for a CSURI value of 2.5 (sensitivity 94.4; specificity 57.5; positive and negative likehood ratio 2.22 and 0.097, respectively). At the validated cut-off value of 2.96 sensitivity was 86.1%, specificity 60.0%, positive and negative likehood ratio 2.15 and 0.23, respectively, showing a lower negative predictive value. Conclusions In patients treated with bosentan, CSURI shows a lower positive predictive value if compared with SSc population observed in our previous validation study, while the negative predictive value can be considered acceptable. The cause of this different result is not evaluable by our study. SSc peripheral microangiopathy is sustained by a multifactorial process, only partially known, involving a complex cytokines and cells network. In this picture, bosentan could reduce the incidence of new DU, without significantly interfere with the parameters included in CSURI calculation. Some Authors observed a general improvement of NVC parameters in SSc patients treated with bosentan. These data are not in contrast with our study since CSURI calculation is obtained by considering the “worst” capillaroscopic image. Moreover, a longer period of observation as in the other studies should more significantly influence changes in NVC parameters. In our previous studies, CSURI showed a higher predictive value than history of DU in detecting SSc patients at risk for new lesions. Anyway in this study this role of primary prevention cannot be applicable. Moreover, a special attention could be pointed on patients with recurrent DU, regardless specific treatments. A combined approach based on clinical picture and CSURI could probably help in managing therapy of SSc patients with DU, but only prospective clinical trial could clarify the correct role of NVC in the evaluation and management of vasoactive treatments. Disclosure of Interest None declared

Unusual association between Budd–Chiari syndrome secondary to antiphospholipid syndrome and relapsing polychondritis: a case report and review of the literature

Relapsing polychondritis is a rare immune-mediated condition, characterized by episodic inflammation of the cartilaginous tissue, in particular the ears, nose, and eyes, and involvement of joints and respiratory tract. Nearly one third of patients showed other associated diseases, such as systemic vasculitides, connective tissue diseases, or myelodysplastic syndromes. Antiphospholipid antibodies can be found in relapsing polychondritis in patients with no clinical thrombotic disease. However, when antiphospholipid syndrome is present, its clinical manifestations can be severe and life threatening. We describe the case of a patient with relapsing polychondritis associated to Budd–Chiari syndrome due to antiphospholipid syndrome. The present clinical observations together with the updated review of the literature suggest a search for antiphospholipid antibodies in all patients with relapsing polychondritis.

Efficacy of Belimumab for active lupus nephritis in a young Hispanic woman intolerant to standard treatment: a case report

BackgroundLupus nephritis (LN) is a frequent severe complication of Systemic Lupus Erythematosus (SLE), especially in patients of non-Caucasian ethnicity. Induction treatment for LN consists in the combination of steroids plus a second agent (cyclophosphamide or mycophenolate mofetil) or, as a second-line, calcineurin inhibitors or Rituximab. Induction treatment for LN can be complicated by a series of side effects, the most severe being serious infections. Belimumab is a fully humanized monoclonal antibody that targets soluble B lymphocyte stimulator (BLyS), approved for treatment of serologically active SLE in addition to standard of care.Case presentationA young Hispanic woman was diagnosed with SLE at the age of 15. After several immunosuppressive treatments for arthritic symptoms (high-dose steroids, mycophenolate mofetil, Rituximab, cyclophosphamide) leading to serious complications and scarce clinical improvement, she developed severe LN. Induction treatment with a combination of intravenous high-dose methylprednisolone and cyclophosphamide was started but, after few days, the patient developed cryptococcal meningitis. After institution of appropriate antifungal therapy, treatment with Tacrolimus was attempted but poorly tolerated by the patient and withdrawn. Eventually, Belimumab was initiated off-label as a last resource to treat LN. Belimumab was well tolerated by the patient and resulted in a rapid and marked improvement in clinical symptoms and reduction in proteinuria, serum complement levels and anti-dsDNA titer; of note, the patient developed no infectious complications.ConclusionsWe report the case of a severe LN in a young Hispanic woman who did not respond to conventional and second-line induction therapies, due both to intolerance and to the development of serious infectious complications. Eventually, Belimumab was successfully added to steroids and was well tolerated by the patient, resulting in a marked improvement in clinical and biochemical parameters. We suggest that Belimumab should be considered as a potentially efficacious treatment in patients with LN who cannot tolerate conventional therapies.

The profiling of axial spondyloarthritis patient candidate to a biologic therapy: Consensus from a Delphi-panel of Italian experts

OBJECTIVE The project aimed to collect expert consensus statements for the profiling of patients with axial spondyloarthritis (axSpA) candidate to biologic agents (bDMARDs) treatment, in order to better define the drivers for the best treatment choice. METHODS The 6 more interesting topics about axSpA patient profiling were identified by the project steering committee and a panel of axSpA Italian experts. A systematic literature review (SLR) was performed for each of the selected topics according to the PICO format. Two rounds of a modified Delphi process were conducted. In the 1st round, the steering committee evaluated the results of the SLR in order to formulate statements for each topic. In the 2nd round, the experts panel discussed, rephrased when needed, and voted the level of agreement (on a 5-point Likert-type scale) for each statement. Consensus was defined as ≥66% agreement. RESULTS The topics selected for the analysis were the differential efficacy of available bDMARDs on enthesitis/dactylitis, uveitis, radiographic progression and cardiovascular involvement, and the clinical response in non radiographic-axSpA and in patients receiving a second-line bDMARD. The Delphi rounds formulated 19 statements, all reaching the defined level of consensus in a second round including 25 rheumatologists highly skilled in the management of axSpA. CONCLUSION Identified consensus statements can help clinicians to apply to routine-care settings the results from clinical studies and international recommendations, providing a guide for individualization of treatment strategy in axSpA patients.

AB0760 Advanced oxidation protein products in serum of patients with systemic sclerosis: a possible indicator of clinical evolution

Background Systemic sclerosis (SSc) is a chronic, multisystem connective tissue disease characterised by by immune dys-regulation, obliterative microvasculopathy and fibrosis. Endothelial dysfunction, immune system imbalance and fibroblast activation constitute the three major factors of the pathogenetic process. In this context, oxidative stress could play a significant role through direct damage of endothelial cells and the persistent activation of the immune system.1,2 Objectives This study investigated the presence of advanced protein oxidation products (AOPP) in serum of patients with SSc and its correlation with disease’s features. Methods 50 patients with SSc (M:F 1:7, mean age 57.3±11.2 SD, mean duration of disease 10±9.1 SD years), were screened for AOPP in the serum, using the AOPP OxiSelect Kit of CELL BIOLABS (San Diego, Ca, USA). Among 50 SSc patients, 39 had limited cutaneous subset, while 11 had the diffuse one. Anamnestic and clinical data were collected for all SSc patients. As a control group 50 consecutive healthy subjects, sex and age matched, were recruited. Results We found serum levels of AOPP increased in the SSc group compared with the controls (p<0.0001) with mean values of 336.9±167.8 mmol/L and 167.5±59.2 mmol/L, respectively. In addition, higher levels of AOPP directly correlated with the diffuse cutaneous subset (p=0.0242), presence of digital ulcers (p=0.005), esophagopathy (p=0.006) and pulmonary fibrosis (p=0.0128). Conclusions Serum AOPP levels are significantly higher in patients with SSc than in controls. In addition, the correlations of AOPP with SSc diffuse cutaneous subset, digital ulcers, and pulmonary involvement (indicative of progressive disease and worse prognosis) suggest a possible role of this marker in the identification of the cases with worse clinical evolution. The data of this preliminary study should be confirmed on larger case series and analysed in prospective studies, in order to understand its eventual usefulness during the follow-up of SSc patients. References [1] Ferri C, Valentini G, Cozzi F, Sebastiani M, Michelassi C, La Montagna G, et al. Systemic Sclerosis: Demographic, Clinical, and Serologic Features and Survival in 1,012 Italian Patients. Medicine (Baltimore). 2002; 81:139–153 [2] Steen VD. The many faces of scleroderma. Rheum Dis Clin North Am. 2008; 34:1–15 Disclosure of Interest None declared

AB0844-HPR Dance class dedicated to scleroderma patients: joint evaluation, functional and quality of life before and after treatment – clinical trial

Background Systemic sclerosis (SSc) is a complex and multi systemic disorder of connective tissue, characterized by progressive thickening and fibrosis of the skin and internal organs, as well as by widespread microvascular damage. A number of studies have described in these patients a high prevalence of depressive symptoms, pain, and body image dissatisfaction. Physical and occupational therapy showed to be useful in preserving mobility in patients with scleroderma but no specific study was found that investigated exactly what kind of activity could improve disability, encourage participation and improve mood. Objectives Dance (as a work of stretching, coordination, strength, aerobic, rhythmic, and recreational activity) could lead not just to accept and reconnect the patients with their bodies, enhance self-expression, address feelings of isolation and depression, but also to improve joint width, functionality, and autonomy. The study aims to compare the effects of dance/movement therapy and standard care with standard care alone in patients with systemic sclerosis. Methods 15 female patients diagnosed with SSc were divided into an experimental group -A-(5 patients enrolled spontaneously, considering that not all are willing to participate) and in a control group-C- (10 patients). The group A followed a dance routine devotedly for 4 months (1 hour twice a week). at T0 and after 4 months were evaluated: Subjective Functionality upper limb (QUICK DASH), Autonomy of daily living (SHAQ Scleroderma- Health Assessment Questionnaire), mood ( CES-D), Fatigue (FS Scale) Objective- Six Minutes Walking Test (6MWT), ROM of the shoulder and hip. In both groups, the changes in the outcome measures between the baseline and 16-week assessments were assessed with a paired t-test. Results There has been a statistically significant improvement in the group A in the objective evaluation: joint ROM of flexion of the hip and shoulder, meters walked in 6MWT(p< 0.03), test Borg dyspnea before the 6MWT is reduced from 0.6 to 0 and after the 6MWT is reduced from 2 to 0.8, while in the control group both remain unchanged (from 0.8 to 1 and 2.1 to 2.2). The self-assessment questionnaires show in the dancer group an improvement in the perception of fatigue (p <0.02) and of mood (p <0.01). No improvement was recorded in HAQ (0.58 to 0.65) and in the functionality of the upper limb (Quick Dash) due to worsening in work items (from 21.9 to 46.2) and a marked improvement in the free time items (from 40.6 to 21.5) probably attributable to awareness of disease. The results of the objective and subjective tests at time 0 and after 4 months were unchanged in the control group. Conclusions The results should be interpreted with caution given the small sample. However, it can be said that the experimental dance program has brought a gain in aerobic performance, perception of fatigue, mood as well as hip and shoulder joints flexibility. The patients indicated that they liked the course and continued to dance even at the end of the trial outside of the dance school References – Maddali Bongi S (2006):Riabilitazione reumatologica. Approccio multidisciplinare. Milano, Edizioni EDRA – Poole JL. Musculoskeletal rehabilitation in the person with scleroderma. Curr Opin Rheumatol. 2010;22(2):205–12 Disclosure of Interest None Declared