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Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Background: Approximately 5% of idiopathic recurrent pericarditis (IRP) patients are refractory or intolerant to NSAIDs, Colchicine and corticosteroids. The empiric treatment approach for these patients includes immunosuppression with Azathioprine (AZA) or immunomodulation with intravenous human immunoglobulin (IVIG). We assessed the efficacy and safety of long-term Anakinra treatment in refractory IRP patients after failure of prior immunosuppressive therapy and/or failure of IVIG., Methods: Clinical data of seven IRP patients were retrospectively analyzed. Treatment efficacy was determined by decrease of IRP recurrence and by the ability to withdraw or taper corticosteroids without a relapse. Safety was assessed by the occurrence of adverse events., Results: 7 IRP patients (4 male, median age 41) with a median disease duration of 4 years (range: 1.25-9 years) were treated with Anakinra (median treatment duration: 20 months). All patients were resistant or intolerant to NSAIDs, Prednisone, Colchicine and at least one immunosuppressive or immunomodulatory drug such as AZA, Methotrexate, Plaquenil, or IVIG. The median number of recurrences before Anakinra was 6 (range: 4-7) and all patients were corticosteroid-dependent and had steroid-related side effects. After initiation of Anakinra, none of the patients had IRP relapse. Prednisone was tapered down to 5 mg/day or less in all patients. Four patients discontinued prednisone altogether. No significant adverse effects have occurred as a result of Anakinra treatment and all patients continued treatment after the study period., Conclusion: Long-term Anakinra is a rapid-acting, efficient and safe steroid sparing agent even for patients with IRP refractory to previous immunosuppressive and/or immunomodulatory agents., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Golimumab is a recombinant human monoclonal antibody targeted against tumour necrosis factor-alpha (TNF-α). Golimumab is effective in the management of patients with active psoriatic arthritis (PsA). The aim of this study is to evaluate the clinical efficacy and survival of golimumab monotherapy versus co-administration with methotrexate (MTX) in patients with PsA in the clinical practice. This retrospective observational trial included patients with PsA. Efficacy was assessed by disease activity scores - DAS28, BASDAI, physician global assessment of disease (PGA) and CRP. Golimumab survival rate was estimated using the Kaplan-Meier analysis and univariate and multivariate Cox regression models. Forty-one patients with PsA were recruited; 26 patients were treated with golimumab, whereas 15 patients received combination therapy with MTX. The treatment resulted in significantly improved clinical measures of disease activity in comparison with baseline, including DAS28 CRP (4.1 vs 2.6, p ≤ 0.0001) and BASDAI (5.6 vs 3.8, p ≤ 0.001). Overall, 29 (71%) patients continued golimumab treatment (18 patients on monotherapy and 11 on combination therapy). The difference in the duration of golimumab survival between the combination therapy and monotherapy groups was not statistically significant (12.5 vs 12 months, p = 0.2). Similar efficacy profiles and survival rates were documented in patients with PsA regardless of the co-administration of methotrexate., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Specific inflammatory pathways and specifically Tumor Necrosis Factor alpha (TNF-α) have been associated with the neurodegeneration in Parkinson's disease (PD). TNFα is also known to play an important role in the pathogenesis of sarcoidosis and TNF blockers can ameliorate the disease. In contrast, multiple sclerosis (MS) is clearly exacerbated by anti- TNF-α medications. We have therefore hypothesized that Parkinson-like disease would be more common in neurosarcoidosis (NS) compared to MS. The aim of this case-control study was therefore to assess the frequency of extrapyramidal signs in patients with NS compared to MS patients. In order to do so the medical records of NS patients and of age and gender matched MS patients were reviewed and data regarding the clinical features, ancillary tests performed, treatment, and outcome were documented. Patients were then examined in a uniform manner for the presence of extrapyramidal signs. We found that in the NS group 8 patients had minor signs, one had mild functional disability and 3 subjects had significant extrapyramidal signs compatible with the diagnosis of Parkinson's disease. All extrapyramidal signs found in 5 of the MS group were minor. The proportional severity of extrapyramidal signs was significantly higher (p=0.045, chi square test) in the NS group compared to the MS group. We conclude that the specificity of extrapyramidal to NS raises the intriguing question of whether specific inflammatory pathways involving TNF-α play a role in the pathogenesis of PD and therefore may be a therapeutic target., (Copyright © 2017 Elsevier GmbH. All rights reserved.)

Background: Previous studies have shown that with prophylactic colchicine 65% of the patients suffering from Familial Mediterranean fever (FMF) will show a complete response, 30% a partial response and about 5% will show minimum or no response. These studies were performed before the isolation of the disease gene. Genotyping enables us to study the response rates according to specific mutations. We have witnessed a large number of M694V homozygotes who do not respond well to colchicine despite being treated with maximal sustained doses., Aim: To assess the response rates to colchicine in M694V homozygote FMF patients in comparison to other prevalent genotypes., Methods: We conducted a telephonic survey which included 112 FMF patients: 40 M694V homozygotes, and 2 comparison groups of 41 M694V/V726A compound heterozygotes and 31 V726A homozygotes. The questionnaire included demographic, social and clinical features, colchicine dose, response rates and reported side effects., Results: M694 homozygotes showed a more severe disease, and were treated with higher doses of colchicine (average dose 1.98±0.56 compared to 1.47±0.58, p=0.0001 and 1.13±0.41, p<0.001 in the M694V/V726A compound heterozygotes and the V726A homozygotes, respectively); Colchicine related side effects were noted in 40% of the M694V homozygotes. The average rate of attacks in treated M694V homozygotes (0.70±1.06) was higher compared to the two other groups (0.14±0.26, p=0.002 and 0.08±0.20, p=0.0009, respectively) and only 25% of them reported no attacks in the last year. None of the patients who took part in this study had amyloidosis. Side effects limiting the dose of colchicine were noted in 40% of the M694V homozygotes., Conclusions: Despite receiving higher doses of colchicine the prevalence of complete responders among M694V homozygotes is much lower than previously appreciated. The results highlight the need for additional treatment modalities for these patients., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Systemic sclerosis is a systemic, inflammatory, autoimmune disease affecting the skin and viscera, manifesting pathologically with microvascular lesions, perivascular infiltration by mononuclear cells and increased deposition of extracellular collagen. The rarity of the disease as well as its propensity to appear in the early 1940s, explain the low frequency of concurrent scleroderma and pregnancy. However, the marked female excess, as well as the trend for increasing maternal age due to social change and assisted reproductive technologies, renders heightened significance to issues of fertility, pregnancy course and pregnancy outcomes. In the past, scleroderma patients were thought to be at high risk for poor fetal and maternal outcome, but more current retrospective studies show that despite an increased frequency of prematurity and small for gestational age infants, overall maternal and neonatal survival is good. Hence, at present, with close monitoring and appropriate therapy most scleroderma patients can sustain a successful pregnancy. Therapy with hydroxychloroquine and low dose steroids as well as judicious use of intravenous immunoglobulins is permitted. Renal crisis remains the most dreaded complication of a scleroderma pregnancy and necessitates prompt institution of ACE inhibitor therapy despite its potential teratogenicity. In order minimize the risk for renal crisis, pregnancies should be avoided in rapidly progressive diffuse disease as such patients are at a greater risk for developing serious cardiopulmonary and renal problems early in the disease. This review shall focus on the bi-directional effects of scleroderma on fertility and pregnancy as well as on the management of pregnancy and delivery in the scleroderma patient., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Introduction: MRI and MRA are used for diagnosis and activity determination of patients with Takayasu's arteritis (TA). However, there is a limited experience regarding the role of MRI in long-term follow-up of those patients. The aim of the present study was to evaluate the clinical usefulness of MRI in the long-term follow-up of patients with Takayasu's disease., Materials and Methods: The clinical data of 11 TA patients, who obtained two or more follow-up MRI scans, was matched with the imaging results. MRI examinations were considered positive for disease activity when one of the following findings was noted: new arterial wall enhancement or interval appearance of anatomical changes (interval dilatation, stenosis or occlusion or new arterial wall irregularity). Conversely, MRI examinations were considered to show signs of improvement when local enhancement disappeared, or when a stenosis was relieved. Disease activity was determined by the combination of worsening localizing ischemic signs and symptoms, systemic signs and symptoms (malaise, fever, etc.), and elevated blood markers (CRP and ESR)., Results: A total of 47 MRI examinations were performed in 11 patients (1 male, mean age 28, range 14-53 years) with a total follow-up time ranging between 12 and 56 months (average 36 months). MRI was positive for active disease at least once in nine out of the 11 patients (82%). The most commonly affected arteries were the aortic arch, the left subclavian artery and the left common carotid artery. No statistically significant correlation was found between clinical activity and MRI signs of activity., Conclusion: Although MRI is a well established modality for primary diagnosis of TA, the present study suggests that it has a limited clinical role in the long-term follow-up of those patients when reactivation of disease is suspected., (Copyright © 2011 Elsevier B.V. All rights reserved.)

Object: Gastrointestinal involvement in adult dermatomyositis (DM) and polymyositis (PM) is usually mild, resulting from myoenteric dismotility. Severe inflammation of the alimentary tract in cases of adult DM and PM is rare. The purpose of this study was to examine the prevalence and clinical characteristics of inflammatory gastrointestinal involvement in patients with DM., Methods: The charts of all cases with polymyositis or dermatomyositis, registered in our rheumatology clinic between 1984 and 2004, were reviewed retrospectively for documentation of severe gastrointestinal involvement. The clinical course and the histopathologic findings in all the patients were noted, and the prevalence of this disorder was computed., Results: Among 48 patients with DM or PM, 3 patients with DM and severe gastrointestinal tract manifestations were identified (6% of the study population). Edematous hyperemic bowel wall, with multiple erosions and ulcerous lesions were the prominent endoscopic findings, whereas diffuse mucosal inflammation and multiple vascular ectasias without vasculitis dominated the histologic picture. The resulting clinical course was notable for recurrent abdominal pain and bloody diarrhea, ending catastrophically in two patients with fatal gastrointestinal perforations, despite aggressive immunosuppressive therapy., Conclusions: Severe inflammatory gastrointestinal tract disease should be recognized as a grave, albeit rare, manifestation of adult DM that portends a poor prognosis and carries a high rate of fatal complications. The role of vasculopathy in the pathogenesis of this syndrome remains to be determined.