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Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are inflammatory diseases that typically affect white individuals >50 years. Women are affected ∼2-3 times more often than men. PMR and GCA occur together more frequently than expected by chance. The main symptoms of PMR are pain and stiffness in the shoulders, and often in the neck and pelvic girdle. Imaging studies reveal inflammation of joints and bursae of the affected areas. GCA is a large-vessel and medium-vessel arteritis predominantly involving the branches of the aortic arch. The typical clinical manifestations of GCA are new headache, jaw claudication and visual loss. PMR and GCA usually remit within 6 months to 2 years from disease onset. Some patients, however, have a relapsing course and might require long-standing treatment. Diagnosis of PMR and GCA is based on clinical features and elevated levels of inflammatory markers. Temporal artery biopsy remains the gold standard to support the diagnosis of GCA; imaging studies are useful to delineate large-vessel involvement in GCA. Glucocorticoids remain the cornerstone of treatment of both PMR and GCA, but patients with GCA require higher doses. Synthetic immunosuppressive drugs also have a role in disease management, whereas the role of biologic agents is currently unclear. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and spinal cord. This study was undertaken to evaluate the clinical features and outcomes among patients with PCNSV who presented with prominent gadolinium meningeal enhancement on magnetic resonance imaging (MRI). METHODS: Through retrospective review using the Mayo Clinic medical records linkage system, we identified 101 consecutive patients with PCNSV based on brain biopsy or conventional angiography (or both) between January 1, 1983, and December 31, 2003. We evaluated data on demographics, clinical findings, laboratory studies, imaging, biopsy of brain or spinal cord (or both), treatment, and neurologic outcome. RESULTS: MRIs showed prominent leptomeningeal enhancement in 8 of 101 patients with PCNSV. In 6 of those 8, cerebral angiography or magnetic resonance angiography results were normal, but biopsy of the brain or spinal cord showed vasculitis in all 8. Granulomatous vascular inflammation was found in 6 specimens and was associated in 4 cases with vascular deposits of beta-amyloid peptide. All 8 patients had a prompt response to therapy, with resolution of the MRI meningeal enhancement. Although 3 of the 8 patients had relapses during followup, the overall outcome was favorable. Patients with meningeal enhancement, compared with patients without enhancement, more commonly had substantial abnormalities of cerebrospinal fluid (100% versus 58%; P = 0.02) and amyloid angiopathy (50% versus 12%; P = 0.03). CONCLUSION: Prominent gadolinium leptomeningeal enhancement on MRI may point to a distinct subtype of PCNSV with small leptomeningeal artery vasculitis and rapid response to therapy. [ABSTRACT FROM AUTHOR]

Background: A reliable alternative to steroids for treating polymyalgia rheumatica has not yet been identified. Although infliximab has been used occasionally in steroid-resistant cases, its efficacy has not been demonstrated in a controlled study.Objective: To compare the efficacy of prednisone plus infliximab with that of prednisone plus placebo in patients with newly diagnosed polymyalgia rheumatica.Design: Randomized, placebo-controlled trial.Setting: 7 rheumatology clinics in Italy.Patients: 51 patients with newly diagnosed polymyalgia rheumatica. Patients with associated giant cell arteritis and those who had been previously treated with steroids or biological or immunosuppressive agents were excluded.Intervention: Initial therapy with oral prednisone tapered from 15 mg/d to 0 mg/d over 16 weeks according to a standard protocol, plus infusions of placebo or infliximab, 3 mg/kg of body weight, at weeks 0, 2, 6, 14, and 22.Measurements: The primary efficacy end point was the proportion of patients without relapse or recurrence through week 52. Secondary outcomes were the proportion of patients no longer taking prednisone, the number of relapses and recurrences, the duration of prednisone therapy, and the cumulative prednisone dose.Results: Four patients (3 in the infliximab group and 1 in the placebo group) did not complete the trial. The proportion of patients who were free of relapse and recurrence at 52 weeks did not differ between groups (6 of 20 patients [30%] in the infliximab group vs. 10 of 27 patients [37%] in the placebo group; adjusted risk difference, -3 percentage points [95% CI, -31 to 24 percentage points]; P = 0.80). In a sensitivity analysis that included dropouts, the best-case scenario yielded a difference of 5 percentage points (CI, -21 to 31 percentage points) between the groups. The secondary outcomes at weeks 22 and 52 did not differ between the groups.Limitations: The study had a small sample and a short follow-up. A low dosage of infliximab was used, and the prednisone dosage was rapidly tapered.Conclusions: Although too small to be definitive, the trial provides evidence that adding infliximab to prednisone for treating newly diagnosed polymyalgia rheumatica is of no benefit and may be harmful. If there is benefit, it is unlikely to be large. Australian Clinical Trials Registry number: ACTRN012606000205538. [ABSTRACT FROM AUTHOR]

Background: Evidence of a dark halo on ultrasonography has been considered a specific sign of giant-cell arteritis and may replace temporal artery biopsy for the diagnosis of giant-cell arteritis in patients with typical clinical manifestations.Objective: To assess the usefulness of temporal artery duplex ultrasonography and to compare this mode of ultrasonography with physical examination of temporal arteries for the diagnosis of giant-cell arteritis in patients with suspected giant-cell arteritis or polymyalgia rheumatica.Design: Diagnostic test study.Setting: Several divisions of Reggio Emilia Hospital, Reggio Emilia, Italy.Patients: 86 consecutive patients with a suspected diagnosis of giant-cell arteritis or polymyalgia rheumatica identified over a 22-month period.Measurements: The temporal arteries were examined in all 86 patients. Duplex ultrasonography of the temporal arteries was then performed by two ultrasonographers who were unaware of the clinical diagnosis. Before corticosteroid therapy was started, temporal artery biopsies were performed in all patients at the site targeted by the ultrasonographer.Results: A hypoechoic halo around the lumen of the temporal arteries had a sensitivity of only 40% (95% CI, 16% to 68%) and a specificity of 79% (CI, 68% to 88%) for the diagnosis of biopsy-proven giant-cell arteritis. The negative likelihood ratio was 0.8 (CI, 0.5 to 1.2), and the positive likelihood ratio was 1.9 (CI, 0.9 to 4.1). When the thickness of the halo was at least 1 mm, specificity increased to 93% (CI, 84% to 98%) and the positive likelihood ratio increased to 5.7 (CI, 2.0 to 16.2); however, sensitivity remained low at 40% (CI, 16% to 68%). On physical examination, temporal artery abnormalities had a higher sensitivity of 67% (CI, 38% to 88%), a higher specificity of 99% (CI, 92% to 100%), and a higher positive likelihood ratio of 47.3 (CI, 6.5 to 342.4) than did ultrasonographic findings. None of the patients with giant-cell arteritis had a normal temporal artery inspection and a hypoechoic halo on ultrasonography.Conclusion: Evidence on ultrasonography of a halo around temporal arteries, either any halo or a halo 1 mm or greater in thickness, only modestly increased the probability of biopsy-proven giant-cell arteritis but did not improve the diagnostic accuracy of a careful physical examination. [ABSTRACT FROM AUTHOR]

Background: Musculoskeletal disorders are the most common extra-intestinal manifestation of inflammatory bowel disease (IBD). Wide ranges of prevalence have been reported depending on the criteria used to define spondylarthropathy and on the selection of patients. We aimed to evaluate the prevalence and clinical spectrum of musculoskeletal manifestations in an inception cohort of European IBD patients. Methods: From 1 October 1991 to 30 September 1993, 202 IBD patients were diagnosed in three centres of two countries (Italy and The Netherlands) by means of a population-based inception cohort study. Of this group of patients, 160 (79%) were interviewed and examined by a rheumatologist and a gastroenterologist in the period June-September 1996. A total of 139/160 patients had an anteroposterior radiograph of the pelvis, and in 140/160 HLA-B27 was determined. Results: 53 (33.1%) of the 160 patients had experienced at least one musculoskeletal manifestation, 29 (18.1%) satisfied the European Spondylarthropathy Study Group (ESSG) criteria for spondylarthropathy and 5 (3.1%) satisfied the modified New York criteria for ankylosing spondylitis. However, 23 (14.4%) patients developed one or more spondylarthropathy-related manifestations without fulfilling any of the classification criteria. In patients satisfying ESSG criteria a significantly higher frequency of women (P = 0.03), of ocular and liver involvement (P = 0.01 and P = 0.03, respectively), and use of immunosuppressive drugs (P = 0.02) was observed. Conclusion: Our study shows a high prevalence of musculoskeletal manifestations in an inception cohort of IBD patients. The clinical spectrum is broader than that defined by spondylarthropathy criteria. [ABSTRACT FROM AUTHOR]

There are no standardised diagnostic criteria for polymyalgia rheumatica. The combination of persistent pain (at least 1 month) with marked morning stiffness in at least 2 of the neck, shoulder or pelvic girdle is characteristic of polymyalgia rheumatica. The other criteria are age >50 years, erythrocyte sedimentation rate (ESR) >40 mm/hour, rapid response to corticosteroids and an absence of other diseases capable of causing the musculoskeletal symptoms. A normal ESR does not exclude a diagnosis of polymyalgia rheumatica. Diagnostic temporal artery biopsy is recommended in all patients suspected of having giant cell arteritis. The segment of temporal artery with abnormality on physical examination should be biopsied. The drugs of choice in the treatment of polymyalgia rheumatica/giant cell arteritis are corticosteroids. An initial prednisone dosage of 40 to 60 mg/day is adequate in almost all cases of giant cell arteritis. Higher dosages and/or intravenous pulse methylprednisolone can be tried on patients with partial response or with recent visual loss. Polymyalgia rheumatica in the absence of giant cell arteritis requires an initial dose of prednisone 10 to 20 mg/day. In some cases of mild polymyalgia rheumatica, a short course of nonsteroidal anti-inflammatory drugs may be tried. Long term corticosteroid therapy in polymyalgia rheumatica and giant cell arteritis is complicated by serious adverse effects in between 48 and 65% of patients. Vertebral fractures and infections are among the most dangerous and frequent complications. Although there are limited data on the use of cytotoxic or immunosuppressive drugs, such as methotrexate, azathioprine and cyclosporin, in these indications, they might be effective either in sparing corticosteroids or in treating patients who do not respond to treatment with corticosteroids. [ABSTRACT FROM AUTHOR]

To evaluate the efficacy of pegylated interferon (PEG-IFN) alpha-2a to treat post-uveitic relapsing macular edema (ME) after withdrawal of non-PEG IFN alpha-2a or 2b to maintain treatment efficacy. This retrospective study investigated subjects with post-uveitic ME who received weekly subcutaneous PEG-IFN alpha-2a injections. Comparisons between baseline central macular thickness (CMT) and best-corrected visual acuity (BCVA) and those at all follow-up visits were made. Six patients (nine eyes) were treated and followed up for six months. CMT (mean [standard deviation]) decreased from 375[117] to 283[39] μm after one month (p < 0.001), remaining significantly lower up to the final follow-up visit at six months (275[38] μm, p = 0.008), and BCVA (0.21[0.16] logMAR at baseline) showed an improvement of 0.12[0.11] logMAR (p = 0.026) at six months. Neither recurrences nor any serious adverse events were recorded. Post-uveitic ME patients were effectively and safely treated with PEG-IFN alpha-2a. [ABSTRACT FROM AUTHOR]

Objective and design: A cross-sectional single-center study was conducted to assess cytokine levels in aqueous humor (AH) and plasma of three different uveitis entities: definite ocular sarcoidosis (OS), definite OS associated with QuantiFERON®-TB Gold test positivity (Q + OS) and presumed tubercular uveitis (TBU). Subjects: Thirty-two patients (15 OS, 5 Q + OS, 12 TBU) were included. Methods: Quantification of selected cytokines was performed on blood and AH samples collected before starting any treatment. Statistical analysis was conducted using the Kruskal–Wallis test, the Mann–Whitney or Fisher test and the Principal Component Analysis (PCA). Results: IL-6, IL-8 and IP-10 levels were higher in AH samples than in peripheral blood. In AH samples, BLC, IL-8 and IP-10 were significantly higher in definite OS than in presumptive TBU. There were no statistically significant differences in terms of cytokine levels between Q + OS and presumptive TBU. PCA showed a similar cytokine pattern in the latter two groups (IFNγ, IL-15, IL-2, IP-10, MIG), while the prevalent expression of BLC, IL-10 and MIP-3 α was seen in definite OS. Conclusions: The different AH and plasma cytokine profiles observed in OS compared to Q + OS and TBU may help to differentiate OS from TBU in overlapping clinical phenotypes of granulomatous uveitis (Q + OS). [ABSTRACT FROM AUTHOR]

Despite intensive investigations, about 30% of stroke cases remains of undetermined origin. After exclusion of common causes of stroke, there is a number of rare heritable and non-heritable conditions, which often remain misdiagnosed, that should be additionally considered in the diagnosis of cryptogenic stroke. The identification of these diseases requires a complex work up including detailed clinical evaluation for the detection of systemic symptoms and signs, an adequate neuroimaging assessment and a careful family history collection. The task becomes more complicated by phenotype heterogeneity since stroke could be the primary or unique manifestation of a syndrome or represent just a manifestation (sometimes minor) of a multisystem disorder. The aim of this review paper is to provide clinicians with an update on clinical and neuroradiological features and a set of practical suggestions for the diagnostic work up and management of these uncommon causes of stroke. The identification of these stroke causes is important to avoid inappropriate and expensive diagnostic tests, to establish appropriate management measures, including presymptomatic testing, genetic counseling, and, if available, therapy. Therefore, physicians should become familiar with these diseases to provide future risk assessment and family counseling. [ABSTRACT FROM AUTHOR]

Summary: Vogt–Koyanagi–Harada (VKH) is an autoimmune disease characterized by inflammation in tissues that contain melanocytes. We aimed to increase the knowledge regarding immunological pathways deregulated in VKH disease. We compared the percentages of circulating natural killer (NK), NK T and T cells expressing the activatory markers: CD16, CD69, NK group 2D (NKG2D), natural cytotoxicity triggering receptor 3 (Nkp30), natural cytotoxicity triggering receptor 1 (Nkp46) and the inhibitory marker: NK group 2 member A (NKG2A) in 10 active VKH patients, 20 control subjects (CTR) and seven patients with Behçet disease (BD) by flow cytometry. Cytotoxic potential of NK cells was determined through the degranulation marker CD107a expression after contact with K562 cells by flow cytometry. Moreover, plasmatic levels of 27 cytokines were determined with a multiplex bead‐based assay. VKH patients showed higher percentages of NKG2Dpos NK and NK T cells versus CTR. The cytotoxic potential of NK cells induced by K562 cells was comparable between VKH patients and CTR. Finally, higher concentrations of interleukin (IL)‐4, IL‐5, IL‐7, IL‐17 and platelet‐derived growth factor‐subunits B (PDGF‐bb) were detected in plasma of VKH patients versus CTR. The immune profile of VKH patients was similar to that of BD patients. [ABSTRACT FROM AUTHOR]

La relation entre les douleurs rachidiennes, caractéristiques de la spondylarthrite ankylosante (SA), les résultats rapportés par les patients et l'activité de la maladie est peu décrite. L'objectif de cette analyse était d'étudier le lien entre une amélioration significative des rachialgies et la qualité de vie, l'activité de la maladie, la capacité fonctionnelle, la fatigue, le sommeil et la productivité au travail chez des patients ayant une SA traités par upadacitinib (UPA) ou placebo (PBO) dans l'étude de phase 2/3 SELECT-AXIS 1 [1]. Des patients avec SA active et réponse inadéquate aux AINS ont reçu UPA 15 mg 1×/j ou un PBO pendant 14 semaines (S) (extension en ouvert jusqu'à S104). L'amélioration cliniquement significative des douleurs rachidiennes évaluées par le patient sur une échelle numérique (0–10) était définie par un score < 4 et une variation par rapport à l'inclusion ≥ 2, à chaque visite et à S52. Ont été évaluées, la proportion de patients ayant une amélioration significative des douleurs rachidiennes à S4 et maintenue à S14, ainsi que la proportion de patients (groupes UPA et PBO combinés) ayant des réponses ≥ à la MCID à S14 pour les questionnaires patients (PRO : BASFI, BASDAI, ASAS HI, ASQoL, FACIT-F, ISI, WPAI) et répondeurs pour les scores d'activité de la maladie (ASAS40, ASAS-PR, ASDAS-LDA, ASDAS-ID, ASDAS-MI, ASDAS-CII, BASDAI50), en fonction de l'amélioration des rachialgies. Les analyses ont été réalisées avec le test de Cochran–Mantel–Haenszel ajusté sur le taux de hsCRP avec imputation des non-répondeurs pour la comparaison des traitements, avec le test du Chi2 pour les données observées pour la comparaison des répondeurs et non-répondeurs. Les valeurs de p < 0,05 étaient nominales. La proportion de patients ayant une amélioration significative des douleurs rachidiennes était significativement plus importante dans le groupe UPA par rapport au groupe PBO (p < 0,001) à partir de S2, avec une poursuite de l'amélioration entre S14 et S52 dans le groupe UPA (Fig. 1). Parmi les patients ayant une amélioration des douleurs rachidiennes à S4, la réponse était maintenue à S14 chez 92,9 % des patients du groupe UPA 15 mg, versus 54,5 % du groupe PBO (p < 0,05). Une proportion significativement plus importante de patients ayant une amélioration significative des douleurs rachidiennes à S14 ont rapporté une variation ≥ MCID pour l'ensemble des PRO et de meilleures réponses pour tous les critères d'évaluation de l'activité de la maladie, (p ≤ 0,0001, Fig. 2 et 3). Une proportion significativement supérieure de patients atteints de SA a obtenu une amélioration importante des douleurs rachidiennes à partir de S2 dans le groupe UPA par rapport au placebo, poursuivie jusqu'à S52 sous UPA. Une amélioration significative des douleurs rachidiennes était associée à une amélioration importante des PRO et de meilleures réponses en termes d'activité de la SA. [ABSTRACT FROM AUTHOR]

Purpose: To assess the efficacy of interferon (IFN) alpha-2a in the treatment of post-uveitic refractory macular edema (ME).Methods: Retrospective cohort of patients with post-uveitic refractory ME, who received subcutaneous IFN alpha-2a injections for at least 3 months. Baseline central macular thickness (CMT) and best-corrected visual acuity (BCVA) were compared with those at follow-up visits up to 12 months.Results: Thirty-seven patients were included. Treatment duration (median [interquartile range]) was 14[8-24] months with a follow-up of 17[10-38] months. CMT (mean [standard deviation]) decreased from 438[140] to 335[119] μm after 1 month (p < 0.0001) and remained significantly lower up to 12 months (286[98] μm, p = 0.001). BCVA (0.48[0.33] logMAR at baseline) improved by 0.26[0.33] logMAR (p = 0.001) at 12 months. There were 14 recurrences. Seven patients had treatment side effects, without serious adverse events.Conclusions: IFN alpha-2a was effective, safe, and well tolerated in treating post-uveitic refractory ME. [ABSTRACT FROM AUTHOR]

Summary: Collaboration between gastroenterologists and rheumatologists is recommended for the correct management of patients with associated spondyloarthritis (SpA) and inflammatory bowel disease (IBD). We aimed to establish the appropriateness of several red flags for a prompt specialist referral. A systematic review of the literature was performed using the GRADE method to describe the prevalence of co‐existing IBD‐SpA and the diagnostic accuracy of red flags proposed by a steering committee. Then, a consensus among expert gastroenterologists and rheumatologists (10 in the steering committee and 13 in the expert panel) was obtained using the RAND method to confirm the appropriateness of each red flag as 'major' (one sufficient for patient referral) or 'minor' (at least three needed for patient referral) criteria for specialist referral. The review of the literature confirmed the high prevalence of co‐existing IBD‐SpA. Positive and negative predictive values of red flags were not calculated, given the lack of available data. A consensus among gastroenterology and rheumatology specialists was used to confirm the appropriateness of each red flag. Major criteria to refer patients with SpA to the gastroenterologist included: rectal bleeding, chronic abdominal pain, perianal fistula or abscess, chronic diarrhoea and nocturnal symptoms. Major criteria to refer patients with IBD to the rheumatologist included: chronic low back pain, dactylitis, enthesitis and pain/swelling of peripheral joints. Several major and minor red flags have been identified for the diagnosis of co‐existing IBD‐SpA. The use of red flags in routine clinical practice may avoid diagnostic delay and reduce clinic overload. 1. A systematic review of the literature was performed to investigate the prevalence of co‐existing IBD‐SpA and the diagnostic accuracy of red flags for specialist referral. 2. A consensus among gastroenterology and rheumatology specialists was used to confirm the appropriateness of each red flag for suspected diagnosis of co‐existing IBD‐SpA and specialist referral. 3. The use of specific red flags as major or minor criteria for specialist referral may avoid diagnostic delay in case of associated IBD‐SpA and reduce clinic overload. [ABSTRACT FROM AUTHOR]

Évaluer l'efficacité du tocilizumab (TCZ) chez les patients atteints de maladie de Behçet réfractaire. Étude multicentrique sur 30 patients remplissant les critères internationaux de la maladie de Behçet et traités par TCZ dans différents centres de référence européens. La réponse clinique a été évaluée à 6 mois (M6) de l'initiation du TCZ. Quatre-vingt-dix pour cent des patients étaient réfractaires ou intolérants aux anti-TNF-α. Dans l'ensemble, le TCZ a été efficace chez 25 (83 %) patients avec des réponses complètes et partielles respectivement chez 18 (60 %) et 7 (23 %) patients. Le taux de réponse complète était de 67, 60, et 42 % respectivement chez les patients présentant des uvéites (18/30), manifestations neurologiques (5/30), et cutanéomuqueuses et/ou articulaires (7/30). Le TCZ a eu un effet d'épargne cortisonique significatif permettant de diminuer la dose quotidienne médiane de prednisone de 20 mg/jour [10 ; 40] à 9 mg/jour [5 ; 13] à 6 mois (p = 0,0006). Le nombre de patients nécessitant un traitement concomitant par disease-modifying antirheumatic drugs (DMARDs) est passé de 7 (23 %) à 4 (13 %) à 6 mois. Des effets indésirables légers à modérés ont été observés chez 6 (20 %) patients et 3 (10 %) ont présenté des événements indésirables graves [pneumonie, perforation intestinale et bactériémie] nécessitant l'arrêt du traitement dans 2 cas. Le TCZ semble une alternative efficace aux anti-TNF-α dans les uvéites et les manifestations neurologiques de la maladie de Behçet. [ABSTRACT FROM AUTHOR]

Abstract: Background: Psoriatic arthritis (PsA) is a chronic inflammatory disease associated with psoriasis (PsO). Early diagnosis and prompt therapeutic intervention are crucial for limiting PsA progression and prevention of disability. Dermatologists are in a privileged position to detect early PsA. The management of patients with PsA in the dermatology setting is widely variable. Objective: To provide practical recommendations for the management of patients with PsA in the dermatology setting including early diagnosis and treatment. Methods: A consensus document was written by an expert panel composed by dermatologists (n = 12) and rheumatologists (n = 6). Eleven highly relevant questions were selected and elaborated with answers/statements based on a narrative literature review. The resulting document was discussed in a face‐to‐face meeting adopting a nominal group technique to reach consensus (i.e. 100% agreement) using the Delphi method. Results: A consensus was achieved in defining the following: the clinical characteristics differentiating inflammatory and non‐inflammatory signs and symptoms of joint disease; the most important differential diagnoses of PsA in clinical practice; the most useful screening questionnaires, serum laboratory tests and imaging techniques for the detection of early PsA; the criteria for dermatologist to refer patients with PsO to rheumatologist; the criteria for the diagnosis of PsA; the selection of the indices that the dermatologist could use for measuring the activity and severity of PsA in clinical practice; when systemic steroids and/or intra‐articular steroid injections are indicated in the treatment of PsA. Finally, systemic treatments including synthetic and biologic disease‐modifying antirheumatic drugs to be considered for the treatment of PsA have been reported. Conclusions: The implementations of these practical recommendations could be very helpful for the management of patients with PsA in the dermatology setting including early diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Background: Giant-cell arteritis commonly relapses when glucocorticoids are tapered, and the prolonged use of glucocorticoids is associated with side effects. The effect of the interleukin-6 receptor alpha inhibitor tocilizumab on the rates of relapse during glucocorticoid tapering was studied in patients with giant-cell arteritis.Methods: In this 1-year trial, we randomly assigned 251 patients, in a 2:1:1:1 ratio, to receive subcutaneous tocilizumab (at a dose of 162 mg) weekly or every other week, combined with a 26-week prednisone taper, or placebo combined with a prednisone taper over a period of either 26 weeks or 52 weeks. The primary outcome was the rate of sustained glucocorticoid-free remission at week 52 in each tocilizumab group as compared with the rate in the placebo group that underwent the 26-week prednisone taper. The key secondary outcome was the rate of remission in each tocilizumab group as compared with the placebo group that underwent the 52-week prednisone taper. Dosing of prednisone and safety were also assessed.Results: Sustained remission at week 52 occurred in 56% of the patients treated with tocilizumab weekly and in 53% of those treated with tocilizumab every other week, as compared with 14% of those in the placebo group that underwent the 26-week prednisone taper and 18% of those in the placebo group that underwent the 52-week prednisone taper (P<0.001 for the comparisons of either active treatment with placebo). The cumulative median prednisone dose over the 52-week period was 1862 mg in each tocilizumab group, as compared with 3296 mg in the placebo group that underwent the 26-week taper (P<0.001 for both comparisons) and 3818 mg in the placebo group that underwent the 52-week taper (P<0.001 for both comparisons). Serious adverse events occurred in 15% of the patients in the group that received tocilizumab weekly, 14% of those in the group that received tocilizumab every other week, 22% of those in the placebo group that underwent the 26-week taper, and 25% of those in the placebo group that underwent the 52-week taper. Anterior ischemic optic neuropathy developed in one patient in the group that received tocilizumab every other week.Conclusions: Tocilizumab, received weekly or every other week, combined with a 26-week prednisone taper was superior to either 26-week or 52-week prednisone tapering plus placebo with regard to sustained glucocorticoid-free remission in patients with giant-cell arteritis. Longer follow-up is necessary to determine the durability of remission and safety of tocilizumab. (Funded by F. Hoffmann-La Roche; ClinicalTrials.gov number, NCT01791153 .). [ABSTRACT FROM AUTHOR]

Dans cette étude rétrospective multicentrique, nous avons compare l'efficacité et la tolérance de tocilizumab IV versus SC chez 109 patients avec une artérite de Takayasu (TAK). Étude rétrospective multicentrique dans les centres en France, Italie, Espagne, Arménie, Israël, Japon, Tunisie, Russie de patients avec une maladie de Takayasu ayant reçu au moins 3 cures de tocilizumab entre janvier 2017 à septembre 2019. Au total, 109 patients ont été inclus, dont 91 avec sous tocilizumab IV et 18 avec une forme SC. Une réponse complète (NIH < 2 avec moins de 7,5 mg/jour de prednisone) à 6 mois a été mise en évidence chez 69 % des patients TAK, dont 57 (70 %) et 11 (69 %) sous tocilizumab IV et SC, respectivement (p = 0,95). Les facteurs associés à la réponse complète au tocilizumab à 6 mois étaient l'âge < 30 ans (HR = 2,85, IC95 % : 1,14–7,12 ; p = 0,027) et le delais entre le diagnostic de TAK et l'initiation du tocilizumab (HR = 1,18, IC95 % : 1,02–1,36 ; p = 0,034). Au cours du suivi médian de 30,1 mois [0,4 ; 105,8] et 10,8 [0,1 ; 46,4] (p < 0,0001) chez les patients ayant reçu du tocilizumab sous forme IV et SC, respectivement, le risque de rechute était significativement plus élevé chez les patients sous tocilizumab SC (HR = 2,55, IC95 % : 1,08–6,02 ; p = 0,033). L'incidence cumulée de rechute à 12 mois chez les patients TAK était de 13,7 % (IC95 % : 7,6–21,5), avec 10,3 % (IC95 % : 4,8–18,4) pour ceux sous tocilizumab IV contre 30,9 % (IC95 % : 10,5–54,2) chez les patients sous tocilizumab SC (p < 0,05). Des événements indésirables sont survenus chez 14 (15 %) patients sous la forme IV et chez 2 (11 %) sous tocilizumab SC. Dans cette étude, on montre une efficacité similaire de la forme SC et IV, avec un risque de rechute plus important en cas de forme SC, et une réponse globale de 70 % chez des TAK DMARDs-refractaires. [ABSTRACT FROM AUTHOR]

The mainstay of therapy of large vessel vasculitides (LVV) remains glucocorticoids (GC). Although most patients initially achieve disease remission, relapses and GC dependence are seen in more than two-thirds of cases. Conventional synthetic disease-modifying antirheumatic drugs (DMARDs) showed little or no steroid sparing effects, while biological agents represent a valid therapeutic option in patients with severe and/or relapsing LVV. [ABSTRACT FROM AUTHOR]

Objective To report the efficacy and safety of anti-TNF agents in patients with severe and/or refractory manifestations of Behçet's disease (BD). Methods We performed a multicenter study of main characteristics and outcomes of anti-TNF alpha treatments [mainly infliximab (62%), and adalimumab (30%)] in 124 BD patients [48% of men; median age of 33.5 (28–40) years]. Results Overall response (i.e. complete and partial) rate was 90.4%. Clinical responses were observed in 96.3%, 88%, 70%, 77.8%, 92.3% and 66.7% of patients with severe and/or refractory ocular, mucocutaneous, joint, gastro-intestinal manifestations, central nervous system manifestations and cardiovascular manifestations, respectively. No significant difference was found with respect to the efficacy of anti-TNF used as monotherapy or in association with an immunosuppressive agent. The incidence of BD flares/patient/year was significantly lower during anti-TNF treatment (0.2 ± 0.5 vs 1.7 ± 2.4 before the use of anti-TNF, p < 0.0001). The prednisone dose was significantly reduced at 6 and 12 months (p < 0.0001). In multivariate analysis, retinal vasculitis was negatively associated with complete response to anti-TNF (OR = 0.33 [0.12–0.89]; p = 0.03). The efficacy and relapse free survival were similar regardless of the type of anti-TNF agent used. After a median follow-up of 21 [7–36] months, side effects were reported in 28% of patients, including infections (16.3%) and hypersensitivity reactions (4.1%). Serious adverse events were reported in 13% of cases. Conclusion Anti-TNF alpha therapy is efficient in all severe and refractory BD manifestations. Efficacy appears to be similar regardless of the anti-TNF agent used (infliximab or adalimumab). [ABSTRACT FROM AUTHOR]

Inflammatory myopathies (IM) are a group of muscle diseases occurring both in children and adults. Nailfold videocapillaroscopy (NVC) alterations are described in IM, but available data are discordant, including differences between polymyositis (PM) and dermatomyositis (DM). The aim of this study was to describe the capillaroscopic differences between PM and DM patients and possible correlation with clinical and serological features. We analyzed 52 unselected patients with IM in a cross-sectional study in a 6-month period. NVC findings of 29 DM and 23 PM patients were compared with those of 52 patients with primary Raynaud's phenomenon. Tortuosities, capillary loss, enlarged and giant capillaries, microhemorrhages, and ramified capillaries were scored by a semiquantitative rating; disorganization of the vascular array, avascular areas, and scleroderma pattern were scored as presence/absence. Sex, mean age, and mean disease duration were similar in both groups. Disorganization of the vascular array, enlarged and giant capillaries, capillary loss, and scleroderma-like pattern were observed almost only in IM patients. Significant differences were observed between PM and DM with higher frequency and mean score of NVC changes in DM. In DM patients with disease duration ≤6 months (14/29 patients), capillary density was significantly reduced ( P = 0.039) and giant capillaries more frequent ( P = 0.027), compared with patients with longer disease duration, while a scleroderma pattern tended to be more frequent in patients with a disease duration of less than 6 months. On the contrary, no differences were observed for ramified capillaries with regard to disease duration. Capillaroscopic alterations are identified only in DM patients as expression of diffuse microangiopathy; surprisingly, more severe changes were associated with shorter disease duration, while persistence of ramified capillaries with long-standing disease. [ABSTRACT FROM AUTHOR]

The original version of this article unfortunately contained a mistake. Figure 3 caption is incorrect and co-author name should be E.Tournier-Lasserve. [ABSTRACT FROM AUTHOR]

L'efficacité et la tolérance d'UPA ont été démontrées chez des patients (pts) atteints de rhumatisme psoriasique (RP) actif dans les essais cliniques de phase 3 SELECT-PsA1 et SELECT-PsA2 [1,2]. Nous présentons ici les données regroupées de tolérance de la période de 24S contrôlée vs PBO du programme clinique. Le programme SELECT-PsA a inclus des pts ayant eu une réponse inadéquate (IR) ou une intolérance à ≥ 1 non-bDMARD et ayant eu une IR ou une intolérance à ≥ 1 bDMARD. Les deux essais ont évalué un PBO, UPA 15 mg et UPA 30 mg 1 ×/j ; SELECT-PsA1 a également inclus l'ADA comme comparateur actif [1,2]. Les données ont été regroupées pour les pts des bras PBO, UPA15 ou UPA30. Les pts pouvaient être traités en monothérapie ou ≤ 2 non-bDMARD concomitants. Les événements indésirables apparus sous traitement (EI) dans les essais ont été analysés et résumés. Les EI rapportés jusqu'à la S24 sont présentés. Au total, 345 pts ont reçu ≥ 1 dose de traitement (PBO : 635, UPA15 : 640, UPA30 : 641, ADA : 429). Les taux d'EI, EI graves et EI ayant entraîné l'arrêt du traitement ont été similaires avec le PBO et UPA15 et plus élevés avec UPA30. Les taux d'infections graves (IG) et d'herpes zoster (HZ) ont été similaires avec le PBO et UPA15 et plus élevés avec UPA30. L'IG la plus fréquente a été la pneumonie. La plupart des cas d'HZ ont inclus un seul dermatome ; aucune atteinte du système nerveux central n'a été rapportée. Les infections opportunistes ont inclus 1 candidose urétrale (UPA15) et 1 cas de chacune des infections suivantes, pneumonie à Pneumocystis jirovecii , cytomégalovirus, candidose trachéale et candidose oropharyngée, avec UPA30. Les affections malignes autres que les cancers cutanés non-mélanomes (CCNM) ont été rapportées à des taux similaires avec UPA15 et UPA30 ; aucun cas n'a été rapporté avec le PBO. Le taux de CCNM a été similaire avec le PBO, UPA15 et UPA30. Le nombre d'EI cardiovasculaires majeurs (MACE) et de thromboembolies veineuses (TEV) confirmés par le comité d'adjudication a été < 0,5 % dans tous les bras, des taux similaires étant rapportés avec le PBO, UPA15 et UPA30 ; tous les pts avaient au moins un facteur de risque. Les TEV ont inclus 1 thrombose veineuse profonde (PBO) et 1 embolie pulmonaire dans chacun des deux bras UPA. Il n'y a eu aucun cas de perforation gastro-intestinale confirmé par le comité d'adjudication. Les troubles hépatiques ont été majoritairement des augmentations transitoires des transaminases. Des élévations des CPK ont été rapportées plus fréquemment dans les bras UPA vs PBO et ont été majoritairement asymptomatiques, n'ayant entraîné que rarement l'arrêt du traitement ; aucun cas de rhabdomyolyse n'a été rapporté. Les cas d'anémie, de neutropénie et de lymphopénie ont été généralement légers ou modérés, non graves et n'ont que rarement entraîné l'arrêt du traitement. Le profil de tolérance a généralement été cohérent entre UPA15 et ADA, à l'exception de taux plus faibles d'HZ, d'anémie et de lymphopénie et de taux plus élevés de troubles hépatiques et de neutropénie dans le bras ADA. Cependant, les taux de la plupart des EI étaient plus élevés avec UPA30 vs ADA, à l'exception des troubles hépatiques. Les profils de tolérance d'UPA15 et d'ADA ont généralement été cohérents, les taux de la plupart des EI étant plus élevés avec UPA30 par rapport à ADA. Jusqu'à S24, le profil de tolérance d'UPA15 et d'UPA30 chez les pts ayant un RP était cohérent avec celui observé avec UPA chez les pts ayant une polyarthrite rhumatoïde [3]. [ABSTRACT FROM AUTHOR]

The endovascular treatment of spontaneous dural cavernous sinus fistula (DAVF) can be accomplished by arterial approach, just with symptoms relief, or by numerous venous approaches through the inferior petrosal sinus, ophthalmic vein, anterior or posterior intercavernous sinus and facial vein. Our case suggests the approach to the cavernous sinus via the foramen ovale and emissary veins puncture as an alternative when there is no possibility of venous approach conventionally described. A 76-year-old woman presented with right conjunctival hyperemia, exophthalmos, intraocular pressure increasing and visual deficits in a period of six months. Angiographic diagnosis of spontaneous DAVF isolated from the cavernous sinus, Barrow Type C, with exclusive venous drainage through the superior ophthalmic vein. Endovascular treatment was performed under general anesthesia. Attempts to approach the cavernous sinus through the inferior petrosal sinus ipsilateral and contralateral intercavernous, facial vein and pterygoid plexus, as well as by dissection and direct puncture of the superior ophthalmic vein were not possible. An approach to the cavernous sinus was performed by puncturing the foramen ovale, catheterization of the emissary vein of the foramen ovale with occlusion of the fistula with microcoils. There was a symptomatic regression with gradual normalization of intraocular pressure, exophthalmos and conjunctival hyperemia in three months. The approach to the cavernous sinus through the foramen ovale and catheterization of the emissary cranial skull base vein is an exception and should be considered in cases of spontaneous and isolated DAVF not accessible by a conventional approach. [ABSTRACT FROM AUTHOR]

The article focuses on the concepts of psoriatic disease, which was introduced to provide a new perspective on psoriasis and related disorder. It mentions that the introduction of the new concept of psoriatic disease represents an opportunity to better understand the complex associations of psoriasis-related disorder at both the pathogenetic and clinical level. It says that patients with psoriasis also reveal an increased occurrence of hyperlipidaemia, hypertension, and cardiovascular disease.

Background As psoriatic disease (PD) is a condition characterized by the combination of inflammatory skin (psoriasis) and osteo-articular manifestations (psoriatic arthritis), its treatment should cover both its clinical components. Objective The objective of this study was to propose a flexible framework for the use of biological agents in PD. Methods The proposal was drawn up by a group of dermatologists and rheumatologist expert in PD and was based on existing evidence and personal opinion. Results The three TNF-α inhibitors (adalimumab, etanercept, infliximab) are effective in all of the psoriatic manifestations and should be used in the case of moderate/severe disease refractory to systemic treatment with non-biological drugs. We propose the following definitions of moderate/severe disease: PASI > 10 or BSA > 10 or DLQI > 10 for plaque-psoriasis; BSA ≥ 10 or DLQI ≥ 10 for the other psoriatic skin lesions; DLQI ≥ 10 or meaningful values of the NAPSI or mNAPSI for psoriatic nail involvement; ≥ 1 inflamed joint + patient global VAS34 + physician's judgement or arthritic joint deformities or radiographic joint damage plus ≥ 5 inflamed small joints or ≥ 1 large joints for peripheral joint involvement; ≥ 1 digit with dactylitis and ≥ 1 enthesitic sites + patient global VAS34 + physician's judgement for dactylitis and enthesitis. BASDAI ≥ 4 + physician's judgement for spondylitis; recurrent flares or risk of developing irreversible damage for uveitis. Other assessment instruments can be used if the physician is more familiar with them and if they have been validated. Conclusion We provide a shared dermatological and rheumatological proposal for the use of biological agents in PD. [ABSTRACT FROM AUTHOR]

Objectives. Chronic periaortitis (CP) is a rare disease with a potentially immune-mediated pathogenesis. The study aims to report the frequency and the clinical characteristics of peripheral inflammatory arthritis in a cohort of CP patients, and to review the literature regarding the association between arthritis and CP. [ABSTRACT FROM PUBLISHER]

Objective. To investigate potential associations between the −463 G/A myeloperoxidase (MPO) promoter polymorphism and susceptibility to, and clinical expression of, Behçet's disease (BD). [ABSTRACT FROM PUBLISHER]

Objective. To evaluate the effects of angiotensin II (Ang II) treatment on apoptosis of fibroblast-like synoviocytes (FLS) from patients with osteoarthritis (OA) and rheumatoid arthritis (RA). [ABSTRACT FROM PUBLISHER]

Objectives. To evaluate the long-term efficacy and safety of infliximab in patients with Behçet's disease (BD) and refractory bilateral posterior uveitis, and to assess the proportion of relapse-free subjects through months 12 and 24. [ABSTRACT FROM PUBLISHER]

Background: Tumor necrosis factor-alpha is present in arteries in giant cell arteritis.Objective: To evaluate the efficacy of infliximab, an anti-tumor necrosis factor-alpha agent, in giant cell arteritis.Design: Randomized, controlled trial.Setting: 22 sites in the United States, the United Kingdom, Belgium, Italy, and Spain.Patients: 44 patients with newly diagnosed giant cell arteritis that was in glucocorticosteroid-induced remission.Intervention: Participants were randomly assigned in a 2:1 ratio to receive infliximab (5 mg/kg of body weight) or placebo. Sixteen patients were assigned to glucocorticosteroid plus placebo, and 28 patients to glucocorticosteroid plus infliximab.Measurements: End points were measured through week 22, when an interim analysis resulted in early stopping of the planned 54-week trial. Primary end points were the number of patients who remained free of relapse through week 22 and adverse events. Secondary end points were time to first relapse, biomarkers, cumulative glucocorticosteroid dose, and the number of patients who remained relapse-free while the glucocorticosteroid dosage was tapered to 10 mg/d.Results: Infliximab therapy did not increase the proportion of patients without relapse at week 22 compared with placebo (43% vs. 50%, respectively; difference, -7 percentage points [95% CI, -38 to 23 percentage points; P = 0.65), nor did it increase the proportion of patients whose glucocorticosteroid dosages were tapered to 10 mg/d without relapse (61% vs. 75%, respectively; difference, -14 percentage points [CI, -42 to 14 percentage points]; P = 0.31). The incidence of infection was 71% with infliximab and 56% with placebo (difference, 15 percentage points [CI, -14 to 45 percentage points]).Limitations: The sample was too small to rule out modest effects of infliximab and included only patients with a new diagnosis. Only one dose of infliximab was evaluated, and the study was terminated early.Conclusions: This trial is too small to draw definitive conclusions, but it provides evidence that using infliximab as maintenance therapy in patients in glucocorticoid-induced remission of newly diagnosed giant cell arteritis is of no benefit and may be harmful. If infliximab has benefit, it is unlikely to be great. ClinicalTrials.gov registration number: NCT00076726. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To investigate potential associations between interleukin-10 (IL-10) promoter polymorphisms and susceptibility to, and clinical features of, giant cell arteritis (GCA). METHODS: A total of 140 patients with biopsy-proven GCA who were residents of Reggio Emilia, Italy, and 200 population-based controls from the same geographic area were genotyped for promoter polymorphisms of the IL-10 gene, by molecular methods. The patients were subgrouped according to the presence or absence of polymyalgia rheumatica (PMR) and ischemic complications (any or all of the following: vision loss, jaw claudication, cerebrovascular accidents, or aortic arch syndrome). RESULTS: The distribution of the C/A 592 genotype differed significantly between the GCA patients and the controls (P(corr) = 0.003). Carriers of the A592 allele (A/A or C/A) were significantly more frequent among the GCA patients than among the controls (P(corr) = 0.004, odds ratio [OR] 2.0 [95% confidence interval (95% CI) 1.3-3.1]). Homozygosity for the A592 allele was significantly more frequent among the GCA patients than among the controls (P(corr) = 0.002, OR 3.4 [95% CI 1.6-7.2]). The distribution of the A/G 1082 genotype was similar in GCA patients and controls. In the haplotype analysis, the frequency of the ATA haplotype was significantly higher in GCA patients than in the controls (P = 0.0001), whereas the frequencies of the ACC and GTA haplotypes were significantly lower (P = 0.0001 for both comparisons). No significant associations were found for comparisons of GCA patients with and those without PMR or GCA patients with and those without ischemic complications. CONCLUSION: Our findings show that the -592 C/A promoter polymorphism of the IL-10 gene is associated with susceptibility to GCA. [ABSTRACT FROM AUTHOR]

La granulomatose éosinophilique avec polyangéite (GEPA) (Churg-Strauss) est une vascularite nécrosante des vaisseaux de petit calibre caractérisée par une éosinophilie sanguine et tissulaire associée à un asthme. Les ANCA sont positifs dans seulement un tiers des cas, le plus souvent dirigés contre la myélopéroxydase (MPO). Les patients ANCA+ ont plus de manifestations neurologiques et rénales, et moins de manifestations cardiaques que les patients ANCA−. Les ANCA dirigés contre la protéinase 3 (PR3) sont rares dans la GEPA, et les caractéristiques des patients avec ANCA-PR3 sont inconnues. L'objectif de cette étude était d'étudier le phénotype des patients avec GEPA et ANCA-PR3. Nous avons réalisé une étude multicentrique européenne incluant 845 patients suivis pour une GEPA, répondant aux critères de classification de l'ACR, à la définition de la consensus de Chapel Hill et/ou aux critères de l'essai MIRRA. Les caractéristiques initiales et de suivi ont été recueillies, et comparées selon le statut des ANCA. Le statut des ANCA et leur spécificité étaient connus pour 734 patients : 508 (69,2 %) étaient ANCA−, 209 (28,5 %) avaient des ANCA-MPO et 17 (2,3 %) avaient des ANCA-PR3. Au diagnostic, les patients avec ANCA-PR3, comparativement aux ANCA-MPO et aux ANCA−, avaient respectivement : moins d'asthme (71 % vs 91 % ou 93 %, p = 0,004), notamment moins d'asthme corticodépendant (21 % vs 34 % ou 46 %, p = 0,007), plus de manifestations cutanées (65 % vs 38 % ou 34 %, p = 0,03), moins d'infiltrats pulmonaires (41 % vs. 40 % ou 58 %, p = 0,03) mais plus de nodules pulmonaires (25 % vs 10 % ou 8 %, p = 0,046), moins de neuropathie périphérique (29 % vs 72 % ou 47 %, p < 0,0001), un taux plus faible d'éosinophiles circulants (2015 [IQR 802–5826] vs 5718 [2330–10 444] ou 3224 [1332–7570], p < 0,0001). La durée médiane de suivi était de 74 (37–116) mois pour l'ensemble de la cohorte. Les patients avec ANCA-PR3 présentaient plus de rechute de vascularite que les patients avec ANCA-MPO ou ANCA− (47 % vs 34 % ou 24 %, respectivement ; p = 0,004). La survie sans rechute était plus courte pour les patients avec ANCA-PR3 (hazard ratio [HR] : 7,32, IC95 % : 2,02–26,5 ; p = 0,002) et ANCA-MPO (HR : 1,71, IC95 % : 1,23–2,37 ; p = 0,002), comparativement aux ANCA− Les doses médianes de prednisone étaient plus élevées à 6 et 24 mois de suivi pour les patients avec ANCA-PR3 que pour les patients avec ANCA-MPO ou ANCA− (p = 0,02 et p = 0,007). À 24 mois de suivi, les patients avec ANCA-PR3, comparativement aux ANCA-MPO et aux ANCA−, avaient moins d'asthme chronique (38 % vs 51 % et 60 %, respectivement, p = 0,04) et plus fréquemment de séquelles rénales (20 % vs 10 % vs 1 %, p < 0,0001). Les caractéristiques des patients avec GEPA et ANCA-PR3 diffèrent de celles des patients avec ANCA-MPO ou ANCA−, particulièrement concernant les manifestations asthmatiques et l'éosinophilie qui sont parmi les signes cardinaux de la GEPA. Une éosinophilie circulante légère à modérée pouvant être observée au cours de la granulomatose avec polyangéite (GPA), ces patients pourraient plutôt correspondre à une forme particulière GPA qu'à un nouveau phénotype de GEPA, avec un risque de rechute similaire à celui des GPA. [ABSTRACT FROM AUTHOR]

Behcet's disease (BD) is a multisystem disease of unknown etiology characterized by chronic relapsing orogenital ulcers, uveitis and systemic involvement including articular, gastrointestinal, cardiopulmonary, neurological and vascular pathology. The incidence and nature of cardiac involvement are not clearly elucidated. Cardiovascular manifestations have been reported in 7–46% of patients and mortality occurs in up to 20% of those patients with marked vascular involvement. Sporadic cases of endocarditis, myocarditis, pericarditis, acute myocardial infarction, aortic aneurysm, ventricular thrombosis, congestive cardiomyopathy and valvular dysfunction have been reported. This review discusses the general aspects of the pathogenic mechanisms and clinical features cardiovascular involvement in BD, and provides the data of cardiovascular involvement in a cohort of Italian BD patients. [ABSTRACT FROM AUTHOR]

Background: Steroids are the standard treatment for polymyalgia rheumatica. The efficacy of the candidate drug methotrexate has not yet been demonstrated in controlled studies.Objective: To compare the efficacy and safety of prednisone plus methotrexate and prednisone alone in patients with polymyalgia rheumatica.Design: Multicenter randomized, double-blind, placebo-controlled trial.Setting: 5 Italian rheumatology clinics.Patients: 72 patients with newly diagnosed polymyalgia rheumatica.Measurements: The proportion of patients no longer taking prednisone, the number of flare-ups, and the cumulative prednisone dose after 76 weeks.Intervention: Prednisone dosage (25 mg/d) was tapered to 0 mg/d within 24 weeks and was adjusted if flare-ups occurred. Oral methotrexate (10 mg) or placebo, with folinic acid supplementation (7.5 mg), was given weekly for 48 weeks.Results: Twenty-eight of 32 patients in the methotrexate group and 16 of 30 patients in the placebo group were no longer taking prednisone at 76 weeks (P = 0.003). The risk difference was 34 percentage points (95% CI, 11 to 53 percentage points). Similar results were obtained after adjustment for C-reactive protein level and duration of symptoms in a multivariate model. Fifteen of 32 patients in the methotrexate group and 22 of 30 patients in the placebo group had at least 1 flare-up by the end of follow-up (P = 0.04). The median prednisone dose was 2.1 g in the methotrexate group and 2.97 g in the placebo group (P = 0.03). The rate and severity of adverse events were similar.Limitations: Follow-up was short, and a high dose of folinic acid and a relatively high starting dosage of prednisone were used. Ten of 72 patients (14%) discontinued treatment or were lost to follow-up.Conclusions: Prednisone plus methotrexate is associated with shorter prednisone treatment and steroid sparing. It may be useful in patients at high risk for steroid-related toxicity. [ABSTRACT FROM AUTHOR]

18F-Fluorodeoxyglucose-positron emission tomography (18F-FDG-PET) is a new functional imaging technique available for clinical and experimental use. 18F-FDG-PET studies can be used for screening, localization and follow-up of hypermetabolic processes including malignancies, infections and autoimmune processes. For several years it has been applied in oncological, cardiological and neurological patients, but nowadays an increasing number of studies favours its use in patients with autoimmune diseases including large vessel arteritis. From the experimental view, this technique has even become more important since the introduction of a small PET scanner for the use in animal models. This review focuses on technical aspects, clinical experiences and experimental and future perspectives of 18F-FDG-PET, with a special emphasis on large vessel vasculitis and other autoimmune diseases. [Copyright &y& Elsevier]

Polymyalgia rheumatica (PMR) is a common disorder of the elderly: the pathogenesis of the syndrome is still debated, though active synovitis of the shoulder has recently been confirmed. To investigate the pathogenesis of this synovitis we evaluated cell adhesion molecule (CAM) expression in shoulder synovial tissue from patients with PMR, correlated synovial expression with the serum levels of soluble forms, and assessed the changes associated with corticosteroid treatment. Arthroscopic synovial biopsies were obtained from 12 untreated and seven corticosteroid (CS)-treated cases. CAM expression was evaluated by MoAb staining on frozen sections and computerized image analysis. Soluble CAM were quantified by ELISA. Endothelial cells expressed intercellular adhesion molecule-1 (ICAM-1), E-and P-selectins. Infiltrating cells were ICAM-1 and β1-integrin-positive, while L-selectin expression was limited to intravascular leucocytes. Synovial lining cells strongly expressed vascular cell adhesion molecule-1 (VCAM-1), and less intensely ICAM-1. Only the soluble form of ICAM-1 (sICAM-1) was elevated in untreated patients. CS treatment was associated with a decrease in ICAM-1, VCAM-1 and E-and P-selectin expression. sICAM-1 levels were in the normal range in treated patients. VLA-5 and 6 expression was widely distributed among cell types, and was not CS-sensitive. Active shoulder synovitis is associated with different CAM expression in PMR. ICAM-1 expression is widely distributed and correlates with elevated levels of the soluble form; it is significantly lower in CS-treated asymptomatic cases. [ABSTRACT FROM AUTHOR]

Près de la moitié des patients atteints d'artérites à cellules géantes (ACG) rechutent sous corticothérapie seule. Le taux de rechute semble plus refléter la durée de la corticothérapie que la dose initiale. Cependant, le taux de rechute varie considérablement dans les études observationnelles et les essais contrôlés randomisés. L'objectif était d'identifier les facteurs prédictifs de rechute chez des patients ayant une ACG, inclus dans 3 cohortes internationales distinctes. Cette étude internationale a inclus des patients âgés de plus de 50 ans, remplissant les critères ACR pour le diagnostic d'ACG suivis en France (n = 351), aux États-Unis (n = 285) et en Italie (n = 142). Le critère de jugement principal était le délai entre le diagnostic et la première rechute ou le décès. La survie sans rechute était estimée par la méthode de Kaplan–Meier. Les modèles à risques proportionnels de Cox ont été stratifiés par cohorte. La performance du modèle a été évaluée à la fois par la concordance statistique et par la courbe d'étalonnage. Le modèle final a été présenté avec les hazard ratios (HR) obtenus après ajustement avec leurs intervalles de confiance à 95 % (IC95 %). Nous avons inclus 778 patients (24 % d'homme ; âge médian 71 [EIQ 61–78] ans). Le suivi médian était de 51 [EIQ 24–102] mois. Après 36 mois de suivi, 382 patients ont rechuté, avec une survie sans rechute à 36 mois de 45,3 % (IC95 % : 41,6–49,2), avec des différences marquées entre les cohortes. Les cohortes française et italienne avaient de meilleurs taux de survie sans rechute que la cohorte américaine. Le modèle final corrigé comprenait les céphalées (HR : 1,18, IC95 % : 1,00–1,39 ; p = 0,052), la présence d'une claudication des membres (HR : 1,34, IC95 % : 0,95–1,89 ; p = 0,091), la présence d'une aortite (HR : 1,20, IC95 % : 0,97–1,48 ; p = 0,096) et le taux de CRP (après transformation logarithmique) (HR : 1,11, IC95 % : 1,02–1,22 ; p = 0,014). Le modèle était bien calibré mais avec une capacité de discrimination relativement limitée. Nous avons mis en place un nomogramme permettant d'estimer la survie sans rechute à 3 ans. Des points (allant de 0 à 100) sont attribués à chacune des quatre variables précédentes. Le score total (allant de 0 à 200) est utilisé pour prédire la survie sans rechute à 3 ans dans chaque cohorte. Le risque de rechute de l'ACG est variable selon les centres. Un modèle comprenant les céphalées, la claudication des membres, la présence d'une aortite et l'élévation de la CRP pourrait aider à identifier les patients à haut risque de rechute. Cependant, ce modèle a une faible capacité de discrimination, possiblement due à l'hétérogénéité observées entre les cohortes. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to a study which examined prevalence of monoclonal gammopathy of undetermined significance (MGUS) in patients treated with tumor necrosis factors (TNF)-alpha blockers for psoriasis or psoriatic arthritis (PsA).

The article describes the case of a 68-year-old woman with immunoglobulin G4 (IgG4)-related sacroiliitis. She presented with sicca syndrome, transmissive hearing loss, chronic sinusitis and lacrimal gland enlargement. The results of lacrimal gland biopsy, F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (CT) scanning and immunohistochemical staining are detailed. The case is said to suggest that IgG4-related inflammation may be detected in the sacroiliac (SI) joints.