RESUMEN
OBJECTIVES: Recently, the total area of the inflammatory infiltrate and the percentage of inflammatory infiltrate have been proposed as novel histopathological parameters to improve the stratification of patients with Sjögren's syndrome (SS) in clinical trials. Both these parameters provide a more accurate assessment of the extent of the infiltrate in minor salivary gland biopsies (MSGBs) and may overcome the bias related to the Focus score (FS). To date, however, only few studies have investigated their clinical value and feasibility. In this study we revised consecutive MSGBs obtained routinely in a real-life clinical setting and correlated the total area of the inflammatory infiltrate and the percentage of inflammatory infiltrate both with the other MSGB histopathological parameters and with patients' clinical features in order to explore their usefulness in SS diagnostic work-up. METHODS: We assessed the area of the inflammatory infiltrate and the percentage of the inflammatory infiltrate in consecutive MSGBs and correlated these parameters with the number of foci, the FS and the presence of ectopic lymphoid structures (ELS). We also correlated these additional parameters with patients' clinical and biological data. RESULTS: We revised 69 MSGB samples: 46 from patients with a diagnosis of SS and 23 from subjects with no SS. The total area of inflammatory infiltrate and the percentage of inflammatory infiltrate appeared significantly higher in patients fulfilling the ACR/EULAR classification criteria for SS and correlated significantly with both the number of foci (p<0.001) and the FS (p<0.001). Particularly, they correlated better with the ELS in MSGBs than the number of foci and the FS. When we limited the analysis to the 32/69 patients with a FS<1, both the total area of the inflammatory infiltrate (p=0.02) and the percentage of the inflammatory infiltrate (p=0.03), but not the number of foci (p=0.12) remained significantly higher in the 10/32 anti-Ro/SSA positive patients fulfilling the ACR/EULAR classification criteria. Finally, the total area of inflammatory infiltrate and the percentage of inflammatory infiltrate correlated significantly with several biological and haematological SS-related abnormalities including hypergammaglobulinaemia, C4 levels, total number of white blood cells and the number of circulating lymphocytes. CONCLUSIONS: The total area of the inflammatory infiltrate and the percentage of the inflammatory infiltrate in SS referral centres, and particularly for selected cases, may maximise the information on disease activity at tissue level, ultimately improving SS patients' assessment.
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Síndrome de Sjögren , Biopsia , Humanos , Hipergammaglobulinemia , Recuento de Leucocitos , Glándulas Salivales Menores , Síndrome de Sjögren/diagnósticoRESUMEN
The pathogenesis of primary Sjögren's syndrome (pSS) remains poorly understood. However, important efforts have been made during the last few months. In this review, following the others of this series we will summarise the most recent literature on pSS pathogenesis focusing in particular on new insights into pSS animal models, genetics and epigenetics, innate and adaptive immune system abnormalities and tertiary lymphoid structures. Hopefully, novel insights into pSS pathogenesis will pave the way to new therapeutic approaches to the disease improving patients' management and prognosis.
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Síndrome de Sjögren , Animales , Epigénesis Genética , Humanos , Pronóstico , Síndrome de Sjögren/diagnóstico , Síndrome de Sjögren/genética , Síndrome de Sjögren/terapiaRESUMEN
Spondyloarthritis (SpA) is an inflammatory condition characterised by a broad spectrum of clinical manifestations, laboratory abnormalities and imaging features that genetically tend to be associated with the major histocompatibility complex class 1 antigen, HLA-B27, and in which both peripheral and axial joints might be affected. In addition to arthritis, the typical musculoskeletal manifestations are enthesitis and dactylitis. Extraarticular manifestations such as acute anterior uveitis (AAU), psoriasis (PsO) and inï¬ammatory bowel disease (IBD) are also typical of SpA. In this article we have reviewed the literature of the past year on one of the most important variants of SpA, i.e. psoriatic arthritis (PsA) (Medline search of articles published from 1st January 2018 to 31st January 2019).
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Artritis Psoriásica , Espondiloartritis , Uveítis Anterior , Artritis Psoriásica/epidemiología , Antígeno HLA-B27 , Humanos , Psoriasis , Espondiloartritis/epidemiología , Uveítis Anterior/epidemiologíaRESUMEN
Spondyloarthritis (SpA) is the umbrella term for a broad spectrum of inflammatory rheumatic diseases with typical but also rather different clinical manifestations, limited laboratory abnormalities and characteristic imaging features. For classification purposes, a so-called non-radiographic form (nr-axSpA) is differentiated from a radiographic one (r-axSpA) which is almost identical to the classical ankylosing spondylitis (AS) that is genetically strongly associated with the major histocompatibility complex class 1 antigen HLA-B27. In axSpA, the axial skeleton is affected by both inflammation and new bone formation, and joints might be affected. Rather typical musculoskeletal manifestations of SpA are enthesitis and dactylitis, the latter mainly in psoriatic arthritis (PsA). Extra-articular manifestations such as acute anterior uveitis (AAU), psoriasis (PsO) and inflammatory bowel disease (IBD) are also typical of SpA. In this paper we review the literature on axial SpA (ax-SpA) of 2018 (Medline search of articles published from 1st January 2018 to 31st January 2019).
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Artritis Psoriásica , Espondiloartritis , Espondilitis Anquilosante , Uveítis Anterior , Antígeno HLA-B27 , Humanos , Espondiloartritis/patologíaRESUMEN
The current treatment approach in rheumatoid arthritis (RA) follows a stepwise management, starting from early introduction of conventional synthetic (cs) disease-modifying anti-rheumatic drugs (DMARDs), moving to biological (b) DMARDs and targeted synthetic (ts) DMARDs. In the last few years, new drugs with different mechanisms of action have demonstrated their efficacy in treating such a disabling condition, and their approval, along with other more "experienced" treatments, has established their effectiveness on disease activity, damage accrual prevention, patients' quality of life improvement, confirming their safety profile. Moreover, new molecular pathways are under investigation as potential targets of new advanced therapies. Clinicians' capability of stratifying treatment strategies and decisions has improved, with several new tools for the optimisation of long-term management of RA; however, a high proportion of patients are refractory to the available drugs. Finally, as RA is a systemic disease, the knowledge in multi-systemic complications of the disease has grown, as well as the possibility in improving extra-articular manifestations of the disease, although certain drugs have potentially relevant non-articular effects, which need to be monitored. This narrative review summarises the most relevant studies published over the last year in the field of treatment of RA, with the major aim to let clinicians and researchers reflect on "what is new", "what is effective" and "what is safe".
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Antirreumáticos , Artritis Reumatoide , Antirreumáticos/uso terapéutico , Quimioterapia Combinada , Humanos , Calidad de VidaRESUMEN
Systemic lupus erythematosus (SLE) is a systemic autoimmune condition characterised by a wide spectrum of clinical manifestations, partly related to the disease itself, but also linked to its comorbidities and drugs adverse reactions. Following the previous annual reviews, we focused on new insights in SLE clinical features, pathogenic pathways, biomarkers of specific organ involvement and therapeutic strategies. We finally concentrated on SLE aspects that could significantly influence patients' quality of life and that need to be investigated in detail through the development and validation of disease-specific patient-reported outcomes.
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Lupus Eritematoso Sistémico , Animales , Biomarcadores/metabolismo , Comorbilidad , Progresión de la Enfermedad , Estado de Salud , Humanos , Inmunosupresores/uso terapéutico , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/epidemiología , Lupus Eritematoso Sistémico/inmunología , Valor Predictivo de las Pruebas , Calidad de Vida , Factores de Riesgo , Resultado del TratamientoRESUMEN
INTRODUCTION: The aim of this study was to investigate the incidence and clinical presentation of SARS-CoV-2 infections in a Systemic Lupus Erythematosus (SLE) cohort; to assess correlations with disease characteristics and rheumatic therapy; and to evaluate the occurrence of treatment discontinuation and its impact on disease activity. MATERIALS AND METHODS: SLE patients monitored by a single Italian centre were interviewed between February and July 2020. Patients were considered to be positive for SARS-CoV-2 infections in case of 1) positive nasopharyngeal swab; 2) positive serology associated with COVID19 suggesting symptoms. The following data were also recorded: clinical symptoms, adoption of social distancing measures, disease activity and treatment discontinuation. RESULTS: 332 patients were enrolled in the study. Six patients (1.8%) tested positive for SARS-CoV-2 infection, with the incidence being significantly higher in the subgroup of patients treated with biological Disease-Modifying Anti-Rheumatic Drugs (p = 0.005), while no difference was observed for other therapies, age at enrollment, disease duration, type of cumulative organ involvement or adoption of social isolation. The course of the disease was mild. Thirty-six patients (11.1%) discontinued at least part of their therapy during this time period, and 27 (8.1%) cases of disease flare were recorded. Correlation between flare and discontinuation of therapy was statistically significant (p<0.001). No significant increase of rate of flare in a subgroup of the same patients during 2020 was observed. CONCLUSION: Treatment discontinuation seems to be an important cause of disease flare. Our findings suggest that abrupt drug withdrawal should be avoided or evaluated with caution on the basis of individual infection risk and comorbidities.