Quantitative chest computed tomography is associated with two prediction models of mortality in interstitial lung disease related to systemic sclerosis.

Objective: In this multicentre study, we aimed to evaluate the capacity of a computer-assisted automated QCT method to identify patients with SSc-associated interstitial lung disease (SSc-ILD) with high mortality risk according to validated composite clinical indexes (ILD-Gender, Age, Physiology index and du Bois index).

Methods: Chest CT, anamnestic data and pulmonary function tests of 146 patients with SSc were retrospectively collected, and the ILD-Gender, Age, Physiology score and DuBois index were calculated. Each chest CT underwent an operator-independent quantitative assessment performed with a free medical image viewer (Horos).

Diagnosis and treatment of rheumatoid arthritis in the Emilia Romagna region: a prospective population-based study.

Objectives: To perform a population-based study in rheumatoid arthritis (RA) patients, in order to evaluate the efficacy and safety of pharmacologic treatments.

Methods: 1087 patients with RA were enrolled; inclusion criteria were: newly diagnosed RA, already diagnosed RA with high disease activity (HDA) (DAS28≥4.2) starting biologic DMARDs (bDMARDs), already diagnosed RA with HDA continuing with conventional DMARDs (cDMARDs).

Interleukins (ILs), a fascinating family of cytokines. Part II: ILs from IL-20 to IL-38.

Every nucleated cell can produce and respond to cytokines, extracellular proteic/glycoproteic mediators that constitute a complex, interconnected, and flexible signaling network, addressed to modulate cell behavior and homeostasis through the interaction with high-affinity surface receptors. These messenger molecules, whose main characteristics are potency, pleiotropism, and redundancy, primarily act in autocrine, paracrine, and juxtacrine way, but can also display systemic activity in endocrine-like modality. They are generally classified according to their cellular sources, three-dimensional structure, or biological functions.

Quantitative CT indexes are significantly associated with exercise oxygen desaturation in interstitial lung disease related to systemic sclerosis.

Introduction: Interstitial Lung Disease (ILD) is the first cause of death related to Systemic Sclerosis (SSc). The ILD severity can be assessed with clinical, functional and radiological outcome. Nevertheless none of them is completely validated in clinical practice.

Quality of life and functional disability in patients with interstitial lung disease related to Systemic Sclerosis.

Background: Systemic Sclerosis (SSc) is a connective disease impairing respiratory function. SSc worsens patients' Health Assessment Questionnaire (HAQ-DI), Short Form 36 Physical and Mental Component Summary (SF36-PCS and SF36-MCS). The aim of this work is to verify whether there is correlation between quality of life and lung interstitiopathy in SSc patients.

Operator-independent quantitative chest computed tomography versus standard assessment of interstitial lung disease related to systemic sclerosis: A multi-centric study.

Purpose: Interstitial lung disease (ILD) related to systemic sclerosis (SSc) is assessed with pulmonary functional tests (PFTs) and semi-quantitative scores based on extent of ILD detectable on chest computed tomography (CT). CT quantitative indexes (QCTIs) are promising tools to assess extent of ILD. This study's aim is to evaluate the validity of QCTI compared with that of chest CT standard evaluation and PFTs.

Systemic sclerosis interstitial lung disease evaluation: comparison between semiquantitative and quantitative computed tomography assessments.

The pulmonary fibrosis extent in systemic sclerosis (SSc) has a prognostic value. Chest Computed Tomography (CT) is the gold standard to detect an interstitial lung disease (ILD). Semi-quantitative scores and quantitative methods can estimate the ILD.

Epigenetics in autoimmune connective tissue diseases.

Unlabelled: Background. Autoimmune connective tissue diseases (ACTDs) encompass a heterogeneous group of chronic immune-mediated inflammatory disorders, primarily affecting connective tissues and clinically characterized by variable multisystem manifestations, frequently overlapping. Environmental factors are thought to promote ACTD development in genetic predisposing/endocrine permissive background through the induction of epigenetic modifications, consisting of stable, heritable, but potentially reversible changes in gene expression, occurring without alterations of the DNA sequence.

Interleukins (ILs), a fascinating family of cytokines. Part I: ILs from IL-1 to IL-19.

Every nucleated cell can produce and respond to cytokines, extracellular proteic/glycoproteic mediators that constitute a complex, interconnected, and flexible signaling network, addressed to modulate cell behavior and homeostasis through the interaction with high-affinity surface receptors. These messenger molecules, whose main characteristics are potency, pleiotropism, and redundancy, primarily act in autocrine, paracrine, and juxtacrine way, but can also display systemic activity in endocrine-like modality. They are generally classified according to their cellular sources, three-dimensional structure, or biological functions.

Drug survival of the first course of anti-TNF agents in patients with rheumatoid arthritis and seronegative spondyloarthritis: analysis from the MonitorNet database.

Objectives: To compare drug survival of different anti-TNF drugs (infliximab, INF, etanercept, ETA, and adalimumab, ADA) in rheumatoid arthritis (RA) and spondyloarthritis (SpA) by analysing data collected from an Italian multicenter observational cohort study.

Methods: All patients with RA or SpA registered in the MonitorNet database who started their first course of anti-TNF therapy were included. Overall drug survival was measured, along with specific reasons of discontinuation (inefficacy or adverse events).

Does early arthritis clinic organisation improve outcomes? What evidence is there? A systematic review.

Objectives: To perform a systematic review aimed to identify studies addressing the effect of the establishment of a structured organisation programme, named early arthritis clinic (EAC), finalized to manage patients with early arthritis (EA) or suspected early rheumatoid arthritis (ERA).

Methods: A literature search was performed until May 2012 using electronic databases. Additional information was obtained through a hand and grey literature search.

Focus on adipokines.

Once considered a passive reservoir for lipid storage and an inert provider of thermal/mechanical insulation, white adipose tissue (WAT) is presently seen as a highly dynamic endocrine organ that actively modulates a variety of physiologic processes, including energy balance, food intake, inflammation, immunity, metabolism, as well as cardio-vascular (CV) and neuroendocrine homeostasis. Actually, other than fatty acids and lipid moieties, WAT secretes a wide range of bioactive factors, considerably different in therms of structure and functions, including cytokines, chemokines, growth factors, complement system molecules, acute phase reactants, and hormones, among which the products predominantly or exclusively synthesized by and released from adipocytes are categorized as "adipokines". The adipokine expression is intimately linked to various parameters of adiposity (such as total body fat, percentage of body fat, and fat distribution), resulting generally (with very few exceptions, such as adiponectin, omentin, and Zinc-alpha2-glycoprotein) in positive correlation with WAT mass.

Psychiatric and neuropsychological manifestations of systemic lupus erythematosus.

Background And Aim: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease which may involve any organ and system, including peripheral, autonomic, and central nervous system (CNS). According to the American College of Rheumatology nomenclature, the term "neuropsychiatric SLE" identifies neurological and psychiatric syndromes occurring in patients at any time, not attributable to other causes, and categorized in three main groups, namely neurological syndromes of CNS, neurological syndromes of peripheral nervous system, and diffuse psychiatric/neuropsychological syndromes. The SLE neurological and psychiatric manifestations are usually reported together, and specific data on SLE psychopathology are limited.

Proteasomes and immunoproteasomes.

In eukaryotic cells, the protein degradation is a highly regulated and selective process. Membrane-associated or extracellular proteins are degraded in lysosomes, whereas intracellular protein dismantling is primarily non-lysosomal, being realized by complex, not-membrane enclosed and energy-dependent effectors, the proteasomes, localized in both cytoplasm and nucleus. In mammals, the proteasomes constitute a structurally and functionally heterogeneous system, with shared general architecture, different molecular compositions and functions, and tissue-specific distribution.

Focus on human natural killer cells.

Human natural killer (NK) lymphocytes were originally identified by their large granular morphology and their ability to "naturally" kill virus-infected and malignant cells without any priming. Lacking the surface markers of either B or T cells, they constitute the third major lymphocytic population, representing 5-15% of circulating lymphocytes. Their functions are tightly regulated by a delicate balance of signals transmitted by at least four different families of germ-line encoded, non-rearranged activating/inhibitory receptors.

Central nervous system effects of natural and synthetic glucocorticoids.

Natural glucocorticoids (NGC) physiologically modulate body homeostasis and coordinate adaptive responses to stress, involving almost all organs and tissues, including brain. Since their therapeutic availability, synthetic GC (SGC) have been successfully prescribed for a variety of diseases. Mounting evidence, however, demonstrated pleiotropic adverse effects (AE), including central nervous system (CNS) disturbances, which are often misdiagnosed or underestimated.

The effector T helper cell triade.

T lymphocytes play crucial role in immune responses. Effector T helper (Th) cells derive from progenitor naïve CD4+ T cells, after maturational process induced by antigenic stimulation. Their commitment depends on complex interactions with antigen-presenting cells in a permissive milieu, including antigenic type and load, costimulatory molecules and cytokine signaling.

Hematologic manifestations of connective autoimmune diseases.

Autoimmune connective tissue diseases (ACTDs) constitute a heterogeneous group of chronic immune-mediated inflammatory disorders, primarily affecting connective tissues and usually characterized by multisystem involvement with variable and frequently overlapping clinical manifestations. Abnormal immune regulation patterns and persistent inflammation are ACTD hallmarks. In such a context, autoimmunity/inflammation-associated cellular and molecular networks drive a complex of reactions that may involve hemopoietic tissue and peripheral blood cells.

Atherogenesis in rheumatoid arthritis: the "rheumatoid vasculopathy"?

Background And Aim: Rheumatoid Arthritis (RA) is associated with accelerated atherogenesis. RA patients have a reduced life expectancy compared to the general population, and cardiovascular (CV) disease (CVD) is recognized as a strong contributor to the excess of morbidity and mortality. Our aim was to review and discuss the recent advances in the knowledge of the RA-associated atherogenesis.

The inflammasomes: the key regulators of inflammation.

Following any threat to tissutal integrity, innate immune system promptly recognizes foreign/damage-associated molecules and orchestrates the global immune response, inducing inflammation, chemotaxis, phagocytosis and production of antimicrobial effector molecules, as well as providing instruction to the adaptive immune system. Innate immune cells detect both exogenous and endogenous danger signals through invariant germline-encoded pattern recognition receptors, including Toll-like receptors, retinoic acid-inducible gene I-like receptors, and nucleotide binding domain and leucine reach repeat containing receptors (NLRs). The recruitment of NLRs, namely IPAF, NAIPs and NALPs, by various potentially harmful stimuli leads to the assembly of inflammasomes, multimeric caspase-activating complexes entailing the sensor NLR, intracellular adaptor proteins, and procaspase-1 and -5.

Remitting seronegative symmetrical synovitis with pitting oedema in a patient with myelodysplastic syndrome and relapsing polychondritis.

Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) may be the inaugural manifestation of different rheumatic diseases of the elderly, malignancies and myelodysplastic syndromes (MDS). Relapsing polychondritis (RP) is a rare systemic disorder characterised by an inflammatory process involving predominantly cartilaginous structures, the cardiovascular system and organs of special sense. We report on a 72-year-old man with RS3PE and MDS, refractory anaemia subtype, diagnosed at the same time as RS3PE.

[Pulmonary hypertension in rheumatic diseases].

In rheumatic diseases (RD) pulmonary hypertension (PH) may result by either direct damage of the pulmonary arteries (isolated PH) or pulmonary interstitial fibrosis and other causes. PH is an important cause of morbidity and mortality in systemic sclerosis in which it is more frequently isolated in the limited cutaneous variant and secondary to interstitial fibrosis in the diffuse type. In isolated PH the main histopathological finding is an occlusive arteriopathy.

[Etiopathogenic and clinical considerations on primary pulmonary hypertension].

Primary pulmonary hypertension is a severe condition of unknown etiology first described by Romberg in 1891. The authors review the literature with particular emphasis on new hypotheses concerning etiopathogenesis, and recent suggestions for management. The former take into account the discovery of endothelium derived relaxing factors and the identification in pulmonary arteries of a polypeptide called endothelin.