Search

Objectives: Interstitial pneumonia with autoimmune features (IPAF) refers to patients with interstitial lung disease and autoimmune features not fulfilling the classification criteria for a specific connective tissue disease. We sought to study the characteristics, disease progression, response to treatment and complications of patients with IPAF in 1-year follow-up period., Methods: Clinical and laboratory findings, comorbidities, medications, pulmonary function tests (PFTs), chest HRCT and complications during the one-year follow-up period were documented for each of the 39 enrolled patients with IPAF., Results: The mean age at the time of IPAF diagnosis was 63.2 (±11) years, and 62% of patients were female. The most common clinical features were arthritis (82%) and rash (54%-not included in the IPAF criteria). Antinuclear antibodies (ANA) (59%) and non-specific interstitial pneumonia (NSIP-61.5%) were the most prevalent autoantibodies and radiological pattern respectively. PFTs at 12 months from baseline stabilized or improved in 79.5% of patients (p> 0.05). Infections were observed in 23.1% of patients during the first and in 12.8% during the second semester of follow-up. Two patients (5.1%) required hospitalization. All infections occurred in patients with non-usual interstitial pneumonia (UIP) pattern (p=0.02)., Conclusions: Arthritis and rash are among the most common features in IPAF suggesting rash could be included into IPAF criteria. Almost 80% of patients had stable/improved PFTs at the end of follow-up. Infections occurred mainly in the first semester of treatment and in patients with non-UIP radiological pattern probably due to higher doses of corticosteroids used in these patients., (© 2020 The Mediterranean Journal of Rheumatology (MJR).)

ANCA-associated vasculitides (AAVs) are characterised by heterogeneous molecular and pathophysiological traits, causing ambiguous differential diagnosis and taxonomy. Response to therapy has proven far from successful, contributing to high mortality. Transcriptome analysis of different vasculitis subtypes adds new leads in elucidating mechanisms of disease and the role of specific cell subsets to them. Recent findings have shown that mitophagy is a procedure whose imbalance could lead to immune dysregulation with certain involvement to autoimmunity. Inflammatory response related mitophagy is yet to be described in AAVs. We here describe a research protocol to investigate mitophagy in monocytes, neutrophils, and T cells in AAV patients, and the relationship of disturbed mitophagy with ANCA seropositivity., (© 2020 The Mediterranean Journal of Rheumatology (MJR).)

The third decade of the 21 st century marks the beginning of a new era in the treatment of rheumatoid arthritis (RA). Recently, after the introduction in clinical practice of different biologics in the first decade, three different oral synthetic targeted agents (JAK inhibitors) have been licensed for the treatment of RA, in patients who had failed or are intolerant to disease modifying anti-rheumatic drugs (DMARDs). Despite the significant progress that these agents bring to the care of RA patients, the risk of infections is still present and clear, given that their risk for serious infections is at least comparable with that of biologic DMARDs, whereas the incidence of herpes zoster is higher than that of bDMARDs. Here, we review the most recent data regarding the risk for serious and opportunistic infections in RA patients treated with biologics or JAK inhibitors, as well the up-to-date approach for managing and preventing such infections in RA patients., (© 2020 The Mediterranean Journal of Rheumatology (MJR).)

Despite the recent advances in treatment, antineutrophil cytoplasmic antibody (ANCA) associated vasculitides (AAV) are among the rheumatic diseases with the highest morbidity and mortality. These outcomes are affected by a variety of factors apart from the disease itself, and are driven by infections, cardiovascular disease and drug toxicity. Even after the induction of remission, patients and their treating physicians are challenged by frequent relapses, accrual of chronic damage and impaired quality of life. Given their rarity along with their heterogeneous disease spectrum, patient registries are of utmost need for the study of all aspects of AAV. Already established AAV registries have a significant contribution in the apprehension of these diseases outside the strict context of clinical trials, and are becoming increasingly important as new drugs and treatment strategies are about to be introduced in daily practice. We here describe the design of a contemporary, web-based and easy-to-use patient registry specifically for patients with AAV, including all the necessary domains suggested by international working groups. This project is anticipated to contribute in better understanding of AAV in our country, with a future prospect of contributing to and sharing data with other international registries., (© 2020 The Mediterranean Journal of Rheumatology (MJR).)

The use of biologic and targeted synthetic disease-modifying anti-rheumatic drugs (tsDMARDs) in rheumatic diseases is constantly increasing during the last decade. Tofacitinib is a new oral Janus Kinase (JAK) inhibitor, approved for rheumatoid arthritis (RA), psoriatic arthritis and ulcerative colitis. Safety data of tofacitinib derived from randomized controlled trials and long-term extension studies has demonstrated a moderate increase in the risk for common serious infections. We describe a case of Pneumocystis jirovecii pneumonia (PJP) in a woman on tofacitinib therapy for RA. Although tofacitinib use has been associated with the development of opportunistic infections, PJP has been rarely reported. PJP should be included in the differential diagnosis of patients with autoimmune disorders under newer oral JAK inhibitors therapy who present with fever, hypoxia and pulmonary infiltrates., (© 2019 The Mediterranean Journal of Rheumatology (MJR).)

Aim of the Study: To evaluate the current disease characteristics, treatment and comorbidities of rheumatoid arthritis (RA) in Greece., Methods: Multicenter, cross-sectional study with a 9-month recruitment period between 2015 and 2016. Demographics, disease characteristics, treatment and comorbidities were collected via a web-based platform., Results: 2.491 RA patients were recruited: 96% from tertiary referral centers, 79% were females with a mean age of 63.1 years and disease duration of 9.9 years. Fifty-two percent were rheumatoid factor and/or anti-CCP positive, while 41% had erosive disease. Regarding treatment, 82% were on conventional synthetic disease modifying anti-rheumatic drugs (csDMARDs), 42% on biologic DMARDs (TNFi: 22%, non-TNFi: 20%) and 40% on corticosteroids (mean daily dose: 5.2 mg). Despite therapy, 36% of patients had moderate and 12% high disease activity. The most frequent comorbidities were hypertension (42%), hyperlipidemia (33%), osteoporosis (29%), diabetes mellitus (15%) and depression (12%). Latent tuberculosis infection (positive tuberculin skin test or interferon gamma release assay) was diagnosed in 13 and 15.3% of patients, respectively. Regarding chronic viral infections, 6.2% had history of herpes zoster while 2% and 0.7% had chronic hepatitis B and C virus infection, respectively. A history of serious infection was documented in 9.6%. Only 36% and 52% of the participants had ever been vaccinated against pneumococcus and influenza virus, respectively., Conclusion: This is one of the largest epidemiologic studies providing valuable data regarding the current RA characteristics in Greece. Half of patients were seropositive but despite therapy, half displayed residual disease activity, while preventive vaccination was limited., (© 2018 The Mediterranean Journal of Rheumatology (MJR).)

Herpes zoster is a common infection especially in elderly persons. It is caused by reactivation of varicella zoster virus (VZV) that has remained dormant within dorsal root ganglia after primary infection. Besides patients with immunodeficiency and malignancies, zoster incidence is also higher in patients with rheumatic diseases compared to the general population. Especially in the group of rheumatoid arthritis (RA) patients being treated with biologics, the risk seems to be steady among regimens with different modes of action (1.6-2.4/100 patients-years). RA patients receiving tumor necrosis factor (TNF) inhibitors are at increased risk during the first year of treatment. The aim of the current research protocol is to evaluate the natural course of herpes zoster and the effect of biologic and conventional synthetic disease modifying anti-rheumatic drugs (DMARDs) on the VZV-specific cell mediated immunity in RA patients after herpes zoster infection. We will - prospectively - include RA patients who develop herpes zoster, while being treated with biologics (anti-TNF, tocilizumab, rituximab, abatacept) and a control group comprised of patients with herpes zoster [RA patients under non-biologic therapies (corticosteroids/csDMARDs) and age- and sex-matched healthy controls). Titers of IgG specific antibodies against VZV glycoprotein gp1 and the percentage and absolute number of VZV-specific activated CD4+ T cells (CD4+CD69+IFN-γ+) at the time of rash onset and during follow-up will be measured by ELISA and flow cytometry respectively. This study will contribute to the better understanding of various aspects of immune response to VZV in the modern treatment era with biologic DMARDs., (© 2017 The Mediterranean Journal of Rheumatology (MJR).)