Acute exacerbation (AE) is a possible manifestation of interstitial lung diseases (ILD) associated to very high mortality. It's defined as clinically significant respiratory deterioration with evidence of new widespread alveolar abnormalities on computed tomography scan. AE is better described in idiopathic pulmonary fibrosis (IPF) but also reported in ILD secondary to connective tissue diseases (CTD) and vasculitis. The main features and the real clinical impact of this severe complication in these patients are not well defined. Aim of our study was to prospectively investigate the incidence, clinical features and outcome of AE in a population of patients with ILD related to CTD and vasculitis. We consecutively enrolled all patients, with ILD secondary to rheumatic systemic diseases, referring to our multidisciplinary outpatient clinic for rare lung diseases. All patients were followed for at least 12 months (range, 12-36 months). At baseline, all patients underwent to a core set of laboratory investigations and periodically followed; data about demographic, disease onset, clinical, serological and therapeutic features were also recorded. AE occurred in 9/78 patients, with an incidence of 5.77/100 patients/year, and 5/9 patients died because of AE. The baseline value of DLCO was significantly associated to the risk of AE at Cox regression. In patients with ILD related to rheumatic systemic diseases AE can occur with an incidence similar to IPF. Rheumatologists should carefully consider this life-threatening complication as a possible natural course of all patients with ILD secondary to systemic rheumatic disease.
In 2019, the novel SARS-CoV-2 infection emerged, causing the disease called COVID-19, which primarily affects the respiratory tract and lung at alveolar and interstitial levels. Systemic sclerosis (SSc) is an autoimmune connective disease characterized by vascular abnormalities and diffuse and progressive fibrosis of the skin and internal organs. Raynaud phenomenon (RP) occurs in virtually all patients affected by SSc and, in most cases, is an onset symptom of the disease; that is, RP may appear several years before overt illness. Although the exact pathophysiologic pathways leading to RP and SSc are still unknown, several infectious agents, especially viruses, have been suggested as possible triggering factors. Here, the authors describe the first case of RP secondary to SSc following SARS-CoV-2 infection.
BACKGROUNDS: Systemic Sclerosis (SSc) is a complex autoimmune and up to fifty percent of patients develop digital ulcers. AIMS: Understand how much infections influence scleroderma digital ulcers’ healing. METHODS: We revised fifty consecutive patients with SSc-related DUs who referred to our Scleroderma Unit. Thirty-five of them who showed clear signs of DUs infection underwent to cutaneous swab and microbiological data collection. We performed 87 cutaneous swabs overall. RESULTS: DUs were recurrent in 58% of the patients and multiple in 60% of patients. Fourty-four swabs (53%) were positive for Staphylococcus Aureus (13% Methicillin-Resistant), 9 were positive for Pseudomonas Aeruginosa (Pseudomonas A.) (10%), and then the others less frequently isolated. Twenty-fifth percent of patients needed hospitalization. CONCLUSIONS: Our data support a patient-tailored approached to DUs, particularly those infected. Self-hygiene and asepsis during dressing procedures are mandatory. Patient must be trained to avoid dangerous behaviors and reduce the risk of infection.
BackgroundSystemic sclerosis (SSc) is a chronic connective tissue disease characterised by endothelial dysfunction, dysregulation of fibroblasts with excessive fibrosis of the skin and internal organs and autoimmune abnormalities. Cardio-pulmonary manifestations are common in SSc and their detection in the early stage of the disease as well as their careful follow-up are mandatory in order to counteract their impact on the overall disease outcome. Despite the need of establishing a proper methodology, literature provides few reports about this issue.ObjectivesTo evaluate the activity of our Cardio-Rheumatology Clinic in order to optimise diagnostic management of cardio-pulmonary disease in SSc patients.MethodsWe retrospectively analysed data from 350 consecutive SSc patients referred to our University-based Rheumatology Centre and SSc Unit (F/M 308/42; lc/dcSSc 45/305; mean age 50.8±14.7 years; mean disease duration 10.9±7.0 years). All patients underwent general and cardio-pulmonary assessment, in particular they were evaluated in the Cardio-Rheumatology Clinic. The following parameters were considered: physical examination; past and current drugs; blood tests, in particular Erythrocyte sedimentation rate-ESR, C-reactive protein-CRP, CPK enzymes, troponin, NT-pro-BNP, d-dimer, serum autoantibodies, 25-OH-vitamin D; capillaroscopy; pulmonary function tests; high resolution scan of the lungs (HRCT); standard electrocardiogram (ECG) and 24 hour Holter ECG monitoring; Doppler echocardiography; cardiac stress test; coronary angiography and right heart catheterization (RHC); cardiac MRI and CT; vascular ultrasound (intima-media-thickness, carotid-femoral and brachial-ankle pulse-wave-velocity). The clinicians decided to perform these examinations according to clinical picture and current methodologies.Abstract AB0724 – Figure 1ResultsIn the last 12 months we assessed 300 patients with 1st-level screening (cardio-rheumatologic evaluation, standard ECG, Doppler echocardiography, pulmonary function tests, thoracic imaging). Among 2nd-level, 30 procedures of 24 hour Holter ECG and 15 RHC tests were performed. Cardiac MRI, coronary CT angiography and vascular ultrasound were assessed, when requested, as 3rd-level examinations (30 procedures). After 1 year we observed a mean time of 10±5 days between request and clinical cardio-rheumatologic evaluation, 20±12 days to perform 1st-level screening, 25±15 days to execute the 2nd-level examinations. Figure-1 shows Cardio-Rheumatology algorithm for the management of SSc cardio-pulmonary disease.ConclusionsThe activity of our Cardio-Rheumatology Clinic optimises the cardio-pulmonary SSc assessment, determining an early detection of these harmful complications with reduced waiting times which are critical issues. Screening algorithms are useful to stratify the risk and to establish the most appropriate diagnostic-therapeutic protocols, improving outcome of scleroderma patients. The development of a cardio-pulmonary risk score and the standardisation of a patient care approach, accor...
BackgroundIn psoriatic arthritis (PsA), low disease activity as defined by the Minimal Disease Activity (MDA) index is considered a good treatment target. However, as MDA is based only on clinical findings, it might not capture pauci-symptomatic inflammation. Sensitive imaging such as ultrasound (US) might disclose residual inflammatory signs in PsA patients in MDA.MethodsIn this cross-sectional multicentre study, adult PsA patients on biologic treatment in MDA for at least 6 months were consecutively enrolled for a thorough clinical and US examination. Data collection included demographics, personal history, main patient's reported outcomes, clinical and US findings of joints, tendon sheaths, tendons, bursae, and entheses involvement. All centers performed the US investigation in B-mode and Power Doppler (PD)-mode using a similar US machine equipped with a 18–6 and 13–5 MHz multifrequency linear probe. Statistical analysis included comparisons between groups and correlation tests.ResultsThe 72 PsA patients enrolled in the study had a median duration of MDA of 12 (6–65) months. Overall, US examination revealed a low number of acute lesions. However, 54% of patients had at least one PD signal in the examined tissues. A joint or enthesis positive PD signal was found in about 19 and 24% of patients, respectively. Synovial hypertrophy, at least one acute entheseal lesions, and bursitis were the most common changes, detected in 41.7, 41.7 and 26% of patients, respectively.ConclusionsPsA patients in a stable state of MDA may still have residual inflammation in peripheral articular structures detectable by US examination.
OBJECTIVE To explore the effect of topical cannabidiol (CBD) in treating digital ulcers in patients with systemic sclerosis (SSc). METHODS In total, 45 patients with SSc who had digital ulcers were consecutively enrolled between January 2019 and December 2019. Of the participants, 25 were treated with CBD during surgical debridement and 20 were treated with standard local therapy. A numeric rating scale for pain and Health Assessment Questionnaire Disability Index were administered at the baseline and at the end of treatment. RESULTS Local treatment with CBD was significantly associated with lower pain scores, higher health assessment scores, and an increase in participants’ total hours of sleep. Patients in the control group more frequently required additional analgesic therapy. CONCLUSIONS Topical CBD may be a valuable tool to treat pain related to digital ulcers in patients with SSc.
BackgroundSystemic Sclerosis (SSc) is a life-threatening connective tissue disease characterized by endothelial dysfunction, autoimmune abnormalities and aberrant fibrosis. Several reports showed an increased risk of cancer in SSc compared to general population, including breast cancer (BC). The relationship between BC and SSc has long been discussed with contradictory results. In our recent Sclero-Breast study we analyzed clinical-pathological features of BC in SSc; we finally observed the development of BC with early stages and good prognosis among these patients.ObjectivesThe aim of this project was to explore the immunohistochemical (IHC) expression of potential biomarkers involved in the molecular pathways at the basis of SSc and BC etiophatogenesis as update analysis from the Sclero-Breast study.MethodsOur observational multicenter retrospective study, performed at Modena University Hospital and Reggio Emilia Hospital in northern Italy, enrolled 33 SSc women with a personal history of BC between January 2017 and December 2019. Clinical and pathological characteristics of BC and SSc were collected. For 22 patients, BC tissues were available and IHC analysis was performed using specific antibodies to evaluate biomarkers and pathways potentially involved. The antibodies used included: PI3K/mTOR/TGFβ/PDGFRα/PDGFRβ/VEGF/EGFR/IL-6/CTLA-4/PDL1. We also reported TILs percentage (stromal tumor-infiltrating lymphocytes) of each sample. The following scores were assigned for general IHC analysis: (-) negative, (1+) positive tumor cells <20%, (2+) positive tumor cells 20-50%, (3+) positive tumor cells >50%. For PDL1 we considered a positivity in case of PD-L1 expression ≥1% in infiltrating inflammatory cells. For TILs quantification we applied the score: 0 (negative), ≤ 50% (low-median expression), >50% (high expression).ResultsThe first IHC analysis was performed on the samples of invasive BC patients (20 pts) and showed a prevalence of high PI3K expression (score of 3+ in 55% of cases) with mTOR overexpression in 45%. A PDL1 positivity was detected in 30%, with high TILs expression in 30%. Biopsies from the 2 pts with ductal carcinoma in situ were characterized by a negativity of almost all parameters, except for a medium-high TILs expression reported (40%/90% respectively). See Figure 1. IHC analysis was also performed according to BC subtypes. The group of HR+/HER2 negative showed high PI3K expression (score 3+) in most of cases (59%) with mTOR overexpression in 50%. CTLA-4 and PDL1 were positive in 25%, with high TILs expression in 25%. HER2 positive patients showed a high PI3K positivity in 50% of cases with mTOR positivity (score 3+) in 25% and high TGFβ expression (score 3+) in 25%. PDL1 was positive in 50% with high TILs expression in 25%. In Triple Negative group, PI3K overexpression was found in 75% of pts with half of cases represented by mTOR score 3+. PDL1 was positive in 50% with high TILs representation (80% of total cell count) in 50% of pts.Figure 1.IHC analysis and TILs% expression in histological samples of SSc patients with invasive BC.ConclusionAccording to our results, SSc patients with BC showed high positivity for PDL1 and high TILs representations in all subtypes. Furthermore, the high expression of PI3K, did not always correlate with mTOR overexpression. Further investigations on larger numbers are needed; however, these aspects seem to confirm that SSc subjects might develop BC at good prognosis, suggesting again a de-escalation strategy of cancer therapies. Finally, the possibility to personalize oncological targeted treatments in this subset of fragile patients could be promising.Disclosure of InterestsNone declared
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