Polymyositis following varicella and mumps infection in adults: report of two cases.

Idiopathic immune myopathies (IIMs) are autoimmune diseases caused by immune-mediated muscle damage. The etiology remains unclear. Epidemiological and experimental studies, both in animals and humans, hint at viruses as major environmental factors able to trigger aberrant immune responses through many different mechanisms. However, only a few cases of either dermatomyositis or polymyositis following a specific viral infection have been reported in the literature. The objective of this study is to describe the clinical features and the treatment strategy of 2 cases of polymyositis developing shortly after chickenpox and mumps, respectively, and to review the existing literature on the topic. The clinical records of the 2 patients suspected to have developed inflammatory myositis following a viral infection were reviewed. Their clinical history, main laboratory findings, and treatment outcome are presented here. Moreover, a literature search was performed in the PubMed and MEDLINE databases to identify reports describing the association between viral infections and IIMs in patients aged ≥18. The 2 patients reported here developed polymyositis shortly after chickenpox and mumps, respectively, suggesting a causal role for viruses in triggering autoimmunity. Only a few reports published between 1990 and 2020 were found in the literature, possibly linking infections to myositis development. Intravenous immunoglobulin and rituximab were effective for the treatment of viral-triggered polymyositis.

The data project: a shared approach between stakeholders of the healthcare system in definition of a therapeutic algorithm for inflammatory arthritis.

Rheumatic musculoskeletal diseases or RMD [rheumatoid arthritis (RA) and spondyloarthritis (SpA)] are systemic inflammatory diseases for which there are no biomarkers capable of predicting treatments with a higher likelihood of response in naive patients. In addition, the expiration of the anti-TNF blocking drugs' patents has resulted in the availability of anti-TNF biosimilar drugs with the same efficacy and safety than originators but at significantly reduced prices. To guarantee a personalized therapeutic approach to RMD treatment, a board of rheumatologists and stakeholders from the Campania region, Italy, developed a clinically applicable arthritis therapeutic algorithm to guide rheumatologists (DATA project). The general methodology relied on a Delphi technique forecast to produce a set of statements that summarized the experts' consensus. Selected clinical scenarios were discussed in light of the available evidence, and there were two rounds of voting on the therapeutic approaches. Separate discussions were held regarding rheumatoid arthritis, psoriatic arthritis, and ankylosing spondylitis. The decision-making factors for each disease were clinical presentation, demographics, and comorbidities. In this paper, we describe a virtuous process between rheumatologists and healthcare system stakeholders that resulted in the development of a shared therapeutic algorithm for RMD patients naive to bDMARDs.

Predictors of in-hospital mortality of COVID-19 patients and the role of telemetry in an internal medicine ward during the third phase of the pandemic.

OBJECTIVE: The first pandemic phase of COVID-19 in Italy was characterized by high in-hospital mortality ranging from 23% to 38%. During the third pandemic phase there has been an improvement in the management and treatment of COVID-19, so mortality and predictors may have changed. A prospective study was planned to identify predictors of mortality during the third pandemic phase. PATIENTS AND METHODS: From 15 December 2020 to 15 May 2021, 208 patients were hospitalized (median age: 64 years; males: 58.6%); 83% had a median of 2 (IQR,1-4) comorbidities; pneumonia was present in 89.8%. Patients were monitored remotely for respiratory function and ECG trace for 24 hours/day. Management and treatment were done following the timing and dosage recommended by international guidelines. RESULTS: 79.2% of patients necessitated O2-therapy. ARDS was present in 46.1% of patients and 45.4% received non-invasive ventilation and 11.1% required ICU treatment. 38% developed arrhythmias which were identified early by telemetry and promptly treated. The in-hospital mortality rate was 10%. At multivariate analysis independent predictors of mortality were: older age (R-R for≥70 years: 5.44), number of comorbidities ≥3 (R-R 2.72), eGFR ≤60 ml/min (RR 2.91), high d-Dimer (R-R for≥1,000 ng/ml:7.53), and low PaO2/FiO2 (R-R for <200: 3.21). CONCLUSIONS: Management and treatment adherence to recommendations, use of telemetry, and no overcrowding appear to reduce mortality. Advanced age, number of comorbidities, severe renal failure, high d-Dimer and low P/F remain predictors of poor outcome. The data help to identify current high-risk COVID-19 patients in whom management has yet to be optimized, who require the greatest therapeutic effort, and subjects in whom vaccination is mandatory.

Clinical application of lung ultrasound score on COVID-19 setting: a regional experience in Southern Italy.

OBJECTIVE: We aimed to assess the correlation between LUS Soldati proposed score and clinical presentation, course of disease and the possible need of ventilation support/intensive care. PATIENTS AND METHODS: All consecutive patients with laboratory confirmed SARS-CoV-2 infection and hospitalized in two COVID Centers were enrolled. All patients performed blood gas analysis and lung ultrasound (LUS) at admission. The LUS acquisition was based on standard sequence of 14 peculiar anatomic landmarks with a score between 0-3 based on impairment of LUS picture. Total score was computed with their sum with a total score ranging 0 to 42, according to Soldati LUS score. We evaluated the course of hospitalization until either discharge or death, the ventilatory support and the transition in intensive care if needed. RESULTS: One hundred and fifty-six patients were included in the final analysis. Most of patients presented moderate-to-severe respiratory failure (FiO2 <20%, PaO2 <60 mmHg) and consequent recommendation to invasive mechanic ventilation (CPAP/NIV/OTI). The median ultrasound thoracic score was 28 (IQR 18-36) and most of patients could be ascertained either in a score 2 (40%) or score 3 pictures (24.4%). The bivariate correlation analysis displayed statistically significant and high positive correlations between the LUS score and the following parameters: ventilation (rho=0.481, p<0.001), lactates (rho=0.464, p<0.001), dyspnea (rho=0.398, p=0.001) mortality (rho=0.410, p=0.001). Conversely, P/F (rho= -0.663, p<0.001), pH (rho = -0.363, p=0.003) and pO2 (rho = -0.400 p=0.001) displayed significant negative correlations. CONCLUSIONS: LUS score improve the workflow and provide an optimal management both in early diagnosis and prognosis of COVID-19 related lung pathology.

Pronounced dys-autonomic symptoms announcing a primary Sjögren's syndrome.

Sjögren's syndrome (SS) is an autoimmune disease that involves the nervous system in about 20% of cases. In 25-92% of patients affected by Sjögren's syndrome, neurological symptoms may precede the sicca syndrome. A 65-year-old male presented with a seven-month history of episodes of near-syncope, constipation, anhidrosis, disabling fatigue and asthenia. Physical examination was unremarkable, whilst the ECG revealed sinus bradycardia. Laboratory tests showed lymphopenia and normal inflammatory markers. In order to assess a potential autonomic neuropathy, "Deep Breathing Test" (E/I 1.02), "Lying to Standing Test" (R/R' 0.95), and "Orthostatic Hypotension Tests" (T 120s Systolic reduction >20 mmHg and Diastolic reduction >10 mmHg) were performed, all of which were abnormal. ECG Holter monitoring revealed sinus bradycardia, and right bundle branch block with 24-h blood pressure monitoring revealing a diurnal hypotensive profile. The patient reported a three-month history of worsening dry mouth. On physical examination, the patient had anisocoria in response to light stimulation. Auto-antibody testing was performed to evaluate the presence of any autoimmune disease. The results of these studies included an abnormal elevation of ANA (1:320 speckled pattern), Ro/SS-a (>240U/l), and La/SS-b (162 U/ml) antibodies. The patient was discharged with a diagnosis of "Autonomic Neuropathy Most Likely Due to Primary Sjögren's Syndrome (SS)" and started the immunotherapy. After one month, he reported a significant improvement in his symptoms with a concomitant normalization of his "Orthostatic Hypotension Tests." This case underlines the potential for dys-autonomic symptoms to precede the onset of sicca syndrome in patients with Sjogren's Syndrome.

Validation of plasma microRNAs as biomarkers for myotonic dystrophy type 1.

Non-invasive and simple to measure biomarkers are still an unmet need for myotonic dystrophy type 1 (DM1). Indeed, muscle biopsies can be extremely informative, but their invasive nature limits their application. Extracellular microRNAs are emerging humoral biomarkers and preliminary studies identified a group of miRNAs that are deregulated in the plasma or serum of small groups of DM1 patients. Here we adopted very stringent selection and normalization criteria to validate or disprove these miRNAs in 103 DM1 patients and 111 matched controls. We confirmed that 8 miRNAs out of 12 were significantly deregulated in DM1 patients: miR-1, miR-27b, miR-133a, miR-133b, miR-206, miR-140-3p, miR-454 and miR-574. The levels of these miRNAs, alone or in combination, discriminated DM1 from controls significantly, and correlated with both skeletal muscle strength and creatine kinase values. Interestingly, miR-133b levels were significantly higher in DM1 female patients. Finally, the identified miRNAs were also deregulated in the plasma of a small group (n = 30) of DM2 patients. In conclusion, this study proposes that miRNAs might be useful as DM1 humoral biomarkers.

Tibialis anterior muscle needle biopsy and sensitive biomolecular methods: a useful tool in myotonic dystrophy type 1.

Myotonic dystrophy type 1 (DM1) is a neuromuscular disorder caused by a CTG repeat expansion in 3'UTR of DMPK gene. This mutation causes accumulation of toxic RNA in nuclear foci leading to splicing misregulation of specific genes. In view of future clinical trials with antisense oligonucleotides in DM1 patients, it is important to set up sensitive and minimally-invasive tools to monitor the efficacy of treatments on skeletal muscle. A tibialis anterior (TA) muscle sample of about 60 mg was obtained from 5 DM1 patients and 5 healthy subjects through a needle biopsy. A fragment of about 40 mg was used for histological examination and a fragment of about 20 mg was used for biomolecular analysis. The TA fragments obtained with the minimally-invasive needle biopsy technique is enough to perform all the histopathological and biomolecular evaluations useful to monitor a clinical trial on DM1 patients.

Glucocorticoids in systemic sclerosis: patients' beliefs and treatment adherence.

OBJECTIVES: To investigate how chronic intake of glucocorticoids (GCs) is perceived by patients with systemic sclerosis (SSc) in terms of necessity and concerns, and whether such beliefs influence treatment adherence. METHOD: Ninety-eight consecutive SSc patients who they had been taking oral GCs for > 3 months prior to the study were enrolled. All patients underwent a clinical evaluation and laboratory investigations, and were asked to complete four self-administered questionnaires: the Beliefs about Medicines Questionnaire (Specific-BMQ), the four-item Morisky Medication Adherence Scale (MMAS-4), the 36-item Short Form Health Survey (SF-36), and the Health Assessment Questionnaire Disability Index (HAQ-DI). Moreover, patients answered two additional questions investigating their knowledge about GC-related adverse events (AEs). RESULTS: Seventy-seven patients (83.6%) believed in the necessity of GCs for maintaining health but 72.8% also reported concerns about potential AEs to GCs. A high adherence rate was recorded in 64.1% of patients. Only about 20% of patients reported knowing that a daily GC dose and treatment duration influence the burden of GC-related AEs. Patients who believed that GC-related AEs were influenced by the dose scored lower concerns (p = 0.043) and were more frequently labelled as accepting (perceiving high necessity and low concerns) (p = 0.009). CONCLUSIONS: The majority of patients taking GCs perceived them as necessary, but high concerns about chronic GC use exist. Patients with a poorer knowledge of the factors associated with GC-related AEs and those with a poorer quality of life were the most worried about these compounds. Experimental studies assessing the efficacy of educational programmes aiming to increase the adherence to GC therapy should be planned in these patients.

Diffusion and applications of musculoskeletal ultrasound in Italian Rheumatology Units.

The Musculoskeletal Ultrasound Study Group of the Italian Society of Rheumatology (SIR) was founded during the 68th SIR Congress, on November 2011. The request of activation of this group was based on the increasing interest and the widespread diffusion of ultrasound in the scientific rheumatology community and on the solid experience of some Italian rheumatologists in the field. The aims of the Study Group are to stimulate the applications and use of ultrasound in the clinical practice at the level of the Italian rheumatology units and, in addition, to develop research projects at a national level...

Severe polymyositis due to Toxoplasma gondii in an adult immunocompetent patient: a case report and review of the literature.

Toxoplasmosis, a worldwide zoonosis caused by a coccidian parasite Toxoplasma gondii, is more often asymptomatic in immunocompetent patients. We report the case of a 38-year-old immunocompetent male with a polymyositis as the presenting manifestation of T. gondii infection. The patient was hospitalized for a 30-day history of fever (T max 39.5°C), muscle pain, and progressive weakness of the muscles. A diagnosis of polymyositis was made, and he was started on corticosteroid treatment, which caused no reduction of symptoms. After finding a positive polymerase chain reaction (PCR) assay for T. gondii, together with additional clinical findings, a diagnosis of acute toxoplasmosis was made. Specific treatment with pyrimethamine and sulfadiazine was started, with a progressive reduction of symptoms and normalization of laboratory tests.

Oral feeding competences of healthy preterm infants: a review.

Background. With increasing sophistication and technology, survival rates hugely improved among preterm infants admitted to the neonatal intensive care unit. Nutrition and feeding remain a challenge and preterm infants are at high risk of encountering oral feeding difficulties. Objective. To determine what facts may impact on oral feeding readiness and competence and which kind of interventions should enhance oral feeding performance in preterm infants. Search Strategy. MEDILINE database was explored and articles relevant to this topic were collected starting from 2009 up to 2011. Main Results. Increasingly robust alertness prior to and during feeding does positively impact the infant's feeding Skills. The review found that oral and non-oral sensorimotor interventions, provided singly or in combination, shortened the transition time to independent oral feeding in preterm infants and that preterm infants who received a combined oral and sensorimotor intervention demonstrated more advanced nutritive sucking, suck-swallow and swallow-respiration coordination than those who received an oral or sensorimotor intervention singly.

Evidence of the contribution of the X chromosome to systemic sclerosis susceptibility: association with the functional IRAK1 196Phe/532Ser haplotype.

OBJECTIVE: Several autoimmune disorders, including systemic sclerosis (SSc), are characterized by a strong sex bias. To date, it is not known whether genes on the sex chromosomes influence SSc susceptibility. Recently, an IRAK1 haplotype that contains the 196Phe functional variant (rs1059702), located on Xq28, was found to confer susceptibility to systemic lupus erythematosus (SLE). This study was undertaken to test for an association between SSc and the IRAK1 SLE risk haplotype. METHODS: We tested for an association with the IRAK1 SLE risk haplotype in a discovery set of 849 SSc patients and 625 controls. IRAK1 rs1059702 was further genotyped in a replication set, which included Caucasian women from Italy (493 SSc patients and 509 controls) and Germany (466 SSc patients and 1,083 controls). RESULTS: An association between the IRAK1 haplotype and SSc was detected in the discovery set. In both the discovery and replication sets, the rs1059702 TT genotype was found to be associated with specific SSc subsets, highlighting a potential contribution to disease severity. A meta-analysis provided evidence of an association of both the T allele and TT genotype with the overall disease, with an odds ratio (OR) of 1.20 and 95% confidence interval (95% CI) of 1.06-1.35 for the T allele (P = 0.003) and an OR of 1.49 and 95% CI of 1.06-2.10 for the TT genotype (P = 0.023). However, the most notable associations were observed with the diffuse cutaneous, anti-topoisomerase I antibody positive, and SSc-related fibrosing alveolitis subsets (OR 2.35 [95% CI 1.51-3.66], P = 1.56 × 10(-4), OR 2.84 [95% CI 1.87-4.32], P = 1.07 × 10(-6), and OR 2.09 [95% CI 1.35-3.24], P = 9.05 × 10(-4), respectively). CONCLUSION: Our study provides the first evidence of an association between IRAK1 and SSc, demonstrating that a sex chromosome gene directly influences SSc susceptibility and its phenotypic heterogeneity.

Police job strain during routine activities and a major event.

BACKGROUND: Special police forces are exposed to periods of intense work stress in ensuring public order. AIMS: To explore the relationship between the work context (routine work or special event) of special force policemen and psychological measures of job strain (demand-control) and effort-reward imbalance. METHODS: All policemen assigned to the G8 meeting in L'Aquila, Italy, in July 2009 were invited to complete a questionnaire while engaged in routine work in January 2009 (Time A) and in June 2009 (Time B), while preparing for the special event. RESULTS: Participation rate in the questionnaire study was 292/294 (99%) members of the special police force. Measures of job strain (-0.39, P < 0.001) and effort-reward imbalance (-0.37, P < 0.001) decreased significantly from Time A to Time B. On average, demand decreased from 14.2 ± 1.9 to 12.6 ± 2.7 (P < 0.001), control increased from 11.8 ± 2.5 to 14.4 ± 3.4 (P < 0.001) and social support increased from 17.8 ± 2.9 to 19.0 ± 3.1 (P < 0.001). At the same time, effort decreased from 17.4 ± 3.2 to 11.8 ± 3.8 (P < 0.001), reward grew from 37.6 ± 5.5 to 45.5 ± 7.4 (P < 0.001) and overcommitment dropped from 7.1 ± 2.1 to 6.6 ± 1.7 (P < 0.001). CONCLUSIONS: In special police forces, routine work may be significantly more stressful than a single critical event.

[The coping and support activities in the phenomenon of mass immigration in Italy: evaluation of work-related stress in police forces]. / L'attività di accoglienza e sostegno nel fenomeno di immigrazione di massa in Italia: valutazione dello stress lavoro-correlato nelle Forze di Polizia.

UNLABELLED: The recent emergence of new democracies in North Africa results in a massive wave of immigration in Europe with thousands landing in recent months on the coast of southern Italy. This phenomenon has caused major problems of public order (PO), worsened by the presence of ever-increasing numbers of immigrants and multiculturalism, exposing the Special Forces of the Italian State Police in periods of intense work-related stress. AIMS: To explore the relationship between work context (OP routine i.e. mass migration control vs. OP extraordinary i.e. the G8 summit) of special force policemen and psychological measures of job strain (demand-control, JCQ and effort-reward imbalance, ERI) related to objective measure (sickness absences). CONCLUSIONS: data showed that in special police forces routine work as migration control is significantly more stressful than a single special event that presents a very high risk for law enforcement, probably due to positive changes in psychosocial work environment among special police force.

[Survival and death causes in 251 systemic sclerosis patients from a single Italian center]. / Sopravvivenza e cause di morte in 251 pazienti affetti da sclerosi sistemica arruolati in un singolo centro italiano.

OBJECTIVE: To investigate survival in Italian systemic sclerosis (SSc) patients from a tertiary center, reporting death causes. MATERIALS AND METHODS: We analyzed the charts of 251 SSc patients prospectively enrolled in our Rheumatology Unit from 2000 to 2008. Baseline characteristics were recorded. In 2008 the vital status and the causes of death were assessed. Overall and subgroup survival were analyzed by the Kaplan-Meier method and the log-rank test. RESULTS: In 2008, 82% of patients were alive, 8% were known to have died and 10% were lost to follow-up. Overall 5- and 8-year survival were 94.8% and 77.1%, respectively. Patients with an age greater than the median value of the cohort (χ²=4.4; p=0.036), diffuse cutaneous SSc (χ²=3.9; p=0.048), digital ulcers (χ²=6; p=0.015), articular (χ²=5.3; p=0.021), lung (χ²=5.6; p=0.018) and heart involvement (χ²=9.3; p=0.002) had a poorer survival than patients without these features. The majority of SSc-related deaths (60%) were secondary to interstitial lung disease and heart involvement (both 33.3%); 50% of non-SSc-related deaths were due to cancer. CONCLUSIONS: Our study reports an improvement in survival of Italian SSc patients during the last decade with respect to the previous ones. Moreover, a reduction in deaths from renal involvement and an increase in deaths from interstitial lung disease were recorded in Italian SSc patients. Our data are consistent with those from recent survival studies carried out on SSc patients from other geographic areas.

Clinical and subclinical atherosclerosis in systemic sclerosis: consequences of previous corticosteroid treatment.

OBJECTIVE: To investigate the prevalence of clinical and subclinical atherosclerosis in systemic sclerosis (SSc) patients and its associated features. METHODS: Fifty unselected SSc patients and 41 controls, matched for sex and age, were investigated for previous cardiovascular events, cardiovascular risk factors, and ultrasonographic features of subclinical atherosclerosis in the carotid arteries, that is intima-media thickness (IMT) > 0.9 mm or plaques. SSc patients were also investigated for disease features and previous treatment. Finally, blood samples were randomly selected from 27 patients and 18 controls to evaluate concentrations of amino-terminal propeptide of type III procollagen (PIIINP), transforming growth factor (TGF)-ß, hepatocyte growth factor (HGF), soluble interleukin-2 receptor (sIL-2R), IL-13, E-selectin, intercellular adhesion molecule (ICAM)-1, plasminogen activator inhibitor (PAI)-1, tissue plasminogen activator (t-PA), D-dimer, and prothrombin fragments (F1+2). RESULTS: Previous cardiovascular events were recorded in three SSc patients and no controls (p > 0.05). Mean IMT (0.613 ± 0.240 vs. 0.654 ± 0.173 mm) did not differ between patients and controls (p > 0.05), but subclinical atherosclerosis was detected in 14/50 SSc patients and 4/41 controls (p = 0.036). At multiple logistic regression analysis, mean IMT was correlated with older age [p = 0.006; odds ratio (OR) 1.276, 95% confidence interval (CI) 1.043-1.516] and a higher cumulative corticosteroid intake (p = 0.017; OR 1.155, 95% CI 1.027-1.300). No correlation was found with any soluble marker of disease activity and of coagulation/fibrinolysis system activation. CONCLUSION: Our study confirms an increased prevalence of subclinical atherosclerosis in SSc patients and demonstrates a hitherto unknown association with corticosteroid cumulative dosage.

Impaired exercise performance in systemic sclerosis and its clinical correlations.

OBJECTIVES: To investigate the prevalence of impaired exercise performance as assessed by a standardized cardiopulmonary exercise test (CPET) in systemic sclerosis (SSc) and to identify the associated disease features. METHODS: Forty-six SSc patients were enrolled and evaluated for clinical and serological SSc subset, extent of skin and internal organ involvement, and disease activity and severity. Exercise performance was subsequently evaluated in these patients and in 23 healthy individuals matched for sex and age, using a standardized CPET. RESULTS: Exercise performance, measured by maximum oxygen uptake (VO2 max < 80% of predicted value), was found to be impaired in 43/46 patients. Stepwise regression analysis showed that VO2 max adjusted for body weight VO2 max/kg) was independently correlated with the severity of heart (p = 0.001) and lung (p = 0.013) involvement, left ventricular diastolic dysfunction (p = 0.009), and the Health Assessment Questionnaire Disability Index (HAQ-DI) score (p = 0.016). CONCLUSIONS: This study demonstrates that physical disability contributes significantly to the development of impaired exercise performance in SSc patients. Cardiopulmonary exercise testing may be included among the battery of tests used to determine the severity of SSc.