The role of the dermatologist in Raynaud’s phenomenon: a clinical challenge

Raynaud’s phenomenon (RP) is a functional vascular disorder involving extremities. In his practice, the dermatologist may frequently encounter RP which affects mainly women and is categorized into a primary benign form and a secondary form associated with different diseases (infections, drugs, autoimmune and vascular conditions, haematologic, rheumatologic and endocrinologic disorders). Still today, the differential diagnosis is a clinical challenge. Therefore, a careful history and a physical examination, together with laboratory tests and nailfold capillaroscopy, is mandatory. RP is generally benign, but a scheduled followâ up for primary RP patients should be established, due to risk of evolution to secondary RP. A combination of conservative measures and medications can help in the management of RP. The importance of avoiding all potential physical, chemical and emotional triggers, as well as quitting smoking, should be strongly suggested to the patient. As firstâ line treatment, dihydropyridine calcium channel blockers should be used. If this approach is not sufficient, prostacyclin derivatives, phosphodiesterases inhibitors and endothelin receptor antagonists can be considered as secondâ line treatment. In cases of acute ischaemia, nifedipine and intravenous prostanoids are helpful. In refractory cases, botulinum injections have shown a significant benefit. The approach to the RP patients requires therefore a coordinated care of specialists together with the primary care physician.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/144681/1/jdv14914_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/144681/2/jdv14914.pd

AB0239 EFFECTS OF DYSMETABOLISMS AND COMORBIDITIES ON THE EFFICACY, SAFETY AND RETENTION RATE OF BIOLOGICAL DMARDS (bDMARD) IN INFLAMMATORY JOINT DISEASES.

Background:bDMARDs have an effect on glucose homeostasis (1), lipoproteins profile (2; 3) and blood pressure (4). However, with the exception of obesity (5; 6), there are no clear data on how bDMARDs work in patients who already have or develop metabolic comorbidities and whether these conditions can impact on their efficacy and safety profile.Objectives:to evaluate, in chronic inflammatory joint diseases, the effect of arterial hypertension (AH), dyslipidemia (DYS) and diabetes mellitus (DM) on efficacy, safety and retention rate of first-line bDMARDs therapy.Methods:a retrospective observational study on the clinical charts of Rheumatoid Arthritis (RA), Psoriatic Arthritis (PsA) or Ankylosing Spondylitis (AS), treated with first on-label bDMARD was performed. Data on adverse events, efficacy and comorbidities at the baseline visit in which the bDMARD was prescribed (BL), the visit performed after 6 months of therapy (6M), and the last visit on treatment (LoT) were collected.Results:383 patients (41,8% RA, 33,4% PsA and 24,8% AS) were included in the study, with the predominance of females (F: 67,36%, M: 32,64%; mean age 51,67 ± 15,11 years). Our data show that the presence of comorbidities had no influence on efficacy of bDMARD, while patients who had DYS at BL manifested a higher rate of systemic adverse events either in the first 6 months of therapy (58,9% vs 43,7%, p=0,040) and also later on (80,36% vs. 66,67%, p=0,046). In addition, patients who developed DYS and AH after the 6M visit reported a higher rate of systemic adverse events at LoT visit, compared to others (DYS: 97,8% vs 66,7%, p<0,001; AH: 86,9% vs 65,2%, p=0,031). For what concerns the retention rate, patients who developed DYS or AH during bDMARD treatment continued the drug for a longer period of time (DYS 95,5 vs 19,6 months, p<0,001; AH 72,1 vs 23,4 months, p<0,001). In particular, patients with AH who concomitantly carried out therapy with ACE-inhibitors (ACEi) and/or angiotensin II receptor blockers (ARB) continued bDMARDs for nearly 20 more months than patients who were not exposed to these drugs (40,5 vs 23,4 months, p=0,001) and more frequently maintained the bDMARDS at LoT (59,42% vs. 47,53%). In case of withdrawal in the ACEi/ARB exposed cohort, this was due to well-being and disease remission rather than inefficacy or adverse reaction (p=0,025). In dyslipidemic patients treated with statins, data showed that bDMARDs were continued for a longer time than in DYS patients treated with other anti dyslipidemic therapies (41,09 vs. 26,50 months, p=0,042).Conclusion:our data suggest that AH and DYS may be associated with higher frequency of adverse events but a better drug retention. The combination of bDMARD and ACEi/ARB may determine a better control of the inflammatory process by inhibition of angiotensin II, favouring the achievement of remission. In AH patients on bDMARDs, ACEi and ARB could therefore represent an useful anti-hypertensive drug choice. Similarly, statins could be the treatment of choice in DYS patients.References:[1]Gonzalez-Gay MA, et al. Clin Exp Rheumatol. 2006.[2]Pollono EN, et al. Clin Rheumatol. 2010[3]van Sijl AM, et al. Semin Arthritis Rheum. 2011.[4]Yoshida S, et al. J Hum Hypertens. 2014.[5]Gremese E, et al. Arthritis Care Res (Hoboken). 2013.[6]Heimans L, et al. Arthritis Care Res (Hoboken). 2013.Disclosure of Interests:Laura Cometi: None declared, Cosimo Bruni Speakers bureau: Actelion, Eli Lilly, Nicolò Chiti: None declared, Lorenzo Tofani: None declared, Francesca Nacci: None declared, Francesca Bartoli: None declared, Silvia Bellando Randone: None declared, Ginevra Fiori: None declared, Serena Guiducci: None declared, Marco Matucci-Cerinic Grant/research support from: Actelion, MSD, Bristol-Myers Squibb, Speakers bureau: Acetelion, Lilly, Boehringer Ingelhei

Quantitative analysis of pulmonary vasculature in systemic sclerosis at spirometry-gated chest CT

Objective To prospectively investigate whether differences in pulmonary vasculature exist in systemic sclerosis (SSc) and how they are distributed in patients with different pulmonary function. Methods Seventy-four patients with SSc undergoing chest CT scan for interstitial lung disease (ILD) screening or follow-up were prospectively enrolled. A thorough clinical, laboratory and functional evaluation was performed the same day. Chest CT was spirometry gated at total lung capacity and images were analysed by two automated software programs to quantify emphysema, ILD patterns (ground-glass, reticular, honeycombing), and pulmonary vascular volume (PVV). Patients were divided in restricted (FVC% &lt;80, DLco%&lt;80), isolated DLco% reduction (iDLco-FVC%≥80, DLco%&lt;80) and normals (FVC%≥80, DLco%≥80). Spearman ρ, Mann-Whitney tests and logistic regressions were used to assess for correlations, differences among groups and relationships between continuous variables. Results Absolute and lung volume normalised PVV (PVV/LV) correlated inversely with functional parameters and positively with all ILD patterns (ρ=0.75 with ground glass, ρ=0.68 with reticular). PVV/LV was the only predictor of DLco at multivariate analysis (p=0.007). Meanwhile, the reticular pattern prevailed in peripheral regions and lower lung thirds, PVV/LV prevailed in central regions and middle lung thirds. iDLco group had a significantly higher PVV/LV (2.2%) than normal (1.6%), but lower than restricted ones (3.8%). Conclusions Chest CT in SSc detects a progressive increase in PVV/LV as DLco decreases. Redistribution of perfusion to less affected lung regions rather than angiogenesis nearby fibrotic lung may explain the results. Further studies to ascertain whether the increase in PVV/LV reflects a real increase in blood volume are needed

Preliminary Validation of the Digital Ulcer Clinical Assessment Score in Systemic Sclerosis

OBJECTIVE: To date, "healed/non-healed" and clinical judgment are the only available assessment tools for digital ulcers (DU) in patients with systemic sclerosis (SSc). The aim of our study is to examine a preliminary composite DU clinical assessment score (DUCAS) for SSc for face, content, and construct validity. METHODS: Patients with SSc presenting at least 1 finger DU were enrolled and assessed with the Health Assessment Questionnaire-Disability Index, Cochin scale, visual analog scale (VAS) for DU-related pain, patient global DU status, and global assessment as patient-reported outcomes (PRO), and physician VAS for DU status (phyGDU) as an SSc-DU expert physician/nurse measure. The DUCAS included 7 DU-related variables selected by a committee of SSc DU experts and weighted on a clinical basis. Face validity was examined by consensus and partial construct validity was tested through convergent correlation with other measures of hand function, using Spearman's correlations. A range of patients with SSc was examined. A linear regression model with backward stepwise analysis was used to determine the relationship of individual variables with the primary clinical parameter, phyGDU. RESULTS: Forty-four patients with SSc (9 males, mean age 55 ± 15 yrs, mean disease duration 9.9 ± 5.8 yrs) were enrolled in the study. Overall DUCAS showed significant positive correlations with all abovementioned PRO (r > 0.4, p < 0.01). When all scores and scales were modeled, only DUCAS significantly predicted phyGDU (r = 0.59, R² = 0.354, Akaike information criterion = 385.4). CONCLUSION: Preliminarily, we suggest that the DUCAS may be a new clinical score for SSc-related DU, having face and content validity and convergent/divergent correlations (construct validity). These early data suggest that this score deserves further evaluation

Oral lactobacillus species in systemic sclerosis

In systemic sclerosis (SSc), the gastrointestinal tract (GIT) plays a central role in the patient’s quality of life. The microbiome populates the GIT, where a relationship between the Lactobacillus and gastrointestinal motility has been suggested. In this study, the analysis of oral Lactobacillus species in SSc patients and healthy subjects using culture-independent molecular techniques, together with a review of the literature on microbiota and lactobacilli in SSc, has been carried out. Twenty-nine SSc female patients (mean age 62) and twenty-three female healthy subjects (HS, mean age 57.6) were enrolled and underwent tongue and gum swab sampling. Quantitative PCR was conducted in triplicate using Lactobacillus specific primers rpoB1, rpoB1o and rpoB2 for the RNA-polymerase β subunit gene. Our data show significantly (p = 0.0211) lower LactobacillusspprpoB sequences on the tongue of patients with SSc compared to HS. The mean value of the amount of Lactobacillus ssprpoB gene on the gumsofSSc patients was minor compared to HS. A significant difference between tongue and gums (p = 0.0421) was found in HS but not in SSc patients. In conclusion, our results show a lower presence of Lactobacillus in the oral cavity of SSc patients. This strengthens the hypothesis that Lactobacillus may have both a protective and therapeutic role in SSc patients

Rheumatic diseases in migrant patients resident in Tuscany: epidemiological data analysis and single-center experience

Objective. In the last decades, the number of foreigners in Tuscany has considerably increased with a multiethnic distribution. We reviewed the main rheumatic diseases in the foreign population resident in Tuscany and also reported the experience at the Rheumatology Division of the University Hospital of Careggi, Florence, in order to identify the areas of origin of these patients and the main rheumatic diseases observed in them. Methods. The collaboration with the Tuscan Region provided data about foreign patients residing in Tuscany on January 1, 2021 (country of origin, chronic diseases). Moreover, we conducted a retrospective review of the clinical charts of our Rheumatologic Division from January 1, 2019, to December 31, 2020. Results. In Tuscany, on January 1, 2021, there were 61,373 patients with chronic inflammatory rheumatic diseases, and 3994 of them (6.51%) were foreigners. Most patients were born in Europe (39.03%), followed by the Balkans (15%), South America (11.27%), and North Africa (10.31%). Inflammatory joint diseases, Sjögren syndrome, and systemic lupus erythematosus were the most frequent diseases. In the period 2019-2020, 511 foreign patients visited our Rheumatology Division and mainly originated from the Balkans (34.64%), South America (18%), and European countries (16.44%). In these patients, chronic inflammatory joint diseases and connective tissue diseases (systemic sclerosis, Sjögren syndrome, and systemic lupus erythematosus) were the most prevalent diseases. Conclusions. This study provides a picture of the rheumatic diseases affecting foreign patients residing in Tuscany that are in agreement with the epidemiological data previously provided

Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey

Background A consensus on digital ulcer (DU) definition in systemic sclerosis (SSc) has been recently reached (Suliman et al., J Scleroderma Relat Disord 2:115-20, 2017), while for their evaluation, classification and categorisation, it is still missing. The aims of this study were to identify a set of essential items for digital ulcer (DU) evaluation, to assess if the existing DU classification was useful and feasible in clinical practice and to investigate if the new categorisation was preferred to the simple distinction of DU in recurrent and not recurrent, in patients with systemic sclerosis (SSc). Methods DeSScipher is the largest European multicentre study on SSc. It consists of five observational trials (OTs), and one of them, OT1, is focused on DU management. The DeSScipher OT1 items on DU that reached ≥ 60% of completion rate were administered to EUSTAR (European Scleroderma Trials and Research group) centres via online survey. Questions about feasibility and usefulness of the existing DU classification (DU due to digital pitting scars, to loss of tissue, derived from calcinosis and gangrene) and newly proposed categorisation (episodic, recurrent and chronic) were also asked. Results A total of 84/148 (56.8%) EUSTAR centres completed the questionnaire. DeSScipher items scored by ≥ 70% of the participants as essential and feasible for DU evaluation were the number of DU defined as a loss of tissue (level of agreement 92%), recurrent DU (84%) and number of new DU (74%). For 65% of the centres, the proposed classification of DU was considered useful and feasible in clinical practice. Moreover, 80% of the centres preferred the categorisation of DU in episodic, recurrent and chronic to simple distinction in recurrent/not recurrent DU. Conclusions For clinical practice, EUSTAR centres identified only three essential items for DU evaluation and considered the proposed classification and categorisation as useful and feasible. The set of items needs to be validated while further implementation of DU classification and categorisation is warranted. Trial registration Observational trial on DU (OT1) is one of the five trials of the DeSScipher project (ClinicalTrials.gov; OT1 Identifier: NCT01836263, posted on April 19, 2013)