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.Background During the first months of the Sars-CoV-2 pandemic, antimalarial drugs were the central axis of the treatment of patients with acute respiratory infection. After that, several studies reported a risk of prolongation of corrected QT interval (QTc) at the electrocardiogram (ECG).Historically, these drugs, have been the common denominator in the treatment of patients with Systemic Lupus Erythematosus (SLE).ObjectivesTo analyze the possible relationship between the use of antimalarial drugs and the electrocardiographic alterations in patients diagnosed with SLE.MethodsCross-sectional study in patients diagnosed with SLE (SLICC 2012). In all of them, we performed a 12-lead ECG at rest. We measured the QT interval: manually and automatically, and its correction was made according to the Hodge formule (QTc)Results91 patients diagnosed with SLE were included in the study. Of the total of patients included in the study, 64 were in current treatment with an antimalarial drug, with a mean of 9.09 (5.73) years of treatment, and a mean cumulative dosage of 813.16 (436.12) gr.Of the patients on current treatment with antimalarial drugs, 4.69% had a prolonged QTc, compared to 3.7% of the patients without current treatment with these drugs.Abstract PO.8.175 Table 1Principal characteristics of the patients included in our study NO antimalarial n= 27 YES antimalarial n= 64 Heart disease 7 (25,93%) 5 (7,81%) Cumulative dosage HCQ (gr) 316,41 (457,28) 813,16 (436,12) ECG disorders 5 (18,52%) 12 (18,75%) Structural disorders 1 (3,7%) 6 (9,38%) Electrical conduction disorders 2 (14,82%) 6 (9,38%) We analyzed the possible relationship between the QTc interval, the current treatment with antimalarial drugs, and the cumulated dosage of this medication. We corrected the lineal regression models by the years of disease evolution, the presence or absence of known heart disease, the women gender, and other treatments such as antiarrhythmics or beta-blockers.We found a statistically significant association between taking antimalarial drugs and the elongated QTc interval (p= 0,001). Nevertheless, in the multivariate analysis, we did not find a significant relationship between the ECG alterations and the treatment with antimalarial drugs.ConclusionsIn our study, we did not observe a direct relationship between the intake of antimalarial drugs and the alteration of the corrected QT interval.

Background:Several drugs have been implicated in the development of de novo systemic lupus erythematosus (SLE), unmasking of quiescent SLE or causing an exacerbation of previously diagnosed SLE.Objectives:Our aim is to describe the causative drugs, clinical and serological features of patients diagnosed of Drug-Induced lupus (DIL) in our Rheumatology DepartmentMethods:A total of 445 patients diagnosed with SLE treated in our Rheumatology Department from 2012 to 2023 were retrospectively screened for the fulfilment of DIL criteria through the search of medical electronic records. Demographic, clinical and laboratory data were summarised using descriptive statisticsResults:We identified 18 patients diagnosed with DIL, representing a prevalence of 4% among all SLE patients in our department. There was a female preponderance and a young age at disease onset. Patients’ clinical and serological characteristics are shown in Table 1. Only three drugs (infliximab, adalimumab and sulfasalazine) were identified as causative agents of DIL, anti-TNF being the most common. Most patients were treated for a condition different from a rheumatic disease, mainly inflammatory bowel disease (IBD). Median time to symptom onset after drug initiation ranged from 3 to 194 weeks (median 50.4). Peripheral arthritis and skin rash were the most frequent symptoms, with 4 patients (22%) presenting both at onset. Serologically, only 2 patients were ANA negative, but tested positive for anti-dsDNA. After drug withdrawal, ANA titre showed a slow decreasing trend over time, as well as anti-dsDNA antibodies. However, only 2 patients lost ANA-positivity through follow-up. Remarkably, more than half of the patients tested positive for antiphospholipid antibodies.Conclusion:DIL showed a prevalence of 4% among SLE cases in our Rheumatology Department. Anti-TNF agents were the most common drugs related to DIL. ANA tended to decrease over time, but only become undetectable in a few patients. Antiphospholipid antibodies are common in our DIL patients. Age at onset is earlier than previously reported, probably because causative drugs are being used in younger populations.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:There is limited information about musculoskeletal involvement in Marfan syndrome, especially concerning bone metabolism.Objectives:Description of the musculoskeletal manifestations in patients diagnosed with Marfan Syndrome (MS) and its association with various clinical manifestations of the disease.Methods:We conducted a retrospective and descriptive study of patients diagnosed with Marfan Syndrome (according to Ghent Criteria) followed in Rheumatology since 2012, collecting demographic, clinical, analytical, and densitometric data.Results:A total of 65 patients were included (52.3% women) with a mean age of 39 (16) years. Genetically confirmed diagnosis was present in 48 cases, with 3 of them as index cases, and the FBN1 gene was the most frequently affected (93.8%).Eighty percent of patients had musculoskeletal involvement (see Table 1), with a history of fractures in 4.6% of patients. About 40% of the performed densitometries showed values in the Osteoporosis range. We observed a tendency for patients with decreased densitometry values to have a higher proportion of aortic dilation (P=0.053) and thoracic wall involvement (P=0.059). Elevated levels of autoantibodies were found in 11 patients.Table 1.Clinical manifestations of patients with MS.VariableN=65n (%)Aortic dilation41 (63.08%)Cardiopathy (mitral, aortic, pulmonary, tricuspid valve disease)30 (46.15%)Marfanoid facies10 (15.38%)Lens subluxation/ Ectopia lentis14 (21.54%)Spinal alignment abnormalities Kyphoscoliosis Scoliosis6 (9.2%)29 (44.6%)Thoracic wall involvement Pectus carinatum Pectus excavatum5 (7.7%)14 (21.5%)Foot involvement (flat, cavus, varus, valgus)19 (29.2%)Knee involvement (genu recurvatum, genu valgum)3 (4.6%)Hip involvement (coxa valga, acetabular protrusion)8 (12.3%)Hand involvement (arachnodactyly)4 (6.15%)Furthermore, a statistically significant association was observed between the presence of aortic dilation and abnormalities of the thoracic wall (P=0.027) and the presence of cardiopathy (P<0.001). There was also a tendency to have greater demineralization of the lumbar spine in patients with cardiopathy, aortic dilation, and thoracic wall abnormalities, with the latter being more frequently observed in male patients.Conclusion:Up to 80% of MS patients presented musculoskeletal manifestations, and 40% showed densitometry values in the osteoporosis range. An association was observed between findings of thoracic wall deformities and the presence of aortic dilation. There was also a tendency to have greater demineralization of the lumbar spine in patients with aortic dilation and thoracic wall abnormalities.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Although the utility of PROs (Patient-Reported Outcomes) has been demonstrated in various systemic autoimmune diseases, including Systemic Sclerosis, there appears to be a lack of data on some PROs in clinical practice for this disease.Objectives:Description of data derived from Patient-Reported Outcomes (PROs) in patients with Systemic Sclerosis (SSc) in real clinical practice.Methods:We conducted a single-center cross-sectional and descriptive study of patients diagnosed with SSc (ACR-EULAR 2013 criteria). Clinical evaluation of patients was conducted, and they systematically completed the Spanish version of the SF36 quality of life questionnaire, as well as the HAQ (Health Assessment Questionnaire) and CHFS (Cochin Hand Function Scale) questionnaires, which assess functional disability. In addition, Rodnan index, EUSTAR activity index, and Medsger severity scale were calculated.Results:A total of 42 patients with SSc were included, with a mean EUSTAR index score of 4.93 (1.45) points, a mean value of 4.8 on the Rodnan scale, and an average value of 4.05 on the Medsger scale.Regarding PROs, in the SF36 quality of life questionnaire, an average score of 51 (29.5) was obtained, and in the HAQ questionnaire, a mean score of 0.78 points, with 64% of patients scoring below 1. The average CHFS score was 14.29 out of a maximum of 90 points, with 71% of cases scoring below 18 points.We observed a statistically significant association between disease severity according to the Medsger scale and hand functional disability assessed through CHFS (P=0.03). A statistically significant association was also found between increased skin thickening according to the Rodnan index and a higher perception of quality of life by SF36 (P=0.03) and a higher perception of disability in both the CHFS scale (P=0.02) and the HAQ (P=0.03).Conclusion:An association was observed between the results obtained with PROs and the assessment of disease activity and severity in patients with Systemic Sclerosis. The role of PROs in the disease can assist us in the management of our patients.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Rheumatoid arthritis (RA) is a systemic autoimmune disease, chronic and inflammatory in nature, which primarily affects the joints and can lead to multisystem involvement. Serologically, a correlation has been observed between the severity of the disease and the presence of certain autoantibodies, such as rheumatoid factor (RF) and anti-citrullinated peptide antibodies (anti-CCP). Recently, anti-carbamylated protein antibodies (anti-CarP) have been described as potential biomarkers for RA, especially in patients who are seronegative for traditionally evaluated antibodies.Objectives:To analyze the relationship between serum levels of anti-CarP antibodies and the serological profile of rheumatoid factor (RF) and anti-CCP in patients with rheumatoid arthritis (RA).Methods:Materials and Methods: A cross-sectional observational study of patients diagnosed with RA and healthy controls (HC). A complete clinical and analytical evaluation was performed, obtaining demographic, clinical, and analytical data. The serological profile of each individual was determined, as well as the levels of anti-CarP antibodies using ELISA.Table 1.FR/anti-CCP(-/-)n=23FR/anti-CCP(-/+)n=10FR/anti-CCP(±)n=7FR/anti-CCP(+/+)n=61Anti-carP, average (Standard Deviation)10.61 (8.33)8.69 (3.99)10.85 (2.45)23.78 (21.06)CRP, average (SD)9.63 (12.49)9.5 (8.27)15.24 (19.34)13.84 (23.25)ESR, average (SD)19.74 (17.66)24.9 (13.28)42.57 (27.09)34.79 (21.65)RF, average (SD)11.26 (4.18)16.2 (7.16)77.29 (56.3)213.38 (339.87)Anti-CCP, average (SD)1.76 (3.37)197.9 (128.25)2.51 (2.69)284.72 (112.34)Results:A total of 101 patients (79% female) with an average age of 45 (±13) years, and 98 healthy controls (67% female) with an average age of 55 (±11) years were included. The patients had significantly higher levels of anti-CarP than the healthy controls (P<0.001), as well as older individuals in general (P=0.004). Upon analyzing the antibody levels, a significant association was observed between elevated levels of anti-CarP and elevated levels of RF (P<0.001). Patients were divided into serological profiles based on the positivity of RF and anti-CCP (see Table 1).Based on the serological profile, patients with double positivity for RF/anti-CCP showed a significantly higher elevation of anti-CarP compared to the rest of the groups (P<0.001). The cutoff value for high levels of anti-CarP was established at twice the standard deviation from the average value of the control population (17.23ng/mL). Thirty-five patients were classified as having elevated anti-CarP, 32 of whom were included in the group with double positivity for RF and anti-CCP (P=0.006), and 3 of them in the group negative for both RF and anti-CCP. No cases of elevated anti-CarP were found in those patients with positive RF or anti-CCP only. Three cases of elevated anti-CarP were also classified among the 98 HCs, without elevation of RF or anti-CCP.Conclusion:Patients with RA have significantly higher serological levels of anti-CarP compared to HCs, and 34.6% of the patients have an elevated anti-CarP profile. High levels of anti-CarP are associated with the combined elevation of RF/anti-CCP. 3% of seronegative RA patients are anti-CarP positive, thereby increasing the seropositive rate from 77% to 80%.REFERENCES:[1] Smolen JS, Aletaha D, Barton A, Burmester GR, Emery P, Firestein GS, et al. Rheumatoid arthritis. Nat Rev Dis Primers. 2018 Feb 8;4:18001.[2] Holers VM. Autoimmunity to citrullinated proteins and the initiation of rheumatoid arthritis. Curr Opin Immunol. 2013;25(6):728-35.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Osteogenesis imperfecta (OI) is a hereditary connective tissue disorder (90% autosomal dominant) characterized by a disruption in type 1 collagen or proteins associated with it. Individuals with OI face an increased risk of fractures due to the lower bone mineral density they exhibit, along with other systemic manifestations.Currently, there is no specific treatment available for these patients, who necessitate a multidisciplinary approach and the use of anti-resorptive drugs in cases of moderate to severe forms with a high risk of new fractures.[1]Objectives:Description of clinical characteristics and evaluation of bone metabolism in patients diagnosed with osteogenesis imperfecta (OI).Methods:Retrospective longitudinal descriptive study in 37 patients with osteogenesis imperfecta (OI) diagnosed through genetic testing or family aggregation (confirmed index case and dominant inheritance).Results:A total of 37 patients were included, comprising 21 females (56.76%) and 16 males (43.24%), with a mean age of 40.24 (17.91) years. Genetic confirmation was present in 23 cases (62.16%), with the most frequent OI subtypes being type I (5), Brück syndrome (2), type III (1), and type 5 (1). The most commonly affected genes were COL1A1 (45.83%) and COL1A2 (41.67%), with a dominant inheritance pattern observed in 92.86% of cases. Fracture history was reported in 97.3% of patients, with an average of 5.65 (3.7) fractures. Recorded fractures included 5 (13.51%) in the spine and 5 (13.51%) in the hip. At the densitometry level (BMD), the initial mineral density was 0.74 g/cm2 (0.14), compared to 0.83 g/cm2 (0.1) in the latest recording, resulting in a delta of 0.08 (0.18). The mean T-Score for the total lumbar spine was -2.54 (1.03). Blood test results showed mean values within the normal range for vitamin D (31.69 ± 13.42), PTH (34.79 ± 14.93), P1NP (43.86 ± 77.15), and β-CTX (0.28 ± 0.44). The alkaline phosphatase mean of 134.24 (121.88) was slightly elevated. Other values such as albumin, glomerular filtration rate, calcium, and phosphorus were within normal ranges. Regarding therapy, 6 patients (16.22%) had not received treatment, 27 (72.71%) received bisphosphonates, and 4 (10.81%) received denosumab. Eleven patients (29.73%) underwent a single line of treatment, 15 (40.54%) underwent two lines, and 5 (13.51%) underwent three lines. Zoledronic acid was the most commonly used bisphosphonate (62.16%), while pamidronate was more prevalent (45.16%) as the initial treatment.Table 1.Treatments used and characteristics in BMD and blood tests.TotalZoledronateNo treatmentDenosumabPamidronateN3723642Age40,24 (17,91)38,13 (17,64)45.17 (17.06)46.5 (25.07)23.5 (3.54)Nº Fractures5,65 (3,7)6.13 (4.03)3.33 (3.08)7.5 (1.29)5 (2.83)Months treatment39,69 (37,88)42.23 (41.8)040.67 (27.02)36.5 (17.68)Initial DMO0,74 (0,14)0.73 (0.15)0.8 (0.08)0.61 (0.11)0.82 (0.07)Last DMO0,83 (0,1)0.84 (0.11)0.78 (0)0.81 (0.18)0.83 (0.12)T-Score-2,54 (1,03)-2.51 (1.04)-2.37 (0.78)-3.22 (0.92)-1.35 (1.48)Vit. D31,69 (13,42)30.72 (11.55)33.96 (16.83)48.57 (17.82)18.85 (0.64)P1NP43,86 (77,15)46 (96.16)45.27 (10.97)28.4 (NA)34.4 (NA)β-CTX0,28 (0,44)0.3 (0.52)0.19 (0.1)0.35 (NA)0.51 (NA)Alkaline phosphatase134,24 (121,88)145.24 (137.99)78.8 (18.01)198.67 (165.66)112 (41.01)Conclusion:Patients with OI in our center exhibit typical mutations and inheritance patterns as described in the literature. Nearly all have a history of previous fractures and demonstrate bone mineral density (BMD) within the range of osteoporosis. The most commonly used treatments are bisphosphonates, with pamidronate in pediatric cases and zoledronic acid in adults being the predominant choices.REFERENCES:[1] Balsa Criado A, Díaz Gonzáles F. Tratado de enfermedades reumáticas, second edition. Chapter 111; 789.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Systemic lupus erythematous (SLE) is an autoimmune disease characterized by deregulation of cytokine production. Interferon (IFN) is a proinflamatory cytokine considered as a key molecule in the SLE etiopathogenesis, being responsible of the differentiation of dendritic cells from monocytes, and indirectly of IL10 upregulation.Objectives:We aimed to analyze the association between inflammatory cytoquine levels (IFN-a2, IFN-b, IFN-g and IL10) and SLE clinical activity for a follow-up period of 12 months in SLE patients.Methods:A longitudinal, observational prospective study with evaluations at baseline and follow-up visits every 3 months (for 1 year) in SLE patients (SLICC 2012 criteria) was performed. In all cases complete laboratory test, clinical evaluation and SLEDAI score was carried out. We analyzed inflammatory cytokines serum levels by colorimetric methods.Results:45 SLE patients (86.7% female) participated in the study, with a mean age at diagnosis of 32.8 (16.2) years and a mean time of disease evolution of 17.9 (11.4) years. The 28.9% of patients showed SLEDAI>6 at the basal visit.The 66.7% of patients were under glucocorticoid treatment, 44.4% under immunosupressants (methotrexate, azatioprine, belimumab or mycophenolate) and 66.7% under antimalarials. SLEDAI and inflammatory cytoquine levels during follow-up is shown in Table 1.Table 1.V0 N=45Mean (DS)V3 N=45Mean (DS)V6 N=45Mean (DS)V9 N=45Mean (DS)V12 N=45Mean (DS)Sledai score6.09 (5.38)3.53 (3.41)5.09 (4.06)3.71 (2.87)3.64 (2.61)Ifn-alpha2 (pg/mL)202.59 (608.11)115.24 (219.33)170.6 (634.44)103.21 (194.39)157.22 (523.12)Ifn-beta (pg/mL)74.61 (91.24)72.65 (114.59)76.2 (101.37)77.23 (114.21)74.55 (97.51)Ifn-gamma (pg/mL)257.18 (413.65)293.62 (395.65)334.26 (485.59)289.19 (354.71)370.53 (794.94)IL-10 (pg/mL)14.35 (21.07)15.48 (16.92)11.79 (15.23)11.02 (11.14)11.86 (11.76)Statistical analysis showed significant association between SLEDAI score and IL-10 (P=0.014) and IFNa2 (0.009), as well as a tendency with IFN-beta (P=0.057), independently of the time of follow-up.Regarding to clinical activity biomarkers, we observed an association between high levels of antidsDNA and elevated IFN-beta (P=0.005) and IFN-gamma (P=0.038), and low levels of C3 and an increment in IL-10 (P=0.006). Patients under antimalarials treatment during follow-up exhibit low levels of IL-10 (P=0.012). No influence of age at diagnosis, time of evolution, vitamin D levels, corticoids and tobacco use in cytoquine levels was observed.Conclusion:We observed an association between IL-10, IFN-alpha2, IFN-beta and IFN-gamma levels with clinical activity, independently of the time of follow-up. IL-10 levels may also be influenced by antimalarial treatment.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Juvenile dermatomyositis (JDM) is a polygenic autoimmune interferonopathy with predominant involvement of the adaptive system. It is the most common inflammatory myopathy in childhood. It is a vasculopathy characterized by muscle weakness, elevation of muscle enzymes and pathognomonic skin lesions such as heliotrope erythema or Gottron papules. The onset is usually between 5 and 14 years and, unlike adults, pediatric patients have more calcinosis, lipodystrophy and skin lesions; Furthermore, it is not usually associated with neoplastic processes. In 60% of the cases, we find myositis-specific antibodies. AntiTIF1 autoantibodies are the most common in white people, with a prevalence between 17 and 35%. AntiMDA5 autoantibodies are more common in Japanese and antiMi2 autoantibodies are detected in between 4 and 10% of cases. Classically, mortality was one third of patients; however, in recent decades, the introduction of new treatments associated with earlier diagnosis has made possible to drastically reduce the mortality, improving the functional prognosis of our patients. However, different results have been reported both in the distribution of specific antibodies and in the clinical evolution of the patients.Objectives:The objective of our study is to review the clinical-analytical characteristics of patients referred to the transition consultation in a tertiary hospital and assess their evolution.Methods:Cross-sectional, single-center study, with retrospective acquisition of variables. Demographic and clinical data were collected in patients with JDM through review of medical records. Data are collected from the baseline visit, considered the one in which the diagnosis is made, from the transition visit and from the last visit in Adult Rheumatology department.Results:14 patients were included (50% men) with a mean age (standard deviation) of 6.53 (3.59) years at the onset of the disease and 6.92 (3.81) years at diagnosis. The median (Q1;Q3) follow-up of the patients is 18.68 (3.95;13.92) years. The mean age of the last outbreak was 14.42(4.65) years. All the patients are Spanish, except for one who is Moroccan. The average age of the patients at the transition consultation was 19.70 (2.90) years. The median follow-up of these patients since the transition consultation is 2.14 (1.52; 4.44) years. At diagnosis, 9(64%) patients presented positive ANAs, all had a speckled nuclear pattern. Regarding the specifics one, we found three AntiMi2, two AntiTIF1, two antiMDA5, one antiRO52 and one AntiSAE2 autoantibodies. Eight patients met definitive disease criteria according to Bohan and Peter. In the remaining six, the diagnosis was probable. 15 patients had CK levels above the laboratory’s upper reference value at the time of diagnosis. Currently, five patients have elevated CKs levels.Table 1 shows the clinical and analytical evolution of the patients throughout the evolutionary course of the disease.Currently, nine patients continue in active treatment, although they have experienced de-escalation since the transition consultation.Table 2 shows the treatments received by the patients.Conclusion:In our series, AntiMi2, anti TIF1 and antiMDA5 autoantibodies have a similar prevalence. Most patients have been treated with a combination of immunosuppressive drugs; Tacrolimus and methotrexate are the most used immunosuppressants with earlier initiation compared to other series published in the literature. At the last visit, 85% of patients are in remission and 35.8% are untreated.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Axial spondyloarthritis (AS) is an autoinflammatory disease in which biologic or targeted synthetic disease modifying drugs (DMARDs) therapy is widely used, with several targets and drugs to chose from. However, some patients are still somewhat refractory to the therapies that are provided to them.Objectives:To analyse the clinical and therapeutic characteristics in AS patients who have received at least three distinct advanced therapy (AT) lines.Methods:This is a retrospective longitudinal observational study of AS patients treated with at least 1 targeted therapy (TT). We analyse the different therapeutic lines administered, their duration and the causes of withdrawal. The patients are classified in non-refractory AS –those who have received up to 2 lines of TT– and refractory AS –those who have received 3 or more lines of therapy–.Results:162 AS patients with TT treatment where included (62.4% men), with a mean age at diagnosis of 41 (13) years and a disease progression time since the beginning of the first TT of 20 (30) months. 59.1% of patients were HLA-B27 positive, 20.5% presented extraarticular manifestations (uveitis and/or inflammatory bowel disease) and 38.3% presented peripheral damage. 10 people required 3 or more different lines of therapy. In those patients, drug survival is shorter (P<0,001). The most used first line treatment was an anti-TNF, although it was less usual in patients with 3 or more therapy lines. The treatment change in second line was mainly due to switching, using cycling in third line of treatment. A tendency to more concomitant DMARD use is observed in people with more therapeutic lines (P=0.06). There is no statistically significant association between singular causes of withdrawal and the use of 3 or more therapeutic families. We also have not found an association between age or sex of patients and the use of more therapeutic lines. A correlation between the use of at least 3 different therapeutic families and the absence of extraarticular manifestations (P=0.01), presence of psoriasis (P=0.022) and anxious-depressive syndrome (P=0.001) have been observed.Table 1.Table 2.Conclusion:6% of our AS patients required the use of at least 3 therapeutic families, with a mean of 4 lines of TT. The treatment with anti-TNF is less used in first and second line in patients who have required at least 3 therapeutic families, and the treatments are mainly modified through switching rather than cycling. The presence of other factors –such as psoriasis or anxious-depressive syndrome– seem to be associated with patients who require 3 or more therapeutic lines.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

Systemic sclerosis (SSc) is often difficult to treat and the evidence in support of many of the usual therapies is limited. Abnormalities in B-cell function in SSc are thought to play a role in disease pathology. Furthermore, B-cell infiltrates have been demonstrated in both the skin and lung specimens in SSc. Rituximab (RTX) is a chimeric monoclonal antibody inhibiting CD20-mediated B-cell proliferation and differentiation that has shown promising results for pulmonary and cutaneous involvement of SSc. Our aim was to assess RTX for pulmonary and diffuse cutaneous involvement in real-life patients diagnosed of SSc. Retrospective observational study of a wide and unselected series of patients diagnosed as SSc from a single university hospital from 2011 to 2022. Patients were classified as SSc following 2013 ACR/EULAR classification criteria. We reviewed pulmonary function through conventional spirometry and diffusing capacity of lung for carbon monoxide (DLCO). Pulmonary involvement was also assessed by high-resolution chest-CT test. Cutaneous involvement was assessed by modified Rodnan skin score (MRSS). We included 15 patients with a median [range] age (at the onset of RTX) of 53 [41-78] years (80% women; 20% men). The median [range] of time since diagnosis and treatment onset was 3 [0-12] years. At the diagnosis of SSc, no one of our patients evidenced a restrictive ventilatory pattern. DLCO was below normal limits in 12 patients (80%). Small airway obstruction expressed according decreased maximal (mid-) expiratory flow (MMEF) 25-75 was present in 1 patient (6.7%). Regarding cutaneous involvement, median MRSS at diagnosis was 18.3 [range 4-26]. RTX intravenous treatment regimen employed was 1000 mg (Day 1, followed by 1000 mg on day 15), with a repeat cycle every 6 months if required. After a median [range] follow-up period of 31 [6-100] months, 12 (80%) patients completed a total of 4 RTX cycles. An analysis of the pulmonary function showed that, after RTX, DLCO decreased in 2 of 15 patients (13.3%) and none presented worsening of MMEF 25-75. In all patients there was an improvement of cutaneous involvement. After a median follow-up of 31 [6-100] months during RTX therapy none of the patients presented any new relapse of cutaneous involvement or MRSS progression. The mean difference between MRSS measurement before and after RTX was 6.4±1.2. Only 2 of the patients (13.3%) had to discontinue RTX in the first term due to infusion-related reaction. After RTX treatment, 4 patients (26.7%) died (prostate cancer, SARS-COV-2 pneumonia, sudden cardiac arrest and ictus) has been well tolerated and no severe infections were reported. In our experience, RTX seems to be a great option in real-life for those patients with pulmonary and diffuse cutaneous involvement due to SSc. However, cardiovascular, infectious and neoplastic complications should be assessed. [1]Thoreau B, Chaigne B, Mouthon L. Role of B-Cell in the Pathogenesis of Systemic Sclerosis. Front Immunol. 2022;13:933468. [2]Morgan K, Woollard C, Beinart D, Host LV, Roddy J. Rituximab treatment for systemic sclerosis-associated interstitial lung disease: a case series of 13 patients. Intern Med J. 2022 Jun 7.Online ahead of print. [3]Narita Y, Funatogawa T, Mii K, Adachi H, et al. Use of Biologics for Systemic Sclerosis and Systemic Sclerosis-Associated Interstitial Lung Disease: Information from a Japanese Hospital Claims Database. Mod Rheumatol. 2022 Jun 2:roac055. Online ahead of print. NIL. None Declared.