Background:Sjögren’s syndrome (SjS) is a systemic autoimmune disease with a broad clinical presentation from dry syndrome to systemic extraglandular manifestations. The diagnosis can be complex since none of the markers, except anti-Ro, is sufficiently sensitive or specific. Although, minor salivary glands biopsy (MSGB), Schirmer’s test and unstimulated whole salivary flows (UWSF) are the hallmark for the diagnosis of this entity, its use is not widespread in some centers.Objectives:The aim of the study was to analyze the usefulness and safety of the diagnostic protocol for the classification of SjS and the immunological and analytical markers in dry syndrome due to SjS.Methods:Prospective observational study of a cohort of patients with sicca syndrome from a reference center. The diagnostic protocol (Schirmer’s test, UWSF and minimally invasive MSGB) was applied in the same consultation. Demographic, clinical, analytical and histological data were reviewed.Results:Over a period of 6 months, 48 patients with dry syndrome were analyzed, of which 39 women (81.2%). The main suspicion was SjS (39), followed by sarcoidosis (3), IgG4-related disease (2) and other diagnoses (4). The mean age was 59.1±4.4 years. Almost half (45.8%) reported xerostomia and 41.6% xerophthalmia. Recurrent parotidomegaly was described in 6 patients (12.5%) and arthralgias in 12 (25%). Immunologically, 23 (47.9%) presented anti-nuclear antibodies, 13 (27.1%) anti-Ro, 4 (8.3%) anti-La, 12 (25%) rheumatoid factor and 15 (31.2%) low C4. Schirmer test was positive in 32 patients (66.7%), UWSF in 22 (45.8%) and 9 (18.8%) had a focus score ≥1, although 16 (33.3%) had focal lymphocytic sialadenitis in the MSGB. A total of 21 (43,8%) patients were classified according to the 2016 ACR/EULAR criteria. 12 (57.1%) were seropositive SjS and 9 (42.9%) seronegative SjS. MSGB sensitivity was 71% and specificity 96%. Patient reported symptoms were unhelpful to differentiate SjS from other causes of dry syndrome. The number of protocols needed to diagnose a SjS was 2.28 (5.33 in seronegative SjS). Complications associated with the procedure were low (1 of 48) and mild (self-limited paraesthesia). Patients with SjS, unlike those with dry syndrome of another etiology, had more anemia (p<0.001), lymphopenia (p=0.022), ESR (p=0.030), beta-2 microglobulin (p=0.011), ANA (p<0.001), anti-ENA (p=0.006), anti-Ro (p<0.001), low C4 (p<0.001) and hypergammaglobulinemia (p=0.002).Conclusion:Immunological and histological manifestations were more predictive than clinical ones to differentiate SjS from other causes of dry syndrome. MSGB is a simple, sensitive, specific and safe procedure. The application of the diagnostic protocol (Schirmer test, UWSF and MSGB) allowed to standardize the classification of SjS and increased the diagnosis of patients with seronegative SjS.References:[1]Ramos-Casals M, Brito-Zerón P, Bombardieri S On behalf of the EULAR-Sjögren Syndrome Task Force Group, et al. EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies.Annals of the Rheumatic Diseases2020;79:3-18.[2]Guellec D, Cornec D, Jousse-Joulin S, et al. Diagnostic value of labial minor salivary gland biopsy for Sjögren’s syndrome: a systematic review.Autoimmun Rev. 2013;12(3):416–420.Disclosure of Interests:None declared
Background: Unlike other systemic autoimmune diseases (rheumatoid arthritis or systemic lupus erythematosus), the mechanisms involved and the association between ANCA vasculitis with cardiovascular risk factors (CVRF) or cardiovascular events (CVE) are unknown. There may be a phenomenon of "early" atherosclerosis that contributes to an increased cardiovascular risk. This process would not be explained only by the coexistence of the classics CVRF. Objectives: We reviewed the prevalence of classical CVRF and CVE in a cohort of patients diagnosed with ANCA vasculitis. We analyzed whether the appearance of these factors was prior to or subsequent to the diagnosis of the disease or during its evolution. Methods: A descriptive cross-sectional analysis of the classic CVRF and CVE was analyzed in a cohort of patients with ANCA positive vasculitis in follow-up in the Autoimmune Diseases Division of a Spanish hospital. The main demographic characteristics, type of vasculitis and the presence of arterial hypertension, type 2 diabetes mellitus (T2DM), dyslipemia, smoking and obesity were reviewed. Likewise we analyzed CVE (heart failure-HF-, acute coronary syndrome-ACS-, stroke or transient ischemic attack-TIA-and peripheral arteriopathy-PA-) and if each factor was presented at the diagnosis of the disease or they appeared during the evolution after starting immunosuppressive treatment. Results: A total of 35 patients were studied: 21 women (60%) and the average age was 53 years old. A number of 15 were microscopic polyangiitis, 9 granulomatosis with polyangiitis and 11 allergic granulomatous angiitis. Twenty one patients presented hypertension, 9 of them (42.9%) developed it after the diagnosis of vasculitis. From 7 patients with diabetes mellitus, 5 of them were before diagnosed with vasculitis. Nineteen presented dyslipemia and 9 of them (47.4%) presented lipid alteration during the evolution of vasculitis. Overweight/obesity was evident in 4 of the 11 cases after the diagnosis of vasculitis. Only 5 patients did not have a cardiovascular event. ACS was observed in 3 patients, HF in 2 and PA in 1 patient. There were no cases of TIA or ischemic stroke. Four of them had dyslipidemia (3 after diagnosis of vasculitis) (p=0.18) and 3 had hypertension (2 after diagnosis of vasculitis, p=0.66). Three patients were overweight or obese (p=0.3) and two had T2DM (p=0.2), both of them appeared after the diagnosis. Previous history of smoking was observed in 4 of the 5 patients (p=0.06). In 3 patients (71.4%) the cardiovascular event was recorded prior to vasculitis diagnosis and only in 2 cases it occurred during the evolution. Conclusions: This study shows that a high percentage of patients with ANCA vasculitis also presents some type of classic CVRF despite of CVE were not elevated. The diagnosis and treatment of ANCA-positive vasculitis did not statiscally correlate with a greater number of CVE, therefore it would be necessary to carry out studies with a larger number of patients in order to establish conclusions. It is not well de...
BackgroundAntisynthethase syndrome (ASS) is a heterogeneous rare inflammatory condition characterized by myopathy, intersticial lung disease (ILD), arthritis, mechanic hands and Raynaud phenomenon (RP). The hallmark of ASS is the presence of antibodies against aminoacyl-transfer RNA synthetases (anti-ARS). Eight different anti-ARS have been described: anti-Jo1, anti-PL7, anti-PL12, anti-EJ, anti-OJ, anti-KS, anti-Zo, anti-Tyr/YRS. Despite recent publications, correlation between autoantibodies clinical pattern and management in ASS is not entirely well-known.ObjectivesThe aim of this study was to define clinical features, autoantibodies and outcomes of a series of ASS patients.MethodsWe retrospectively analyzed the epidemiology, clinical data, lung function parameters, muscle enzymes, electromyogram (EMG) and autoantibodies pattern and its relationship with clinical manifestations, treatment management and outcomes of 17 patients recruited between 2005 and 2016 from the Autoimmune Diseases Division of a Spanish hospital.ResultsA total of 17 patients were reviewed (15 female). Mean age at diagnosis was 55 years. Median time delay to diagnosis was 5 months. Median follow-up was 65 months. Main clinical symptoms were dyspnea (76.5%), polyartrhitis (58.6%), muscle weakness and myalgias (52.9%), RP (41.1%) and mechanic hands (23.5%). Lung involvement was defined by HRCT compatible with ILD (64.7%) and abnormal functional exploration (47.1%): restrictive pattern (87.5%) and diminished DLCO (61%). Muscle involvement was defined by elevated CK (52.9%) with a median maximum value of 517 IU/L, myopathic pattern on 8 of 13 performed EMG (61.1%) with myositis found in 4 of them (50%), and inflammatory myositis in 5 of 8 performed biopsies (62.5%). Anti-ARS findings were anti-Jo1 (11), PL12 (2), PL7 (1), anti-EJ (2) and one patient with both PL7 and PL12. Anti-Jo1 predominant clinical pattern was ILD (72.7%), followed by myopathy (63.6%) and concomitant myopathy and ILD (45.5%). Anti-PL12 was associated with ILD, RP, and esophageal involvement and no myopathy. Anti-PL7 patient showed mild myopathy and cutaneous association alone. A combination of anti-PL12 and PL7 was described in one patient who developed ILD with severe myopathy. Anti-EJ patients had pulmonary involvement but no evidence of muscle disease. There was no evidence of cancer in any of our patients. Corticosteroids therapy was administered in most of them (88.2%), and corticodependence was highlighted, being necessary at times to associate one or more immunosuppressants.ConclusionsRegardless of ASS being a rare disease, 17 patients were collected. Anti-Jo1 was the most described antibody. It is important to note that one patient was found to be positive for both anti-PL7 and PL12 meanwhile they were described as exclusive, showing overlap of clinical pattern with severe muscle injury. This finding suggests that positive results for more than one ASS antibody infer more severity. In contrast with previous literature, pulmonary was more frequent than muscle involve...
Background:Myositis-specific antibodies (MSA) are highly specific and useful to classify patients as having syndromes with distinct clinical features and prognosis. MSA are almost always mutually exclusive and quite specific, adding value as a useful biomarker for diagnosis. Although individual autoantibodies aren’t sensitive enough to detect the full spectrum of idiopathic inflammatory myopathies (IIM), the sensitivity of a myositis panel is increasing as more autoantibodies are discovered, and as better assays become available.Objectives:We aimed to analyze the usefulness of a myositis-specific immunoblot for the diagnosis of IIM in a hospital cohort from January 2019 to December 2020. We also seek to correlate immunological findings with the risk of associated interstitial lung disease (ILD), cancer, or death.Methods:Retrospective single-center observational study conducted in a Spanish tertiary hospital. In patients with high clinical suspicion of IIM, a myositis immunoblot was performed including Jo1, PL-7, PL-12, EJ, SRP, Mi2, Ku, MDA-5, TIF1-γ, HMGCR, PM-Scl and Ro52 antibodies. The demographic characteristics, the risk of ILD, cancer and death were analyzed.Results:In a cohort of 313 patients with high suspicion of IIM, 87 patients (27.8%) presented a positive MSA (MSA+ve). The mean age at diagnosis was 56.7±16.9 years, with no significant differences between MSA+ve and MSA-ve (p=0.597). Most of the patients were women with significant differences between both groups (80.5% MSA+ve vs 68.1% MSA-ve, p=0.030).IIM were classified as antisynthetase syndrome (ARS) (38%), dermatomyositis (DM) (31%), overlap myopathy (OM) (16.9%) and necrotizing myopathy (NM) (14.1%) according to the manifestations and MSA found (Jo1, PL-12, PL-7, EJ in ARS; Mi-2, MDA-5 and TIF1-γ in DM; Ku and PM-Scl in OM; HMGCR and SRP in NM). The most frequent MSA were anti-Jo1 (16.9%), TIF1-γ (15.5%), Ku (12.7%), Mi-2 (9.9%), PL-7 (9.9%), HMCGR (8.5%), PL-12 (7%), MDA-5 (5.6%), SRP (5.6%) and EJ (4.2%). The presence of Ro52 associated with other MSA was found in 20 patients (22.9%).ILD was the most frequent manifestation (45.2% of the MSA+ve). Non-specific interstitial pneumonia (NSIP) was the most frequent ILD (39.5%), followed by usual interstitial pneumonia (34.2%). The main risk factors associated with IIM-ILD were some subtypes of the MSAs (p<0.001), the association of Ro52 (p<0.001), and older age (p=0.027). Among the IIM, ARS and OM (p<0.001) were more frequently associated with IIM-ILD. The MSAs most associated with IIM-ILD were Jo1, PL-7, PM-Scl, Ku and SRP (p<0.001).Cancer was found in 9.6% of MSA+ve patients. The most frequent tumors were gynecological (37.5%), followed by gastrointestinal (25%) and breast cancer (12.5%). Factors associated with cancer were age (p=0.010), TIF1-γ (p<0.001), SRP (p=0.004), PL-12 (p=0.013), PL-7 (p=0.047) and HMGCR (p=0.027).The mortality of these patients was 3.5%. There were no differences regarding MSA+ve/-ve (p = 0.911). However, MDA-5 (p=0.033) and older age (p=0.001) were associated with higher mortality. There were no significant differences between the IIM classifications, the associated SAD, the presence of cancer or ILD. However, longer follow-up periods and future studies are necessary to confirm these results.Conclusion:The use of a myositis blot allowed classifying, stratifying the risk of ILD, the risk of cancer and the risk of mortality in IIM. IIM-ILD was the most frequent complication, usually manifested as NSIP. The associated risk factors were ARS, OM, some MSAs, Ro52+ and older age. Cancer was a serious and frequent manifestation in these patients, especially in patients with TIF1-γ and other MSAs, so it is essential to know the risk factors and perform an early screening, especially in older patients.A better knowledge of the serological profiles of IIM will provide more individualized approaches and better risk stratification, helping in the management and treatment of these patients.References:[1]Satoh et al. Clin Rev Allergy Immunol. 2017 Feb;52(1):1-19.[2]Betteridge et al. J Intern Med. 2016 Jul;280(1):8-23.Disclosure of Interests:None declared
Background:Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by multiorgan involvement. Pulmonary hypertension (PH) is an uncommon manifestation with high morbidity and mortality whose characteristics, prevalence and evolution in SLE are not completely defined.Objectives:Using data of patients from the inception cohort Registro Español de Lupus Eritematoso Sistémico (RELES), we aimed to to identify the factors associated with pulmonary hypertension (PH) in systemic lupus erythematosus (SLE).Methods:Prospective observational study on a multicenter Spanish inception cohort. Patients with SLE, diagnosed by the American College of Rheumatology (ACR) criteria, since January 2009, who had at least one transthoracic echocardiogram (TTE) performed were selected. Demographic data, diagnostic criteria, follow-ups, treatments and SLEDAI were analyzed.Results:Of 289 patients diagnosed with SLE with TTE performed, 15 (5.2%) patients were identified to have PH. Mean age was 56,9±7,7 years, of which 93,3% (14) were women and 80% (12) Caucasian. The ACR score at diagnosis was 4.66. Mean SLEDAI was 15. Only 5 patients had dyspnea at the time of diagnosis. Mean pulmonary arterial systolic pressure was 49.2±5.6 mmHg. Among the PH, 4 patients had pericarditis (26.6%), 3 (20%) valvulopathies (1 antiphospholipid syndrome), 1 patient pulmonary embolism and 1 shrinking lung. Multivariable analysis indicated that pericarditis (odds ratio (OR)=2.53), and valvulopathies (OR 8.96) were independently associated with the development of PH in SLE. Having PH was associated with older age at diagnosis (p<0.001), more dyspnea (p<0.001), higher ESR (p=0.007), more serositis (p<0.001), higher SLEDAI (p=0.011), higher SLICC (p <0.001), higher number of admissions (p=0.006) and higher mortality (p=0.003).Conclusion:PH in SLE is a serious comorbidity with high mortality. In the RELES cohort it was associated with increased disease activity, pericarditis and valvulopathies. Performing TTE in patients with SLE may favor early diagnosis and treatment.References:[1]Kim JS, Kim D, Joo YB, et al. Factors associated with development and mortality of pulmonary hypertension in systemic lupus erythematosus patients.Lupus. 2018;27(11):1769–1777.[2]Bazan IS, Mensah KA, Rudkovskaia AA, et al. Pulmonary arterial hypertension in the setting of scleroderma is different than in the setting of lupus: A review.Respir Med. 2018;134:42–46.Disclosure of Interests:Jorge Álvarez Troncoso: None declared, Ángel Robles Marhuenda: None declared, Francesca Mitjavila Villero: None declared, Francisco José García Hernández: None declared, Adela Marín Ballvé: None declared, Antoni Castro Consultant of: Actelion pharmaceuticals, GSK, MSD., Gonzalo Salvador Cervelló: None declared, Eva Fonseca: None declared, Isabel Perales Fraile: None declared, Guillermo Ruiz-Irastorza: None declared
Background:Cardiac involvement is one of the most important causes of disability and mortality in patients with systemic lupus erythematosus (SLE). Transthoracic echocardiography (TTE) is a sensitive and specific technique in detecting cardiac abnormalities, particularly mild pericarditis, valvular lesions and myocardial dysfunction in SLE.Objectives:Using data of patients from the inception cohort Registro Español de Lupus Eritematoso Sistémico (RELES), we aimed to analyse the echocardiographic features of cardiac involvement of systemic lupus erythematosus (SLE).Methods:Prospective observational study on a multicenter Spanish inception cohort. Patients with SLE, diagnosed by the American College of Rheumatology (ACR) criteria, since January 2009, who had at least one TTE performed were selected. Demographic data, diagnostic criteria, follow-ups, treatments and SLEDAI were analyzed.Results:We included 289 patients diagnosed with SLE with TTE performed. The mean age was 40.5 ± 1.9 years, of which 86.9% (251) were women and 82.4% (238) Caucasian. The ACR score at diagnosis was 4.98 ± 0.1. Most frequent SLE manifestations were arthritis (59.2%), photosensitivity (49.5%), malar rash (39.1%) and serositis (31.1%). The main immunological findings were: ANA (97.6%), anti-DNA (66.4%), hypocomplementemia (58.7%), antiphospholipid antibodies (31.5%). One third (31.5%) of the TTE performed were pathological. Of these, 13.8% had pericardial effusion, 13.3% valvulopathy, 6.5% myocardial dysfunction, 5.2% pulmonary hypertension and 3.2% myocardiopathy. Regarding valvulopathies, 9,5% presented valvular dysfunction, 3.2% valvular thickening and 0.6% vegetation. The most frequently injured valve was the mitral (9.1%), followed by the aortic (2.8%). The majority of patients (88.26%) were asymptomatic at the time of TTE. However, patients with pathological TTE had more dyspnea than those in the normal TTE group (24.7% vs. 5.8%, p<0.001). Presenting a pathological TTE was associated with higher SLICC score (p<0.001), greater number of admissions (p<0.001) and mortality (p=0.002). A higher SLEDAI was also associated with higher mortality (p<0.001).Conclusion:Cardiac involvement in SLE is not only related to damage accrual but can also be an early manifestation (beyond pericarditis), especially in active SLE. TTE assessment should be considered as a part of routine examination for SLE due to the high prevalence of heart disease even in asymptomatic patients.References:[1]Doria A, Iaccarino L, Sarzi-Puttini P, Atzeni F, Turriel M, Petri M. Cardiac involvement in systemic lupus erythematosus.Lupus. 2005;14(9):683–686.[2]Chen J, Tang Y, Zhu M, Xu A. Heart involvement in systemic lupus erythematosus: a systemic review and meta-analysis.Clin Rheumatol. 2016;35(10):2437–2448.Disclosure of Interests:Jorge Álvarez Troncoso: None declared, Ángel Robles Marhuenda: None declared, Francesca Mitjavila Villero: None declared, Francisco José García Hernández: None declared, Adela Marín Ballvé: None declared, Antoni Castro Consultant of: Actelion pharmaceuticals, GSK, MSD., Gonzalo Salvador Cervelló: None declared, Eva Fonseca: None declared, Isabel Perales Fraile: None declared, Guillermo Ruiz-Irastorza: None declared
Background:Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EPGA). Renal involvement is frequent in AAV and is an important factor for morbidity and mortality.Objectives:The main objective of this study was to analyze the demographic, clinical, histological and therapeutic characteristics of renal involvement in patients with AAV and the risk of renal replacement therapy (RRT) or death.Methods:Retrospective observational study of 56 patients with AAV fulfilling classificatory criteria and renal involvement diagnosed between 1995 and 2020 from a Spanish tertiary centre. We studied the histological involvement (according to the 2010 classification in focal, crescentic, mixed or sclerotic), immunofluorescence (IF) and the treatment received with the risk of RRT or death.Results:We included 56 patients diagnosed with AAV and renal involvement. The mean age was 61.08±4.05 years; 58.9% were women. The mean follow-up time of these patients was 16.14± 8.80 years. Only 57.1% of patients presented systemic involvement.Most frequent non-renal AAV manifestations were lung involvement (39.3%), central nervous system (30.4%), otorhinolaryngology (ORL) (14.3%), skin (8.9%) and cardiac involvement (8.9%). Main immunological findings were ANCA-MPO+ (69.6%), ANCA-PR3+ (23.2%), ANCA-negative (5.4%). Low C3 was found in 19.6% patients. Histologic classification (HC) and need of RRT is described in table 1. Main HC in renal AAV was crescentic, mixed, focal and sclerotic respectively. Eight patients had not biopsy performed. IF was positive for C3 deposits in 20 patients (35.7%). Half of the patients presented <50% normal glomeruli.The treatment of renal involvement in AAV in our cohort was as follows: 83.9% (47) corticosteroids (CS) and cyclophosphamide (of which 40 received intravenous and 7 oral cyclophosphamide; and 12 patients associated plasma exchange (PE) with this treatment), 5.36% CS alone, 2 patients received CS and mycophenolate; 1 CS and rituximab, 1 CS and PE, and 2 patients received no treatment. A total of 13 patients received PE and 18 RRT. The mean time to RRT was 65.44±32.72 months. Relapses were not uncommon, 33.93% of the patients presented ≥1 relapse and 10.71% presented ≥2.Infections were very frequent since they were present in 91.07% of the patients. Other frequent non-immunological complications observed in the follow-up of these patients were thrombosis in 31.14%, cardiovascular events in 28.57% and cancer in 19.64%.Patients with ANCA-PR3+ were younger at diagnosis (p<0.001), were more likely to present cardiac (p=0.045) and ORL involvement (p<0.001). However, neither ANCA-PR3+ nor ANCA-MPO+ were specifically associated with the need of RRT or higher risk of death in our cohort. Use of CS alone for the treatment of renal AAV was associated with higher mortality (p=0.006) but CS and cyclophosphamide with lower mortality (p=0.044). ANCA-negative patients were more likely to receive no treatment. Having <50% normal glomeruli and C3 deposits on IF were associated with an increased need for RRT. Presenting focal disease on HC was protective for the need of RRT. Older age at diagnosis, systemic involvement of AAV and need of RRT was associated with higher mortality.Conclusion:AAVs are complex vasculitides with frequent renal involvement. Increased C3 deposition, non-focal histological forms, and <50% normal glomeruli were related to the need for RRT. In turn, the need for RRT, a later age at diagnosis, and systemic involvement were associated with higher mortality. Holistic and multidisciplinary early management of AAVs in experience centers can help improve renal prognosis and decrease mortality.References:[1]Binda et al. ANCA-associated vasculitis with renal involvement. J Nephrol. 2018 Apr;31(2):197-208.[2]Kronbichler et al. Clinical associations of renal involvement in ANCA-associated vasculitis. Autoimmun Rev. 2020 Apr;19(4):102495.Disclosure of Interests:None declared
BackgroundThe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are rare multisystem autoimmune diseases of unknown cause, characterised by inflammatory cell infiltration causing necrosis of blood vessels. The AAV comprise granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). The AAV are autoimmune diseases with potentially severe systemic involvement that require prolonged immunosuppressive therapy. Infection is a frequent complication in AAV and is associated with increased morbidity and mortality.ObjectivesThe aim of this study was to define epidemiology, ANCA patterns, treatments, infections and outcomes of a series of 39 patients with AAV.MethodsWe retrospectively analysed 39 patients diagnosed with AAV between 1995 and 2017 from the Internal Medicine Department of a Spanish referral centre.ResultsA total of 39 patients were reviewed. 23 female (58.9%). Mean age at diagnosis was 55.6 years. Median time delay to diagnosis was 7.6 weeks. Median follow-up was 91.3 months. Most frequent AAV was MPA with 18 patients (46.2%), followed by GPA with 11 (28.2%) and EGPA with 10 (25.6%). 6 patients (15.4%) had a concomitant autoimmune disease: Systemic sclerosis,2 Antiphospholipid syndrome,2 Lupus1 and Sjögren.1 Only 2 patients (5.1%) had previous infection with hepatitis C virus. Regarding the treatments, all patients received corticoids (bolus 24 patients, 61.5%), 29 (74.4%) cyclophosphamide, 10 (25.6%) rituximab, 19 (48.7%) azathioprine, 4 (10.3%) mycophenolate and 1 (2.6%) methotrexate. 16 patients presented post-treatment lymphopenia, 5 pancytopenia, and 15 hypogammaglobulinemia. 21 patients (53.8%) presented any infection after the diagnosis. The most frequent were bacterial infections (15 patients), presenting 9 patients with sepsis criteria (7 due to gram-negative organisms). 9 opportunistic infections were described: 3 infections by cytomegalovirus, 5 by tuberculosis and 1 by Mycobacterium avium. There were no cases of Pneumocystis jirovecii despite the fact that only 16 patients (41%) performed primary prophylaxis. The factors associated with increased risk of infections were: lymphopenia, pancytopenia and increased BVAS (p<0.05). 6 patients had died at the time of the study (3 associated with infections, 2 with neoplasms and 1 directly with AAV). The Charlson index performed at the time of the study was the best predictor of mortality (p<0’01).ConclusionsInfections were a frequent complication in patients with AAV and one of the main causes of mortality. Risk factors were lymphopenia, pancytopenia and increased BVAS. Bacterial infections were the most frequent but opportunistic infections must be taken into account.Reference[1] Kronbichler A, Jayne DR, Mayer G. Frequency, risk factors and prophylaxis of infection in ANCA-associated vasculitis. Eur J Clin Invest. 2015Mar;45(3):346–68.Disclosure of InterestNone declared
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