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Objective To determine the reasons for hospitalisation in patients with systemic lupus erythematosus (SLE) admitted to the Hospital de Clinicas "Jose de San Martin" Buenos Aires, Argentina. Methods We retrospectively analysed the clinical histories of SLE patients (SLICC 2012) during or prior to hospitalisation, from 1-2014 to 12-2017. Demographic data, reasons for hospitalisation, treatments, SLEDAI-2 K and comorbid conditions. Results 121 hospitalisations corresponding to 72 patients, 25 patients (34.7%) were hospitalised more than once. Females (83.3%), the median length of disease until admission was 5 years. There was more than one reason for hospitalisation in 32, a total of 164 reasons. The main reason was relapse of disease (52.4%). The most frequent manifestations were renal involvement, serositis and pulmonary involvement. Infections were the second reason for hospitalisation (26.8%); urinary tract, pneumonia and soft tissue. Cardiovascular involvement 4.9%. Hospitalisation in a closed unit 14.05%, mortality 2.48%. Conclusion Relapse of disease and infections were the main reasons for hospitalisation; admission secondary to infection was higher than that reported in other series.

Background The use of Hydroxychloroquine (HCQ) in Systemic Lupus Erythematosus (SLE) has been shown to decrease disease activity and all cause mortality. Retinal toxicity (RT) may limit its use and the pathogenesis of RT has not been fully established. The aim of this study is to evaluate retinal toxicity in our SLE cohort. Methods Our electronic SLE database (Jan 2014–June 2018) was evaluated. Patients fulfilling ACR 1997 criteria, with at least 1 year follow-up, at leats 12 week of HCQ treatment and computerized visual field (CVF) study performed who suspended HCQ due to RT were included. Patients with other HCQ suspension causes (intolerance, hypersensibility, muscular toxicity, skin rash) were excluded. RT was defined as diminution in visual acuity and/or worsening in CVF below reference levels that required HCQ suspension or dose adjustment. Other possible causes of retinal alteration (diabetes, arterial hypertension), cumulative dose of HCQ at the time of suspension, time of HCQ exposure and accrual damage by SLICC-DI at the time of suspension and at the last recorded visit were assessed. Results 231 patients evaluated. 33 included. 10 (30%) reinitiated treatment at a later time, 5 with dose adjustment. HCQ suspension was definite in 23 (69.7%, 9.9% of the global population). Patients with RT had mean age 44.5 (DS ±12,6) years; Ethnicity: Amerindian 6,06%, Caucasian 6,06% and Mestizo 87,8%; 12 (36.3%) had arterial hypertension and 2 (6.06%) diabetes. Median Cumulative dose of HCQ at suspension was 576 gr (IQR 144–1008). 9 (27.2%) patiens had cumulative dose >1000 gr and 13 (39.3%)>5 years of exposure. Mean SLICC-DI at suspension was 0.8 (DS ±1,2) and mean SLICC-DI at last visit was 1,17 (DS ±1,7). 22 (70.9%) patients with CVF worsening were receiving HCQ>5 mg/kg (real weight) and 23 (74.1%) were receiving HCQ>6.5 mg/kg (ideal weight) Conclusions We observed a similar frecuency of HCQ suspension due to RT as reported in other studies. In our patients, there was no difference in RT between patients receiving We found a higher frecuency of RT in patients with cumulative dose of HCQ 1000 gr of cumulative dose. A raise in the SLICC-DI score was observed after HCQ discontinuation, reaffirming the role of HCQ therapy in SLE. A limitation is that RT was defined by CVF and was not routinely assessed by higher complexity studies (OCT). As a strength, RT was studied in the Mestizo population of Argentina, the dominant ethnicity, which might not be fully represented in previous international studies. Funding Source(s): None

Background Neonatal lupus (NL) is a disease in children of mothers who have specific anti-Ro/La IgG autoantibodies by passive transplacental transfer. LN is characterized by skin and cardiac involvement, as well as cytopenias, hepatic or neurological manifestations. NL can be diagnosed intra-uterus or in neonatal period, being self-limiting in several months or be irreversible. Congenital heart block (CHB) in a structurally normal heart, is perhaps the most serious manifestation with an estimated mortality rate of around 19%. Objetives: to estimate the frequency of NL in children of mothers with anti-Ro/La in reference centers in the management of pregnancy and autoimmune diseases in Argentina, and to describe maternal and children features. Methods A descriptive multicenter study was conducted in reference centers in the management of autoimmune diseases and pregnancy in Argentina. Inclusion criteria were the presence of positive maternal serology anti-Ro/La and at least one pregnancy. Demographic and maternal-fetal clinical data were obtained from the clinical histories and each center completed a data collection form created for this study. We defined a NL case (born or not) who presented, pre and/or postpartum, characteristic skin lesions, cytopenias, cardiac involvement (CHB, endocardial fibroelastosis and dilated cardiomyopathy), hepatic or neurological manifestations. Ethnicity was classified using GLADEL groups. NL frequency was calculated dividing the number NL cases by the number of mothers with positive anti-Ro/La serology. Results 18 reference centers in the management of autoimmune diseases and pregnancy participated in this study in 7 different geographic areas of Argentina (6 of Buenos Aires, 6 of Cordoba, 1 of Jujuy, 1 of Mendoza, 1 of Santa Fe, 1 of Santiago del Estero and 2 of Tucuman). 193 mothers with positive anti-Ro/La serology were included with 364 pregnancies. 19 cases NL cases were reported (10 diagnosed during pregnancy and 9 in post-partum. The frequency of NL was estimated at 9.8% [95% CI 6.3–14.9] (CHB=6.2% [IC95% 3.5–10.7]). Table 1 describes the maternal and fetal characteristics of NL cases. The most frequent manifestations were skin (n=7) and cardiac involvement (n=12). In 1 case, there was a history of NL in a previous pregnancy. Of the patients with CHB, 5 required a pacemaker. Conclusions In conclusion, the frequency of NL in our multicentric cohort is greater than other international cohorts. Differences could be related to genetic/environmental factors as well as methodological limitations and selection bias. Funding Source(s): None

Background Rheumatoid factor (RF) in SLE is found in around 25% of patients. Authors associate it with cutaneous involvement, Sicca, anti Ro (+) and a protective role in glomerulonephritis (GMN), suggested by the finding of less frequent and less severe nephritis in lupus patients with RF(+) as in patients with RA. The proposed protective mechanisms of RF include preventing the complement from joining immune-complexes (IC) and avoiding the IC deposit in the glomerulus. We aimed to determine the clinical and immunological profile of SLE patients with and without RF and to determine the association between RF and GMN. Methods Descriptive, retrospective study of SLE (SLICC 2012) patients selected from Rheumatology department of Guemes Hospital, from January 2015-August 2018. We collected data on demographics, cutaneous-articular, renal, hematological and CNS involvement. Immunoserology: RF IgM by immunoturbidimetric (+)>30 UI/ml, anti Ro, anti La, anti Sm, anti RNP (ELISA), ANA (Hep 2), Anti DNA (Crithidia lucilliae), C3, C4, aPL at least one of the following: LI, anti 2GP1 IgG/IgM, Anti aCL IgG/IgM. Statistical Analysis: Epiinfo version 7.2.0.1. Results We reviewed 147 clinical histories, 107 with RF ordered. Female 93/107 (86.9%), mean age 41.6 years (SD ±13.8). Disease duration 98 months (SD ±80.6). Tobacco exposure 10/107 (9.35%). RF (+) 22/107 (20.5%), RF median titer 228.8 IU/ml (31–2825 IU/ml). RF level in patients with GMN was 142 IU/ml (39–191), without GMN 258 IU/ml (31–2825) p=0.46. RF level in patients with anti Ro (+) was 248 IU/ml (31–2825), Ro (-) 52.5 IU/ml (31–74) p=0.37. RF (-) subgroup showed statistically significant association with female sex, discoid lesions and aPL presence. RF(+) was associated with Ab Ro, La and patients with RA. (Table 1). Not found significant association with: rash, photosensivity, oral ulcer, joint pain, arthritis, serositis, neurologic and hematological involvement. In those with GMN, we found a significant association with Ab Ro(+) in the absence of RF (table 1). Conclusions Similar RF frequency was found as the published literature. There was no association with nephritis but the mean RF titer was higher in patients without GMN. The detection of Ab clusters associated with RF may be useful to suggest absence of renal involvement. Funding Source(s): None

Background In SLE there is five times more risk of cardiovascular events (CV) compared to the general population. In Argentina, a prevalence of metabolic syndrome (MetS) was reported in 28%. The SLICC cohort found 36.5% of MS in the first 2 years of diagnosis. The neutrophil to lymphocyte ratio (NLR) is a marker of inflammation that relates the absolute count of neutrophils and lymphocytes, is a tool in the assessment of CV risk and systemic endothelial dysfunction. The NLR values reported in healthy is 1.65 (±1.47). Studies in SLE show that NLR is a marker of activity and nephritis. We aimed to determine the association of MetS with and without renal involvement, the relationship between NLR and MetS and relationship between NLR and disease activity. Methods Descriptive, cross-sectional study. Patients with SLE (SLICC 2012) of 18 years followed at the Guemes Hospital were included, between 06/2013 to 07/2018. Acute CV events, infections, pregnancy, diabetes and chronic kidney disease were excluded. Risk factors nontraditional were determined: antiphospholipid (aPL), GC, SLEDAI. MetS (NCEP ATP III criteria): Weight, height, abdominal perimeter (AP) and blood pressure. PCR, neutrophil and lymphocyte count, glucose, CT, TG, LDL, urea, creatinine, proteinuria/24hs, Anti DNA, C3, C4, aPL: LA, Anti B2GP and Anti aCL antibody. Statistical Analysis: Epi Info 7.2.0. Results A total of 42 patients were reviewed, 12 were excluded (incomplete data). Of 30 patients: 23 women (77%), mean age 39.3 (±SD 14.3), evolution of disease: 35 months (±SD 18.9), tobacco exposure: 11 (37%). Nephritis in 17 patients (51%). MetS: 13 (43.3%), components: AP increased 15 (50%), HDL low 11 (37%), hypertension 11 (37%), high TG 8 (27%), hyperglycemia 3 (10%). Overweight: 17 (57%). Mean BMI 27.4 (±DS 6.2). Characteristics of patients with and without MetS and relationship MetS and SLE with NLR. (Table). Conclusions In our series, a greater frequency of MetS was found than literature. No relationship was found between MetS with renal involvement; however patients with MetS had higher proteinuria and elevated CRP. No relationship was found between the NLR with MetS and nephritis. The NLR was higher in patients with SLEDAI 4. The high prevalence of MetS, proteinuria, elevated CRP and NLR in our population suggest persistent inflammatory activity and probable CV morbidity, so it is important to detect it from the onset of the disease. Funding Source(s): None

Introduction No inherent renal lesions are known in rheumatoid arthritis (RA), but urinary abnormalities and renal dysfunction have been described. Objective First, we describe the histopathological findings of renal biopsies (RBs) in patients with RA and associated clinical manifestations. Second, we evaluated time evolution of RA and the relationship between drugs and renal disease. Last, we investigate whether changes in the management of RA from 1976 to 2015 influenced RBs indication, frequency, and type of histopathological findings. Patients and methods This is a retrospective and observational study conducted at a university hospital from Argentina. Patients with a diagnosis of RA (ACR, 1987) and RBs between 1976 and 2015 were included. Sixty-five patients met the inclusion criteria. The histopathological findings and associated clinical manifestations were evaluated. Time evolution of RA and the relationship between drugs and renal disease were also determined. To clarify these issues, we characterized 3 groups according to changes in the management of RA: 1976-1989, 1990-2002, and 2003-2015. Results The most common histopathological finding was renal amyloidosis in 31% (n = 20), followed by mesangial glomerulonephritis in 18% (n = 12), membranous nephropathy in 17% (n = 11), extracapillary proliferative glomerulonephritis in 15% (n = 10), focal segmental glomerular sclerosis in 9% (n = 6), minimal change nephropathy in 5% (n = 3), and tubulointerstitial nephritis in 5% (n = 3). Time evolution of renal amyloidosis was significantly higher than other RBs (15 ± 12 vs 7 ± 6.5 years). Nephrotic syndrome was the most common clinical manifestation (60%) followed by hematuria (46%) with or without proteinuria. Membranous nephropathy was related to the use of gold salts in 45% of cases, and its frequency decreased since 1990. Before 2003, renal amyloidosis was the leading cause of kidney disease, but mesangial glomerulonephritis reached the same frequency between 2003 and 2015. We found that RBs decreased 20% in the second period (1990-2002) and 40% in the last period (2003-2015). Nephrotic syndrome remained the main RB indication during the entire study period. Conclusion This is the first report on RBs findings in patients with RA from Latin America. We found a significant reduction in RBs frequency and modified histological patterns throughout the study period, although RB indication was not modified. Changes in the management of RA might have influenced these findings.

Objective: To investigate the prevalence and associations with clinical manifestations of anti- P ribosomal antibodies in patients with juvenile-onset and adult-onset systemic lupus erythematosus (SLE). Methods: Clinical and serological data of 30 patients with juvenile-onset SLE (age at onset younger than 16 years old) were compared with data of 92 patients with adult-onset SLE. Symptoms occurring during the entire disease course were considered. Anti- P ribosomal antibodies were tested by ELISA.

Objective To investigate the prevalence and associations with clinical manifestations of anti-P ribosomal antibodies in patients with juvenile-onset and adult-onset systemic lupus erythematosus (SLE). Methods Clinical and serological data of 30 patients with juvenile-onset SLE (age at onset younger than 16 years) were compared with data of 92 patients with adult-onset SLE. Symptoms occurring during the entire disease course were considered. Anti-P ribosomal antibodies were tested by ELISA. Results Anti-P ribosomal antibodies were found significantly more often in pediatric-onset SLE patients (26.7% vs 6.5%; OR=5.21 [95% CI=1.6–16.5], P =.003). Alopecia (OR=10.11, 95% CI=1.25–97) and skin rash (non-discoid) (OR=4.1, 95% CI=1.25–13.89) were significantly associated with anti-P ribosomal antibodies. Conclusion Anti-ribosomal P antibodies are more often found in patients with juvenile SLE. Alopecia and skin rash were the only clinical manifestations associated to anti-ribosomal P antibodies.

Diffuse alveolar haemorrhage (DAH) is a manifestation of several immune and nonimmune diseases.The objective of this study was to characterize the clinical characteristics and short-term outcomes of patients with immune-mediated DAH requiring hospital admission.A retrospective study from December 2010 to December 2015, was conducted by analyzing the clinical records of 39 patients with DAH with a proven immunological origin. The diagnosis of individual collagen vascular diseases (CVD) was made according to the criteria of the corresponding societies.Thirty-nine patients were included (median age 44.8 years, range 16-76). The main causes of DAH were ANCA-related vasculitis (74.3%) mainly granulomatosis with polyangiitis (n = 14) and microscopic polyangiitis (n = 13). Thirty patients (76.9%) had hemoptysis. An alveolar airspace filling pattern was found in most of patients (59%). All the patients had a drop in hemoglobin level that ranged from 1.0 to 3.0 g/dL. BAL fluid was macroscopically bloody in 43.6% of patients (n = 17) and showed siderophagic alveolitis on BAL cytology in 100%. All patients received high doses of corticosteroids. Other additional treatments were antibiotics (53,8%, n = 21), intravenous cyclophosphamide (87.2%, n = 34), plasma exchange (35.9%, n = 14); intravenous immunoglobulin (12.8%, n = 5) and rituximab in 5 patients (12.8%). Mortality rate was higher amongst patients who required dialysis (50.0 vs 15.4%, p = 0.045), with SaO290% at admission (50.0 vs 5.3%, p = 0.003) or those who required mechanical ventilation (76.9 vs 6.8%, p = 0.001).DAH may present without hemoptysis and requires an early bronchoscopy to confirm the diagnosis and exclude infection. Other characteristics could be included in the abstract which are relevant to the paper. (relation between mortality, dialysis, ventilation, etc.).

Introduction Several studies have shown that Hispanic patients generally experience a worse renal prognosis than non-Hispanic white patients with lupus. To date, there is no report on American College of Rheumatology (ACR) renal response criteria (ACR-RRC) in patients from Latin America. Objective To evaluate treatment response in patients with proliferative and membranous lupus nephritis (LN) according to ACR-RRC. Methods A retrospective study (2001–2011) was performed in our hospital and the data collected included clinical information, renal assessment and immunological parameters. Details related to treatment received during induction and maintenance therapy were also recorded. Results The study included forty-three Latin American patients (37 women) from Argentina. Mean follow-up was 54 months. The regimen used for induction therapy included intravenous cyclophosphamide (IV-CYC) for six months in 36 patients and only seven received mycophenolate mofetil (MMF) given twice daily for a total dose of 2.5 ± 0.5 grams a day. For the maintenance period, six patients continued with quarterly IV-CYC, 20 patients received MMF (1.5 ± 0.5 grams a day) and 17 patients received azathioprine (AZA) 1.5 – 2 mg/kg/day. ACR-RRC observed after induction therapy was complete response in 19%, partial response in 9%, improvement in 42%, unchanged in 14%, and 16% had deterioration despite treatment. ACR-RRC observed after maintenance therapy was complete response in 30%, partial response in 23%, improvement in 19%, unchanged in 16%, and 12% had deterioration. Relapse was observed in 11 patients (25%). Ten out of 11 cases (91%) who relapsed did not achieve a complete response at the end of the induction therapy. Conclusion In Latin American patients from Argentina, only 19% could achieve a complete response after induction therapy and 30% achieved a complete response after maintenance therapy. Failing to obtain a complete response after induction therapy was related to an increased risk of relapse during long-term follow-up in our study.

Introduction Infections are the leading cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). Invasive fungal infections (IFI) comprise a group of diseases caused by Cryptococcus, Histoplasma, Aspergillus and Candida. Few studies of IFI have been published in patients with SLE and associated factors have not been completely defined. Objectives The objectives of this paper are to estimate the frequency of IFI in admitted patients with SLE in our hospital, to determine the risk factors associated with IFI in our patients with SLE, and to compare IFI group with a control group (SLE without IFI). Methods The medical charts of patients with IFI (EORTC/MSG, 2008) and SLE (ACR, 1997) admitted to our hospital from June 2001 until June 2012 were reviewed. To identify factors associated with IFI, we developed a case-control study (SLE + IFI vs SLE alone) in a one to three ratio adjusted for sex and age and hospitalization for other reasons. Comparison was made of demographic characteristics, duration of disease and disease activity previous to IFI diagnosis, especially three months before fungal infection. We defined severe activity as SLEDAI ≥ 8. Infection by fungi of the genus Candida was considered only in its disseminated form. Results Ten cases of IFI were identified in 208 patients with SLE admitted between June 2001 and June 2012. We included 40 patients with SLE (10 with IFI and 30 controls). Of the SLE-IFI patients, eight were women and the average age was 27.5 years (range, 19–42 years). Fungal isolation: eight Cryptococcus neoformans, one Histoplasma capsulatum and one Candida albicans. Sites affected: five in peripheral blood, five in central nervous system (CNS), four in skin/soft tissue and one in pleura. Mortality was 40% ( p = 0.002), with Cryptococcus neoformans being the most common fungus. The SLE disease activity was severe in 70% of infected patients and no significant difference with the control group was found ( p = 0.195). We also found no association with leukopenia, lymphopenia, hypocomplementemia, hypogammaglobulinemia or anti-DNA positivity; neither with meprednisone doses >20 mg/day or intravenous methylprednisolone pulse therapy before fungal infection. The use of immunosuppressive therapy with azathioprine showed a significant association ( p = 0.017). Cyclophosphamide ( p = 0.100) or mycophenolate mofetil ( p = 0.256) did not show similar results. Conclusion The frequency of IFI in hospitalized SLE patients in our hospital was 4.8%. Cryptococcus neoformans was the most common etiologic agent and was primarily responsible for the deaths in this cohort. These data are consistent with publications in East Asia rather than North America where Candida spp. is more common. Unlike other publications, previous immunosuppression with azathioprine was the only risk factor associated with the development of the infection. Invasive fungal infection should be suspected in hospitalized patients with SLE and immunosuppression with CNS or atypical cutaneous manifestation of SLE in order to start appropriate treatment early and obtain better outcome.

Background ACR and EULAR guidelines recommend performing renal biopsy in every SLE patient with proteinuria above 0,5g/24h. Some patients show an active urine analysis with dysmorphic erythrocytes but proteinuria Objectives To analyzed clinical manifestations, SLEDAI and histological classes found in patients with SLE and proteinuria Methods Retrospective study of 33 adult patients with SLE (ACR 1997) and proteinuria We analyzed clinical manifestations and SLEDAI at the time of renal biopsy. All renal samples were studied by light microscopy and immunofluorescence and were classified according to the ISN RPS 2003. Data were analyzed using SPSS 17.0 program. Results There were 25 women (76%) and 8 men (24%), average age of 34 years (19-65) IQR 17. Median of disease: 120 months (36-264) IQR 96. Cutaneous involvement was found in 28 patients (84.8%), arthritis in 21 (63.6%), hematological features in 17 (51.5%), serositis in 9 (27.3%), and no patient had neurological criteria. Anti DNA was positive in 24 patients (72.7%) and hypocomplementemia 20 (60.6%). Mean SLEDAI was 15 (DS 6.06). Conclusions We found high prevalence of proliferative forms in SLE patients with low proteinuria ( References George K Bertsias et al, Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis, Ann Rheum Dis 2012;71:1771–1782. Hahn BH et al, American College of Rheumatology guidelines for screening, treatment, and management of lupus nephritis, Arthritis Care Res 2012 Jun; 64(6):797-808. Sociedad Argentina de Reumatologia y Sociedad Argentina de Nefrologia, Primer Consenso Argentino sobre Diagnostico y Tratamiento de la Nefropatia Lupica, Rev Arg Reumatol. 2011; 22 (4): 06-37. Disclosure of Interest : None Declared