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Resumen Se describe el caso de un paciente varon de 52 anos que consulta al servicio de reumatologia por presentar adenopatias indoloras en las regiones cervical, axilar, supraclaviculares y en la nuca, asi como fiebre, aumento de tamano de parotidas y submaxilares. Se realizan estudios complementarios que arrojan como resultado anemia normocitica-normocromica, trombocitopenia y eosinofilia, alteracion de la funcion renal con hipoalbuminemia y hematuria, FAN 1/5.120, Sm+, ACL+ y biopsia de los tejidos comprometidos, por lo que se arriba al diagnostico de enfermedad de Rosai-Dorfman y enfermedad relacionada con IgG4. Se discuten diagnosticos diferenciales de adenopatias cervicales, axilares e inguinales, fiebre, compromiso renal y hematologico.

Objective The objective is to describe the main characteristics of patients with systemic lupus erythematosus (SLE) in Argentina and to examine the influence of ethnicity on the expression of the disease. Patients and methods RELESSAR is a multicentre register carried out by 106 researchers from 67 rheumatologic Argentine centres. It is a cross-sectional study of SLE (1982/1997 ACR) patients. RELESSAR electronic database includes demographic, cumulative SLE manifestations, SELENA-SLEDAI, SLICC-SDI, Katz’s severity and Charlson’s comorbidity indexes and treatment patterns. Results We included 1,610 patients, 91.7% were female with a median age at diagnosis of 28.1 ± 12.8; 96.2% met ≥4 ACR 1982/97 criteria. Frequent manifestations were arthritis (83.5%), malar rash (79.5%), photosensitivity (75.3%), haematological (63.8%) and renal disease (47.4%), antinuclear antibodies (96%), anti-dsDNA (66.5%) and anti-Smith antibodies (29%). The mean Selena-SLEDAI score at last visit was 3.18 (SD 4.3) and mean SDI was 1 (SD 1.3). The accumulated treatments most frequently used were antimalarials (90.4%), corticosteroids (90%), azathioprine (31.8%), intravenous cyclophosphamide (30.2%), mycophenolate mofetil or mycophenolic acid (24.5%), methotrexate (19.3%), belimumab 5.3% and rituximab 5.1%. Refractory lupus was diagnosed in 9.3% of the cases. The main causes of death were lupus activity (25.0%), activity and concomitant infections (25.0%), infections (18.2%), vascular disease (13.6%) and cancer (4.5%). Mortality was associated with higher SLEDAI, Katz, damage indexes and comorbidities. Of the 1610 patients included, 44.6% were Caucasian, 44.5% Mestizo, 8.1% Amerindian and 1.2% Afro-Latin American. Mestizo patients had higher male representation, low socioeconomic status, more inadequate medical coverage, fewer formal years of education and shorter disease duration. Polyadenopathies and Raynaud’s phenomenon were more frequent in Caucasians. In the logistic regression analysis higher damage index (OR 1.28, CI 95% 1.02–1.61, p = 0.03) remained associated to mestizo ethnicity. Conclusions This study represents the largest number of adult patients with SLE studied in Argentina. Caucasian patients were differentiated by having Raynaud’s phenomenon and polyadenopathy more frequently, while patients of Mestizo origin had higher damage indexes.

Cocaine use is associated with several rheumatic syndromes. A series of cases is presented of 10 patients (7 male and 3 female), age at onset: 19–38 years-old. They consulted due to a variety of symptoms: fever, arthritis, swollen hands, stroke, fetal losses, arterial hypertension, alveolar hemorrhage, leucocytoclastic vasculitis, genital bleeding, among others. Half of them showed positive ANA and Ro antibodies, lymphopenia and positive P-ANCA. Treatments with steroids in bolus, cyclophosphamide and rituximab were used, with a good response. This series highlights clinical manifestations at onset that can mimic primary rheumatic diseases. Rheumatologists should consider cocaine use in the differential diagnoses of vasculitis and pseudovasculitis syndromes.

Cocaine use is associated with several rheumatic syndromes. A series of cases is presented of 10 patients (7 male and 3 female), age at onset: 19-38 years-old. They consulted due to a variety of symptoms: fever, arthritis, swollen hands, stroke, fetal losses, arterial hypertension, alveolar hemorrhage, leucocytoclastic vasculitis, genital bleeding, among others. Half of them showed positive ANA and Ro antibodies, lymphopenia and positive P-ANCA. Treatments with steroids in bolus, cyclophosphamide and rituximab were used, with a good response. This series highlights clinical manifestations at onset that can mimic primary rheumatic diseases. Rheumatologists should consider cocaine use in the differential diagnoses of vasculitis and pseudovasculitis syndromes. RESUMEN El uso de cocaína se asocia con varios síndromes reumáticos. Se trata de 10 pacientes (7 varones y 3 mujeres) con una edad al inicio del cuadro entre 19-38 anos, quienes consultaron por una variedad de síntomas: fiebre, artritis, tumefacción de manos, accidente isquémico transitorio, pérdidas fetales, hipertensión arterial, hemorragia alveolar, vasculitis leucoci-toclástica y hemorragia genital, entre otros. La mitad de estos pacientes presentaron ANA y anticuerpo anti-Ro positivos, linfopenia y p-ANCA positivo. Fueron tratados con corticoides intravenosos en bolo, ciclofosfamida y rituximab, con buena respuesta. Esta serie resalta las manifestaciones clínicas que, al inicio del cuadro, pueden imitar una enfermedad reumática primaria. Los reumatólogos debieran considerar a la cocaína en el diagnóstico diferencial de las vasculitis y los síndromes seudovasculíticos.

BackgroundThe goal of targeted treatment in patients with Systemic Lupus Erythematosus (SLE) is to achieve clinical remission or low disease activity, with the best quality of life, low damage rates and better survival 1-4. RELESSAR is a multicenter, cross-sectional study registry of ≥18 years SLE (ACR 97) patients 5.ObjectivesTo describe demographic, clinical characteristics and treatments in SLE patients according to disease activity state. To evaluate the proportion of SLE and refractory SLE patients that are potentially candidates for Belimumab treatment (Active SLE despite standard treatment including increased acDNA autoantibodies and low complement).MethodsWe evaluated demographic and clinical data, treatments, score of damage (SLICC), activity (SLEDAI) and comorbidity (Charlson), hospital admissions and severe infections. The patients were compared according to disease activity: remission (SLEDAI = 0 and without corticosteroids), low disease activity (LDA, SLEDAI> 0 and ≤4 and without corticosteroids) and non-optimal control (SLEDAI> 4 and any dose of corticosteroids). Refractory SLE was defined according to Rituximab (RTX) use, non-response to cyclophosphamide or two or more immunosuppressant or splenectomized patients. Potential use of Belimumab according approved prescription in Argentina was analyzed.ResultsOverall, 1277 patients were analyzed: 299 (23.4%) were in remission, 162 (12.7%) in LDA and 816 (63.9%) with non-optimal control of the disease.Patients in non-optimal control group were younger, less frequently female and they showed less time of disease and lower socioeconomic status (p < 0.001). They were also more prevalent mestizos (p= 0.004), had higher SLEDAI and SLICC indexes (p Two hundred and one SLE patients fulfilled the use of Belimumab prescription criteria but only 45/201 patients (22,3%) received it in the last visit. Malar rash was the only clinical variable associated with the use of Belimumab (72.7% vs 29.8% p= 0.005).Seventy-six patients classified as refractory SLE (15.7%) and 56/76 (75.7%) never received Belimumab. Patients on Belimumab therapy were associated to treatment with lower doses of corticoids (p= 0.018) and lower rate of hospital admission caused by SLE flare (p= 0.027).ConclusionA high percentage of patients had uncontrolled disease upon entry into the registry and were potential candidates for treatment with Belimumab. The patients who received biologic treatment showed the benefit of requiring fewer doses of corticosteroids and having a lower rate of hospitalizations.References[1]Mok CC. Treat-to-target in systemic lupus erythematosus: Are we there yet? Expert Rev Clin Pharmacol. 2016;9(5).[2]Morand EF, Mosca M. Treat to target, remission and low disease activity in SLE. Vol. 31, Best Practice and Research: Clinical Rheumatology. 2017.[3]Golder V, Tsang-A-Sjoe MWP. Treatment targets in SLE: Remission and low disease activity state. Rheumatol (United Kingdom). 2020;59.[4]Ruiz-Irastorza G, Bertsias G. Treating systemic lupus erythematosus in the 21st century: new drugs and new perspectives on old drugs. Vol. 59, Rheumatology (United Kingdom). 2021.[5]Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum [Internet]. 1997;40(9):1725. Available from: http://www.ncbi.nlm.nih.gov/pubmed/9324032Disclosure of InterestsRosana Quintana: None declared, Lucila Garcia: None declared, Paula Alba: None declared, Susana Roverano: None declared, Analia Alvarez: None declared, Cesar Graf: None declared, Cecilia Pisoni: None declared, Alberto Spindler: None declared, Catalina Gomez: None declared, Heber Matias Figueredo: None declared, Silvia Papasidero: None declared, Raul Horacio Paniego: None declared, Maria DeLaVega: None declared, Emma Estela Civit De Garignani: None declared, Luciana Gonzalez Lucero: None declared, Victoria Martire: None declared, Rodrigo Águila Maldonado: None declared, Sergio Gordon: None declared, Carla Gobbi: None declared, Romina Nieto: None declared, Gretel Rausch: None declared, Vanina Góngora: None declared, Maria Agustina D´Amico: None declared, Diana Dubinsky: None declared, Alberto Omar Orden: None declared, Johana Zacariaz: None declared, Julia Romero: None declared, Mariana Alejandra Pera: None declared, Oscar Rillo: None declared, Roberto Baez: None declared, Valeria Arturi: None declared, Andrea Gonzalez: None declared, Florencia Vivero: None declared, Marcela Schmid: None declared, Victor Caputo: None declared, Maria Silvia Larroude: None declared, Graciela Gomez: None declared, Graciela Rodriguez: None declared, Josefina Marin: None declared, Maria Victoria Collado: None declared, Marisa Jorfen: None declared, Zaida Bedran: None declared, Judith Sarano: None declared, David Zelaya: None declared, MONICA SACNUN: None declared, Pablo Finucci: None declared, Romina Rojas Tessel: None declared, Maria Emilia Sattler: None declared, MAXIMILIANO MACHADO ESCOBAR: None declared, Pablo Astesana: None declared, Ursula Vanesa Paris: None declared, Alberto Allievi: None declared, Juan Manuel Vandale: None declared, Bernardo Pons-Estel: None declared, Guillermo Pons-Estel: None declared, Mercedes García Grant/research support from: GSK grant

La enfermedad de Erdheim-Chester (EEC) es una histiocitosis de células no Langerhans de presentación proteiforme y escaso conocimiento. Se presenta una serie de 19 casos de 4 centros, registrados de junio de 2012 a junio de 2019. Se incluyeron aquellos pacientes con clínica, anatomía patológica e inmunohistoquímica característica de la enfermedad. Se excluyeron pacientes con hallazgos indefinidos. Resultados: al igual que en la literatura, el compromiso más frecuente fue el óseo por imagenología, la mitad de estos sintomático. Nuestra serie presenta mayor incidencia de mujeres (casi 70%). Siete pacientes presentaron nódulo mamario como forma de presentación. La mayoría recibieron dosis media de esteroides asociado a otra droga inmunosupresora. La mortalidad fue del 16%. Conclusión: comunicamos una serie de pacientes con la EEC mencionando las características más destacables. Es llamativo el número de pacientes con afectación mamaria, por lo cual proponemos téngase en cuenta en el diagnóstico diferencial de la patología tumoral mamaria. Erdheim-Chester disease (ECD) is a non-Langerhans histiocytosis, protein manifestations at start and little known. We included 19 patients from June 2012 to June of 2019. Inclusion criteria: clinical features, histopathology and immunostaining compatible with ECD. We excluded patients with undefined features. Results: Bones were the most frequent affected, half of them were asymptomatic. Seventy per cent of the patients were women, and 7 of them developed a nodule breast as first manifestation of ECD. The patients were treated with corticosteroids associated or not with immunosuppressants. The mortality rate was 16%. Conclusion: We reported a series of patients with ECD, enhancing the most frequent features. It is striking the number of patients with breast involvement; we propose to include the Erdheim-Chester disease in differential diagnosis of breast tumor.

Pulmonary haemorrhage (PH) in systemic lupus erythematosus (SLE) is a rare but potentially fatal complication due to its high mortality. Early treatment benefits the outcome. Reports on predictive factors of PH in SLE patients are scarce.To describe a case series of PH in SLE patients that were attended in the Rheumatology Section of the J. M. Cullen Hospital and to compare this data with published results.Patients with SLE (1982-1997 ACR criteria) and PH diagnosed by clinical criteria (cough, dyspnoea, haemoptysis), haemoglobin below 12 g/dL or drop greater than 2 points, new radiological infiltrate and bronchioalveolar lavage, monitored between June 1987 and December 2019 were studied. Demographic, clinical, laboratory, treatment and prognosis data related to PH were analysed.From a database of 306 SLE patients, 25 (8.2%) developed 29 episodes of PH. PH was the first manifestation of SLE in 8 patients. Renal involvement was the most frequent manifestation prior to the development of PH. SLE activity (measured by SLEDAI) was high during the episodes (mean: 16.8). Renal failure (p = 0.027) and mechanical respiratory support (p = 0.006) were related to mortality (40.7%) with statistical significance. Patients with SLEDAI higher to 10 at SLE onset showed more likelihood of developing PH. The OR was 2.68 (p = 0.046).Although treatment in SLE has progressed in recent years, PH continues to be a rare and severe complication of this disease. When a PH is suspected, studies to confirm it must be done rapidly, since early diagnosis and aggressive treatment have been shown to improve survival. We observed that patients with renal involvement and mechanical respiratory support had higher mortality than SLE patients without them.

Resumen Varon de 47 anos que consulta por perdida de peso, dolor con tumefaccion en pantorrillas, fiebre, hipertension arterial, orquitis y oligoartritis. Laboratorio: anemia y aumento de enzimas musculares. Resonancia magnetica: hiperintensidad en gemelos (miositis). Histologia de musculo: infiltrado inflamatorio con atrofia y regeneracion perifascicular. Tratamiento: pulsos de metilprednisolona y ciclofosfamida. Mialgias, tumefaccion muscular y deambulacion dificultosa son hallazgos comunes en poliarteritis nodosa (PAN), no asi la miositis demostrada histologicamente y mas infrecuente aun como forma de inicio de esta vasculitis.

Objective The aim of this study was to measure presenteeism (productivity impairment while the patient is at work) and the related risk factors in patients with systemic lupus erythematosus (SLE) from Argentina. Methods A total of 130 consecutive (1997 American College of Rheumatology (ACR) criteria) working patients with SLE were assessed using a standardized data collection form. Sociodemographic, disease and work-related variables were collected. The Work Productivity and Activity Impairment (WPAI) questionnaire was performed. Results Overall, 130 patients were included in the analysis; 91% were women, and the mean age was 39 years (range 19–77). A total of 43% were White, 43% Mestizo and 13% Amerindian. Overall, 38% were single and 38% were married. A total of 75% had more than 12 years of formal education. The median disease duration was 7 years (interquartile range 25–75 (IQR) 4–13). Median Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score was 0 (IQR 0–2), and median Systemic Lupus International Collaborating Clinics/ACR Damage Index (SLICC-SDI) score was 0 (IQR 0–1). Lupus quality of life (LupusQoL) domains scores were: physical health 87 (IQR 70–96), emotional health 78 (IQR 54–91), burden to others 75 (IQR 50–92), intimate relationships 87 (IQR 50–100), and body image 85 (IQR 70–100). Absenteeism was 8%, presenteeism was 19%, and overall work impairment (absenteeism + presenteeism) was 26%. In the multiple regression analysis, considering presenteeism as dependent variable, (adjusting by age, disease duration, >12 years of education, Non-white race, Visual Analogue Scale (VAS) pain, VAS fatigue, SLICC-SDI, LupusQoL, physical and emotional domains), we found that SLICC-SDI (odds ratio (OR) 1.68, confidence interval (CI) 1–2.7) and Non-white race (OR 3.27, CI 1.04–10) were related to presenteeism and >12 years of education (OR 0.30, CI 0.09–0.98) and higher scores of LupusQoL emotional health domain (OR 0.95, CI 0.92–0.98) were protective. Conclusions organ damage and Non-white race were significantly associated with presenteeism while >12 years of education and higher scores of LupusQoL emotional health domain were protective.