Management of non-renal non-neurologic persistent lupus activity in real world patients from Argentina.

Management of systemic lupus erythematosus patients is challenging because of disease heterogeneity. Although treatment of renal nephritis is more standardized, treating non-renal lupus activity remains controversial. Our objective was to identify non-renal, non-neurologic persistent active systemic lupus erythematosus patients in our cohort and described therapeutic behaviors in them. All systemic lupus erythematosus patients (American College of Rheumatology and/or Systemic Lupus Erythematosus International Collaborating Clinics criteria) seen at a university hospital between 2000 and 2017 were included and electronic medical records manually reviewed. Persistent lupus activity was defined as a patient with a Systemic Lupus Erythematosus Disease Activity Index score ≥ 6 (without renal and central nervous system manifestations) despite being on a stable treatment regimen for ≥ 30 days. Stable treatment could include prednisone alone (7.5-40 mg/d) or combined with antimalarial drugs and immunosuppressant therapies. A total of 257 lupus patients were included, 230 females (89.5%, 95% confidence interval 85.1-92.7), mean age at diagnosis 29.9 years (SD 16.4). After a median cohort follow-up of 5.7 years (interquartile range 2.4-10.2), 14 patients (5.4%, 95% confidence interval 3.2-9.0) showed persistent non-renal non neurologic lupus activity, with a median disease duration of 11.3 years (interquartile range 3.6-19.4). At that time, 12/14 (85.7 %, 95% confidence interval 52.6-97.0%) had low complement and 11/14 (78.6 %, 95% confidence interval 46.5-93.9%) had positive antiDNA antibodies. The main reasons for being refractory were mucocutaneous disease (50%, 95% confidence interval 23.5-76.5) and arthritis (42.9%, 95% confidence interval 18.5-71.2). Therapeutic choices after being refractory were: only increasing corticosteroid dose in one patient, starting rituximab in four, belimumab in eight, and in one mycophenolate and rituximab; with good response in all of them. In conclusion, 5.4% of systemic lupus erythematosus patients in our cohort were considered to have non-renal non neurologic persistent lupus activity, with mucocutaneous and arthritis the main manifestations. In total, 92.8% of these patients started a biologic treatment at this point (rituximab or belimumab).

Remission criteria and activity indices in psoriatic arthritis.

Remission criteria and activity indices used in rheumatoid arthritis (RA) are often applied in psoriatic arthritis (PsA). Some new indices have been specifically developed for PsA. Our objective was to evaluate the performance of different remission criteria and activity indices in PsA. This is a cross-sectional study that includes consecutive patients with PsA. Information necessary to complete the following indices was captured: Composite Psoriatic Disease Activity Index (CPDAI), Psoriatic Arthritis Screening and Evaluation (PASE), Disease Activity Index for Psoriatic Arthritis (DAPSA), Disease Activity Score in 28 Joints (DAS28), Simplified Disease Activity Index (SDAI), Clinical Disease Activity Index (CDAI), and American College of Rheumatology and European League Against Rheumatism (ACR/EULAR) Boolean RA remission criteria. Patients were classified according to activity categories (remission, low, medium, or high disease activity). Correlation between indices was established. Fifty-five patients were included. Mean age was 53 years (SD = 12), and 35 (63.6 %) were males. Mean PsA disease duration was 5.9 years (SD = 8.5), and mean psoriasis duration was 15.9 (SD = 12.6). We found important differences in the percentage of patients classified as in remission by applying different remission criteria: DAS28 = 33 % (95 % confidence interval (CI) 20-45) vs ACR/EULAR = 4 % (95 % CI 1-17). Particularly, DAS28 and minimal disease activity seemed to be less stringent in PsA than the other indices. Of the specific PsA indices evaluated, CPDAI showed the poorest correlation with all the other activity measurements, although differences were not statistically significant in most cases. Disease activity in PsA is measured by many different indices. In spite they all showed good correlations between them, they classified different patients in different disease status.

Prevalencia de la enfermedad de Pompe entre pacientes con elevación de creatinin-kinasa (CK) / Prevalence of Pompe diseases among patients with elevated phosphocreatine kinase

Introducción: La prevalencia exacta de la enfermedad de Pompe en lapoblación general es desconocida, y la frecuencia estimada varía entrelas diferentes formas clínicas y grupos étnicos. Existe un gran númerode pacientes con elevación de enzimas musculares que no tienen undiagnóstico definitivo. Un porcentaje de ese grupo de pacientes podríatener enfermedad de Pompe. Objetivos: 1) Determinar la prevalencia de la enfermedad de Pompeentre pacientes mayores de un año de edad, con elevación persistente de creatin-kinasa (CK) de causa desconocida. 2) Describir las manifestaciones clínicas y demográficas de los pacientes con CPK elevada. Materiales y métodos: Se incluyeron a todos los pacientes con aumentode CK en sangre, definida como la detección de al menos un valor >500 UI/L y otro mayor de 200 UI/L, durante un período mínimode 1 año, entre los años 2010-2013, tomados de la base de datos dellaboratorio del Hospital Italiano de Buenos Aires. Se excluyeron a lospacientes bajo tratamiento actual con agentes hipolipemiantes; pacientestratados con agentes hipolipemiantes, en quienes la CK no senormalizó luego de suspender el tratamiento; pacientes tratados conagentes hipolipemiantes que interrumpieron el tratamiento durante untiempo menor de 9 meses antes del período de inclusión y pacientescon enfermedad muscular inflamatoria (pacientes con criterios diagnósticos probables o definidos de Bohan & Peter). Los pacientes condiagnóstico probable de miositis por cuerpo de inclusión (biopsia notípica) podían ser incluidos. Se les realizó evaluación clínica (mediciónde la fuerza muscular), cuestionario sobre síntomas musculares y se lesextrajo sangre para test enzimático en papel de filtro para enfermedadde Pompe.

Introduction: The exact prevalence of Pompe disease in the generalpopulation is unknown, and the estimated frequency varies among differentethnic groups and clinical forms. A large number of patients withelevated muscle enzymes do not have a definitive diagnosis. A significant percentage of these patients may have a Pompe disease. Objectives: To determine the prevalence of Pompe disease among patients with persistently elevated CK (over one year) of unknown cause.To describe the demographic and clinical manifestations of the patients with elevated CK. Patients and methods: We included all patients with increased bloodCK, defined as a value >500 IU/L and another greater than 200 IU/L forat least 1 year between 2010 and 2013. Patients were selected fromthe database of the laboratory of the Italian Hospital of Buenos Aires. Weexcluded patients under current treatment with lipid-lowering agents; patients who have been treated with lipid-lowering agents, in whom CPK has not normalized after discontinuation of therapy; patients whohave been treated with lipid-lowering agents and discontinued for atime less than 9 months before the inclusion period and patients with inflammatory muscle disease: patients with probable or definite criteriadiagnoses (Bohan & Peter criteria). Patients with a diagnosis of probableinclusion body myositis (not typical biopsy) were included. Patients underwent clinical evaluation (measurement of muscle strength), muscle symptoms questionnaire and had blood taken for enzyme test on filter paper for Pompe disease.