Active lupus in Argentina: Results of a multicenter and national registry.

OBJECTIVE: To evaluate the association between patients' characteristics and disease activity in an Argentine lupus registry. METHODS: Cross-sectional study. Disease activity was stratified into: Remission off-treatment: SLEDAI = 0, without prednisone and immunosuppressive drugs. Low disease activity Toronto Cohort (LDA-TC): SLEDAI ≤2, without prednisone or immunosuppressive drugs. Modified lupus low disease activity (mLLDAS): SLEDAI score of ≤4, with no activity in major organ systems and no new features, prednisone of ≤10 mg/day and/or immunosuppressive drugs (maintenance dose) and Active disease: SLEDAI score of >4 and prednisone >10 mg/day and immunosuppressive drugs. A descriptive analysis and logistic regression model were performed. RESULTS: A total of 1346 patients were included. Of them, 1.6% achieved remission off steroids, 0.8% LDA-TC, 12.1% mLLDAS and the remaining 85.4% had active disease. Active disease was associated with younger age (p ≤ 0.001), a shorter time to diagnosis (p ≤ 0.001), higher frequency of hospitalizations (p ≤ 0.001), seizures (p = 0.022), serosal disease (p ≤ 0.001), nephritis (p ≤ 0.001), higher SDI (p ≤ 0.001), greater use of immunosuppressive therapies and higher doses of prednisone compared to those on mLLDAS. In the multivariable analysis, the variables associated with active disease were the presence of pleuritis (OR 2.1, 95% CI 1.2-3.9; p = 0.007), persistent proteinuria (OR 2.5, 95% CI 1.2-5.5; p ≤ 0.011), nephritis (OR 2.5, 95% CI 1.2-5.6; p = .018) and hospitalizations (OR 8.9, 95% CI 5.3-16.0; p ≤ 0.001) whereas age at entry into the registry was negatively associated with it (OR 0.9, 95% CI 0.9-1.0; p = 0.029). CONCLUSION: Active disease was associated with shorter time to diagnosis, worse outcomes (SDI and hospitalizations) and renal, neurological and serosal disease.

Multicenter lupus register from Argentina, the RELESSAR database: Influence of ethnicity on disease phenotype.

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.

Ultrasound nail imaging on patients with psoriasis and psoriatic arthritis compared with rheumatoid arthritis and control subjects.

OBJECTIVE: The objective of this study was too show findings at finger nails level revealed by high-frequency gray-scale ultrasound (US) and power Doppler in patients with psoriatic arthritis (PsA),and cutaneous psoriasis compared with rheumatoid arthritis and control subjects. METHODS: We studied 35 patients with PsA, 20 with cutaneous psoriasis, and control groups (28 control subjects and 27 patients with rheumatoid arthritis). All nails of both hands were observed (1097 nails, 3 excluded because of trauma). In every patient, we classified the morphologic abnormalities disclosed in ventral and dorsal nail plates. We also measured the distance between ventral plate and the bone margin of the distal phalanx at the right index finger. RESULTS: All patients and control subjects presented abnormalities in the US imaging. Those with psoriatic arthritis and cutaneous psoriasis showed a higher number of compromised nails. When classifying those abnormalities using the typology of Wortsman et al, patients with psoriatic arthritis showed loosening of the borders of the ventral plate (type II), whereas patients with cutaneous psoriasis showed focal hyperechoic involvement of the ventral plate without involvement of the dorsal plate (type I). Patients of the control group could not be classified, although 31 of 55 showed thinning of the ventral plate without hyperechoic deposits. Nineteen of 35 patients with psoriatic arthritis, and 10 of 20 patients with cutaneous psoriasis did not show any nail clinical alterations. Nevertheless, the US detected type II alterations in the first group and type I in the second group. Patients with psoriatic arthropathy had power Doppler increases in distal interphalangeal joints and nail beds in a statistically significant form (P = 0.0001).When measuring the distance between the ventral plate and the bone margin of the distal phalanx, there was homogeneity among samples in patients and control subjects. A receiver operating characteristic curve analysis determined that a cut point of 2 mm clearly defined these 2 groups. There was a significant difference (P < 0.0001) between the mean distance ventral plate-osseous margin of the distal phalanx in psoriatic arthritis patients (P = 0.001) and patients with cutaneous psoriasis (P = 0.005) versus rheumatoid arthritis patients (P = 0.548). CONCLUSIONS: As a predominant finding in our study, we observed abnormalities of the ventral plate in patients with PsA (type II) and abnormalities (type I) in patients with cutaneous psoriasis. We found a significant difference between the mean distance ventral plate-osseous margin of the distal phalanx in patients with PsA and patients with cutaneous psoriasis versus control subjects. Ultrasound imaging could be a feasible and sensitive tool to describe, measure, and follow morphologic characteristics and changes of the nail in psoriatic and/ or psoriatic arthritis patients with or without clinical nail lesions.

Diffuse alveolar haemorrhage in systemic lupus erythematosus patients.

INTRODUCTION: 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. OBJECTIVE: 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. METHODS: 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. RESULTS: 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 = .027) and mechanical respiratory support (p = .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 = .046). CONCLUSIONS: 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.

Calcification of the transverse ligament of the atlas in chondrocalcinosis.

OBJECTIVE: This study seek to establish the prevalence of calcification of the transverse ligament of the atlas (TLA) and of crowned dens syndrome (CDS) in patients with articular chondrocalcinosis. It also seeks to assess the main computed tomography appearances of these calcifications. PATIENTS AND METHODS: Twenty-eight patients with articular chondrocalcinosis were prospectively evaluated for evidence of calcifications of the transverse ligament of the atlas. A group of patients with gout, fibromyalgia, and osteoarthritis were used as control. RESULTS: Twenty-eight patients with articular chondrocalcinosis were prospectively evaluated for evidence of calcifications of the transverse ligament of the atlas. Twenty of the patients (71%) showed this calcification by computed tomography, disclosing 3 patterns of calcification: curvilinear in shape, thick, and forming a double band and mottled. Calcifications were not found in the controls (P < 0.0001). Nine patients were symptomatic (crowned dens syndrome). Patients with chondrocalcinosis and calcification of the transverse ligament of the atlas were older than the rest of the patients. Unlike the patient group, the majority of the patients in the control group were male (P = 0.02). CONCLUSION: The presence of calcifications of the transverse ligament of the atlas in patients with chondrocalcinosis occurs more frequently than that reported earlier. The CT scan is the most sensitive method to detect it. Taking into account that calcifications of TLA may manifest as CDS in a high percentage of these patients, such possibility should be considered in all patients with neck pain, stiffness, fever, and inflammatory response so as to avoid invasive diagnostic methods and more aggressive treatments than the use of nonsteroidal anti-inflammatory drugs.

Degos cutaneous disease with features of connective tissue disease.

A 30-year-old woman was referred on April 2002 for a plaque that involved the internal aspect of the right leg, an erythema nodosum-like lesion on the lower extremities, and periarthritis on her left ankle. Subsequently, the patient developed anular, atrophic, growing, porcelain-white papules, with a thin rim of erythema and telangiectases over her upper and lower extremities. Clinically and histologically, these lesions were the characteristics of Degos disease. Despite arthritis and myositis that required treatment, low level C3 and C4, positive antinuclear antibodies, and elevated anticardiolipin antibodies only once, in a follow-up of 6 years the patient never developed a specific connective tissue disease or other systemic involvement. In conclusion, because clinical and histological findings of Degos disease might mimic connective tissue diseases, rheumatologists must be aware that this reaction pattern can be seen in a wide clinical spectrum of diseases.

Diffuse Alveolar Haemorrhage in Systemic Lupus Erythematosus Patients. / Hemorragia alveolar difusa en pacientes con lupus eritematoso sistémico.

INTRODUCTION: 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. OBJECTIVE: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Myositis as the initial presentation of panarteritis nodosa. / Miositis como forma de presentación de panarteritis nodosa.

A 47-year-old man presented with weight loss, bilateral calf pain, fever, hypertension, orchitis and oligoarthritis. Lab tests: anemia and elevated muscle enzymes. Resonance magnetic imaging: hyperintensity in gastrocnemius muscles (myositis). Histologic exam of the muscles: inflammatory infiltrate with atrophy and perifascicular regeneration. Treatment: methylprednisone (bolus) and cyclophosphamide. Muscle pain and swelling and difficulty in walking are common in panarteritis nodosa (PAN), whereas histologically demonstrated myositis is not. Even more rare is myositis as the initial presentation of this vasculitis.

Can nailfold videocapillaroscopy images be interpreted reliably by different observers? Results of an inter-reader and intra-reader exercise among rheumatologists with different experience in this field.

Nailfold videocapillaroscopy (VCP) allows non-invasive assessment of the microcirculation. Adequate training in this field is relevant for rheumatologists. There is increasing evidence of the reliability of VCP findings among different readers. Objective: To evaluate inter- and intra-reader agreement of rheumatologists to identify normal images and systemic sclerosis (SSc) patterns on VCP ("early," "active," and "late" proposed by Cutolo et al.). Thirteen rheumatologists with different experience in nailfold VCP received training to standardize reading criteria. They rated 60 VCP images from healthy and SSc patients at baseline and 4 weeks later, using an electronic platform. The reading of an expert was considered the gold standard. Data were analyzed using Cohen's kappa for concordance and Student's t test and ANOVA to compare kappa means for inter-reader, intra-reader, and inter-pattern readings. Mean inter-reader and intra-reader kappa were 0.45 and 0.49, respectively, (moderate agreement). Kappa scores were higher among experienced vs inexperienced readers (inter-reader kappa 0.58 vs 0.34, p = 0.001, intra-reader kappa 0.65 vs 0.37, p = 0.01). Agreement was substantial (kappa = 0.61) for the identification of normal vs abnormal images and higher for the identification of active (0.48, p = 0.009) and late SSc patterns (0.56, p = 0.008) than for the early SSc pattern (0.35, p = 0.003). There is moderate agreement among rheumatologists for the identification of SSc videocapillaroscopy patterns (higher among experienced rheumatologists) and substantial agreement, regardless of previous experience in VCP, in the identification of normal and abnormal images. Agreement for the identification of active and late patterns is higher than for the early pattern.

Chronic intestinal pseudo-obstruction in patients with systemic lupus erythematosus: report of four cases.

Chronic intestinal pseudo-obstruction (CIPO), a recently recognized manifestation of systemic lupus erythematosus (SLE) with only 23 cases reported in the English literature, may appear as a complication or as the initial presentation of SLE and usually occurs during the setting of an active lupus. The pathogenic mechanism in SLE is unknown. We describe four additional cases with clinical, radiological, and manometric features of CIPO. As SLE-related CIPO usually responds to treatment with high doses of corticosteroids and/or immunosuppressive and prokinetic agents, a high level of awareness of this complication is needed to avoid unnecessary surgical intervention.

Frecuencia de eventos cardiovasculares en pacientes con lupus eritematoso sistémico / Frequency of cardiovascular events in patients with systemic lupus erythematosus

Resumen Introducción: el progreso en los tratamientos para el lupus eritematoso sistémico (LES) resultó en una disminución de la mortalidad; sin embargo, la enfermedad cardiovascular y las complicaciones infecciosas aún son las principales causas de muerte. La evidencia apoya la participación del sistema inmunológico en la generación de la placa aterosclerótica, así como su conexión con las enfermedades autoinmunes. Objetivos: describir la frecuencia de eventos cardiovasculares (ECV) en el Registro de Lupus Eritematoso Sistémico de la Sociedad Argentina de Reumatología (RELESSAR) transversal, así como sus principales factores de riesgo asociados. Materiales y métodos: estudio descriptivo y transversal para el cual se tomaron los pacientes ingresados en el registro RELESSAR transversal. Se describieron las variables sociodemográficas y clínicas, las comorbilidades, score de actividad y daño. ECV se definió como la presencia de al menos una de las siguientes patologías: enfermedad arterial periférica, cardiopatía isquémica o accidente cerebrovascular. El evento clasificado para el análisis fue aquel posterior al diagnóstico del LES. Se conformaron dos grupos macheados por edad y sexo 1:2. Resultados: 1515 pacientes mayores de 18 años participaron del registro. Se describieron 80 pacientes con ECV (5,3%). En este análisis se incluyeron 240 pacientes conformando dos grupos. La edad media fue de 47,8 (14,4) y 47,6 (14,2) en el grupo con y sin ECV respectivamente. Los pacientes con ECV tuvieron mayor duración del LES en meses, mayor índice de Charlson, mayor SLICC (Systemic Lupus International Collaborating Clinics/American College of Rheumatology), mayor frecuencia de manifestaciones neurológicas, síndrome antifosfolípido, hospitalizaciones y uso de ciclofosfamida. Las únicas variables asociadas en el análisis multivariado fueron el índice de Charlson (p=0,004) y el SLICC (p<0,001). Conclusiones: los ECV influyen significativamente en nuestros pacientes, y se asocian a mayor posibilidad de daño irreversible y comorbilidades.

Abstract Introduction: progress in treatments for systemic lupus erythematosus (SLE) has resulted in a decrease in mortality; however, cardiovascular and infectious diseases remain the leading causes of death. Evidence supports the involvement of the immune system in the generation of atherosclerotic plaque, as well as its connection to autoimmune diseases. Objectives: to describe the frequency of cardiovascular disease (CVD) in the cross-sectional RELESSAR registry, as well as its associated variables. Materials and methods: a descriptive and cross-sectional study was performed using patients admitted to the cross-sectional RELESSAR registry. Sociodemographic variables, clinical variables, comorbidities, activity and damage scores were described. CVD was defined as at least one of the following: peripheral arterial disease, ischemic heart disease, or cerebrovascular accident. All patients with at least one CVD were included in our analysis (heart attack, central nervous system vascular disease, and peripheral arteries atherosclerotic disease). The event classified for the analysis was that after the diagnosis of SLE. SLE diagnosis was previous to CVD. Two groups matched by age and sex, 1:2 were formed. Results: a total of 1515 patients older than 18 years participated in the registry. Eighty patients with CVD (5.3%) were described in the registry. Two-hundred and forty patients were included, according to two groups. The mean age was 47.8 (SD 14.4) and 47.6 (SD 14.2) in patients with and without CVD, respectively. Patients with CVD had a longer duration of SLE in months, a higher Charlson index, a higher SLICC, increased frequency of neurological manifestations, antiphospholipid syndrome, hospitalizations, and use of cyclophosphamide. The associated variables in the multivariate were the Charlson Index (p=0.004) and the SLICC (p<0.001). Conclusions: CVDs have a significant influence on our patients, being associated with a greater possibility of damage and comorbidities.

Pachydermodactyly: four additional cases.

Pachydermodactyly (PDD) is a rare, benign form of digital fibromatosis characterized by an asymptomatic soft tissue swelling affecting the skin of the lateral aspects of the proximal interphalangeal joints of the fingers, and it sometimes can be misdiagnosed with some rheumatic condition. Recognition on these features should lead to its more frequent diagnosis. The purpose of our study is to report four additional cases of PDD and discuss the differential diagnosis.

Accrual of organ damage over time in Argentine patients with systemic lupus erythematosus: a multi-centre study.

The goals of this study were to ascertain damage in patients with systemic lupus erythematosus (SLE) from five rheumatologic centres in Argentina and to examine overall damage, damage by domain and damage by item within each domain. We performed a retrospective observational study including patients with SLE (ACR 1997 revised and modified criteria) from five rheumatology centres in Argentina. Organ damage was scored using the SLICC/ACR DI (SDI), ascertained at years 1, 2, 5 and 10. Three centres provided information up to the fifth year. Of the 197 patients, 88.3% were women and their mean age was 33.2 years. The mean disease duration and follow-up were 7.6 and 5.3 years, respectively. Damage accrued gradually over time with SDI ranging from 0.52 (+/-1.1) at year 1 up to 2.46 (+/-2.1) at year 10. The renal system was the most involved system, followed by the neuropsychiatric, the cardiovascular and the musculoskeletal systems. Proteinuria, cognitive impairment, pericarditis, avascular necrosis, cataract and alopecia were the predominant items in their respective systems. Systems such as peripheral vascular, pulmonary, gastrointestinal, diabetes, malignancy and premature gonadal failure were not frequent. Overall SDI had a gradual increase over time. Damage in each domain of SDI, except for diabetes, had a similar behaviour. Behaviour of items in each domain varied.

Cervical lymph nodes in rheumatology: A diagnostic dilemma

The case is presented on a 52-year-old male patient, who was seen in the Rheumatology department. He had painless lymph nodes in the cervical, axillary, supraclavicular, and neck region. He also had a fever, and parotid and submaxillary gland enlargement. Complementary studies were performed, showing normocytic-normochromic anemia, thrombocytopenia and eosinophilia, impaired renal function with hypoalbuminaemia and hematuria, ANA 1/5120, Sm+, ACL+. Biopsies were also performed on the compromised tissues, reaching the diagnosis of Rosai-Dorfman Disease and IgG4-related Disease. Differential diagnoses of cervical, axillary and inguinal lymph nodes, with fever, renal and hematological compromise are discussed.

Se describe el caso de un paciente varón de 52 años que consulta al servicio de reumatología por presentar adenopatías indoloras en las regiones cervical, axilar, supraclaviculares y en la nuca, así como fiebre, aumento de tamaño de parótidas y submaxilares. Se realizan estudios complementarios que arrojan como resultado anemia normocítica-normocrómica, trombocitopenia y eosinofilia, alteración de la función renal con hipoalbuminemia y hematuria, FAN 1/5.120, Sm+, ACL+ y biopsia de los tejidos comprometidos, por lo que se arriba al diagnóstico de enfermedad de Rosai-Dorfman y enfermedad relacionada con IgG4. Se discuten diagnósticos diferenciales de adenopatías cervicales, axilares e inguinales, fiebre, compromiso renal y hematológico.

Lupus en Argentina. Pacientes no respondedores al tratamiento estándar y belimumab como posible opción. Datos del registro RELESSAR / Lupus in Argentina. Non-responders to standard treatment and belimumab as a possible option. RELESSAR record data

Introducción: el lupus es una enfermedad compleja y varias veces de difícil abordaje. Alcanzar la remisión es uno de los objetivos, incorporando opciones terapéuticas. Objetivos: describir las características generales de los pacientes según el estado de la enfermedad y el uso de belimumab. Materiales y métodos: estudio de corte transversal, registro RELESSAR. Se definió el estado de la enfermedad como: remisión: SLEDAI=0 y sin corticoides; baja actividad de la enfermedad: SLEDAI >0 y ≤4 y sin corticoides; control no óptimo: SLEDAI >4 y cualquier dosis de corticoides. Resultados: se incluyeron 1.277 pacientes, 23,4% en remisión, 12,6% en baja actividad y 63,8% con control no óptimo. En este último grupo eran más jóvenes y con menor duración de la enfermedad; presentaban mayores índices de actividad y cronicidad, y mayor empleo de inmunosupresores. Solo el 22,3% de los pacientes con criterio potencial de uso de belimumab (lupus eritematoso sistémico activo a pesar del tratamiento estándar) lo recibía en ese momento. Las variables asociadas a hospitalizaciones fueron: terapia con corticoides, ciclofosfamida y mayor SLICC. Conclusiones: se refleja la complejidad del manejo de estos pacientes y se visualizan aspectos estructurales como la desigualdad. El uso del belimumab resultaría beneficioso en los pacientes seleccionados.

Introduction: lupus is a complex disease and often difficult to approach. Achieving remission is one of the objectives, incorporating therapeutic options. Objectives: to describe the characteristics of the patients and the use of belimumab, according to the status of the disease. Materials and methods: cross-sectional study. Patients of the RELESSAR registry. Stratification: Remission: SLEDAI=0 and without corticosteroids. Low disease activity SLEDAI> 0 and ≤4 and without corticosteroids and non-optimal control: SLEDAI> 4 and any dose of corticosteroids. Results: a total of 1,277 patients were included, 23.4% in remission, 12.6% in low disease activity and 63.8% in non-optimal control. The last group was younger and had a shorter duration of the disease. They had higher activity and chronicity indices and greater use of immunosuppressants. Only 22.3% of the patients with potential criteria for the use of belimumab (activity disease despite standard treatment) were receiving it. The variables associated with hospitalizations were: corticosteroids, cyclophosphamide and higher SLICC. Those associated with severe infection: mycophenolate mofetil, azathioprine, corticosteroids, and higher SLICC. Conclusions: the complexity of the management of these patients is reflected, visualizing structural aspects such as inequality. The use of belimumab could be beneficial in selected patients.

Lupus en Argentina. Pacientes no respondedores al tratamiento estándar y belimumab como posible opción. Datos del registro RELESSAR / Lupus in Argentina. Non-responders to standard treatment and belimumab as a possible option. RELESSAR record data

Introducción: el lupus es una enfermedad compleja y varias veces de difícil abordaje. Alcanzar la remisión es uno de los objetivos, incorporando opciones terapéuticas. Objetivos: describir las características generales de los pacientes según el estado de la enfermedad y el uso de belimumab. Materiales y métodos: estudio de corte transversal, registro RELESSAR. Se definió el estado de la enfermedad como: remisión: SLEDAI=0 y sin corticoides; baja actividad de la enfermedad: SLEDAI >0 y ≤4 y sin corticoides; control no óptimo: SLEDAI >4 y cualquier dosis de corticoides. Resultados: se incluyeron 1.277 pacientes, 23,4% en remisión, 12,6% en baja actividad y 63,8% con control no óptimo. En este último grupo eran más jóvenes y con menor duración de la enfermedad; presentaban mayores índices de actividad y cronicidad, y mayor empleo de inmunosupresores. Solo el 22,3% de los pacientes con criterio potencial de uso de belimumab (lupus eritematoso sistémico activo a pesar del tratamiento estándar) lo recibía en ese momento. Las variables asociadas a hospitalizaciones fueron: terapia con corticoides, ciclofosfamida y mayor SLICC. Conclusiones: se refleja la complejidad del manejo de estos pacientes y se visualizan aspectos estructurales como la desigualdad. El uso del belimumab resultaría beneficioso en los pacientes seleccionados.

Introduction: lupus is a complex disease and often difficult to approach. Achieving remission is one of the objectives, incorporating therapeutic options. Objectives: to describe the characteristics of the patients and the use of belimumab, according to the status of the disease. Materials and methods: cross-sectional study. Patients of the RELESSAR registry. Stratification: Remission: SLEDAI=0 and without corticosteroids. Low disease activity SLEDAI> 0 and ≤4 and without corticosteroids and non-optimal control: SLEDAI> 4 and any dose of corticosteroids. Results: a total of 1,277 patients were included, 23.4% in remission, 12.6% in low disease activity and 63.8% in non-optimal control. The last group was younger and had a shorter duration of the disease. They had higher activity and chronicity indices and greater use of immunosuppressants. Only 22.3% of the patients with potential criteria for the use of belimumab (activity disease despite standard treatment) were receiving it. The variables associated with hospitalizations were: corticosteroids, cyclophosphamide and higher SLICC. Those associated with severe infection: mycophenolate mofetil, azathioprine, corticosteroids, and higher SLICC. Conclusions: the complexity of the management of these patients is reflected, visualizing structural aspects such as inequality. The use of belimumab could be beneficial in selected patients.

Focal myositis presenting as pseudothrombophlebitis of the arm.

We described a 21-year-old woman with a diagnosis of Sjögren syndrome that came for consultation with a localized mass over her left arm of fast growth. Lab results were normal; a Doppler ultrasound showed the presence of partial thrombosis in the left axillary vein; a magnetic resonance imaging showed edema on the biceps muscle and the biopsy of the mass disclosed the presence of severe lymphocyte infiltrate within the connective tissue and scarce muscle fibers. Immunostaining showed positive results for antígeno comun leucocitario in spanish (leukocyte common antigen) (ACL) and CD3. Those results are consistent with the diagnosis of focal myositis. The patient was treated with low doses of prednisone and methotrexate, with good response.

Calciphylaxis associated with rheumatoid arthritis: Communication of another case. / Calcifilaxis asociada a artritis reumatoide: comunicación de otro caso.

Calciphylaxis has been defined as tissue sensitivity to calcification, described mainly in patients with chronic renal insufficiency, renal transplant, or parathyroid dysfunction. There are few cases described in patients with rheumatoid arthritis that do not suffer from renal failure or hyperparathyroidism. Another case is presented of calciphylaxis in a woman with inactive rheumatoid arthritis.

Cytidine deaminase in polymyalgia rheumatica and elderly onset rheumatoid arthritis.

Serum cytidine deaminase (CD) as a marker of inflammatory disease was assessed in 44 patients and 47 controls to differentiate polymyalgia rheumatica (PMR) from elderly onset rheumatoid arthritis (EORA). The patients were divided into four groups: PMR with and without synovitis and seropositive and seronegative EORA. No statistically significant differences were found when serum CD levels of seropositive EORA patients were compared with serum CD of PMR patients without synovitis, neither when serum CD levels of all PMR patients were compared with a seronegative EORA group, nor when serum CD levels of PMR patients with synovitis were compared with those with EORA. Nevertheless, statistically significant differences were detected between EORA's serum CD levels and the control group (p=0.023). This difference was 10% when comparing CD levels of PMR patients with the control group (p=0.070). We did not demonstrate that serum CD levels could be a useful tool to differentiate PMR from EORA, but these findings could nevertheless reflect the presence of an inflammatory disease.