Immunoglobulins in systemic sclerosis management. A large multicenter experience.

OBJECTIVE: To analyze the effectiveness and safety of intravenous immunoglobulin (IVIG) given in routine care to patients with systemic sclerosis (SSc). METHODS: A retrospective multicenter observational study was conducted in SSc patients treated with IVIG. We collected data on epidemiological parameters and clinical outcomes. Firstly, we assessed changes in organ manifestations during IVIG treatment. Secondly, we analyzed the frequency of adverse effects. The following parameters were collected from baseline to the last follow-up: the patient's weight, modified Rodnan Skin Score (mRSS), modified manual muscle strength scale (MRC), laboratory test(creatine kinase(CK), hemoglobin and protein levels), The University of California Los Angeles Scleroderma Clinical Trials Consortium gastrointestinal tract 2.0 (UCLA GIT 2.0) questionnaire, pulmonary function tests, and echocardiography. RESULTS: Data were collected on 78 patients (82% females; 59% with diffuse SSc). Inflammatory idiopathic myopathy was the most frequent concomitant overlap disease (41%). The time since Raynaud's phenomenon and SSc onset were 8.8 ± 18 and 6.2 ± 6.7 years respectively. The most frequent IVIG indication was myositis (38/78), followed by gastrointestinal (27/78) and cutaneous (17/78) involvement. The median number of cycles given were 5. 54, 53 and 9 patients have been treated previously with glucocorticoids, synthetic disease-modifying antirheumatic drugs and biologic therapies respectively. After IVIG use we found significant improvements in muscular involvement (MRC ≥ 3/5 92% IVIG, p = 0.001 and CK levels from 1149 ± 2026 UI to 217 ± 224 UI, p = 0.02), mRSS (15 ± 12.4 to 13 ± 12.5, p = 0.015) and improvement in total score of UCLA GIT 2.0 (p = 0.05). None Anti-RNA polymerase III patients showed an adequate response in gastrointestinal involvement (0/7) in comparison with other antibodies (0 vs. 25, p = 0,039). Cardiorespiratory involvement remained stable. A total of 12 adverse events were reported with only one withdrawn due to serious adverse effect. CONCLUSIONS: this study suggest that IVIG may improve myositis, gastrointestinal and skin involvement in SSc patients treated in routine care and seems to have a good safety profile.

Efectos de la intervención desde terapia ocupacional sobre la percepción del dolor crónico y la calidad de vida en pacientes con artritis reumatoide / Effects of an occupational therapy intervention in chronic pain perception and quality of life of patients with rheumatoid arthritis

Objetivos: determinar si una propuesta de intervención experimental desarrollada desde el campo de trabajo de la terapia ocupacional, dirigida a la reestructuración del desempeño ocupacional en pacientes con artritis reumatoide, reduce los niveles de dolor percibidos y mejora la autoeficacia y la calidad de vida de los mismos. Esta intervención se llevó a cabo de forma grupal, utilizando modelos de formación y enseñanza de estrategias a los pacientes. Material y método: estudio de intervención controlado aleatorizado de un programa grupal de terapia ocupacional de siete sesiones, de dos horas cada una, realizadas en cinco semanas. Resultados: se detectó una mejora en las puntuaciones de autoeficacia (p < 0,001) y de calidad de vida (p = 0,001; p = 0,08), así como de las puntuaciones de intensidad de dolor medidas con MPQ (McGill Pain Questionnaire), aunque los valores recogidos con EVA (escala visual analógica) se mantienen en el tiempo. Conclusiones: el programa resultó eficaz en la modificación de las variables estudiadas, apreciándose cambios significativos tanto en intensidad de dolor, como en los valores referidos a autoeficacia y calidad de vida. Es recomendable repetir el trabajo con una muestra mayor que permita obtener datos más consistentes (AU)

Objectives: To determine if an occupational therapy based intervention focused on performance reorganization on patients with rheumatoid arthritis reduces pain and improves self-efficacy and quality of life. Material and methods: Randomized controlled intervention study through a group program of seven occupational therapy sessions, conducted in five weeks. Results: We found an improvement in self-efficacy (p<0,001) and quality of life scores (p = 0,001, p = 0,08), as well as pain intensity MPQ (McGill Pain Questionnaire) measures, although the values collected with VAS (visual analogue scale) maintained over time. Conclusions: The program was effective in changing the studied variables, although a new study with a larger sample is required (AU)

Enfermedades inflamatorias mediadas por inmunidad y otras comorbilidades en pacientes con psoriasis. Características basales de la cohorte de pacientes con psoriasis del estudio AQUILES / Immune-Mediated Inflammatory Diseases and Other Comorbidities in Patients With Psoriasis: Baseline Characteristics of Patients in the AQUILES Study

Introducción y objetivos: Los pacientes con psoriasis presentan con frecuencia comorbilidades, incluyendo otras enfermedades inflamatorias mediadas por inmunidad (EIMI) y factores de riesgo cardiovascular (FRCV). El objetivo de este trabajo es describir la prevalencia basal de otras EIMI y comorbilidades en una cohorte de pacientes con psoriasis. Pacientes y métodos: AQUILES es un estudio observacional prospectivo multicéntrico de 3 cohortes de pacientes (psoriasis, espondiloartritis y enfermedad inflamatoria intestinal [EII]), para evaluar la coexistencia de EIMI y otras comorbilidades. En la cohorte con psoriasis se incluyeron pacientes ≥ 18 años atendidos en consultas hospitalarias de dermatología. Se recogió información sobre datos demográficos y clínicos de acuerdo a un protocolo preespecificado. Resultados: Se incluyeron 528 pacientes con psoriasis (edad media: 46,7 años; 60,2% hombres; 39,8% mujeres; 89,8% psoriasis en placas; mediana de PASI 3,2 [1,5-7,4]). Presentaron otra EIMI 82 pacientes (15,5% [IC 95%: 12,7-18,9]). El 14,0% (IC 95%: 11,3-17,2) presentó espondiloartritis (la mayoría de estos artritis psoriásica [prevalencia 13,1%, IC 95%: 10,5-16,2), el 1,3% EII (IC 95%: 0,6-2,7) y el 0,2% uveítis (IC 95%: 0,1-1,4). La presencia de artritis psoriásica se asoció al sexo masculino (OR: 1,75 [0,98-2,98]) y a la duración de la psoriasis > 8 años (OR: 4,17; [1,84- 9,44]) respecto a < 4 años. El 73,1% presentó al menos un FRCV: tabaquismo (40,5%); obesidad (26,0%); dislipidemia (24,8%); hipertensión arterial (24,3%) y diabetes mellitus (12,3%). Conclusión: Los pacientes con psoriasis presentaron una prevalencia del 15,5% de otras EIMI, discretamente superior a la de población general. Casi tres cuartas partes tuvieron al menos un FRCV (AU)

Introduction and objectives: Patients with psoriasis often have comorbidities, including other immune-mediated inflammatory diseases (IMIDs), and cardiovascular risk factors. In this article we describe the baseline prevalence of comorbidities----including other IMIDs----in a cohort of patients with psoriasis. Patients and methods: AQUILES was a prospective observational multicenter study of 3 patient cohorts (patients with psoriasis, spondyloarthritis, or inflammatory bowel disease) undertaken to investigate the prevalence of comorbidities, including other IMIDs, in these settings. The psoriasis cohort comprised patients aged at least 18 years who were seen in hospital dermatology clinics. A predefined protocol was used to collect demographic and clinical data. Results: The study enrolled 528 patients with psoriasis (60.2% men and 39.8% women). Mean age was 46.7 years; 89.8% of the participants had plaque psoriasis, and the median Psoriasis Area Severity Index score (PASI) was 3.2 (1.5-7.4). Comorbid IMIDs were present in 82 (15.5%) of the patients (CI 95%, 12.7%-18.9%). Spondyloarthritis was observed in 14% of patients (95% CI, 11.3%-17.2%), mostly in the form of psoriatic arthritis, for which the overall prevalence was 13.1% (95% CI, 10.5%-16.2%). Inflammatory bowel disease was present in 1.3% (95% CI, 0.6%-2.7%) and uveitis in .2% (95% CI, 0.1%-1.4%). Psoriatic arthritis was associated with male sex (odds ratio, 1.75 [.98-2.98]) and a disease duration of over 8 years (OR, 4.17 [1.84-9.44] vs a duration of < 4 years). In 73.1%, at least 1 cardiovascular risk factor was identified: smoking (40.5%), obesity (26.0%), dyslipidemia (24.8%), hypertension (24.3%), and diabetes mellitus (12.3%). Conclusion: In patients with psoriasis the prevalence of other IMIDs was 15.5%, a level slightly higher than that found in the general population. Nearly three-quarters of these patients had at least 1 cardiovascular risk factor (AU)

Immune-mediated inflammatory diseases and other comorbidities in patients with psoriasis: baseline characteristics of patients in the AQUILES study.

INTRODUCTION AND OBJECTIVES: Patients with psoriasis often have comorbidities, including other immune-mediated inflammatory diseases (IMIDs), and cardiovascular risk factors. In this article we describe the baseline prevalence of comorbidities-including other IMIDs-in a cohort of patients with psoriasis. PATIENTS AND METHODS: AQUILES was a prospective observational multicenter study of 3 patient cohorts (patients with psoriasis, spondyloarthritis, or inflammatory bowel disease) undertaken to investigate the prevalence of comorbidities, including other IMIDs, in these settings. The psoriasis cohort comprised patients aged at least 18 years who were seen in hospital dermatology clinics. A predefined protocol was used to collect demographic and clinical data. RESULTS: The study enrolled 528 patients with psoriasis (60.2% men and 39.8% women). Mean age was 46.7 years; 89.8% of the participants had plaque psoriasis, and the median Psoriasis Area Severity Index score (PASI) was 3.2 (1.5-7.4). Comorbid IMIDs were present in 82 (15.5%) of the patients (CI 95%, 12.7%-18.9%). Spondyloarthritis was observed in 14% of patients (95% CI, 11.3%-17.2%), mostly in the form of psoriatic arthritis, for which the overall prevalence was 13.1% (95% CI, 10.5%-16.2%). Inflammatory bowel disease was present in 1.3% (95% CI, 0.6%-2.7%) and uveitis in .2% (95% CI, 0.1%-1.4%). Psoriatic arthritis was associated with male sex (odds ratio, 1.75 [.98-2.98]) and a disease duration of over 8 years (OR, 4.17 [1.84-9.44] vs a duration of < 4 years). In 73.1%, at least 1 cardiovascular risk factor was identified: smoking (40.5%), obesity (26.0%), dyslipidemia (24.8%), hypertension (24.3%), and diabetes mellitus (12.3%). CONCLUSION: In patients with psoriasis the prevalence of other IMIDs was 15.5%, a level slightly higher than that found in the general population. Nearly three-quarters of these patients had at least 1 cardiovascular risk factor.

Biomarkers predicting a need for intensive treatment in patients with early arthritis.

The heterogeneous nature of rheumatoid arthritis (RA) complicates early recognition and treatment. In recent years, a growing body of evidence has demonstrated that intervention during the window of opportunity can improve the response to treatment and slow- or even stop-irreversible structural changes. Advances in therapy, such as biologic agents, and changing approaches to the disease, such as the treat to target and tight control strategies, have led to better outcomes resulting from personalized treatment to patients with different prognostic markers. The various biomarkers identified either facilitate early diagnosis or make it possible to adjust management to disease activity or poor outcomes. However, no single biomarker can bridge the gap between disease onset and prescription of the first DMARD, and traditional biomarkers do not identify all patients requiring early aggressive treatment. Furthermore, the outcomes of early arthritis cohorts are largely biased by the treatment prescribed to patients; therefore, new challenges arise in the search for prognostic biomarkers. Herein, we discuss the value of traditional and new biomarkers and suggest the need for intensive treatment as a new surrogate marker of poor prognosis that can guide therapeutic decisions in the early stages of RA.

Lupus eritematoso subagudo inducido por terbinafina en un paciente con lupus eritematoso sistémico / Lupus erythematosus induced by terbinafine in a patient with systemic lupus erythematosus

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The effects of rituximab on the lipid profile of patients with active systemic lupus erythematosus: results from a nationwide cohort in Spain (LESIMAB).

INTRODUCTION: Patients with systemic lupus erythematosus (SLE) have increased cardiovascular risk related to lipid changes induced by inflammatory activity, proteinuria and treatments. Our objective was to analyse lipid changes in a cohort of patients with SLE resistant to standard treatments who were treated with rituximab. METHODS: The study population comprised a retrospective multicentre, national cohort of patients with SLE resistant to standard treatments who were treated with rituximab. The basic lipid profile, concomitant treatment and disease activity were analysed at the start of the treatment, 24 weeks later, and at the end of the follow-up period. The effects of the main lupus variables and therapy on the lipid changes were analysed. RESULTS: Seventy-nine patients with active lupus treated with rituximab were assessed during 149.3 patient-years. Prior to the treatment, 69% had dyslipidaemia. The most frequent abnormalities were a low-density lipoprotein (LDL) level of ≥100 mg/dl (34%) and a high-density lipoprotein (HDL) level of <50 mg/dl (27%). Baseline total cholesterol (TC) and LDL levels correlated with the degree of proteinuria, while the concentration of triglycerides (TGs) correlated with the SLE Disease Activity Index (SLEDAI). TGs were reduced at short- and long-term follow-up after rituximab treatment. A multiple linear regression analysis identified that the reduction of the lupus inflammatory activity, particularly changes in proteinuria, was the only independent variable that was positively associated with the reduction in TGs after 24 weeks (p=0.001) and with TC (p=0.005) and TGs (p<0.001) at the end of the follow-up period. CONCLUSION: Our results suggest that rituximab may improve the long-term lipid profile of patients with SLE refractory to standard treatment, mainly by reducing inflammatory activity.

Multicenter longitudinal study of B-lymphocyte depletion in refractory systemic lupus erythematosus: the LESIMAB study.

OBJECTIVE: This study aimed to investigate the effectiveness and safety of single and repeated courses of rituximab in patients with refractory lupus. METHODS: LESIMAB is a multicenter, retrospective, longitudinal study of lupus patients who have not responded to standard therapy and have been treated with rituximab. Response rates at six months and at follow-up were defined as efficacy outcomes. Complete response was defined as a SELENA-SLEDAI score ≤ two and a SELENA-SLEDAI Flare Index of zero. Partial response was defined as a reduction in the SELENA-SLEDAI score of ≥four points with no new or worsening of symptoms. Adverse events were collected. RESULTS: Seventy-three (62.9%) of 116 patients achieved a response at six months (complete in 22 and partial in 51). Ninety-seven (77.6%) of 128 patients achieved a response after a mean follow-up of 20.0 ± 15.2 months (complete in 50 and partial in 47). High baseline SLEDAI score, previous treatment with ≥100 mg/day prednisone, and no history of severe hematologic flare were associated with response after the first treatment course. The median time to response was 6.5 months (95% CI, 5.0-8.0). Thirty-seven patients (38.1%) relapsed after the first infusion. The flare was severe in seven cases and mild to moderate in 29 cases. Serious infection rate was 12.6/100 patient-years. A schedule of four weekly doses was associated with more serious infections. Six patients died: two of infection and four of lupus complications. CONCLUSION: Rituximab can be an effective treatment option for patients who have refractory lupus with severe or life-threatening disease with an acceptable tolerance profile.

A novel subaerial Dunaliella species growing on cave spiderwebs in the Atacama Desert.

Strategies for life adaptation to extreme environments often lead to novel solutions. As an example of this assertion, here we describe the first species of the well-known genus of green unicellular alga Dunaliella able to thrive in a subaerial habitat. All previously reported members of this microalga are found in extremely saline aquatic environments. Strikingly, the new species was found on the walls of a cave located in the Atacama Desert (Chile). Moreover, on further inspection we noticed that it grows upon spiderwebs attached to the walls of the entrance-twilight transition zone of the cave. This peculiar growth habitat suggests that this Dunaliella species uses air moisture condensing on the spiderweb silk threads as a source of water for doing photosynthesis in the driest desert of the world. This process of adaptation recapitulates the transition that allowed land colonization by primitive plants and shows an unexpected way of expansion of the life habitability range by a microbial species.

Ancient photosynthetic eukaryote biofilms in an Atacama Desert coastal cave.

Caves offer a stable and protected environment from harsh and changing outside prevailing conditions. Hence, they represent an interesting habitat for studying life in extreme environments. Here, we report the presence of a member of the ancient eukaryote red algae Cyanidium group in a coastal cave of the hyperarid Atacama Desert. This microorganism was found to form a seemingly monospecific biofilm growing under extremely low photon flux levels. Our work suggests that this species, Cyanidium sp. Atacama, is a new member of a recently proposed novel monophyletic lineage of mesophilic "cave" Cyanidium sp., distinct from the remaining three other lineages which are all thermo-acidophilic. The cave described in this work may represent an evolutionary island for life in the midst of the Atacama Desert.

Are the C-reactive protein values and erythrocyte sedimentation rate equivalent when estimating the 28-joint disease activity score in rheumatoid arthritis?

UNLABELLED: A formula for calculating disease activity score with 28 joint counts (DAS28) with C-reactive protein (CRP) instead of the erythrocyte sedimentation rate (ESR) has been proposed. OBJECTIVE: Here we analyze the factors that contribute to the differences in the DAS28 when calculated using either the ESR (DAS28-ESR) or the CRP values (DAS28-CRP). METHODS: We analyzed the data from 587 visits made by 220 patients with early arthritis. The age at the onset of the disease was 51+/-16 years old and 76.3% of the patients were women. The disease evolution at the first visit was 5 months and at each visit information related to several variables was collected, including that necessary to calculate the DAS28-ESR and DAS28-CRP. We defined a new variable DIFDAS=DAS28-ESR-DAS28-CRP to analyze which independent variables account for differences between the two indexes. RESULTS: There was a correlation between the two indexes of 0.91 (p<0.0001), although the DAS28-ESR value obtained was higher than that of DAS28-CRP at approximately 90% of the visits. Significantly, the difference between both indexes was higher than 0.6 in 44% of the visits studied. A multivariate analysis showed that female gender and disease duration were associated with the higher values obtained for DAS28-ESR when compared to those of DAS28-CRP. CONCLUSION: Our data show that DAS28-ESR and DAS28-CRP are not fully equivalent, because the former usually produces higher values. This finding is particularly relevant in females and patients with a long disease duration.

Psychosis leading to the diagnosis of unrecognized systemic lupus erythematosus: a case report.

We present the case of a woman whose psychiatric clinical picture was the major manifestation of SLE with unrecognized secondary antiphospholipid syndrome. The atypical onset and the subsequent clinical course of the psychiatric manifestations led to the diagnosis. This case demonstrates the heterogeneous progress of SLE, the increasing relevance of the antiphospholipid antibodies in the central nervous system and the difficulty in making an early diagnosis.

Vasculitis asociadas a ANCA en artritis reumatoide. Descripción de un caso de poliangeitis microscópica y otro de enfermedad de Wegener / Vasculitis associated with anti-neutrophil cytoplasmic autoantibodies in rheumatoid arthritis. Report of a case of microscopic polyangiitis and another case of Wegener’s granulomatosis

La vasculitis es una complicación infrecuente de la artritis reumatoide que se asocia con un aumento claro de la morbimortalidad, aunque son muy raras las manifestaciones sistémicas como glomerulonefritis, vasculitis cerebral o vasculitis pulmonar. A su vez, las vasculitis sistémicas con afectación renal se asocian en menos del 5% a poliartritis franca y la asociación con artritis reumatoide es excepcional. La determinación de los anticuerpos anticitoplasma de neutrófilo (ANCA), utilizados en el contexto clínico apropiado, se ha convertido en una importante herramienta diagnóstica de las vasculitis sistémicas de pequeño vaso. Presentamos 2 pacientes diagnosticados de artritis reumatoide que posteriormente desarrollaron vasculitis sistémica, en los que la determinación de ANCA fue decisiva en el diagnóstico precoz(AU)

Vasculitis is an uncommon complication of rheumatoid arthritis that is associated with a clear increase in morbidity and mortality, although systemic manifestations such as glomerulonephritis, cerebral vasculitis or pulmonary vasculitis are very rare. Systemic vasculitis with renal involvement is associated with overt polyarthritis in less than 5% and association with rheumatoid arthritis is exceptional. Determination of anti-neutrophil cytoplasmic autoantibodies (ANCA), used in the appropriate clinical context, has become an important diagnostic tool in small-vessel systemic vasculitides. We present two patients with rheumatoid arthritis who subsequently developed systemic vasculitis. ANCA determination was decisive in the early diagnosis of these patients(AU)

[Vasculitis associated with anti-neutrophil cytoplasmic autoantibodies in rheumatoid arthritis. Report of a case of microscopic polyangiitis and another case of Wegener's granulomatosis]. / Vasculitis asociadas a ANCA en artritis reumatoide. Descripción de un caso de poliangeitis microscópica y otro de enfermedad de Wegener.

Vasculitis is an uncommon complication of rheumatoid arthritis that is associated with a clear increase in morbidity and mortality, although systemic manifestations such as glomerulonephritis, cerebral vasculitis or pulmonary vasculitis are very rare. Systemic vasculitis with renal involvement is associated with overt polyarthritis in less than 5% and association with rheumatoid arthritis is exceptional. Determination of anti-neutrophil cytoplasmic autoantibodies (ANCA), used in the appropriate clinical context, has become an important diagnostic tool in small-vessel systemic vasculitides. We present two patients with rheumatoid arthritis who subsequently developed systemic vasculitis. ANCA determination was decisive in the early diagnosis of these patients.

Molecular approach for analysis of model fungal genes encoding ligninolytic peroxidases in wood-decaying soil systems.

AIMS: Test the use of nondegenerated consensus polymerase chain reaction (PCR) primers targeting lip and mnp sequences to detect ligninolytic fungi in wood-decaying soil systems, avoiding the need for enrichment or isolation on traditional fungal media culture. METHODS AND RESULTS: The PCR primers were tested with total DNA isolated from incubations of wood-soil systems inoculated or not with the white-rot fungi Phanerochaete chrysosporium, or a white-rot sample obtained from a Nothofagus forest. The PCR products for lip and mnp sequences were only obtained in soil with P. chrysosporium-colonized wood chips. In these soil samples, reverse transcription-PCR analysis of lip and mnp PCR products indicated expression of LipA, LipB, LipJ and MnP isoenzymes. SIGNIFICANCE AND IMPACT OF THE STUDY: This is the first assessment of the use of consensus PCR primers for direct detection of ligninolytic peroxidase genes in wood-decaying soil systems.

Increased serum levels of interleukin-15 in rheumatoid arthritis with long- term disease.

OBJECTIVE: To study the serum levels of IL-15 in patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), seronegative spondyloarthropathies (SSd) and healthy donors. METHODS: The IL-15 serum levels were measured by ELISA in sera from 50 RA patients, 30 patients with SLE, 30 patients with SSd and 30 healthy donors. In RA patients, several clinical and demographic parameters were also obtained at the time of sample collection. IL-15 levels were compared in different RA subpopulations (positive or negative rheumatoid factor [RF], long term or recent onset disease, high or low disease activity). In addition, the possible association with other demographic and clinical parameters (gender, age, disease duration, etc) was also analysed. RESULTS: RA patients had significantly higher serum levels of IL-15 (102.4 +/- 150 pg/ml; p = 0.0001) than SLE patients (9.8 +/- 15.3 pg/ml), SSd patients (7.9 +/- 14.6 pg/ml) and healthy donors' (5.2 +/- 11.6 pg/ml). RA patients with a disease evolution less than 2 years showed lower IL-15 levels (33.7 +/- 62.2 pg/ml) than those with long-term disease (152.4 +/- 64.6 pg/ml; p = 0.004). In addition, a significant correlation between IL15 in serum and the number of disease-modifying antirheumatic drugs (DMARDs) prescribed was detected in RA patients (r = 0.42; p = 0.002). No association between IL-15 levels and age, gender, RF or disease activity was observed in this group. CONCLUSION: IL-15 is elevated in RA patients, specially in those with long term disease, compared to other rheumatic disorders. This finding supports that IL-15 is involved in the perpetuation of RA synovitis.