Analyzing Gender Disparities in Ankylosing Spondylitis: Exploring the Factors Behind Severe Sacroiliac Joint Disease Without Spinal Involvement.

We have read with interest the paper by Ridley et al1 on the radiographic damage discordance between the sacroiliac joints (SIJs) and the spine in patients with ankylosing spondylitis (AS) from the Prospective Study of Outcomes in AS (PSOAS) cohort, which includes patients from the United States and Australia. Their results have encouraged us to investigate the prevalence of severe SIJ disease without evidence of concomitant syndesmophytes in the spine in our patient cohort from Spain.

Exploring radiographic patterns of the cervical spine, including zygapophyseal joints, in axial spondyloarthritis.

INTRODUCTION: The assessment of the cervical spine (CS) in axial spondyloarthritis (axSpA) and its radiographic characteristics, including the zygapophyseal joints (ZJ), may be helpful for an accurate diagnosis, establishing a prognosis and enhancing treatment decisions. OBJECTIVES: To describe the prevalence and characteristics of CS involvement in patients with axSpA and perform a comparison between groups according to cervical radiographic damage. METHODS: Patients who fulfilled the Assessment of SpondyloArthritis International Society classification criteria were included from January 2011 to January 2021. Sociodemographic, clinical, radiographic and treatment variables were gathered. Patients were categorised into 'CS group' (Bath Ankylosing Spondylitis Radiology Index ≥2 or De Vlam score ≥3 for ZJ) and 'no CS group' as controls. ZJ fusion and interobserver reliability in ZJ scoring were analysed. RESULTS: A total of 340 patients were included, 244 (71.7%) men, with mean age 57±15 years. CS involvement was observed in 181 (53.2%) patients. Patients in the CS group, as compared with no CS group, were predominantly men, older, had a higher body mass index, higher prevalence of smoking, showed higher disease activity, worse functionality and mobility, as well as more structural damage. Sixty-nine patients with CS involvement had ZJ fusion at some level. These patients showed worse mobility and more radiographic damage. Overall, ZJ involvement was observed in 99 patients (29.1%), 20 of whom did not present with vertebral body involvement. CONCLUSION: Radiographic evaluation of CS is relevant in patients with axSpA and should be assessed routinely. Evaluation of the ZJ is particularly significant, as it is related to higher disease activity and worse function.

Usefulness of 18F-FDG PET-CT for assessing large-vessel involvement in patients with suspected giant cell arteritis and negative temporal artery biopsy.

OBJECTIVE: To investigate the usefulness of 18F-FDG PET-CT for assessing large-vessel (LV) involvement in patients with suspected giant cell arteritis (GCA) and a negative temporal artery biopsy (TAB). METHODS: A retrospective review of our hospital databases was conducted to identify patients with suspected GCA and negative TAB who underwent an 18F-FDG PET-CT in an attempt to confirm the diagnosis. The gold standard for GCA diagnosis was clinical confirmation after a follow-up period of at least 12 months. RESULTS: Out of the 127 patients included in the study, 73 were diagnosed with GCA after a detailed review of their medical records. Of the 73 patients finally diagnosed with GCA, 18F-FDG PET-CT was considered positive in 61 cases (83.5%). Among the 54 patients without GCA, 18F-FDG PET-CT was considered positive in only eight cases (14.8%), which included 1 case of Erdheim-Chester disease, 3 cases of IgG4-related disease, 1 case of sarcoidosis, and 3 cases of isolated aortitis. Overall, the diagnostic performance of 18F-FDG PET-CT for assessing LV involvement in patients finally diagnosed with GCA and negative TAB yielded a sensitivity of 83.5%, specificity of 85.1%, and a diagnostic accuracy of 84% with an area under the ROC curve of 0.844 (95% CI: 0.752 to 0.936). The sensitivity was 89% in occult systemic GCA and 100% in extracranial LV-GCA. CONCLUSION: Our study confirms the utility of 18F-FDG PET-CT in patients presenting with suspected GCA and a negative TAB by demonstrating the presence of LV involvement across different subsets of the disease.

Performance of the new 2022 ACR/EULAR classification criteria for giant cell arteritis in clinical practice in relation to its clinical phenotypes.

BACKGROUND: To examine the performance of the new 2022 American College of Rheumatology (ACR)/EULAR classification criteria for giant cell arteritis (GCA) in routine clinical care, compared with the 1990 ACR GCA classification criteria. METHODS: The fulfilment of 2022 ACR/EULAR and 1990 ACR criteria was tested in our real-life cohort of GCA patients with proven vasculitis by temporal artery biopsy or imaging (a necessary pre-requisite to apply the new criteria is the presence of a confirmed diagnosis of medium- or large-vessel vasculitis). The performance of classification criteria was evaluated in all patients with GCA across different subsets of the disease. Patients with GCA were compared with unselected controls with suspected GCA. RESULTS: A total of 136 patients with proven GCA were identified. The new criteria had a sensitivity of 92.6% and a specificity of 85.2%. According to the clinical phenotypes, the sensitivity was 98.8% in cranial GCA, 92% in extracranial large vessel (LV) GCA and 75% in occult systemic GCA. These data are much better than those observed with the 1990 ACR classification criteria, which showed a sensitivity of 66.1% and a specificity of 85.1% for the total sample, with a sensitivity of 89.1% in cranial GCA, 24% in extracranial LV-GCA and 35.7% in occult systemic GCA. Ten (7.4%) patients in our cohort did not fulfil either of the criteria sets (8 with occult systemic GCA and 2 with extracranial LV-GCA). The sensitivity of the new criteria in patients with occult systemic and extracranial LV-GCA could be greatly improved assigning more weight (3 points) to some imaging findings (axillary involvement and FDG-PET activity throughout the aorta). CONCLUSION: Our study confirms that the new classification criteria are more sensitive in real-life settings than the old ACR criteria across all clinical phenotypes.

Efficacy and safety of leflunomide in the management of large vessel vasculitis: A systematic review and metaanalysis of cohort studies.

OBJECTIVE: The search for new glucocorticoid-sparing disease-modifying anti-rheumatic drugs continues to be an unmet need in large vessel vasculitis (LVV). This report aims to assess the effectiveness and safety of leflunomide (LEF) in Takayasu arteritis (TA) and giant cell arteritis (GCA). METHODS: We systematically reviewed the literature, searching for studies evaluating the efficacy of LEF in LVV. A meta-analysis was conducted using the random-effects method. RESULTS: The literature search identified eight studies that assessed LEF in TAK and seven in GCA. All were uncontrolled observational studies with a high risk of bias, implying a low or very-low certainty of evidence. In TAK, the pooled proportion of patients achieving at least a partial remission was 75% (95% CI: 0.64-0.84), angiographic stabilization was observed in 86% (0.77-0.94) and relapses in 12% (0.05-0.21). The mean reduction in the prednisolone dose (MRPD) after LEF treatment was 15.7 mg/d (10.28-21.16). Adverse events were observed in 8% of patients (0.02-0.16). Comparison of LEF with methotrexate (MTX) or cyclophosphamide revealed LEF to be superior in terms of remission induction, relapse prevention, and tolerance. When compared with tofacitinib, both drugs demonstrated comparable efficacy. In GCA, the pooled proportion of patients achieving at least a partial remission was 60% (0.17-0.95). The MRPD after LEF treatment was 15.63 mg/d (1.29-32.55) and 53% of the patients were able to discontinue glucocorticoids (0.25 - 0.80). Relapses were observed in 21% of cases (0.14- 0.28) and adverse events in 28% (0.12-0.46). Comparison of LEF with MTX showed similar efficacy and tolerance. CONCLUSION: LEF is well tolerated and might be effective for patients with TAK and GCA.

Bone health and predictors of 15-year mortality in a physically active population.

OBJECTIVE: To analyse determinants of mortality at 15 years in a population over 60 years of age and physically active. METHODS: This is a prospective longitudinal study. After 15 years of participating in an active ageing programme, participants were contacted by telephone to verify their state of health and to determine whether in that time they had had any fractures. RESULTS: 561 individuals over 60 years of age were included, 82% of whom were women. Only differences in densitometric data, FRAX values and history of previous fracture at baseline characteristics were found between the group that died at 15 years and the group that remained alive. The only variables that were related to mortality risk were the basal data of the densitometric t-score (OR = .50, P < .001) and history of fracture in any location (OR = 2.44, P < .033). CONCLUSIONS: The value of bone mineral density could be considered as a useful biomarker to calculate the risk of mortality in people over 60 years old with a physically active lifestyle.

Salud ósea y predictores de mortalidad a 15 años en una población físicamente activa / Bone Health and Predictors of 15-Year Mortality in a Physically Active Population

Objetivo: Analizar determinantes de mortalidad a 15años en relación con la salud ósea en una población de mayores de 60años y físicamente activos. Métodos: Estudio longitudinal prospectivo. A los 15años de participar en un programa de envejecimiento activo, y de los que se disponía de datos de salud ósea, se contactó telefónicamente con los participantes para constatar el estado vital y conocer si en ese intervalo de tiempo habían tenido alguna fractura, y para determinar la asociación entre la puntuación basal del FRAX, los datos densitométricos y la mortalidad al cabo del tiempo.Resultados: Se incluyeron 561 individuos mayores de 60años, de los que el 82% eran mujeres. Solo se encontraron diferencias en las características basales entre el grupo que falleció a los 15años y el grupo que siguió con vida en los datos densitométricos y en los valores del FRAX, así como en el antecedente de algún tipo de fractura. Las únicas variables que se relacionaron con el riesgo de mortalidad fueron los datos basales del T-score densitométricos (OR=0,50; p<0,001) y el antecedente de fractura en cualquier localización (OR=2,44; p<0,033).Conclusiones: El valor de la densidad mineral ósea podría considerarse como un biomarcador útil para calcular el riesgo de mortalidad en mayores de 60años con una vida físicamente activa.(AU)

Objective: To analyse determinants of mortality at 15years in a population over 60years of age and physically active. Methods: This is a prospective longitudinal study. After 15years of participating in an active aging programme, participants were contacted by telephone to verify their state of health and to determine whether in that time they had had any fractures. Results: A total of 561 individuals over 60years of age were included, 82% of whom were women. Only differences in densitometric data, FRAX values and history of previous fracture at baseline characteristics were found between the group that died at 15years and the group that remained alive. The only variables that were related to mortality risk were the basal data of the densitometric T-score (OR=.50, P<.001) and history of fracture in any location (OR=2.44, P<.033). Conclusions: The value of bone mineral density could be considered as a useful biomarker to calculate the risk of mortality in people over 60years old with a physically active lifestyle.(AU)

Bone Health and Predictors of 15-Year Mortality in a Physically Active Population. / Salud ósea y predictores de mortalidad a 15 años en una población físicamente activa.

OBJECTIVE: To analyse determinants of mortality at 15years in a population over 60years of age and physically active. METHODS: This is a prospective longitudinal study. After 15years of participating in an active aging programme, participants were contacted by telephone to verify their state of health and to determine whether in that time they had had any fractures. RESULTS: A total of 561 individuals over 60years of age were included, 82% of whom were women. Only differences in densitometric data, FRAX values and history of previous fracture at baseline characteristics were found between the group that died at 15years and the group that remained alive. The only variables that were related to mortality risk were the basal data of the densitometric T-score (OR=.50, P<.001) and history of fracture in any location (OR=2.44, P<.033). CONCLUSIONS: The value of bone mineral density could be considered as a useful biomarker to calculate the risk of mortality in people over 60years old with a physically active lifestyle.

Tocilizumab in Behçet's disease with refractory ocular and/or neurological involvement: response according to different clinical phenotypes.

OBJECTIVES: Anti-IL6R tocilizumab (TCZ) therapy has proved to be useful in the treatment of refractory ocular and/or neurological involvement of Behçet's disease (BD). However, TCZ efficacy in other BD manifestations remains unclear. In this study we aimed to assess the efficacy of TCZ in the different clinical phenotypes of BD. METHODS: This is a multicentre study of BD patients treated with TCZ, due to refractivity to standard systemic treatment. RESULTS: We studied 16 patients (10 men/6 women); mean age 36.5±18.2 years. The main clinical manifestations at TCZ onset were ocular, oral and/or genital ulcers, arthritis, folliculitis and/or neurological involvement. Before TCZ, they had received several conventional and/or biological immunosuppressants, such as methotrexate, cyclosporine, adalimumab or infliximab. TCZ was used in monotherapy or combined with conventional immunosuppressive drugs. The main indications for TCZ prescription were refractory uveitis (n=14) and refractory neurobehçet (n=2). After a median [IQR] follow-up of 20 [9-45] months using TCZ, neurological and ocular domains improved in most cases with complete remission in most patients with uveitis. Articular and peripheral venous manifestations also experienced a favourable evolution. However, oral/genital ulcers, skin lesions and intestinal manifestations followed a torpid course. CONCLUSIONS: TCZ is effective in BD with major clinical involvement. However, it does not seem to be effective in oral/genital ulcers or skin lesions.

Response to interleukin-6 receptor antagonists in patients with rheumatoid arthritis is independent of the number of prior used TNF inhibitors: A systematic review and metaanalysis.

OBJECTIVE: To investigate whether early response to tocilizumab (TCZ) and sarilumab (SAR) therapy in patients with active rheumatoid arthritis (RA) is influenced by previous use of biologic agents. METHODS: We performed a systematic literature review and a meta-analysis of original studies that analyzed the effectiveness of TCZ or SRL in subgroups of RA patients, including biologic-naïve patients versus those with inadequate response to at least one biologic DMARD (bDMARD), and patients with failure to 1 versus≥2 bDMARDs. RESULTS: The study selection process finally included 17 articles corresponding to 14 studies, including 7 randomized controlled trials (RCTs). Although the existing literature that compared the response in biologic-naïve patients versus those with inadequate response to at least one bDMARD showed conflicting results, meta-analysis of 6 published studies revealed a significantly higher likelihood of remission (RR=1.3; 95% CI: 1.2-1.5) and low activity disease (RR=1.3; 95% CI: 1.2-1.4) in the biologic-naïve group at week 24. However, differences between groups were not clinically meaningful in all studies and not always maintained after 6 to 12months of treatment. In addition, data from RCT RADIATE and TARGET suggest that the response to IL-6 pathway inhibitors seems to be similar, regardless of the number of tumor necrosis factor inhibitors (TNFis) previously tested. CONCLUSION: Disease activity was more rapidly reduced in the early stages of treatment in biologic-naïve patients. However, near similar efficacy can be expected in patients who experienced a failure of at least one bDMARD (mainly TNFis) beyond the first 6 to 12months of treatment, suggesting that the response occurs independently of the number of prior TNFis.

Epidemiology of osteoporosis and its determinants in physically active Majorcan elderly.

OBJECTIVE: To estimate the prevalence and distribution of determinants of osteoporosis (OP) in a population of physically active Majorcans over 60. METHODS: Health survey in which consecutive women and men above 60 years old visiting sports facilities during a two-month period were recruited. All underwent a densitometry of the lumbar spine (LS) and femoral neck (FN). Osteoporosis was defined according to the World Health Organization densitometric criteria (T-score <2.5 SD in the LS or FN, and osteopenia if the result was between -2.5 and -1 SD). As osteoporosis shows substantial differences between genders, the study of its determinants was conducted independently for men and women. RESULTS: The sample included 731 subjects (86% female), with an average age of 70 (SD 5) among men and 65 (8) among women. The overall prevalence of osteoporosis was 35.7% in the LS, 8.9% in the FN and 39.4% in the LS and/or FN. The analysis by gender showed a higher prevalence of osteoporosis in women than in men (43.8 % vs. 11.1%). The presence of osteoporosis increased with age in men and women (7.8% for 61-75 years old vs 22.7% > 75 years old for men and 48.5% for 61-75 years old vs 62.7% > 75 for women). CONCLUSIONS: Densitometric osteoporosis is frequent among physically active elderly population, and higher than expected in a largely sunlight-exposed area.

Rituximab effect in severe progressive connective tissue disease-related lung disease: preliminary data.

Progressive connective tissue disease (CTD)-related lung disease is a challenging condition that requires lung transplantation in some patients. Treatment with rituximab may improve lung function. To evaluate the effect of rituximab in patients with progressive CTD-related lung disease who met criteria for inclusion in waiting list for a lung transplant. Retrospective study of patients with progressive CTD-related lung disease with criteria for lung transplant (FVC < 60% and/or DLCO < 40%) that started treatment with rituximab because of disease progression. Clinical variables, pulmonary function tests and chest computed tomography were used to monitor the effect of rituximab. The cohort included 18 patients; systemic sclerosis (7), rheumatoid arthritis (5), systemic lupus erythematosus (4), Sjögren syndrome (1) and antisynthetase syndrome (1). The radiologic patterns observed were: usual interstitial pneumonia (1), non-specific interstitial pneumonia (9), lymphoid interstitial pneumonia (1), emphysema-usual interstitial pneumonia (1), shrinking lung syndrome (3) and undetermined pattern (3). Over the previous year to rituximab initiation a decline in FVC (- 3.8%, p = 0.095) and DLCO (- 8.4%, p = 0.004) was observed. After 2 years of treatment, DLCO significantly improved (+ 12.4%, p < 0.001 at 1 year and + 15.3%, p = 0.001 at 2 years). Six patients (33.3%) presented adverse events related to rituximab. No patient required lung transplant or died during the study period. Rituximab is an effective treatment for patients with severe and progressive CTD-related lung disease, which allows to delay lung transplantation in some cases.

High prevalence of gallstone disease in rheumatoid arthritis: A new comorbidity related to dyslipidemia? / Alta prevalencia de litiasis biliar en la artritis reumatoide: ¿una nueva comorbilidad relacionada con la dislipidemia?

Objective: To assess the prevalence of gallstone disease and identify associated risk factors in rheumatoid arthritis (RA) patients compared to the general population. Methods: Eighty-four women with rheumatoid arthritis were included in the study. Each patient was assessed via a structured interview, physical examination, abdominal ultrasound and blood test including lipid profile. The prevalence of gallstone disease in rheumatoid arthritis was compared with data from a study of the Spanish population matched by age groups. Results: Twenty-eight of the 84 women had gallstone disease (33.3%). RA women with and without gallstone disease were similar in most of the variables assessed, except for older age and menopausal status in the former. A greater prevalence of gallstone disease was seen in rheumatoid arthritis patients compared to the general population of the same age; however, the differences were significant only in women aged 60 or older (45.5% versus 23.1% respectively, P-value .008). The age-adjusted OR of developing gallstone disease in RA women compared with general population women was 2,3 (95% CI: 1.3-4.1). A significantly higher HDL3-c subfraction and higher apoA-I/HDL and HDL3-c/TC ratios were observed in patients with gallstone disease. Conclusion: Women with rheumatoid arthritis may have a predisposition to gallstones that can manifest in middle or older age compared with women in the general population. This situation could be related to chronic inflammation and HDL metabolism

Objetivo: Evaluar la prevalencia de litiasis biliar e identificar los factores de riesgo asociados en pacientes con artritis reumatoide (AR) en comparación con la población general. Métodos: Ochenta y cuatro mujeres con AR fueron incluidas en el estudio. Cada paciente fue evaluada a través de una entrevista estructurada, un examen físico, una ecografía abdominal y un análisis de sangre que incluía el perfil lipídico. La prevalencia de litiasis biliar en AR se comparó con los datos de un estudio de la población española emparejada por grupos de edad. Resultados: Veintiocho de las 84 mujeres tenían litiasis biliar (33,3%). Las pacientes con y sin colelitiasis fueron similares en la mayoría de las variables evaluadas, a excepción de la edad más avanzada y mayor prevalencia de estado menopáusico en las pacientes con AR. Las pacientes con AR presentaban una mayor prevalencia de litiasis biliar en comparación con la población general de la misma edad; sin embargo, estas diferencias solo fueron significativas en mujeres de 60 años o más (45,5% vs. 23,1% respectivamente, p-valor 0,008). La OR ajustada por edad de presentar litiasis biliar en mujeres con AR respecto a mujeres de la población general fue de 2,3 (IC del 95%: 1,3-4,1). Se observó una subfracción de c-HDL3 significativamente más alta y una relación mayor de apoA-I/HDL y c-HDL3/TC en las pacientes con litiasis biliar. Conclusión: Las mujeres con AR pueden tener una mayor predisposición a la presencia de litiasis biliar en comparación con las mujeres en la población general, sobre todo en edades más avanzadas. Esta situación podría estar relacionada con la inflamación crónica y el metabolismo de las HDL

Effectiveness and safety of rituximab for the treatment of refractory systemic sclerosis associated calcinosis: A case series and systematic review of the literature.

OBJECTIVE: To analyze the effectiveness and safety of rituximab (RTX) for the treatment of refractory systemic sclerosis (SSc)-associated calcinosis. METHODS: We undertook an observational study of patients with this complication treated with 1 or more cycles of RTX (1 g × 2 weeks) and evaluated for at least 12 months after RTX treatment in a single center. The primary outcome measures of the study were the improvement of calcinosis symptoms (pain, signs of local inflammation, and new episodes of skin ulceration) and the radiologic evolution of the calcification(s). RESULTS: We treated 8 patients with refractory SSc-related calcinosis with RTX (off-label use). The main indications for RTX were complicated calcinosis unresponsive to previous therapies with concomitant arthritis in 2 patients and refractory arthritis or interstitial lung fibrosing disease in the remaining 6 patients. The mean number of RTX cycles administered was 3.12 ±â€¯2.1 (range, 1-7), the median duration of RTX treatment was 9 months (interquartile range [IQR], 7.5-36 months), and the median follow-up after the first infusion of RTX dose was 19 months (IQR, http://catsalut.gencat.cat/web/.content/minisite/catsalut/proveidors_professionals/medicaments_farmacia/phf_mhda/informes_camse/esclerosi_sistemica/Dictamen-CAMS_-ES_-web.pdf (n.d.) 5-45 months). Four patients (50%) had a significant improvement in clinical symptoms (sustained improvement in the visual analog scale for pain of at least 50% and no new episodes of local inflammation or skin ulceration). Two of these patients (25%) also had a complete resolution or significant reduction in the size of the calcification(s) on X-ray, according with the radiographical scoring system for calcinosis developed by the Scleroderma Clinical Trials Consortium. In the remaining 4 patients (50%), RTX did not provide any significant clinical or radiologic benefit for calcinosis. The frequency of adverse effects was low, occurring in only 1 patient (12.5%), who developed upper respiratory tract infections not requiring hospitalization. CONCLUSION: Our preliminary data suggest that RTX may be helpful as a rescue therapy in selected cases of severe and refractory SSc-related calcinosis.

High prevalence of gallstone disease in rheumatoid arthritis: A new comorbidity related to dyslipidemia?

OBJECTIVE: To assess the prevalence of gallstone disease and identify associated risk factors in rheumatoid arthritis (RA) patients compared to the general population. METHODS: Eighty-four women with rheumatoid arthritis were included in the study. Each patient was assessed via a structured interview, physical examination, abdominal ultrasound and blood test including lipid profile. The prevalence of gallstone disease in rheumatoid arthritis was compared with data from a study of the Spanish population matched by age groups. RESULTS: Twenty-eight of the 84 women had gallstone disease (33.3%). RA women with and without gallstone disease were similar in most of the variables assessed, except for older age and menopausal status in the former. A greater prevalence of gallstone disease was seen in rheumatoid arthritis patients compared to the general population of the same age; however, the differences were significant only in women aged 60 or older (45.5% versus 23.1% respectively, P-value .008). The age-adjusted OR of developing gallstone disease in RA women compared with general population women was 2,3 (95% CI: 1.3-4.1). A significantly higher HDL3-c subfraction and higher apoA-I/HDL and HDL3-c/TC ratios were observed in patients with gallstone disease. CONCLUSION: Women with rheumatoid arthritis may have a predisposition to gallstones that can manifest in middle or older age compared with women in the general population. This situation could be related to chronic inflammation and HDL metabolism.

Primary respiratory disease in patients with systemic lupus erythematosus: data from the Spanish rheumatology society lupus registry (RELESSER) cohort.

BACKGROUND: The purpose of this study was to assess the prevalence, associated factors, and impact on mortality of primary respiratory disease in a large systemic lupus erythematosus (SLE) retrospective cohort. METHODS: All adult patients in the RELESSER-TRANS (Registry of Systemic Lupus Erythematosus Patients of the Spanish Society of Rheumatology [SER], cross-sectional phase) registry were retrospectively investigated for the presence of primary pleuropulmonary manifestations. RESULTS: In total 3215 patients were included. At least one pleuropulmonary manifestation was present in 31% of patients. The most common manifestation was pleural disease (21%), followed by lupus pneumonitis (3.6%), pulmonary thromboembolism (2.9%), primary pulmonary hypertension (2.4%), diffuse interstitial lung disease (2%), alveolar hemorrhage (0.8%), and shrinking lung syndrome (0.8%). In the multivariable analysis, the variables associated with the development of pleuropulmonary manifestation were older age at disease onset (odds ratio (OR) 1.03, 95% confidence interval (CI) 1.02-1.04), higher SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) scores (OR 1.03, 95% CI 1.00-1.07), the presence of Raynaud's phenomenon (OR 1.41, 95% CI 1.09-1.84), secondary antiphospholipid syndrome (OR 2.20, 95% CI 1.63-2.97), and the previous or concomitant occurrence of severe lupus nephritis, (OR 1.48, 95% CI 1.12-1.95) neuropsychiatric manifestations (OR 1.49, 95% CI 1.11-2.02), non-ischemic cardiac disease (OR 2.91, 95% CI 1.90-4.15), vasculitis (OR 1.81, 95% CI 1.25-2.62), hematological manifestations (OR 1.31, 95% CI 1.00-1.71), and gastrointestinal manifestations, excluding hepatitis (OR 2.05, 95% CI 1.14-3.66). Anti-RNP positivity had a clear tendency to significance (OR 1.32, 95% CI 1.00-1.75; P = 0.054). The development of pleuropulmonary manifestations independently contributes to a diminished survival (hazard ratio of 3.13). However, not all complications will influence the prognosis in the same way. Whereas the occurrence of pleural disease or pulmonary thromboembolism has a minimal impact on the survival of these patients, the remaining manifestations have a major impact on mortality. CONCLUSION: Except for pleural disease, the remaining respiratory manifestations are very uncommon in SLE (<4%). Pleuropulmonary manifestations independently contributed to a decreased survival in these patients.

The value of repeat biopsy in lupus nephritis flares.

Whether a repeat renal biopsy is helpful during lupus nephritis (LN) flares remains debatable. In order to analyze the clinical utility of repeat renal biopsy in this complex situation, we retrospectively reviewed our series of 54 LN patients who had one or more repeat biopsies performed only on clinical indications. Additionally, we reviewed 686 well-documented similar cases previously reported (PubMed 1990-2015).The analysis of all patients reviewed showed that histological transformations are common during a LN flare, ranging from 40% to 76% of cases. However, the prevalence of transformations and the clinical value of repeat biopsy vary when they are analyzed according to proliferative or nonproliferative lesions.The great majority of patients with class II (78% in our series and 77.5% in the literature review) progressed to a higher grade of nephritis (classes III, IV, or V), resulting in worse renal prognosis. The frequency of pathological conversion in class V is lower (33% and 43%, respectively) but equally clinically relevant, since almost all cases switched to a proliferative class. Therefore, repeat biopsy is highly advisable in patients with nonproliferative LN at baseline biopsy, because these patients have a reasonable likelihood of switch to a proliferative LN that may require more aggressive immunosuppression.In contrast, the majority of patients (82% and 73%) with proliferative classes in the reference biopsy (III, IV or mixed III/IV + V), remained into proliferative classes on repeat biopsy. Although rebiopsy in this group does not seem as necessary, it is still advisable since it will allow us to identify the 18% to 20% of patients that switch to a nonproliferative class. In addition, consistent with the reported clinical experience, repeat biopsy might also be helpful to identify selected cases with clear progression of proliferative lesions despite the initial treatment, for whom it is advisable to intensify inmunosuppression. Thus, our experience and the literature data support that repeat biopsy also brings more advantges than threats in this group.The results of the repeat biopsy led to a change in the immunosuppresive treatment in more than half of the patients on average, intensifying it in the majority of the cases, but also reducing it in 5% to 30%.