Differences in clinical manifestations and increased severity of systemic lupus erythematosus between two groups of Hispanics: European Caucasians versus Latin American mestizos (data from the RELESSER registry).

BACKGROUND: Systemic lupus erythematosus (SLE) is regarded as a prototype autoimmune disease because it can serve as a means for studying differences between ethnic minorities and sex. Traditionally, all Hispanics have been bracketed within the same ethnic group, but there are differences between Hispanics from Spain and those from Latin America, not to mention other Spanish-speaking populations. OBJECTIVES: This study aimed to determine the demographic and clinical characteristics, severity, activity, damage, mortality and co-morbidity of SLE in Hispanics belonging to the two ethnic groups resident in Spain, and to identify any differences. METHODS: This was an observational, multi-centre, retrospective study. The demographic and clinical variables of patients with SLE from 45 rheumatology units were collected. The study was conducted in accordance with Good Clinical Practice guidelines. Hispanic patients from the registry were divided into two groups: Spaniards or European Caucasians (EC) and Latin American mestizos (LAM). Comparative univariate and multivariate statistical analyses were carried out. RESULTS: A total of 3490 SLE patients were included, 90% of whom were female; 3305 (92%) EC and 185 (5%) LAM. LAM patients experienced their first lupus symptoms four years earlier than EC patients and were diagnosed and included in the registry younger, and their SLE was of a shorter duration. The time in months from the first SLE symptoms to diagnosis was longer in EC patients, as were the follow-up periods. LAM patients exhibited higher prevalence rates of myositis, haemolytic anaemia and nephritis, but there were no differences in histological type or serositis. Anti-Sm, anti-Ro and anti-RNP antibodies were more frequently found in LAM patients. LAM patients also had higher levels of disease activity, severity and hospital admissions. However, there were no differences in damage index, mortality or co-morbidity index. In the multivariate analysis, after adjusting for confounders, in several models the odds ratio (95% confidence interval) for a Katz severity index >3 in LAM patients was 1.45 (1.038-2.026; p = 0.02). This difference did not extend to activity levels (i.e. SLEDAI >3; 0.98 (0.30-1.66)). CONCLUSION: SLE in Hispanic EC patients showed clinical differences compared to Hispanic LAM patients. The latter more frequently suffered nephritis and higher severity indices. This study shows that where lupus is concerned, not all Hispanics are equal.

Predictors of fatigue and persistent fatigue in early rheumatoid arthritis: a longitudinal observational study, data from the ESPOIR cohort.

OBJECTIVE: To determine, in a cohort of patients with early rheumatoid arthritis (RA), factors associated with fatigue at baseline, describe its evolution over 5 years of follow-up, and determine baseline predictors of persistent fatigue. METHOD: We selected patients fulfilling the 2010 American College of Rheumatology/European League Against Rheumatism criteria for RA included in the ESPOIR cohort. Using bivariable and multivariable logistic regression models, we examined baseline variables associated with baseline fatigue (defined by visual analogue scale fatigue > 20) and baseline predictors of persistent fatigue (if the patient experienced fatigue at all visits during the 5 year follow-up period). RESULTS: We analysed 673 patients; 80.7% reported fatigue at baseline. At baseline, fatigue was associated with female gender, younger age, greater severity of morning stiffness, sleep problems, higher Health Assessment Questionnaire levels, presence of sicca symptoms, history of thyroid problems, and presence of psychological distress (depressive or anxiety symptoms). At 5 years of follow-up, the percentage of fatigued patients who reported fatigue at all time-points since baseline was 24.6% (referred to as 'persistent fatigue'). Independent baseline predictors were presence of sicca symptoms, greater severity of morning stiffness, and psychological distress. CONCLUSIONS: Fatigue is a frequent symptom in RA. The presence of sicca symptoms, greater severity of morning stiffness, and presence of psychological distress at baseline were associated with baseline fatigue and persistent fatigue at 5 years. We did not observe any association between baseline fatigue or persistent fatigue and the Disease Activity Score based on 28-joint count-erythrocyte sedimentation rate.

A genome-wide association study identifies a new locus associated with the response to anti-TNF therapy in rheumatoid arthritis.

Anti-Tumor Necrosis Factor (anti-TNF) drugs are biologic agents commonly used to treat rheumatoid arthritis (RA). However, anti-TNFs are not effective in approximately one out of four treated patients. We conducted a Genome-Wide Association Study (GWAS) to identify the genetic variation associated with the response to anti-TNF therapy in RA. In the discovery stage, 372 RA patients treated with an anti-TNF agent (infliximab, adalimumab or etanercept) were analyzed and treatment response was defined at 12 weeks of therapy. We found a genome-wide significant association in the MED15 gene with the response to etanercept (P<1.5e-8). Using an independent cohort of 245 RA patients, we performed a replication study of the most significant GWAS associations. We replicated the association at the MED15 locus and found suggestive evidence of association in the previously associated MAFB locus. The results of this study suggest novel mechanisms associated with the response to anti-TNF therapies.

Shotgun Metagenomics Reveals Taxonomic and Functional Patterns of the Microbiome Associated with Barbour's Seahorse (Hippocampus barbouri).

This study aimed to investigate the taxonomic and functional patterns of the microbiome associated with Barbour's seahorse (Hippocampus barbouri) using a combination of shotgun metagenomics and bioinformatics. The analyses revealed that Pseudomonadota and Bacillota were the dominant phyla in the seahorse skin microbiome, whereas Pseudomonadota and, to a lesser extent, Bacillota and Bacteroidota were the dominant phyla in the seahorse gut microbiome. Several metabolic pathway categories were found to be enriched in the skin microbiome, including amino acid metabolism, carbohydrate metabolism, cofactor and vitamin metabolism, energy metabolism, nucleotide metabolism, as well as membrane transport, signal transduction, and cellular community-prokaryotes. In contrast, the gut microbiome exhibited enrichment in metabolic pathways associated with the metabolism of terpenoids and polyketides, biosynthesis of other secondary metabolites, xenobiotics biodegradation and metabolism, and quorum sensing. Additionally, although the relative abundance of bacteriocins in the skin and gut was slightly similar, notable differences were observed at the class level. Specifically, class I bacteriocins were found to be more abundant in the skin microbiome, whereas class III bacteriocins were more abundant in the gut microbiome. To the best of our knowledge, this study represents the first comprehensive examination of the taxonomic and functional patterns of the skin and gut microbiome in Barbour's seahorse. These findings can greatly contribute to a deeper understanding of the seahorse-associated microbiome, which can play a pivotal role in predicting and controlling bacterial infections, thereby contributing to the success of aquaculture and health-promoting initiatives.

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 systematic review of loneliness and social isolation scales used in epidemics and pandemics.

The COVID-19 pandemic has heightened social isolation and loneliness. There is a lack of consensus on rating scales to measure these constructs. Our objectives were to identify commonly used loneliness and social isolation scales over the last two decades and test their user characteristics. 7928 articles were searched in PubMed/MEDLINE, CINAHL, Web of Science, and APA PsychINFO databases. 41 articles were included based on study criteria. Among fourteen scales reported, UCLA 3-item loneliness scale was found to be most commonly used. The scale is specifically developed for telephone use and is the fastest taking less than a minute for self-administration.

The feasibility of utilizing the systolic pre-ejection interval as a determinant of pacing rate.

Rate-modulated pacing modes adjust the stimulus rate by responding to sensed alterations in physiologic indexes of metabolic demand. This study was designed to determine whether right ventricular pre-ejection interval, measured in patients by a prototype pacemaker system capable of tracking intraventricular volume, changes predictably with exercise and, if so, whether it can be used in an algorithm to vary heart rate appropriately. This system utilizes intraventricular electrical impedance measurements of injected microampere currents to determine intracavitary volume changes. Five pacemaker-dependent patients underwent temporary insertion of a tripolar electrode connected to an external device that sensed cardiac signals, generated an impedance wave form and produced stimuli at rates dependent on pre-ejection interval. Pre-ejection interval did not change as a result of variations in pacing rate itself (347 +/- 41 ms at 70 beats/min versus 321 +/- 19 ms at 130 beats/min), but consistently decreased during graded exercise (by 23% from baseline). During rate-modulated pacing based on pre-ejection interval, heart rate significantly increased during exercise compared with ventricular demand pacing (by 46 +/- 6 versus 7 +/- 6 beats/min, respectively), and increased appropriately during burst exercise. Thus, the pre-ejection interval appears to be a specific, reliable physiologic determinant of pacing rate during exertion, which may be applicable in implantable rate-modulated pacemakers.

Comparison of subcutaneous unfractionated heparin with a low molecular weight heparin (Fragmin) in patients with venous thromboembolism and contraindications to coumarin.

Intravenous heparin followed by oral anticoagulant therapy (e. g. with coumarin) is still the most widely used treatment for deep venous thromboembolism. Self-administered subcutaneous injections of heparin have been thought of as a promising alternative to coumarin, but the high doses required for ongoing prophylaxis have raised concerns about the possible development of bone disease. Certainly, long-term heparin therapy has been reported to cause osteoporosis in both laboratory animals and humans. This study aimed to compare the efficacy and safety of unfractionated (UF) heparin with that of a low molecular weight heparin (Fragmin, Kabi Pharmacia) in the prevention of recurrent deep venous thrombosis (DVT) and pulmonary embolism (PE) in a consecutive series of patients with contraindications to coumarin therapy. The patients comprised 40 men and 40 women, aged between 19 and 92 years (mean age, 68 years). They had all previously been diagnosed as having acute DVT and had been treated with conventional doses of heparin while in hospital. All patients had at least one of the following conditions: recent blood loss (either spontaneous or during admission while receiving heparin therapy); active gastroduodenal ulcer disease; psychological or physical inability or unwillingness to understand and accept the need for regular laboratory monitoring during coumarin treatment; chronic alcoholism; dementia; pregnancy; recent neurosurgery, and pericardial effusion; or were over 80 years of age. They were randomly allocated to receive either UF heparin, 10,000 IU s.c. b.d., or Fragmin, 5000 IU anti-Factor Xa s.c. b.d., for a period of 3-6 months.(ABSTRACT TRUNCATED AT 250 WORDS)

G-CSF after autologous bone marrow transplantation for malignant diseases in children. Spanish Working Party for Bone Marrow Transplantation in Children.

The use of recombinant human granulocyte-stimulating factor (G-CSF) has been shown to effectively accelerate granulocytic recovery after autologous bone marrow transplantation (BMT) in adults. The experience, however, is limited in children. We evaluated the hematopoietic reconstitution in 41 consecutive children undergoing autologous BMT for hematologic malignancies (21 acute lymphoblastic leukemia, five non-Hodgkin's lymphoma) and solid tumours (seven neuroblastoma, two brain tumor, three Ewing's sarcoma, two Wilms' tumor, one rhabdomyosarcoma). Their ages ranged from 2 to 16 years (mean 7.2 years). rhG-CSF was given at a dose of 10 micrograms/kg/day i.v. in a 2h infusion from day +1 until +28 or until the absolute neutrophil count (ANC) was > 1 x 10(9)/L. These patients were compared with a similar historical control group of 38 children who did not receive rhG-CSF after autologous BMT. The number of cells infused was similar in both groups. At the dose and schedule used in the present study, rhG-CSF was well tolerated and no side-effects were observed. The number of cell infused was similar in both groups. At the dose and schedule used in the present study, rhG-CSF was well tolerated and no side-effects were observed. Our data show that rhG-CSF accelerates engraftment and reduces the number of febrile days and antibiotic use. Furthermore, patients who were treated had less infections.

Relation between postoperative antithrombin III concentrations and site of operation.

Antithrombin III concentrations were measured in 305 patients before and at intervals after operation for up to 12 days. The blood concentrations fell significantly after both major and minor surgery, but there was no relation between the expected degree of surgical trauma or the amount of blood transfused and the fall in concentration of antithrombin III. Some surgical procedures such as cholecystectomy, laparotomy, hernia repair, highly selective vagotomy, and transurethral resection of the prostate were associated with a greater depletion of antithrombin III than were other common operations and it is suggested that there are factors related to the actual site of the operation which affect the concentration of circulating antithrombin III in the postsurgical period.

Pseudotumoral articular amyloidosis.

We present a 71-year-old man with primary amyloidosis characterized by several outstanding clinical features, namely hip involvement of a pseudotumoral appearance, periarticular osteolytic lesions and a pathological fracture. Kappa light chains in the synovial fluid were observed. The patient survived for 90 months without developing plasma cell dyscrasia, and with preservation of vital functions despite parenchymal involvement of multiple organs.

Aseptic spondylodiskitis in rheumatic diseases.

Aseptic diskitis is relatively common. Several rheumatic diseases involving the spine may have this complication. As this condition mimic infectious diskitis, it is important to recognize it. Clinically, it is characterised by vertebral pain of an inflammatory nature, occasionally accompanied by fever and an increase in the erythrocyte sedimentation rate. Radiologically, the decrease in the articular space and the irregularity of the vertebral plates are of particular importance. Although uncommon, it is necessary to bear in mind this possibility when the etiological search for a possible infectious diskitis proves fruitless. A MEDLINE (1986-2000) and PUBMED (1966-2000) search of relevant articles was performed. Descriptors used were aseptic diskitis, spondylodiskitis, pseudodiskitis and pseudoarthrosis.

Gout and secondary amyloid.

A case of gout and secondary amyloid is described. This rare association is described and the literature is reviewed.

Achilles tendinitis as the presentation form of Löfgren's syndrome.

Löfgren's syndrome is characterised by bilateral hilar adenopathy arthritis and erythema nodosum. Achilles tendinitis as the presentation form of Löfgren's syndrome is very unusual. Herein we present a case of bilateral achilles tendinitis as the presentation form of Löfgren's syndrome.

Air in the oesophagus: a sign of oesophageal involvement in systemic sclerosis.

An air oesophagogram, defined as a column of air involving the entire oesophagus, seen on a lateral chest X-ray was observed in 6 (20%) of 30 consecutive patients with systemic sclerosis (SSc) but in none of the controls. The presence of this sign was unrelated to the clinical subset of SSc and to age but was associated with the symptom of regurgitation.

Systemic lupus erythematosus, berry aneurysm and subarachnoid haemorrhage.

A 57-year-old woman with SLE and subarachnoid haemorrhage is described. The aetiology of the haemorrhage was a saccular aneurysm. The literature is reviewed.

Occult liver involvement by polyarteritis nodosa.

The diagnosis of panarteritis nodosa is usually difficult because of the variability of clinical manifestations. Herein is presented a case of occult liver involvement by polyarteritis nodosa. Several biopsies were performed but the diagnosis was made with the contribution of a hepatic arteriography.

[The SAPHO syndrome: a study of 16 cases]. / Síndrome SAPHO: estudio de 16 casos.

The SAPHO syndrome is characterized by synovitis, acne, palmo-plantar pustulosis, hyperostosis and osteitis. SAPHO syndrome has been occasionally described in Spain. We present our experience of 16 cases with the SAPHO syndrome diagnosed between 1984-1995. The predominant clinical symptoms were: anterior thoracic pain (14 cases), sacroiliac pain (7 cases) and peripheral arthritis (2 cases). Cutaneous involvement was characterized by palmo-plantar pustulosis (8 cases) acne (3 cases) and psoriasis (2 cases). The histocompatibility antigen B27 was negative in all cases. A computerized tomographic study revealed involvement of sterno-costo-clavicular and manubriosternal joints (14 cases) and sacroiliitis (8 cases).

[Spinal cord compression: an unusual clinical manifestation of retroperitoneal fibrosis]. / Síndrome de compresión medular: una rara forma de presentación de la fibrosis retroperitoneal.

INTRODUCTION: Retroperitoneal fibrosis is a rare disorder consisted of a periaortic fibrous mass that compresses retroperitoneal organs. CASE REPORT: We report a case of retroperitoneal fibrosis diagnosed after a spinal cord compression. Magnetic resonance imaging scan showed a prevertebral mass which affected a vertebral body. It extended to the epidural space and compressed the spinal cord. The final diagnosis was established through the mass biopsy by means of mediastinoscopy. After prednisone treatment, both clinical remission and mass reduction in the follow-up MRI were observed. CONCLUSIONS: The spinal cord compression is an extremely unusual initial manifestation of retroperitoneal fibrosis. Nevertheless, it should be considered in the differential diagnosis of spinal cord compression syndrome, specially given the possibility of its medical treatment instead of aggressive surgical treatment.