Differences between radiographic and non-radiographic axial spondyloarthritis patients in a Mexican cohort.

To compare the demographic, clinical, and laboratory characteristics, disease onset, and clinical features of radiographic axial Spondyloarthritis (r-axSpA) and non-radiographic axial Spondyloarthritis (nr-axSpA) patients. All patients who attended outpatient spondylarthritis (SpA) clinics at Hospital General de Mexico and the Instituto Nacional de la Nutrición from 1998 to 2005 and met the rheumatologist diagnostic criteria for SpA were selected. Then the SpA patients were classified by European Spondyloarthropathy Study Group criteria (ESSG). We selected SpA patients with axial presentation as axial SpA (axSpA), and they were classified as r-axSpA if they met modified New York (mNY) criteria for sacroiliitis and as nr-axSpA if they did not meet mNY criteria; to compared clinical, demographic, and laboratory test between the subgroups. It included 148 SpA patients; 55 (37.2%) patients had r-axSpA, and 70 (47.3%) had nr-axSpA. The nr-axSpA patients had a lower proportion of males (58.6% vs 78.2%, P < 0.05), lower HLA-B27 frequency (54.3%. vs. 92.7%, P < 0.05), were older at disease onset (21 vs 16 years; P < 0.01) and had a higher frequency of infections at disease onset (9.1% vs 32.9, P < 0.05) than r-axSpA. BASFI (2.9 vs 4.8; P < 0.0001), Dougados functional index (7 vs. 14; P < 0.05), and BASDAI (4.1 vs. 5.2; P < 0.001) were lower in patients with nr-axSpA than r-axSpA, respectively. The factors that most influenced the presentation of r-axSpA were history of uveitis (OR 14, 95% CI 2.3-85), HLA-B27 (OR 7.97, 95% CI, 2.96-122), male sex (OR 6.16, 95% CI, 1.47-25.7), axial enthesopathy count (OR 1.17 95% CI, 1.03-1.33). This study provides insight into the differences between nr-axSpA and r-axSpA in Mexico. Patients with r-axSpA were mainly male, with a younger presentation age, a higher prevalence of HLA-B27, more history of uveitis, fewer episodes of dactylitis, more axial enthesopathy, and higher disease activity than nr-axSpA.

A Practical Overview of the Articular Manifestations of Systemic Lupus Erythematosus.

Although it is widely known that joint involvement is the most frequent and prevalent manifestation of systemic lupus erythematosus (SLE), not having a validated organ-specific index for this domain in order to guide its treatment has been a major limitation. In addition, its clinical importance had been underestimated since it was not a vital risk domain; it was never the center of treatment, under the premise that in most cases its progression was slow and did not lead to significant functional disability. However, this concept has been changing due to the greater description of erosions both in ultrasonography and in osteoarticular magnetic resonance, so their identification can establish a more appropriate treatment time and thus avoid joint deformities, which in some cases can become irreversible. Recently, anifrolumab and belimumab have been able to significantly reduce the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and British Isles Lupus Assessment Group (BILAG) index scores, along with improvement in quality of life indices and a significant decrease in the required dose of glucocorticoids. Despite this, the ideal moment to consider biological therapy in this domain is not clear, since the clinical examination can sometimes be biased by the pain associated with fibromyalgia or the fatigue associated with SLE. For this reason, perhaps ultrasonography or magnetic resonance imaging, apart from differentiating the joint phenotype, can identify patients in time to define the onset of disease-modifying antirheumatic drugs and rationalize the use of glucocorticoids. The objective of this review is to characterize in detail the joint manifestations of SLE to offer the clinician a practical view of its diagnosis and treatment.

Causes of death in patients with autoimmune and rheumatic diseases-a 16-year autopsy-based study.

Introduction: the autopsy is an essential medical procedure; however, its use has declined over the decades. In autoimmune and rheumatological diseases, anatomical and microscopic diagnosis is critical to diagnose of the cause of death. For this reason, our objective is to describe the cause of death in patients diagnosed with autoimmune and rheumatic diseases who underwent an autopsy in a Pathology reference center in Colombia. Materials and methods: a retrospective and descriptive study of autopsy reports. Results: between January 2004 and December 2019, 47 autopsies of patients with autoimmune and rheumatological diseases were performed. Systemic lupus erythematosus and rheumatoid arthritis were the most common diseases. The leading cause of death was related to infections, being opportunistic infections in the majority of the cases. Conclusions: our autopsy-based study was focused on patients with autoimmune and rheumatological conditions. Infections are the leading cause of death, particularly opportunistic infections, diagnosed mainly by microscopy. Thus, the autopsy should continue to be considered the "gold standard" to determine the cause of death in this population.

Interstitial Lung Disease in Mixed Connective Tissue Disease: An Advanced Search.

The spectrum of pulmonary manifestations associated with mixed connective tissue disease ranges from pulmonary hypertension and interstitial lung disease to pleural effusions, alveolar hemorrhage, and complications from the thromboembolic disease. Interstitial lung disease in mixed connective tissue disease is a frequently occurring entity, although in most cases it tends to be self-limited or slowly progressive. Despite this, a significant percentage of patients may present a progressive fibrosing phenotype, thus posing a great challenge regarding its therapeutic approach, given the scarcity of clinical studies that compare the efficacy of immunosuppressants available to date. Due to this, many recommendations are extrapolated from other diseases with similar characteristics such as systemic sclerosis and systemic lupus erythematosus. That is why it is proposed to carry out an advanced search of the literature in order to clarify its clinical, radiological, and therapeutic characteristics to achieve its evaluation from a holistic point of view.

Clinical Diagnosis of Chikungunya Infection: An Essential Aid in a Primary Care Setting Where Serological Confirmation Is Not Available.

BACKGROUND: Chikungunya virus (CHIKV) diagnosis has become a challenge for primary care physicians in areas where the Zika virus and/or Dengue virus are present. Case definitions for the three arboviral infections overlap. METHODS: A cross-sectional analysis was carried out. A bivariate analysis was made using confirmed CHIKV infection as the outcome. Variables with significant statistical association were included in an agreement consensus. Agreed variables were analyzed in a multiple regression model. The area under the receiver operating characteristic (ROC) curve was calculated to determine a cut-off value and performance. RESULTS: 295 patients with confirmed CHIKV infection were included. A screening tool was created using symmetric arthritis (4 points), fatigue (3 points), rash (2 points), and ankle joint pain (1 point). The ROC curve identified a cut-off value, and a score ≥ 5.5 was considered positive for identifying CHIKV patients with a sensibility of 64.4% and a specificity of 87.4%, positive predictive value of 85.5%, negative predictive value of 67.7%, area under the curve of 0.72, and an accuracy of 75%. CONCLUSION: We developed a screening tool for CHIKV diagnosis using only clinical symptoms as well as proposed an algorithm to aid the primary care physician.

Causes of death in patients with autoimmune and rheumatic diseases—a 16-year autopsy-based study

ABSTRACT Introduction the autopsy is an essential medical procedure; however, its use has declined over the decades. In autoimmune and rheumatological diseases, anatomical and microscopic diagnosis is critical to diagnose of the cause of death. For this reason, our objective is to describe the cause of death in patients diagnosed with autoimmune and rheumatic diseases who underwent an autopsy in a Pathology reference center in Colombia. Materials and methods a retrospective and descriptive study of autopsy reports. Results between January 2004 and December 2019, 47 autopsies of patients with autoimmune and rheumatological diseases were performed. Systemic lupus erythematosus and rheumatoid arthritis were the most common diseases. The leading cause of death was related to infections, being opportunistic infections in the majority of the cases. Conclusions our autopsy-based study was focused on patients with autoimmune and rheumatological conditions. Infections are the leading cause of death, particularly opportunistic infections, diagnosed mainly by microscopy. Thus, the autopsy should continue to be considered the "gold standard" to determine the cause of death in this population.

Perimyocarditis With Acute Heart Failure as the First Manifestation of Systemic Lupus Erythematosus.

Cardiac abnormalities are common in patients with systemic lupus erythematosus (SLE). However, many of them tend to be mild or asymptomatic and can be recognized by non-invasive studies such as transthoracic echocardiography and cardiac magnetic resonance imaging (CMR). However, heart failure secondary to perimyocarditis as the initial manifestation of SLE remains an extremely rare form of presentation. Below, we present the case of an adult female patient who initially consulted due to symptoms of acute dyspnea, atypical chest pain, and edema of the lower limbs, who underwent a chest X-ray as part of the initial studies, which described an increase in the cardiac silhouette associated with diffuse opacities in both lung fields. The admission electrocardiogram only showed sinus tachycardia and nonspecific alterations of the T wave, with an initial report of frankly elevated cardiac biomarkers compatible with acute myocardial injury together with the positivity of specific antibodies for SLE.

A Practical Perspective of the Hematologic Manifestations of Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease with an unknown etiology that can affect any organ or system of the human body. Hematological, renal, or central nervous system manifestations in these patients result in great morbidity because high doses of glucocorticoids, cytotoxic medications, or biological drugs are required to control these manifestations. It is noteworthy that hematological involvement predominates during the first years of the disease and tends to last over time, with the premise that it may be the initial manifestation of the disease. Clear examples of this are the cases of hemolytic anemia and immune thrombocytopenia that can be initially classified as idiopathic or primary to be later classified as secondary when associated with infections, medications, neoplasms, or autoimmune diseases. The spectrum of hematologic manifestations in SLE is very broad, including lymphopenia, anemia, thrombocytopenia, or pancytopenia. In some cases, lymphadenopathy and splenomegaly are also identified. The vast majority of these manifestations denote high disease activity. However, many of these alterations have a multifactorial cause that must be taken into account to adopt a more complete therapeutic approach. The objective of this review is to characterize in detail the hematological manifestations of SLE to offer clinicians a practical vision of its diagnosis and treatment.

Obstetric Antiphospholipid Syndrome From the Perspective of a Rheumatologist.

Antiphospholipid syndrome (APS) is an autoimmune disease that can lead to thrombotic or obstetric complications. Recent histopathological studies have shown the absence of placental thrombosis, leading to the consideration of other pathophysiological pathways such as inflammation and complement activation. Due to this, various clinical studies are being carried out with different drug agents in order to avoid their complications. The combination of prophylactic heparin treatment and low doses of aspirin today result in successful pregnancies in most cases. Despite this, a minority of patients require alternative therapies to avoid recurrent miscarriage and decrease obstetric morbidity. Thanks to the better understanding of its pathophysiology, other treatments such as low doses of glucocorticoids, hydroxychloroquine (HCQ), immunoglobulin, pravastatin, and plasmapheresis have been considered in refractory cases, achieving favorable results. Despite the great advances regarding its treatment, unfortunately, there are no treatments with a good level of evidence to reduce late obstetric complications. The evaluation of preconception risk factors, as well as the antiphospholipid antibody profile, is necessary to establish individual risk and thus anticipate possible complications.

HIV and COVID-19 Coinfection: A Synergism That Results in More Severe Forms of Reactive Arthritis.

Reactive arthritis is defined as arthritis that arises after infection, where pathogens cannot grow in the affected joints. Although the human immunodeficiency virus and severe acute respiratory syndrome coronavirus 2 are not among the most commonly implicated pathogens, there is growing evidence that they have major implications in the genesis of reactive arthritis. However, there are no described cases of coinfection of both entities that cause reactive arthritis at the same time, and the alterations involved in the immune system that could cause the change of certain clinical characteristics to more severe forms of the disease are unknown. The following describes the case of a male patient in his third decade of life who has an unusual and severe presentation of reactive arthritis associated with coinfection by COVID-19 and the human immunodeficiency virus.

Extra-Articular Manifestations in Reactive Arthritis due to COVID-19.

Reactive arthritis (ReA) is defined as arthritis that arises after infection, where pathogens cannot grow in the affected joints. Formerly, the clinical triad of postinfectious arthritis, urethritis, and conjunctivitis was called Reiter's syndrome; however, these clinical signs only represented a subset of patients with ReA. Due to the great diversity of its manifestations, its diagnosis is a challenge and can be overlooked in clinical practice. Additionally, it is associated with a variety of extra-articular manifestations that may be present either in the acute or chronic phase of the disease. Despite the cardinal clinical presentation characteristics of ReA, no case has been described in the literature that is diagnosed by the presence of classic extra-articular manifestations without objective joint involvement after COVID-19 infection. This report describes the case of a female patient in her third decade of life with an unusual presentation of ReA and focuses on her extra-articular manifestations.

Aortitis: An Unusual Inflammatory Complication of Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can involve any organ system. Vasculitides are classified according to the predominant vessel involved such as large vessel, medium vessel, or small vessel vasculitis. Of these, Takayasu arteritis, Behcet's disease, relapsing polychondritis, and immunoglobulin G4 (IgG4)-related disease predominantly involve large vessels. The most common form of vasculitis seen in SLE is small vessel vasculitis. Aortitis in SLE is an extremely rare complication. This is a case report of a 21-year-old female patient with a history of SLE with overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis associated with antiphospholipid syndrome (APS), who presented with a one-week history of left-back burning lumbar pain, radiating to the flank, which increased with changes in position associated with intermittent claudication. In the angiography images and the positron emission tomography (PET) scan, a hypometabolic left para-aortic oval image was noted, corresponding to the presence of a contained hematoma in an abdominal aorta rupture. Later, she underwent vascular surgery and hemodynamics, performing thoracoabdominal aortic reconstruction together with aortorenal bypass and left nephrectomy. Pathology fundings of the left kidney correspond with class IV lupus nephritis, and the resection sample of the thoracoabdominal aneurysm showed a marked thinning and fragmentation of elastic fibers, areas of fibrosis of the wall with severe IgG4 negative lymphoplasmacytic infiltrate in the immunohistochemical study, establishing the diagnosis of aortitis.

Lupus Cystitis, From Myth to Reality: A Narrative Review.

Systemic lupus erythematosus is a multisystemic disease that usually involves the urinary tract, often in the form of lupus nephritis. However, another form of compromise of this system is lupus cystitis, which, despite being an unusual condition, turns out to be a challenging diagnosis due to the spectrum of nonspecific abdominal and urinary symptoms. Although the exact pathophysiological mechanism of bladder inflammation remains to be established, the role of small vessel vasculitis measured by immune complexes continues to be supported as a central axis for considering possible therapeutic targets. Additionally, there are no clinical studies that dictate a guideline regarding its treatment, however, the evidence from most cases described in the literature suggests the initiation of pulses of methylprednisolone and cyclophosphamide in treatment regimens similar to those of lupus nephritis. Despite its low prevalence, obstructive complications and kidney damage can lead to increased morbidity and mortality.

Current Evidence for IL-17/23 Blockade for the Treatment of Lupus Nephritis.

In the current medical literature, there is increasing evidence for the involvement of the interleukin (IL)-17/23 axis and the potential role of Th17 cells in the pathogenesis of lupus nephritis. Knowledge about the interaction of these immunological pathways in the development of autoimmune diseases has led to the identification of new therapeutic strategies aimed at blocking them. For this reason, the main objective of this review focuses on knowing the recent evidence of the different anti-IL-17/23 treatment strategies in lupus nephritis and their future perspectives. A non-systematic narrative review of the literature was carried out following the objective of having the most representative information on the different anti-IL-17/23 drugs available together with the description of the pathophysiological mechanisms of this pathway involved in systemic lupus erythematosus and lupus nephritis. Despite the great existing theoretical foundation, today few clinical studies support the use of these therapies in both contexts. Nevertheless, the publication of research with a better methodology is expected to approve the indication of some of these drugs in lupus nephritis. However, the clinical response seen with ustekinumab and secukinumab in clinical studies and case reports published to date has been encouraging.

Terapia biológiaa en patología pulmonar: parte i generalidades y clasificaciónn parte ii: terapias específicas en asma, epoc, enfermedad pulmonar parinquematosa difusa, cáncer pulmonar / Terapiabiológicaa em patología pulmonar / Terapia biológica en patología pulmonar parte I: generalidades y clasificación parte II: terapias específicas en asma, EPOC, enfermedad pulmonar parinquematosa difusa, cáncer pulmonar

El avance de la investigación médica, en los campos de la biología molecular y la ingeniería genética, ha traído consigo el desarrollo de una serie de nuevos medicamentos dirigidos a bloquear diferentes vías de la respuesta inmune celular. La terapia biológica, nombre con el cual se reconoce a estos nuevos medicamentos, ofrece una nueva oportunidad terapéutica para el manejo de enfermedades crónicas progresivas. En las enfermedades pulmonares crónicas como el asma, la enfermedad pulmonar obstructiva crónica (EPOC), la enfermedad pulmonar parenquimatosa difusa (EPPD) y el cáncer de pulmón, el tratamiento con medicamentos biológicos ha aportado importantes avances para comprender con mayor claridad estas enfermedades y en algunos casos gracias a la eficacia de los mismos, mejorar la calidad de vida de los pacientes que las presentan. Debido al número cada vez mayor de medicamentos de terapia biológica y su aplicación terapéutica creciente en enfermedades inflamatorias crónicas y cáncer, creemos necesario revisar su estado actual en el manejo de la patología pulmonar crónica.

The advancement of medical research in molecular biology and genetic engineering has given rise to the development of new drugs aimed at blocking different pathways of cellular immune responses. Biological therapy is a new therapeutic option for progressive chronic disease management. In chronic lung diseases such as asthma, chronic obstructive pulmonary disease (COPD), diffuse parenchymal lung disease (DPLD) and lung cancer, treatment with biologics has made important advances in the understanding of these diseases, and in some cases, due to their effectiveness, has contributed to the improvement in life quality of patients who suffer them. Due to the increasing number of biological therapy drugs and their therapeutic application in chronic inflammatory diseases and cancer, it is relevant to review their current status in the management of chronic lung diseases.

O avanço da pesquisa médica, nos campos da biologia molecular e da engenharia genética, trouxe consigo o desenvolvimento de uma série de novos medicamentos dirigidos a bloquear diferentes vias da resposta imune celular. A terapia biológica, nome com o qual são conhecidos estes novos medicamentos, oferece uma nova oportunidade terapêutica para o tratamento de doenças cônicas progressivas. Nas doenças pulmonares crônicas como a asma, a doença pulmonar obstrutiva crônica (DPOC), a doença parenquimatosa difusa pulmonar (DPDP) e o câncer de pulmão, o tratamento com medicamentos biológicos tem contribuído com importantes avanços para compreender com maior claridade estas doenças e em alguns casos graças à eficácia dos mesmos, melhorar a qualidade de vida dos pacientes que as apresentam. Devido ao número cada vez maior de medicamentos de terapia biológica e sua aplicação terapêutica crescente em doenças inflamatórias crônicas e câncer, acreditamos que é necessário revisar seu estado atual no tratamento da patologia pulmonar Crônica.