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Resumen La fibrosis de la medula osea es una enfermedad rara que se puede ver asociada con enfermedades autoinmunes como el lupus eritematoso sistemico (LES) y que puede llegar a ser confundida con las manifestaciones propias de la enfermedad. Se presenta el caso de una paciente con mielofibrosis autoinmune en el contexto de LES.

Intravenous immunoglobulin (IVIg) is an anti-inflammatory drug with an unclear role in the treatment of patients with lupus nephritis (LN). This systematic review evaluates the evidence for IVIg in the care of patients with LN.A systematic search was done in the PubMed, EMBASE, BVS and OVID databases - All EBM Reviews following the PRISMA methodology (registration in PROSPERO CRD42021236662). The variables were extracted: indications for use, dosage, partial or complete response, adverse reactions, initiation of renal replacement therapy, reduction of proteinuria, and mortality. The quality assessment was done with the "The Joanna Briggs Institute (JBI) Critical Appraisal tools for use in Systematic Reviews Checklist". In addition, synthesis reports were prepared through the Synthesis Without Meta-analysis - SWiM guide.A total of 2328 articles were obtained (28 were considered for inclusion). When the studies were evaluated, IVIg therapy was found to be between 60% to 70% effective (except for patients with class V LN) with overall responses (complete + partial) even for patients who are refractory to first line treatment. Normalization (0.5 g) of nephrotic proteinuria occurred in 24% of cases with infrequent adverse events and a mortality plus dialysis composite of 11.5% and 24.1% (most representative study).In patients with LN refractory to conventional treatment or co-infection situations, the reported data seem to demonstrate effectiveness of IVIg therapy. There are few adverse reactions and caution is exercised when using it on patients with class V NL. However, given the lack of controlled studies with long-term follow-up, these data should be interpreted cautiously thus encouraging the development of high-quality RCTs.

RESUMEN Introducción: El síndrome de Sjögren (SS) es una enfermedad autoinmune, con compromiso multisistémico, especialmente de la función de las glándulas exocrinas. Los pacientes cursan con una alta carga de la enfermedad que compromete su calidad de vida, asociado sobre todo a fatiga crónica, ansiedad, depresión y disfunción sexual, entre otras dificultades. Objetivo: Describir las generalidades de las principales escalas (específicas y no específicas) para la evaluación de la calidad de vida en pacientes con SS. Métodos: Se realizó una revisión narrativa y ampliada de la literatura. Se hizo una búsqueda en las diferentes bases de datos (PubMed, Embase, Cochrane, BVS) y de literatura gris, seleccionando los artículos relacionados con el tema y realizando una extracción de datos de estos. Resultados: Se encontraron 3 escalas realizadas específicamente para pacientes con SS: Sjögren’s Syndrome Patient Reported Index, Psychometric Testing of the Primary Sjögren’s Syndrome Quality of Life Questionnaire y Profile of Fatigue and Discomfort, y 14 escalas realizadas en el contexto de otras enfermedades (no específicas), utilizadas en pacientes con SS. Conclusiones: Como en otras afecciones, en pacientes con SS es importante la evaluación de la calidad de vida. A tal efecto se cuenta con múltiples escalas, a parte de las 3 citadas, elaboradas específicamente para este tipo de pacientes, sin dejar de lado las escalas no específicas, como el Beck Depression Inventory y el Ocular Surface Disease Index, que tienen su mayor utilidad en la evaluación específica de síntomas asociados a esta enfermedad. Resultados: Se encontraron 3 escalas realizadas específicamente para pacientes con SS: Sjögren’s Syndrome Patient Reported Index, Psychometric Testing of the Primary Sjögren’s Syndrome Quality of Life Questionnaire y Profile of Fatigue and Discomfort, y 14 escalas realizadas en el contexto de otras enfermedades (no específicas), utilizadas en pacientes con SS. ABSTRACT Introduction: Sjögren’s syndrome (SS) is an autoimmune disease, with multisystemic invol- vement, particularly compromising the function of exocrine glands. The patients have a high burden of the disease associated with chronic fatigue, anxiety, depression, sexual dysfunction, among others. Objective: To describe the generalities of the main scales (specific and non-specific) for the evaluation of quality of life in patients with SS. Methods: A narrative review and expanded review was performed, including a search conducted in the different databases (PubMed, Embase, Cochrane, VHL) and grey literature. Articles related to the topic were selected and data extracted from them. Results: Three scales were developed specifically for patients with SS, and included Sjögren’s Syndrome Patient Reported Index, Psychometric Testing of the Primary Sjögren’s Syndrome Quality of Life Questionnaire, and Profile of Fatigue and Discomfort, and 14 scales performed in context of other diseases (non-specific) used in patients with SS. Conclusions: As is the case in other diseases, it is important to evaluate the quality of life in patients with SS. There are multiple scales for this evaluation, including the 3 scales mentioned before, specifically developed for these types of patients. The non-specific scales worth noting are, the Beck Depression Inventory and the Ocular Surface Disease Index, having their greatest use in the specific evaluation of symptoms associated with this disease.

espanolAntecedentes No es claro si los pacientes con algun grado de inmunosupresion tienen peores desenlaces en la infeccion por SARS-CoV-2, en comparacion con la poblacion sana. Objetivo Realizar una revision narrativa de la informacion disponible sobre infeccion por SARS-CoV-2 en pacientes inmunosuprimidos, especialmente pacientes con cancer, trasplantados, con patologias neurologicas, inmunodeficiencias primarias y secundarias. Resultados Los pacientes con cancer y tratamiento reciente del mismo (quimioterapia o cirugia) e infeccion por SARS-CoV-2 tienen mayor riesgo de peores desenlaces. En los pacientes trasplantados (renal, cardiaco y hepatico), con patologias neurologicas (esclerosis multiple [EM], neuromielitis optica [NMODS], miastenia grave [MG]), inmunodeficiencias primarias e infeccion por virus de inmunodeficiencia humana (VIH) en asociacion con uso de inmunosupresores, los estudios no han mostrado tendencia a peores desenlaces. Conclusion Dada la poca evidencia con que contamos hasta el momento no es claro el comportamiento de la infeccion por SARS-CoV-2 en pacientes con inmunosupresion, pero los estudios actuales no han mostrado peores desenlaces en este tipo de pacientes, a excepcion de los pacientes con cancer. EnglishBackground It is not clear whether patients with some degree of immunosuppression have worse outcomes in SARS-CoV-2 infection, compared to healthy people. Objective To carry out a narrative review of the information available on infection by SARS-CoV-2 in immunosuppressed patients, especially patients with cancer, transplanted, neurological diseases, primary and secondary immunodeficiencies. Results Patients with cancer and recent cancer treatment (chemotherapy or surgery) and SARS-CoV-2 infection have a higher risk of worse outcomes. In transplant patients (renal, cardiac and hepatic), with neurological pathologies (multiple sclerosis [MS], neuromyelitis optica [NMODS], myasthenia gravis [MG]), primary immunodeficiencies and infection with human immunodeficiency virus (HIV) in association with immunosuppressants, studies have shown no tendency for worse outcomes. Conclusion Given the little evidence we have so far, the behaviour of SARS-CoV-2 infection in immunosuppressed patients is unclear, but current studies have not shown worse outcomes, except for patients with cancer.

Pachymeningitis is an uncommon disease with multiple etiologies with fibrosis of leptomeninges. One type of this disease is related to immunoglobulin G4 (IgG4). The most frequent manifestations include chronic cephalgia and focal neurological deficits. The case of a 31-year-old man with chronic cephalgia localized to the left hemicranium and left dysmetria is presented. Nuclear magnetic resonance imaging showed image hyperintensity and perilesional edema located at the infratentorial and left parietal occipital levels. The results of cerebral angiography and a cerebrospinal fluid examination were normal. A biopsy of the central nervous system (CNS) ruled out neoplastic lesions, granulomatous lesions, infection, and arteriovenous malformation and indicated thickened meningeal fragments. Elevated serum IgG4 levels were found. The antinuclear antibodies (ANAs), antibodies to extractable nuclear antigens (ENAs), anti-native DNA antibodies (anti-DNA), topoisomerase I antibodies (Scl-70), antibodies to cyclic citrullinated peptides (anti-CCP), and rheumatoid factor (RF) were negative. After an initial diagnosis of idiopathic hypertrophic cranial pachymeningitis, the patient received treatment with high doses of glucocorticosteroids for two years, without response, and developed Cushing's syndrome, cataracts, and osteoporosis. During the follow-up, dactylitis, distal upper limb sclerosis, and Raynaud's phenomenon occurred. A capillaroscopy indicated capillaries with dilated loops, microhemorrhaging, disorganization of the capillary architecture, and megacapillaries, which led to a diagnosis of limited systemic scleroderma. Although an association between scleroderma and pachymeningitis was originally proposed, review of the literature suggests that these are two different entities. Treatment with methotrexate, 25 mg weekly, allowed the withdrawal of glucocorticoids and resolution of neurological symptoms.

8 páginas Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease expressed in genetically predisposed individuals. The interaction of a trigger factor together with the failure of the mechanisms for handling potential reactive autoantigens leads to the development of immune complex responsible for the damage of the target organs. Elements of both innate and adaptive immunity have been implicated in the pathophysiology of the disease, but given the heterogeneity of the SLE, we still do not have a biological marker of activity of the disease with high sensitivity, specificity and predictive value. Recently, a cell population was described as lymphocytes T helper 17 (Th17), so called because of their production of Interleukin 17 (IL-17), cytokine that mediates physiological and pathophysiological processes implicated in the development of inflammatory conditions such as SLE. It has been postulated that serum IL-17 may be a biomarker that meets these parameters; however, data on this topic is still incomplete. We present a review of the ontogenetic mechanisms of Th17 lymphocytes, an explanation of its pathophysiological role in SLE and clinical studies that support and discuss the role of Th17 lymphocytes related cytokines as biomarkers of disease activity in SLE. El lupus eritematoso sistémico (LES) es una enfermedad autoinmune, inflamatoria, crónica, que se desarrolla en individuos genéticamente predispuestos. La interacción de un factor detonante en conjunto con la falla de los mecanismos de depuración de potenciales autoantígenos reactivos, dará lugar a la formación de complejos inmunes responsables del daño tisular de los órganos blanco. En la fisiopatología han sido implicados una variedad de elementos pertenecientes a la inmunidad innata y adaptativa. Una población celular descrita recientemente es la de linfocitos Th17, designados así por la producción de la IL-17; citosina que media procesos fisiológicos y fisiopatológicos; estos últimos responsables del desarrollo de condiciones inflamatorias como las del LES. Dada la heterogeneidad de esta enfermedad, en la actualidad no se cuenta con un biomarcador de actividad lo suficientemente sensible, específico y con un grado de predicción, que permita una confiable toma de decisiones clínicas. Con la intención de suplir dicha carencia, se ha postulado que los niveles séricos de IL-17, pueden ser un biomarcador que cumpla con dichos parámetros. Sin embargo, la información al respecto no es conclusiva. A continuación se presenta una revisión sobre los mecanismos ontogénicos de los linfocitos Th17, la argumentación de su rol fisiopatológico en LES y los estudios clínicos que apoyan y debaten el protagonismo de las citosinas relacionadas con linfocitos Th17 como biomarcadores de actividad de la enfermedad en LES.