Investigating the concomitance of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides and inflammatory bowel disease (IBD).

OBJECTIVE: To assess relationship between Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis and inflammatory bowel disease (IBD). METHODS: This is a retrospective study design. The patients were identified using a preset criteria of patients who have the diagnosis of ANCA associated vasculitis including a diagnosis of granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) or eosinophilic granulomatosis with polyangiitis (EGPA) with overlapping inflammatory bowel disease (Crohn's disease or ulcerative colitis) in the time period from 01/01/2020 to 08/03/2023. Subsequently data from each patient was collected that will include baseline demographics, disease characteristics, disease activity, treatment information, multiorgan involvement, and pathology findings which were then analyzed. RESULTS: 39 patients were identified that met criteria. 20 patients carried a diagnosis of GPA, 6 had MPA and 4 patients had EGPA. 20 patients with GPA had inflammatory bowel disease, 13 with ulcerative colitis and 6 with Crohn's disease while 1 GPA patient had unspecified inflammatory bowel disease. 4 patients with EGPA had inflammatory bowel disease, 2 with ulcerative colitis and 2 with Crohn's disease. 6 patients with MPA had inflammatory bowel disease, 4 with ulcerative colitis and 2 with Crohn's disease. IBD diagnosis preceded the diagnosis of ANCA vasculitis in 77.8 % of the cases. CONCLUSION: Objective observation and deductions from this study raise the concern for a possible pathogenic association of ANCA associated vasculitis and inflammatory bowel disease and more research is needed to identify any causal association or influence of the two systemic disease on each other.

Nailfold videocapillaroscopy in antineutrophil cytoplasmic antibody-associated vasculitis.

OBJECTIVE: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of illnesses that cause inflammation and alterations to small vessels in the body. Some of the most common and detrimental manifestations, including alveolar hemorrhage and glomerulonephritis, are caused by this capillary inflammation. We sought to clarify whether patients with AAV would have abnormal nailfold capillaries when evaluated with nailfold videocapillaroscopy. METHODS: Patients with a current diagnosis of AAV and a control group were identified for enrollment. Nailfold videocapillaroscopy images were used for a semiquantitative analysis on capillary density, morphology, dilation, and microhemorrhage after review by 2 rheumatologists. Disease characteristics, occurrence of recent disease flare, and presence of ANCA were recorded. RESULTS: Thirty-three patients with a diagnosis of AAV and 21 controls were recruited. The AAV group had a median age of 59 and 17 (52%) were women. Granulomatosis with polyangiitis was the most common diagnosis (19 [58%]), followed by eosinophilic granulomatosis with polyangiitis (7 [21%]) and microscopic polyangiitis (7 [21%]). Twenty-seven patients (82%) had positive ANCA tests. After assessment of capillary density, dilation, morphology, microhemorrhages, and disorganization, there were no statistically significant differences between the 2 groups. CONCLUSION: There was no evidence of differences in nailfold capillaroscopy abnormalities between those diagnosed with AAV and the control group. While this cohort was relatively small, we did not find a high enough prevalence or specific phenotype of capillary abnormalities that could aid in diagnosis or prognostication of these diseases in the clinical setting.

Central Retinal Artery Occlusion Associated with Takayasu Arteritis.

Takayasu arteritis is a chronic inflammatory vasculitis with granulomatous panarteritis particularly impacting large vessels including the aorta and its branches, especially the subclavian arteries, with clinical manifestation dependent on the involved artery. Sequelae of the active disease vary, including stenosis, occlusions, or aneurysmal dilatations of the large vessels. The prevalence of Takayasu arteritis is higher in the Asian population and in Japan, but quite low in the United States, varying from 0.9-8.4 per million people. Ocular manifestations are rare and lead to a delay in diagnosis and appropriate treatment. Ocular manifestations include Takayasu retinopathy, anterior ischemic optic neuropathy (AION), retinal artery occlusion (RAO) and retinal vein occlusion (RVO). We present two cases in which central retinal artery occlusion (CRAO) was associated with Takayasu arteritis. CRAO is an ophthalmic emergency with an incidence of 1.9 per 100,000 person years in the United States; only 5% of cases are arteritic, which can be observed with inflammatory vasculitides secondary to the formation of immune deposits.

Bilateral Lumbosacral Plexopathy As the Initial Manifestation of Systemic Sarcoidosis: A Case Report.

Neurosarcoidosis is one of the most relevant involvements in systemic sarcoidosis and can be the initial presentation. Its diagnosis is often considered difficult because of unusual clinical manifestations or diagnostic mimics. The peripheral nervous system is less frequently involved than the central nervous system, although it may also lead to irreversible neurologic impairment. Lumbosacral plexopathy in sarcoidosis is a rare presentation and has been scarcely described in anecdotal case reports and small case series. We describe the case of a 61-year-old female who presented with right inguinal pain, right thigh weakness, and gait limitation, with imaging evidence of bilateral lumbosacral plexopathy as the initial manifestation of systemic sarcoidosis and subsequently developed joint and pulmonary involvement. This case report aims to bring awareness of this involvement as a possible initial manifestation of systemic sarcoidosis and mention key features of the differential diagnosis. Prompt recognition and treatment may prevent neurologic impairment.

Treatment of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis With Diffuse Alveolar Hemorrhage With Avacopan.

OBJECTIVE: Avacopan, an activated complement factor 5 receptor antagonist, has been approved as adjunct therapy for severe active antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Current evidence supports the management of AAV presenting with diffuse alveolar hemorrhage (DAH) by administering glucocorticoids combined with either rituximab or cyclophosphamide in addition to supportive care. The role of avacopan in patients with DAH as a primary severe disease manifestation of AAV has not been well established. Furthermore, concerns remain regarding timely access to avacopan, the best glucocorticoid tapering regimen, and long-term efficacy and safety of the drug. We sought to identify clinical features and outcomes of patients presenting with DAH secondary to AAV who received avacopan in addition to glucocorticoids and rituximab or cyclophosphamide. METHODS: We performed a retrospective cohort study of all consecutive patients presenting with DAH as part of active severe granulomatosis with polyangiitis or microscopic polyangiitis. Demographic and clinical characteristics were collected at presentation and follow-up. RESULTS: Fifteen patients met inclusion criteria and were observed for a median time of 17 weeks (interquartile range [IQR] 6-37 weeks) after initiation of avacopan. Patients were predominantly female and White, had never smoked, and were a median age of 66 years (IQR 52-72 years) at diagnosis. The majority had newly diagnosed severe AAV with renal involvement. Three patients progressed to respiratory failure. The timing of avacopan introduction and patterns of glucocorticoid tapers varied widely in this cohort. Two serious adverse events related to infection were observed, including one opportunistic infection leading to the patient's death, although neither was directly attributed to avacopan administration. CONCLUSION: We describe the clinical course of patients who presented with the severe AAV disease manifestation of DAH and received avacopan as adjunct therapy. Most patients achieved remission during follow-up, and adverse events, including infection, were observed.

A retrospective analysis of the prevalence and impact of associated comorbidities on fibromyalgia outcomes in a tertiary care center.

Background: This retrospective study was designed to analyze the prevalence and impact of associated comorbidities on fibromyalgia (FM) outcomes (functionality, pain, depression levels) for patients who participated in an intensive multicomponent clinical program in a tertiary care center. Methods: Participants included a sample of 411 patients diagnosed with FM at a large tertiary medical center using the 2016 ACR criteria. Patients completed an intensive 2-day cognitive behavioral treatment (CBT) program, filled out the Fibromyalgia Impact Questionnaire Revised (FIQR), the Center for Epidemiologic Studies Depression Scale (CES-D), the Pain Catastrophizing Scale (PCS), and were followed for 6 months after treatment completion. T-tests were performed to analyze differences between the presence or absence of select comorbidities for the three outcomes at follow-up. Statistically significant comorbidities (p < 0.05) were used as predictors in multivariable logistic regression models. Results: The FM associated comorbidities in this cohort that had significant impact on the measured outcome domains after treatment program completed were Obesity (FIQR p = 0.024), Hypothyroidism (CES-D p = 0.023, PCS p = 0.035), Gastroesophageal reflux disease GERD (PCS p < 0.001), Osteoarthritis (CES-D p = 0.047). Interestingly, Headache, the most frequent FM associated comorbidity in this cohort (33.6%), did not have a significant impact on the outcome domains at follow-up. Obesity (18.2%) was the only FM associated comorbidity significantly impacting all three outcome domains at follow-up. Conclusion: The present study suggests that addressing obesity may significantly impact outcomes in FM patients.

Interstitial lung involvement in systemic sclerosis / Compromiso intersticial pulmonar en la esclerosis sistémica

ABSTRACT Introduction: Systemic sclerosis can involve the lung parenchyma leading to serious complications and even death. Objectives: To describe the most relevant aspects of interstitial lung disease related to systemic sclerosis emphasizing diagnosis and treatment. Materials and methods: A literature review was performed searching in the databases Medline and EMBASE using the MeSH terms «Scleroderma, Systemic", «Lung Diseases, Interstitial¼ and «Pulmonary Fibrosis¼ Results and conclusions: Interstitial lung disease is a common clinical manifestation of systemic sclerosis and one of the main causes of death. Treatment options are limited and have a modest effect in most of the cases.

RESUMEN Introducción: La esclerosis sistémica puede potencialmente comprometer el parénquima pulmonar, llevando a serias complicaciones e incluso a la muerte. Objetivos: Describir los aspectos más relevantes en cuanto a las generalidades de la enfermedad pulmonar intersticial en esclerosis sistémica, su diagnóstico y su tratamiento. Materiales y métodos: Se realizó una búsqueda de literatura en las bases de datos Medline y EMBASE utilizando los términos MeSH «Scleroderma, Systemic¼, «Lung Diseases, Interstitial¼ y «Pulmonary Fibrosis¼. Resultados y conclusiones: La enfermedad pulmonar intersticial es una manifestación frecuente de la esclerosis sistémica y una de las principales causas de muerte en los pacientes que la padecen. Las opciones terapéuticas son limitadas y su efecto es, en muchos casos, modesto.

Enfermedad relacionada con IgG4: revisión concisa de la literatura / IgG4-related disease: a concise review of the current literature

La enfermedad relacionada con IgG4 (ER-IgG4) es una entidad recientemente nominada para definir diversas enfermedades caracterizadas por infiltración linfoplasmocítica, fibrosis, presencia de un número aumentado de células IgG4+ y, en gran parte de los casos, niveles aumentados de IgG4 sérica, afectando frecuentemente el páncreas, las glándulas salivales y los ganglios linfáticos pero pudiendo comprometer casi cualquier estructura de la anatomía humana. Aunque su etiología se desconoce, se han realizado avances en el conocimiento de sus bases fisiopatológicas e inmunológicas, al igual que del rol de las células inflamatorias en el desarrollo de daño del órgano blanco. No existe hasta la fecha un consenso internacional sobre su diagnóstico, lo que no ha impedido avances terapéuticos muy importantes en su control y búsqueda de remisión. Se hace una revisión acerca de la historia, hipótesis sobre la etiología de la enfermedad, sus manifestaciones clínicas, abordaje diagnóstico y terapéutico (AU)

IgG4-related disease is the term used to refer to a condition characterized by a lymphoplasmacytic infiltrate, fibrosis and an increased number of IgG4+ cells present in tissue, in most cases, with an elevated serum IgG4 level. This disease frequently affects the pancreas, salivary glands and lymph nodes, but can involve almost any tissue. Its etiology and the exact role of the different inflammatory cells in the damage to the target organ is still unclear. As yet, there is no international consensus about diagnostic criteria for the disease, but there are important advances in its treatment and in the quest to achieve remission. We include a review of the history, possible pathogenesis, clinical manifestations, diagnostic approach and available therapeutic approaches (AU)

Sonoanatomía de la rodilla / Knee sonoanatomy

La rodilla es una región anatómica vasta en estructuras que pueden ser afectadas por diversas enfermedades de etiología diversa. La exploración física no siempre brinda un diagnóstico certero. La ultrasonografía es una técnica de imagen que ha demostrado validez y reproducibilidad para el estudio de la rodilla tanto para complementación diagnóstica como para evaluar la respuesta al tratamiento en varias enfermedades, así como servir de guía para aspiración o infiltraciones. Por ello es importante conocer la anatomía y la exploración por ultrasonografía de la región.

The knee is a vast region in anatomical structures that may be affected by various diseases of diverse etiology. Physical examination does not always provide an accurate diagnosis. Ultrasonography is an imaging technique that has proven validity and reproducibility for the study of the knee for both diagnostic and complementation to assess response to treatment in various diseases and to guide aspiration or infiltration. It is therefore important to know the anatomy and U.S. exploration of the region.