Navigating the diagnostic maze: A case presentation of C1q vasculitis mimicking hypocomplementemic urticarial vasculitis in a patient with systemic lupus erythematosus.

In rare instances, patients with SLE may exhibit atypical clinical manifestations, such as Hypocomplementemic Urticarial Vasculitis, which can pose diagnostic challenges. Here, we present a case report of a 28-year-old female with a history of SLE with lupus nephritis clase IV who developed HUV-like symptoms, ultimately leading to a diagnosis of C1q Vasculitis. This case underscores the importance of considering C1q Vasculitis in SLE patients presenting with HUV-like features and highlights Rituximab as a promising therapeutic option for managing this rare condition.

Systemic lupus erythematosus/ANCA-associated vasculitis overlap: An explanation for atypical lupus manifestation.

The coexistence of systemic lupus erythematosus (SLE) and ANCA-associated vasculitis (AAV) as an overlapping syndrome is not common. Here, we report a case of a 33-year-old woman, with recent SLE diagnosis due to skin, kidney, articular, and immunologic compromise, in whom a chest CT scan showed bilateral nodules, consolidations, and tree-in-bud pattern; thoracoscopic lung biopsy revealed diffuse non-caseating granulomas, without other features of sarcoid, organizing pneumonia, or hypersensitivity pneumonitis with high positive p-ANCA titers. Overlap between SLE and AAV was a possible explanation for lupus granulomatous pneumonitis, and for this reason, a multidisciplinary meeting was held to evaluate complex patients with interstitial lung diseases patients.

Prevalence and demographic characteristics of Behcet disease in Colombia: data from the national health registry 2012-2016.

To estimate prevalence and describe the main demographic characteristics of Behcet disease in Colombia. Cross-sectional study, based on official Ministry of Health registry data. 523 cases of Behcet disease (ICD-10 code: M352) were reported between 2012 and 2016, for a prevalence in people over 18 years old of 1.10 per 100,000 inhabitants, of which 68% are women, with a female-to-male ratio of 2.2:1, with greater prevalence within the 45-49 age group. This is the first study that shows demographic and epidemiological information on Behcet disease in Colombia. Prevalence seems to be low when compared with other similar studies in the region.

Prevalence of polymyalgia rheumatica in Colombia: data from the national health registry 2012-2016.

Polymyalgia rheumatica (PMR) affects elderly patients and is characterized by pain and stiffness of the shoulder girdle, pelvic girdle and cervical region, which can be associated with the presence of giant cell arteritis. Data on the epidemiology of this disease in Latin America are scarce. In Colombia, the Ministry of Health introduced SISPRO, a tool to collect nation-wide information from the health system. The information collected from SISPRO is available for scientific analysis. Using SISPRO data for the years 2012-2016, an analysis was made on the prevalence and characteristics of patients diagnosed with PMR. This is a descriptive epidemiological study using the International Statistical Classification of Diseases and Related Health Problems as search terms related to PMR, based on SISPRO data. Criteria for diagnosis are not explicitly addressed in each individual case. National records report 19,901 individuals diagnosed with PMR and estimated prevalence of 2 cases per 1000 inhabitants over 50 years old (based on a total population of 47,663,162), being more frequent in women (86% of cases), with a female/male ratio of 6.2:1. This is the first study that describes the demographic characteristics of PMR in Colombia. Our results are consistent with the age-related increase in prevalence and gender ratio. Likewise, there are differences between regions, which may be related to ancestry and environmental factors, which require further studies.

Lupus enteritis as systemic lupus erythematosus main manifestation: Two case reports.

Lupus enteritis denotes inflammation of the intestinal walls resulting from the influence of systemic lupus erythematosus. It represents a rare manifestation associated with notable morbidity and mortality, marked by nonspecific gastrointestinal symptoms. In this article, we present two cases of individuals experiencing severe gastrointestinal symptoms. They had a personal or familial history of autoimmunity with intestinal involvement consistent with the presentation of lupus enteritis. Following treatment with glucocorticoids and immunomodulators, both patients exhibited a satisfactory clinical evolution. While lupus enteritis remains an uncommon occurrence, its clinical significance is undeniable. Hence, it is imperative to maintain a high level of clinical suspicion to facilitate prompt diagnosis and treatment.

Prevalence of axial spondyloarthritis in Colombia: data from the National Health Registry 2017-2021.

INTRODUCTION: Registries allow ascertaining the epidemiology of chronic diseases such as axial spondyloarthritis (axSpA). The Colombian Ministry of Health has implemented a National Health Registry (SISPRO) that collects data from each medical contact in the system, which provides close to universal coverage (around 98%). OBJECTIVE: To establish the 5-year prevalence of axSpA in Colombia, and to describe its demographics, using data from January 1st, 2017, to December 31st, 2021. METHODS: We performed an observational, cross-sectional study using the International Statistical Classification of Diseases and Related Health Problems as search terms related to ax-SpA, based on SISPRO data. We estimated the prevalence using three approaches: (1) ankylosing spondylitis (AS) diagnoses; (2) diagnoses compatible with axSpA; and (3) diagnoses compatible with axSpA, including sacroiliitis. We calculated prevalence per 100,000 inhabitants. RESULTS: Based on our three approaches, patients with a primary diagnosis compatible with ax-SpA ranged between 12,684 and 117,648, with an estimated 5-year adjusted prevalence between 26.3 and 244 cases per 100,000 inhabitants (0.03-0.2%). The male-to-female ratio ranged between 1.2:1 and 0.4:1, which was markedly skewed towards a higher prevalence in women when we included the code for sacroiliitis. We found the highest frequency of cases in the 50-54 years group. A differential prevalence was observed between different regions in our country, particularly in regions known to have European ancestors. CONCLUSION: This is the first study that describes demographic characteristics of ax-SpA in Colombia and offers valuable information for stakeholders. Key Points • Using the official country-level health database, the prevalence of axSpA in Colombia ranges between 26.3 and 244 cases per 100,000 inhabitants (0.03% - 0.2%) • The prevalence of axSpA peaked among the 50-54 years patient group, suggesting an increased survival • Nations with a substantial admixture, such as Colombia, may present a differential prevalence of axSpA among regions within the country • Including the ICD-10 code for sacroiliitis (M46.1) in epidemiological studies probably overestimates the frequency of axSpA.

Fever of unknown origin: A 12-year case series in Colombia.

BACKGROUND: Fever of unknown origin (FUO) is a diagnostic challenge with highly heterogeneous causes. Its etiology can change according to the studied regions, and the chance of reaching a diagnosis depends on available resources. The aim of this study is to describe the clinical characteristics, etiology and the usefulness of diagnostic aids in cases of FUO managed over 12 years in a Colombian reference center. METHODOLOGY: Single-institution retrospective case series. All cases of FUO between 2006 and 2017 were identified with the help of an electronic medical record search software. Cases of adults with fever for more than three weeks who remained undiagnosed after three days of hospitalization are described. RESULTS: Of 1,009 cases evaluated, 112 cases met the inclusion criteria (median age 43 years, 66% men). The etiologies identified were infectious (31.2%), inflammatory (20.5%), neoplastic (14.3%), and miscellaneous (2.7%) diseases. 31.2% remained without etiological diagnosis. The most frequent conditions were tuberculosis (17%), Hodgkin's lymphoma (7.1%), systemic lupus erythematosus (6.3%), disseminated histoplasmosis, and adult Still's disease. Contrast tomography and biopsies were the studies that most frequently supported or confirmed the final diagnosis. CONCLUSIONS: This series of contemporary Latin American cases suggests that the categories of FUO etiologies are similar to those reported in studies from developed countries, with tuberculosis being the most frequent cause in our setting. Our results highlight the importance of tomography-guided invasive studies in the diagnostic approach to FUO.

Human Genetic Host Factors and Its Role in the Pathogenesis of Chikungunya Virus Infection.

Chikungunya virus (CHIKV) is an alphavirus from the Togaviridae family that causes acute arthropathy in humans. It is an arthropod-borne virus transmitted initially by the Aedes (Ae) aegypti and after 2006's epidemic in La Reunion by Ae albopictus due to an adaptive mutation of alanine for valine in the position 226 of the E1 glycoprotein genome (A226V). The first isolated cases of CHIKV were reported in Tanzania, however since its arrival to the Western Hemisphere in 2013, the infection became a pandemic. After a mosquito bite from an infected viremic patient the virus replicates eliciting viremia, fever, rash, myalgia, arthralgia, and arthritis. After the acute phase, CHIKV infection can progress to a chronic stage where rheumatic symptoms can last for several months to years. Although there is a great number of studies on the pathogenesis of CHIKV infection not only in humans but also in animal models, there still gaps in the proper understanding of the disease. To this date, it is unknown why a percentage of patients do not develop clinical symptoms despite having been exposed to the virus and developing an adaptive immune response. Also, controversy stills exist on the pathogenesis of chronic joint symptoms. It is known that host immune response to an infectious disease is reflected on patient's symptoms. At the same time, it is now well-established that host genetic variation is an important component of the varied onset, severity, and outcome of infectious disease. It is essential to understand the interaction between the aetiological agent and the host to know the chronic sequelae of the disease. The present review summarizes the current findings on human host genetics and its relationship with immune response in CHIKV infection.

Can presence of HLA type I and II alleles be associated with clinical spectrum of CHIKV infection?

Host immune response and virulence factors are key to disease susceptibility. However, there are no known association studies of human leukocyte antigen (HLA) class I and II alleles with chikungunya virus (CHIKV) infection in the Latin American population. Here, we aimed to identify HLA alleles present in patients with CHIKV infection versus healthy controls as well as the allelic association with the clinical spectrum of the disease. We conducted a cross-sectional analysis of a community cohort and included patients aged 18 years and older with serologically confirmed CHIKV infection. HLA typing of HLA-A, HLA-B, and HLA-DRB1 alleles was performed. Two-by-two tables were used to establish associations between allele presence and clinical characteristics. Data from 65 patients with confirmed CHIKV infection were analyzed for HLA typing. CHIKV infection was significantly associated with the presence of HLA-A*68 [p = .005; odds ratio (OR): 8.90; 95% confidence interval (CI): 1.88-42.13], HLA-B*35 (p = .03; OR: 2.01; 95% CI: 1.06-3.86), HLA-DRB*01 (p <.001; OR: 5.70; 95% CI: 1.95-16.59), HLA-DRB1*04 (p <.001; OR: 7.37; 95% CI: 3.33-16.30), and HLA-DRB1*13 (p = .004; OR: 3.75; 95% CI: 1.50-9.39) alleles in patients versus healthy subjects. A statistically significant relationship was found between the presence of a rash on the face or abdomen and the presence of HLA-DRB1*04 (p = .028; OR: 3.2; 95% CI: 1.11-9.15 and p = .007; OR: 4.33; 95% CI: 1.45-12.88, respectively). Our study demonstrated that, in our cohort, HLA type I and type II alleles are associated with CHIKV infection, and an HLA type II allele is associated with dermatological symptoms. Further research is needed to establish a path for future investigation of genes outside the HLA system to improve knowledge of the pathophysiology of CHIKV infection and its host-pathogen interaction.

Prevalence of osteoporosis in Colombia: Data from the National Health Registry from 2012 to 2018.

INTRODUCTION: Osteoporosis is considered a healthcare problem due to the increased risk of fractures and high cost of care. In Colombia, the Ministry of Health introduced SISPRO, a tool to collect nationwide information from the health system. The information collected from SISPRO is available for scientific analysis. This article presents an analysis of the prevalence and characteristics of patients with osteoporosis using data from 2012 to 2018. AIM: To estimate prevalence of osteoporosis between January 2012 to December 2018 and describe the patients' demographic characteristics. METHODS: This is a descriptive epidemiological study using the International Statistical Classification of Diseases and Related Health Problems related to osteoporosis as search terms using the SISPRO database. RESULTS: National records report 249,803 patients over 50 years old diagnosed with osteoporosis. The estimated prevalence is 2440 cases per 100,000 inhabitants over 50 years old (based on a total population of 10,236,132), being more frequent in women (92% of cases), with a female/male ratio of 12.3:1. CONCLUSION: This study shows a lower prevalence than previous estimates or projections. Given these findings we think it is necessary to act to promote health policies for patients with osteoporosis.

Prevalence of systemic sclerosis in Colombia: Data from the National Health Registry 2012-2016.

Introduction: Systemic sclerosis is an autoimmune disease that characteristically presents with fibrosis and vasculopathy. In Latin America, the information on the epidemiology of this disease is scarce and records are essential to know its behavior in the populations. In Colombia, the Ministry of Health uses the SISPRO tool to collect information on the unified national health system which offers nearly universal coverage (around 95%). This public information makes it possible to perform epidemiological studies on different diseases. Objectives: Using the SISPRO data corresponding to the years 2012-2016, we analyzed the prevalence and characteristics of patients with systemic sclerosis. Methods: A descriptive cross-sectional study was performed based on the SISPRO data; we used as keywords the diagnoses of the International Classification of Diseases related to the diagnosis of systemic sclerosis. Results: A total of 11,300 individuals diagnosed with systemic sclerosis were documented. The estimated prevalence was 23.7 cases per 100,000 inhabitants (based on a total population of 47,663,162); this disease is more frequent in the age group of 65-69 years in females (77%), and has a female:male ratio of 3.27:1. Conclusion: This study describes the epidemiological characteristics of systemic sclerosis in Colombia, based on official statistics from the Ministry of Health. The results provide a new vision of this disease in Latin America and open the door to future research, in order to better understand the particular characteristics of this disease in our country and our region.

Prevalence and Demographic Characteristics of Sjögren's Syndrome in Colombia, Based on Information from the Official Ministry of Health Registry. / Prevalencia y características demográficas del síndrome de Sjögren en Colombia, según información del Sistema Integral de Información de la Protección Social.

OBJECTIVE: To calculate the prevalence and describe the main demographic characteristics of Sjögren's syndrome in adults in Colombia. MATERIAL AND METHODS: Descriptive cross-sectional study which utilized data from the Integral Information System of Social Protection of the Ministry of Health of the Republic of Colombia during the years 2012 to 2016. RESULTS: 58,680 cases of Sjögren's syndrome were identified, with a prevalence in those over 18 years of age of 0.12%; 82% were women, with a female:male ratio of 4.6:1, with a higher prevalence in the age group of 65 to 69 years. The departments with the highest numbers of cases were Bogotá DC (24,885), Antioquia (9,040) and Valle del Cauca (5,277); however, the departments with the highest prevalences were Caldas (0.42%), Bogotá DC (0.32%) and Antioquia (0.14%). CONCLUSIONS: We present demographic and epidemiological information on Sjögren's syndrome in Colombia. There are very few epidemiological studies of this disorder. However, a prevalence similar to that reported in countries of the region such as Brazil (0.17%) and Argentina (0.17%) was documented.

Coexistence of Systemic Lupus Erythematosus and Myasthenia Gravis: An Unusual Case of Polyautoimmunity. / Coexistencia de lupus eritematoso sistémico y miastenia gravis. Una expresión infrecuente de poliautoinmunidad.

The relevance of polyautoimmunity, defined as the presence of 2or more autoimmune diseases in the same individual, is one of the issues not yet elucidated in medical practice. The coexistence of myasthenia gravis (MG) and systemic lupus erythematosus (SLE) is a clinical challenge due to the possible differential diagnoses of muscle involvement in patients with SLE. We present the case of a patient who came to the emergency room of Hospital Universitario San Ignacio in Bogotá, Colombia, with a previous diagnosis of SLE, who developed acute weakness in the context of a systemic infection, with a clinical and electrophysiological diagnosis of MG.

Association of ERAP2 polymorphisms in Colombian HLA-B27+ or HLA-B15+ patients with SpA and its relationship with clinical presentation: axial or peripheral predominance.

OBJECTIVE: To determine the association between endoplasmic reticulum aminopeptidase (ERAP)1 and ERAP2 single-nucleotide polymorphisms (SNPs) and human leukocyte antigens (HLA)-B27+ or HLA-B15+ patients with spondyloarthritis (SpA). METHODS: 104 patients with SpA according to Assessment of Spondyloarthritis International Society criteria were included in the study. HLA typing was performed by PCR. The polymorphisms were determined by real-time PCR on genomic DNA using customised probes for SNPs rs27044, rs17482078, rs10050860 and rs30187 in ERAP1, and rs2910686, rs2248374 and rs2549782 in ERAP2. RESULTS: 70 of the104 patients with SpA were HLA-B27+ and 34 were HLA-B15+. The distribution of ERAP1 and ERAP2 SNPs between the HLA-B15+ and HLA-B27+ patients with SpA did not reveal differences. Likewise, no differences in the frequencies of ERAP1 SNP haplotypes and alleles HLA-B15 or HLA-B27 were found. Interestingly, however, the frequencies of three particular haplotypes formed by ERAP2 SNPs rs2549782/rs2248374/rs2910686 varied between HLA-B15+ and HLA-B27+ patients: the ERAP2 SNPs haplotype TGT was more common in HLA-B15+ patients with SpA (OR 2.943, 95% CI 1.264 to 6.585; P=0.009), whereas the ERAP2 SNP haplotypes TGC and CAT were more associated with HLA-B27+ patients with SpA: (OR 4.483, 95% CI 1.524 to 13.187; p=0.003) and (OR 9.014, 95% CI 1.181 to 68.807; p=0.009), respectively. CONCLUSION: An association was found between HLA-B15+ patients with SpA and haplotype TGT of ERAP2 SNPs. On the other hand, HLA-B27+ patients with SpA were associated with ERAP2 haplotypes TGC and CAT. These associations could be related to the clinical presentation of the disease, specifically with a peripheral or axial predominance, respectively.

Interosseous atrophy beyond rheumatoid arthritis, a manifestation of amyotrophic lateral sclerosis

Interosseous atrophy of the hand is not an exclusive finding of rheumatoid arthritis, and in the case of amyotrophic lateral sclerosis it has a significant diagnostic and prognostic value. The physical examination and evidence of the "split hand" sign or sign of the double anatomical snuffbox is a finding that should alert the clinician to the need for complementary studies in order to rule out neurological pathology.

La atrofia de interóseos de la mano no es un hallazgo exclusivo de la artritis reumatoide, en el caso de la esclerosis lateral amiotrófica tiene un valor diagnóstico y pronóstico significativo, por lo que la exploración física y la evidencia del signo de la mano partida o signo de la doble tabaquera anatómica es un hallazgo que debe alertar al clínico de la necesidad de realizar estudios complementarios para descartar una enfermedad neurológica.

Síndrome de Dressler: el retorno de una enfermedad olvidada / Dressler's syndrome: the return of a forgotten entity

Resumen El síndrome de Dressler es una enfermedad infrecuente que fue descrita por primera vez en 1956 por el doctor William Dressler. Corresponde a un cuadro de pericarditis secundaria, en ocasiones asociada a efusión pericárdica de aparente etiología autoinmunitaria, que hace parte de los síndromes de lesión poscardiaca. Se presenta un caso de síndrome de Dressler en un paciente joven.

Abstract Dressler's syndrome is a rare entity which was first described in 1956 by Dr. William Dressler. It consists of secondary pericarditis, at times associated with pericardial effusion with an apparently autoimmune etiology, which is one of the post-cardiac injury syndromes. We present a case of Dressler's syndrome in a young patient.

Prevalencia de osteoporosis en Colombia: datos del registro nacional de salud del 2012 al 2018 / Prevalence of osteoporosis in Colombia: Data from the National Health Registry from 2012 to 2018

Introducción: La osteoporosis es considerada un problema de salud pública, ya que, al aumentar el riesgo de fracturas, genera un alto coste para el sistema de salud. En Colombia, el Ministerio de Salud utiliza la herramienta SISPRO para recolectar información del sistema de salud. La información almacenada en SISPRO es pública y disponible para la investigación. Este artículo presenta un análisis de la prevalencia y características de los pacientes con osteoporosis a partir de los datos del 2012 al 2018. Objetivos: Estimar la prevalencia de osteoporosis entre enero de 2012 a diciembre de 2018 y describir las características demográficas de los pacientes. Métodos: Estudio descriptivo de corte transversal en el que se tomaron los datos de SISPRO, utilizando como palabras clave los diagnósticos del manual internacional de enfermedades relacionados con el diagnóstico de osteoporosis. Resultados: Se documentaron 249.803 individuos mayores de 50 años con diagnóstico de osteoporosis, estimando una prevalencia de 2.440 casos por 100.000 habitantes mayores de 50 años (basado en una población total de 10.236.132), siendo más frecuente en mujeres (92%), con una relación mujer a hombre de 12,3:1. Conclusión: Este estudio muestra una baja prevalencia respecto a las estimaciones y proyecciones. Dados estos resultados es necesario trabajar en promover políticas en salud para los pacientes con osteoporosis.(AU)

Introduction: Osteoporosis is considered a healthcare problem due to the increased risk of fractures and high cost of care. In Colombia, the Ministry of Health introduced SISPRO, a tool to collect nationwide information from the health system. The information collected from SISPRO is available for scientific analysis. This article presents an analysis of the prevalence and characteristics of patients with osteoporosis using data from 2012 to 2018. Aim: To estimate prevalence of osteoporosis between January 2012 to December 2018 and describe the patients’ demographic characteristics. Methods: This is a descriptive epidemiological study using the International Statistical Classification of Diseases and Related Health Problems related to osteoporosis as search terms using the SISPRO database. Results: National records report 249,803 patients over 50 years old diagnosed with osteoporosis. The estimated prevalence is 2,440 cases per 100,000 inhabitants over 50 years old (based on a total population of 10,236,132), being more frequent in women (92% of cases), with a female/male ratio of 12.3:1. Conclusion: This study shows a lower prevalence than previous estimates or projections. Given these findings we think it is necessary to act to promote health policies for patients with osteoporosis.(AU)

Refractory Immunological Thrombocytopenia Purpura and Splenectomy in Pregnancy.

Thrombocytopenia is defined as a platelet count of less than 100,000 platelets per microlitre (mcL). Thrombocytopenia develops in approximately 6-7% of women during pregnancy and at least 3% of these cases are caused by immunological platelet destruction. Herein, we present a pregnant woman who develops at the first trimester autoimmune thrombocytopenia purpura associated with positive antiphospholipid antibodies. The disease was refractory to pharmacological treatments but had a favourable response to splenectomy. The patient carried the pregnancy to term without complication and gave birth to a healthy baby girl.

Outcome of patients with autoimmune diseases in the intensive care unit: a mixed cluster analysis.

OBJECTIVES: The interest on autoimmune diseases (ADs) and their outcome at the intensive care unit (ICU) has increased due to the clinical challenge for diagnosis and management as well as for prognosis. The current work presents a-year experience on these topics in a tertiary hospital. METHODS: The mixed-cluster methodology based on multivariate descriptive methods such as principal component analysis and multiple correspondence analyses was performed to summarize sets of related variables with strong associations and common clinical context. RESULTS: Fifty adult patients with ADs with a mean age of 46.7±17.55 years were assessed. The two most common diagnoses were systemic lupus erythematosus and systemic sclerosis, registered in 45% and 20% of patients, respectively. The main causes of admission to ICU were infection and AD flare up, observed in 36% and 24%, respectively. Mortality during ICU stay was 24%. The length of hospital stay before ICU admission, shock, vasopressors, mechanical ventilation, abdominal sepsis, Glasgow score and plasmapheresis were all factors associated with mortality. Two new clinical clusters variables (NCVs) were defined: Time ICU and ICU Support Profile, which were associated with survivor and no survivor variables. CONCLUSIONS: Identification of single factors and groups of factors from NCVs will allow implementation of early and aggressive therapies in patients with ADs at the ICU in order to avoid fatal outcomes.

Síndrome de Sjögren neuropsiquiátrico / Neuropsychiatrie Sjögren's Syndrome

RESUMEN El síndrome de Sjögren primario (SSp) es una enfermedad autoinmune que afecta principalmente al tejido glandular. A pesar de ello, puede involucrar otros sistemas, siendo el compromiso neuropsiquiátrico una manifestación extraglandular común. Su presentación clínica varía ampliamente según el dominio que se encuentre afectado, y por tanto puede dividirse en tres grandes categorías: sistema nervioso central, sistema nervioso periférico y psiquiátrico. Algunas de estas complicaciones comparten mecanismos fisiopatológicos comunes, entre los principales la vasculitis/vasculopatía, la infiltración linfocítica y la presencia de anticuerpos antineuronales. La diversidad en la presentación clínica de esta entidad impide hacer una aproximación diagnóstica común, por lo cual la utilización de estudios específicos depende de un adecuado reconocimiento y de la localización por parte del clínico. El tratamiento debe dirigirse al mecanismo fisiopatológico implicado y, de acuerdo con el tipo de manifestación, puede incluso estar limitado al manejo sintomático.

ABSTRACT Primary Sjögren's syndrome is an autoimmune disease that mainly involves glandular tissue. Despite this, it can potentially develop systemic involvement, within which neuropsychiatric manifestations are common. The clinical presentation may vary widely depending on the domain affected, and may thus be classified into three categories: central nervous system, peripheral nervous system, and psychiatric. Some of these complications share a common pathophysiology, amongst which are vasculitis/ vasculopathy, lymphocytic infiltration and positive antineuronal antibodies. The wide clinical presentation makes it difficult to establish a common diagnostic approach, making it essential for the clinician to recognise and localise the type of compromise, so that diagnostic tools can be more advantageously employed. Treatment must be directed towards the underlying pathophysiology, and depending on the type of compromise, it can even be limited solely to the management of symptoms.