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Background: Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disorder with a wide array of clinical manifestations. Lymphadenopathy is occasionally observed in patients with SLE, and although it is sometimes a feature of active disease, it is rarely the initial presenting feature at the time of diagnosis. Case presentation: We report the case of a 35-year-old woman with clinical features of generalized lymphadenopathy, fatigue, fever, and malaise of approximately 3 months duration with no other constitutional disorders or systemic involvement suggestive of SLE. Despite extensive laboratory, radiological, and histological evaluations for malignancies and infectious causes of generalized lymphadenopathy, all results returned normal. About a month later, she developed inflammatory polyarthritis, with a markedly elevated erythrocyte sedimentation rate (ESR) of 120 mm/h, and a subsequent serologic workup confirmed the diagnosis of SLE. Autoimmune serology testing revealed positive results for various antibodies, including antinuclear antibody (ANA) at a titre of 1:5120, homogenous and speckled pattern, anti-Sm, anti-dsDNA, U1-snRNP, Ku, SSA-AntiRo, SSB-AntiLa, anti-SM/RNP, anti-Histone, ribosomal-p protein, and anti-nucleosome antibodies. The diagnosis of SLE was established using the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ ACR) criteria. She was on oral prednisolone, hydroxychloroquine, and calcium/vitamin D3 tablets. The patient improved remarkably, all swollen lymph nodes regressed spontaneously and she had no further need for a hematology review. Conclusion: While similar cases have been reported in Europe and some parts of Asia, in Africa, a careful search of the literature revealed that such cases have been reported only from Egypt in North Africa and not from other parts of the continent; hence, this case report highlights the importance of recognizing atypical presentations of SLE to improve awareness and diagnosis in different regions. [ABSTRACT FROM AUTHOR]

Type 1 diabetes mellitus (T1DM) is an autoimmune illness that targets specific organs and associated with other autoimmune conditions. Dermatomyositis is an idiopathic systemic autoimmune condition involving skeletal muscles and skin. Co-occurrence of both disorders is quite rare. We diagnosed dermatomyositis in a young male with T1DM who presented with muscle weakness and cutaneous manifestations. There is an additional metabolic hurdle in T1DM, as high doses of glucocorticoids used in dermatomyositis management are related to glycemic instability which requires regular blood sugar monitoring and insulin dose titration accordingly. Considering the prognostic consequences, early diagnosis and management of dermatomyositis is essential. [ABSTRACT FROM AUTHOR]

Immunological illnesses related to wheat represent an epidemiologically relevant phenomenon at a pediatric age. The term "Wheat-related disorders" involves a spectrum of diseases: celiac disease, IgE-mediated wheat allergy, non-IgE mediated wheat allergy, wheat-related eosinophilic esophagitis, and non-celiac gluten sensitivity. Their pathogenesis is different. At the same time, wheat represents their common point. This article aims to the state-of-the-art and new clinical evidence in pediatric age. [ABSTRACT FROM AUTHOR]

Statin-induced immune-mediated necrotising myopathy (IMNM) is an inflammatory myopathy that can present as proximal muscle weakness and, in some cases, as dysphagia and respiratory distress. In this report, we present a case of statin-induced IMNM in a 78-year-old male. The patient had significantly high levels of creatinine kinase and myoglobinuria and experienced gradual weakness in the proximal muscles for 1 month after initiating a 20 mg dose of Atorvastatin 10 months before admission. Rapid clinical improvement was observed with the use of high-dose glucocorticoids in conjunction with methotrexate. [ABSTRACT FROM AUTHOR]

Objective To examine whether patients with ANCA-associated vasculitis (AAV) have an increased risk of cardiovascular disease in the months prior to diagnosis of AAV. Methods Using a nested case–control framework, patients with granulomatosis with polyangiitis and microscopic polyangiitis were identified through the Danish Nationwide Registries from 1996 to 2021 and matched 1:3 with age- and sex-matched controls without AAV. Each control was assigned the same index date (date of AAV diagnosis) as their corresponding case. Conditional logistic regression was used to compute adjusted hazard ratios (HRs) for major adverse cardiovascular events (MACE), ischaemic heart disease, coronary angiogram, heart failure, venous thromboembolism, atrial fibrillation, ischaemic stroke, pericarditis and ventricular arrhythmias/implantable cardioverter defibrillator implantation/cardiac arrest (VA/ICD/CA) within 12 months, 6 months, 3 months, 2 months and 1 month before index date. Results A total of 2371 patients with AAV (median age 63 years, 53.7% male) were matched with 7113 controls. The prevalence of any cardiovascular outcome and MACE within 12 months preceding index date were 10.3% and 2.4% for AAV, compared with 3.8% [HR 3.05 (95% CI 2.48–3.75)] and 1.3% [HR 1.98 (95% CI 1.39–2.82)] of controls. The risk of cardiovascular outcomes was similarly increased in temporal proximity to the diagnosis, with the highest HR at 1 month prior to index date: any cardiovascular outcome [HR 10.73 (95% CI 7.05–16.32)] and MACE [HR 5.78 (95% CI 2.67–12.52)]. In individual analysis, a significantly higher rate was observed for all outcomes (excluding VA/ICD/CA). Conclusions AAV disease is associated with an increased risk of cardiovascular disease in the months preceding diagnosis, which underlines the importance of early clinical vigilance towards cardiovascular disease. [ABSTRACT FROM AUTHOR]

* Context.--Antinuclear antibodies (ANAs) against certain antigens are useful for identifying autoimmune disorders. Although new solid phase--based immunoassays have been developed for evaluating ANAs, the conventional line immunoassay (LIA) is commonly used in clinical practice. Objective.--To compare the clinical performance of 2 newly developed methods for detecting specific ANAs with LIA. Design.--Six hundred ninety-six serum samples were collected from 559 patients with autoimmune disease (AID) and 137 controls. The samples were screened by using the LIA, digital liquid chip method (DLCM), and chemiluminescent immunoassay (CLIA) for specific ANAs. The agreement across assays and the clinical performance of each assay in diagnosing ANA-associated rheumatic diseases (AARDs) were evaluated. Results.--Almost perfect agreement was observed among all assays for anti-centromere protein B (κ = 0.85-0.97), anti-ribosome P (κ = 0.85-0.88), anti-SSA 52 (κ = 0.86-0.89), and anti-SSA 60 (κ = 0.89-0.91); moderate to substantial agreement was detected for the autoantibodies against Sm, Jo-1, ribonucleoprotein, Scl-70, and SSB (κ = 0.55-0.80). LIA exhibited better sensitivity for diagnosing AARDs, while DLCM and CLIA demonstrated higher specificity. In the subset of AIDs, especially systemic lupus erythematosus, antibody positive percentages varied greatly between assays. Conclusions.--The 3 assays showed comparable qualitative agreement; however, the standardization of testing for ANAs remains challenging owing to intermanufacturer variations. Moreover, DLCM and CLIA exhibited better specificity in distinguishing non-AID individuals, whereas LIA was more sensitive in diagnosing AARDs. [ABSTRACT FROM AUTHOR]

Aim: Despite intensive research on elevated IgG4 concentrations in serum, little is known about the importance of selective IgG4 subclass deficiency. We investigated the clinical and immunological characteristics of 55 patients with selective IgG4 subclass deficiency. Materials and Methods: IgG subclass analyses performed in our hospital over 3 years were examined. The clinical features of patients with selective IgG4 subclass deficiency were reviewed, and the definitive diagnoses and the reasons for IgG subclass analysis were recorded. Results: Of 1,675 IgG subclass analyses performed, 55 were indicative of selective IgG4 subclass deficiency. The final diagnoses associated with selective IgG4 subclass deficiency were variable. The most common clinical finding was recurrent infections (33 patients, 60%), and most had upper respiratory tract infections (18 patients, 54.5%). Allergic diseases were noted in 25 patients (45.4%) and were the second most common conditions. Allergic rhinitis was the most frequent allergic disease. Autoimmune diseases were noted in 18 patients (32.7%), Hashimoto thyroiditis being the most frequent. Conclusion: This is the first study of adult selective IgG4 subclass deficiency at a single tertiary medical center in Turkey. In patients with IgG4 deficiency, while the primary concern is the presence of recurrent infections, consideration should also be given to allergic and autoimmune diseases. [ABSTRACT FROM AUTHOR]

Aim: Hashimoto's thyroiditis (HT), the leading cause of hypothyroidism in developed nations, is an autoimmune condition characterized by the progressive destruction of the thyroid gland through various cell- and antibody-mediated immune mechanisms. C-reactive protein-to-albumin ratio (CAR) is a known inflammatory marker easily obtained from a routine blood test. In this context, this study aims to investigate the efficacy of CAR instead of conventionally used thyroid antibodies in predicting the diagnosis and prognosis of HT. Materials and Methods: The participants in this prospective cross-sectional study were adults aged 18 years and above diagnosed with Hashimoto's thyroiditis (HT), seeking medical care at the Internal Medicine and Family Medicine Departments of Recep Tayyip Erdoğan University Training and Research Hospital in Rize, Turkey, during the period from April to July 2022. An age- and gender-matched control group was created from healthy individuals who applied to the outpatient clinics for routine check-ups. The patient and control groups were compared based on C-reactive protein (CRP), hemoglobin, and albumin serum levels of free triiodothyronine (FT3), free thyroxine (FT4), and thyroid stimulating hormone (TSH). Results: The patient and control groups comprised 142 HT patients and 62 healthy individuals. CRP level and CAR value were significantly higher. However, albumin and hemoglobin levels were significantly lower in the patient group than in the control group. [1.5 (0.4-5.6) mg/dL vs. 0.98 (0.3-2.6) mg/dL, p<0.001; 58 (11.5-193.1) vs. 29.5 (8.1-72.2) p<0.001; 2.9 (2.1-4.1) g/dL vs. 3.3 (2.3-4.2) g/dL, p<0.001; and 12±1.1 g/L vs. 12.7±1.760 g/L, p=.005; respectively]. The multivariate analysis revealed CRP, CAR, albumin, FT3, and hemoglobin as significant predictors of HT, with CAR being the most promising one among them [Odds Ratio (OR): 1.11, 95% confidence interval (CI): 1.38-1.19, p=0.003]. Conclusion: This study's findings indicated that CAR is an easily measurable, reliable, and cost-effective marker of HT that can be used as an alternative to conventional thyroid antibodies in primary care centers. [ABSTRACT FROM AUTHOR]

Simple Summary: Autoimmune atrophic gastritis can lead to serious conditions, including malabsorption and vitamin deficiencies, that may cause anemia, neurological disorders, and gastric malignancies. This paper provides recent evidence on the pathogenesis, diagnosis, clinical presentation, risk of malignancies, endoscopic surveillance, and treatment of autoimmune atrophic gastritis. This review provides a valuable update for healthcare professionals and researchers, and the findings may help improve the diagnosis and management of patients with autoimmune atrophic gastritis, leading to improved outcomes and shaping future research directions. Autoimmune atrophic gastritis (AAG) is a chronic condition characterized by the presence of atrophy in the oxyntic mucosa due to anti-parietal cell antibodies. This review provides a comprehensive and up-to-date overview of autoimmune atrophic gastritis, reporting recent evidence on epidemiology, pathogenesis, diagnosis, clinical presentation, risk of malignancies, and management. The prevalence of AAG has been estimated at between 0.3% and 2.7% in the general population. The diagnosis of AAG is based on a combination of the serologic profile and the histological examination of gastric biopsies. Patients with AAG are often asymptomatic but can also have dyspeptic or reflux symptoms. The atrophy of the oxyntic mucosa leads to iron and vitamin B12 malabsorption, which may result in anemia and neurological affections. Autoimmune atrophic gastritis is associated with an increased risk of type I neuroendocrine tumors (NETs) and gastric cancer, with an incidence rate of 2.8% and 0.5% per person/year, respectively. Management is directed to reinstate vitamins and iron and to prevent malignancies with endoscopic surveillance. In conclusion, atrophic autoimmune gastritis is an infrequent condition, often asymptomatic and misdiagnosed, that requires an early diagnosis for appropriate vitamin supplementation and endoscopic follow-up for the early diagnosis of NETs and gastric cancer. [ABSTRACT FROM AUTHOR]

Detection of specific antinuclear antibodies is very important in term of diagnosis, prognosis and management of patients with systemic autoimmune rheumatic diseases. Chemiluminescence microarray immunoassay (CLMIA) is a microdot array-based method that allows simultaneous detection of multiple antinuclear antibodies, which received increasing attention. A CLMIA method that can detect 14 kinds of antinuclear antibodies was established and optimized. Basic performance and diagnostic performance of CLMIA was evaluated by comparing it with line immunoassay (LIA) and indirect immunofluorescence (IIF). Through conditional exploration, the optimal blocking time and blocking temperature were determined to be 18 h and 25 °C, respectively. The enzyme-labeled secondary antibody reaction concentration was 0.1 μg/mL, the incubation temperature of serum and enzyme-labeled secondary antibody were 30 °C, and the incubation time of serum and enzyme-labeled secondary antibody were 40 min. After parameter optimization, CLMIA demonstrated high accuracy with a relative bias <15 %; high sensitivity with detection limits below 3 IU/mL for dsDNA and below 1 RU/mL for other ANAs; and high reproducibility with both intra-assay and inter-assay coefficients of variation (CV) <15 %.The CLMIA detection method established in this study was also demonstrated to have good clinical diagnostic performance, showing the highest area under curve (AUC=0.87, p=0.042 and p=0.03). The CLMIA and LIA revealed substantial to good agreements on specific antinuclear antibodies except anti-dsDNA, with the Cohen's kappa from 0.72 to 0.89. Samples that produced discrepant results between the CLMIA and LIA methods were further analyzed. Upon additional testing, most of these samples were ultimately determined to have been correctly detected by the CLMIA assay rather than the LIA assay, suggesting that CLMIA also shows some superiority in diagnosing dsDNA. The CLMIA could become a potential routine method for detecting ANAs with the advantages of good detection performance. [ABSTRACT FROM AUTHOR]

The Treponema pallidum particle agglutination (TPPA) has been used for decades for serological diagnostics of syphilis but is no longer available. Therefore, we evaluated the Treponema IgG ELISA (TpG) alone and in combination with the Treponema IgM ELISA (TpG+M, both from Euroimmun) as possible substitutes for TPPA as a confirmatory test in a two-tier syphilis screening algorithm. Furthermore, we investigated whether a TPPA titer of 5,120 which is used as cut-off for therapeutic decision in pregnant women in Germany can be transferred to an appropriate cut-off value of the TpG. All serum samples with reactive syphilis screening test (CLIA, Diasorin) within a 13-months period were included (n=739). In addition to TPPA and rapid plasma reagin test both ELISA tests were done in all samples. Sensitivity, specificity, positive and negative predictive values were 92.2, 100, 100, and 74.5 % for TpG, and 93.2, 85.4, 96.6, and 74.1 % for TpG+M. By ROC analysis the cut-off of TpG corresponding to a TPPA titer ≥5,120 was calculated to be 54 RU/mL with a sensitivity of 99.6 % and a resulting specificity of 58.6 %. TpG appears suitable to substitute TPPA as a confirmatory test for syphilis diagnostics but TpG-negative samples have to be evaluated by further tests like FTA-Abs or immunoblot. Treponema IgM determined in addition to TpG did not improve the test performance compared to the TPPA as a reference standard. Valid prediction of a TPPA titer ≥5,120 from TpG result appears not reasonable. [ABSTRACT FROM AUTHOR]

Hypothyroid myopathy is uncommon in childhood. Severe hypothyroid myopathy observed in paediatric practice is a part of Kocher–Debré–Semelaigne syndrome (KDSS, OR-PHA:2349), a rare disorder characterised by muscular pseudohypertrophy and long-standing moderate-to-severe hypothyroidism. We present a pubertal girl with KDSS diagnosed with severe myopathy and significantly limited mobility and progressively increasing pains in the lumbar area, hip joints, and the lower limbs. Additionally, the patient presented metabolic syndrome with severe obesity, growth retardation, and educational difficulties. In this case, adequate hormone replacement therapy with Levothyroxine evoked full recovery of the myopathy and a significant reversal in the patient's general condition. In conclusion, emphasizing the knowledge related to KDSS can improve the diagnosis and prognosis of the condition. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 34-year-old patient with autoimmune Glial Fibrillary Acidic Protein (GFAP) astrocytopathy, a rare central nervous system inflammatory disorder. Topics discussed include the diagnostic challenges and imaging findings related to autoimmune GFAP astrocytopathy, the impact of the condition on neurological and functional abilities, and the effectiveness of a multidisciplinary rehabilitation approach in improving patient outcomes.

Paediatric acute-onset neuropsychiatric syndrome (PANS) and paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) are disorders that affect children, and can have a significant effect on a child’s life, even when they reach adulthood. [ABSTRACT FROM AUTHOR]

Paediatric acute-onset neuropsychiatric syndrome (PANS) and paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) are disorders that affect children, and can have a significant effect on a child’s life, even when they reach adulthood. [ABSTRACT FROM AUTHOR]

Sociologists have a rich history of studying the process of diagnosis and how people experience illness. Yet, the sociology of diagnosis and illness experience literatures have seldom been fully integrated. Instead, these literatures highlight one element of the illness journey, wherein scholars either primarily study diagnostic processes and categories or people's illness experiences. Drawing on empirical studies that examine diagnosis and experiences of illness in varied settings (diagnosis during breast cancer surveillance, diagnosis and experience of autoimmune illness and incarcerated women's experiences of diagnoses and illness), in this article we build on our concept of regimes of patienthood to explain how diagnostic journeys, and the relations and power dynamics that manifest during this time, shape the illness experience and practices of patienthood. We construct a classification of diagnostic processes grounded in our empirical research that span (1) sudden diagnoses, (2) long, changing diagnostic journeys and (3) diagnostic journeys marked by disbelief and denial of care. Our findings demonstrate how diagnostic journeys and illness experiences are intertwined, with different diagnostic pathways impacting how illness is experienced. Analysing these categories collectively demonstrates that diagnostic journeys, while heterogenous, shape the practices that patients develop to manage health conditions and navigate unequal health‐care encounters. [ABSTRACT FROM AUTHOR]

Interleukins are a group of proteins that have a wide range of complex functions and are believed to be involved in several diseases and conditions. In particular, interleukin-40 (IL-40) is a recently identified cytokine associated with B cells that was first introduced by Catalan et al. in 2017. This cytokine has several roles in the body, including functioning in the formation of B cells in the bone marrow, IgA production, and expression in the intestinal microbiome. Moreover, IL-40 appears to be involved in numerous autoimmune and inflammatory conditions, such as rheumatoid arthritis, systemic lupus erythematosus, primary Sjogren's syndrome, ankylosing spondylitis, type 2 diabetes, Graves' disease, and hepatic cell carcinoma. Our understanding of this molecule is quite restricted due to its novelty. However, because of its inflammatory characteristics, there is a high probability that it contributes to a variety of inflammatory disease complications. The aim of the present review is to highlight all available data on the importance of assessing IL-40 levels in human diseases up to now, which could be used as a diagnostic biomarker for the onset or progression of numerous inflammatory diseases. [ABSTRACT FROM AUTHOR]

Introduction: Lupus is a chronic systemic disorder with a complex etiology, and viral infections have contributed to its development and progression. Human herpesvirus-8 (HHV-8) has been diagnosed in many patients with autoimmune disorders; however, its role in lupus has not been well investigated. The present study aimed to compare the frequency and viral load of HHV-8 in patients with lupus and healthy population. Material & Methods: In this cross-sectional study, 70 patients with lupus and 70 healthy individuals who were matched for gender and age were included. After the extraction of viral DNA, the HHV-8 viral load was measured using the Real-time polymerase chain reaction (PCR) technique, and the results were analyzed by SPSS software. Results: Patients with lupus consisted of 59 females (84.3%) and 11 males (15.7%) with a mean age of 31.1±8.4 years, and the control group consisted of 58 females (82.9%) and 12 males (17.1%) with a mean age of 33.5±11.2 years, none of them were positive for HHV-8 genome. Discussion & Conclusion: To date, there is no report in this field from Iran, and this study is the first survey. Regarding the high accuracy of the Real-time PCR technique and lack of HHV-8 genome in the blood samples of Iranian patients with lupus, it seems that none of the individuals, even in the case of viral infection, were infected with the active form of HHV-8, which is compatible with the previous studies. [ABSTRACT FROM AUTHOR]

La médecine interne est une spécialité médicale mal connue du grand public et parfois mal identifiée. À l'heure de la surspécialisation et de l'ultra-technicité, elle se caractérise par une approche globale centrée sur l'évaluation clinique. Contrairement à ce qui est observé dans la plupart des pays développés où les maladies auto-immunes systémiques sont prises en charge par les spécialistes d'organe en fonction de leur mode de présentation, les internistes français sont en première ligne pour diagnostiquer et prendre en charge ces maladies et leur formation multidisciplinaire leur donne toute légitimité pour justifier ce positionnement. Les internistes ont un exercice principalement hospitalier et sont également fortement impliqués dans la prise en charge des patients relevant d'hospitalisations non programmées en aval des urgences et en lien avec la médecine de ville. Afin de mieux définir la médecine interne, son positionnement dans les activités de soins, mais aussi d'enseignement et de recherche et ses perspectives, les internistes ont organisé les états généraux de la médecine interne qui ont eu lieu le 28 septembre 2023 à Paris. Construits autour de groupes de réflexions portant sur les activités de soins, d'enseignement et de recherche, ces états généraux avaient pour objectif d'améliorer la visibilité de la médecine interne et des internistes. Cet article relate les débats qui ont animé cette réunion et met en avant les principales idées qui ont émergé. Ces états généraux constituent une étape fondatrice, et seront suivis d'une Conférence de concertation qui permettra de mieux identifier et faire connaître la médecine interne et les internistes, quels que soient leurs types et lieux d'exercice. Internal medicine is a medical specialty that is often poorly understood by the general public and sometimes misidentified. In an era of increasing subspecialization and high technicality, it is characterized by a comprehensive approach centered on clinical evaluation. Unlike what is observed in most developed countries, where systemic autoimmune diseases are managed by organ specialists based on their mode of presentation, French internists are at the forefront for diagnosing and managing these diseases. Their multidisciplinary training gives them legitimacy to justify this role. Internists also play a crucial role in the management of patients requiring unplanned hospitalizations downstream from emergency departments and in connection with primary care. Internists primarily practice in a hospital setting, with a specific position in the French healthcare system aligned with the training frameworks of all medical specialties. To better define internal medicine, its role in care activities, as well as in education and research, internists organized a General Assembly of internal medicine that took place on September 28, 2023, in Paris. Structured around think tanks focusing on care, education, and research activities, the general assembly aimed to improve visibility on internal medicine and internists. This article recounts the discussions that animated this meeting and highlights the main ideas that emerged. These general assemblies constitute a foundational step and will be followed by a Consultation Conference in order to better identify and promote internal medicine and internists, regardless of their types and places of practice. [ABSTRACT FROM AUTHOR]

A 60-year-old male presented with extreme fatigue and weight loss 3 weeks after testing positive for coronavirus disease 2019. The course of coronavirus disease 2019 was mild, without pulmonary involvement. The patient showed symptoms and laboratory signs of new-onset diabetes without ketoacidosis and acute primary adrenal insufficiency, the latter of which was confirmed with an adrenocorticotrophic hormone stimulation test. Glutamic acid decarboxylase-65 antibody and antibodies to the adrenal cortex were positive in high titers. Previous medical records of the patient revealed no hyperglycemia or electrolyte abnormalities prior to current admission, but an earlier test result was compatible with primary hypothyroidism which was associated with autoimmune thyroiditis. He was therefore diagnosed with autoimmune polyglandular syndrome type II. Clinical and laboratory findings improved after starting appropriate hormone replacement therapies along with insulin. Coronavirus disease 2019 may induce a variety of autoimmune disorders in susceptible subjects. In our case, coronavirus disease 2019 might have simply precipitated type 1 diabetes and adrenal insufficiency in whom autoimmune thyroiditis may have been the first manifestation of an undiagnosed autoimmune polyglandular syndrome type II. Coronavirus disease 2019 might have triggered autoimmunity in the patient who was possibly predisposed to autoimmune disorders. Anyhow, more research is required to evaluate the possible link between coronavirus disease 2019 and autoimmune disorders. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Few studies have evaluated racial and ethnic differences in several immune-mediated inflammatory diseases (IMIDs) or overlap syndrome (the co-occurrence of ≥ 2 IMIDs). We assessed associations between race and ethnicity and prevalence of IMIDs and overlap syndrome using US claims and electronic health records from 2021. STUDY DESIGN: Retrospective cohort study of 10.8 million adults. METHODS: We identified the 10 most prevalent IMIDs among frequently discussed IMIDs. We estimated associations between the 5 most prevalent IMIDs and overlap syndrome in Hispanic and non-Hispanic Asian and Black adults using non-Hispanic White adults as the referent and stratifying by sex and age (20-39, 40-59, and ≥ 60 years). RESULTS: Inflammatory bowel disease (IBD), systemic lupus erythematosus (SLE), multiple sclerosis (MS), and rheumatoid arthritis (RA) were the most prevalent IMIDs in all races and ethnicities. We observed positive associations (P < .0001) between Hispanic and non-Hispanic Black adults and SLE, Asian women of all ages and Asian men younger than 60 years and SLE, Black women younger than 60 years and MS, and Hispanic and non-White women 60 years or older and RA. Hispanic and non-White adults of all age groups had inverse associations (P < .0001) with IBD. Non-Hispanic Black adults of all ages and Hispanic and non-Hispanic Asian women 40 years or older had inverse associations (P < .0001) with psoriasis/psoriatic arthritis. Overlap syndrome was rare among all groups, with some variation in which IMIDs co-occurred. CONCLUSIONS: We found racial and ethnic differences in the prevalence and co-occurrence of IMIDs in this sample of US adults. Because misdiagnoses are relatively frequent for patients with IMIDs, awareness of racial and ethnic variations in IMIDs could aid early diagnosis and improve disease management. [ABSTRACT FROM AUTHOR]

Calprotectin (CLP) is a calcium-binding protein produced by neutrophils and monocytes in the course of inflammation. Today, the role of faecal CLP in chronic IBD is well known, but in recent years attention has shifted towards circulating CLP. In fact, this molecule can be measured in different biological fluids: blood, saliva and urine, using different analytic methods that are described in this review. Furthermore, different data confirm the relevant role of serum CLP in autoimmune diseases. In this review we will highlight the correlation between high levels of circulating CLP and specific autoantibodies of major autoimmune pathologies paving the way to the employment of CLP measurement as useful biomarker for monitoring outcome in different pathologies. [ABSTRACT FROM AUTHOR]

The role of complement in human autoimmune, inflammatory, and infectious diseases is reviewed, focusing on clinical applicability. A typical case is presented in which serum testing for C3 and C4 is performed to help assess a syndrome with a broad differential diagnosis. The review includes a discussion of complement deficiency states, consumption of complement by diseases characterized by immune‐complex formation and deposition, usefulness and interpretation of laboratory tests for complement, and development of drugs targeting specific components of the complement pathway for a growing number of indications. [ABSTRACT FROM AUTHOR]

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Introduction: Vitiligo is a chronic skin condition caused by progressive cutaneous hypomelanosis. Aim: The aim of the study was to determine its epidemiology and clinical aspects. As considerable progress has been made in understanding of the pathogenesis of vitiligo and its classification as an autoimmune disease, the paper pays particular attention to coexisting autoimmune or atopic diseases. Material and methods: The study included 55 patients attending the Diagnostic and Treatment Center of Skin Diseases in Lodz. Data were collected during outpatient dermatological consultation. Results: The most common type of vitiligo was nonsegmental (85.5%) followed by segmental (12.7%) and unclassified (2.1%). The first skin lesions were mostly located on the hands (45.5%) and face (38.2%). Older patients with higher body mass index tended to demonstrate a higher body surface area. Of the patients, 63.6% demonstrated an autoimmune or atopic comorbidity, the most common of which were type 1 diabetes mellitus (18.2%), psoriasis (16.4%) or Hashimoto's thyroiditis (14.5%). Location on the face was associated with a significantly greater incidence of autoimmune or atopic co-morbidities. Conclusions: A facial location may serve as a predictive factor for other autoimmune or atopic diseases in vitiligo patients. Determining clinical factors in vitiligo patients which could be associated with a higher risk of autoimmune comorbidities may allow for their early diagnosis and suitable treatment. [ABSTRACT FROM AUTHOR]

Autoimmune polyglandular syndrome type 2 (APS-2) is the coexistence of Addison disease and at least one of the disorders like autoimmune thyroid diseases and/or type 1 diabetes mellitus. We discuss the case of 16.5-year-old girl who had been diagnosed with APS-2 at the early age of 13.5 years. The girl presented recurrent abdominal pain and emesis for about 3 years, occurring about every 6 months. Moreover, in physical examination, characteristic skin hyperpigmentation furrows in the hands, nipples, and mucosa in the oral cavity were noticed. Due to the clinical picture and laboratory data, autoimmune thyroiditis and primary adrenal insufficiency were diagnosed. After initiating substitution treatment, the girl developed full-blown diabetes mellitus, probably masked earlier after primary adrenal insufficiency. It should be kept in mind that during the diagnosis of autoimmune disease, other autoimmune disorders may coexist; thus, such patients should be under specialists' close supervision. [ABSTRACT FROM AUTHOR]

The article presents the discussion on case study of 2-month-old previously healthy, full-term female with fever, nonbloody emesis, and melena. Topics include physical exam was notable for pulmonary rales, abdominal distention, and signs of severe dehydration but no dysmorphic features or palpable lymphadenopathy; and moderate tricuspid valve regurgitation, and mild mitral valve regurgitation.

Glial fibrillary acidic protein (GFAP) astrocytopathy is a rare autoimmune inflammatory disorder affecting the central nervous system, involving the meninges, brain parenchyma, and spinal cord. The distinctive radiologic feature observed on magnetic resonance imaging (MRI) is characterized by periventricular radial and linear contrast enhancement. This case report details a 45-year-old male who initially exhibited constitutional symptoms, followed by encephalitis, lower limb weakness, and urinary retention. The MRI findings revealed meningoencephalitis with longitudinal extensive myelitis. Notably, the cerebrospinal fluid analysis confirmed the presence of anti-GFAP antibodies. [ABSTRACT FROM AUTHOR]

Introduction. 3-Hydroxy-3-methylglutaryl coenzyme A reductase (HMGCR) inhibitors are widely used worldwide to treat dyslipidaemia and prevent cardiovascular events. Statins can cause a wide variety of muscle injuries ranging from myalgia to severe rhabdomyolysis. In most cases, these symptoms are mild and self-limiting and do not require specific treatment besides drug withdrawal. Statin-induced autoimmune necrotizing myopathy (SINAM) is a rare but potentially fatal complication, characterized by the subacute onset of progressive proximal muscle weakness and considerably high creatine phosphokinase (CK) levels in patients exposed to statins. The diagnosis is supported by the presence of antibodies HMGCR, which allows the differentiation from other forms of necrotizing autoimmune myopathies. Symptoms usually progress even after statin discontinuation and can determine severe muscle damage. Summary. We describe the case of a 77-year-old man who developed SINAM after 5 years of statin use. He suffered from muscle functional impairment mainly involving proximal lower limb muscles which progressed to the point that he almost became bedridden. Initial treatment with prednisone alone was not effective, and he required a combination therapy with steroids, methotrexate, and intravenous immunoglobulins. After 5 months of therapy and rehabilitation, he showed complete laboratory response and muscle strength recovery. Conclusion. Recognizing SINAM is paramount in order to promptly start treatment and avoid permanent muscle damage. Using a combination therapy from the beginning could contribute to a better outcome. Prompt statin cessation, categorization of the muscle disease by autoantibody testing, imaging, and histology, exclusion of malignancy, and anti-inflammatory therapy with corticosteroids, antimetabolites, immunoglobulins, and in some cases rituximab are currently accepted approaches to this entity. [ABSTRACT FROM AUTHOR]

Commonly, herpes simplex virus (HSV) causes infectious encephalitis among children. A neurological relapse after primary HSV encephalitis in the weeks or months after presentation is well recognized. Relapsing symptoms of post-HSV encephalitis can present either as a true relapse or an immune-mediated disorder. A relationship is predicted between immune-mediated disorder and N-methyl-D-aspartate receptor (NMDAR) antibodies. This study presents two cases of patients suffering from anti-NMDAR encephalitis that appeared after treatment for proven HSV encephalitis. The first patient was treated immediately after the presentation as autoimmune encephalitis and had an excellent outcome. The second patient had delayed initiation of treatment and suffered from intractable epilepsy and severe global developmental delay. An important role is played by recognizing anti-NMDAR encephalitis symptoms and its variable presentation for timely diagnosis and quick initiation of treatment for anti-NMDAR encephalitis, thus, improving the outcome for those patients. [ABSTRACT FROM AUTHOR]

Hypertrophic pachymeningitis is a rare disorder of the dura mater of the spine or brain. It can be caused by inflammatory, infective or neoplastic conditions or can be idiopathic. We report a man with hypertrophic pachymeningitis and bilateral chronic subdural haematoma caused by IgG4- related disease. We highlight the diagnostic challenges and discuss possible underlying mechanisms of subdural haematoma formation in inflammatory conditions. Isolated IgG4- related hypertrophic pachymeningitis with chronic subdural haematoma is very rare; previously reported cases have suggested a possible predilection for men in their sixth decade, presenting with headache as the dominant symptom. Given the rarity and complexity of the condition, it should be managed in a multidisciplinary team setting. [ABSTRACT FROM AUTHOR]

Autoimmune diseases (ADs) affect about 5% of the general population, causing various systemic and/or topical clinical manifestations. The oral mucosa is often affected, sometimes as the only involved site. The misdiagnosis of oral ADs is an underreported issue. This narrative review focuses on diagnostic delay (DD) in oral ADs (oral lichen planus [OLP], oral Pemphigus Vulgaris, mucous membrane pemphigoid, oral lupus erythematosus, orofacial granulomatosis, oral erythema multiforme [EM], and Sjogren syndrome). Extensive literature research was conducted via MEDLINE, Embase and Google Scholar databases for articles reporting the time spent to achieve the correct diagnosis of oral ADs. Only 16 studies reported DD in oral ADs. Oral autoimmune vesiculobullous diseases are usually diagnosed after 8 months from the initial signs/symptoms, the Sjogren Syndrome diagnosis usually requires about 73 months. No data exist about the DD in OLP, oral lupus erythematosus, orofacial granulomatosis, and oral EM. The diagnosis of oral ADs can be difficult due to the non‐specificity of their manifestations and the unawareness of dentists, physicians, and dental and medical specialists about these diseases. This can lead to a professional DD and a consequential treatment delay. The delay can be attributed to the physicians or/and the healthcare system (Professional Delay) or the patient (Patient's Delay). [ABSTRACT FROM AUTHOR]

Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APCED) syndrome is a rare autosomal recessive syndrome. There is a loss of function mutation in the autoimmune regulator (AIRE) gene. It is characterized by two of the following conditions: chronic mucocutaneous candidiasis, hypoparathyroidism, or Addison's disease. Here, we describe one such mysterious case of APCED syndrome, which posed a diagnostic challenge. [ABSTRACT FROM AUTHOR]

New-onset refractory status epilepticus (NORSE) is a rare, life-threatening clinical presentation in patients without a known history of epileptic seizures. Autoimmune encephalitis is the most common cause identified in adults; however, in up to 50% of cases, no cause can be found. We present a case of a previously healthy 26-year-old male admitted to the intensive care medicine with NORSE, whose condition improved with the initiation of immunotherapy. Later, he was diagnosed with anti-leucine-rich glioma-inactivated 1 (anti-LGI1) antibody encephalitis. Despite prompt initiation of immunotherapy, cognitive function deterioration and resistant seizures persisted. NORSE is a critical condition that requires urgent treatment. In patients with a negative initial work-up, a preliminary diagnosis of autoimmune encephalitis should be considered. It is critical to begin immunotherapy before the autoimmune encephalitis panel results, as early treatment improves outcomes and long-term prognosis. [ABSTRACT FROM AUTHOR]

Background. Autoimmune pancreatitis (AIP) usually responds dramatically to steroid therapy. Occasionally, however, misdiagnosed patients have undergone pancreaticoduodenectomy. This study is aimed at providing useful information to improve the accuracy of diagnosis before surgery and thus avoid unnecessary resections in patients with AIP. Methods. From January 2015 to February 2020, a series of patients were enrolled, having undergone pancreaticoduodenectomy for presumed malignancy. AIP diagnoses were confirmed by postoperative pathology. The demographic and clinical data of the AIP patients were evaluated. The main diagnostic criteria (HISORt, Asian, and ICDC) for AIP were applied to assess whether and how unnecessary surgery could have been avoided. Results. A total of 124 cases of pancreaticoduodenectomy were performed for presumed malignancy. Six patients were diagnosed with benign disease and five with AIP. The prevalences of benign disease and AIP were 4.8% and 4%, respectively. Four patients were female and 1 male, with a mean age of 60.0 years old. Jaundice, pain, and weight loss were observed in 100%, 20%, and 40% of AIP patients, respectively. The radiologic features of the AIP patients were a diffusely enlarged gland (40.0%), a focally enlarged gland (40.0%), pancreatic ductal dilatation (60.0%), upstream parenchymal atrophy (20.0%), bile duct thickening (66.0%), and bile duct stricture (40.0%). Based on the diagnostic criteria for AIP, surgery could have been avoided in two cases. Conclusions. IgG4 measurement and integrated use of major diagnostic criteria should be emphasized in every patient eligible for pancreaticoduodenectomies. [ABSTRACT FROM AUTHOR]

Cardiac masses are rare entities that can be challenging in clinical diagnosis and management. Cardiac masses can be detected incidentally in patients with an asymptomatic course or may cause systemic inflammation findings due to inflammatory cytokine release or symptoms such as shortness of breath, chest pain, syncope, sudden cardiac death, and mortality due to the location of the mass. Cardiac masses associated with systemic inflammatory disorders are uncommon in this disease group. This case report will present a case with an asymptomatic IgG4‐related left atrial mass detected in routine echocardiographic control imaging due to rheumatic valve disease. [ABSTRACT FROM AUTHOR]

Introduction: Delayed enhancement‐magnetic resonance imaging (DE‐MRI) has demonstrated that nonischemic cardiomyopathy is mainly characterized by intramural or epicardial fibrosis whereas global endomyocardial fibrosis suggests cardiac involvement in autoimmune rheumatic diseases or amyloidosis. Conduction disorders and sudden cardiac death are important manifestations of autoimmune rheumatic diseases with cardiac involvement but the substrates of ventricular arrhythmias in autoimmune rheumatic diseases have not been fully elucidated. Methods and Results: 20 patients with autoimmune rheumatic diseases presenting with ventricular tachycardia (VT) (n = 11) or frequent ventricular extrasystoles (n = 9) underwent DE‐MRI and/or endocardial electroanatomical mapping of the left ventricle (LV). Ten patients with autoimmune rheumatic diseases underwent VT ablation. Global endomyocardial fibrosis without myocardial thickening and unrelated to coronary territories was detected by DE‐MRI or electroanatomical voltage mapping in 9 of 20 patients with autoimmune rheumatic diseases. In the other patients with autoimmune rheumatic diseases, limited regions of predominantly epicardial (n = 4) and intramyocardial (n = 5) fibrosis or only minimal fibrosis (n = 2) were found using DE‐MRI. Endocardial low‐amplitude diastolic potentials and pre‐systolic Purkinje or fascicular potentials, mostly within fibrotic areas, were identified as the targets of successful VT ablation in 7 of 10 patients with autoimmune rheumatic diseases. Conclusion: Global endomyocardial fibrosis can be a tool to diagnose severe cardiac involvement in autoimmune rheumatic diseases and may serve as the substrate of ventricular arrhythmias in a substantial part of patients. [ABSTRACT FROM AUTHOR]

Objective: To study the neurological manifestations of glutamic acid decarboxylase (GAD 65) autoimmunity in Indian patients. Methods: Retrospective study conducted in a tertiary care referral hospital in South India. Patients who tested positive for GAD 65 antibodies from February 2013 to July 2019 were included. Results: We identified 922 patients who underwent GAD 65 testing, of which 81 tested positive (8.78%) [mean age 55.42 years (SD 17.39, range 9-86 years, median age 57 years)]. Males (n = 47) outnumbered the females (n = 34). All the GAD values measured were <5000 IU/ml. There were 34 cases (42%) of atypical parkinsonism (16/34, 47% fulfilled the diagnostic criteria for autoimmune atypical parkinsonism) in our series forming the most common group with GAD 65 positivity, followed by autoimmune encephalitis (8 cases, 9.88%). Men were more affected with atypical parkinsonism (22/34; 64.70%), stiff person syndrome (2/3; 66.66%), and neuropathy (4/7; 57.1%) while women were more with autoimmune encephalitis (6/8; 75%). Eighteen (22.6%) had underlying autoimmunity (three had type 1 diabetes mellitus). Six (7.4%) had underlying neoplasm. Thirty three out of 43 patients responded to immunotherapy (76.74%). Five had spontaneous improvement. Conclusion: Glutamic acid decarboxylase65 antibody values were much lower in our study population. Male dominant autoimmunity was seen unlike that in Western literature. The most striking was the high preponderance of atypical parkinsonism in GAD 65 positive patients. We also found that GAD 65 positivity is a useful marker for a positive response to immunotherapy in suspected autoimmune neurological syndromes irrespective of their titers. [ABSTRACT FROM AUTHOR]

Objective The aim of this study was to examine appropriate utilization of antinuclear antibody (ANA) screening tests with follow-up subserology tests (reflex testing) for diagnosing systemic autoimmune rheumatic disorder (SARD). Methods We conducted a retrospective chart review of 3003 SARD-test orders at an academic teaching hospital from January to December 2019. Testing patterns were categorized as American College of Rheumatology (ACR)-recommended reflex testing, panel testing, or single subserology testing. We described testing patterns, assessed their diagnostic accuracy, and explored factors associated with reflex testing. Results Reflex testing accounted for 79.7% of SARD test-ordering, whereas improper testing (panel or single subserology) accounted for the other 20.3%. Reflex testing was associated with significantly more SARD diagnoses than improper testing (P  = .004). Testing patterns were significantly associated with race/ethnicity (P  = .008), with reflex testing being less frequent than improper testing in Hispanics and Whites. Conclusion In summary, one-fifth (20.3%) of testing patterns for suspected SARD did not follow the ACR-recommended guidelines for using reflex testing. Use of reflex testing was associated with an increased frequency of SARD diagnosis. [ABSTRACT FROM AUTHOR]

Background: Insulin autoimmune syndrome (IAS) is a rare cause of hypoglycemia characterized by high levels of blood insulin autoantibodies. It has been documented that drugs containing sulfhydryl groups may result in IAS. In this study, we present two cases of IAS induced by methimazole, along with their corresponding treatments and a long-term follow-up after hospitalization. Case presentation: We report two patients with Grave's disease (GD), carrying the HLA-DRB1 04:06 genotype, who experienced hypoglycemic episodes after taking methimazole. Inpatient treatments helped return their blood glucose levels to normal. Although no recurrences of hypoglycemia were present in the two cases studied, insulin autoantibodies remained positive for the previous follow-up sessions, which turned negative only three years after discharge. Conclusions: GD patients who carry the HLA-DRB1 04:06 genotype are prone to IAS if they take drugs containing sulfhydryl groups. It may take time for the elimination of insulin autoantibodies after the recovery from the hypoglycemic episode in IAS patients. [ABSTRACT FROM AUTHOR]

Objectives : Anti-leucine glioma-inactivated protein 1 (anti-LGI1) autoimmune encephalitis (AE) presents as subacute memory loss, behavioral changes, and seizures. Diagnosis and treatment delays can result in long term sequelae, including cognitive impairment. 18F-FDG PET/CT may be more sensitive than MRI in patients with AE. Our objective was to determine if anti-LGI1 is associated with a distinct pattern of FDG uptake and whether this pattern persists following treatment. Methods: Nineteen18F-FDG PET/CT brain scans (13 pre-treatment, 6 convalescent phase) for 13 patients with anti-LGI1 were studied using NeuroQ™ and CortexID™. The sensitivity of the PET images was compared to MRI. The Z scores of 47 brain regions between the pre-treatment and next available follow-up images during convalescence were compared. Results: All 18F-FDG PET/CT scans demonstrated abnormal FDG uptake, while only 6 (42.9%) pre-treatment brain MRIs were abnormal. The pre-treatment scans demonstrated hypermetabolism in the bilateral medial temporal cortices, basal ganglia, brain stem, and cerebellum and hypometabolism in bilateral medial and mid frontal, cingulate, and parietotemporal cortices. Overall, the brain uptake during convalescence showed improvement of the Z scores towards 0 or normalization of previous hypometabolic activity in medial frontal cortex, inferior frontal cortex, Broca's region, parietotemporal cortex, and posterior cingulate cortex and previous hypermetabolic activity in medial temporal cortices, caudate, midbrain, pons and cerebellum. Conclusions: Brain FDG uptake was more commonly abnormal than MRI in the pre-treatment phase of anti-LGI1, and patterns of dysmetabolism differed in the pre-treatment and convalescent phases. These findings may expedite the diagnosis, treatment, and monitoring of anti-LGI1 patients. [ABSTRACT FROM AUTHOR]

Sjögren's disease (SD), also known as Sjögren's syndrome (SS) or Gougerot-Sjögren's syndrome in France, is a rare systemic autoimmune disease in its primary form and is characterised by tropism for the exocrine glandular epithelia, particularly the salivary and lacrimal glands. The lymphocytic infiltration of these epithelia will clinically translate into a dry syndrome which, associated with fatigue and pain, constitutes the symptom triad of the disease. In about one third of patients, SD is associated with systemic complications that can affect the joints, skin, lungs, kidneys, central or peripheral nervous system, and lymphoid organs with an increased risk of B-cell lymphoma. SD affects women more frequently than men (9/1). The peak frequency is around the age of 50. However, the disease can occur at any age, with paediatric forms occurring even though they remain rare. SD can occur alone or in association with other systemic autoimmune diseases. In its isolated or primary form, the prevalence of SD is estimated to be between 1 per 1000 and 1 per 10,000 inhabitants. The most recent classification criteria were developed in 2016 by EULAR and ACR. The course and prognosis of the disease are highly variable and depend on the presence of systemic involvement and the severity of the dryness of the eyes and mouth. The current approach is therefore to identify at an early stage those patients most at risk of systemic complications or lymphoma, who require close follow-up. On the other hand, regular monitoring of the ophthalmological damage and of the dental status should be ensured to reduce the consequences. [ABSTRACT FROM AUTHOR]

Background: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a novel autoimmune encephalitis (AE) first identified in 2007. It provides a new direction for clinicians when encountering unexplained symptoms such as seizures, psychotic behavioral abnormalities, speech disorders, and involuntary movements. Most patients have a good prognosis after immunotherapy, but some may experience relapses. Case presentation: We report a Chinese female patient diagnosed with anti-NMDAR encephalitis. Over the past 30 years, the patient had experienced eight episodes with seizures as the first symptom, which eventually progressed to autoimmune-associated epilepsy. In the last two episodes, both serum and cerebrospinal fluid of the patient were negative for AE-related antibodies, and brain magnetic resonance imaging (MRI) revealed abnormal hyperintensity in the bilateral hippocampi. The patient's symptoms were poorly controlled by immunotherapy but well controlled by anti-seizure medicines. Conclusions: Patients with a long history of AE and multiple relapses that start with seizures may display alterations of brain structure. Physicians should pay attention to autoimmune-associated epilepsy. [ABSTRACT FROM AUTHOR]

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The article describes the case of unilateral parotid mass and facial palsy in a patient with relapsing immunoglobulin G4-related disease. Topics discussed include the patient's medical history, symptoms presented by the patient, result of the patient's magnetic resonance imaging and biopsy, and treatments given to the patient.

The article presents questions and answers related to the potential causes of hydroureteronephrosis in a 12-year-old boy who experiences symptoms like chronic constipation.

The article discusses the results of a study on the effectiveness of antineutrophilic cytoplasmic antibody-myeloperoxidase (ANCA-MPO) assay in the diagnosis of vasculitis. Topics mentioned include the correlation of atypical p-ANCA and p-ANCA patterns with the ANCA-MPO test, the sensitivity, discriminatory ability, and specificity of ANCA-MPO assay for diagnosing ANCA-associated vasculitis and drug-induced vasculitis, and the characteristics of patients.

The presented case highlights a rare instance of relapsing polychondritis (RP) manifesting as seronegative limbic encephalitis, an uncommon neurological complication. A 70-year-old female patient with a history of RP-related inflammation, along with neuropsychiatric symptoms, was diagnosed through multidisciplinary collaboration. Swift administration of steroid therapy, followed by azathioprine, led to remarkable physical and cognitive recovery. This case emphasises the importance of a multidisciplinary approach in diagnosing and treating complex autoimmune disorders with neurological manifestations. [ABSTRACT FROM AUTHOR]