Sjögren-related cardiomyopathy presenting with cardiogenic shock

Previous reports have described non-ischaemic cardiomyopathy related to a variety of autoimmune diseases. However, very few case reports describe Sjögren disease as a contributing factor to cardiomyopathy. We report the case of a 69-year-old woman with a history of Sjögren disease who presented with cardiogenic shock. Laboratory testing and cardiac MRI revealing apical septal late gadolinium enhancement were consistent with an autoimmune aetiology. After ruling out ischaemic, infectious and other possible causes, the patient’s clinical presentation was thought to be related to underlying Sjögren disease. She was treated with intravenous steroids and evidence-based heart failure therapy, but she eventually died after having declined heart transplantation. Given the rarity of Sjögren disease, no diagnostic criteria or standard treatment has been established for cardiomyopathy related to this disease. Diagnosis should be considered in patients who show evidence of autoimmune processes after other possible causes are ruled out.

Bilateral endogenous endophthalmitis complicated by scleral perforation: an unusual presentation

Endogenous endophthalmitis complicated by necrotising scleritis has rarely been reported in the literature. We, hereby, report a case of bilateral scleral perforation with endogenous endophthalmitis in an 87-year-old female patient with diabetes who presented as bilateral orbital cellulitis. Systemic workup ruled out autoimmune aetiology. The culture and sensitivity of exudates exuding from the scleral perforation showed Escherichia coli. The Patient was managed conservatively with parenteral and topical antibiotics along with steroid, but the vision could not be salvaged. The report emphasizes on atypical presentation of endogenous endophthalmitis. In old and immunosuppressed individuals presenting with a clinical picture of bilateral orbital cellulitis with profound vision loss, endogenous endophthalmitis should be ruled out.

Feline temporal lobe epilepsy: seven cases of hippocampal and piriform lobe necrosis in England and literature review

Case series summary Seven cases of feline hippocampal and piriform lobe necrosis (FHN) are described, with particular emphasis on clinical, radiographic and histopathological correlations. FHN is an uncommon acute epileptic condition resembling human autoimmune limbic encephalitis and temporal lobe epilepsy. Seizures are typically focal and feature uni- or bilateral orofacial or head twitching, hypersalivation, lip smacking, mydriasis, vocalisation and motionless staring, with inter-ictal behavioural changes such as unprovoked aggression and rapid running. Emerging evidence supports an autoimmune aetiology, although disruption of hippocampal architecture secondary to brain neoplasia has also been recognised. Most commonly, however, the underlying cause remains unknown. Diagnosis is achieved clinically and with brain MRI; electroencephalography and voltage-gated potassium channel-complex autoantibodies are currently the subject of research. Affected cats are frequently refractory to conventional antiepileptic treatment. Relevance and novel information Following a review of the literature, including potential complicating factors and comparisons with human medicine, the hippocampus and piriform lobe are proposed as the neuroanatomical localisation for focal seizures with orofacial involvement in cats, regardless of aetiology.

A Rare Case of Recurrent Lymphocytic Hypophysitis Spanning 8 Years

Background: Lymphocytic Hypophysitis (LH) is predominantly a self-limiting condition. Reports of recurrent LH have been limited to case series, predominantly within first two years of initial presentation (1). There is paucity of data on long term follow up of these cases and late recurrence of LH is considered very rare (2). Clinical Case: We describe the clinical course of a 47-year female, who first presented to us, 8 years back, with headache, visual disturbance and secondary amenorrhea. Her MRI pituitary was suggestive of a pituitary mass, which was removed by trans-sphenoidal surgery. Histopathology of the mass was suggestive of LH. Patient had symptomatic improvement, and remained well on out-patient follow-up, on hormone replacement therapy. She took replacement dose of glucocorticoid for 4 years after the initial surgery and then it was discontinued. However, she re-presented to us with headache and visual disturbance this time associated with xerostomia and xerophthalmia. In this presentation, due to presence of sicca symptoms a suspicion of autoimmune aetiology was kept. Her lab parameters were consistent with pan hypopituitarism along with new appearance of anti-TPO antibodies. She underwent two consecutive trans-sphenoidal surgeries for relief of pressure symptoms, and has been started immunosuppressive doses of glucocorticoids along with methotrexate. Other causes of recurrent hyophysitis, as IgG4 related Hypophysitis, were excluded by immunohistochemistry and normal serum IgG4 level (0.72g/l). She has since been discharged, and remains well on outpatient follow-up with no mass effect. MRI pituitary done at 2 months follow up is not suggestive of residual regrowth or mass effect. Conclusion: This case highlights the importance of long term follow up of LH patients. Other autoimmune aetiology maybe considered in cases unresponsive to standard treatment, necessitating titration of additional immunosuppressive therapy. References: 1.M.N. Joshi et al, Hypophysitis: diagnosis and treatment, European Journal of Endocrinology 2018, 179, R151-R1632.Honegger et al, Treatment of Primary Hypophysitis in Germany: Journal Clinical Endocrinology and Metabolism, September 2015, 100(9):3460 –3469

Antibody Negative Autoimmune Encephalitis: A Case Report

Encephalitis is characterized by inflammation of the brain. Literature describes autoimmune as one of the most common aetiology of non-infectious encephalitis. Given the similarities in clinical, imagological and laboratory findings with viral encephalitis and due to the wide variety of clinical features, the diagnosis is rather challenging and therefore physicians need an increased clinical suspicion to make the correct diagnosis. We report a case of a 35-year-old male with no past medical history that presented with two episodes of autoimmune encephalitis in a 6-month period. Despite having the typical clinical presentation and imagological findings consistent with autoimmune encephalitis, this case had negative results for antibodies, which delayed the diagnosis. It is essential to highlight the importance of considering the hypothesis of autoimmune aetiology on the differential diagnosis of all patients presenting with clinical and magnetic resonance imaging results suggestive of probable encephalitis, regardless of the negative antibodies results. This case clearly depicts the difficulties of diagnosing and treating an autoimmune encephalitis. The main goal of this case report is to increase awareness towards early diagnosis to promptly implement a specific treatment that has proven to improve the outcome and prognosis.

Acute CMV hepatitis in an immunocompetent patient

A previously well and immunocompetent 64-year-old woman presented with fever of unknown origin and acute hepatitis. Besides headache and nausea, she had no other symptoms. Her clinical examination was unremarkable with no clear focus of infection. She was thoroughly investigated and her biochemical profile suggested a viral or autoimmune aetiology. Multiple imaging modalities gave no further insight. Her serology and subsequent nucleic acid amplification indicated reactivation of latent cytomegalovirus (CMV). Her symptoms resolved with supportive care and no anti-viral therapy was needed. This case report highlights CMV reactivation leading to acute hepatitis in a well, immunocompetent patient.

Adult-onset autoimmune diabetes: comparative analysis of classical and latent presentation

Introduction Adult-onset autoimmune diabetes (AID) has two different phenotypes: classic type 1 diabetes mellitus (T1DM), with insulin requirement just after diagnosis, and latent autoimmune diabetes in adults (LADA). The purpose of this study is to characterize patients with AID followed on a tertiary centre, comparing classic T1DM and LADA. Methods We collected data from patients with diabetes and positive islet autoantibodies, aged 30 years old and over at diagnosis. Patients who started insulin in the first 6 months were classified as T1DM and patients with no insulin requirements in the first 6 months were classified as LADA. Data regarding clinical presentation, autoantibodies, A1C and C-peptide at diagnosis, pharmacologic treatment and complications were analysed. Results We included 92 patients, 46 with classic T1DM and 46 with LADA. The percentage of females was 50% in T1DM group and 52.1% in LADA group. The median age at diagnosis was 38 years (IQR–15) for T1DM and 42 years (IQR–15) for LADA (p = 0.057). The median time between diagnosis of diabetes and diagnosis of autoimmune aetiology was 0 months in T1DM group and 60 months in LADA group (p < 0.001). The mean BMI at diagnosis was 24.1 kg/m2 in T1DM group and 26.1 kg/m2 in LADA group (p = 0.042). In T1DM group, 67.4% of the patients had more than one positive autoantibody, comparing to 41.3% of LADA patients (p = 0.012). There was no statistical difference in what concerns to title of GAD autoantibodies, A1C and C-peptide at diagnosis of autoimmune aetiology. The presence of symptoms at diagnosis was associated with T1DM group (p < 0.001). The median daily insulin dose was 40 IU for T1DM (0.58 IU/kg) and 33.5 IU for LADA (0.57 IU/kg), with no statistical difference. LADA patients were more often under non-insulin antidiabetic drugs (p = 0.001). At 10 years follow up, 21.1% of T1DM patients and 63.3% of LADA patients had microvascular complications (p = 0.004). Diabetic nephropathy was present in 23.5% of T1DM patients and 53.3% of LADA patients (p = 0.047). At the last evaluation, 55.6% of T1DM and 82.6% of LADA patients had metabolic syndrome and this difference was independent of diabetes duration. Conclusion Patients with classic T1DM presented more often with symptoms, lower BMI and higher number of autoantibodies, which may be related to a more aggressive autoimmune process. Patients with LADA developed more frequently microvascular complications for the same disease duration, namely diabetic nephropathy, and had more often metabolic syndrome.

Adult-onset autoimmune diabetes: comparative analysis of classical and latent presentation

Introduction: Adult-onset autoimmune diabetes (AID) has two different phenotypes: classic type 1 diabetes mellitus (T1DM), with insulin requirement just after diagnosis, and latent autoimmune diabetes in adults (LADA). The purpose of this study is to characterize patients with AID followed on a tertiary centre, comparing classic T1DM and LADA.Methods: We collected data from patients with diabetes and positive islet autoantibodies, aged 30 years old and over at diagnosis. Patients who started insulin in the first 6 months were classified as T1DM and patients with no insulin requirements in the first 6 months were classified as LADA. Data regarding clinical presentation, autoantibodies, A1C and C-peptide at diagnosis, pharmacologic treatment and complications were analysed. Results: We included 92 patients, 46 with classic T1DM and 46 with LADA. The percentage of females was 50% in T1DM group and 52.1% in LADA group. The median age at diagnosis was 38 years (IQR – 15) for T1DM and 42 years (IQR – 15) for LADA (p=0.057). The median time between diagnosis of diabetes and diagnosis of autoimmune aetiology was 0 months in T1DM group and 60 months in LADA group (p<0.001). The mean BMI at diagnosis was 24.1Kg/m2 in T1DM group and 26.1Kg/m2 in LADA group (p=0.042). In T1DM group, 67.4% of the patients had more than one positive autoantibody, comparing to 41.3% of LADA patients (p=0.012). There was no statistical difference in what concerns to title of GAD autoantibodies, A1C and C-peptide at diagnosis of autoimmune aetiology. The presence of symptoms at diagnosis was associated with T1DM group (p<0.001). The median daily insulin dose was 40IU for T1DM (0.58IU/Kg) and 33.5IU for LADA (0.57IU/Kg), with no statistical difference. LADA patients were more often under non-insulin antidiabetic drugs (p=0.001). At 10 years follow up, 21.1% of T1DM patients and 63.3% of LADA patients had microvascular complications (p=0.004). Diabetic nephropathy was present in 23.5% of T1DM patients and 53.3% of LADA patients (p=0.047). At the last evaluation, 55.6% of T1DM and 82.6% of LADA patients had metabolic syndrome and this difference was independent of diabetes duration. Conclusion: Patients with classic T1DM presented more often with symptoms, lower BMI and higher number of autoantibodies, which may be related to a more aggressive autoimmune process. Patients with LADA developed more frequently microvascular complications for the same disease duration, namely diabetic nephropathy, and had more often metabolic syndrome.

Adult-onset Autoimmune Diabetes: Comparative Analysis of Classical and Latent Presentation

Introduction: Adult-onset autoimmune diabetes (AID) has two different phenotypes: classic type 1 diabetes mellitus (T1DM), with insulin requirement just after diagnosis, and latent autoimmune diabetes in adults (LADA). The purpose of this study is to characterize patients with AID followed on a tertiary centre, comparing classic T1DM and LADA.Methods: We collected data from patients with diabetes and positive islet autoantibodies, aged 30 years old and over at diagnosis. Patients who started insulin in the first 6 months were classified as T1DM and patients with no insulin requirements in the first 6 months were classified as LADA. Data regarding clinical presentation, autoantibodies, A1C and C-peptide at diagnosis, pharmacologic treatment and complications were analysed. Results: We included 92 patients, 46 with classic T1DM and 46 with LADA. The percentage of females was 50% in T1DM group and 52.1% in LADA group. The median age at diagnosis was 38 years (IQR – 15) for T1DM and 42 years (IQR – 15) for LADA (p=0.057). The median time between diagnosis of diabetes and diagnosis of autoimmune aetiology was 0 months in T1DM group and 60 months in LADA group (p<0.001). The mean BMI at diagnosis was 24.1Kg/m2 in T1DM group and 26.1Kg/m2 in LADA group (p=0.042). In T1DM group, 67.4% of the patients had more than one positive autoantibody, comparing to 41.3% of LADA patients (p=0.012). There was no statistical difference in what concerns to title of GAD autoantibodies, A1C and C-peptide at diagnosis of autoimmune aetiology. The presence of symptoms at diagnosis was associated with T1DM group (p<0.001). The median daily insulin dose was 40IU for T1DM (0.58IU/Kg) and 33.5IU for LADA (0.57IU/Kg), with no statistical difference. LADA patients were more often under non-insulin antidiabetic drugs (p=0.001). At 10 years follow up, 21.1% of T1DM patients and 63.3% of LADA patients had microvascular complications (p=0.004). Diabetic nephropathy was present in 23.5% of T1DM patients and 53.3% of LADA patients (p=0.047). At the last evaluation, 55.6% of T1DM and 82.6% of LADA patients had metabolic syndrome (p=0.005). Conclusion: Patients with classic T1DM presented more often with symptoms, lower BMI and higher number of autoantibodies, which may be related to a more aggressive autoimmune process. Patients with LADA developed more frequently microvascular complications for the same disease duration, namely diabetic nephropathy, and had more often metabolic syndrome.