Gastric Cancer Screening in Common Variable Immunodeficiency.

Individuals with common variable immunodeficiency (CVID) have an increased risk of gastric cancer, and gastrointestinal lymphoma, yet screening for premalignant gastric lesions is rarely offered routinely to these patients. Proposed screening protocols are not widely accepted and are based on gastric cancer risk factors that are not applicable to all CVID patients. Fifty-two CVID patients were recruited for screening gastroscopy irrespective of symptoms or blood results and were compared to 40 controls presenting for gastroscopy for other clinical indications. Overall, 34% of CVID patients had intestinal metaplasia (IM), atrophic gastritis or moderate to severe non-atrophic gastritis, which can increase the risk of gastric cancer, compared to 7.5% of controls (p < 0.01). Focal nodular lymphoid hyperplasia, a precursor lesion for gastrointestinal lymphoma, was seen in eight CVID patients (16%), one of whom was diagnosed with gastrointestinal lymphoma on the same endoscopy. High-risk gastric pathology was associated with increased time since diagnosis of CVID, smoking, Helicobacter pylori, a low-serum pepsinogen I concentration, and diarrhea, but not pepsinogen I/II ratio, iron studies, vitamin B12 levels or upper gastrointestinal symptoms. There was a lower rate of detection of IM when fewer biopsies were taken, and IM and gastric atrophy were rarely predicted by the endoscopist macroscopically, highlighting the need for standardized biopsy protocols. The prevalence of premalignant gastric lesions in patients with CVID highlights the need for routine gastric screening. We propose a novel gastric screening protocol to detect early premalignant lesions and reduce the risk of gastric cancer and gastric lymphoma in these patients.

Myasthenia gravis and concurrent myositis following PD-L1 checkpoint inhibitor for non-small cell lung cancer.

BACKGROUND: There are increasing reports of myasthenia gravis (MG) following oncological treatment with immune checkpoint inhibitors (ICIs). METHODS AND RESULTS: A 66-year-old man with stage 3A lung adenocarcinoma was treated with second weekly infusions of durvalumab, a programmed cell death ligand-1 inhibitor, at a dose of 10 mg/kg. After the fourth infusion, he developed diplopia, dyspnoea and constitutional symptoms including headache, weakness and anorexia. 1 month later, he developed dysphagia and dysphonia. Examination showed proximal limb weakness with fatigability. An ice pack test was positive. Blood tests revealed a raised creatine kinase and positive PM-Scl75 antibody. Antititin antibody was strongly positive in the serum and cerebrospinal fluid. Antibodies for acetylcholinesterase receptor and antimuscle-specific kinase were negative. Electromyography showed myopathic changes. The patient was treated with steroids, pyridostigmine, mycophenolate mofetil and intravenous immunoglobulin. Eight weeks after treatment initiation ptosis, eye movements and limb strength were markedly improved and repeat creatine kinase was normal. CONCLUSION: Clinicians using ICIs should have a high index of suspicion for ICI-induced MG and concurrent myositis as disease can be severe and is associated with high mortality rates.

Anaphylaxis audit in a busy metropolitan Emergency Department: a review of real life management compared to best practice.

BACKGROUND: Deficiencies in anaphylaxis management in Emergency Departments is well recognised despite established guidelines for its treatment. OBJECTIVE: To identify deficiencies in the management of anaphylaxis in a busy metropolitan Emergency Department and determine if an education intervention could correct these. METHODS: Paediatric and adult admissions to the Emergency Department of a busy hospital were tracked over a 10-month period with a targeted educational program being instituted at 5 months. The electronic records were retrospectively reviewed looking for cases of anaphylaxis and milder forms of immediate type allergic reactions presenting with a combination of urticaria and nonairway threatening angioedema. Anaphylaxis presentation was graded using the Brown grading system. Use of all medication during resuscitation was documented. Observation period before discharge and referral to specialist unit for follow-up was noted. RESULTS: In the first 5 months, 38 patients fulfilled our criteria. Three had severe anaphylaxis, 13 had moderately severe anaphylaxis and 12 had urticaria and angioedema without anaphylaxis. Anaphylaxis was not always recognised or graded leading to inappropriate management with adrenaline often being withheld. Promethazine, usually given in parenteral form, was frequently administered. Observation time was often inadequate. Referral to an immunologist was not universally followed through. Following the educational intervention 58 patients fulfilled our criteria over the next 5 months. The appropriate use of adrenaline increased by 21% and the use of sedating antihistamines decreased by 16%, while the number of referrals to an immunologist increased by 24%. There was an 11% reduction in the number of patients who were observed for at least 4 hours. CONCLUSION: A number of deficiencies in the management of anaphylaxis presentations have been identified. Targeted educational activities aimed at the Emergency Department hospital staff may improve outcomes.

A case study of apple seed and grape allergy with sensitisation to nonspecific lipid transfer protein.

Lipid transfer proteins can be an important cause of allergy given their stability and high degree of protein sequence homology. We describe the case of a child who developed two separate episodes of anaphylaxis after consuming apple seed and grape, with evidence that nonspecific lipid transfer proteins may have been responsible for these reactions. Lipid transfer protein allergy should be considered when anaphylaxis is inconsistent, such as in patients who can tolerate fruit pulp but react to fresh whole fruit juices.

Pseudohypercalcaemia in mixed cryoglobulinaemia (IgMkappa/polyclonal IgG): a rare complication of Sjögren's syndrome.

This is the first reported case of pseudohypercalcaemia associated with type 2 cryoglobulinaemia, secondary to primary Sjögren's syndrome. Pseudohypercalcaemia is an asymptomatic syndrome and does not require specific treatment. However, cryoglobulins have been demonstrated to bind to calcium, affecting the cryoprecipitability, and consequently, altering the pathogenicity of the cryoglobulins. This first report highlights potential for further research into this intriguing phenomenon.