High prevalence of response to PPI treatment in children and adolescents with eosinophilic esophagitis in southern Brazil.

Introduction: Eosinophilic esophagitis is a newly recognized entity, in which there is significant evidence available that clearly demonstrates the positive impact of PPIs on reducing esophageal eosinophilia in individuals across different age groups, including children, adolescents, and adults. Multiple mechanisms have been proposed to explain how this treatment effect occurs. In Brazil, there seems to be a lack of studies that have prospectively assessed the clinical and therapeutic response rate in pediatric patients with EoE. The objective of this study was to prospectively evaluate the clinical and therapeutic response of pediatric patients with EoE in a medical center located in southern Brazil, by investigating the effectiveness of PPI treatment. Methods: This study is a clinical, prospective, open trial that took place in a pediatric hospital located in southern Brazil. The focus of the study was on patients diagnosed with Eosinophilic Esophagitis (EoE) who were given treatment using omeprazole/esomeprazole at a dosage of 1 mg.kg per dose, twice daily, for a period of 8-12 weeks. Following the treatment period, the patients underwent another endoscopy. Patients who exhibited 15 or less eosinophils in the biopsy conducted after the treatment were considered as responders. Results: A total of 27 patients was evaluated (74.1% boys). The average age (± standard deviation) was 8 years (±4). Nineteen patients (70.3%) were considered as responders to PPI treatment: 6 patients-22.2%-exhibited a complete response (defined as having 5 or fewer eosinophil per high power field. Additionally, 13 patients-48.1%-demonstrated a partial response, characterized by eosinophil counts exceeding 5 but less than 15 eos/hpf. When comparing the responder and non-responder groups at presentation, a statistical difference was observed in the prevalence of food refusal as a presenting symptom. Food refusal was found to be more prevalent in the non-responder group (87.5% vs. 26.3%, P = 0.008). No differences were observed in terms of atopy history and endoscopic scores. Upon comparing the histological findings from the post-treatment endoscopy of the two groups, it was observed that PPI responders exhibited a greater tendency to decrease basal cell hyperplasia (P = 0.06) and intercellular edema (P = 0.08). Conclusion: In this group of pediatric patients with EoE in Southern Brazil most patients showed a high prevalence of histological, endoscopic, and clinical response to PPI treatment. PPIs showed efficacy in Brazilian patients with EoE, most of whom would probably not be able to adequately undergo other treatments. Clinical Trial Registration: https://ensaiosclinicos.gov.br/rg/RBR-2ntbth9, identifier (U1111-1301-1842).

Eosinophilic Pneumonia Induced by Daptomycin.

Daptomycin-induced eosinophilic pneumonia (DIEP) is a rare but serious complication associated with the use of this broad-spectrum antibiotic. We present the case of a teenager with a history of nasopharyngeal cancer who developed DIEP while receiving daptomycin to treat an infection associated with an implanted chamber catheter. Symptoms included recurrent dyspnea and peripheral eosinophilia, with radiological findings consistent with DIEP. The pathophysiology involves an immune response triggered by daptomycin, resulting in eosinophilic pulmonary inflammation. Diagnosis requires a thorough evaluation of medical history, clinical laboratory tests, and radiological findings. The main treatment involves discontinuation of daptomycin and, in severe cases, the use of steroids. It is essential to consider DIEP in patients with respiratory failure and bilateral pulmonary opacities who have used daptomycin and to suspect it in those with blood eosinophilia or in bronchoalveolar lavage.

Fatal Itching and Failing Liver: A Case Report and Literature Review of Rare, Atypical DRESS (Drug Rash with Eosinophilia and Systemic Symptoms) Syndrome.

DRESS (Drug Rash with Eosinophilia and Systemic Symptoms) syndrome is a rare, life-threatening, hypersensitivity reaction. The prolonged course and non-specific symptoms of the condition make diagnosis challenging. We present a case of DRESS syndrome that was misdiagnosed as urticaria. Investigations revealed deranged liver and kidney functions and abnormal blood count. The presented case emphasizes the need to have a high suspicion for DRESS syndrome in patients who present with jaundice, generalized rash, acute renal failure, and acute liver failure.

The "great imitator": IgG4-related disease of the oral cavity. Two case reports and scoping review.

This study aimed to review the lesser-known intraoral manifestations of immunoglobulin G4-related disease (IgG4-RD). In this paper we report an unprecedented case of oral IgG4-RD mimicking angiolymphoid hyperplasia with eosinophilia (ALHE), and another case presenting as plasma cell gingivitis. We then performed a scoping review of published cases of IgG4-RD involving the oral cavity. The following data were collected for each case: age, sex, intraoral site(s) involved, clinical appearance, imaging features, serum IgG4 values, histopathology, treatment, and follow-up duration. Fifty-one cases of oral IgG4-RD were published in literature. The hard palate and jaw bones were the two main locations reported, while the histological identification of a IgG4/IgG plasma cells ratio ≥40% was fundamental for diagnosis. Conversely, the pathological features of storiform fibrosis and obliterative phlebitis were not common. Future reports regarding oral IgG4-RD should report clear adherence to the recognized international diagnostic criteria of the disease.

Dapagliflozin-induced drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Followed By Acute Interstitial Nephritis (AIN): A Case Report.

A 78-year-old male was admitted to the hospital with acute renal failure and generalized erythema after starting dapagliflozin 10 mg/day for chronic kidney disease (CKD). A skin biopsy revealed superficial perivascular dermatitis with eosinophils. A renal biopsy revealed lymphocytic and eosinophilic infiltration of the interstitium, and focal tubulitis. The patient was diagnosed with a dapagliflozin-induced drug reaction with eosinophilia and systemic symptoms (DRESS), followed by acute interstitial nephritis (AIN), and prednisolone therapy was therefore initiated. The patient's renal function improved, and erythema disappeared. To our knowledge, this is the first report of DRESS caused by dapagliflozin, and the patient was successfully treated with prednisolone.

Severe cutaneous adverse reactions associated with the immune checkpoint inhibitors: A case/non-case analysis using the Food and Drug Administration Adverse Event Reporting System.

BACKGROUND/OBJECTIVES: The immune checkpoint inhibitors (ICIs) have been increasingly associated with severe cutaneous adverse reactions (SCARs). These reactions, including Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), drug reaction with eosinophilia and systemic symptoms (DRESS) and acute generalized exanthematous pustulosis (AGEP) are uncommon but potentially lethal. Despite the severity of these reactions and growing association with the ICIs, their specific risk and mortality rates have been largely unexplored. METHODS: A case/non-case analysis was performed using data from the United States Food and Drug Administration (FDA) Adverse Event Reporting System (FAERS) to examine the reporting odds ratios (RORs) for ICI-associated SCARs cases under two conditions: (1) ICIs compared with all drugs in FAERS and (2) ICIs compared with a reference group of pooled anticancer drugs to control for underlying malignancy. RESULTS: A statistically significant ROR for SJS (ROR: 5.44), TEN (ROR: 5.81) and DRESS (ROR: 1.38) were identified under Condition 1. Under Condition 2, this significance was maintained for SJS (ROR: 7.31), TEN (ROR: 7.40) and DRESS (ROR: 3.90), and mild significance was identified for AGEP (ROR: 1.89). Mortality rates for the ICIs were increased compared with the anticancer medications (28.5% vs. 24.5% for SJS, 55.3% vs. 46% for TEN, 3.0% vs. 2.1% for AGEP and 7.1% vs. 6.1% for DRESS). CONCLUSIONS: Our results suggest an association between SCARs and the ICIs independent of cancer status.

Histopathology of Eosinophilic Gastrointestinal Diseases Beyond Eosinophilic Esophagitis.

Eosinophilic gastrointestinal diseases (EGID), such as eosinophilic gastritis (EoG), eosinophilic enteritis, and eosinophilic colitis (EoC), are chronic inflammatory conditions characterized by persistent gastrointestinal symptoms and elevated levels of activated eosinophils in the gastrointestinal tract. EoG and eosinophilic duodenitis (EoD) are strongly associated with food allergen triggers and TH2 inflammation, whereas EoC shows minimal transcriptomic overlap with other EGIDs. The level of expression of certain genes associated with TH2 immune response is associated with certain histopathologic findings of EoG, EoD, and EoC. Current immune therapy for EoG depletes tissue eosinophilia with persistence of other histopathologic features of disease.

Drug-induced hypersensitivity syndrome related to piperacillin-tazobactam: a case report and review of the literature.

Allergic reactions to drugs caused by piperacillin-tazobactam are common in clinical practice. However, we also found a few cases of drug-induced hypersensitivity syndrome (DiHS)/Drug reaction with eosinophilia and systemic symptoms (DRESS) caused by piperacillin-tazobactam in our clinical work. We report a case of a 60-year-old female patient who was treated with piperacillin-tazobactam anti-infective therapy after the diagnosis of hematogenous lung abscess, developed fever, rash, and blood abnormalities after 26 days of application, and was later diagnosed as DIHS, which was improved after the administration of glucocorticoid and anti-allergic drugs. In addition, we also retrospectively analyzed 17 cases of DiHS caused by piperacillin-tazobactam from the PubMed databases between March 1980 and September 2023. The majority of the patients had an incubation period of more than 14 days, and the common clinical features included elevated eosinophil count/percentage, fever, rash, liver damage, and lymph node enlargement. After treatment with topical or systemic glucocorticoids, 16 of the 17 patients improved and one died because of the underlying condition. The clinical features of DiHS were diverse and included a long incubation period, skin rash, elevated eosinophils, and impaired organ function. Since some patients have atypical clinical features, clinicians should raise awareness of the disease, recognize these features early, and treat them promptly.

Coexistence of Eosinophilic Granulomatosis With Polyangiitis and Allergic Bronchopulmonary Aspergillosis: A Fascinating Relationship.

Elevated eosinophil counts are associated with various diseases, including eosinophilic granulomatosis with polyangiitis (EGPA) and allergic bronchopulmonary aspergillosis (ABPA). EGPA is a rare small-vessel vasculitis characterized by asthma, eosinophilia, fleeting pulmonary infiltrates, and systemic manifestations. ABPA, initiated by immune reactions against Aspergillus fumigatus in the airways, presents with poorly controlled asthma, wheezing, hemoptysis, productive cough, and systemic symptoms, which result in characteristic central bronchiectasis. Fleeting pulmonary opacities are common radiologic findings. We present a case of ABPA in a patient with a prior EGPA diagnosis under treatment with mepolizumab 300 mg monthly and review eight similar cases from the literature. In these cases, EGPA and ABPA diagnoses preceded each other or were concurrent. Treatment of the latter improved control of both diseases. IL-5 is pivotal in EGPA pathogenesis, and mepolizumab, targeting IL-5, has been effective in EGPA treatment. Our patient received mepolizumab for EGPA and continued it post-ABPA diagnosis, showing favorable outcomes. This suggests mepolizumab as a therapeutic link between EGPA and ABPA. Mepolizumab therapy holds promise for managing both EGPA and ABPA. Double-blind placebo-controlled studies are warranted to establish its efficacy and safety for ABPA, emphasizing the need for further research in this area.

Heparin-induced DRESS syndrome in a paediatric patient and successful anaesthetic management in cardiovascular bypass surgery: case report.

BACKGROUND: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome is a severe adverse drug reaction marked by delayed hypersensitivity reactions causing skin and systemic complications. DRESS diagnosis is challenging due to the variety of clinical presentations and symptom overlap with other conditions. The perioperative period in these patients requires precise pharmacological strategies to prevent complications associated with this syndrome. The treatment of DRESS induced by unfractionated heparin during cardiopulmonary bypass (CPB) surgery presents some challenges that must be considered when selecting an anticoagulant to avoid side effects. In this case, bivalirudin, a direct thrombin inhibitor, is indicated as an alternative to heparin in patients undergoing CPB. However, in contrast to heparin/protamine, there is no direct reversal agent for bivalirudin. CASE PRESENTATION: We report the case of an 11-year-old male diagnosed with native aortic valve endocarditis and thrombosis in his left lower extremity. During valvular replacement surgery, systemic unfractionated heparin was administered. Postoperatively, the patient developed fever, eosinophilia and pruritic rash. Warm shock and elevated alanine transaminase (ALT) and aspartate transaminase (AST) levels followed, leading to the diagnosis of DRESS syndrome. Treatment with methylprednisolone resulted in complete resolution of symptoms. Seven years later, the patient was readmitted due to insufficient anticoagulation and a thrombus in the prosthetic aortic valve, presenting a recurrent DRESS episode due to the administration of unfractionated heparin, which was later replaced with low-molecular-weight heparin during hospitalization. Treatment with corticosteroids and antihistamines was initiated, resulting in the resolution of this episode. Ultimately, the patient required the Ross procedure. During this intervention the anticoagulation strategy was modified, unfractionated heparin was replaced with bivalirudin during the procedure and fondaparinux was administered during the postoperative period. This resulted in stable transaminases levels and no eosinophilia. CONCLUSION: The severity of DRESS Syndrome underscores the importance of early recognition, heightened monitoring, and a comprehensive approach tailored to each patient's needs. This particular case highlights the significance of this approach and may have a substantial clinical impact since it provides alternatives to heparin, such as bivalirudin and fondaparinux, in the anticoagulation strategy of CPB for patients who have a hypersensibility reaction to this medication; thus, enhancing clinical outcomes by minimizing risks linked to adverse drug reactions.

[Syndromic approach in migrant patients: Fever, diarrhea, anemia, eosinophilia and chronic cough]. / Abordaje sindrómico en el paciente inmigrante: fiebre, diarrea, anemia, eosinofilia y tos crónica.

Migrant patients share the same diseases as natives, but biological or environmental differences may lead to distinct prevalence and manifestations of certain syndromes. Some common conditions in Primary Care stand out, such as fever, diarrhea, anemia, eosinophilia, and chronic cough, where it is important to have a special consideration. Fever may indicate a serious imported illness, and malaria should always be ruled out. Diarrhea is generally of infectious origin, and in most cases, management is outpatient. Anemia may indicate malnutrition or malabsorption, while eosinophilia may indicate a parasitic infection. Lastly, chronic cough may be a sign of tuberculosis, especially in immigrants from endemic areas. Family medicine holds a privileged position for the comprehensive, culturally sensitive, and person-centered approach to these conditions.

Sudden Visual Loss due to Arteritic Anterior Ischaemic Optic Neuropathy: A Rare Manifestation of Eosinophilic Granulomatosis with Polyangiitis.

Background: eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem inflammatory disease characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of various organs. While the eye is uncommonly affected in patients with EGPA, multiple ophthalmic manifestations have been reported, which can result in serious visual impairment without timely treatment. Case report: we report the case of a 79-year-old woman with a history of asthma and nasal polyps who presented with low-grade fever, mild alteration of mental status, and fatigue. Chest X-ray revealed bilateral interstitial infiltrates. Lab tests showed elevated C-reactive protein level and eosinophilia (eosinophil count, 4.6 x109 cells/l); blood cultures and parasitological examination of stools tested negative. Four days after presentation, the patient reported sudden and severe blurring of vision in her left eye. Ophthalmological examination revealed bilateral swollen optic disc and visual field loss, more severe in the left eye. A diagnosis of EGPA complicated by arteritic anterior ischaemic optic neuropathy (A-AION) was proposed, while an alternative or concurrent diagnosis of giant cell arteritis was ruled out based on clinical picture. Immunosuppressive treatment with high-dose intravenous glucocorticoids was promptly started. The patient's visual defect did not improve; however, two months later, no worsening was registered on ophthalmic reassessment. Conclusions: A-AION is an infrequent but severe manifestation of EGPA, requiring prompt diagnosis and emergency-level glucocorticoid therapy to prevent any further vision loss. Disease awareness and a multidisciplinary approach are crucial to expedite diagnostic work-up and effective management of EGPA-related ocular complications. LEARNING POINTS: Arteritic ischaemic optical neuropathy is a potential cause of sudden and severe visual loss in eosinophilic granulomatosis with polyangiitis (EGPA) patients.Visual loss due to arteritic ischaemic optical neuropathy is rarely reversible; however, a timely glucocorticoid treatment may prevent further progression of visual impairment.Multidisciplinary approach is crucial to expedite diagnostic work-up and effective management of EGPA patients with ocular complications.

Navigating Asymptomatic Idiopathic Pneumoperitoneum: A Case Report and a Literature Review.

Pneumoperitoneum refers to the presence of free air in the abdominal cavity, typically indicating viscus perforation requiring urgent surgical intervention. Occasionally, pneumoperitoneum occurs without organ perforation, termed 'spontaneous' or 'non-surgical' pneumoperitoneum. We present the case of a 65-year-old male referred to the emergency department after a seizure episode. The patient reported no abdominal pain or fever, and examination revealed no other signs of peritonitis. An erect chest X-ray showed air under the diaphragm, and a subsequent computed tomography (CT) scan confirmed free intraperitoneal air in the abdomen. The patient underwent a prompt exploratory laparotomy to assess for abdominal perforation, but the findings were negative. He had an uneventful post-operative hospital course and was discharged nine days after admission. This case underlines the importance of considering spontaneous idiopathic pneumoperitoneum (SIP) in asymptomatic patients and discusses management options.

A Rare Pediatric Case of Allopurinol-Induced Drug Reaction With Eosinophilia and Systemic Symptoms (DRESS) Successfully Treated With Intravenous Immunoglobulins.

Allopurinol-induced drug reaction syndrome with eosinophilia and systemic symptoms (A-DRESS) is a well-described condition in adults, whereas it is uncommon among children. We describe a case of A-DRESS in a 16-year-old male with steroid-dependent nephrotic syndrome. He presented a life-threatening clinical course with persisting fever, skin rash, eosinophilia, lymphadenopathy, distributive shock, and herpesvirus 6 detection. The withdrawal of allopurinol and a combination of intravenous immunoglobulins (IVIGs) and systemic corticosteroids led to the patient's recovery without sequelae. Drug reaction with eosinophilia and systemic symptoms (DRESS) in pediatrics is rare and can present in a severe form. Early diagnosis and timely treatment are critical for prognostic purposes. This report suggests the potentially crucial role of IVIG in the treatment of patients with A-DRESS.

Interstitial Pneumonia Associated with Nodal T-follicular Helper Cell Lymphoma: A Case Report.

Nodal T-follicular helper cell lymphoma (nTFHL), a hematologic neoplasm originating from T-follicular helper (TFH) cells, occasionally presents with pulmonary radiographic abnormalities, without neoplastic cellular infiltration. However, the precise mechanisms underlying non-neoplastic pulmonary opacities in patients with nTFHL remain unclear. Previous reports have shown that TFH cell abnormalities are associated with collagen disease and interstitial pneumonia with autoimmune features (IPAF). We herein report a patient with nTFHL accompanied by interstitial pneumonia diagnosed via lung and lymph node biopsies. These findings suggest the need to rule out nTFHL before diagnosing IPAF.

Toxicology and safety study of L-tryptophan and its impurities for use in swine.

L-tryptophan, an essential amino acid for physiological processes, metabolism, development, and growth of organisms, is widely utilized in animal nutrition and human health as a feed additive and nutritional supplement, respectively. Despite its known benefits, safety concerns have arisen due to an eosinophilia-myalgia syndrome (EMS) outbreak linked to L-tryptophan consumed by humans. Extensive research has established that the EMS outbreak was caused by an L-tryptophan product that contained certain impurities. Therefore, safety validations are imperative to endorse the use of L-tryptophan as a supplement or a feed additive. This study was conducted in tertiary hybrid [(Landrace × Yorkshire) × Duroc] pigs to assess general toxicity and potential risks for EMS-related symptoms associated with L-tryptophan used as a feed additive. Our investigation elucidated the relationship between L-tryptophan and EMS in swine. No mortalities or clinical signs were observed in any animals during the administration period, and the test substance did not induce toxic effects. Hematological analysis and histopathological examination revealed no changes in EMS-related parameters, such as eosinophil counts, lung lesions, skin lesions, or muscle atrophy. Furthermore, no test substance-related changes occurred in other general toxicological parameters. Through analyzing the tissues and organs of swine, most of the L-tryptophan impurities that may cause EMS were not retained. Based on these findings, we concluded that incorporating L-tryptophan and its impurities into the diet does not induce EMS in swine. Consequently, L-tryptophan may be used as a feed additive throughout all growth stages of swine without safety concerns.

[When eosinophilia is not only a marker of asthma ]. / Cas clinique. Quand l'éosinophilie n'est pas qu'un marqueur d'asthme...

We report the case of a patient who has been hospitalized for dyspnea. Investigations revealed airway obstruction, eosinophilia, elevated IgE and elevated exhaled nitric oxide. Patient improved with oral corticosteroids (OCS). However, the patient presented two exacerbations requiring OCS during the next twelve months. Chest CT scan revealed two multiloculated parenchymal lesions. Lab test was positive for Echinococcus and Western-Blot confirmed infection with Echinococcus granulosus. Bronchoalveolar lavage confirmed the presence of 6 % eosinophils. Echinococcus granulosis is a zoonotic larval infection caused by a tapeworm larva. Patients with this disease may be asymptomatic for years. Early identification and management, in a multidisciplinary team, are essential and rely mainly on surgical intervention and antiparasitic treatments. This article presents the case of a young patient with pulmonary echinococcosis.

Nous rapportons le cas d'un patient ayant été hospitalisé dans un contexte d'obstruction bronchique, avec une légère éosinophilie, une élévation des IgE et du monoxyde d'azote dans l'air exhalé, qui a évolué favorablement sous corticostéroïdes oraux (CSO). L'évolution est marquée par deux exacerbations d'asthme d'évolution favorable sous CSO dans les douze mois de suivi. Une tomodensitométrie thoracique révèle la présence de deux lésions pulmonaires kystiques. Les sérologies infectieuses mettent en évidence une positivité pour l'espèce -Echinococcus et une confirmation pour l'Echinococcus granulosus. Le lavage broncho-alvéolaire retrouve une hyperéosinophilie à 6 %. L'échinococcose kystique est une infection larvaire zoonotique causée par une larve de taenia. Les patients atteints de cette maladie peuvent être asymptomatiques pendant de nombreuses années. Une identification précoce et une prise en charge adéquate, en équipe pluridisciplinaire, sont primordiales et reposent essentiellement sur une intervention chirurgicale et des traitements anti-parasitaires. Cet article présente le cas d'un jeune patient atteint d'une échinococcose kystique pulmonaire.

Peripheral Blood Eosinophilia in Patients with Diabetic Foot Infection Receiving Long-Term Antibiotic Therapy.

Background: The eosinophil level in peripheral blood increases in response to various conditions, the most common being medication use. Since the outcome of increased levels of eosinophils can range from a benign finding to extensive damage to host organs and systemic consequences, this finding raises concern among clinicians. We aimed to assess the prevalence of prolonged antibiotic-therapy-induced eosinophilia and possible outcomes. Methods: We conducted a retrospective cohort study of diabetic patients admitted to the orthopedic department from December 2016 through December 2020 due to a moderate to severe diabetic foot infection and who received at least 14 days of antibiotic therapy. Patients were identified retrospectively through the orthopedic department registry, and their files were reviewed, extracting demographics, laboratory test results, antibiotic treatment, and outcomes. Results: The cohort included 347 patients; a total of 114 (32.8%) developed eosinophilia during the follow-up period. Patients who developed eosinophilia had a significantly longer duration of antibiotic treatment (p < 0.001) and a significantly longer hospitalization (p = 0.001). For multivariable analysis, the independent risk factors predicting drug-induced eosinophilia included older age, higher eosinophil count on admission (per quantile) and higher platelet count on admission (per quantile) (p = 0.012, p < 0.001, p = 0.009, respectively). There was no evidence of complications in patients who developed eosinophilia compared to patients who did not. No significant association with a specific type of antibiotic was found. Conclusions: We found a higher incidence of drug-induced eosinophilia than expected or previously described. The factors associated with eosinophilia included age and higher baseline eosinophil and platelet levels but not antibiotic type.