Tongue Edema Research Articles

The Clinical Usability Evaluation of an Attachable Video Laryngoscope in the Simulated Tracheal Intubation Scenario: A Manikin Study.

The aim of this study was to assess the usefulness of an attachable video laryngoscope (AVL) by attaching a camera and a monitor to a conventional Macintosh laryngoscope (CML). Normal and tongue edema airway scenarios were simulated using a manikin. Twenty physicians performed tracheal intubations using CML, AVL, Pentax Airwayscope® (AWS), and McGrath MAC® (MAC) in each scenario. Ten physicians who had clinical experience in using tracheal intubation were designated as the skilled group, and another ten physicians who were affiliated with other departments and had little clinical experience using tracheal intubation were designated as the unskilled group. The time required for intubation and the success rate were recorded. The degree of difficulty of use and glottic view assessment were scored by participants. All 20 participants successfully completed the study. There was no difference in tracheal intubation success rate and intubation time in the normal airway scenario in both skilled and unskilled groups. In the experienced group, AWS had the highest success rate (100%) in the tongue edema airway scenario, followed by AVL (60%), MAC (60%), and CML (10%) (p = 0.001). The time required to intubate using AWS was significantly shorter than that with AVL (10.2 s vs. 19.2 s) or MAC (10.2 s vs. 20.4 s, p = 0.007). The difficulty of using AVL was significantly lower than that of CML (7.8 vs. 2.8; p < 0.001). For the experienced group, AVL was interpreted as being inferior to AWS but better than MAC. Similarly, in the unskilled group, AVL had a similar success rate and tracheal intubation time as MAC in the tongue edema scenario, but this was not statistically significant. The difficulty of using AVL was significantly lower than that of CML (8.8 vs. 3.3; p < 0.001). AVL may be an alternative for VL.

Recent safety and efficacy findings from a phase 1b/2 open-label combination study of ASP-1929 photoimmunotherapy with anti-PD-1 therapy in EGFR-expressing advanced head and neck squamous cell carcinoma (HNSCC).

6083 Background: In the challenging landscape of advanced HNSCC, patients (pts) have overall low survival and high locoregional (LR) recurrence rates. While LR progression is a key contributor to morbidity and mortality, LR control remains low despite aggressive therapy including surgery, radiation, chemotherapy, and immunotherapy. ASP-1929 photoimmunotherapy (PIT) combines EGFR-targeting cetuximab, with a light-activatable dye, IRDye 700DX, and a laser light for localized drug activation. This novel approach has demonstrated rapid and selective tumor necrosis in preclinical studies, and a manageable safety profile in HNSCC early clinical trials. Phase I/II and preclinical data indicate a potential synergy in anti-tumor activity of PIT when combined with anti-PD-1 therapy. Here we present updated interim evaluation findings based on a recent data cut from this study initially presented at the 2023 American Head and Neck Society meeting. Methods: A phase 1b/2 open-label study of pts with recurrent locally advanced (rLA) and/or metastatic (m) HNSCC evaluating the safety and efficacy of ASP-1929 PIT in combination with anti-PD-1 (pembrolizumab) therapy (NCT04305795). Pts with measurable disease by modified RECIST 1.1, at least 1 lesion accessible to light illumination, combined positive score (CPS) ≥1, and who were not candidate for LR therapy could be eligible for the study. Treatment included ASP-1929 PIT (infusion day 8, target tumors illuminated 24±4 hours later) plus anti-PD-1 (days 1&amp;22) during a 6-week cycle for up to 24 months. Primary objectives: safety/tolerability; objective response rate (ORR). Secondary objectives: overall survival (OS); progression-free survival (PFS); duration of response (DOR). Planned sample size: 26 HNSCC pts. Results: Of the 19 r/m HNSCC pts enrolled, 18 pts received both study therapies (PIT-evaluable). Data cut-off was August 31, 2023 (previously reported data cut-off: October 4, 2022). Efficacy results updated for the 18 PIT-evaluable pts: ORR of 33.3% (95% CI 13.3-59.0), including 4 CRs (22.2%) and 2 PRs (11.1%). Median OS not reached; 24-month OS rate estimate was 52.4% (95%CI 25.9-73.4). No change in median PFS, 2.9 months (95%CI 1.4-14.6). Median DOR was not reached at data cut-off (range 2.8+ to 18.0+ months). Safety data remained consistent with the data previously presented. Most common serious adverse reactions were dysphagia (10.5%) and tongue edema (10.5%). There were two grade 4 events: laryngeal edema (PIT-related) and tumor hemorrhage due to advanced disease, and no fatal events. Conclusions: ASP-1929 PIT in combination with anti-PD-1 therapy was generally well tolerated. Initial data demonstrate promising overall survival and response rates with this combination therapy in pts with rLA and/or m HNSCC lacking LR treatment options. Clinical trial information: NCT04305795 .

Drug-induced Tongue Disorders: A Comprehensive Literature Review.

Some drugs cause tongue disorders as adverse effects. Most of the druginduced tongue disorders are benign and will resolve after drug discontinuation. However, the changes in the color or appearance of the tongue may frighten patients and decrease compliance with drug therapy. To review the literature to find all reports of drug-induced tongue disorders, their presentation, management, and outcome of patients Methods: The search was conducted in Google Scholar and PubMed using key words "ageusia," "burning tongue," "coated tongue," "drug-induced taste disturbances," "dysgeusia," "glossitis," "glossodynia," "hairy tongue," "hypogeusia," "stomatodynia," "stomatopyrosis," "swollen tongue" "tongue discoloration," "tongue irritation," "tongue numbness, "tongue oedema," and "tongue ulcer. All reports that were published from 1980 to 2022 in the English language were included in the study. Reports that were not in English language but had English abstracts with adequate data for extraction were also included. A total of 208 case reports and case series were included. The most reported drug classes were antineoplastic and immunomodulating agents and anti-infectives for systemic use, and the most common tongue disorders were tongue discoloration and black hairy tongue. Having good oral hygiene and discontinuing the offending drug could manage and resolve the problem. Drug-induced tongue disorders are not rare adverse drug reactions. They are benign in most cases, and withholding offending agents results in significant improvement or complete resolution of tongue lesions.

Tongue edema as an adverse drug reaction to low-dose olanzapine in a cancer patient receiving palliative care.

Olanzapine is an atypical neuroleptic indicated for treatment of various psychiatric disorders, but it has also several indications in palliative care (PC) patients: opioids misuse, nausea not related to chemotherapy, anorexia-cachexia syndrome, and sleep and mood disorders. Peripheral and facial edema are a rare side effect of the treatment with olanzapine. I report a case of an advanced cancer patient cared receiving PC who developed moderate tongue edema on day 1 of a low dose of olanzapine. A patient with advance and metastatic colon cancer presented moderate tongue edema on day 1 of a low dose (2.5 mg) of olanzapine for the treatment of his nausea, anorexia-cachexia syndrome, and mood disorder (mainly anxiety). The patient discontinued the drug with resolution of the edema. The day after he called our outpatients' service, a prompt physical examination, together with blood tests, excluded other differential diagnosis. To the best of our knowledge, this is the second case reporting head and neck localized edema due to olanzapine treatment in a patient with advanced cancer receiving PC. Considering the increasing use of olanzapine as off-label treatment in these patients (often for cluster symptoms), our report could help clinicians in daily practice and researchers on put a deeper focus on indications for olanzapine in PC.

Role of epinephrine in attenuating cytokine storm, decreasing ferritin, and inhibiting ferroptosis in SARS-CoV-2

BackgroundSevere acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is the virus responsible for coronavirus disease 2019. It presents one of the most threatening pandemics in the history of humanity. The mortality and morbidity represent an unprecedented challenge to the modern medical era. SARS-CoV-2 results in acute respiratory distress syndrome, high concentrations of proinflammatory mediators, cytokine storm (CS) due to massive release of cytokines, hypercoagulation, and hemoglobin disintegration. Dysregulation of iron homeostasis, iron overload as indicated by high ferritin level, and ferroptosis are major factors in the pathogenesis of the disease. We report a case of SARS-CoV-2 in which the use of epinephrine (Epi) resulted in an unexpected attenuation of CS, decreasing ferritin level and inhibiting ferroptosis.Case presentationA 64-year-old male patient with a history of multiple medical comorbidities had been diagnosed with SARS-CoV-2. Further evaluation showed marked increase in inflammatory markers, severe hyperferritinemia, and lymphopenia in laboratory blood tests. The characteristic score of CS was strongly positive, and in addition to regular treatment, the patient received Epi due to development of acute generalized skin rash, severe itching, and edema of lips and tongue. Epi may have successfully terminated not only the acute cutaneous condition, but also have attenuated CS, decreased ferritin level, and other inflammatory markers in addition to complete patient’s recovery.ConclusionEpinephrine may attenuate CS and inhibit ferroptosis which is an iron-dependent, non-apoptotic mode of cell death. Epi interacts with ferric and/or ferrous iron and built a stable complex that impedes activation of beta-adrenergic receptors. Epi may cause marked decrease of ferritin and other inflammatory markers. Epi may be used to decrease iron overload which is associated with many medical diseases like type 2 diabetes mellitus and cardiometabolic diseases such as coronary heart disease and cerebrovascular disease. As a new clinical indication extensive studies are required for further assessment and possible therapeutic uses.

Detection of tongue edema caused by endotracheal intubation tube in ICU patients by ultrasonography: a prospective, observational, clinical study.

We aimed to determine the tongue edema that may develop due to the pressure exerted by the endotracheal intubation (ETI) tube on the tongue during the follow-up period of mechanical ventilation in patients intubated in the intensive care unit (ICU) by submental ultrasonography (USG). Also, we aimed to investigate the effect of tongue edema on the oxygen saturation values (SpO2) measured two h after extubation. One hundred patients aged 18-65 years, who were followed up with mechanical ventilation in the ICU from the 0th day of intubation, were included. Patients (n = 57) who were followed up on mechanical ventilation for four days or longer after ETI were included in the study group. Those who were followed up on mechanical ventilation for three days or less after endotracheal intubation and were extubated during this period were included in the control group (n = 43). The tongue cross-sectional areas (TSAs) of patients in both groups were measured twice with submental USG. The first measurement (TSA1) was performed on the 0th day of ETI in all patients in both groups. The second measurement (TSA2) was performed on the 4th day of ETI in the study group and just before extubation in the control group. The difference between TSA2 and TSA1 was defined as tongue edema. Also, the effect of tongue edema on the oxygen saturation levels measured at the 2nd h after extubation of the patients in the control group was investigated. The tongue edema was more prevalent in the study group (p < 0.01). A significant negative correlation was found between the SpO2 levels two hours after extubation and the increase in the mean TSA values indicating tongue edema (p < 0.01). The oxygen saturations of the patients with tongue edema were 4% lower than those without tongue edema. Tongue edema may develop due to the long-term application of pressure of the ETI tube on the tongue and may impair oxygenation after extubation. NCT05249738.

Tongue Edema Caused by Taking Nifedipine Controlled-Release Tablets.

Tongue Edema Caused by Taking Nifedipine Controlled-Release Tablets.

Clinical profile of paraphenylenediamine intoxication

Hair dyes are widely used for decorative skin paint, nail coloring, and hair dye. Hair dye toxicity has been attributed to adding a synthetic dye paraphenylenediamine (PPD). PPD is added to natural henna to accentuate the dark color and shorten the duration of application. PPD self-harm or accidental toxicity is well-known in developing countries and extensively reported in the medical literature. Acute intoxication with PPD causes characteristic severe angioedema of the pharynx and larynx which usually necessitates tracheostomy, and it is associated with facial and tongue edema. PPD poisoning results in multisystem disorders and can cause rhabdomyolysis and acute kidney injury, aggressive gastrointestinal manifestations, pancarditis, and arrhythmias. This form of severe poisoning is fatal if not treated early and properly. There is no specific antidote and treatment is mainly supportive and helpful only if initiated early.

Intoxicação espontânea e experimental por Myrocarpus frondosus em bovinos

ABSTRACT: An outbreak of photosensitization in Holstein and Jersey cattle occurred after the passage of an extratropical cyclone in Southern Brazil. On that occasion, several trees fell, including some of the species Myrocarpus frondosus (Fabaceae family) popularly known as “gabreúva”, “cabriúna” or “gabriúna”. Five heifers consumed the leaves of this plant, and all of them became ill. The main clinical signs were decreased appetite, permanence in the water, decreased ruminal movements, dry stools, congested and icteric sclera and conjunctivae, dark urine and restlessness when exposed to the sun. One heifer showed circling, incoordination, restlessness and death six days after the onset of clinical signs. From the seventh day, three heifers showed improvement; one remained apathetic, with jaundiced mucous membranes, ulceration, and scaling on the tongue, increased respiratory rate, and fever, and died 18 days after ingestion of the plant. At necropsy, photosensitivity lesions were observed on the depigmented skin, snout, teats, and ear tip, characterized by discontinuity of the skin with the formation of crusts, inflammatory exudate associated with redness of the skin, in addition to ulceration in the mouth, snout and ventral portion of the tongue and jaundice and edema in the submandibular region. The liver was enlarged, with an orange color, full gallbladder, and wall edema. Through microscopy, liver lesions were characterized by moderate and diffuse necrosis of hepatocytes, vacuolar and centrilobular degeneration, bilirubin retention and proliferation of biliary epithelium. The literature shows no reports of M. frondosus toxicity in cattle. Experiments were carried out in cattle to clarify the possible clinical-pathological picture produced by this plant. A survey of the epidemiological data of the diagnosed spontaneous outbreak was carried out, and subsequently, three cattle received the plant’s green leaves, and a fourth received the dried leaves. After administering the plant, blood samples were collected daily for blood count, measurement of liver enzymes (gamma glutamyl transferase, alkaline phosphatase, alanine aminotransferase, aspartate transferase), bilirubin, measurement of vital parameters and liver biopsy from the onset of signs. In case of death, a necropsy was performed with collection of viscera samples for macro and microscopic evaluation. The experimental part was developed at the Laboratory of Animal Pathology, CAV-UDESC. The single dose of 33g/kg of green leaves caused severe clinical signs, while the administration of 22g/kg and 11g/kg caused mild to moderate signs of the disease. The supply of desiccated leaves in a single dose did not cause clinical manifestations. M. frondosus can be blamed as a cause of spontaneous hepatogenous photosensitization in cattle. Experimentally, the ingestion of green leaves of this plant, in doses greater than 11g/kg can lead to clinical changes, with subsequent recovery, or progress to death with severe liver damage.

Study of postoperative laryngopharyngeal discomfort: protocol for a single-centre cohort study

IntroductionPostoperative laryngopharyngeal discomfort after extubation can lead to severe throat pain, dysphagia, or postoperative tongue oedema. Possible mechanisms include increased oral pressure, obstruction of venous and lymphatic return in the...

A randomized crossover comparison of the bébé VieScope and direct laryngoscope for pediatric airway management by nurses in medical simulation settings

ABSTRACT Background Airway management in life-threatening emergencies is essential for children, and endotracheal intubation is the gold standard. It protects against regurgitation and enables mechanical ventilation. New types of airway management equipment are being developed and implemented to meet the needs of medical personnel. Research design and methods This prospective, randomized, cross-over simulation trial evaluated the success of endotracheal intubation in three scenarios: normal airway (scenario-A), tongue edema (scenario-B), and continuous chest compression (scenario-C), using the bébé Vie Scope™ laryngoscope (VieScope) and the Macintosh blade laryngoscope (MAC) as a comparative tool performed by nurses with limited tracheal intubation experience. Results The results of the study showed that in scenario-A, there were no significant differences in the first attempt success rate or endotracheal intubation time between VieScope and MAC. However, VieScope was associated with better visualization of the glottis. In scenarios B and C, VieScope was significantly more effective than MAC in terms of first-pass success rate, time to intubate, Cormack-Lehane grade, POGO score, and ease of endotracheal intubation. Conclusions Bébé VieScope may be useful for endotracheal intubation in pediatric patients, particularly in cases of tongue edema and ongoing chest compression, providing a higher first-pass success rate than conventional laryngoscopes.

Life-threatening non-angioneurotic swelling of the tongue as a complication of frenotomy in an eight-year-old boy

Introduction: Frenotomy is considered a safe procedure. The rare complications include bleeding, scarring and occasionally others such as those related to airway obstruction and hypovolemic shock. Case report: An 8-year-old boy with a history of tongue oedema and allergy underwent frenotomy under local anaesthesia. The patient was discharged home without any complications. The following day, a massive swelling of the tongue appeared, requiring hospitalisation. Revision surgery was immediately performed and haematoma was ruled out as a cause of the oedema. COVID-19 infection was detected. As the patient’s general condition had deteriorated, he was intubated and fed through a nasogastric tube. A subsequent pharmacological treatment using intravenous steroids and antihistamines was administered, causing the tongue swelling to gradually subside. The patient was extubated on the 4th day following his admission to hospital. Quincke’s oedema, allergic reaction to an inhalant or food allergens, and the local anaesthetics were excluded as possible causes of the oedema. The cause was not determined, though a hypothesis was put forward that it was related to the COVID-19 infection. The patient was discharged from hospital after 14 days in good general condition. Conclusion: A history of tongue swelling increases the risk of complications after frenotomy and requires an extended observation period

Lingual abscess, an uncommon cause of tongue oedema: Case series

Lingual abscess is a rare but potentially life-threatening medical entity. Consequently, it requires high medical suspicion in order to prevent misdiagnosis. Unilateral tongue oedema constitutes the characteristic clinical feature.

Sudden Death Due to Undiagnosed Ludwig’s Angina: An Autopsy Case Report

Ludwig’s angina is a rapidly progressive and gangrenous cellulitis in the submandibular, sublingual spaces, and neck. It is characterized by an elevated tongue and laryngeal edema, resulting in airway compromise and, rarely, in sudden death. The most common cause is an odontogenic infection of a molar tooth. Infection can spread to the superior mediastinum and buccal area through anatomical structures. Herein, we report the sudden death of a 45-year-old male who was not diagnosed with Ludwig’s angina. He died in the emergency room 20 minutes after loss of consciousness. Intubation was impossible owing to severe laryngeal edema. The C-reactive protein level was 33.81 mg/dL. On autopsy, a diffuse light green abscess of the submandibular space was detected, along with severe edema of the epiglottis, aryepiglottic fold, and vocal cords. Subsequently, we confirmed that the airway obstruction was caused by Ludwig’s angina based on a literature review. In conclusion, this report emphasizes the need to identify the infection source and perform a detailed dissection according to the anatomical structure in the autopsy procedure for Ludwig’s angina.

ENT manifestations of Kala Pathar poisoning; An experience at Nishter Hospital, Multan, Pakistan.

Objective: To know the demographic representation along with possible ENT manifestations of Black Stone Poisoning. Study Design: Cross Sectional study. Setting: Emergency and Wards in Nishtar Hospital Multan. Period: April 2018 to December 2018. Material &amp; Methods: The study was executed in total of 156 Patients with history of Black stone ingestion in Emergency and wards in Nishtar Hospital Multan. All included patients were having history of black stone ingestion along with ENT complications following black stone intake. All cases were excluded who were dead or unresponsive. Results: Most of the cases, 119 (76.3%) were females, between the age group of 11-20 years (41.66%) and 129 (82.7%) belonged to rural areas. Out of 156 subjects, 137 (87.8%) ingested black stone with intentions of suicide. It was noted that 102 (74.4%) patients ingested black stone due to family related issues. Most common ENT manifestations were hoarseness of voice in 149 (95.5%), uvula and pillars edema in 143 (91.7%), tongue edema in 140 (89.7%) and neck swelling in 131 (84.0%). Conclusion: Women more than men, young people more than any of the other ages, rural population more than urban and people living in stressful environment are prone to Black stone ingestion. Lips and tongue edema, neck swelling, odynophagia, vestibulitis, limited mouth opening, ulceration of buccal and nasal mucosa and epistaxis are the commonest ENT manifestations of black stone poisoning.

Safety of Cefazolin Test Dose in Patients With Penicillin Allergy Just Prior to Cardiac Device Implantation: A Single-Centre Experience

BackgroundCephalosporins are the cornerstone of cardiac device infection prophylaxis. Owing to fears of cross-reactivity, penicillin-allergic patients are exposed to potentially more-toxic drugs, with decreased efficacy. We evaluated the safety of a cefazolin test dose (CTD) in self-reported penicillin-allergic patients. MethodsIn this single-centre study, we evaluated consecutive patients with chart documentation of penicillin allergy undergoing cardiac device implantation, over a 2-year period. A CTD was performed if no cephalosporin allergy or severe anaphylactic reaction to penicillin had been documented. Patients were given 2 doses of 100 mg IV cefazolin, and if no allergic reaction occurred after 5 minutes, the full dose (1800 mg) was administered in the electrophysiology laboratory just before the implantation procedure. ResultsA total of 2200 patients were included. The frequency of reported penicillin allergy was 9.3% (n = 204). In 80% of cases, the type of allergic reaction was not reported in medical notes or was unknown by the patient. A CTD was performed in 67.6% of patients with a penicillin allergy (n = 138). A total of 5 adverse events occurred (3.6% of patients [95% confidence interval, 1.1%-6.1%]) — 4 skin rashes and 1 tongue edema. These 5 patients became asymptomatic after antihistaminic and corticosteroid IV treatment. Even if the test dose was negative, 79% of patients also were administered vancomycin before the procedure, as it requires a 1-hour infusion prior to the CTD in the implantation procedure room. ConclusionA CTD in most penicillin-allergic patients appears to be safe and allows its use per recommended guidelines.

Retromolar intubation with video intubating stylet in difficult airway: A randomized crossover manikin study

ObjectiveDifficult airway situations, such as trismus and neck rigidity, may prohibit standard midline orotracheal intubation. An alternative route of intubation from the retromolar space using a fiberoptic scope or rigid intubation stylet has been reported. There is no study investigating the applicability of retromolar intubation using a video intubating stylet. This study comparatively analyzed difficult airway management using a video intubating stylet in the retromolar and standard midline approaches. MethodsA randomized crossover manikin study was conducted between January 2021 and June 2021 at a tertiary teaching hospital. Thirty-six emergency medicine residents and attending physicians were enrolled, and all participated in an educational course regarding video intubating stylet in standard midline and retromolar approaches. Then, they performed both intubation approaches in a randomized order on a manikin seven times with different airway settings each time. The duration of successful intubation, first attempt success rate, overall success rate, number of attempts, and self-reported difficulty were recorded and compared. ResultsThirty-six emergency physicians were included in the study. Compared with the standard midline approach, the use of the retromolar approach significantly reduced the duration of successful intubation in difficult airway scenarios such as limited mouth opening and neck rigidity with (44.77 [28.58–63.65] vs. 120 [93.86–120] s, p < 0.001) and without tongue edema (31.5 [22.57–57.74] vs. 44.72 [36.23–65.34] s, p = 0.012). Furthermore, the retromolar approach increased the first attempt success rate in scenarios of limited mouth opening and neck rigidity with (91.67% vs. 16.67%, p < 0.001) and without (97.22% vs. 72.22%, p = 0.012) tongue edema. The self-reported difficulty was also significantly lower with the retromolar approach than with the standard approach in the above two scenarios. ConclusionsThe retromolar approach for intubation using a video intubating stylet may be a promising choice for selected patients with a combination of difficult airway features such as limited mouth opening, neck rigidity, and edematous tongue.

Awake percutaneous tracheostomy in neurosurgical patients: clinical cases and literature review

Transoral or combined transnasal-transoral approach is sometimes used for tumor resection in patients with skull base and vertebral neoplasms. In such cases, percutaneous tracheostomy before surgical intervention is advisable. Tracheostomy facilitates surgical access, eliminates intraoperative risk of endotracheal tube kinking and provides airway protection from aspiration in early postoperative period in case of bulbar disorders, hypopharynx and tongue edema. The authors present two patients with massive proliferation of pathological tissue in nasopharynx and oropharynx that excluded tracheal intubation before tracheostomy. These patients underwent awake percutaneous tracheostomy.

Anaphylactic shock with pulmonary eosinophilic infiltration due to honeybee attack in a donkey: case report

ABSTRACT A case of a donkey attacked by Africanized honeybee is reported here with clinical signs of agitation, dehydration, congestion of the ocular mucous membranes, tongue edema, tachycardia and inspiratory dyspnea, and progression to death. At necropsy, diffuse, severe subcutaneous edema at face and cervical regions and severe diffuse pulmonary hyperemia with abundant edema without parenchymal collapse were observed. Microscopically, marked, diffuse deep dermis and panniculus carnosus edema and marked diffuse alveolar edema, with moderate population of eosinophils predominantly around larger caliber vessels were noted. The final diagnosis of anaphylactic shock was supported by history, clinical signs, and anatomic pathology findings. This is the first report of a honeybee attack with pulmonary eosinophilic infiltration in a mammal.

RECURRENT ANGIOEDEMA IN SLE PATIENT

TOPIC: Allergy and Airway TYPE: Medical Student/Resident Case Reports INTRODUCTION: The pathophysiology of angioedema is broad. It may be hereditary, idiopathic, or a reaction to a range of medications or allergens; there have been a few case reports describing recurrent angioedema in patients with autoimmune conditions such as systemic lupus erythematosus (SLE). We present the case of a patient with SLE who suffered from recurrent angioedema of the face, including lips, tongue, and throat, without urticaria, with normal C1-Inhibitor levels. CASE PRESENTATION: A 69-year-old female with a history of SLE and recurrent angioedema presented with progressive swelling of eyes, lips, and tongue for one week. She did not consume angiotensin-converting enzyme inhibitors and reported no medication changes or exposure to unknown substances. Physical examination revealed facial, periorbital, and tongue edema with respiratory distress. Initial lab work was unremarkable. Histamine blockers and steroids were immediately administered, and she was intubated due to respiratory compromise. Racemic epinephrine was also initiated, and tracheostomy was performed for definitive airway management. Workup for infection was unrevealing. C1-Inhibitor, CH50, and C4 levels were not deficit. Once her respiratory status was stabilized, she was discharged to a long-term care facility. DISCUSSION: Angioedema, defined as localized subcutaneous or submucosal swelling due to the extravasation of fluid into interstitial tissues, maybe mast cell-mediated or kinin-mediated. The former is often associated with urticaria and is triggered by an allergic reaction or medications. At the same time, the latter occurs in the absence of a specific trigger and is associated with hereditary C1-Inhibitor deficiency or dysfunction. Our patient had normal C1 esterase inhibitor levels, rendering the diagnosis of hereditary angioedema less likely. Furthermore, she reported taking no medications known to cause angioedema and had not been exposed to any new allergens before the presentation. Literature states that patients with SLE characteristically exhibit reduced levels of components of the classical complement pathway. The defect can either originate from a lymphoproliferative disease that catabolizes C1-Inhibitor or circulating antibodies that inactivate C1-Inhibitor. However, our patient did not fulfill the above-mentioned criteria. Even though similar cases have been reported in the literature, the occurrence is still scarce. CONCLUSIONS: SLE has rarely been associated with recurrent angioedema. The pathophysiology of such is unclear. Our patient's late age of presentation and lack of family history of angioedema favored SLE as the independent risk factor for angioedema. Cl-inhibitor dysfunction in SLE is heterogeneous and distinct from that reported in either hereditary or acquired angioedema. Hence, more work needs to be done for early recognition and prompt prevention. REFERENCE #1: Dunckley H, Gatenby PA, Hawkins B, Naito S, Serjeantson SW. Deficiency of C4A is a genetic determinant of systemic lupus erythematosus in three ethnic groups. J Immunogenet. 1987 Aug-Oct;14(4-5):209-18. doi: 10.1111/j.1744-313x.1987.tb00383.x. PMID: 3502648. REFERENCE #2: Alsenz J, Bork K, Loos M. Autoantibody-mediated acquired deficiency of C1 inhibitor. N Engl J Med. 1987 May 28;316(22):1360-6. doi: 10.1056/NEJM198705283162202. PMID: 3494945. REFERENCE #3: Thong BY, Thumboo J, Howe HS, Feng PH. Life-threatening angioedema in systemic lupus erythematosus. Lupus. 2001;10(4):304-308. doi:10.1191/096120301680417011 DISCLOSURES: No relevant relationships by Asim Amjad, source=Web Response No relevant relationships by Zamara Hamid, source=Web Response No relevant relationships by Dan Kazmierski, source=Web Response No relevant relationships by Si Li, source=Web Response No relevant relationships by Pius Ochieng, source=Web Response No relevant relationships by NISHANT SHARMA, source=Web Response