European journal of case reports in internal medicine最新文献

{"title":"A Rare Case of Hyponatremia Caused by Urinary Retention.","authors":"Oton Mahnič, Patrick Bader","doi":"10.12890/2024_004945","DOIUrl":"https://doi.org/10.12890/2024_004945","url":null,"abstract":"<p><p>An elderly man was hospitalized after a fall following an episode of dizziness. During the initial examination, he was found to have a distended bladder, caused by urinary retention. Moreover, he was diagnosed with severe hypoosmotic hyponatremia. After the urinary retention was resolved, the patient developed severe diuresis, and the hyponatremia corrected rapidly and spontaneously.</p><p><strong>Learning points: </strong>Hyponatremia is a common affliction in the elderly population, with diverse causes, some of which are still not well understood.In cases of hyponatremia, urinary retention should be considered a possible cause.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 11","pages":"004945"},"PeriodicalIF":0.0,"publicationDate":"2024-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11542961/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142616915","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Complex Antiphospholipid Syndrome Successfully Controlled for 17 Years with Personalized Enoxaparin Therapy.","authors":"Mafalda Vasconcelos, Patrícia Moreira, Teresa Costa E Silva, João Espírito Santo, Carla Noronha","doi":"10.12890/2024_004877","DOIUrl":"https://doi.org/10.12890/2024_004877","url":null,"abstract":"<p><p>Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by the presence of antiphospholipid antibodies and venous and arterial thrombotic events, including obstetric complications. We describe the case of a 56-year-old female diagnosed with APS with triple antibody positivity and multiple disease-associated manifestations, namely recurrent purpuric lesions, adrenal insufficiency due to infarction, acalculous cholecystitis, and three spontaneous abortions. Her follow-up was marked by severe thrombotic and haemorrhagic events, notably splanchnic vein thrombosis and haemorrhagic shock after a renal biopsy, as well as the diagnosis of systemic lupus erythematosus 8 years after the APS diagnosis. Chronic anticoagulation with enoxaparin, with dosage guided by anti-factor Xa activity, resulted in stability without complications over 17 years. This case emphasizes the importance of personalized therapeutic strategies and close monitoring in patients with APS.</p><p><strong>Learning points: </strong>This patient presented with rare manifestations of antiphospholipid syndrome (APS), such as acalculous cholecystitis, Budd-Chiari syndrome, and Addison's disease, all linked to ischemic events caused by vascular thrombosis. These atypical presentations highlight the varied and severe clinical spectrum of APS, requiring physicians to be vigilant about unusual complications.After multiple severe thrombotic and haemorrhagic events, the patient was successfully managed with long-term enoxaparin, with dose adjusted according to anti-factor Xa activity (which allowed for a significant dose reduction in comparison with the predicted empiric dose).The patient remained event-free for 17 years, reflecting the importance of personalizing anticoagulation therapy.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 11","pages":"004877"},"PeriodicalIF":0.0,"publicationDate":"2024-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11542954/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142616922","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Unique Case of Hypervirulent <i>Klebsiella Pneumoniae</i> Invasive Syndrome with Endogenous Endophthalmitis and Left Renal Vein Thrombosis without Liver Abscess.","authors":"Ghazal Talal Saeed, Montaser Nabeeh Al Smady, Gunjan Awatramani, Hessa Alqasimi, Mohammed Amaan Khokar, Mohamed Awad, Khadija Hafidh","doi":"10.12890/2024_004927","DOIUrl":"https://doi.org/10.12890/2024_004927","url":null,"abstract":"<p><strong>Introduction: </strong>This case report presents a rare instance of hypervirulent <i>Klebsiella pneumoniae</i> invasive syndrome (KPIS) without hepatic involvement. It highlights an atypical presentation as this syndrome is characterized by liver abscesses.</p><p><strong>Case presentation: </strong>A 54-year-old female of South Asian descent presented to the emergency department with abdominal pain, fever, chills, nausea, and vomiting. Lab investigations showed hyperglycaemia, leukocytosis, and elevated procalcitonin. Imaging revealed bilateral acute pyelonephritis with early abscess formation in the left kidney, bilateral pleural effusions, diffuse multi-lobar consolidations, minimal ascites, and left renal vein thrombosis. The patient was treated as a case of septic shock secondary to hypervirulent <i>K. pneumoniae</i> with complications evolving into acute pyelonephritis and endogenous endophthalmitis. <i>K. pneumoniae</i>, known for its polysaccharide capsule, can lead to severe complications in immunocompromised patients, such as endogenous endophthalmitis, liver abscess, and pyelonephritis. Endophthalmitis, a critical ocular emergency, can result in permanent vision loss. The patient's ocular outcomes remain uncertain due to a lack of follow-up, but prompt interdisciplinary intervention led to the resolution of other comorbidities.</p><p><strong>Conclusion: </strong>This case highlights the importance of considering hypervirulent <i>K. pneumoniae</i> as a cause of severe infection without liver abscess. It also underscores the importance of immediate multidisciplinary management to address the systemic complications associated with this infection and the importance of connecting different symptoms to one aetiology.</p><p><strong>Learning points: </strong><i>Klebsiella pneumoniae</i> invasive syndrome (KPIS) should be considered in patients with positive <i>Klebsiella pneumoniae</i> infection affecting more than one organ system.KPIS may present atypically, such as in the absence of liver abscesses and with the presence of thrombosis.Hypervirulent and hypermucoviscous strains of <i>Klebsiella pneumoniae</i> should be considered in cases of KPIS.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 11","pages":"004927"},"PeriodicalIF":0.0,"publicationDate":"2024-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11542939/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142616919","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Epstein-Barr Virus-Driven T-Cell Lymphoma with Haemophagocytic Lymphohistiocytosis: A Life-Threatening Disorder Extending Beyond Childhood.","authors":"Chalothorn Wannaphut, Landon Kozai, Toshiaki Takahashi, Sharina Macapagal, Yoshito Nishimura","doi":"10.12890/2024_004931","DOIUrl":"https://doi.org/10.12890/2024_004931","url":null,"abstract":"<p><p>An 18-year-old previously healthy Filipino male presented with abdominal pain, vomiting, dyspnoea and fever. Initial investigations revealed severe hepatosplenomegaly, pancytopaenia, elevated liver enzymes, coagulopathy and extremely high ferritin levels. Bone marrow biopsy confirmed an abnormal CD8+ T-cell population with haemophagocytosis. Extensive workup was performed, and he was ultimately diagnosed with haemophagocytic lymphohistiocytosis (HLH) secondary to Epstein-Barr virus-positive T-cell lymphoma of childhood (EBV-TCL), a rare and aggressive malignancy. Despite the initiation of modified dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide (SMILE) chemotherapy along with high-dose glucocorticoids, the patient did not respond to the treatment and expired. This case underscores the critical importance of early recognition and prompt intervention in EBV-TCL-associated HLH which is a unique condition and a rare entity. The diagnosis of this entity can be particularly challenging, given its rapid progression and high mortality rate. Therefore, timely diagnosis and the initiation of appropriate therapy are essential for improving patient outcomes. General medicine providers play a key role in identifying warning signs to avoid delays in treatment initiation.</p><p><strong>Learning points: </strong>EBV-associated T-cell lymphoma with haemophagocytic lymphohistiocytosis can affect not only children but also adolescents and young adults, highlighting the need for awareness of the high fatality risk.Early recognition of EBV-associated haemophagocytic lymphohistiocytosis (EBV-HLH) is crucial; when a patient presents with fever, pancytopaenia and hepatosplenomegaly.Future prospective studies are warranted to determine the treatment strategy and the optimal patient population that requires early bone marrow transplantation when initial treatment is inadequate.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 11","pages":"004931"},"PeriodicalIF":0.0,"publicationDate":"2024-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11542956/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142616926","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Newest \"Co-Members\" of the 1000s Club: A Case of Severe Transaminitis Secondary to Epstein-Barr and Dengue Virus Co-Infection in a Returning Traveler.","authors":"Parsa Tahvildar, Marina Atalla, Rabia Tahir, Andrew Cheung","doi":"10.12890/2024_004748","DOIUrl":"https://doi.org/10.12890/2024_004748","url":null,"abstract":"<p><strong>Background: </strong>Timely identification of the etiology of transaminitis is critical in informing subsequent management as strategies can vary from supportive care to urgent transplant assessment. This is especially important in returning travelers as there may be multiple causes of injury that need to be addressed.</p><p><strong>Case report: </strong>We present a case of severe transaminitis secondary to non-hepatitis viral co-infections. A 28-year-old south Asian male returning traveler presented with an acute liver injury (aspartate aminotransferase/alanine aminotransaminase levels of ≥4000 IU/l) and marked jaundice. A thorough and expanded work-up of acute hepatitis was negative aside from positive mononucleosis spot testing and positive dengue fever serologies. This atypical presentation of mononucleosis and dengue fever was managed conservatively, and the patient was discharged with outpatient follow-up after an eight-day admission.</p><p><strong>Conclusions: </strong>Usually, non-hepatitis viruses typically do not present with severe transaminitis or hyperbilirubinemia. These viruses, such as infectious mononucleosis and dengue fever, may work synergistically to cause an elevated inflammatory response, resulting in severe transaminitis in returning travelers. In the absence of a classic clinical presentation, clinicians should be aware of co-infections in returning travelers and test for them based on a thorough history and physical examination.</p><p><strong>Learning points: </strong>The differential diagnosis for severe transaminitis is narrow and commonly includes viral hepatitis (A-E), drug-induced liver injury, vascular and autoimmune causes; however other causes exist, and greater clinical awareness is needed.This case study demonstrates that even in the absence of a classic clinical presentation; in returning travelers, clinicians should have a low index of suspicion to order appropriate screening serologies based on a thorough history and physical examination as they can be sensitive diagnostic tools in detecting the etiology of severe transaminitis.In rare cases, non-hepatitis virus may act synergistically to cause severe transaminitis and should be considered in returning travelers when viral hepatitis serologies are negative.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 11","pages":"004748"},"PeriodicalIF":0.0,"publicationDate":"2024-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11542953/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142617131","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Fungal Necrotizing Fasciitis Due to Mucormycosis Following Contaminated Substance Inoculation: A Report of Two Cases.","authors":"Orivaldo Alves Barbosa, Eliseu Sousa do Amaral, Gabriel Pinheiro Furtado, Viviane Correa Filomeno da Silva, Moreno de Alencar Isabele, Karina Aguiar de Freitas","doi":"10.12890/2024_004914","DOIUrl":"https://doi.org/10.12890/2024_004914","url":null,"abstract":"<p><p>Mucormycosis is a life-threatening fungal infection caused by Mucorales fungi, characterized by angioinvasion and tissue necrosis. While it predominantly affects immunocompromised individuals, it can also occur in immunocompetent patients through traumatic inoculations, such as contaminated surgical dressings or injections. We report two cases of cutaneous mucormycosis following the shared use of contaminated intramuscular corticosteroid injections (dexamethasone). Case 1 involved a 54-year-old male farmer with rapidly progressing necrotic lesions and multi-organ failure, resulting in death despite aggressive treatment. Case 2 involved a 48-year-old female nursing technician who developed similar necrotizing lesions and also succumbed to multi-organ failure after extensive debridement and antifungal therapy. These cases underscore the rapid deterioration associated with mucormycosis, an infection with a high mortality rate even in immunocompetent individuals. The hallmark of the disease is rapidly progressing necrosis due to vascular invasion and thrombosis. Mucormycosis following contaminated injections, though rare, is devastating. Early recognition and aggressive treatment, including surgical debridement and antifungal therapy, are critical but may not always prevent fatal outcomes. Strict infection prevention precautions and epidemiological surveillance are needed to prevent iatrogenic infections.</p><p><strong>Learning points: </strong>Direct inoculation of Mucorales species through contaminated injections is a rare but serious route of infection, highlighting the importance of proper handling of medical equipment.Mucormycosis can occur in immunocompetent individuals following traumatic events or contaminated procedures, and it often leads to rapid tissue necrosis and high mortality if not promptly treated.Early surgical debridement and antifungal therapy are critical for managing mucormycosis, though survival remains poor even with aggressive intervention.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 11","pages":"004914"},"PeriodicalIF":0.0,"publicationDate":"2024-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11542960/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142616930","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Secondary Amyloidosis Treated with Tocilizumab as a Complication of Temporal Arteritis.","authors":"Maysoun Kudsi, Ghina Haidar, Dani Abouharb, Lana Abouharb","doi":"10.12890/2024_004895","DOIUrl":"https://doi.org/10.12890/2024_004895","url":null,"abstract":"<p><strong>Background: </strong>Temporal arteritis is a large-vessel vasculitis mostly seen in the elderly. Amyloidosis may be secondary to a chronic inflammation of body organs. Here, we present the second case report of temporal arteritis complicated by amyloidosis that was successfully treated by tocilizumab.</p><p><strong>Case presentation: </strong>A 64-year-old female presented complaining of fatigue, fever, and diarrhea accompanied by abdominal pain. One year before presentation, she was diagnosed with temporal arteritis. She was treated with 15 mg/day oral prednisone for the last 6 months, with partial remission, but persistence of the fatigue and an elevated erythrocyte sedimentation rate (ESR, 56 mm/h). Physical examination showed tenderness of both temporal arteries and a soft abdomen. Colon tissue biopsy showed amyloid depositions in the vessels and stroma that were positive for Congo red staining. Tocilizumab was started with 8 mg/kg intravenous, the diarrhea resolved, and the arthralgia improved within 1 month, with a decrease in the ESR to 8 mm/h, and a C-reactive protein (CRP) level of 0.98 mg/dl. Monthly tocilizumab therapy remains efficacious 12 months later and was stopped due to lack of tocilizumab from the hospital. No side effects of tocilizumab were registered.</p><p><strong>Conclusion: </strong>Chronic inflammation may be complicated by amyloidosis in patients with rheumatic diseases and genetic predisposition. Therefore, it is important to screen for intestinal Amyloid A (AA) amyloidosis in individuals with gastrointestinal disorders complicated by rheumatic disorders. AA amyloidosis may be complicated by temporal arteritis and presented with gastrointestinal symptoms such as diarrhea.</p><p><strong>Learning points: </strong>Amyloidosis is manifested by the deposition of insoluble protein aggregates in organs.Amyloid A (AA) amyloidosis occurs as a complication of chronic inflammation in patients with a genetic predisposition to rheumatic diseases.Temporal arteritis complicated with AA amyloidosis is extremely rare.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 11","pages":"004895"},"PeriodicalIF":0.0,"publicationDate":"2024-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11542947/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142617139","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Gynaecomastia Revealing Amiodarone-Induced Hypergonadotropic Hypogonadism: An Overlooked Complication?","authors":"Lynda Vickya Bombil, Corinne Jonas, Etienne Delgrange","doi":"10.12890/2024_004726","DOIUrl":"https://doi.org/10.12890/2024_004726","url":null,"abstract":"<p><p>We report a case of primary hypogonadism induced by amiodarone and review the relevant literature. Amiodarone has a well-established and extensive profile of side effects including thyroid toxicity, corneal deposits and skin discoloration. In rare cases, epididymitis or orchitis may occur. This inflammation can lead to testicular atrophy, inducing primary hypogonadism. We present the case of a 57-year-old patient, treated with amiodarone for several years, initially presenting with testicular pain followed by gynaecomastia, and finally loss of libido indicative of hypergonadotropic hypogonadism. Imaging confirmed bilateral testicular atrophy. Amiodarone was discontinued, and androgen replacement therapy was initiated. This case, combined with data from the literature, highlights the importance of careful monitoring of patients on amiodarone to identify symptoms or clinical signs suggesting testicular dysfunction, to diagnose and treat consequent hypogonadism.</p><p><strong>Learning points: </strong>This case highlights the causal relationship between prolonged amiodarone use and the development of hypogonadism.The underlying mechanism involves the destructive effect of amiodarone on testicular tissue, leading to primary hypogonadism through testicular atrophy.It is crucial to monitor patients for clinical signs of hypogonadism after prescribing amiodarone to ensure timely diagnosis and treatment of any resulting hypogonadism.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 11","pages":"004726"},"PeriodicalIF":0.0,"publicationDate":"2024-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11542941/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142617009","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effects of Combined Respiratory Physiotherapy with High-Flow Nasal Cannula and Venturi Mask in Spinal Cord Injury: A Single-Subject Research Study and Literature Review.","authors":"Loredana Raciti, Gianfranco Raciti, Antonio Ammendolia, Sandro Maria Distefano, Rocco Salvatore Calabrò, Maria Pia Onesta, Gaetano Prosperini","doi":"10.12890/2024_004891","DOIUrl":"https://doi.org/10.12890/2024_004891","url":null,"abstract":"<p><strong>Background: </strong>The level of spinal cord injury affects the severity of respiratory impairment and the alteration of respiratory pattern and gas exchanges. Lesions at the C3-C5 level (phrenic nerve nucleus) cause disruption of descending input with paralysis of the main inspiratory muscle, often requiring tracheostomy and prolonged mechanical ventilation. Oxygen therapy is essential to switch from ventilatory support to removal of the endotracheal tube to correct residual difficulties in oxygenation management.</p><p><strong>Case presentation: </strong>A 58-year-old man had tracheostomy and tetraparesis as complication of tonsillectomy and adenoidectomy treatment for a history of obstructive sleep apnea. A respiratory rehabilitation program with protocol of oxygen therapy with high flow cannula alternated with a low-flow system by Venturi mask during daytime hours only was started. The patient was constantly monitored with capillary partial oxygen saturation to obtain adequate oxygenation (≥ 94%) and registered every 15 minutes in the clinical chart. There was gradual improvement of respiratory function. Oxygen by Venturi mask was gradually reduced due to improvement of partial pressure oxygen values. Over the course of days, the optimal results of respiratory parameters led to a gradual weaning from the Venturi mask until the complete discontinuation of the low-flow system during daytime and decreased of the high-flow fraction of inspired oxygen to the maximal tolerated level during nighttime.</p><p><strong>Conclusions: </strong>Implementing a combined protocol of nighttime oxygen with high flow cannula and daytime Venturi mask improves intensive motor training of patients by promoting the acquisition of ability to perform chair/bed transitions and to be able to achieve standing and begin gait training. More research is needed whether or noted to determine the role of this promising approach in patients with severe SCI and in other critically ill patients.</p><p><strong>Learning points: </strong>This is a presentation of a new respiratory training protocol of combined oxygen therapy by high flow cannula and Venturi mask in a patient with cervical spinal cord injury and respiratory failure.Due to this new respiratory training, from the weaning of ventilation devices the patient had greater independence and improved quality of life, with more intensive motor training in the gym, improvement of the motor program and the patient tolerance to training.Oxygen therapy with high flow cannula and Venturi mask could be valid and reliable combined respiratory training to improve the respiratory pattern in severely hypoxemic and hypercapnic patients, without adverse events.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 11","pages":"004891"},"PeriodicalIF":0.0,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11542948/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142616925","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Intravenous Anakinra for Treating Macrophage Activation Syndrome in Adult-Onset Still's Disease.","authors":"Berivan Bitik, Mustafa Şenturk, Seda Kibaroglu, Tulin Yildirim, Mehmet Engin Tezcan, Pınar Zeyneloglu, Ahmet Eftal Yucel","doi":"10.12890/2024_004788","DOIUrl":"https://doi.org/10.12890/2024_004788","url":null,"abstract":"<p><strong>Background: </strong>Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by fever, rash, arthritis, and multi-organ involvement. Macrophage activation syndrome (MAS), a serious complication of AOSD, poses significant diagnostic and therapeutic challenges.</p><p><strong>Case presentation: </strong>A 32-year-old male was diagnosed with AOSD in 2020 after being hospitalized for a fever of unknown origin and elevated liver enzymes. The patient was initially treated with corticosteroids and methotrexate but subsequently discontinued both treatment and follow-up. In September 2023, he presented with fever, sore throat, and elevated inflammatory markers. After screening for infections, methylprednisolone (MP) treatment was initiated because of AOSD activation. The following day, the patient was admitted to the intensive care unit due to an altered state of consciousness. Brain magnetic resonance imaging revealed brainstem involvement. Empirical treatments were initiated, including intravenous MP, and immunoglobulin therapy. Due to suspected macrophage activation syndrome (MAS), anakinra (ANA) infusion was initiated. Significant improvement was observed after the ANA infusion.</p><p><strong>Conclusion: </strong>This case highlights the complex management of severe AOSD complications, emphasizing the role of early recognition, aggressive therapy, and multidisciplinary care in improving outcomes.</p><p><strong>Learning points: </strong>Macrophage activation syndrome (MAS) is a serious complication of adult-onset Still's disease characterized by systemic inflammation. Early recognition and prompt initiation of treatment are crucial due to the high mortality rate associated with MAS, especially when neurologic symptoms are present.Clinicians should not delay treatment pending confirmatory diagnostic tests when MAS is suspected, as early intervention can significantly impact patient outcomes.Anakinra, an interleukin-1 inhibitor, is typically administered subcutaneously but has shown promise when administered intravenously, particularly in severe cases of MAS.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 11","pages":"004788"},"PeriodicalIF":0.0,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11542943/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142617121","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}