European journal of case reports in internal medicine最新文献

{"title":"Postpartum Hellp Syndrome Associated With Posterior Reversible Encephalopathy Syndrome.","authors":"Rita Pinto Medeiros, Maria Ruão, Pedro Vita, Raquel Monte, Aníbal Marinho","doi":"10.12890/2024_005019","DOIUrl":"10.12890/2024_005019","url":null,"abstract":"<p><p>Haemolysis, elevated liver enzymes and low platelets (HELLP) syndrome is a poorly understood, life-threatening multisystemic condition related to pregnancy with a rapid onset, typically observed in patients with severe pre-eclampsia. Various mechanisms may lead to diffuse endothelial damage associated with HELLP and possible brain involvement. A comprehensive review of PubMed, Embase and Cochrane databases was conducted to examine the clinical, laboratory and radiological features associated with postpartum HELLP syndrome, particularly its potential association with posterior reversible encephalopathy syndrome (PRES). This review was prompted by a rare case we encountered. The literature review identified 10 articles documenting this association during pregnancy and 7 articles reporting it in the postpartum period. Our patient developed multiple organ dysfunction (renal, hepatic, haemolysis and thrombocytopenia) postpartum associated with an arterial hypertensive profile. The patient experienced seizures along with the maintenance of an arterial hypertensive profile resistant to pharmacological therapy. Brain magnetic resonance imaging allowed the diagnosis of PRES and pituitary adenoma/apoplexy. Follow-up led to complete recovery.</p><p><strong>Learning points: </strong>Early identification of posterior reversible encephalopathy syndrome (PRES) is essential after diagnosis of haemolysis, elevated liver enzymes and low platelets (HELLP) syndrome.Early magnetic resonance imaging is required to identify PRES lesions and guide therapy.There needs to be an opportunity to discuss therapeutic options.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 12","pages":"005019"},"PeriodicalIF":0.0,"publicationDate":"2024-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11716313/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142946928","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 Rare Case of Pneumomediastinum, Pneumoretroperitoneum and Subcutaneous Emphysema Due to Crystal Methamphetamine Abuse.","authors":"Abdulmajeed K Abdulmajeed, Mansour O Almehrzi, Saba A Al-Bazzaz, Farah A Khalid, Sherif A Fathi, Ashraf F Hefny","doi":"10.12890/2024_005058","DOIUrl":"10.12890/2024_005058","url":null,"abstract":"<p><strong>Background: </strong>Crystal methamphetamine abuse is a growing concern due to its significant adverse effects on various organ systems.</p><p><strong>Case description: </strong>This report presents a rare case of pneumomediastinum, pneumoretroperitoneum and subcutaneous emphysema resulting from crystal methamphetamine abuse. The exact mechanism linking methamphetamine abuse to pneumomediastinum remains ambiguous. Pneumomediastinum is uncommon with drug abuse, especially if it is smoked and the subject coughs against a closed glottis; however, it is rare to cause such severe presentation.</p><p><strong>Conclusions: </strong>This report discusses the toxic effects of crystal methamphetamine on the respiratory system. It emphasises the importance of increased awareness among clinicians regarding these complications to facilitate timely intervention and prevent life-threatening outcomes.</p><p><strong>Learning points: </strong>Pneumomediastinum, pneumoretroperitoneum and subcutaneous emphysema is a rare complication of crystal methamphetamine (ice) abuse.There is a need to increase awareness among clinicians regarding the potential severe pulmonary complications associated with methamphetamine abuse.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 12","pages":"005058"},"PeriodicalIF":0.0,"publicationDate":"2024-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11716305/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142946795","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 Rare Case of Limited Granulomatosis with Polyangiitis Presenting as Bilateral Parotitis.","authors":"Vandana Bandari, Sandra Apenteng, Aaradhana Kaul","doi":"10.12890/2024_004992","DOIUrl":"10.12890/2024_004992","url":null,"abstract":"<p><strong>Background: </strong>Granulomatosis with polyangiitis (GPA) is a rare autoimmune vasculitis affecting small and medium-sized vessels, commonly involving the respiratory tract and kidneys. Salivary gland involvement, particularly bilateral parotitis, is an uncommon presentation of GPA.</p><p><strong>Case report: </strong>We report the case of a 38-year-old Asian male who presented with left ear pain and parotid swelling after a water park visit. Initially treated with antibiotics for suspected otitis externa, his symptoms worsened, leading to bilateral parotitis, facial palsy, and otomastoiditis. Imaging revealed parotid abscesses and lab results showed elevated antineutrophil cytoplasmic antibodies (ANCA), specifically cytoplasmic ANCA directed against proteinase 3, confirming the diagnosis of GPA. Cultures and autoimmune workups for other causes, such as Sjogren's syndrome and immunoglobulin G4-related disease, were negative. Treatment with high-dose corticosteroids and methotrexate resulted in significant clinical improvement.</p><p><strong>Conclusion: </strong>This case highlights the importance of considering GPA in atypical presentations such as parotitis, even in the absence of renal involvement. Early diagnosis and appropriate immunosuppressive therapy are critical to prevent further complications in multisystem involvement.</p><p><strong>Learning points: </strong>This case highlights the importance of recognizing atypical manifestations of granulomatosis with polyangiitis (GPA), such as bilateral parotitis, which aids in diagnosing and treating rare presentations more effectively.It emphasizes the need to expand differential diagnoses of salivary gland involvement, guiding the distinction of GPA from other similar conditions when antibiotics fail.By highlighting a rare presentation of GPA, this case improves diagnostic understanding in autoimmune diseases, enabling earlier interventions and leading to better patient outcomes.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 12","pages":"004992"},"PeriodicalIF":0.0,"publicationDate":"2024-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11716301/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142946768","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":"Tigecycline-Induced Acute Pancreatitis with Electrocardiogram Changes in Renal Insufficiency Elderly.","authors":"Hong Chin Wee, Ru Shing Ng, Loke Meng Ong","doi":"10.12890/2024_005033","DOIUrl":"10.12890/2024_005033","url":null,"abstract":"<p><strong>Background: </strong>The prevalence of multidrug-resistant and extensively drug-resistant pathogens has led to increased reliance on broad-spectrum antimicrobials, such as tigecycline. This medicine is commonly used to treat complicated skin and intraabdominal infections as well as community-acquired pneumonia. However, the increasing use of tigecycline has been linked to serious complications, including acute pancreatitis.</p><p><strong>Case description: </strong>We present a case of tigecycline-induced acute pancreatitis in a 78-year-old man with complex medical conditions, including type 2 diabetes mellitus, chronic renal insufficiency, and triple vessel disease. The patient was initially treated with tigecycline for bilateral dry gangrene of his toes. Six days after initiation of the tigecycline treatment, the patient developed symptoms of acute pancreatitis with electrocardiogram changes. Magnetic resonance imaging confirmed the diagnosis of acute pancreatitis. Tigecycline was stopped promptly, and supportive treatment was initiated. The patient recovered from the acute pancreatitis and was discharged home. He later passed away at home due to his underlying cardiac disease.</p><p><strong>Conclusions: </strong>This case highlights the potential complications of tigecycline therapy, particularly in patients with significant comorbidities.</p><p><strong>Learning points: </strong>Elderly patients with complex medical issues, such as diabetes mellitus type 2, chronic renal insufficiency, and cardiovascular disease, are at higher risk for adverse drug reactions. Recognizing these risk factors is important for management.Addressing the use of magnetic resonance imaging for diagnosing acute pancreatitis in this specific patient due to their already compromised kidneys highlights the need for a tailored approach. Most commonly, angiographic transformers or computed tomography scan are used for high-risk patients.The symptoms of acute pancreatitis and the presence of cardiac diseases cause problems in managing patients. Changes on the electrocardiogram may suggest cardiac overload; as a result, constant follow-up is necessary for patients suffering from underlying cardiac conditions.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 12","pages":"005033"},"PeriodicalIF":0.0,"publicationDate":"2024-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11716299/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142946946","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":"Atypical Presentation of Andersen-Tawil Syndrome: Heart Failure with Reduced Ejection without Periodic Paralysis or Dysmorphic Features.","authors":"Mustafa Shehzad, Dawood Shehzad, Muhammad Ahmad, Sundus Huma, Shaheer Minhas, Abdul Wassey","doi":"10.12890/2024_005029","DOIUrl":"10.12890/2024_005029","url":null,"abstract":"<p><strong>Background: </strong>Andersen-Tawil syndrome (ATS) is a rare autosomal dominant disorder caused by variants in the <i>KCNJ2</i> gene. It is associated with periodic paralysis, dysmorphic features and cardiac arrhythmias. The syndrome exhibits incomplete penetrance, leading to a broad spectrum of clinical manifestations, making diagnosis challenging.</p><p><strong>Case description: </strong>A male in his mid-20s with class 3 obesity presented to the emergency department with a week-long history of worsening chest pain, orthopnoea and fatigue. His family history was significant for sudden cardiac death affecting both his mother and brother, with his father having died from complications of Wolff-Parkinson-White syndrome. Cardiovascular examination revealed an S3 heart sound and elevated brain natriuretic peptide levels at 875 pg/ml, with undetectable troponins; potassium level on admission was 3.6 mEq/l. An electrocardiogram showed normal sinus rhythm, first-degree heart block, left atrial enlargement, left bundle branch block and a prolonged QTc of 486 ms. A transthoracic echocardiogram demonstrated a 15-20% reduction in ejection fraction with global left ventricular hypokinesis and left atrial enlargement. Ultimately, he was diagnosed with non-ischaemic dilated cardiomyopathy and referred for genetic testing, which revealed a <i>KCNJ2</i> variant. Shortly after discharge, he experienced a 55-second run of ventricular tachycardia, necessitating the placement of a single-chamber ICD. Currently, the patient remains on guideline-directed medical therapy and is listed for a heart transplant.</p><p><strong>Conclusions: </strong>Dilated cardiomyopathy, although a rare manifestation in ATS, can profoundly increase the risk of fatal arrythmias, necessitating the need for a low threshold of suspicion, to ensure timely diagnosis and management.</p><p><strong>Learning points: </strong>Andersen-Tawil syndrome (ATS) can present with heart failure with reduced ejection fraction, even without the typical triad of periodic paralysis, dysmorphic features and arrhythmias. Early genetic testing is essential for confirming ATS, especially in atypical cases with unexplained heart failure or arrhythmias.ATS has incomplete penetrance so it may present without the typical features, making diagnosis challenging and requiring a high suspicion in cases with unexplained arrhythmias or family history of sudden cardiac death.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 12","pages":"005029"},"PeriodicalIF":0.0,"publicationDate":"2024-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11716310/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142946802","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":"Macrophage Activation Syndrome in a Patient with Systemic Lupus Erythematous Triggered by Tuberculous Meningitis.","authors":"Noura Alowais, Noura Zamani, Faisal Elbadawi, Hassan Ahmed, Pankhi Dutta, Aesha Sharif","doi":"10.12890/2024_005026","DOIUrl":"10.12890/2024_005026","url":null,"abstract":"<p><strong>Background: </strong>Hemophagocytic lymphohistiocytosis (HLH), is characterized by systemic uncontrolled inflammation resulting from immune dysregulation secondary to various triggers, including genetics, infections, autoimmune diseases, and malignancies. Macrophage activation syndrome (MAS) is an immune dysregulation phenomenon, in which an underlying rheumatological disease is present. We report a rare, interesting case of a middle-aged female, with a systemic lupus erythematosus (SLE) flare complicated by macrophage activation syndrome (MAS), in which tuberculous meningitis (TBM) was the identified trigger.</p><p><strong>Case report: </strong>A 48-year-old female with an African ethnic background, diagnosed with SLE, presented with a 2-week history of high-grade fever, cough, and worsening arthralgia. She was initially admitted with suspected SLE flare secondary to community acquired pneumonia and was started on broad-spectrum antibiotics. Later during her hospital stay, she underwent extensive workup because of her clinical condition, as she had continuous spikes of fever, progressive cytopenia, accompanied with acute confusion state despite treatment. She was ultimately diagnosed with SLE flare complicated by MAS secondary to TBM in view of persistent fever, neuropsychiatric symptoms, hyperferritinemia and hypertriglyceridemia, supported by bone marrow aspiration findings, a tuberculosis (TB) positive contact and supporting cerebrospinal fluid analysis suggestive of TBM. She was immediately started on pulse doses of steroids and anti-tuberculosis medications in which significant clinical improvement was observed. She was discharged home, with continuity of care done at the outpatient department in addition to continuity of physical rehabilitation.</p><p><strong>Conclusion: </strong>Given the wide range of clinical presentations and similarities between SLE and the life-threatening MAS, it is essential to maintain a high level of suspicion to establish the diagnosis and promptly start the necessary treatment to ensure better clinical and survival outcomes.</p><p><strong>Learning points: </strong>The wide range of clinical presentation of hemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS) makes it a challenging diagnosis. Hence, a high index of suspicion is necessary to establish the diagnosis and promptly start the necessary treatment.The mainstay of treatment is the early identification and management of the underlying etiology of MAS along with high-dose glucocorticoids and other immunosuppressive therapy targeting the immune dysregulation pathway.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 12","pages":"005026"},"PeriodicalIF":0.0,"publicationDate":"2024-12-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11716302/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142946831","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":"Budd-Chiari Syndrome as an Initial Manifestation of Incomplete Systemic Lupus Erythematosus.","authors":"Cecilia Porcu, Nathalie Merkel, Tanja Fusi-Schimdhauser","doi":"10.12890/2024_005015","DOIUrl":"10.12890/2024_005015","url":null,"abstract":"<p><p>This article describes a case of a 26-year-old female with a history of Evan's syndrome who presented with severe exertional dyspnoea and abdominal discomfort. The patient was diagnosed with chronic Budd-Chiari syndrome, a rare vascular disorder characterized by obstruction of the hepatic vein. We discuss the risk factors, the clinical manifestations, and diagnostic methods for Budd-Chiari syndrome, as well as the possible association with an underlying incomplete systemic lupus erythematosus. The importance of close follow-up and timely diagnosis for preventing disease progression and reducing mortality is emphasized. The article concludes by highlighting the need for further monitoring to identify any symptoms or signs suggesting a progression to complete lupus erythematosus.</p><p><strong>Learning points: </strong>Budd-Chiari syndrome can present as the initial manifestation of a broader autoimmune disorder such as incomplete systemic lupus erythematosus.Evan's syndrome, when associated with other thrombotic conditions like antiphospholipid syndrome, may indicate an underlying, evolving autoimmune process.Early recognition and management of incomplete lupus are crucial to prevent progression to a full-blown systemic lupus erythematosus and associated complications.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 12","pages":"005015"},"PeriodicalIF":0.0,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11716300/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142946771","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":"Can Severe Hypertriglyceridemia Cause Angina in the Absence of Significant Coronary Artery Lesions?","authors":"Mustafa Zain, Ahmad Rasheed Alsaadi","doi":"10.12890/2024_005043","DOIUrl":"10.12890/2024_005043","url":null,"abstract":"<p><p>Hyperlipidaemia is considered one of the most common risk factors for ischemic heart disease. Severely elevated triglyceride levels may lead to hyperviscosity which may contribute to endothelial dysfunction, tissue ischemia and chylomicronaemia. This case report features a 39-year-old, non-smoker male with a past medical history of hypertension and no family history of coronary artery disease, who presented to the clinic with angina. The electrocardiogram and echocardiogram were normal. Laboratory tests were normal except for severely elevated triglyceride levels. A dobutamine stress echocardiogram was positive, so the patient underwent coronary angiography which revealed normal coronary arteries with slow flow in all of the coronary arteries (TIMI 2 flow grade). The patient was then discharged on high-intensity statin, fenofibrate, isosorbide mononitrate, nebivolol, and was given lifestyle modification instructions. After 3 months the patient came back to the clinic for follow-up. He was in good general condition and symptom free. Laboratory tests (including lipid profile) were within normal limits.</p><p><strong>Learning points: </strong>Understanding the effects of severely elevated triglyceride levels on coronary endothelial function.Prevention of harmful effects resulting from severely elevated triglyceride levels.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 12","pages":"005043"},"PeriodicalIF":0.0,"publicationDate":"2024-11-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11623367/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142799813","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":"Amyopathic Dermatomyositis with a Rapidly Progressing Interstitial Pneumonia.","authors":"Darija Logvinova, Dace Žentiņa, Kristīne Ivanova, Inita Buliņa, Zaiga Kravale","doi":"10.12890/2024_005036","DOIUrl":"10.12890/2024_005036","url":null,"abstract":"<p><strong>Background: </strong>Clinically amyopathic dermatomyositis (CADM) is a rare subtype of idiopathic inflammatory myositis often linked with the presence of autoantibodies targeting melanoma differentiation-associated protein 5 (MDA5). Patients with CADM are at increased risk of developing rapidly progressing interstitial lung disease, which significantly increases both morbidity and mortality compared to other forms of inflammatory myopathies. While there is no standardized treatment regimen, current therapeutic strategies are generally focused on combination immunosuppressive therapies. Despite early diagnosis and immunosuppressive therapy, the disease remains highly aggressive and is associated with a poor prognosis.</p><p><strong>Case report: </strong>This report describes the case of a 63-year-old previously healthy male who developed acute interstitial pneumonia. Polymerase chain reaction testing for pneumonia pathogens and routine autoimmune antibody screening were both negative. Despite treatment with corticosteroids and broad-spectrum antibiotics, the patient's condition continued to deteriorate. A multidisciplinary team was assembled, and a myositis antibody panel was ordered, which led to the diagnosis of anti-MDA5 associated clinically amyopathic dermatomyositis. The patient was initiated on treatment with cyclophosphamide, intravenous immunoglobulin, and a calcineurin inhibitor. However, his condition remained critical, and he ultimately succumbed to respiratory failure.</p><p><strong>Conclusion: </strong>In all cases of rapidly progressive interstitial pneumonia of unclear aetiology, anti-MDA5-associated interstitial lung disease should be considered, regardless of the presence or absence of extrapulmonary manifestations. Despite early recognition and aggressive immunosuppressive therapy, patients with anti-MDA5-associated rapidly progressive interstitial lung disease face a mortality risk of up to 80%. A multidisciplinary approach, with collaboration between specialized centres, is crucial for early diagnosis and timely initiation of treatment.</p><p><strong>Learning points: </strong>Anti-melanoma differentiation-associated protein 5 (anti-MDA5) associated clinically amyopathic dermatomyositis (CADM) is an extremely rare disease associated with significantly higher morbidity and mortality compared to other inflammatory myopathies.This report describes a unique case of a patient who presented with an acute interstitial pneumonia and rapidly progressing respiratory failure due to an undiagnosed anti-MDA5 amyopathic dermatomyositis, without any of the typical dermatomyositis symptoms or physical exam findings.Diagnosis of anti-MDA5 amyopathic dermatomyositis is challenging and standardized treatments for this disease have not been fully developed, which highlights the importance of multidisciplinary approach and collaboration between medical centres.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 12","pages":"005036"},"PeriodicalIF":0.0,"publicationDate":"2024-11-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11716308/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142946797","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 Case Report of Mesenteric Panniculitis and Fibrous Dysplasia with Primary Metabolic Syndrome.","authors":"Birgit Khandalavala, Ryder Cuppett","doi":"10.12890/2024_004922","DOIUrl":"10.12890/2024_004922","url":null,"abstract":"<p><strong>Introduction: </strong>Mesenteric panniculitis (MP) is a rare clinically diagnosed disease resulting from fibro-inflammation in the intestinal adipose tissue. Precedent intra-abdominal inflammation or trauma is often evident. Fibrous dysplasia (FD) is a benign uncommon childhood disease of the bones, with fibrous connective tissue replacing bone tissue. FD can cause pain, disfigurement, and entrapment of critical neighbouring structures. Adult-onset craniofacial involvement is rare. Metabolic syndrome is characterized by obesity and chronic subclinical inflammation, promoting abnormal cellular fibrosis and proliferation. The concomitant incidence of MP and FD in an older female adult with metabolic syndrome is unprecedented. An aberrant metabolic contributory pathophysiology for both MP and FD could be postulated.</p><p><strong>Case report: </strong>A 60-year-old Caucasian woman with primary long-standing well-treated metabolic syndrome with obesity presented for revision bariatric surgery. She had previous intra-abdominal surgeries and occasional intermittent abdominal pain. The surgery was abandoned due to extensively inflamed intestinal mesentery. Radiological studies and intra-abdominal biopsy confirmed MP. She subsequently developed pronounced unilateral facial swelling and radiological studies confirmed FD. Treatment has been suboptimal for both MP and FD. She developed venous thromboembolism on tamoxifen treatment for MP. The patient currently reports abdominal pain and swelling from MP, and facial disfigurement, pain, and tinnitus from FD. Blood markers for inflammation have remained consistently high, and there is statin intolerance. Most recently liver steatosis and diabetes have developed.</p><p><strong>Conclusion: </strong>A case of MP and FD with primary metabolic syndrome is unique. An increased awareness of such rare cases could lead to further research, and critically needed treatments to optimize outcomes.</p><p><strong>Learning points: </strong>Mesenteric panniculitis (MP) is a rare disease as is craniofacial fibrous dysplasia (FD) in adults. The incidence of these two rare diseases in an older adult with primary metabolic syndrome is previously unknown, making this case the first of its kind.Metabolic syndrome is common condition of the industrialized population, and an aberrant common \"systemic\" metabolic pathophysiology could contribute significantly to the inflammatory and fibrotic cellular changes inherent to both these two localized diseases of MP and FD.Knowledge about rare diseases, particularly if clustered with a primary systemic disease, could lead to better treatment outcomes in the future and promote novel therapeutic targets.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 12","pages":"004922"},"PeriodicalIF":0.0,"publicationDate":"2024-11-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11623366/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142799527","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}