Case Reports in Immunology最新文献

{"title":"IgG4-Related Sclerosing Disease Causing Spinal Cord Compression: The First Reported Case in Literature","authors":"Nooraldin Merza, A. Taha, J. Lung, Anthony W. Benderman, S. Wright","doi":"10.1155/2019/3618510","DOIUrl":"https://doi.org/10.1155/2019/3618510","url":null,"abstract":"Immunoglobulin G4-related disease (IgG4-RD) is known for forming soft tissue mass lesions that may have compressive effects. It is an extremely rare disease that most frequently affects the pancreas causing autoimmune pancreatitis. It can also affect the gallbladder, salivary glands, and lacrimal glands causing respective organ-specific complications. In our report, we describe an IgG4-RD case that affected the spinal cord. A 60-year-old female presented with cervical spinal cord compression caused by IgG4-RD leading to several neurological deficits. Pathological examination of the excisional biopsy of the mass revealed dense lymphoplasmacytic cells infiltration and stromal fibrosis with IgG4 and plasma cells. The patient showed a dramatic response to the administration of systemic steroids with almost resolution of her neurological symptoms. This case highlights the first case in literature for IgG4-RD of the extradural tissue causing spinal compression. Hereby, we also demonstrate the dramatic response of IgG4-RD to the administration of systemic steroids as the patient had no recurrence after 5 years of close follow-up, the longest reported period of follow-up reported in the literature to date.","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":"6 1","pages":""},"PeriodicalIF":1.0,"publicationDate":"2019-06-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82534311","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Severe Myositis, Myocarditis, and Myasthenia Gravis with Elevated Anti-Striated Muscle Antibody following Single Dose of Ipilimumab-Nivolumab Therapy in a Patient with Metastatic Melanoma.","authors":"Mahdieh Fazel,&nbsp;Patrick M Jedlowski","doi":"10.1155/2019/2539493","DOIUrl":"https://doi.org/10.1155/2019/2539493","url":null,"abstract":"<p><p>Immune checkpoint inhibitors targeting programmed cell death protein 1 and cytotoxic T-lymphocyte associated protein 4 have improved survival in patients with metastatic melanoma, especially in combination (i.e., ipilimumab-nivolumab). Postmarketing surveillance has identified rare but at times life-threatening adverse effects associated with these agents in combination and as monotherapy, which include myocarditis, myositis, myasthenia gravis (MG), and hepatotoxicity. Further evaluation of immune checkpoint therapy-induced MG identified the rapid clinical progression, prolonged treatment/supportive therapy course, and higher frequency of myasthenic crisis in these patients versus those with idiopathic MG. More rapid incorporation of aggressive treatment options (i.e., intravenous immunoglobulin, plasmapheresis) may be necessary in these cases. Anti-striational antibodies are often detected in individuals with myasthenia gravis and concurrent myositis and myocarditis. A high-index of suspicion is necessary to assist with rapid treatment initiation as these patients can rapidly deteriorate into respiratory compromise. A case of a 78-year-old woman with metastatic melanoma status after combination therapy with ipilimumab-nivolumab that developed transaminitis, myositis, myocarditis, and myasthenia gravis (with positive anti-striational antibodies) five days after the first cycle, is presented. Despite high dose intravenous methylprednisolone and intravenous immunoglobulin treatment, she ultimately entered hospice care eight days after hospital admission, 36 days after her first cycle.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":" ","pages":"2539493"},"PeriodicalIF":1.0,"publicationDate":"2019-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/2539493","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37320191","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":"Parry-Romberg Syndrome with Uhthoff's Phenomena: A Spectrum of Autoimmune Disease?","authors":"Samuel Asanad","doi":"10.1155/2019/1752456","DOIUrl":"https://doi.org/10.1155/2019/1752456","url":null,"abstract":"<p><p>Parry-Romberg syndrome (PRS) is a rare disorder characterized by unilateral facial atrophy. Currently, the pathogenesis of PRS is poorly understood and no definitive treatment is available. This article reports the case of a 51-year-old woman with progressive hemifacial atrophy following herpes zoster infection, who presented with a concomitant chronic history of heat-induced diplopia. Magnetic resonance imaging showed unilateral cerebral white matter, periventricular, and medial longitudinal fasciculus lesions. The patient's diplopia resolved following treatment with valacyclovir. Infection has been previously considered as potential cause of PRS. However, herpes-induced PRS with ophthalmologic manifestations of Uhthoff's phenomena has not previously been reported. The present case suggests that PRS may possibly have an autoimmune etiology resembling that of multiple sclerosis.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":" ","pages":"1752456"},"PeriodicalIF":1.0,"publicationDate":"2019-04-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/1752456","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37281446","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":"Vancomycin-Induced Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome Masquerading as Elusive Sepsis.","authors":"Sumon Roy,&nbsp;Vinay P Goswamy,&nbsp;Kirolos N Barssoum,&nbsp;Devesh Rai","doi":"10.1155/2019/1625010","DOIUrl":"https://doi.org/10.1155/2019/1625010","url":null,"abstract":"<p><p>We present a unique case of vancomycin-induced drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome masquerading as elusive endocarditis. A 37-year-old female actively using intravenous drugs presented with worsening right upper extremity pain, fever, and chills. Workup revealed methicillin-resistant staphylococcus aureus (MRSA) bacteremia and multiple right-sided septic pulmonary emboli. Echocardiogram was negative for vegetation. Vancomycin was initiated for bacteremia management suspected secondary to right upper extremity abscesses. However, despite resolution of abscesses, fevers persisted, raising suspicion for endocarditis not detected by echocardiogram. On hospital day 25, the patient began showing signs of DRESS syndrome, ultimately manifesting as transaminitis, eosinophilia, and a diffuse, maculopapular rash. Vancomycin was switched to Linezolid and she improved on high dose steroids. The persistent fevers throughout this hospital course were thought to be an elusive endocarditis before DRESS syndrome fully manifested. Although Vancomycin-induced DRESS is uncommon, this case highlights the importance of identifying early signs of significant adverse effects.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":" ","pages":"1625010"},"PeriodicalIF":1.0,"publicationDate":"2019-04-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/1625010","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37242484","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":"Anti-N-Methyl-D-Aspartate Encephalitis as Paraneoplastic Manifestation of Germ-Cells Tumours: A Cases Report and Literature Review.","authors":"Claudia Geraldine Rita,&nbsp;Israel Nieto Gañan,&nbsp;Adriano Jimenez Escrig,&nbsp;Ángela Carrasco Sayalero","doi":"10.1155/2019/4762937","DOIUrl":"https://doi.org/10.1155/2019/4762937","url":null,"abstract":"<p><p>Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is the most common form of autoimmune encephalitis, caused by the interaction between an antibody and its target, located on glutamate receptor type N-methyl-D-aspartate (NMDA) of neuronal surface. There is a wide spectrum of clinical features starting by a viral-like prodrome, followed by symptoms such as psychosis, aggressive behaviour, memory loss, seizures, movement disorders, and autonomic instability. Up to 50% of the affected young female patients have germ-cells tumours as ovarian teratoma, making it essential to establish an early diagnosis through detection of specific antibodies in serum and cerebrospinal fluid (CSF). This retrospective observational study was performed in patients whom positive anti-NMDA receptor antibodies have been tested, associated with clinical manifestations that suggest autoimmune encephalitis and a germ-cell tumour confirmed by pathology. Six patients have tested positive for anti-NMDA receptor antibodies associated with a germ-cell tumour and clinical manifestations of autoimmune encephalitis. Management includes aggressive immunosuppression and surgical removal.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":" ","pages":"4762937"},"PeriodicalIF":1.0,"publicationDate":"2019-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/4762937","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37133939","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":"Castleman Disease in a Patient with Common Variable Immunodeficiency.","authors":"Luisa Ricciardi,&nbsp;Fabiana Furci,&nbsp;Antonio Ieni,&nbsp;Antonio Macrì","doi":"10.1155/2019/5476383","DOIUrl":"https://doi.org/10.1155/2019/5476383","url":null,"abstract":"<p><p>Common variable immunodeficiency (CVID) is a primary immunodeficiency due to a disorder of the adaptive immune system which causes hypogammaglobulinemia and therefore an increased susceptibility to infection; noninfectious, inflammatory conditions including systemic autoimmunity and lymphoproliferative complications are also commonly associated with CVID. Castleman disease (CD) is a systemic disease clinically characterized by diffuse lymphadenopathy, splenomegaly, anemia, and systemic inflammatory symptoms. This makes CD a great mimicker of more common benign and malignant masses in the neck, chest, abdomen, and pelvis. A novel case of primary immunodeficiency (CVID) in a middle-aged woman, who developed multicentric CD (MDC) with splenomegaly, is described. The authors suggest that the onset of MCD and of the correlated splenomegaly was due to incorrect management of the hypogammaglobulinemia as immunoglobulin G (IgG) levels were not kept within normal ranges. Correct management of the hypogammaglobulinemia allowed splenectomy to be performed without any infectious surgical complications. MCD is reported for the first time in association with an adult case of CVID. The above reported case highlights the need for a timely correct diagnosis and treatment of CVID to avoid complications, which could cause recourse to splenectomy, such as in our case or development of malignancies.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":" ","pages":"5476383"},"PeriodicalIF":1.0,"publicationDate":"2019-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/5476383","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37248219","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 Forgotten Cause of Allergy at ER That Is Still Difficult to Diagnose and Treat at Poor Resource Setting: Angioedema after Using Angiotensin Converting Enzyme Inhibitors for 4 Years.","authors":"A A Nilanga Nishad,&nbsp;K Arulmoly,&nbsp;S A S Priyankara,&nbsp;P K Abeysundara","doi":"10.1155/2019/1676391","DOIUrl":"https://doi.org/10.1155/2019/1676391","url":null,"abstract":"<p><p>Angiotensin converting enzyme inhibitors (ACEi) are the most commonly used antihypertensives. Therefore, ACEI induced angioedema (ACEi-AE) is not uncommon. Physicians tend to miss the diagnosis whenever a patient is taking the drug for years due to misbelief of \"a drug that was taken for years may not be the cause for an allergic reaction or an angioedema\". But ACEi can induce angioedema after many years of usage as well as sometimes after stopping the drug even. Most of the emergency physicians and centers are not aware of clinical diagnosis and diagnostic criteria including available diagnostic tests and more importantly the treatment options of ACEi-AE. Therefore not only the diagnosis is delayed or missing but also proper treatment options are not practiced at many emergency rooms and at wards.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":" ","pages":"1676391"},"PeriodicalIF":1.0,"publicationDate":"2019-01-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2019/1676391","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36922723","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 of Eosinophilic Granulomatosis with Polyangiitis Complicated with A IgG4 Related Disease Like Symptoms.","authors":"Suguru Sato,&nbsp;Julia Morimoto,&nbsp;Yasuharu Oguchi,&nbsp;Takashi Umeda,&nbsp;Takaya Kawamata,&nbsp;Mami Rikimaru,&nbsp;Tatsuhiko Koizumi,&nbsp;Ryuichi Togawa,&nbsp;Yasuhito Suzuki,&nbsp;Yuki Sato,&nbsp;Manabu Uematsu,&nbsp;Hiroyuki Minemura,&nbsp;Takefumi Nikaido,&nbsp;Atsuro Fukuhara,&nbsp;Junpei Saito,&nbsp;Kenya Kanazawa,&nbsp;Yoshinori Tanino,&nbsp;Mitsuru Munakata,&nbsp;Yoko Shibata","doi":"10.1155/2018/3763084","DOIUrl":"https://doi.org/10.1155/2018/3763084","url":null,"abstract":"<p><p>We report a case of eosinophilic granulomatosis with polyangiitis (EGPA) complicated with a IgG4 related disease like symptoms presenting as eyelid swellings. In the present case, the serum level of IgG4 and the ratio of IgG4 to IgG were generally increased by the disease course of EGPA. Considering the course of clinical symptoms, there is a possibility that orbital manifestations were one of the clinical features during the disease course of EGPA while the histological features of right eyelid tissue and other ocular manifestations were consistent with the diagnosis of IgG4 related disease.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":"2018 ","pages":"3763084"},"PeriodicalIF":1.0,"publicationDate":"2018-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2018/3763084","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36896927","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":"Hereditary Angioedema Type II: First Presentation in Adulthood with Recurrent Severe Abdominal Pain.","authors":"Mohamed Abuzakouk,&nbsp;Nada AlMahmeed,&nbsp;Esat Memisoglu,&nbsp;Martine McManus,&nbsp;Aydamir Alrakawi","doi":"10.1155/2018/7435870","DOIUrl":"https://doi.org/10.1155/2018/7435870","url":null,"abstract":"<p><p>A 27-year-old Emirate man presented to Cleveland Clinic Abu Dhabi emergency department with a 4 year history of recurrent episodes of severe swellings affecting different parts of his body. He used to get 2 swelling episodes every week affecting either his face, hands, feet or scrotum and severe abdominal pain twice a week. Abdominal CT scan and a colonoscopy showed bowel wall oedema. There was no family history of similar complaint or of hereditary angioedema (HAE). Complement studies confirmed the diagnosis of HAE type II. He was commenced on danazol 100 mg twice daily and his symptoms resolved. This case report highlights the importance of considering HAE in patients with recurrent unexplained abdominal pain even in the absence of positive family history of HAE.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":"2018 ","pages":"7435870"},"PeriodicalIF":1.0,"publicationDate":"2018-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2018/7435870","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36737048","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":"An X-Linked Hyper-IgM Patient Followed Successfully for 23 Years without Hematopoietic Stem Cell Transplantation.","authors":"Necil Kutukculer,&nbsp;Neslihan Edeer Karaca,&nbsp;Guzide Aksu,&nbsp;Ayca Aykut,&nbsp;Erhan Pariltay,&nbsp;Ozgur Cogulu","doi":"10.1155/2018/6897935","DOIUrl":"https://doi.org/10.1155/2018/6897935","url":null,"abstract":"<p><p>When caring for patients with life-limiting diseases, improving survival and optimizing quality of life are the primary goals. For patients with X-linked hyper-IgM syndrome (XHIGM), the treatment modality has to be decided for a particular patient regarding hematopoietic stem cell transplantation or intravenous immunoglobulin replacement therapy with <i>P. jiroveci</i> prophylaxis. A seven-year-old male patient was admitted with recurrent upper and lower respiratory tract infections and recurrent otitis media. His initial immunologic evaluation revealed low IgG and normal IgA and IgM levels with normal lymphocyte phenotyping and inadequate specific antibody responses. He was diagnosed as common variable immunodeficiency and began to receive intravenous immunoglobulin (IVIG) (0.5 gm/kg) with four-week intervals. During follow-up for 23 years under IVIG therapy, he was extremely well and never had severe infections. In 2017, targeted next generation sequencing was performed in order to understand his molecular pathology. A previously described hemizygous c.31C>T(p.Arg11Ter) mutation was found in <i>CD40LG</i> gene. The mother was heterozygous carrier for this mutation and his sister did not have any mutation. Flow cytometric analysis for <i>CD40LG</i> expression on activated T cells showed highly decreased, but not absent, <i>CD40LG</i> expression. In conclusion, diagnostic delay is a clinical problem for patients with <i>CD40LG</i> deficiency, because of low or normal IgM levels, showing that all the hypogammaglobulinemic patients, not only with high serum IgM levels, but also with normal to low IgM levels, have to be examined for <i>CD40LG</i> expression on activated T lymphocytes. Secondly, type of <i>CD40LG</i> mutations leads to enormous interpatient variations regarding serum IgM levels, CD40LG levels on activated T cells, age at diagnosis, severity of clinical findings, and follow-up therapies with or without hematopoietic stem cell therapy.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":"2018 ","pages":"6897935"},"PeriodicalIF":1.0,"publicationDate":"2018-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1155/2018/6897935","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36645919","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}