Case Reports in Immunology最新文献

{"title":"Interleukin-6 Inhibitor-Induced Leukocytoclastic Vasculitis: A Case Report With a Literature Review.","authors":"Tatevik Aloyan, Dinara Salimova, Ebrahim Mohamed, Hina Wazir","doi":"10.1155/crii/8863357","DOIUrl":"10.1155/crii/8863357","url":null,"abstract":"<p><strong>Background: </strong>Leukocytoclastic vasculitis (LCV) is a known hypersensitivity reaction to biologic agents, often linked to tumor necrosis factor-α (TNF-α)inhibitors. We present a rare case of LCV in a patient receiving tocilizumab, a monoclonal antibody directed against interleukin-6 (IL-6)receptor.</p><p><strong>Case presentation: </strong>A 33-year-old Asian female with seropositive rheumatoid arthritis presented to the rheumatology clinic complaining of a new rash that started a few days after an infusion of tocilizumab. Her rheumatoid arthritis had been managed with upadacitinib for several years, which was discontinued due to persistent transaminitis. She was started on tocilizumab after a 4-month break from biologics. Following the first tocilizumab infusion, she recalled having transient fatigue and several red dots on her forearms and feet. A few days after the second infusion, she developed a purpuric rash on her lower extremities and forearms. Skin biopsy confirmed LCV. The rash resolved slowly in a month after discontinuation of tocilizumab and prescription of prednisone 20 mg daily. At her 3-month follow-up, the patient remained in remission, and her rheumatoid arthritis was uneventfully managed with abatacept.</p><p><strong>Conclusions: </strong>While most cases of biologic-associated LCV are induced by TNF-α inhibitors, only two known cases of tocilizumab-induced hypersensitivity vasculitis have been published in the literature. Our case represents only the third reported instance in the literature, highlighting the need to raise awareness of tocilizumab as a potential cause of leukocytoclastic vasculitis and the importance of prompt recognition and management.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":"2025 ","pages":"8863357"},"PeriodicalIF":1.5,"publicationDate":"2025-11-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12665487/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145662306","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":"Granulomatosis With Polyangiitis (GPA) Presenting With Painless Scleritis and Ocular Hypertension: Case Report.","authors":"Khaled A Elubous, Hady Saheb, Karin M Oliver","doi":"10.1155/crii/6631904","DOIUrl":"https://doi.org/10.1155/crii/6631904","url":null,"abstract":"<p><p>This case report describes a 67-year-old male who presented with a 2-month history of painless left eye redness and muffled hearing. Ophthalmologic examination revealed elevated intraocular pressure (IOP) and significant conjunctival injection without associated pain or visual disturbance. Blood was observed in Schlemm's canal (SC), and a thorough investigation for elevated episcleral venous pressure (EVP) was performed. Imaging, including magnetic resonance imaging (MRI) and cerebral angiogram, was unremarkable. A rheumatologic workup led to the diagnosis of granulomatosis with polyangiitis (GPA). The patient's ocular symptoms improved significantly with systemic steroid treatment. He was subsequently managed with rituximab and avacopan as per standard GPA therapy. This case highlights the importance of considering vasculitis in patients presenting with unexplained elevation of EVP, and painless scleritis, particularly when there are also extraocular complaints.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":"2025 ","pages":"6631904"},"PeriodicalIF":1.5,"publicationDate":"2025-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12646726/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145640661","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":"Hemolytic Transfusion Reaction Due to Anti-A₁ Antibody During Pregnancy: Case Report.","authors":"Suhalika Sahni, Katharine Sweeney, Franklin Njoku, David Allison","doi":"10.1155/crii/7705026","DOIUrl":"10.1155/crii/7705026","url":null,"abstract":"<p><p>The ABO blood group is the most clinically relevant system in transfusion medicine. Approximately 20% of individuals with blood group A of European descent belong to a weak A subgroup, most commonly A₂, which may produce anti-A₁ antibodies. These antibodies are usually cold-reactive IgM and rarely cause hemolysis, but can occasionally be clinically significant when reactive at 37°C. We describe a pregnant woman with sickle cell disease (HbS/β⁰ thalassemia) and prior hyperhemolysis syndrome who developed a severe delayed hemolytic transfusion reaction (DHTR) after transfusion of A₁ red blood cells (RBCs). Anti-A₁ was identified posttransfusion, confirming her as a non-A₁ subtype. Notably, she also experienced hemolysis following group O red cell transfusion, consistent with hyperhemolysis. This case highlights the rare but serious potential of anti-A₁ to cause DHTR, particularly in high-risk populations, and underscores the importance of increased vigilance when managing transfusion in sickle cell disease.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":"2025 ","pages":"7705026"},"PeriodicalIF":1.5,"publicationDate":"2025-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12615022/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145542534","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":"IgG4-Related Disease Inflamatory Pseudotumor Affecting the Sternoclavicular Joint: A Clinical Challenge.","authors":"Orivaldo Alves Barbosa, Talita Guimarães Andrade, Sergio Saldanha Freire Simões, Andre Luis Coutinho de Araújo Macedo, Dower Frota Barroso, João Paulo Uchoa Fontenele, José Walter Correia","doi":"10.1155/crii/6543528","DOIUrl":"10.1155/crii/6543528","url":null,"abstract":"<p><p>IgG4-related disease (IgG4-RD) is a rare, progressive, and immune-mediated fibroinflammatory disorder that primarily affects middle-aged men and is more prevalent in Asian populations. Although extensively studied, its pathophysiology remains incompletely understood. This case report describes a 44-year-old male presenting with multiple abscesses and progressive inflammatory symptoms, ultimately diagnosed with IgG4-RD with musculoskeletal involvement. Imaging and histopathological evaluation confirmed osteolytic lesions and significant IgG4-positive plasma cell infiltration. Soft tissue tumors in IgG4-RD are exceptionally rare, further emphasizing the uniqueness of this case. The patient showed clinical improvement with corticosteroid therapy. This case highlights the importance of considering IgG4-RD in the differential diagnosis of soft tissue and bone lesions and underscores the need for a multidisciplinary diagnostic approach.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":"2025 ","pages":"6543528"},"PeriodicalIF":1.5,"publicationDate":"2025-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12411044/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145016432","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":"Successfully Overcoming Allergy to Anakinra Through Intravenous Desensitization in a Child \"Case Report\".","authors":"Tariq Al Farsi","doi":"10.1155/crii/6246850","DOIUrl":"10.1155/crii/6246850","url":null,"abstract":"<p><p>Anakinra, a recombinant interleukin-1 (IL-1) receptor antagonist, is effective in treating autoinflammatory conditions in children; however, it may elicit a hypersensitive reaction, thus requiring desensitization. We report a case wherein a novel intravenous Anakinra desensitization protocol was applied for a child with hypersensitivity and needle phobia. A 10-year-old girl with tumor necrosis factor receptor-associated periodic syndrome (TRAPS) and Anakinra hypersensitivity underwent a 12-step intravenous desensitization protocol at the Royal Hospital, Oman. Intravenous desensitization was successful without adverse events. Subsequent subcutaneous Anakinra administration was well tolerated without further hypersensitivity or inflammatory episodes. This case demonstrates successful intravenous desensitization against Anakinra in a child with TRAPS and needle phobia, thus enabling continued treatment. This approach may benefit others facing similar challenges and warrants further research.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":"2025 ","pages":"6246850"},"PeriodicalIF":1.5,"publicationDate":"2025-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12390699/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144972973","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":"Diagnostic Dilemma: Adult Onset Still's Disease Mimicking Lymphoma-A Case Report and Literature Review.","authors":"Mohannad N AbuHaweeleh, Al-Jouhara Albaloshi, Mohammed Al-Hor, Majed Al-Theyab, Ahmad Almaslamani, Basant Elsayed, Moaz O Moursi, Abdelrahman Hamad","doi":"10.1155/crii/5533371","DOIUrl":"10.1155/crii/5533371","url":null,"abstract":"<p><p><b>Introduction:</b> Adult-onset Still's disease (AOSD) is a rare systemic inflammatory condition with hallmark features of spiking fevers, arthritis, and a salmon-colored maculopapular rash. It typically affects young adults, with a bimodal age distribution of 15-25 and 36-46 years. The prevalence of AOSD ranges from 1 to 34 cases per million people, with an incidence rate of 0.16-0.4 per 100,000 individuals. AOSD's etiology remains unclear but is thought to involve genetic and environmental factors. Diagnosis relies on clinical criteria, such as the Yamaguchi criteria, and exclusion of other conditions. Misdiagnosis is common, particularly in regions where infections and malignancies with overlapping features are prevalent. This case report highlights a diagnostically challenging case of AOSD in a young woman, emphasizing the importance of thorough evaluation and accurate diagnosis. <b>Case Presentation:</b> A 21-year-old female with no prior comorbidities presented with persistent fever, a transient salmon-colored rash, and polyarthritis involving the wrists, shoulders, ankles, and small joints. Three months prior, she had been treated for left submandibular sialadenitis attributed to mumps. Laboratory work revealed elevated C-reactive protein (CRP), ferritin, and LDH levels with a normal erythrocyte sedimentation rate (ESR) and autoimmune profile. Imaging studies, including PET-CT, suggested malignancy, raising suspicion of lymphoma. However, lymph node biopsy showed reactive hyperplasia without evidence of malignancy. Ultimately, a diagnosis of AOSD was established based on clinical and laboratory findings. The patient was initiated on anakinra and prednisolone, leading to significant improvement. At a 2-month follow-up, she had complete resolution of symptoms. <b>Conclusion:</b> AOSD presents a significant diagnostic challenge due to its rarity and symptom overlap with infections, malignancies, and autoimmune diseases. This case highlights the importance of a thorough clinical evaluation and the application of established diagnostic criteria to facilitate early diagnosis and timely management.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":"2025 ","pages":"5533371"},"PeriodicalIF":1.5,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12356662/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144875809","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":"Major Histocompatibility Complex Class II Deficiency Beyond Infancy.","authors":"Aziza Bachir Kattra, Ibtihal Benhsaien, Asmaa Drissi Bourhanbour, Zahra Aadam, Abderrahmane Errami, Fatima Ailal, Ahmed Aziz Bousfiha, Jalila El Bakkouri","doi":"10.1155/crii/8570051","DOIUrl":"10.1155/crii/8570051","url":null,"abstract":"<p><p>Major histocompatibility complex class (MHC)-II deficiency is a rare autosomal recessive combined immunodeficiency, accounting for 4.1% of inborn errors of immunity (IEI) cases in North Africa and the Middle East. Most patients do not survive beyond the age of 10 years. The cases described in this study are rare and unusual for MHC-II deficiency. We report the cases of four unrelated patients of Moroccan origin with MHC-II deficiency. Immunophenotyping of lymphocyte subpopulations and analysis of human leukocyte antigen-DR (HLA-DR) expression were performed using flow cytometry. Genetic analysis was conducted through direct sequencing. The mean age of our patients was 18.75 years (range 16-26 years); the mean age at diagnosis was 14.07 years, and the mean age of onset of symptoms was 5.25 months. The clinical presentation is characterized by recurrent pulmonary infections with predominant bronchial dilatation and hemorrhagic rectocolitis. The diagnosis was confirmed in all patients by absence of HLA-DR expression and detection of the c.338-25_338del mutation in <i>RFXANK</i>. Three (75%) of our patients are still alive and are on monthly intravenous immunoglobulin (IVIG) therapy. It is important to consider MHC-II deficiency in the differential diagnosis of combined immunodeficiencies across all age groups. Further studies are needed to elucidate the various phenotypes associated with this condition.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":"2025 ","pages":"8570051"},"PeriodicalIF":1.5,"publicationDate":"2025-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12318625/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144776408","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":"Coexistence of a Leaky SCID Phenotype With Hyperphenylalaninemia in an Adult Case.","authors":"Ugur Musabak, Tuba Erdogan, Muserref Sule Akcay, Serdar Ceylaner","doi":"10.1155/crii/9988821","DOIUrl":"10.1155/crii/9988821","url":null,"abstract":"<p><p>In recent years, due to the widespread use of advanced molecular diagnostic methods, it has become clear that individuals in particular born from consanguineous marriages may be carriers of different genetic diseases. For this reason, cases where diseases related to inborn errors of immunity (IEI) and metabolism errors are detected in the same patient are encountered more frequently. In patients affected by different genetic defects, the pathophysiology is more complex, and disease management is more difficult. In this article, we aimed to draw attention to this complex genetic carrier state in a male with primary immunodeficiency (PID). In the patient who presented with recurrent lower respiratory tract infections, bronchiectasis, asthma and nasal polyps, and antibody deficiencies as well as cellular immunodeficiency findings were detected in the immunological analyses. In the whole exome sequencing (WES) study, three different variants were detected, two in genes related to PIDs (DCLRE1C and TNFRSF13B) and one in the gene related to phenylalanine metabolism (phenylalanine hydroxylase (PAH)). In the light of the current findings, the patient was evaluated as having leaky severe combined immunodeficiency (SCID) with immune phenotype T-B-natural killer (NK)+ and hyperphenylalaninemia (HPA). This case showed us that metabolic diseases may accompany a delay in the diagnosis of SCID and patients should be evaluated with a multidisciplinary approach.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":"2025 ","pages":"9988821"},"PeriodicalIF":0.7,"publicationDate":"2025-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11944794/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143732214","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":"Hemizygous Moesin (MSN) Gene Deletion in an Adult With Chronic Neutropenia.","authors":"Yandy Marx Castillo-Aleman, Francisco Sotomayor-Lugo, Sherjeel Sana, Nameer Abdul Raheem Kadhum Al-Saadawi, Yendry Ventura-Carmenate, David Dennison, Gianina Statache, Julieta Osorio-Zuluaga, Ahmad Raza, David Grossman","doi":"10.1155/crii/3860726","DOIUrl":"https://doi.org/10.1155/crii/3860726","url":null,"abstract":"<p><p>X-linked moesin-associated immunodeficiency (X-MAID) is a recently identified combined immunodeficiency caused by a mutation in the moesin (<i>MSN</i>) gene. It is characterized by cytopenias, hypogammaglobulinemia, poor immune response to vaccine antigens, and increased susceptibility to early-life infections. We report a patient with adult-onset neutropenia, lymphopenia, inadequate response to the pneumococcal polysaccharide vaccine (PPSV23), and recurrent bacterial infections associated with a hemizygous <i>MSN</i> deletion. Notably, the patient has no history of significant childhood infections, cytopenias, or hypogammaglobulinemia. Although only a few cases have been documented worldwide, we underscore the importance of whole-genome sequencing (WES) in diagnosing this atypical immunodeficiency disease in adulthood. Moreover, this report may shed light on our understanding of further variants of X-MAID and enrich the known spectrum of the disease.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":"2024 ","pages":"3860726"},"PeriodicalIF":0.7,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11707056/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142956495","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":"Allergic Contact Dermatitis Reaction to Permanent Tattoo Containing Paraphenylenediamine: A Case Report.","authors":"Eliot Parascandolo, Samuel Puglisi, Miguel Marenco, Gregory Puglisi","doi":"10.1155/2024/2118096","DOIUrl":"10.1155/2024/2118096","url":null,"abstract":"<p><p>Paraphenylenediamine (PPD) is a well-known culprit allergen in the literature and clinical practice. Although this has been described in temporary tattoos, the definite implication of PPD in permanent tattoos has not been described. We report a patient who developed severe allergic contact dermatitis (ACD) requiring skin grafting after receiving a permanent tattoo with ink containing PPD. A 30-year-old female with a past history of atopic dermatitis and psoriasis presented with a 2-week history of cutaneous reaction to a recent tattoo. The patient noticed inflammation and irritation of the tattoo site the day after administration. The patient was previously identified on patch testing to have a PPD allergy after evaluation for dermatitis after hair dye application. Following the tattoo placement, she applied soap and bacitracin cream which she had used several years prior on a similar tattoo. On presentation 2 weeks later, she was found to have a deep ulcerated plaque with an indurated border encompassing the area of the tattoo. She was referred to the emergency department and admitted for treatment, ultimately requiring debridement and skin grafting. The patient obtained the safety data sheets for the tattoo inks which revealed PPD as an ingredient in every color. We believe this is the first confirmed case of PPD being implicated as the causative agent for ACD to a permanent tattoo. Tattoo ink is unregulated, and formulas are proprietary which makes safe practice difficult for patients with sensitivities. We advocate for consistent ingredient labeling, regulation, and transparency within the tattoo ink industry.</p>","PeriodicalId":42865,"journal":{"name":"Case Reports in Immunology","volume":"2024 ","pages":"2118096"},"PeriodicalIF":0.7,"publicationDate":"2024-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11446613/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142366871","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}