Atypical presentation of anti-glomerular basement membrane disease. Pediatric Case

Atypical presentation of anti-glomerular basement membrane disease. Pediatric Case | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Article Atypical presentation of anti-glomerular basement membrane disease. Pediatric Case Veronica Alejandra Bravo Díaz, Xavier Miranda, Ana Lucia Saez, and 1 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-7264487/v1 This work is licensed under a CC BY 4.0 License Status: Under Revision Version 1 posted 5 You are reading this latest preprint version Abstract We describe the clinical case of a 13-year-old girl diagnosed with anti-glomerular basement membrane disease and central nervous system involvement. The patient presented with a generalized tonic‒clonic seizure as the first clinical finding secondary to an intraparenchymal hemorrhage and cerebral vasculitis. Acute renal injury was also detected without the need for renal replacement therapy, hematuria, arterial hypertension, significant proteinuria, and pulmonary hemorrhage with diffuse ground‒glass alveolar infiltrate. The diagnosis was established through renal biopsy, which revealed a pattern of extracapillary glomerulonephritis with linear deposition of IgG and the presence of anti-GBM antibodies in the plasma. In our case, the patient was treated with pulses of corticosteroids, cyclophosphamide, and plasmapheresis. She evolved with normalization of renal function, albuminuria, and no neurological sequelae on imaging. Anti-glomerular basement membrane disease is a vasculitis of small vessels that affects the kidneys (90%) and lungs (60%) and is the cause of 1–2% of acute glomerulonephritis and 10–15% of rapidly progressive glomerulonephritis. Cerebral vasculitis rapidly progressive glomerulonephritis (RPGN) pulmonary hemorrhage antibodies against the glomerular basement membrane (Ac anti-GBM) Figures Figure 1 What is new? This case highlights the neurological involvement of a patient diagnosed with anti-GBM disease, along with moderate kidney involvement and good clinical evolution with immunosuppressive treatment. Introduction Anti-GBM disease is a rare immunological disorder in the pediatric population characterized by the presence of circulating antibodies that act against an intrinsic antigen of the glomerular basement membrane, specifically the alpha 3 chain of type IV collagen of the glomerular basement membrane. It is associated with rapidly progressive glomerulonephritis (RPGN) in 40–60% of patients with concomitant alveolar hemorrhage, regardless of the presence of anti-glomerular basement membrane antibodies (anti-GBMs). Central nervous system involvement is quite exceptional in the absence of anti-neutrophil cytoplasmic antibodies (ANCAs). Although other antigens present in GBM have been described in recent decades, the critical role of T lymphocytes has also been emphasized. The exact mechanism that induces the formation of autoantibodies is unknown, but environmental and infectious factors, among other factors, may trigger the autoimmune response in genetically susceptible individuals. Triggering factors such as immunomodulatory drugs and vaccines are involved in atypical forms of the disease, and although infrequent, cerebral vasculitis has also been reported. We present the case of a teenager with anti-GBM disease with renal, pulmonary, neurological, and vascular involvement. Case Report We present the case of a 13-year-old female patient without a significant personal or family medical history who was admitted to a primary hospital due to generalized tonic‒clonic seizures in the context of a skin infection. Two blood cultures were taken, and she was empirically treated with intravenous antibiotics. She evolved with acute deterioration of consciousness requiring mechanical ventilation assistance. A central nervous system (CNS) CT scan was performed, revealing intraparenchymal hemorrhage, and the patient was subsequently referred to our institution, where complementary studies were conducted, highlighting the findings from the echocardiogram of dilation of the aortic root and refringence of both coronary arteries, associated with a serum creatinine level of 1.92 mg/dl (eGFR 37 ml/m/1.73 m2), microhematuria, and a protein/creatinine ratio of 0.8 mg/mg, with preserved diuresis and hypertension. Immunological, infectious, and blood studies were requested. Seven days after her admission, she presented with respiratory failure requiring assisted ventilation; a lung CT scan revealed bilateral interstitial infiltrates compatible with intraalveolar bleeding. Autoimmune serologies were as follows : ANA + 640; anti-GBM antibodies + IFI ; ANCA, anti-DNA and ENAs were negative; and complement levels were normal. Microbiological tests for IgG against CMV, HSV, EBV, HBV, HCV, HIV, toxoplasmosis, and VZV were negative. Abdominal ultrasound revealed mildly increased echogenicity in both kidneys. The diagnosis of anti-GBM antibody disease was suspected, and treatment was immediately initiated with 3 intravenous pulses of methylprednisolone (15 mg/kg/dose). The patient received 7 sessions of plasmapheresis until the anti-GBM antibodies were undetectable and 6 pulses of cyclophosphamide at 15 mg/kg/d. The renal biopsy revealed that 6 of 27 glomeruli with crescents (27%) and 4 globally sclerosed glomeruli demonstrated mild mesangial proliferation and endocapillary proliferation. Immunofluorescence analysis revealed the following: IgG +++/++++, with linear effects on capillary walls and Bowman's capsule globally and diffusely. A pattern of extracapillary glomerulonephritis associated with linear IgG deposition confirmed the diagnosis of anti-GBM disease. Owing to the deterioration of the sense, a brain MRI Angio was performed, reporting microbleeds in the hemispheres and cerebellum with a decrease in arterial caliber and a rosary appearance in segments of the middle cerebral artery and vertebral artery, findings compatible with CNS vasculitis. Finally, the patient showed progressive neurological improvement and discontinued immunosuppression 6 months after starting treatment. Renal function also normalized. Discussion Anti-MBG disease is a rare condition in the pediatric population. We report a clinical case of a previously healthy 13-year-old girl who presented with an atypical manifestation of the disease with cerebral vascular involvement, a rarely described finding. Neurological involvement was her initial clinical manifestation. The presence of vasculitis on angiography suggests an additional inflammatory mechanism mediated by autoimmunity. Central nervous system manifestations are extremely rare in patients with anti-GBM disease, with only a few cases reported in the literature (8–10). Gittins reported on a teenage patient diagnosed with anti-GBM disease who presented with seizures, lacked anti-neutrophil cytoplasmic antibodies (ANCAs), and whose anti-GBM antibody titers had normalized after 3 weeks of plasmapheresis and immunosuppressive therapy. On the other hand, Jee Young Kim described the case of a previously healthy 34-year-old patient who experienced generalized tonic‒clonic seizures associated with recurrent hemoptysis for 1 month and was subsequently diagnosed with diffuse alveolar hemorrhage. The brain MRI revealed multiple vascular infarcts; at the renal level, he presented only hematuria, and the anti-GBM and ANCA antibodies were negative. Immunofluorescence revealed abundant linear IgG deposits in the pulmonary and renal tissues, suggesting a diagnosis of anti-GBM disease, and the patient received 14 sessions of plasmapheresis with pulse methylprednisolone as treatment. Although the 'gold standard' for diagnosing CNS vasculitis is biopsy of the leptomeninges and brain, in our patient, which coincides with the majority of reported cases in the literature, we diagnosed CNS vasculitis on the basis of clinical characteristics and MRI findings. In accordance with the KDIGO guidelines, we administered 3 pulses of methylprednisolone along with 6 pulses of cyclophosphamide and 7 sessions of plasmapheresis. After completing the treatment, the patient did not experience another episode of intra-alveolar hemorrhage, and her renal and neurological function progressively improved. Currently, she is not on maintenance immunosuppression, as it is not recommended for her underlying diagnosis. In summary, we believe that our patient had an atypical presentation of anti-GBM disease, as she exhibited extrarenal involvement (ANCA-negative CNS vasculitis) without severe compromise or requirements for renal replacement therapy, as typically required by patients with typical anti-GMB disease. We can conclude that anti-GBM antibody disease can manifest with multisystemic involvement, including cerebral vasculitis. Therefore, we emphasize the importance of a multidisciplinary approach as well as early diagnosis and immunosuppressive treatment to improve the prognosis of pediatric patients. References Bharati J, Kenar D, Jhaveri, Alan D, Salama, and Oni L . Anti–Glomerular Basement Membrane Disease. Recent Updates. Adv in Kidney Disease and Health 2024;31(3):206-215 https://doi.org/10.1053/j.akdh.2024.04.007 Reggiani F, L’Imperio V, Calatroni M, Pagni F, Sinico R. Goodpasture syndrome and anti-glomerular basement membrane disease. Clinical and Experimental Rheumatology 2023:964- 974 Gittins N, Basu A, Eyre J, Moghal N y col. Cerebral vasculitis in a teenager with Goodpasture syndrome nephropathy dialysis transplantation 2004; 19 (12): 3168- 3171 Jee Young Kim, Kook Jin Ahn, Jung Im Jung y col .Imaging findings of Central nervous system vasculitis associated with Goodpasture Syndrome : Case report.Korean J radiologist 2007; 8: 545 - 547 KDIGO. Clinical practice guideline on glomerular disease 2021. Additional Declarations We declare no conflicts of interest. Family signed consent for publish the case . Cite Share Download PDF Status: Under Revision Version 1 posted Editorial decision: Major Revisions Needed 25 Aug, 2025 Reviewers agreed at journal 13 Aug, 2025 Reviewers invited by journal 12 Aug, 2025 First submitted to journal 05 Aug, 2025 Editor assigned by journal 01 Aug, 2025 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-7264487","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":499634852,"identity":"e16b4114-2692-40b4-aec4-31fbcb97b7a7","order_by":0,"name":"Veronica Alejandra Bravo Díaz","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA/klEQVRIie3PMUvEMBTA8SeF69LSTTwK8RMIrxQEl/Or5DjoLQc6ZrjhQOh07jkU/RBC53cE2iUfQLCDt3SOm6OtmQTTw80hfzI98uMlAD7fP42+D0D4aQTZyfsxQpbAVGqCaJjw42ssSePSEhgj2NwflBHtTSIXlO2eWnZ9qg79lhm72DiIbpBId1fyteD8o+ryKC2wJ4v8kn4nU1kA7UuFoDXud5Wab1M+EJpXLvLcWXKudXYXPw5kaUZJcjaxBJttHsSbgazGtyRR/zCtO8yasjiRtcqjt9UtcXT/ZRLWgRGiRaaCGsxasfBh+WKMmDEXcYV/u+7z+Xy+n30BI9ltsnSyLoEAAAAASUVORK5CYII=","orcid":"https://orcid.org/0009-0006-1116-9508","institution":"Ricardo Gutierrez Children's Hospital: El Hospital de Ninos Ricardo Gutierrez","correspondingAuthor":true,"prefix":"","firstName":"Veronica","middleName":"Alejandra Bravo","lastName":"Díaz","suffix":""},{"id":499634853,"identity":"4125551f-69b0-46b1-adf7-87f76c7ef113","order_by":1,"name":"Xavier Miranda","email":"","orcid":"","institution":"El Hospital de Niños Ricardo Gutierrez: El Hospital de Ninos Ricardo Gutierrez","correspondingAuthor":false,"prefix":"","firstName":"Xavier","middleName":"","lastName":"Miranda","suffix":""},{"id":499634854,"identity":"235700e2-a292-4ee9-af8d-edc6d9f746ce","order_by":2,"name":"Ana Lucia Saez","email":"","orcid":"","institution":"El Hospital de Niños Ricardo Gutierrez: El Hospital de Ninos Ricardo Gutierrez","correspondingAuthor":false,"prefix":"","firstName":"Ana","middleName":"Lucia","lastName":"Saez","suffix":""},{"id":499634855,"identity":"e4d4ffa3-ecec-41ee-8d95-b5a0cdf46b66","order_by":3,"name":"Miguel Liern","email":"","orcid":"","institution":"El Hospital de Niños Ricardo Gutierrez: El Hospital de Ninos Ricardo Gutierrez","correspondingAuthor":false,"prefix":"","firstName":"Miguel","middleName":"","lastName":"Liern","suffix":""}],"badges":[],"createdAt":"2025-07-31 17:06:15","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-7264487/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-7264487/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":89838821,"identity":"573130c0-db4b-4f16-8e9a-9dd99f229016","added_by":"auto","created_at":"2025-08-25 15:07:21","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":115883,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eTimeline of the clinical case\u003c/strong\u003e\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-7264487/v1/b31cb96156231d23be55720a.png"},{"id":89838824,"identity":"120884ae-3fd9-4565-a7ae-6068055a7567","added_by":"auto","created_at":"2025-08-25 15:07:26","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":347344,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7264487/v1/357b83af-387f-41b2-b7c0-374a0f61d6f2.pdf"}],"financialInterests":"\u003cp\u003eWe declare no conflicts of interest.\u003c/p\u003e\n\u003cp\u003eFamily signed consent for publish the case .\u003c/p\u003e","formattedTitle":"Atypical presentation of anti-glomerular basement membrane disease. Pediatric Case","fulltext":[{"header":"What is new?","content":"\u003cp\u003eThis case highlights the neurological involvement of a patient diagnosed with anti-GBM disease, along with moderate kidney involvement and good clinical evolution with immunosuppressive treatment.\u003c/p\u003e"},{"header":"Introduction","content":"\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eAnti-GBM disease is a rare immunological disorder in the pediatric population characterized by the presence of circulating antibodies that act against an intrinsic antigen of the glomerular basement membrane, specifically the alpha 3 chain of type IV collagen of the glomerular basement membrane.\u003c/span\u003e\u003c/p\u003e\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eIt is associated with rapidly progressive glomerulonephritis (RPGN) in 40–60% of patients with concomitant alveolar hemorrhage, regardless of the presence of anti-glomerular basement membrane antibodies (anti-GBMs). Central nervous system involvement is quite exceptional in the absence of anti-neutrophil cytoplasmic antibodies (ANCAs).\u003c/span\u003e\u003c/p\u003e\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eAlthough other antigens present in GBM have been described in recent decades, the critical role of T lymphocytes has also been emphasized. The exact mechanism that induces the formation of autoantibodies is unknown, but environmental and infectious factors, among other factors, may trigger the autoimmune response in genetically susceptible individuals.\u003c/span\u003e\u003c/p\u003e\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eTriggering factors such as immunomodulatory drugs and vaccines are involved in atypical forms of the disease, and although infrequent, cerebral vasculitis has also been reported. We present the case of a teenager with anti-GBM disease with renal, pulmonary, neurological, and vascular involvement.\u003c/span\u003e\u003c/p\u003e"},{"header":"Case Report","content":"\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eWe present the case of a 13-year-old female patient without a significant personal or family medical history who was admitted to a primary hospital due to generalized tonic‒clonic seizures in the context of a skin infection. Two blood cultures were taken, and she was empirically treated with intravenous antibiotics. She evolved with acute deterioration of consciousness requiring mechanical ventilation assistance. A central nervous system (CNS) CT scan was performed, revealing intraparenchymal hemorrhage, and the patient was subsequently referred to our institution, where complementary studies were conducted, highlighting the findings from the echocardiogram of dilation of the aortic root and refringence of both coronary arteries, associated with a serum creatinine level of 1.92 mg/dl (eGFR 37 ml/m/1.73 m2), microhematuria, and a protein/creatinine ratio of 0.8 mg/mg, with preserved diuresis and hypertension.\u003c/span\u003e\u003c/p\u003e\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eImmunological, infectious, and blood studies were requested. Seven days after her admission, she presented with respiratory failure requiring assisted ventilation; a lung CT scan revealed bilateral interstitial infiltrates compatible with intraalveolar bleeding.\u003c/span\u003e\u003c/p\u003e\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eAutoimmune serologies were as follows\u003c/span\u003e: \u003cspan type=\"BoldItalicSmallCaps\" class=\"BoldItalicSmallCaps\" name=\"Emphasis\"\u003eANA + 640; anti-GBM antibodies + IFI\u003c/span\u003e; \u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eANCA, anti-DNA and ENAs were negative; and complement levels were normal. Microbiological tests for IgG against CMV, HSV, EBV, HBV, HCV, HIV, toxoplasmosis, and VZV were negative. Abdominal ultrasound revealed mildly increased echogenicity in both kidneys.\u003c/span\u003e\u003c/p\u003e\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eThe diagnosis of anti-GBM antibody disease was suspected, and treatment was immediately initiated with 3 intravenous pulses of methylprednisolone (15 mg/kg/dose). The patient received 7 sessions of plasmapheresis until the anti-GBM antibodies were undetectable and 6 pulses of cyclophosphamide at 15 mg/kg/d.\u003c/span\u003e\u003c/p\u003e\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eThe renal biopsy revealed that 6 of 27 glomeruli with crescents (27%) and 4 globally sclerosed glomeruli demonstrated mild mesangial proliferation and endocapillary proliferation. Immunofluorescence analysis revealed the following: IgG +++/++++, with linear effects on capillary walls and Bowman's capsule globally and diffusely. A pattern of extracapillary glomerulonephritis associated with linear IgG deposition confirmed the diagnosis of anti-GBM disease.\u003c/span\u003e\u003c/p\u003e\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eOwing to the deterioration of the sense, a brain MRI Angio was performed, reporting microbleeds in the hemispheres and cerebellum with a decrease in arterial caliber and a rosary appearance in segments of the middle cerebral artery and vertebral artery, findings compatible with CNS vasculitis. Finally, the patient showed progressive neurological improvement and discontinued immunosuppression 6 months after starting treatment. Renal function also normalized.\u003c/span\u003e\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eAnti-MBG disease is a rare condition in the pediatric population. We report a clinical case of a previously healthy 13-year-old girl who presented with an atypical manifestation of the disease with cerebral vascular involvement, a rarely described finding. Neurological involvement was her initial clinical manifestation. The presence of vasculitis on angiography suggests an additional inflammatory mechanism mediated by autoimmunity.\u003c/span\u003e\u003c/p\u003e\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eCentral nervous system manifestations are extremely rare in patients with anti-GBM disease, with only a few cases reported in the literature (8–10). Gittins reported on a teenage patient diagnosed with anti-GBM disease who presented with seizures, lacked anti-neutrophil cytoplasmic antibodies (ANCAs), and whose anti-GBM antibody titers had normalized after 3 weeks of plasmapheresis and immunosuppressive therapy. On the other hand, Jee Young Kim described the case of a previously healthy 34-year-old patient who experienced generalized tonic‒clonic seizures associated with recurrent hemoptysis for 1 month and was subsequently diagnosed with diffuse alveolar hemorrhage. The brain MRI revealed multiple vascular infarcts; at the renal level, he presented only hematuria, and the anti-GBM and ANCA antibodies were negative. Immunofluorescence revealed abundant linear IgG deposits in the pulmonary and renal tissues, suggesting a diagnosis of anti-GBM disease, and the patient received 14 sessions of plasmapheresis with pulse methylprednisolone as treatment.\u003c/span\u003e\u003c/p\u003e\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eAlthough the 'gold standard' for diagnosing CNS vasculitis is biopsy of the leptomeninges and brain, in our patient, which coincides with the majority of reported cases in the literature, we diagnosed CNS vasculitis on the basis of clinical characteristics and MRI findings.\u003c/span\u003e\u003c/p\u003e\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eIn accordance with the KDIGO guidelines, we administered 3 pulses of methylprednisolone along with 6 pulses of cyclophosphamide and 7 sessions of plasmapheresis. After completing the treatment, the patient did not experience another episode of intra-alveolar hemorrhage, and her renal and neurological function progressively improved. Currently, she is not on maintenance immunosuppression, as it is not recommended for her underlying diagnosis.\u003c/span\u003e\u003c/p\u003e\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eIn summary, we believe that our patient had an atypical presentation of anti-GBM disease, as she exhibited extrarenal involvement (ANCA-negative CNS vasculitis) without severe compromise or requirements for renal replacement therapy, as typically required by patients with typical anti-GMB disease.\u003c/span\u003e\u003c/p\u003e\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eWe can conclude that anti-GBM antibody disease can manifest with multisystemic involvement, including cerebral vasculitis. Therefore, we emphasize the importance of a multidisciplinary approach as well as early diagnosis and immunosuppressive treatment to improve the prognosis of pediatric patients.\u003c/span\u003e\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eBharati J, Kenar D, Jhaveri, Alan D, Salama, and Oni L . Anti\u0026ndash;Glomerular Basement Membrane Disease. Recent Updates. Adv in Kidney Disease and Health 2024;31(3):206-215 \u003cem\u003ehttps://doi.org/10.1053/j.akdh.2024.04.007\u003c/em\u003e\u003c/li\u003e\n\u003cli\u003eReggiani F, L\u0026rsquo;Imperio V, Calatroni M, Pagni F, Sinico R. Goodpasture syndrome and anti-glomerular basement membrane disease. Clinical and Experimental Rheumatology 2023:964- 974\u003c/li\u003e\n\u003cli\u003eGittins N, Basu A, Eyre J, Moghal N y col. Cerebral vasculitis in a teenager with Goodpasture syndrome nephropathy dialysis transplantation 2004; 19 (12): 3168- 3171\u003c/li\u003e\n\u003cli\u003eJee Young Kim, Kook Jin Ahn, Jung Im Jung y col .Imaging findings of Central nervous system vasculitis associated with Goodpasture Syndrome : Case report.Korean J radiologist 2007; 8: 545 - 547\u003c/li\u003e\n\u003cli\u003eKDIGO. Clinical practice guideline on glomerular disease 2021.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"pediatric-nephrology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pnep","sideBox":"Learn more about [Pediatric Nephrology](http://link.springer.com/journal/467)","snPcode":"467","submissionUrl":"https://www.editorialmanager.com/pnep/default2.aspx","title":"Pediatric Nephrology","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"Cerebral vasculitis, rapidly progressive glomerulonephritis (RPGN), pulmonary hemorrhage, antibodies against the glomerular basement membrane (Ac anti-GBM)","lastPublishedDoi":"10.21203/rs.3.rs-7264487/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7264487/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cspan type=\"SmallCaps\" class=\"SmallCaps\" name=\"Emphasis\"\u003eWe describe the clinical case of a 13-year-old girl diagnosed with anti-glomerular basement membrane disease and central nervous system involvement. The patient presented with a generalized tonic‒clonic seizure as the first clinical finding secondary to an intraparenchymal hemorrhage and cerebral vasculitis. Acute renal injury was also detected without the need for renal replacement therapy, hematuria, arterial hypertension, significant proteinuria, and pulmonary hemorrhage with diffuse ground‒glass alveolar infiltrate. The diagnosis was established through renal biopsy, which revealed a pattern of extracapillary glomerulonephritis with linear deposition of IgG and the presence of anti-GBM antibodies in the plasma. In our case, the patient was treated with pulses of corticosteroids, cyclophosphamide, and plasmapheresis. She evolved with normalization of renal function, albuminuria, and no neurological sequelae on imaging. Anti-glomerular basement membrane disease is a vasculitis of small vessels that affects the kidneys (90%) and lungs (60%) and is the cause of 1\u0026ndash;2% of acute glomerulonephritis and 10\u0026ndash;15% of rapidly progressive glomerulonephritis.\u003c/span\u003e\u003c/p\u003e","manuscriptTitle":"Atypical presentation of anti-glomerular basement membrane disease. Pediatric Case","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-08-25 14:59:17","doi":"10.21203/rs.3.rs-7264487/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Major Revisions Needed","date":"2025-08-25T12:54:39+00:00","index":"","fulltext":""},{"type":"reviewerAgreed","content":"","date":"2025-08-13T20:33:58+00:00","index":0,"fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-08-13T00:20:47+00:00","index":"","fulltext":""},{"type":"submitted","content":"Pediatric Nephrology","date":"2025-08-05T14:43:45+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-08-01T15:52:16+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"pediatric-nephrology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pnep","sideBox":"Learn more about [Pediatric Nephrology](http://link.springer.com/journal/467)","snPcode":"467","submissionUrl":"https://www.editorialmanager.com/pnep/default2.aspx","title":"Pediatric Nephrology","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false}}],"origin":"","ownerIdentity":"20faf9b3-c420-4ebc-b494-f010066d0fa1","owner":[],"postedDate":"August 25th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"in-revision","subjectAreas":[],"tags":[],"updatedAt":"2025-08-25T16:55:38+00:00","versionOfRecord":[],"versionCreatedAt":"2025-08-25 14:59:17","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-7264487","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7264487","identity":"rs-7264487","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}