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Scientific paper - Case study
Pulmonary Hemorrhage and Crescentic Glomerulonephritis in a Patient with Seropositive Anti-Glomerular Basement Membrane Disease and Anti-Neutrophil Cytoplasmic Antibodies
Acta clinica Croatica, 61 (2022), 3; 528-532. https://doi.org/10.20471/acc.2022.61.03.19
Sreter, Katherina Bernadette; Pavlović, Draško; Tomić, Monika; Šenjug, Petar; Galešić Ljubanović, Danica

Institutional repository: Repository of the Sestre milosrdnice University Hospital Center

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Sreter, K. B., Pavlović, D., Tomić, M., Šenjug, P. & Galešić Ljubanović, D. (2022). Pulmonary Hemorrhage and Crescentic Glomerulonephritis in a Patient with Seropositive Anti-Glomerular Basement Membrane Disease and Anti-Neutrophil Cytoplasmic Antibodies. Acta clinica Croatica, 61. (3), 528-532. doi: 10.20471/acc.2022.61.03.19

Sreter, Katherina Bernadette, et al. "Pulmonary Hemorrhage and Crescentic Glomerulonephritis in a Patient with Seropositive Anti-Glomerular Basement Membrane Disease and Anti-Neutrophil Cytoplasmic Antibodies." Acta clinica Croatica, vol. 61, no. 3, 2022, pp. 528-532. https://doi.org/10.20471/acc.2022.61.03.19

Sreter, Katherina Bernadette, Draško Pavlović, Monika Tomić, Petar Šenjug and Danica Galešić Ljubanović. "Pulmonary Hemorrhage and Crescentic Glomerulonephritis in a Patient with Seropositive Anti-Glomerular Basement Membrane Disease and Anti-Neutrophil Cytoplasmic Antibodies." Acta clinica Croatica 61, no. 3 (2022): 528-532. https://doi.org/10.20471/acc.2022.61.03.19

Sreter, K. B., et al. (2022) 'Pulmonary Hemorrhage and Crescentic Glomerulonephritis in a Patient with Seropositive Anti-Glomerular Basement Membrane Disease and Anti-Neutrophil Cytoplasmic Antibodies', Acta clinica Croatica, 61(3), pp. 528-532. doi: 10.20471/acc.2022.61.03.19

Sreter KB, Pavlović D, Tomić M, Šenjug P, Galešić Ljubanović D. Pulmonary Hemorrhage and Crescentic Glomerulonephritis in a Patient with Seropositive Anti-Glomerular Basement Membrane Disease and Anti-Neutrophil Cytoplasmic Antibodies. Acta clinica Croatica [Internet]. 2022 [cited 2024 December 24];61(3):528-532. doi: 10.20471/acc.2022.61.03.19

K. B. Sreter, D. Pavlović, M. Tomić, P. Šenjug and D. Galešić Ljubanović, "Pulmonary Hemorrhage and Crescentic Glomerulonephritis in a Patient with Seropositive Anti-Glomerular Basement Membrane Disease and Anti-Neutrophil Cytoplasmic Antibodies", Acta clinica Croatica, vol. 61, no. 3, pp. 528-532, 2022. [Online]. Available at: https://urn.nsk.hr/urn:nbn:hr:220:354994. [Accessed: 24 December 2024]

Metadata
Title (english)Pulmonary Hemorrhage and Crescentic Glomerulonephritis in a Patient with Seropositive Anti-Glomerular Basement Membrane Disease and Anti-Neutrophil Cytoplasmic Antibodies
Title (croatian)Plućno krvarenje i glomerulonefritis s polumjesecima u pacijentu sa seropozitivnom antiglomerularnom bolesti bazne membrane i anti-neutrofilnim citoplazmičnim antitijelima
AuthorKatherina Bernadette Sreter
AuthorDraško Pavlović
AuthorMonika Tomić
AuthorPetar Šenjug
AuthorDanica Galešić Ljubanović
Author's institution(Sestre milosrdnice University Hospital Center)
Scientific / art field,
discipline and subdiscipline		BIOMEDICINE AND HEALTHCARE
Clinical Medical Sciences
Abstract (english)
Anti-glomerular basement membrane (anti-GBM) disease is an acute and life-threatening systemic autoimmune disorder. The coexistence of circulating anti-neutrophil cytoplasmic antibodies (ANCA) and anti-GBM disease, the so-called double-positive disease (DPD), is exceptionally rare. We report a unique case of DPD manifesting as pulmonary-renal syndrome (PRS) in a 46-year-old woman who first presented with clinical and radiological suspicion of pneumonia. Chest computed tomography scan later revealed bilateral alveolar hemorrhage. Kidney biopsy showed necrotizing crescentic (100% glomeruli) glomerulonephritis. On immunofluorescence microscopy, glomeruli were global linear positive for IgG, confirming anti-GBM disease. Double positivity was detected for circulating anti-myeloperoxidase ANCA (p-ANCA) and anti-GBM antibodies. Acute renal failure evolved rapidly. Therapeutic plasma exchange (TPE) and hemodialysis (HD) were initiated early in combination with intravenous pulse corticosteroid therapy followed by oral methylprednisolone and cyclophosphamide. Pulmonary hemorrhage resolved, but renal function could not be preserved. The patient remains HD dependent. This case report highlights that pulmonary symptomatology may be the leading clinical presentation of PRS, with initially normal renal function at DPD onset. Early recognition and diagnosis are therefore crucial to timely clinical intervention. The role of prompt kidney biopsy and initiation of TPE and HD in PRS must not be underestimated.
Abstract (croatian)
Bolest protiv glomerularne bazalne membrane (anti-GBM) je akutna i po život opasna sistemska autoimuna bolest. Pojava cirkulirajućih antitijela na citoplazmu granulocita (engl. anti-neutrophil cytoplasmic antibody, ANCA) u bolesnika sa anti-GBM glomerulonefritisom, tako zvana dvostruko pozitivna bolest (engl. double-positive disease, DPD), je vrlo rijetka. Prikazujemo 46 godišnju bolesnicu u koje je prvo klinički i radiološki postavljena sumnja na upalu pluća, a koja se kasnije manifestirala sa pulmo-renalnim sindromom (PRS) odnosno DPD. Kompjutorskom tomografijom pluća dokazano je alveolarno krvarenje. Biopsijom bubrega dokazan je nekrotizirajući glomerulonefritis (100 % glomerula). Imunoflorescencija je pokazala pozitivne linearne IgG depozite, što odgovara anti-GBM glomerulonefritisu. U bolesnice su dokazana antitijela na mijeloperoksidazu p-ANCA i antitijela na glomerularnu bazalnu membranu. Liječena je terapijskom izmjenom plazme (engl. therapeutic plasma exchange, TPE), hemodijalizom te kombinacijom parenteralne pulsne terapije kortikosteroidima, kasnije oralnom primjenom metilprednisolona i ciklofosfamida. Došlo je do regresije krvarenja u plućima ali se bubrežna funkcija nije oporavila, zbog čega smo nastavili s redovitim hemodijalizama. Ovaj prikaz bolesnice pokazuje kako u DPD, plućna simptomatologija može biti vodeći simptom PRS sa urednom bubrežnom funkcijom u početku. Rano prepoznavanja i dijagnoza su značajni za pravovremeni početak liječenja. Potrebno je naglasiti značaj rane biopsije bubrega, ranog početka TPE te po potrebi i nadomještanje bubrežne funkcije hemodijalizom.
Keywords (english)
Keywords (croatian)
Languageenglish
Languagecroatian
Publication typeScientific paper - Case study
Publication statusPublished
Peer reviewPeer review
Publication versionPublished version
Journal titleActa clinica Croatica
Numberingvol. 61, no. 3, pp. 528-532
p-ISSN0353-9466
e-ISSN1333-9451
DOIhttps://doi.org/10.20471/acc.2022.61.03.19
URN:NBNurn:nbn:hr:220:354994
Publication2022
Document URLhttps://hrcak.srce.hr/296211
Type of resourceText
Access conditionsOpen access
Terms of useLicence In copyright icon
Created on2024-09-23 10:55:40