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The Presence of ANCA in IgA Crescentic Nephropathy Does Not Lead to Worse Prognosis with Intensive Rescue Treatment Agraz Pamplona, Irene Castañeda Amado, Zaira Ivette Sanz Martinez, Maria Teresa Gabaldon Dominguez, Alejandra Bermejo Garcia, Sheila Viñas Gimenez, Laura Bury, Roxana Bolufer Cardona, Monica Lopez Martinez, Marina Bestard Matamoros, Oriol Soler Romeo, Maria Jose Ramos Terrades, Natalia Institut Català de la Salut [Agraz I, Castañeda Z, Bermejo S, Bury R, Bolufer M, López-Martínez M, Ramos N, Bestard O, Soler MJ] Servei de Nefrologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Reference Center for Complex Glomerular Diseases (CSUR), Barcelona, Spain. [Sanz-Martínez MT, Viñas Gimenez L] Servei d’Immunologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Gabaldón A] Servei d’Anatomia Patològica, Vall d’Hebron Hospital Universitari, Barcelona, Spain Vall d'Hebron Barcelona Hospital Campus Medicaments immunosupressors Glomerulonefritis Autoanticossos DISEASES::Immune System Diseases::Autoimmune Diseases::Glomerulonephritis, IGA CHEMICALS AND DRUGS::Amino Acids, Peptides, and Proteins::Proteins::Blood Proteins::Immunoproteins::Immunoglobulins::Antibodies::Autoantibodies::Antibodies, Antineutrophil Cytoplasmic CHEMICALS AND DRUGS::Chemical Actions and Uses::Pharmacologic Actions::Physiological Effects of Drugs::Immunologic Factors::Immunosuppressive Agents ENFERMEDADES::enfermedades del sistema inmune::enfermedades autoinmunes::glomerulonefritis por IgA COMPUESTOS QUÍMICOS Y DROGAS::aminoácidos, péptidos y proteínas::proteínas::proteínas sanguíneas::inmunoproteínas::inmunoglobulinas::anticuerpos::autoanticuerpos::anticuerpos anticitoplasma de neutrófilos COMPUESTOS QUÍMICOS Y DROGAS::acciones y usos químicos::acciones farmacológicas::efectos fisiológicos de los fármacos::factores inmunitarios::inmunosupresores Nefropatía por IgA; Autoanticuerpos anticitoplasma de neutrófilos IgA nephropathy; Antineutrophil cytoplasmic autoantibodies Nefropatia per IgA; Anticossos anticitoplasma de neutròfils Background: Immunoglobulin A nephropathy (IgAN) is the most common glomerulonephritis worldwide. The concomitant presence of both crescentic proliferation and anti-neutrophil cytoplasmic autoantibodies (ANCA) in this pathology represents a rare coincidence. However, it is not clear to what extent the presence of ANCA (IgA or IgG) in these patients could have any clinical significance. The aim of the current work is to describe the presence of ANCA (IgA or IgG) in patients with IgAN and crescentic proliferation and its possible clinical implications. Methods: We retrospectively recruited all patients in our center with a histological diagnosis of IgAN with crescentic proliferation between January 2013 and December 2020. The main demographic and clinicopathologic data, fundamental histological characteristics, as well as the treatments implemented and main kidney outcomes, were collected and analyzed at a 6 and 12-month follow-up. Results: Between January 2013 and December 2020, a total of 17 adults were diagnosed with concomitant crescentic proliferation through a kidney biopsy of IgAN. Five (29.4%) patients showed ANCA, three (60%) showed IgA-ANCA and two (40%) showed IgG-ANCA. All ANCA-positive patients had some degree of crescentic proliferation. At diagnosis, the mean age of patients was 48 years old (range: 27–75). Nine of them were women (52%) and the most common clinical presentation was hypertension (71%). At the time of biopsy, the mean serum creatinine and proteinuria were 2.2 mg/dL (DS 1.42) and 3.5 g/mgCr (DS 1.22), respectively, with no statistical differences between ANCA-positive and -negative patients. Histological analyses showed that 11 out of the 12 (91%) ANCA-negative IgAN patients displayed less than 25% cellular crescents, whereas 100% of ANCA-positive IgAN patients displayed more than 25% cellular crescents (p = 0.04). Notably, five (30%) patients displayed fibrinoid necrosis, with four of them (80%) being IgAN-ANCA-positive (p = 0.01). Only one ANCA-negative patient needed renal replacement therapy (RRT) upon admission (5%). The mean serum creatinine and proteinuria were 1.94 mg/dL (DS 1.71) and 1.45 g/gCr (DS 1.78), respectively, within 6 months of immunosuppressive therapy. At 12-month follow-up, the mean creatinine was 1.57 mg/dL (DS 1). Four (23.5%) patients needed RRT at the end of the follow-up and four (23.5%) patients died. Conclusions: Probably due to the limited number of IgAN-ANCA-positive and IgAN-ANCA-negative patients, no significant differences were found between the clinical and laboratory characteristics. IgAN-ANCA-negative patients seemed to display less extracapillary proliferation than IgAN-ANCA-positive patients, who tended to show significantly higher fibrinoid necrosis. There were no differences regarding renal prognosis and patient survival after aggressive immunosuppressive therapy within 6 and 12 months when comparing the two samples. 2022-12-22T07:19:28Z 2022-12-22T07:19:28Z 2022-11-30 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Agraz I, Castañeda Z, Sanz-Martínez MT, Gabaldón A, Bermejo S, Viñas Gimenez L, et al. The Presence of ANCA in IgA Crescentic Nephropathy Does Not Lead to Worse Prognosis with Intensive Rescue Treatment. J Clin Med. 2022 Nov 30;11(23):7122. 2077-0383 https://hdl.handle.net/11351/8708 10.3390/jcm11237122 36498699 http://hdl.handle.net/11351/8708 eng Journal of Clinical Medicine;11(23) https://doi.org/10.3390/jcm11237122 Attribution 4.0 International http://creativecommons.org/licenses/by/4.0/ info:eu-repo/semantics/openAccess application/pdf MDPI Scientia