Síndrome Pulmón-Riñón como Presentación Inicial de Poliangeítis Microscópica ANCA-Asociada: Reporte de Caso
Resumen
El síndrome pulmón-riñón, definido por la concurrencia de hemorragia alveolar difusa (HAD) y glomerulonefritis de rápida progresión, constituye una emergencia médica de alta morbimortalidad cuyo reconocimiento temprano resulta determinante para el pronóstico. Una de sus causas más relevantes son las vasculitis asociadas a anticuerpos anticitoplasma de neutrófilo (ANCA), grupo de trastornos crónicos caracterizados por inflamación necrotizante de pequeños y medianos vasos, que engloba tres entidades: granulomatosis con poliangeítis (GPA), poliangeítis microscópica (MPA) y granulomatosis eosinofílica con poliangeítis (EGPA) (Chung et al., 2021; Grayson et al., 2022; Lyons et al., 2022; Suppiah et al., 2022). La poliangeítis microscópica es una vasculitis necrotizante pauciinmune que afecta principalmente capilares, vénulas y arteriolas, manifestándose con mayor frecuencia como glomerulonefritis necrotizante y/o capilaritis pulmonar. Los anticuerpos ANCA están presentes en más del 90% de los pacientes con MPA; la HAD y la falla renal aguda son sus complicaciones agudas más graves y requieren diagnóstico y tratamiento inmediatos por el riesgo vital que implican (Jayne, 2021; Nasser & Cottin, 2021). La HAD compromete la función respiratoria en más del 90% de los casos, requiriendo manejo agresivo en unidad de cuidados intensivos (UCI). En el presente artículo se describe el caso de una paciente femenina de 44 años que debutó con síndrome pulmón-riñón como manifestación inicial de MPA, con diagnóstico confirmado mediante clínica, laboratorio y biopsia, y en quien se instauró tratamiento exitoso con pulsos de metilprednisolona, recambio plasmático y rituximab como terapia de inducción.
Descargas
Citas
Alberici, F., Flossmann, O., Lamprecht, P., Loudon, K. W., Padoan, R., Popov, T., Salvarani, C., & Mohammad, A. J. (2025). Antineutrophil cytoplasmic antibody-associated vasculitis: insights into relapse risk and future management directions. Frontiers in Immunology, 16(1655326), 1655326. https://doi.org/10.3389/fimmu.2025.1655326
Bitton, N., Alley, E., Gonter, A., Chang, L., & Namjouyan, K. (2025). Plasmapheresis saves the day: A therapeutic approach in severe diffuse alveolar hemorrhage associated with granulomatosis with polyangiitis. American Journal of Respiratory and Critical Care Medicine, 211(Supplement_1), A5750–A5750. https://doi.org/10.1164/ajrccm.2025.211.abstracts.a5750
Casal Moura, M., Merkel, P. A., Jayne, D., Cid, M. C., Basu, N., Hellmich, B., Terrier, B., Rutgers, A., Gordon, J., Verhoeven, P., Kullman, J., Langford, C. A., Bajema, I. M., Geetha, D., Fervenza, F. C., Kitching, A. R., Stone, J. H., Specks, U., & Kronbichler, A. (2025). Challenges in the diagnosis, classification and prognosis of ANCA-associated vasculitis. Nature Reviews. Rheumatology, 21(12), 719–736. https://doi.org/10.1038/s41584-025-01306-w
Chung, S. A., Langford, C. A., Maz, M., Abril, A., Gorelik, M., Guyatt, G., Archer, A. M., Conn, D. L., Full, K. A., Grayson, P. C., Ibarra, M. F., Imundo, L. F., Kim, S., Merkel, P. A., Rhee, R. L., Seo, P., Stone, J. H., Sule, S., Sundel, R. P., … Mustafa, R. A. (2021). 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis & Rheumatology, 73(8), 1366–1383. https://doi.org/10.1002/art.41773
Delestre, F., Charles, P., Karras, A., Pagnoux, C., Néel, A., Cohen, P., Aumaître, O., Faguer, S., Gobert, P., Maurier, F., Samson, M., Godmer, P., Bonnotte, B., Cottin, V., Hanrotel-Saliou, C., Le Gallou, T., Carron, P.-L., Desmurs-Clavel, H., Direz, G., … French Vasculitis Study Group (FVSG). (2024). Rituximab as maintenance therapy for ANCA-associated vasculitides: pooled analysis and long-term outcome of 277 patients included in the MAINRITSAN trials. Annals of the Rheumatic Diseases, 83(2), 233–241. https://doi.org/10.1136/ard-2023-224623
Grayson, P. C., Ponte, C., Suppiah, R., Robson, J. C., Craven, A., Judge, A., Khalid, S., Hutchings, A., Luqmani, R. A., Watts, R. A., Merkel, P. A., & DCVAS Study Group. (2022). 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with polyangiitis. Annals of the Rheumatic Diseases, 81(3), 309–314. https://doi.org/10.1136/annrheumdis-2021-221794
Guillevin, L. (2025). How to prescribe rituximab wisely for antineutrophil cytoplasmic antibody-associated vasculitides. Clinical and Experimental Rheumatology, 43(4), 575–578. https://doi.org/10.55563/clinexprheumatol/b1ec25
Harigai, M., & Takada, H. (2022). Avacopan, a selective C5a receptor antagonist, for anti-neutrophil cytoplasmic antibody-associated vasculitis. Modern Rheumatology, 32(3), 475–483. https://doi.org/10.1093/mr/roab104
Hellmich, B., Sanchez-Alamo, B., Schirmer, J. H., Berti, A., Blockmans, D., Cid, M. C., Holle, J. U., Hollinger, N., Karadag, O., Kronbichler, A., Little, M. A., Luqmani, R. A., Mahr, A., Merkel, P. A., Mohammad, A. J., Monti, S., Mukhtyar, C. B., Musial, J., Price-Kuehne, F., … Jayne, D. (2024). EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Annals of the Rheumatic Diseases, 83(1), 30–47. https://doi.org/10.1136/ard-2022-223764
Konda, R., Rajasekaran, A., & Rizk, D. V. (2024). Antineutrophil cytoplasmic antibody-associated vasculitis. Current Opinion in Nephrology and Hypertension, 33(5), 503–511. https://doi.org/10.1097/MNH.0000000000001004
Mendes, R. de S., Silva, P. L., Robba, C., Battaglini, D., Lopes-Pacheco, M., Caruso-Neves, C., & Rocco, P. R. M. (2024). Advancements in understanding the mechanisms of lung-kidney crosstalk. Intensive Care Medicine Experimental, 12(1), 81. https://doi.org/10.1186/s40635-024-00672-1
Murray, J., O’Brien, S., & Mitchell, P. D. (2025). Diffuse alveolar hemorrhage. Seminars in Respiratory and Critical Care Medicine. https://doi.org/10.1055/a-2725-7274
Puéchal, X. (2025). Update on targeted treatments for ANCA-associated vasculitis. Joint, Bone, Spine: Revue Du Rhumatisme, 92(1), 105768. https://doi.org/10.1016/j.jbspin.2024.105768
Pyo, J. Y., Lee, L. E., Park, Y.-B., & Lee, S.-W. (2023). Comparison of the 2022 ACR/EULAR classification criteria for antineutrophil cytoplasmic antibody-associated vasculitis with previous criteria. Yonsei Medical Journal, 64(1), 11–17. https://doi.org/10.3349/ymj.2022.0435
Rathmann, J., & Mohammad, A. J. (2024). Classification criteria for ANCA associated vasculitis - ready for prime time? Current Rheumatology Reports, 26(9), 332–342. https://doi.org/10.1007/s11926-024-01154-9
Robson, J. C., Grayson, P. C., Ponte, C., Suppiah, R., Craven, A., Judge, A., Khalid, S., Hutchings, A., Watts, R. A., Merkel, P. A., Luqmani, R. A., & DCVAS Investigators. (2022). 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis. Annals of the Rheumatic Diseases, 81(3), 315–320. https://doi.org/10.1136/annrheumdis-2021-221795
Sada, K.-E., Kaname, S., Higuchi, T., Furuta, S., Nagasaka, K., Nanki, T., Tsuboi, N., Amano, K., Dobashi, H., Hiromura, K., Bando, M., Wada, T., Arimura, Y., Makino, H., & Harigai, M. (2023). Validation of new ACR/EULAR 2022 classification criteria for anti-neutrophil cytoplasmic antibody-associated vasculitis. Modern Rheumatology, 34(1), 144–150. https://doi.org/10.1093/mr/road017
Sattui, S. E., Jiang, B., Fu, X., Cook, C., Srivatsan, S., Williams, Z. K., Katz, G., Zhang, Y., & Wallace, Z. S. (2024). The effects of age and frailty on the risks of end-stage renal disease, death, and severe infection in older adults with antineutrophil cytoplasmic antibody-associated vasculitis: a retrospective cohort study. The Lancet. Rheumatology, 6(11), e771–e779. https://doi.org/10.1016/S2665-9913(24)00193-0
Sharma, P., Zonozi, R., & Geetha, D. (2024). ANCA-associated vasculitis. Advances in Kidney Disease and Health, 31(3), 194–205. https://doi.org/10.1053/j.akdh.2024.04.005
Smith, R. M., Jones, R. B., Specks, U., Bond, S., Nodale, M., Al-Jayyousi, R., Andrews, J., Bruchfeld, A., Camilleri, B., Carette, S., Cheung, C. K., Derebail, V., Doulton, T., Ferraro, A., Forbess, L., Fujimoto, S., Furuta, S., Gewurz-Singer, O., Harper, L., … RITAZAREM co-investigators. (2023). Rituximab versus azathioprine for maintenance of remission for patients with ANCA-associated vasculitis and relapsing disease: an international randomised controlled trial. Annals of the Rheumatic Diseases, 82(7), 937–944. https://doi.org/10.1136/ard-2022-223559
Suppiah, R., Robson, J. C., Grayson, P. C., Ponte, C., Craven, A., Khalid, S., Judge, A., Hutchings, A., Merkel, P. A., Luqmani, R. A., Watts, R. A., & DCVAS INVESTIGATORS. (2022). 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis. Annals of the Rheumatic Diseases, 81(3), 321–326. https://doi.org/10.1136/annrheumdis-2021-221796
Tesar, V., Hartinger, J. M., & Hruskova, Z. (2024). Avacopan as an add-on therapy for ANCA-associated vasculitis: a pharmacological overview. Expert Review of Clinical Pharmacology, 17(12), 1099–1113. https://doi.org/10.1080/17512433.2024.2432500
Trivioli, G., Marquez, A., Martorana, D., Tesi, M., Kronbichler, A., Lyons, P. A., & Vaglio, A. (2022). Genetics of ANCA-associated vasculitis: role in pathogenesis, classification and management. Nature Reviews. Rheumatology, 18(10), 559–574. https://doi.org/10.1038/s41584-022-00819-y
Derechos de autor 2026 Jerson Zuñiga Pacheco, Emily Carpio Moya, Naomi Ordoñez Calderon, Martha García Montiel, Sara Barahona Vizueta
Esta obra está bajo licencia internacional Creative Commons Reconocimiento 4.0.
.png)
.png)
1.png)
