PMID- 31385079 OWN - NLM STAT- MEDLINE DCOM- 20210209 LR - 20210209 IS - 1437-160X (Electronic) IS - 0172-8172 (Linking) VI - 40 IP - 2 DP - 2020 Feb TI - The complexity of classifying ANCA-associated small-vessel vasculitis in actual clinical practice: data from a multicenter retrospective survey. PG - 303-311 LID - 10.1007/s00296-019-04406-5 [doi] AB - The different sets of criteria for diagnosis or classification of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) lead to numerous overlapping and reclassified diagnoses in clinical practice. We designed this study to assess the difficulties in classifying patients with AAV. As a secondary objective, different variables were tested to predict prognosis. We conducted a retrospective chart review in a Western Spain multicentre survey. A total of 115 adult patients diagnosed with AAV from 2002 to 2013 and followed for at least 3 years were included. They were classified according to (1) Chapel Hill Consensus Conference (CHCC), (2) European Medicines Agency algorithm and (3) French Vasculitis Study Group/European Vasculitis Society phenotypes. Fifty-three patients (46%) had neither distinctive histopathological data of a single AAV definition nor any surrogate markers for granulomatous inflammation and thus did not fulfill any diagnostic criteria. Ocular, ear, nose, throat, skin, and lung involvement were more frequent with proteinase 3 (PR3) antibodies, whereas peripheral neuropathy was more frequent with myeloperoxidase (MPO) antibodies. When the disease was severe at diagnosis, the HR for mortality was 10.44. When induction treatment was not given in accordance with the guidelines, the HR for mortality was 4.00. For maintenance treatment, the HR was 5.49 for mortality and 2.48 for relapse. AAV classification is difficult because many patients had neither specific clinical data nor distinctive histological features of a single CHCC definition. A structured clinical assessment of patient severity is the best tool to guide the management of AAV. FAU - Corral-Gudino, Luis AU - Corral-Gudino L AUID- ORCID: 0000-0003-0151-5420 AD - Internal Medicine Department, Hospital El Bierzo, Ponferrada, Leon, Spain. lcorral@saludcastillayleon.es. AD - Internal Medicine Department, Hospital Universitario Rio Hortega (HURH), Calle Dulzaina, 2, 47012, Valladolid, Spain. lcorral@saludcastillayleon.es. FAU - Gonzalez-Vazquez, Elvira AU - Gonzalez-Vazquez E AUID- ORCID: 0000-0002-3362-6451 AD - Internal Medicine Department, Complejo Hospitalario de Ourense, Ourense, Spain. FAU - Calero-Paniagua, Ismael AU - Calero-Paniagua I AUID- ORCID: 0000-0003-0553-6157 AD - Internal Medicine Department, Hospital Virgen de la Luz, Cuenca, Spain. FAU - Perez-Garrido, Laura AU - Perez-Garrido L AUID- ORCID: 0000-0003-1756-3507 AD - Service of Rheumatology, Hospital Universitario de Salamanca, Salamanca, Spain. FAU - Cusacovich, Ivan AU - Cusacovich I AUID- ORCID: 0000-0002-4984-0639 AD - Internal Medicine Department, Hospital Universitario de Burgos, Burgos, Spain. FAU - Rivas-Lamazares, Alicia AU - Rivas-Lamazares A AUID- ORCID: 0000-0001-7301-7637 AD - Internal Medicine Department, Hospital El Bierzo, Ponferrada, Leon, Spain. FAU - Quesada-Moreno, Alba AU - Quesada-Moreno A AUID- ORCID: 0000-0002-6648-3645 AD - Service of Rheumatology, Hospital Universitario de Salamanca, Salamanca, Spain. FAU - Gonzalez-Fernandez, Ana AU - Gonzalez-Fernandez A AUID- ORCID: 0000-0001-5872-0594 AD - Internal Medicine Department, Hospital El Bierzo, Ponferrada, Leon, Spain. FAU - Mora-Pena, Damian AU - Mora-Pena D AUID- ORCID: 0000-0001-5113-5077 AD - Internal Medicine Department, Hospital Virgen de la Luz, Cuenca, Spain. FAU - Lerma-Marquez, Jose Luis AU - Lerma-Marquez JL AUID- ORCID: 0000-0002-5054-0211 AD - Service of Nephrology, Hospital Universitario de Salamanca, Salamanca, Spain. FAU - Del-Pino-Montes, Javier AU - Del-Pino-Montes J AUID- ORCID: 0000-0002-3145-6137 AD - Service of Rheumatology, Hospital Universitario de Salamanca, Salamanca, Spain. LA - eng GR - BIO/SA/12/13/SACYL NHS/ PT - Journal Article PT - Multicenter Study DEP - 20190805 PL - Germany TA - Rheumatol Int JT - Rheumatology international JID - 8206885 RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - EC 1.11.1.7 (Peroxidase) RN - EC 3.4.21.76 (Myeloblastin) SB - IM MH - Aged MH - Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/*classification/immunology/*pathology/*physiopathology MH - Antibodies, Antineutrophil Cytoplasmic/immunology MH - Churg-Strauss Syndrome/classification/immunology/pathology/physiopathology MH - Epistaxis/immunology/pathology/physiopathology MH - Eye Diseases/immunology/pathology/physiopathology MH - Female MH - Gastrointestinal Diseases/immunology/pathology/physiopathology MH - Granulomatosis with Polyangiitis/classification/immunology/pathology/physiopathology MH - Humans MH - Hypertension/immunology/pathology/physiopathology MH - Kidney Diseases/immunology/pathology/physiopathology MH - Kidney Failure, Chronic/physiopathology MH - Lung Diseases/immunology/pathology/physiopathology MH - Male MH - Microscopic Polyangiitis/classification/immunology/pathology/physiopathology MH - Middle Aged MH - *Mortality MH - Myeloblastin/immunology MH - Peripheral Nervous System Diseases/immunology/pathology/physiopathology MH - Peroxidase/immunology MH - Primary Prevention MH - Prognosis MH - Proportional Hazards Models MH - Recurrence MH - Retrospective Studies MH - Severity of Illness Index MH - Sinusitis/immunology/pathology/physiopathology MH - Skin Diseases/immunology/pathology/physiopathology OTO - NOTNLM OT - Anti-neutrophil cytoplasmic antibody-associated vasculitis OT - Classification OT - Eosinophilic granulomatous with polyangiitis (Churg-Strauss) OT - Granulomatosis with polyangiitis OT - Microscopic polyangiitis OT - Prognosis EDAT- 2019/08/07 06:00 MHDA- 2021/02/10 06:00 CRDT- 2019/08/07 06:00 PHST- 2019/05/18 00:00 [received] PHST- 2019/07/29 00:00 [accepted] PHST- 2019/08/07 06:00 [pubmed] PHST- 2021/02/10 06:00 [medline] PHST- 2019/08/07 06:00 [entrez] AID - 10.1007/s00296-019-04406-5 [pii] AID - 10.1007/s00296-019-04406-5 [doi] PST - ppublish SO - Rheumatol Int. 2020 Feb;40(2):303-311. doi: 10.1007/s00296-019-04406-5. Epub 2019 Aug 5.