ABSTRACT
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.
Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/classification , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/pathology , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/physiopathology , Mortality , Aged , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/immunology , Antibodies, Antineutrophil Cytoplasmic/immunology , Churg-Strauss Syndrome/classification , Churg-Strauss Syndrome/immunology , Churg-Strauss Syndrome/pathology , Churg-Strauss Syndrome/physiopathology , Epistaxis/immunology , Epistaxis/pathology , Epistaxis/physiopathology , Eye Diseases/immunology , Eye Diseases/pathology , Eye Diseases/physiopathology , Female , Gastrointestinal Diseases/immunology , Gastrointestinal Diseases/pathology , Gastrointestinal Diseases/physiopathology , Granulomatosis with Polyangiitis/classification , Granulomatosis with Polyangiitis/immunology , Granulomatosis with Polyangiitis/pathology , Granulomatosis with Polyangiitis/physiopathology , Humans , Hypertension/immunology , Hypertension/pathology , Hypertension/physiopathology , Kidney Diseases/immunology , Kidney Diseases/pathology , Kidney Diseases/physiopathology , Kidney Failure, Chronic/physiopathology , Lung Diseases/immunology , Lung Diseases/pathology , Lung Diseases/physiopathology , Male , Microscopic Polyangiitis/classification , Microscopic Polyangiitis/immunology , Microscopic Polyangiitis/pathology , Microscopic Polyangiitis/physiopathology , Middle Aged , Myeloblastin/immunology , Peripheral Nervous System Diseases/immunology , Peripheral Nervous System Diseases/pathology , Peripheral Nervous System Diseases/physiopathology , Peroxidase/immunology , Primary Prevention , Prognosis , Proportional Hazards Models , Recurrence , Retrospective Studies , Severity of Illness Index , Sinusitis/immunologyABSTRACT
No disponible
Subject(s)
Humans , Male , Middle Aged , Arthritis, Psoriatic/drug therapy , Ustekinumab/therapeutic use , Toe Joint/physiopathology , Arthritis, Psoriatic/complications , Nail Diseases/etiology , Arthralgia/drug therapyABSTRACT
El síndrome SAPHO (sinovitis, acné, pustulosis, hiperostosis y osteítis) comprende un conjunto de manifestaciones cutáneo-osteoarticulares. Se han descrito algunas complicaciones graves que pueden aparecer durante la evolución de la enfermedad, como la trombosis venosa, principalmente en pacientes que desarrollan afectación inflamatoria grave de la pared torácica anterior. El objetivo de la presente revisión fue analizar los casos descritos en la literatura médica relacionados con la presencia de complicaciones trombóticas en pacientes diagnosticados de síndrome SAPHO e intentar establecer los probables factores de riesgo y su posible mecanismo patogénico. Se analizaron 11 artículos publicados de casos clínicos aislados o series de casos, con un total de 144 pacientes, que describen en total 15 casos de trombosis venosa. Se exponen las características clínicas de estos pacientes, se evalúa si cumplen los criterios de clasificación ASAS para espondiloatritis axial y periférica, y se resalta la necesidad de realizar un diagnóstico y tratamiento precoces (AU)
SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a cluster of osteo-cutaneous manifestations that can lead to serious complications such as thrombosis of the subclavian vein or superior vena cava, mainly in patients with significant inflammatory involvement of the anterior-chest-wall. The objective of this study was to review the cases published in the medical literature related with the presence of thrombotic complications in patients diagnosed with SAPHO syndrome and to try to determine their possible pathogenic mechanism and risk factors. We analyzed 11 published reports of isolated clinical cases or case series, a total of 144 patients, which described a total of 15 cases of venous thrombosis. The clinical characteristics of these patients, evaluated to determine whether they meet the ASAS criteria for axial and peripheral spondyloarthritis, is analyzed the need for early diagnosis and treatment is highlighted (AU)
Subject(s)
Humans , Male , Female , Adolescent , Young Adult , Adult , Middle Aged , Acquired Hyperostosis Syndrome/complications , Acquired Hyperostosis Syndrome/epidemiology , Risk Factors , Venous Thrombosis/complications , Venous Thrombosis/therapy , Venous Thrombosis , Early Diagnosis , Anticoagulants/therapeutic use , Acquired Hyperostosis Syndrome/physiopathology , Acquired Hyperostosis Syndrome , Subclavian Vein/pathology , Subclavian Vein , Tomography, Emission-Computed , Radionuclide Imaging/methodsABSTRACT
SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a cluster of osteo-cutaneous manifestations that can lead to serious complications such as thrombosis of the subclavian vein or superior vena cava, mainly in patients with significant inflammatory involvement of the anterior-chest-wall. The objective of this study was to review the cases published in the medical literature related with the presence of thrombotic complications in patients diagnosed with SAPHO syndrome and to try to determine their possible pathogenic mechanism and risk factors. We analyzed 11 published reports of isolated clinical cases or case series, a total of 144 patients, which described a total of 15 cases of venous thrombosis. The clinical characteristics of these patients, evaluated to determine whether they meet the ASAS criteria for axial and peripheral spondyloarthritis, is analyzed the need for early diagnosis and treatment is highlighted.
