Laure Delaval1, Maxime Samson2, Flora Schein3, Christian Agard4, Ludovic Tréfond5, Alban Deroux6, Henry Dupuy7, Cyril Garrouste5, Pascal Godmer8, Cédric Landron9, François Maurier10, Guillaume le Guenno5, Virginie Rieu5, Julien Desblache11, Cécile-Audrey Durel12, Laurence Jousselin-Mahr13, Hassan Kassem14, Grégory Pugnet15, Vivane Queyrel16, Laure Swiader17, Daniel Blockmans18, Karim Sacré19, Estibaliz Lazaro7, Luc Mouthon20, Olivier Aumaître5, Pascal Cathébras3, Loic Guillevin20, Benjamin Terrier20. 1. Centre de Référence des Maladies Auto-Immunes Systémiques Rares d'Ile-de-France, Hôpital Cochin, Paris, France. 2. Centre Hospitalier Universitaire (CHU) de Dijon Bourgogne, Dijon, France. 3. Hôpital Nord, Saint-Étienne, France. 4. CHU de Nantes, Nantes, France. 5. CHU de Clermont-Ferrand, Clermont-Ferrand, France. 6. CHU de Grenoble Alpes, Grenoble, France. 7. Hôpital Haut-Lévêque, Pessac, France. 8. Centre Hospitalier Bretagne Atlantique, Vannes, France. 9. CHU de Poitiers, Poitiers, France. 10. Hôpitaux Privés de Metz, Metz, France. 11. Centre Hospitalier de Pau, Pau, France. 12. Hôpital Edouard Herriot and Hospices Civiles de Lyon, Lyon, France. 13. Hôpital St. Antoine, AP-HP, Paris, France. 14. Centre Hospitalier Sud Essonne Dourdan-Etampes, Dourdan, France. 15. CHU de Toulouse, Toulouse, France. 16. Hôpital Pasteur 2, Nice, France. 17. CHU de Marseille, Hôpital de la Timone, Marseille, France. 18. University Hospital Gasthuisberg, Leuven, Belgium. 19. Hôpital Bichat-Claude Bernard, AP-HP, Paris, France. 20. Centre de Référence des Maladies Auto-Immunes Systémiques Rares d'Ile-de-France, Hôpital Cochin, and Université Paris Descartes, Paris, France.
Abstract
OBJECTIVE: Temporal arteritis (TA) is a typical manifestation of giant cell arteritis (GCA). Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are rarely revealed by TA manifestations, leading to a risk of misdiagnosis of GCA and inappropriate treatments. This study was undertaken to describe the clinical, biologic, and histologic presentations and outcomes in cases of TA revealing AAV (TA-AAV) compared to controls with classic GCA. METHODS: In this retrospective case-control study, the characteristics of patients with TA-AAV were compared to those of control subjects with classic GCA. Log-rank test, with hazard ratios (HRs) and 95% confidence intervals (95% CIs), was used to assess the risk of treatment failure. RESULTS: Fifty patients with TA-AAV (median age 70 years) were included. Thirty-three patients (66%) presented with atypical symptoms of GCA (ear, nose, and throat involvement in 32% of patients, and renal, pulmonary, and neurologic involvement in 26%, 20%, and 16% of patients, respectively). Blood samples were screened for ANCAs at the time of disease onset in 33 patients, and results were positive in 88%, leading to a diagnosis of early TA-AAV in 20 patients. The diagnosis of AAV was delayed a median interval of 15 months in 30 patients. Compared to controls with GCA, patients with TA-AAV were younger (median age 70 years versus 74 years), were more frequently men (48% versus 30%), and had high frequencies of atypical manifestations and higher C-reactive protein levels (median 10.8 mg/dl versus 7.0 mg/dl). In patients with TA-AAV, temporal artery biopsy (TAB) showed fibrinoid necrosis and small branch vasculitis in 23% of patients each, whereas neither of these characteristics was evident in controls with GCA. Treatment failure-free survival was comparable between early TA-AAV cases and GCA controls, whereas those with delayed TA-AAV had a significantly higher risk of treatment failure compared to controls (HR 3.85, 95% CI 1.97-7.51; P < 0.0001). CONCLUSION: TA-AAV should be considered diagnostically in cases of atypical manifestations of GCA, refractoriness to glucocorticoid treatment, or early relapse. Analysis of TAB specimens for the detection of small branch vasculitis and/or fibrinoid necrosis could be useful. Detection of ANCAs should be performed in cases of suspected GCA with atypical clinical features and/or evidence of temporal artery abnormalities on TAB.
OBJECTIVE:Temporal arteritis (TA) is a typical manifestation of giant cell arteritis (GCA). Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are rarely revealed by TA manifestations, leading to a risk of misdiagnosis of GCA and inappropriate treatments. This study was undertaken to describe the clinical, biologic, and histologic presentations and outcomes in cases of TA revealing AAV (TA-AAV) compared to controls with classic GCA. METHODS: In this retrospective case-control study, the characteristics of patients with TA-AAV were compared to those of control subjects with classic GCA. Log-rank test, with hazard ratios (HRs) and 95% confidence intervals (95% CIs), was used to assess the risk of treatment failure. RESULTS: Fifty patients with TA-AAV (median age 70 years) were included. Thirty-three patients (66%) presented with atypical symptoms of GCA (ear, nose, and throat involvement in 32% of patients, and renal, pulmonary, and neurologic involvement in 26%, 20%, and 16% of patients, respectively). Blood samples were screened for ANCAs at the time of disease onset in 33 patients, and results were positive in 88%, leading to a diagnosis of early TA-AAV in 20 patients. The diagnosis of AAV was delayed a median interval of 15 months in 30 patients. Compared to controls with GCA, patients with TA-AAV were younger (median age 70 years versus 74 years), were more frequently men (48% versus 30%), and had high frequencies of atypical manifestations and higher C-reactive protein levels (median 10.8 mg/dl versus 7.0 mg/dl). In patients with TA-AAV, temporal artery biopsy (TAB) showed fibrinoid necrosis and small branch vasculitis in 23% of patients each, whereas neither of these characteristics was evident in controls with GCA. Treatment failure-free survival was comparable between early TA-AAV cases and GCA controls, whereas those with delayed TA-AAV had a significantly higher risk of treatment failure compared to controls (HR 3.85, 95% CI 1.97-7.51; P < 0.0001). CONCLUSION:TA-AAV should be considered diagnostically in cases of atypical manifestations of GCA, refractoriness to glucocorticoid treatment, or early relapse. Analysis of TAB specimens for the detection of small branch vasculitis and/or fibrinoid necrosis could be useful. Detection of ANCAs should be performed in cases of suspected GCA with atypical clinical features and/or evidence of temporal artery abnormalities on TAB.
Authors: Hélène Greigert; André Ramon; Georges Tarris; Laurent Martin; Bernard Bonnotte; Maxime Samson Journal: J Clin Med Date: 2022-01-05 Impact factor: 4.241
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Authors: David Faz-Muñoz; Andrea Hinojosa-Azaola; Juan M Mejía-Vilet; Norma O Uribe-Uribe; Marina Rull-Gabayet; Wallace Rafael Muñoz-Castañeda; Nancy Janeth Vargas-Parra; Eduardo Martín-Nares Journal: Immunol Res Date: 2022-04-21 Impact factor: 4.505