Cerebral Amyloid Angiopathy-Related Inflammation and Biopsy-Positive Primary Angiitis of the CNS: A Comparative Study.
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Grangeon Lou, Boulouis Grégoire, Capron Jean, Bala Fouzi, Renard Dimitri, Raposo Nicolas, Ozkul-Wermester Ozlem, Triquenot-Bagan Aude, Ayrignac Xavier, Wallon David, Gerardin Emmanuel, Kerschen Philippe, Sablot Denis, Formaglio Maïté, Pico Fernando, Turc Guillaume, Verny Marc, Humbertjean Lisa, Gaudron Marie, Vannier Stéphane, Dequatre Nelly, Guillon Benoit, Isabel Clothilde, Arquizan Caroline, Detante Olivier, Godard Sophie, Casolla Barbara, Levraut Michael, Gollion Cédric, Gerfaud-Valentin Mathieu, Kremer Laurent, Daelman Laure, Lambert Nicolas, Lanthier Sylvain, Poppe Alexandre, Régent Alexis, Weisenburger-Lile David, Verdure Pierre, Quesney Gérald, Vautier Mathieu, Wacongne Anne, Thouvenot Eric, Pariente Jérémie, Coulette Sarah, Labauge Pierre M, Olivier Nadège, Allou Thibaut, Zephir Hélène, Néel Antoine, Bresch Saskia, Terrier Benjamin, Martinaud Olivier, Schneckenburger Romain, Papo Thomas, Comarmond-Ortoli Chloé, Jouvent Eric, Subréville Marie, Poncet-Megemont Louis, Khatib Muhammad Amer, Lun François, Henry Carole, Magnin Eloi, Thomas Quentin, Graber Mathilde, Boukriche Yassine, Blanchet-Fourcade Geneviève, Ratiu Diana, Pagnoux Christian, Touzé Emmanuel, de Boysson Hubert, Alamowitch Sonia, Nehme Ahmad
Аннотация
METHODS: Patients with CAA-RI and BP-PACNS were enrolled from 2 retrospective multicenter cohorts. Patients with CAA-RI were biopsy-positive or met probable clinicoradiologic criteria. Patients with BP-PACNS had histopathologic confirmation of CNS angiitis, with no secondary etiology. A neuroradiologist read brain MRIs, blinded to the diagnosis of CAA-RI or BP-PACNS. Clinicoradiologic features were compared using univariable logistic regression models. Relapse rates were compared using a univariable Fine-Gray subdistribution hazard model, with death as a competing risk.
RESULTS: This study enrolled 104 patients with CAA-RI (mean age 73 years, 48% female sex) and 52 patients with BP-PACNS (mean age 45 years, 48% female sex). Patients with CAA-RI more often had white matter hyperintense lesions meeting the probable CAA-RI criteria (93% vs 51%, p < 0.001), acute subarachnoid hemorrhage (15% vs 2%, p = 0.02), cortical superficial siderosis (27% vs 4%, p < 0.001), ≥1 lobar microbleed (94% vs 26%, p < 0.001), past intracerebral hemorrhage (17% vs 4%, p = 0.04), ≥21 visible centrum semiovale perivascular spaces (34% vs 4%, p < 0.01), and leptomeningeal enhancement (70% vs 27%, p < 0.001). Patients with BP-PACNS more often had headaches (56% vs 31%, p < 0.01), motor deficits (56% vs 36%, p = 0.02), and nonischemic parenchymal gadolinium enhancement (82% vs 16%, p < 0.001). The prevalence of acute ischemic lesions was 18% in CAA-RI and 22% in BP-PACNS (p = 0.57). The features with the highest specificity for CAA-RI were acute subarachnoid hemorrhage (98%), cortical superficial siderosis (96%), past intracerebral hemorrhage (96%), and ≥21 visible centrum semiovale perivascular spaces (96%). The probable CAA-RI criteria had a 71% sensitivity (95% CI 44%-90%) and 91% specificity (95% CI 79%-98%) in differentiating biopsy-positive CAA-RI from BP-PACNS. The rate of relapse in the first 2 years after remission was lower in CAA-RI than in BP-PACNS (hazard ratio 0.46, 95% CI 0.22-0.96, p = 0.04).
CONCLUSION: Clinicoradiologic features differed between patients with CAA-RI and those with BP-PACNS. Specific markers for CAA-RI were hemorrhagic signs of subarachnoid involvement, past intracerebral hemorrhage, ≥21 visible centrum semiovale perivascular spaces, and the probable CAA-RI criteria. A biopsy remains necessary for diagnosis in some cases of CAA-RI. The rate of relapse in the first 2 years after disease remission was lower in CAA-RI than in BP-PACNS.