Acute pericarditis with pleuropulmonary involvement, fever and elevated C-reactive protein: A systemic autoinflammatory disease? A cohort study

• Published in: European journal of internal medicine Vol. 113; pp. 45 - 48
• Main Authors: Pisacreta, Anna Maria, Mascolo, Ruggiero, Nivuori, Mariangela, Dominioni, Costanza Caccia, Gabiati, Claudia, Trotta, Lucia, Pancrazi, Massimo, Marco, Giacomo Di, Carollo, Chiara, Pedroli, Alice, Casarin, Francesca, Tombetti, Enrico, Bizzi, Emanuele, Imazio, Massimo, Brucato, Antonio
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.07.2023
• Subjects: Autoinflammatory diseases; C-Reactive Protein - analysis; Cohort Studies; Female; Fever; Hereditary Autoinflammatory Diseases - complications; Humans; IL-1; Neutrophil-to-lymphocyte ratio; Pericarditis; Pleural effusion; Pleural Effusion - complications; Pleuritis; Pleural effusion; Pericarditis; Autoinflammatory diseases; Neutrophil-to-lymphocyte ratio; IL-1; Pleuritis
• ISSN: 0953-6205; 1879-0828
• DOI: 10.1016/j.ejim.2023.03.034

•Aim of this study is to increase awareness in the scientific community for the earlier identification and differential diagnosis of pericarditis with systemic involvement, especially in the severe forms.•Acute pericarditis with fever, elevated CRP and particularly pleuropulmonary involvement shows strong similarities with systemic autoinflammatory diseases associated to inflammosome activation.•Neutrophilia, lymphopenia and high neutrophil/lymphocyte ratio are other features of this subset.•Correct recognition of this phenotype might help clinicians to reach appropriate diagnoses, avoiding delays, and on the other hand to individuate possible candidates for anti IL-1 therapies. This cohort study describes a systemic phenotype of pericarditis, comparing this phenotype with other forms of pericarditis. Patients in our center were enrolled in a prospectively maintained registry from 2019 to 2022. 412 patients with idiopathic recurrent pericarditis were analyzed. “Systemic inflammatory” subset was defined as the presence of all the following criteria: fever ≥38C°, CRP ≥2 times normal values, pleural effusion detected with any imaging techniques. The absence of any of the 3 criteria was defined as “isolated” subset. We found that 211 (51.2%) of 412 patients (188 female) presented the systemic subset and the variables significantly associated with this subset in univariate analysis (p<0.001) were: higher mean age: 45.5 (±SD 17.2) vs 39.9 (±SD 16.4) years, higher mean CRP values: 128.8 vs 49.9 mg/L, higher proportion of pericardiocentesis: 19% vs 1.5%, higher mean leukocyte count: 13,143.3 vs 9910.3/mm3, higher mean neutrophils number: 10,402.5 vs 6779.8 /mm3 and lower mean lymphocyte count: 1693.9 vs 2079.3 /mm3. As results the neutrophil-to-lymphocyte ratio was higher in systemic inflammatory phenotype: 6.6 vs 3.4 (p< 0.001). Anti-IL1 therapy was started more frequently in the systemic subgroup (26%) than in the isolated subset (7.5%) (p < 0.001). On multivariate analysis neutrophil count and lymphopenia were statistically associated with the systemic subset (p < 0.001). This results demonstrate the relevance of the systemic inflammatory phenotype, characterized by pleural effusions, confirming its analogy with autoinflammatory diseases, thus possibly requiring an eventual escalation of therapy to IL-1 inhibitors. [Display omitted]