POS1193 CLINICAL FEATURES AND THE COURSE OF COVID-19 IN PATIENTS WITH FAMILIAL MEDITERRANEAN FEVER
Authors: A. Avanoglu Guler, T. Yüce İnel, H. Karadeniz, R. Bilici Salman, H. Satiş, H. Küçük, M. A. Ozturk, B. Goker, S. Haznedaroglu, İ. Sari, T. Kasifoglu, A. Tufan
Journal: Annals of the Rheumatic Diseases (2021)

Background: The novel coronavirus 2019 (COVID-19) has a wide range of clinical presentation from asymptomatic or mild viral infection to severe life-threating complications, including acute respiratory distress syndrome (ARDS), which develop as a result of immune system dysregulation, exaggerated immune response, and cytokine release syndrome [1]. Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disorder characterized by dysfunction of the innate immune system and excessive production of proinflammatory cytokines, including interleukin (IL)-1β, IL-6, interferon-gamma, and tumor necrosis factor-alpha, all of which have increased in severe cases in COVID-19 [1-3]. Objectives: The aim of this study is to report clinical characteristics and outcome of FMF patients with COVID-19. Methods: This study included 48 consecutive FMF patients who were diagnosed COVID-19 by SARS-CoV-2 nucleic acid RT-PCR in nasopharyngeal swab or sputum, or symptoms and computed tomography findings suggestive for COVID-19. Data on demographic and clinical characteristics of FMF disease, clinical course and outcome of COVID-19 were evaluated. Results: The median age of patients was 35.5 (29-43.5) years, ranging from 18 to 87 years. The median disease duration of FMF was 10 (6-16) years. The most common presenting symptoms and signs of patients during attacks were peritonitis (85.5%), fever (81.3%), and pleuritis (48%). Twelve (25%) patients had amyloidosis. Comorbidities were present in half of patients with ankylosing spondylitis (21%) and hypertension (17%) being the most frequently seen. Two-third of patients (66%) were in remission for FMF. The median dosage of colchicine treatment for FMF was 1.5 (IQR 1) mg/day. 90% of patients continued colchicine treatment for FMF during the COVID-19 course. The baseline characteristics and treatment modalities of patients were demonstrated in Table 1. Forty-six patients presented with at least one COVID-19 symptoms. Fever (73%), myalgia/arthralgia (69%), and cough (60%) were the most common symptoms. 16 patients were admitted at hospital, 5 of them required oxygen therapy and 2 patients with amyloidosis developed ARDS and went to intensive care unit (ICU) for invasive mechanical ventilation (IMV). One patient who had been followed-up at ICU died. Conclusion: Our FMF patients with COVID-19 have similar clinical features and outcomes as general population. Dysregulation of innate immune system in FMF might not be risk factors for COVID-19. Besides, colchicine and IL-1 inhibitors intake might have protective and preventive effects on COVID-19 progression. Table 1. Baseline characteristics, treatment modalities and outcome of patients Age years, median (IQR ) 39.4 (15) Gender (F/M ) 25/23 FMF Disease duration, years, median (IQR ) 10 (10) Mutations* Monoallelic mutation, n (%) 6 (14) Biallelic mutation, n (%) 34 (81) Homozygous M694V mutation 18 (43) Heterozygous M694V mutation 15 (36) Comorbidities, n (% ) 24 (50) FMF treatment Colchicine 1 mg/day, n (%) 17 (35.5) Colchicine 1.5 mg/day, n (%) 18 (37.5) Colchicine 2 mg/day, n (%) 13 (27) Anakinra, n (%) 12 (25) Canakinumab, n (%) 3 (6.3) Positive SARS-Cov-2 RT-PCR, n (% ) 42 (87.5) Interstitial pneumonia in CT scan, n (% ) 15 (31.3) COVID-19 treatment, n (% ) 46 (95.8) Outpatient treatment, n (% ) 32 (66.7) Hospitalized, not required supplemental oxygen, n (% ) 9 (18.8) Hospitalized, required supplemental oxygen, n (% ) 5 (10.4) ICU, required IMV, n (% ) 2 (4.2) Outcome Recovered, n (% ) 47 (98) Deceased, n (% ) 1 (2) Complications, n (% ) 3 (6.3) *42 patients were included in the analysis References: [1]Tufan A, Avanoğlu Güler A, Matucci-Cerinic M. COVID-19, immune system response, hyperinflammation and repurposing antirheumatic drugs. Turkish journal of medical sciences. 2020;50:620-632. [2]Hausmann JS. Targeting cytokines to treat autoinflammatory diseases. Clinical immunology (Orlando, Fla.). 2019;206:23-32. [3]Tufan A, Lachmann HJ. Familial Mediterranean fever, from pathogenesis to treatment: a contemporary review. Turkish journal of medical sciences. 2020;50:1591-1610. Disclosure of Interests: None declared.