A single-center COVID-19 vaccine experience with CoronaVac and BNT162b2 in familial Mediterranean fever patients.

AIM: To determine frequency of adverse events and attacks related to vaccination in recipients of CoronaVac and BNT162b2 in familial Mediterranean fever (FMF) patients, and to search whether history of prior COVID-19 or a booster dose increases occurrence of adverse events/attacks. METHODS: FMF patients were surveyed for administration of any COVID-19 vaccine and vaccine-related adverse events or FMF attacks. Demographic, clinical, vaccine-related data, history of COVID-19 infection before or after vaccination, adherence to FMF treatment during vaccination were collected. RESULTS: A total of 161 vaccinated FMF patients were included. Ninety-three patients out of 161 had reported suffering from an adverse event/attack after a vaccine dose. There were 54.7% of BNT162b2 recipients who reported any adverse event after any vaccine dose in comparison to 29.9% of CoronaVac recipients (P < .001). There were 22.2% of BNT162b2 recipients who reported suffering from a FMF attack within 1 month after vaccination in comparison to 19.4% of CoronaVac recipients (P = .653). When patients with or without adverse event/attack were compared, no significant differences were observed in means of demographics, comorbid diseases, disease duration, total vaccine doses, or treatments adhered to for FMF. Rates of adverse events/attacks were similar between patients with and without prior COVID-19. In booster recipients, adverse events/attacks were most frequent after the booster dose. CONCLUSIONS: A considerable number of FMF patients suffered from vaccine-related adverse events/attacks, particularly with BNT162b2. No serious events or mortalities due to vaccination were detected. Demographics, clinical characteristics and prior history of vaccination did not significantly affect these results. We observed an increased rate of adverse events/attacks with booster dose administration.

Clinical course of COVID-19 infection in paediatric familial Mediterranean fever patients.

OBJECTIVE: To evaluate the course of coronavirus-19 (COVID-19) infection in paediatric familial Mediterranean fever (FMF) patients and to investigate the risk factors for COVID-19 infection. METHODS: Medical records of 100 consecutive paediatric FMF patients and their COVID-19 infection status were evaluated. Age- and gender-matched control group consisted of 51 patients with positive results for severe acute respiratory syndrome coronavirus 2. RESULTS: Twenty-five of 100 paediatric FMF patients were detected to have COVID-19 infection. A history of contact with a COVID-19 case was present in ∼95% of patients in both the FMF and control groups with COVID-19 infection. Asymptomatic infection was detected in two patients in the paediatric FMF group (8.0%) and 17 patients in the control group (33.3%) (P = .017). Mild disease was observed in 23 paediatric FMF patients (92.0%) and 28 control patients (54.9%) (P = .001), whereas moderate disease was present in only 6 control patients (11.7%) (0 vs 11.7%, P = .074). Severe or critical disease was not observed in any patients. CONCLUSION: Paediatric FMF patients receiving colchicine had no moderate COVID-19 disease compared to the control group. We suggest that colchicine use may tune down the severity of the disease even if it does not prevent COVID-19 infection.

Challenges in diagnosing COVID-19 related disease in pediatric patients with rheumatic disease.

OBJECTIVES: Multisystem inflammatory syndrome in children (MIS-C) is a rare but severe condition associated with coronavirus disease 2019. Here we aimed to raise awareness for the symptoms of MIS-C in patients with rheumatic diseases, emphasizing the challenges of the differential features. METHODS: We retrospectively evaluated the demographic and clinical characteristics, laboratory and imaging findings, treatments, and outcomes of six MIS-C patients with previous rheumatic disease. RESULTS: Three of the patients had familial Mediterranean fever (FMF), one had juvenile dermatomyositis, one had systemic juvenile idiopathic arthritis (JIA), and another patient had oligoarticular JIA. All FMF patients presented with fever and abdominal pain, two also had chest pain. The patient with systemic JIA presented with fever, rash, and myalgia. All patients had elevated inflammatory markers and high d-dimer levels. Chest imaging of two FMF patients showed infiltrations compatible with pneumonia. One FMF patient had mildly decreased systolic functions with a shortening fraction of 48% in his echocardiography. Intravenous immunoglobulin and methylprednisolone were administered to all patients. Anakinra was given to four patients. CONCLUSIONS: Clinical and laboratory signs of MIS-C may overlap with the findings of various rheumatic diseases, and this may cause a delay in diagnosis.

Incidence and course of COVID-19 hospitalizations among patients with familial Mediterranean fever.

OBJECTIVES: To evaluate the incidence of hospitalization for coronavirus disease 2019 (COVID-19) in patients with FMF, as compared with the general population, and to compare the disease course between FMF inpatients, and age-, sex-, ethnicity- and comorbidity-matched non-FMF COVID-19 inpatients. METHODS: We used electronic medical records to obtain data about the total number of the insured population and the number of FMF patients in the two largest health management organizations in Jerusalem, Clalit and Meuhedet. The total number of COVID-19 inpatients at the Hadassah Medical Center, including those with FMF, for the period between 1 February 2020 and 10March 2021, was retrieved from the electronic medical records of Hadassah. COVID-19 course was compared between the FMF inpatient group and age-, sex-, ethnicity- and comorbidity-matched non-FMF COVID-19 inpatients. Each FMF inpatient was matched with two non-FMF controls. RESULTS: We found no statistically significant difference in the odds of hospitalization for COVID-19 between FMF patients and the non-FMF population (0.46% vs 0.41%, P = 0.73). Furthermore, we found similar disease severity and therapeutic approach in FMF COVID-19 inpatients and matched non-FMF COVID-19 inpatients. CONCLUSIONS: Neither FMF nor baseline colchicine therapy, appear to affect the incidence of hospitalization for COVID-19 or the disease course, in terms of severity and therapeutic approach.

The impact of COVID-19 on familial Mediterranean fever: a nationwide study.

The study aimed to evaluate the impact of the coronavirus disease 2019 (COVID-19) in patients with familial Mediterranean fever (FMF) and to assess the relationships between FMF characteristics and severe COVID-19 outcomes such as hospitalization. The study was planned within a national network of 21 different centers. Demographics, FMF-related clinical and genetic characteristics, and COVID-19 outcomes were obtained. A total of 822 patients with FMF (mean age of 36 years) were included in the study. Fifty-nine of them (7%) had a COVID-19 diagnosis confirmed by real-time PCR test or chest CT findings. Most FMF patients with COVID-19 (58) had mild and moderate disease activity. All patients were on colchicine treatment. However, 8 of them (13.6%) were not compliant with colchicine use and 9 of them (15.3%) were colchicine resistant. Twelve FMF patients with COVID-19 were hospitalized. There were 4 patients requiring oxygen support. COVID-19 related complications were observed in 2 patients (1 thromboembolism, 1 acute respiratory distress syndrome). Hospitalized COVID-19 patients with FMF were older than non-hospitalized patients (median ages: 51 and 31 years, respectively; p: 0.002). Other FMF-related characteristics were similar between the groups. FMF-related characteristics were not found to be associated with poor outcomes in COVID-19. Thus, FMF may not be a risk factor for poor COVID-19 outcomes.

COVID-19 in patients with familial Mediterranean fever treated with colchicine: case based review.

Coronavirus disease 2019 (COVID-19) refers to the clinical picture of an important and severe infectious disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Considering the current knowledge on the pathophysiology and clinical manifestations of COVID-19, it is safe to state that both COVID-19 and inflammatory rheumatic disorders cause a cytokine storm and merit treatment with anti-rheumatic drugs. Three patients, who were on regular follow-up due to the diagnosis of familial Mediterranean fever (FMF), contracted COVID-19 infection; and their pre-clinical and post-clinical data as well as laboratory, prognosis and treatment data were investigated. Effects of colchicine in FMF patients who contracted COVID-19 infection were presented in this study. All the cases recovered from COVID-19 without complications. The present study suggests that colchicine can positively affect the prognosis of COVID-19 in FMF patients; therefore, experience of rheumatologists in the use of anti-inflammatory drugs can be highly instrumental in management of COVID-19 patients.

COVID-19 outcomes in patients with familial Mediterranean fever: a retrospective cohort study.

Aim of this study is to investigate the course of coronavirus disease 2019 (COVID-19), in our cohort of familial Mediterranean fever (FMF) patients in means of mortality, admission to hospital and/or intensive care unit and length of hospital stay.A retrospective cohort was formed from patients who have previously been followed with a diagnosis of FMF. Patients of this cohort were retrospectively evaluated for a positive severe acute respiratory syndrome-coronavirus 2 (SARS-CoV 2) polymerized chain reaction (PCR) test result and information regarding hospitalisation, intensive care unit admission and mortality were collected from medical records.Out of a total 496 FMF patients, 34 were detected to have a positive SARS-CoV 2 PCR test. Eighty-five point three percent of these patients were under colchicine treatment and 17.6% were under interleukin (IL)-1 inhibitor treatment. Eight of the 34 patients (23.9%) were found to be hospitalized, one of them was admitted to the intensive care unit and died thereafter (2.9%). An increasing trend in the frequency of comorbid diseases (presence of at least one comorbidity 64.7% in all patients vs 75.0% in hospitalized patients) and IL-1 inhibitor usage (17.6% in all patients vs 50.0% in hospitalized patients) was observed in hospitalized patients.Rates of comorbid diseases and IL-1 inhibitor use for FMF were observed to be increased in FMF patients hospitalized for COVID-19.

Familial Mediterranean Fever and COVID-19: Friends or Foes?

Familial Mediterranean Fever (FMF) and COVID-19 show a remarkable overlap of clinical symptoms and similar laboratory findings. Both are characterized by fever, abdominal/chest pain, elevation of C-reactive protein, and leukocytosis. In addition, colchicine and IL-1 inhibitors treatments that are effective in controlling inflammation in FMF patients have recently been proposed for off-label use in COVID-19 patients. Thus, FMF may resemble a milder recapitulation of the cytokine storm that is a hallmark of COVID-19 patients progressing to severe disease. We analyzed the sequence of the MEFV-encoded Pyrin protein - whose mutations cause FMF- in mammals, bats and pangolin. Intriguingly, although Pyrin is extremely conserved in species that are considered either a reservoir or intermediate hosts for SARS-CoV-2, some of the most common FMF-causing variants in humans are present as wildtype residues in these species. We propose that in humans, Pyrin may have evolved to fight highly pathogenic infections.

Management of childhood-onset autoinflammatory diseases during the COVID-19 pandemic.

Concerns regarding the comorbidity as a significant risk factor for Coronavirus Disease-2019 (COVID-19), gave rise to an urgent need for studies evaluating patients with chronic conditions such as autoinflammatory diseases (AIDs). We prepared a web-based survey investigating the clinical findings and contact histories among pediatric patients with AIDs. Confirmed COVID-19 cases, patients with contact history and those with symptoms which were highly suggestive of COVID-19 were called via phone or recruited to a video or face to face appointment. Data of AIDs were obtained from their medical records, retrospectively. Laboratory and screening findings were confirmed by our national health registry website. There were 404 patients (217 female) eligible for the enrollment. During pandemic, 375 (93%) were on colchicine treatment and 48 (11.8%) were receiving biologic treatment. Twenty-four out of 404 patients were admitted to hospital due to COVID-19 suspicion. Severe acute respiratory syndrome coronavirus-2 (SARS CoV-2) was identified through rhinopharyngeal swabs in seven patients, six of whom were only on colchicine treatment. Only one patient with no finding of any severe respiratory complications was hospitalized. All of seven patients recovered completely. Among patients on biologic drugs, neither a symptom nor a positive polymerase chain reaction test for COVID 19 was detected. In conclusion, pediatric patients with AIDs, those receiving biologic treatment and/or colchicine, may not be at increased risk for neither being infected nor the severe disease course.