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Open Access 06.01.2024 | Brief Report

Characteristics of COVID-19 and Impact of Disease Activity in Patients with Adult-Onset Still’s Disease

verfasst von: Xinyue Hong, Xiaoming Wang, Ningqi Dai, Yue Sun, Honglei Liu, Xiaobing Cheng, Junna Ye, Hui Shi, Qiongyi Hu, Jianfen Meng, Zhuochao Zhou, Chengde Yang, Jialin Teng, Yutong Su, Huihui Chi

Erschienen in: Rheumatology and Therapy | Ausgabe 1/2024

Abstract

Introduction

This study aimed to characterize the morbidity, hospitalization, and mortality rates among patients with adult-onset Still’s disease (AOSD) affected by coronavirus disease 2019 (COVID-19) and explore the impact of COVID-19 on the disease activity of AOSD.

Methods

Data on the clinical and demographic characteristics, COVID-19-related symptoms, and outcomes were retrospectively collected. Patients were stratified according to COVID-19 severity and associations between risk factors and outcomes were analyzed using multivariate logistic regression. The disease activity of patients with AOSD flares after COVID-19 was described.

Results

A total of 188 patients with AOSD were followed up, of whom 75.5% (n = 142) had a confirmed or highly suspected COVID-19. Patients on medium or high-dose oral glucocorticoids or Janus kinase (JAK) inhibitors were at increased risk of developing moderate to severe COVID-19. Six patients suffered flares of AOSD following COVID-19 in a short period; however, the relapse rate was not statistically increased compared with patients without COVID-19.

Conclusion

Patients with AOSD receiving medium or high-dose glucocorticoid therapy or JAK inhibitors had worse COVID-19 outcomes. Further work is needed to explore risk factors affecting COVID-19 outcomes and the impact of COVID-19 on disease activity in AOSD.

Xinyue Hong, Xiaoming Wang, and Ningqi Dai contributed equally to this work.

Huihui Chi, Yutong Su, and Jialin Teng are co-corresponding authors.

Key Summary Points

Limited data exist regarding the effects of SARS-CoV-2 infection in patients with AOSD, and the association between risk factors and severe outcomes of COVID-19 remains unclear.

This study analyzed the factors associated with COVID-19 severity in patients with AOSD and described the impact of COVID-19 on disease activity.

COVID-19 outcomes were worse in patients with AOSD who received medium or high-dose glucocorticoids or Janus kinase (JAK) inhibitors, and age and comorbidities were also risk factors for more severe COVID-19 in patients with AOSD.

Further follow-up is required to observe whether SARS-CoV-2 infection increases the risk of AOSD flares.

Introduction

Adult-onset Still’s disease (AOSD) is an auto-inflammatory disease of unknown etiology, characterized by high fever, transient rash, leukocytosis, arthralgia, and arthritis. The pathogenesis is associated with the overactivation of macrophages and neutrophils by danger signals which activate the inflammasome, leading to the overproduction of pro-inflammatory cytokines [1]. Although the exact pathogenesis of AOSD remains largely unknown, viral or bacterial infections are considered potential triggers [2], and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may also be involved as pathogen-associated molecular patterns (PAMPs). With the coronavirus disease 2019 (COVID-19) outbreak, lots of people were infected worldwide. There are significant similarities between AOSD and COVID-19, including a range of clinical and laboratory features such as persistent fever, high serum ferritin, and the tendency to develop cytokine release syndrome [3]. The activation of NACHT, LRR, and PYD domains-containing protein 3 (NLRP3) was important in both diseases [1, 4]. There have been several case reports of AOSD following SARS-CoV-2 infection and vaccination [5, 6]. Thus, the excessive inflammatory responses that occur in patients with AOSD and patients with severe COVID-19 may share similar mechanisms.

In December 2022, following a change in relevant prevention policies in China, the population had a concentration of infections, mainly with the Omicron strain. Although several previous studies have assessed the effects of SARS-CoV-2 infection in rheumatic diseases, data from patients with AOSD are limited and it is unclear which risk factors are associated with severe outcomes of COVID-19 in this population. Following this outbreak of COVID-19, we retrospectively collected and analyzed clinical data on COVID-19 in patients with AOSD up to March 15, 2023. Our study aimed to describe the factors associated with COVID-19 severity in patients with AOSD and assess the impact of COVID-19 on disease activity.

Patients and Methods

Study Design and Population

We conducted a retrospective observational cohort study among patients with AOSD under long-term outpatient follow-up at the Department of Rheumatology and Immunology, Ruijin Hospital, China. The patients were diagnosed with AOSD according to the Yamaguchi criteria. The study was conducted between December 15, 2022 and March 15, 2023. As a result of the COVID-19 pandemic, patient data collection was conducted remotely using internet-based methods. Reputable online platforms and telecommunication tools were used to establish contact with patients and obtain their clinical information. Informed consent was obtained electronically, with detailed explanations of the study purpose, procedures, and confidentiality measures provided. Secure online data collection instruments, including web-based questionnaires and electronic medical record forms, were designed to capture relevant information while ensuring data reliability and accuracy. This study was approved by the Institutional Research Ethics Committee of Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, China with Institutional Review Board number KY2022-182, and was performed in compliance with the Declaration of Helsinki with written informed consent from the subjects.

Data Collection

Demographic data, clinical characteristics, treatments, and outcomes were collected. COVID-19 was confirmed as positive for SARS-CoV-2 by polymerase chain reaction (PCR) or antigen. COVID-19 was suspected if patients reported associated symptoms (new onset of fever and presence of at least one respiratory symptom) and a history of epidemiological exposure. Symptoms collected for COVID-19 included fever, sore throat, cough, expectoration, nasal congestion, runny nose, dyspnea, diarrhea, anosmia or ageusia, muscle soreness, conjunctivitis, and fatigue. The severity of COVID-19 was classified as mild (did not or mildly interfered with daily activity), moderate (severely interfered with daily activity), or severe (required urgent medical attention or hospitalized medications). Medium or high-dose oral glucocorticoids were defined as more than 7.5 mg/day [7].

The definition of AOSD flare includes the presence of at least two AOSD-related symptoms together with elevated levels of inflammatory indicators such as C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and ferritin and requiring medication adjustment [8]. The AOSD flare within 3 months after the infection of SARS-CoV-2 was considered a post-infection disease flare. The systemic score was assessed in all patients with disease flares. The score included 12 items: fever, typical rash, pleuritis, pneumonia, pericarditis, hepatomegaly or abnormal liver function tests, splenomegaly, lymphadenopathy, leukocytosis of more than 15,000/mm³, sore throat, myalgia, and abdominal pain.

Statistical Analysis

Medians (with interquartile range) or mean (with standard deviation) for continuous variables and frequencies (with proportions) for categorical variables were presented. Baseline characteristics and medication were summarized and stratified by COVID-19 severity. The relationship between each risk factor (comorbidities, vaccination, and medicine use) and COVID-19 severity was further assessed using logistic regression models after adjusting by age, gender, and body mass index (BMI). Binary covariates with a total sample size less than 5 were not included. Odds ratios (ORs) and 95% confidence intervals (95% CIs) were reported. A two-sided p value less than 0.05 was considered statistically significant. All analyses were performed using SPSS software (version 26.0).

Results

Baseline Characteristics

A total of 188 patients with AOSD were ultimately followed up, of whom 24.5% (n = 46) had no evidence of SARS-CoV-2 infection and 75.5% (n = 142) had confirmed or highly suspected COVID-19 (Fig. 1), similar to the infection rate of the general population [9]. Of the patients with COVID-19, the median age was 40.0 (32.0–51.0) years, 80.3% were female, and 50.7% of the patients had received SARS-CoV-2 vaccination (Table 1). Over half of the patients were treated with oral glucocorticoids or conventional synthetic disease-modifying antirheumatic drugs (DMARDs), including methotrexate, cyclosporine, hydroxychloroquine, and 11.3% (n = 16) of patients were treated with targeted synthetic DMARDs, mainly Janus kinase inhibitors (JAK inhibitors). Preexisting conditions included hypertension (14.1%), diabetes (8.5%), heart failure (1.4%), chronic obstructive pulmonary disease (COPD) or asthma (1.4%), chronic kidney disease (1.4%), and malignant tumor (0.7%). Fever and cough were the most common symptoms in patients with COVID-19, existing in 83.1% (n = 118) and 80.3% (n = 114) of patients, respectively.

Table 1

Comparison of baseline characteristics in patients with AOSD with confirmed or highly suspected COVID19

	Total (n = 142)	Mild^a (n = 131)	Moderate^b or severe^c (n = 11)	p value
Age, years	40.0 (32.0–51.0)	39.0 (31.0–50.0)	61.0 (49.0–72.0)	0.001
Female	114 (80.3)	104 (79.4)	10 (90.9)	0.598
BMI, kg/m²	22.9 ± 3.5	22.8 ± 3.4	23.7 ± 4.2	0.322
COVID-19 vaccination before the infection	72 (50.7)	69 (52.7)	3 (27.3)	0.192
1 dose	8 (5.6)	8 (6.1)	0
2 doses	24 (16.9)	22 (16.8)	2 (18.2)
3 doses	40 (28.2)	39 (29.8)	1 (9.1)
Inactivated vaccine	68 (47.9)	65 (49.6)	3 (27.3)
Adenovirus vaccine	1 (0.7)	1 (0.8)	0
Recombinant protein subunit vaccine	3 (2.1)	3 (2.3)	0
Interval between vaccination and COVID-19 infection^d, months	10.3 ± 6.8	10.5 ± 6.8	5.3 ± 3.5	0.136
Comorbidities
Hypertension	20 (14.1)	16 (12.2)	4 (36.4)	0.078
Diabetes mellitus	12 (8.5)	10 (7.6)	2 (18.2)	0.520
Heart failure	2 (1.4)	1 (0.8)	1 (9.1)	0.358
COPD or asthma	2 (1.4)	0	2 (18.2)	0.005
Chronic kidney disease	2 (1.4)	1 (0.8)	1 (9.1)	0.358
Malignant tumor	1 (0.7)	1 (0.8)	0	1.000
Smoking history	2 (1.4)	2 (1.5)	0	1.000
Pregnancy or lactation	2 (1.4)	2 (1.5)	0	1.000
Inactive AOSD before COVID-19^e	104 (73.2)	98 (74.8)	6 (54.5)	0.145
Systemic score^f	0.11 ± 0.31	0.09 ± 0.29	0.27 ± 0.47	0.061
Treatments
Drug withdrawal	62 (43.7)	60 (45.8)	2 (18.2)	0.145
Glucocorticoid therapy^g	57 (40.1)	51 (38.9)	6 (54.5)	0.310
Medium or high-dose glucocorticoid	24 (16.9)	20 (15.1)	4 (36.4)	0.169
Glucocorticoid dosage, mg/day	0 (0–5.0)	0 (0–5.0)	2.5 (0–17.5)	0.168
csDMARDs	67 (47.2)	59 (45.0)	8 (72.7)	0.146
b/tsDMARDs	16 (11.3)	14 (10.7)	2 (18.2)	0.796
Methotrexate	34 (23.9)	29 (22.1)	5 (45.5)	0.082
Cyclosporine	28 (19.7)	26 (19.8)	2 (18.2)	1.000
Hydroxychloroquine	29 (20.4)	26 (19.8)	3 (27.3)	0.844
JAK inhibitor	13 (9.2)	11 (8.4)	2 (18.2)	0.592
COVID-19 confirmed	111 (78.2)	100 (76.3)	11 (100.0)	0.122
COVID-19 suspected	31 (21.8)	31 (23.7)	0	0.122
COVID-19 symptoms
Fever	118 (83.1)	109 (83.2)	9 (81.8)	1.000
Sore throat	65 (45.8)	62 (47.3)	3 (27.3)	0.333
Cough	114 (80.3)	106 (80.9)	8 (72.7)	0.794
Expectoration	64 (45.1)	58 (44.3)	6 (54.5)	0.511
Nasal congestion	43 (30.3)	41 (31.3)	2 (18.2)	0.570
Runny nose	44 (31.0)	42 (32.1)	2 (18.2)	0.537
Dyspnea	21 (14.8)	10 (7.6)	11 (100.0)	0
Diarrhea	24 (16.9)	21 (16.0)	3 (27.3)	0.591
Anosmia or ageusia	50 (35.2)	48 (36.6)	2 (18.2)	0.367
Muscle soreness	86 (60.6)	78 (59.5)	8 (72.7)	0.590
Conjunctivitis	17 (12.0)	15 (11.5)	2 (18.2)	0.859
Fatigue	95 (66.9)	85 (64.9)	10 (90.9)	0.153
Hospitalized for COVID-19	7 (4.9)	0	7 (63.6)	0
AOSD flare after COVID-19	6 (4.2)	5 (3.8)	1 (9.1)	0.389
Death	1 (0.7)	0	1 (9.1)	0.077

Values are presented with a median (IQR), mean ± SD, or number (%)

COVID-19 coronavirus disease 2019, BMI body mass index, IQR interquartile range, JAK inhibitor Janus kinase inhibitor, DMARDs disease-modifying antirheumatic drugs, csDMARDs conventional synthetic DMARDs, bDMARDs biologic DMARDs, tsDMARDs targeted synthetic DMARDs, AOSD adult-onset Still’s disease, COPD chronic obstructive pulmonary disease, SD standard deviation

^aMild: did not or mildly interfered with daily activity

^bModerate: severely interfered with daily activity

^cSevere: required urgent medical attention or hospitalized medications

^dInterval between vaccination and COVID-19 infection: interval between last vaccine dose and first onset of COVID-19 symptoms

^eInactive AOSD before COVID-19: inactive for at least 3 months, and treated with prednisolone (or equivalent) dose ≤ 7.5 mg daily

^fSystemic score: included fever, typical rash, pleuritis, pneumonia, pericarditis, hepatomegaly or abnormal liver function tests, splenomegaly, lymphadenopathy, leukocytosis more than 15,000/mm³, sore throat, myalgia, and abdominal pain

^gGlucocorticoid therapy: including prednisone and prednisone equivalents

The characteristics of patients with mild COVID-19 were then compared with those of patients with moderate or severe COVID-19 (Table 1). The median age of the two groups of patients was 39.0 and 61.0 years (p = 0.001), respectively, with no significant differences in gender or BMI. There was also no significant difference between the two groups in terms of glucocorticoid and DMARDs treatment. Comorbidity with COPD or asthma was associated with the severity of COVID-19. In patients with mild disease, fever and cough remained the most common COVID-19 symptoms and dyspnea was more common in patients with severe disease.

COVID-19 Outcomes

In our study population, the COVID-19-related hospitalization rate was 4.9%, 11 cases out of 142 participants with AOSD were moderate or severe, and the mortality rate associated with COVID-19 was 0.7% (1 out of 142). This deceased patient was a 44-year-old woman with a history of asthma and pulmonary hypertension. Before the infection, she had an occasional fever and mild arthralgia and was orally treated with 17.5 mg/day of methylprednisolone, cyclosporine, and JAK inhibitors. After being infected with SARS-CoV-2, she developed COVID-19-associated pneumonia, which improved with treatment. However, within a week of being negative for SARS-CoV-2 by PCR, she developed severe lethal hemophagocytic lymphohistiocytosis (HLH).

Risk Factors for Severity of COVID-19 in Patients with AOSD

In further analysis of factors affecting COVID-19 severity, vaccination has an OR of 0.342 (95% CI 0.078–1.507) in the adjusted covariates model (Fig. 2). Concerning medication use, medium or high-dose glucocorticoid therapy was a risk factor with an OR of 4.667 (95% CI 1.011–21.549). JAK inhibitor was associated with moderate to severe COVID-19, with an OR of 10.783 (95% CI 1.240–93.771). Among patients with AOSD treated with methotrexate, hydroxychloroquine, and cyclosporine, there was no significant difference in the severity of COVID-19.

Detailed Information on Patients with AOSD and Post-Infection Disease Flare

Of the patients infected with SARS-CoV-2, six presented with AOSD flare. The demographic and clinical characteristics of these patients are shown in Table 2. One of the patients was male and the rest were female. Before the SARS-CoV-2 infection, two patients had withdrawn from treatment. Five patients were hospitalized for AOSD-related symptoms and one patient was treated on an outpatient basis. Most patients demonstrated elevated leukocytes and neutrophils, four patients suffered from impaired liver function, and all showed elevated inflammatory indicators.

Table 2

Demographic and clinical characteristics of patients with AOSD with post-infection disease flare

	Patient 1	Patient 2	Patient 3	Patient 4	Patient 5	Patient 6
Age, years	36	44	21	27	45	61
Sex	Male	Female	Female	Female	Female	Female
BMI, kg/m²	24	22.4	18.1	18.1	21.9	18.1
Comorbidities	No	Heart failure, asthma	No	No	No	No
Treatments before COVID-19	Glucocorticoid	Glucocorticoid, cyclosporine, tofacitinib	Glucocorticoid, cyclosporine, hydroxychloroquine	No	No	Glucocorticoid, methotrexate, hydroxychloroquine
Interval between COVID-19 and AOSD flare, days	76	33	4	35	7	65
AOSD symptoms of flare	Fever, sore throat, myalgia, arthralgia	Fever, rash, arthralgia, dyspnea	Fever, myalgia, arthralgia	Fever, rash, arthralgia	Fever, rash, sore throat, arthralgia	Fever, sore throat, myalgia
Systemic score^a	5	7	4	4	4	4
Leukocyte count (× 10⁹/L)	24.79	2.62	17.35	5.28	13.16	2.7
Neutrophil percentage (%)	89.7	62.3	95.6	73	85	70.8
Hb (g/L)	130	89	96	92	139	133
ALT (IU/L)	225	394	232	5	10	23
AST (IU/L)	208	508	57	30	23	63
CRP (mg/L)	208	23	74	125	64	13
ESR (mm/h)	61	17	69	36	67	22
Ferritin (ng/ml)	1223.2	> 15,000	585	3291	420.1	8852.5
Outcome	Hospitalization	Death	Hospitalization	Hospitalization	Hospitalization	Ambulatory

BMI body mass index, COVID-19 coronavirus disease 2019, AOSD adult-onset Still’s disease, Hb hemoglobin, ALT alanine aminotransferase, AST aspartate aminotransferase, CRP C-reactive protein, ESR erythrocyte sedimentation rate

^aSystemic score: included fever, typical rash, pleuritis, pneumonia, pericarditis, hepatomegaly or abnormal liver function tests, splenomegaly, lymphadenopathy, leukocytosis more than 15,000/mm³, sore throat, myalgia, and abdominal pain

Discussion

Since the beginning of December 2022, following a relevant policy change in China, there has been a large outbreak of SARS-CoV-2 infection, predominantly with the Omicron strain [10]. The COVID-19 epidemic has significantly influenced patients with rheumatic diseases, and patients with AOSD are no exception. The present study analyzed the clinical characteristics of patients with AOSD and COVID-19 and the impact of various factors on the prognosis of the infectious process.

The infection rate of SARS-CoV-2 in patients with AOSD was 75.5%, including patients with confirmed and highly suspected COVID-19, consistent with the general population (63.52% confirmed rate up to 25 December 2022) [9]. Of note, the infection rate might be underestimated, because some patients might be asymptomatic. Among all patients with AOSD with confirmed or suspected COVID-19, the COVID-19-related hospitalization rate was 4.9% (7 out of 142) and the mortality rate was 0.7% (1 out of 142). Given the low hospitalization rate in our study, extrapolation to the general population is challenging, although previous data showed similar critical or mortality rates (533 of 48,234 [1.1%]) [11]. Remarkably, the shortage of medical resources due to this large outbreak in a short period may have prevented the hospitalization of some patients with COVID-19, resulting in a lower hospitalization rate. The cause of death in one patient was considered to be severe HLH, one of the most serious complications of AOSD. However, it is challenging to determine whether the cause of her death was the onset of COVID-19, the flare of AOSD, or a combination of both. Additionally, the COVID-19 probably worsened her pulmonary hypertension. Similarly, patients with severe COVID-19 may also develop secondary HLH, which usually occurs in patients presenting with acute respiratory distress syndrome (ARDS) [12]. The HLH of this patient might be secondary to AOSD because the pneumonia was not severe.

The patients with AOSD in this study received mainly inactivated SARS-CoV-2 vaccines, and our previous study has shown that inactivated vaccines did not cause disease relapse in clinically inactive patients with AOSD and that adverse effects were mild and self-limiting [13]; however, the efficacy of inactivated vaccines is unclear. Following logistic regression analysis of risk factors for COVID-19 severity, although no statistical difference was reached, it was found that vaccination may result in a superior outcome for patients, consistent with the findings in the general population [11] and other populations with rheumatic diseases [14], but our conclusion requires further validation. Possible reasons include the long interval between vaccination and infection, and the suppression of the immunogenicity of the vaccine due to the use of immunosuppressive drugs or glucocorticoids, which requires further validation. In addition, not surprisingly, age and the presence of comorbidities (COPD or asthma and possible hypertension) increase the risk of deterioration of COVID-19 in patients with AOSD, which is consistent with the previous studies.

In terms of medication use, medium or high-dose oral glucocorticoids (> 7.5 mg/day) were associated with more severe COVID-19, which is concordant with data from patients with other rheumatic diseases [15‐17]. The mechanisms involved have not been fully elucidated, but this is not surprising since glucocorticoids are comprehensive inhibitors of the host inflammatory response to defend against the virus. Moreover, patients on medium or high-dose glucocorticoids may still have immune dysregulation which contributes to the worse outcome of COVID-19. Although hydroxychloroquine was originally proposed as a therapeutic agent for COVID-19, it was not shown to be protective in preventing adverse patient outcomes in our cohort, in line with previous reports on patients with rheumatic diseases [17], including systemic lupus erythematosus (SLE) [18]. The use of JAK inhibitors is strongly associated with an adverse prognosis of COVID-19 in patients with rheumatic diseases [15, 19], and similar to previous studies, we found that patients receiving JAK inhibitors had a worse outcome. While caution is needed in drawing this conclusion since the number of patients using JAK inhibitors in our study was so small. However, one study found that baricitinib reduced mortality in patients hospitalized for COVID-19 [20]. This may be related to the timing of drug use, where baseline use of JAK inhibitor at the initial phase of SARS-CoV-2 infection may suppress the immune response against the virus, whereas initiation of JAK inhibitor at the time of clinical deterioration may attenuate the abnormal systemic inflammatory response.

Our study identified six patients with AOSD suffering from AOSD flares within 3 months of SARS-CoV-2 infection, four of whom were hospitalized and one died, suggesting that SARS-CoV-2 might cause flares of AOSD. During the follow-up period, no disease flares were reported in patients with AOSD without COVID-19, and there was no statistical difference compared to patients with COVID-19 (p = 0.339). A case of AOSD onset after COVID-19 has been reported previously [5]. The potential pathogenic role of various pathogens in AOSD, particularly viruses, has attracted extensive attention. The formation of a cytokine storm is the key to the pathogenesis of AOSD. The starting point of the cytokine storm may arise from specific viral danger signals. Both severe COVID-19 and AOSD can be grouped as hyperferritinemia syndrome, share the same clinical features of high inflammation and cytokine storm, and possibly have the same molecular mechanisms [21]. Studies have shown that ferritin induces NETs (neutrophil extracellular traps) release and promotes systemic inflammation in AOSD [22], and a ferritin–NETs–cytokine storm loop may also be present in COVID-19 due to the formation of NETs and hyperferritinemia syndrome which are characteristic of patients with severe COVID-19. A potentially faulty immune response against SARS-CoV-2 may have triggered AOSD in patients.

This is the first study to evaluate the impact of COVID-19 in patients with AOSD and, as a result of a short-term outbreak of COVID-19, participants were infected with a similar strain of SARS-CoV-2. However, there are limitations. First, because of the rareness of the disease, the sample size was limited. In addition, owing to the retrospective observational study setting, unmeasured confounding factors may affect the results.

Conclusion

Our study demonstrates that patients with SARS-CoV-2 infection receiving medium or high-dose oral glucocorticoids and JAK inhibitors were associated with a worse outcome. Age and comorbidities were also risk factors. Vaccination was a possible protective factor from severe COVID-19 in patients with AOSD. SARS-CoV-2 infection was at risk of predisposing to AOSD flares. Additional study is warranted to confirm these observations. Strategies are needed to improve the outcome of patients with COVID-19 on glucocorticoids and immunomodulating agents.

Acknowledgements

We thank the participants of the study.

Declarations

Conflict of Interest

All authors declare they have no conflict of interest.

Ethical Approval

This study was approved by the Institutional Research Ethics Committee of Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, China with Institutional Review Board number KY2022-182, and was performed in compliance with the Declaration of Helsinki with written informed consent from the subjects.

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Feist E, Mitrovic S, Fautrel B. Mechanisms, biomarkers and targets for adult-onset Still’s disease. Nat Rev Rheumatol. 2018;14(10):603–18.CrossRefPubMedPubMedCentral

Giacomelli R, Ruscitti P, Shoenfeld Y. A comprehensive review on adult onset Still’s disease. J Autoimmun. 2018;93:24–36.CrossRefPubMed

Meng J, Ma Y, Jia J, et al. Cytokine storm in coronavirus disease 2019 and adult-onset Still’s disease: similarities and differences. Front Immunol. 2020;11:603389.CrossRefPubMed

Sefik E, Qu R, Junqueira C, et al. Inflammasome activation in infected macrophages drives COVID-19 pathology. Nature. 2022;606(7914):585–93.CrossRefPubMedPubMedCentral

Bamidis AD, Koehler P, di Cristanziano V, et al. First manifestation of adult-onset Still’s disease after COVID-19. Lancet Rheumatol. 2021;3(5):e319–21.CrossRefPubMedPubMedCentral

Palassin P, Bres V, Hassan S, et al. Comprehensive description of adult-onset Still’s disease after COVID-19 vaccination. J Autoimmun. 2023;134:102980.CrossRefPubMed

Buttgereit F, Boers M, Burmester G-R, et al. Standardised nomenclature for glucocorticoid dosages and glucocorticoid treatment regimens: current questions and tentative answers in rheumatology. Ann Rheum Dis. 2002;61:718–22.CrossRefPubMedPubMedCentral

Meng J, Chi H, Wang Z, et al. Characteristics and risk factors of relapses in patients with adult-onset Still’s disease: a long-term cohort study. Rheumatology (Oxford). 2021;60(10):4520–9.CrossRefPubMed

Sichuan Center for Disease Control and Prevention. Survey on the COVID-19 infection situation in Sichuan Province (Second Round). 2022. https://www.sccdc.cn/Article/View?id=26473.

10.

Lu G, Ling Y, Jiang M, et al. Primary assessment of the diversity of Omicron sublineages and the epidemiologic features of autumn/winter 2022 COVID-19 wave in Chinese mainland. Front Med. 2023. https://doi.org/10.1007/s11684-022-0981-7.CrossRefPubMedPubMedCentral

11.

Wu Q, Wang H, Cai J, et al. Vaccination effects on post-infection outcomes in the Omicron BA.2 outbreak in Shanghai. Emerg Microbes Infect. 2023;12(1):e2169197.CrossRefPubMedPubMedCentral

12.

McGonagle D, Sharif K, O’Regan A, Bridgewood C. The role of cytokines including interleukin-6 in COVID-19 induced pneumonia and macrophage activation syndrome-like disease. Autoimmun Rev. 2020;19(6): 102537.CrossRefPubMedPubMedCentral

13.

Hong X, Pan H, Su Y, et al. Inactivated SARS-CoV-2 vaccine does not increase the risk of relapse in patients with clinically inactive adult-onset Still’s disease. Rheumatology (Oxford). 2022;62:2262–6.CrossRef

14.

Cordtz R, Kristensen S, Westermann R, et al. COVID-19 infection and hospitalization risk according to vaccination status and DMARD treatment in patients with rheumatoid arthritis. Rheumatology (Oxford). 2022;62(1):77–88.CrossRefPubMed

15.

Haberman RH, Castillo R, Chen A, et al. COVID-19 in patients with inflammatory arthritis: a prospective study on the effects of comorbidities and disease-modifying antirheumatic drugs on clinical outcomes. Arthritis Rheumatol. 2020;72(12):1981–9.CrossRefPubMedPubMedCentral

16.

Ugarte-Gil MF, Alarcon GS, Izadi Z, et al. Characteristics associated with poor COVID-19 outcomes in individuals with systemic lupus erythematosus: data from the COVID-19 Global Rheumatology Alliance. Ann Rheum Dis. 2022;81(7):970–8.CrossRefPubMed

17.

Gianfrancesco M, Hyrich KL, Al-Adely S, et al. Characteristics associated with hospitalisation for COVID-19 in people with rheumatic disease: data from the COVID-19 Global Rheumatology Alliance physician-reported registry. Ann Rheum Dis. 2020;79(7):859–66.CrossRefPubMed

18.

Teh CL, Cheong YK, Wan-Musa WR, Wan-Mohd-Akbar SA, Mat-Husin N, Gun SC. COVID-19 among Malaysian patients with systemic lupus erythematosus on hydroxychloroquine. Ann Rheum Dis. 2021;80(5):e69.CrossRefPubMed

19.

Sparks JA, Wallace ZS, Seet AM, et al. Associations of baseline use of biologic or targeted synthetic DMARDs with COVID-19 severity in rheumatoid arthritis: results from the COVID-19 Global Rheumatology Alliance physician registry. Ann Rheum Dis. 2021;80(9):1137–46.CrossRefPubMed

20.

RECOVERY Collaborative Group. Baricitinib in patients admitted to hospital with COVID-19 (RECOVERY): a randomised, controlled, open-label, platform trial and updated meta-analysis. Lancet. 2022;400(10349):359–68.CrossRef

21.

Colafrancesco S, Alessandri C, Conti F, Priori R. COVID-19 gone bad: a new character in the spectrum of the hyperferritinemic syndrome? Autoimmun Rev. 2020;19(7): 102573.CrossRefPubMedPubMedCentral

22.

Jia J, Wang M, Meng J, et al. Ferritin triggers neutrophil extracellular trap-mediated cytokine storm through Msr1 contributing to adult-onset Still’s disease pathogenesis. Nat Commun. 2022;13(1):6804.CrossRefPubMedPubMedCentral

Titel: Characteristics of COVID-19 and Impact of Disease Activity in Patients with Adult-Onset Still’s Disease
verfasst von: Xinyue Hong
Xiaoming Wang
Ningqi Dai
Yue Sun
Honglei Liu
Xiaobing Cheng
Junna Ye
Hui Shi
Qiongyi Hu
Jianfen Meng
Zhuochao Zhou
Chengde Yang
Jialin Teng
Yutong Su
Huihui Chi
Publikationsdatum: 06.01.2024
Verlag: Springer Healthcare
Erschienen in: Rheumatology and Therapy / Ausgabe 1/2024
Print ISSN: 2198-6576
Elektronische ISSN: 2198-6584
DOI: https://doi.org/10.1007/s40744-023-00632-3

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Characteristics of COVID-19 and Impact of Disease Activity in Patients with Adult-Onset Still’s Disease