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Background: Cardiac involvement in adult-onset Still’s disease (AOSD) represents a relatively frequent and potentially severe complication. However, its predictive factors and management strategies remain poorly defined. We conducted a retrospective multicenter study to describe the manifestations, treatments, and outcomes of cardiac involvement in AOSD.
Objectives: The aims of this work were to describe the clinical and paraclinical profile of patients with AOSD who present with cardiac involvement, to try to establish the predictive factors for developing a cardiac complication, and to assess the response to treatment.
Methods: Patients were included from three different databases originating from French hospitals. All met the Yamaguchi and/or Fautrel criteria. Demographic, clinical, biological data, as well as treatmentof 66 patients with cardiac involvement (AOSD + C group) were collected, analyzed, and compared to those of 126 controls without cardiac involvement (AOSD – C group).
Results: Cardiac involvement occurred at AOSD onset, before or during the diagnostic stage in 62/66 (94%) cases, before any specific treatment. The AOSD + C group had more active disease at diagnosis including more frequent odynophagia, hepatosplenomegaly, myalgia, macrophage activation syndrome (MAS), and pleural effusion (Table 1). Biologically, they showed higher levels of white blood cells, neutrophils, ferritin, and C-reactive protein (CRP). Cardiac complications included pericarditis in 36/66 (55%) cases (with tamponade in 6 patients), myocarditis in 29 (44%) cases, and non-infectious endocarditis in 1 (1%) case (Table 2). Cardiac symptoms were non-specific, more often presenting as chest pain (74%) or dyspnea (53%). Signs of right heart failure were present in 16% of patients, while 10% had no cardiac symptoms (cardiac involvement discovered during routine evaluation). Predictive factors for cardiac involvement included a high modified Pouchot score, hepatomegaly, pleural effusion, myalgia, and elevated CRP. Less than 50% of patients treated with glucocorticoids (GC) alone achieved complete remission, whereas 90-100% of those who combined GC with anakinra or tocilizumab achieved remission. Although 50% of patients were initially managed in intensive care units, there was no death.
Conclusion: This study represents the largest reported series of AOSD patients with cardiac involvement. The fact that nearly 10% of patients were asymptomatic (raises the question of systematic screening, particularly in cases of highly active AOSD. Our findings support the early use of IL-1 or IL-6 inhibitors in combination with GC, along with multidisciplinary management.
Characteristics and manifestations of AOSD at diagnosis in patients with and without cardiac involvement.
| AOSD – C (n = 126) | AOSD + C (n = 66) | p-value | |
|---|---|---|---|
| Demography | |||
| Median year (range) of AOSD diagnosis | 2016 (1980 – 2023) | 2014 (1980 – 2023) | 0.824 |
| Male n/N (%) | 47/126 (37) | 31/66 (47) | 0.254 |
| Age at AOSD diagnosis (years): median [Q1-Q3] | 36 [25 – 52] | 30 [22 – 51] | 0.157 |
| Follow-up (months): median [Q1-Q3] | 20.5 [0 – 31] | 22.50 [9 – 57] | 0.360 |
| Classification criteria fulfillment | |||
| Yamaguchi criteria | 92/126 (73) | 60/66 (91) | 0.007 |
| Fautrel criteria | 103/126 (82) | 60/66 (91) | 0.141 |
| At least one criteria | 126/126 (100) | 66/66 (100) | - |
| Clinical manifestations at diagnosis | |||
| Fever >39°C: n/N (%) | 126/126 (100) | 66/66 (100) | - |
| Arthralgia : n/N (%) | 110/126 (87) | 54/66 (81) | 0.420 |
| Arthritis n/N (%) | 49/126 (39) | 14/36 (39) | 1.000 |
| Sore throat n/N (%) | 72/126 (57) | 51/66 (77) | 0.017* |
| Maculopapular rash n/N(%) | 88/126 (70) | 43/66 (65) | 0.617 |
| Lymphadenopathy n/N (%) | 39/126 (31) | 20/62 (32) | 0.989 |
| Hepatomegaly n/N (%) | 10/126 (8) | 21/62 (34) | 0.001* |
| Splenomegaly n/N (%) | 10/126 (8) | 13/62 (21) | 0.020* |
| Myalgia n/N (%) | 20/126 (16) | 35/66 (53) | 0.001* |
| Pneumonia n/N (%) | 3/124 (2) | 1/66 (2) | 1.000 |
| Pleural effusion n/N (%) | 4/126 (3) | 46/66 (70) | 0.001* |
| MAS n/N (%) | 2/126 (2) | 8/66 (12) | 0.003* |
| Biological manifestations at diagnosis | |||
| WBC (G/L) median [Q1-Q3] | 14 [11 – 18] | 23 [17.0 – 28.9] | 0.001* |
| PMNs (G/L) median [Q1-Q3] | 11 [8 – 16] | 20 [14.1 – 26.9] | 0.001* |
| Percentage of neutrophils % median [Q1-Q3] | 83 [74 – 88] | 89 [81.8 – 92.1] | 0.001* |
| CRP (mg/L) median [Q1-Q3] | 132 [65 – 205] | 328 [225.25 – 401.25] | 0.001* |
| SF (μmol/L) median [Q1-Q3] | 1649 [494 – 6529] | 3593 [1149 – 10908] | 0.001* |
| Glycosylated ferritin (%) median [Q1-Q3] | 10.50 [8 – 18.25] | 9 [7 – 16] | 0.131 |
| Elevated enzyme livers, n/N (%) | 46/126 (36%) | 29/66 (44) | 0.397 |
| Modified Pouchot Systemic Score at diagnosis | |||
| Mean (SD) | 4 (1)
| 7 (2)
|
0.001*
|
Characteristics of cardiac events in the AOSD + C group.
| AOSD + C group N = 66 | |
|---|---|
| Cardiac events n/N (% ) | |
| Pericarditis | 36/66 (55) |
| Myocarditis | 29/66 (44) |
| • Of which myopericarditis | 18/29 (27) |
| Non-infective endocarditis | 1/66 (1) |
| Time of appearance of cardiac involvement: n/N (% ) | |
| Before or during the diagnosis of AOSD | 62/66 (94) |
| After the first year of diagnosis | 4/66 (6) |
| Clinical cardiac manifestations n/N (% ) | |
| Chest pain | 42/57 (74) |
| Dyspnea | 30/57 (53) |
| Right heart failure | 9/57 (16) |
| Asymptomatic (incidentally discovered) | 6/57 (10) |
| Examinations: n/N (% ) | |
| Electrocardiogram abnormality | 32/57 (56) |
| • T wave modification | • 10 (31) |
| • Diffuse ST shift | • 10 (31) |
| • PR segment under-shift | • 6 (19) |
| • Atrial fibrillation | • 3 (9) |
| • Complete heart block | • 1 (3) |
| • Right bundle branch block | • 2 (6) |
| High troponin levels | 23/33 (70) |
| Cardiac ultrasound abnormality* | 48/57 (84) |
| • Pericardial effusion | • 36 (75) |
| • Signs of tamponade** | • 6 (13) |
| • Left ventricular dysfunction | • 5 (10) |
| • 15 mm oscillating mitral valvular vegetation | • 1 (2) |
| Cardiac MRI abnormality | 25/29 (86) |
| • Pericardial effusion | • 5 (20) |
| • Myocardial gadolinium enhancement | • 20 (80) |
REFERENCES: NIL.
Acknowledgements: All the doctors who participated in the observational call of the Club of Inflammatory Rheumatism.
Disclosure of Interests: None declared.
© The Authors 2025. This abstract is an open access article published in Annals of Rheumatic Diseases under the CC BY-NC-ND license (