Macrophage activation syndrome complicating adult-onset still's disease: A diagnostic challenge

Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases. This case demonstrates the significant challenges and therapeutic considerations in adult-onset Still’s disease (AOSD) complicated with MAS at initial presentation, which will be discussed. MAS in our patient was refractory to the first-line therapy with high-dose corticosteroids, early administration of anakinra at a standard dosage and subsequent add-on treatments with cyclosporine A, IVIG, etoposides and tocilizumab. At 2 months after presentation, the patient was still critically ill with clinical, laboratory and histological signs of an active uncontrolled MAS. Notably, adoption of anakinra at a high dosage finally induced remission. This case confirms that adjusted dosage of anakinra is an effective therapeutic strategy in a severe AOSD-related MAS. It is tempting to speculate that anakinra at a high dosage, if used earlier, would have significantly changed the course of the disease in our patient and could have led to earlier remission.

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Adult-onset Still’s disease with macrophage activation syndrome diagnosed and treated based on cytokine profiling: a case-based review

Rheumatology International ◽

10.1007/s00296-019-04446-x ◽

2019 ◽

Vol 40 (1) ◽

pp. 145-152 ◽

Cited By ~ 3

Author(s):

Ken Goda ◽

Tsuneaki Kenzaka ◽

Masahiko Hoshijima ◽

Akihiro Yachie ◽

Hozuka Akita

Keyword(s):

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Adult Onset Still’S Disease ◽

Adult Onset ◽

Still’S Disease ◽

Still's Disease ◽

Yamaguchi Criteria ◽

Adult Onset Still's Disease ◽

Cytokine Profiling ◽

Activation Syndrome

Abstract Adult-onset Still’s disease (AOSD) is a relatively rare systemic inflammatory disorder and is diagnosed using various sets of classification criteria, with the Yamaguchi criteria as the most widely used criteria. Herein, we present the case of a 21-year-old woman admitted with a high fever, lasting for over 1 month, who did not fulfill the Yamaguchi criteria. However, by analyzing the inflammatory cytokine profile, we defined this case as AOSD based on a greatly elevated serum interleukin-18 level. In addition, we predicted the occurrence of macrophage activation syndrome by a characteristic increase in the soluble tumor necrosis factor receptor II level, which allowed a timely intervention for this malicious complication. Therefore, we suggest that cytokine profiling will be useful for the diagnosis and management of AOSD.

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Increased level of H-ferritin and its imbalance with L-ferritin, in bone marrow and liver of patients with adult onset Still's disease, developing macrophage activation syndrome, correlate with the severity of the disease

Autoimmunity Reviews ◽

10.1016/j.autrev.2015.01.004 ◽

2015 ◽

Vol 14 (5) ◽

pp. 429-437 ◽

Cited By ~ 32

Author(s):

Piero Ruscitti ◽

Paola Cipriani ◽

Paola Di Benedetto ◽

Francesco Ciccia ◽

Vasiliki Liakouli ◽

...

Keyword(s):

Bone Marrow ◽

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Adult Onset Still’S Disease ◽

Adult Onset ◽

Still’S Disease ◽

Still's Disease ◽

Severity Of The Disease ◽

Adult Onset Still's Disease ◽

Activation Syndrome

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POS1344 EVALUATING THE MULTIVISCERAL INVOLVEMENT ON ADULT-ONSET STILL’S DISEASE TO RETRIEVE IMAGING-BASED DIFFERENCES IN PATIENTS WITH AND WITHOUT MACROPHAGE ACTIVATION SYNDROME; RESULTS FROM A SINGLE-CENTRE OBSERVATIONAL STUDY

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.1600 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 954.2-955

Author(s):

I. DI Cola ◽

F. Bruno ◽

O. Berardicurti ◽

R. Monti ◽

A. Conforti ◽

...

Keyword(s):

Ct Scan ◽

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Adult Onset Still’S Disease ◽

Adult Onset ◽

Still’S Disease ◽

Still's Disease ◽

Life Threatening ◽

Adult Onset Still's Disease ◽

Activation Syndrome

Background:Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder usually affecting young adults, burdened by life-threatening complications, mainly macrophage activation syndrome (MAS), a secondary form of hemophagocytic lymphohistiocytosis [1]. In this context, the importance of an accurate assessment of AOSD is suggested to promptly recognise the multivisceral involvement of the disease which is associated with life-threatening complications. The assessment of the most aggressive subsets of the disease could guide the clinicians when to apply additional resources but avoiding unnecessary expenditures in patients with a less severe clinical picture.Objectives:In this study, we aimed at describing the multivisceral involvement of the disease to retrieve imaging-based differences in AOSD patients with and without MAS.Methods:The present evaluation has been designed as a cross-sectional study to descriptively compare the multivisceral involvement in AOSD patients with and without MAS. Patients admitted to our Institution, who underwent a total body CT scan, were selected from our historical cohort and assessed. Clinical and CT scan characteristics of AOSD patients with and without MAS were compared. Clinical and CT scan characteristics of AOSD patients with and without MAS were analysed by parametric or non-parametric t tests for all continuous variables, and chi squared test was used for categorical ones, as appropriate. Furthermore, possible correlations among radiological outcomes with laboratory markers and systemic score were estimated by using a point-biserial coefficient correlation.Results:This study evaluated 39 AOSD patients (men 64.1%), mean age of 48.8±16.6 years). Out of those, 14 patients (35.9%) were complicated by MAS. These patients showed higher values of ferritin [AOSD: 770.0 (1306.5) ng/mL vs MAS: 2926.3 (4918.5) ng/mL p=0.003] and systemic score (AOSD: 4.6±1.4 vs MAS: 6.9±1.7, p<0.0001). AOSD patients with MAS presented a higher prevalence of lung disease than others (AOSD: 56.0% vs MAS 85.7% p=0.048). Lung disease correlated with the systemic score (coefficient 0.491, p=0.003). AOSD patients with MAS were more frequently characterised by hepatomegaly (AOSD: 12.0% vs MAS: 50.0% p=0.019) and splenomegaly (AOSD: 16.0% vs MAS 50.0% p=0.033), respectively, than others. Hepatomegaly correlated with CRP (coefficient 0.421, p=0.016), ferritin (coefficient 0.397, p=0.020), and systemic score (coefficient 0.391, p=0.022). Furthermore, the presence of splenomegaly correlated with the systemic score (coefficient 0.439, p=0.009). CT scan features of abdominal effusions were more frequently observed in AOSD patients with MAS than those without this complication (AOSD: 12.0% vs 57.1% p=0.007). Finally, a higher percentage of AOSD patients with MAS showed a significant lymph node enlargement, either mediastinal or abdominal, than others on CT scan (AOSD: 36.0% vs MAS 71.4% p=0.048). The presence of lymphadenomegaly correlated with the systemic score (coefficient 0.368, p=0.032).Conclusion:Our findings showed a higher prevalence of multiorgan involvement in AOSD patients with MAS, suggesting imaging-based differences, although other studies are needed to fully assess this issue. Pulmonary disease, hepatomegaly, splenomegaly, lymph nodes enlargement, and abdominal effusions were associated with these more aggressive patients.References:[1]Giacomelli R, Ruscitti P, Shoenfeld Y. A comprehensive review on adult onset Still’s disease. J Autoimmun. 2018 Sep;93:24-36.Disclosure of Interests:None declared

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