Macrophage activation syndrome and pulmonary arterial hypertension in a patient with adult-onset Still disease

Zeng, Furong; Deng, Guangtong; Luo, Hongyu; Zuo, Xiangsheng; Xie, Yanli

doi:10.1097/md.0000000000017427

Cited by 3 publications

(1 citation statement)

References 27 publications

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“…In the present study, TCZ effectively reduced the activity of acute AOSD in both the relapsed and first-episode groups. However, MAS development has been reported in several cases [ 21 , 22 ], and caution should be exercised when administering TCZ. Yamabe et al reported secondary MAS in six of their 20 patients with AOSD who received TCZ [ 23 ], suggesting that MAS after TCZ use may be more common than expected.…”

Section: Discussionmentioning

confidence: 99%

Optimal time of starting tocilizumab in acute phase of adult-onset Still’s disease and comparison of its efficacy with that of methotrexate: a case series and a review of the literature

Suzuki,

Kataoka,

Otani

et al. 2024

Clin Rheumatol

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Adult-onset still’s disease is a rare condition that is generally treated by glucocorticoids. Importantly, due to the limited established treatments, glucocorticoid-refractory cases are particularly difficult to treat. Between December 2009 and August 2022, nine patients with adult-onset Still’s disease were treated with tocilizumab (tocilizumab group). The therapeutic efficacy and safety of tocilizumab initiation in the acute phase were evaluated in cases of initial onset and recurrence. We also compared the efficacy of tocilizumab with that of methotrexate (methotrexate group, n = 13), which has been the drug of choice for adjunctive therapy. Tocilizumab demonstrated the expected efficacy in all four patients who received it at relapse and in three of the five patients who received it at the initial onset. However, two patients developed macrophage activation syndrome following treatment. A comparison of treatment effects between the methotrexate and tocilizumab groups revealed that the ferritin and C-reactive protein levels, severity score, and glucocorticoid doses decreased over time in both groups; nonetheless, the tocilizumab group experienced a more stable effect. Tocilizumab is undoubtedly a valuable treatment option for adult-onset Still’s disease, especially when administered at relapse. This suggests that it shows both high safety and good efficacy. Nevertheless, a larger sample size is required to validate the efficacy and safety of tocilizumab compared with those of the existing alternatives. Key Points • We examined the significance of TCZ in terms of therapeutic efficacy, reduction in glucocorticoid usage, and safety in patients with AOSD. •We compared the therapeutic efficacy of TCZ with that of MTX, which is often used to treat glucocorticoid-resistant AOSD. •TCZ is undoubtedly a valuable treatment option for AOSD, especially when administered at relapse, suggesting both high safety and good efficacy.

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Section: Discussionmentioning

confidence: 99%

Optimal time of starting tocilizumab in acute phase of adult-onset Still’s disease and comparison of its efficacy with that of methotrexate: a case series and a review of the literature

Suzuki,

Kataoka,

Otani

et al. 2024

Clin Rheumatol

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Biallelic mutations in the CFHR genes underlying atypical hemolytic uremic syndrome in a patient with catastrophic adult-onset Still's disease and recurrent macrophage activation syndrome: A case report

Dillemans,

Bekhuis,

Betrains

et al. 2023

Clinical Immunology

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A case of glucocorticoid-resistant adult Still’s disease complicated by pulmonary hypertension and interstitial lung disease

Kusaka

Miyagawa

Kosaka

et al. 2022

Modern Rheumatology Case Reports

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Adult Still’s disease (ASD) is rarely complicated by pulmonary hypertension (PH). A 76 year-old woman experienced ASD relapse with repeated exacerbation of PH and interstitial lung disease (ILD). Although she had been treated with immunosuppressive agents and pulmonary vasodilators, the ASD relapsed with fever, rash, increased inflammatory responses and exacerbated ILD and PH. The pathology of PH appeared to encompass groups 1 (pulmonary arterial hypertension [PAH]), 1ʹ (pulmonary veno-occlusive disease [PVOD]), and 3. Remission induction therapy with high-dose glucocorticoid and tocilizumab was administered, and switching or adding pulmonary vasodilators was also attempted. As the disease activity of ASD decreased, the mean pulmonary arterial pressure and pulmonary vascular resistance improved. PH is an extremely rare form of organ dysfunction in individuals with ASD. Like other systemic autoimmune diseases, PH (PAH or PVOD) can determine the prognosis of ASD. Because of PH’s rarity, it is important to sufficiently evaluate its pathology, considering the possibility that PH is not clinically classified as PAH (group 1), and to administer immunosuppressive therapy and vasodilators according to the pathology.

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Macrophage activation syndrome and pulmonary arterial hypertension in a patient with adult-onset Still disease

Cited by 3 publications

References 27 publications

Optimal time of starting tocilizumab in acute phase of adult-onset Still’s disease and comparison of its efficacy with that of methotrexate: a case series and a review of the literature

Optimal time of starting tocilizumab in acute phase of adult-onset Still’s disease and comparison of its efficacy with that of methotrexate: a case series and a review of the literature

Biallelic mutations in the CFHR genes underlying atypical hemolytic uremic syndrome in a patient with catastrophic adult-onset Still's disease and recurrent macrophage activation syndrome: A case report

A case of glucocorticoid-resistant adult Still’s disease complicated by pulmonary hypertension and interstitial lung disease

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