Remission of steroid-resistant Still's disease treated with anakinra, evidenced by FDG-PET/CT examination: case report

Adam, Zdeněk; Skřičková, Jana; Krtička, M.; Řehák, Zdeněk; Koukalová, Renata; Sprláková, A; Krejčí, Marta; Pour, Luděk; Adamová, Zdeňka; Pourová, Eva; Král, Zdeněk

doi:10.36290/vnl.2017.179

Cited by 7 publications

(6 citation statements)

References 84 publications

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“…Our study demonstrates that cladribine has moderate clinical efficacy in patients with ECD, with an overall clinical response rate of 52% (9 of 17) and an overall radiological response rate of 54% (8 of 15). To date, 17 case reports of ECD have been documented, with a collective response rate to cladribine approaching 60% . Similar to our series, these case reports demonstrated a response with cladribine at various ECD sites, such as central nervous system, bone, pleura, and pericardium.…”

Section: Discussionsupporting

confidence: 88%

“…It has also been shown to have activity in other lymphoid cancers (chronic lymphocytic leukemia, lymphoplasmacytic lymphoma, and certain low-grade lymphomas), as well as Langerhans cell histiocytosis. Increasing numbers of case reports demonstrate activity of cladribine in the treatment of ECD . However, contemporary trial or prospective data are lacking to help discern the response rate and outcomes with this agent.…”

Section: Introductionsupporting

confidence: 75%

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Clinical and Radiologic Responses to Cladribine for the Treatment of Erdheim-Chester Disease

et al. 2017

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Section: Discussionsupporting

confidence: 88%

Section: Introductionsupporting

confidence: 75%

Clinical and Radiologic Responses to Cladribine for the Treatment of Erdheim-Chester Disease

et al. 2017

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“…( 16) According to a case report, once a day administration of IL-1 receptor antagonist Anakinra led to the reduction of the dose of corticosteroidswhich was useful in controlling the side effects of corticosteroids such as the decompensation of Diabetes Mellitus. However, due to the hefty cost, it is not commonly used, (17) and it was not used in our patients either. Therefore, wherever possible, tapering off steroids and switching to such alternatives can ensure better treatment compliance and prevent glucocorticoid related side effects.…”

Section: Discussionmentioning

confidence: 99%

Clinical Spectrum of Adult-Onset Still’s Disease in Patients Presenting At A Tertiary Care Hospital in Pakistan: A 16-Year Follow-Up Study

Riaz,

Arshad,

Baqir

et al. 2023

Preprint

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Objectives Adult-onset Still’s Disease (AOSD) is a rare systemic auto-inflammatory disorder with a low incidence rate and limited data. This study aims to lay down the clinical and laboratory spectrum, associated complications, and outcomes of patients who presented to a tertiary care center – in order to identify any regional variation in the presentation and enable physicians to develop a thought process in patients without characteristic features. Materials and Methods We reviewed the records of patients from January 2005 - February 2021 who presented with symptoms consistent with AOSD, using the Yamaguchi criteria. The patients’ clinical symptoms, laboratory values, therapeutic management, complications, and outcomes were analysed. Descriptive statistics were produced to illustrate the pattern of the disease. Results The mean age of patients was 37.7 years, with a male to female ratio of 1:1. Fever and arthralgia was seen in 90%, a salmon-coloured rash in 60%, and leukocytosis in 80%. 30% presented with a sore throat and recent lymphadenopathy, 40% with hepatomegaly and/or splenomegaly on imaging, 100% with abnormal liver function tests. Steroids were used as first-line treatment. Most patients did not present with common, previously reported, complications of AOSD. Conclusions The clinical aspects in this study are, to a large part, comparable to those seen in other parts of the world. Thus, a comprehensive analysis of AOSD would enable physicians to broaden their minds and keep it as a top differential in atypical presentations – enhancing patient care by preventing a delay in diagnosis and initiation of prompt management.

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“…Biological agents can be useful in AOSD refractory to DMARD and Steroid treatment [22]. Biologics, like IL-1 receptor antagonist Anakinra, can reduce corticosteroid doses and manage side effects, e.g., diabetes decompensation [23]. Other IL-1 inhibitors (rilonacept and canakinumab), TNF alpha inhibitors and anti-IFNγ antibody(emapalumab) are alternatives [2,24,25].…”

Section: Discussionmentioning

confidence: 99%

Clinical spectrum of adult-onset Still’s disease in patients presenting at a tertiary care hospital in Pakistan: A 16-year retrospective descriptive study

Riaz,

Baqir,

Isra

et al. 2023

Preprint

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Introduction: Adult-onset Still's Disease (AOSD) is a rare systemic auto-inflammatory disorder with limited available data and regional variations in presentation. This study aims to shed light on the clinical and laboratory spectrum and associated complications of AOSD patients at a tertiary care center. The goal is to facilitate early diagnosis and management in cases lacking characteristic features. Methods: We conducted a retrospective review of patient records from January 2005 to February 2021, utilizing the Yamaguchi criteria for AOSD diagnosis. Clinical symptoms, laboratory values, therapeutic approaches and complications were analyzed. Descriptive statistics were employed to illustrate disease patterns. Results: The study included patients with a mean age of 37.6 years, with a male-to-female ratio of 3:5. Fever and arthralgia were prevalent in 100% of cases, while a salmon-colored rash was observed in 62.5%, and leukocytosis in 75%. Additional symptoms included sore throat and recent lymphadenopathy in 37.5%, hepatomegaly and/or splenomegaly on imaging in 50%, and abnormal liver function tests in 87.5% cases. Steroids were the primary first-line treatment. Notably, most patients did not exhibit the common complications previously reported in AOSD. Conclusion: The clinical findings in our study closely resemble those reported in other regions, emphasizing the need for a comprehensive analysis of AOSD. This study underscores the importance of considering AOSD as a potential diagnosis, even in atypical presentations, to enhance patient care by minimizing delays in diagnosis and enabling prompt management. Key points • This study presents the first national data on AOSD in Pakistan • AOSD mostly affects younger adults, with a mean age of 37.6 years • Common AOSD symptoms include fever, arthralgia, leukocytosis, high ferritin levels, and abnormal liver function, but the rash's prevalence varies by region.

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Remission of steroid-resistant Still's disease treated with anakinra, evidenced by FDG-PET/CT examination: case report

Cited by 7 publications

References 84 publications

Clinical and Radiologic Responses to Cladribine for the Treatment of Erdheim-Chester Disease

Clinical and Radiologic Responses to Cladribine for the Treatment of Erdheim-Chester Disease

Clinical Spectrum of Adult-Onset Still’s Disease in Patients Presenting At A Tertiary Care Hospital in Pakistan: A 16-Year Follow-Up Study

Clinical spectrum of adult-onset Still’s disease in patients presenting at a tertiary care hospital in Pakistan: A 16-year retrospective descriptive study

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