Research & Literature

Efficacy and safety of biological agents in adult-onset Still's disease.

Cavalli G, Franchini S, Aiello P, et al.

Scandinavian journal of rheumatology 2015; (44(4)):309-14 doi:10.3109/03009742.2014.992949.

Clinical features and prognosis of adult-onset Still's disease: 75 cases from China.

Liu Z, Lv X, Tang G

International journal of clinical and experimental medicine 2015; (8(9)):16634-9.

Febrile Exanthem with Hyperferritinemia.

Caputo GM, Dyer JM, Ryan EF

The Journal of clinical and aesthetic dermatology 2015; (8(11)):53-5.

Response rate of initial conventional treatments, disease course, and related factors of patients with adult-onset Still's disease: Data from a large multicenter cohort.

Kalyoncu U, Solmaz D, Emmungil H, et al.

Journal of autoimmunity 2016; (69()):59-63.

The role of 18F-fluorodeoxyglucose positron emission tomography in the assessment of disease activity of adult-onset Still's disease.

An YS, Suh CH, Jung JY, et al.

The Korean journal of internal medicine 2017; (32(6)):1082-1089 doi:10.3904/kjim.2015.322.

Validation of the Fautrel classification criteria for adult-onset Still's disease.

Lebrun D, Mestrallet S, Dehoux M, et al.

Seminars in arthritis and rheumatism 2018; (47(4)):578-585 doi:10.1016/j.semarthrit.2017.07.005.

Macrophage activation syndrome in Still's disease: analysis of clinical characteristics and survival in paediatric and adult patients.

Ruscitti P, Rago C, Breda L, et al.

Clinical rheumatology 2017; (36(12)):2839-2845 doi:10.1007/s10067-017-3830-3.

Oral mucosa lesions as atypical manifestation of adult-onset Still´s disease.

Brance ML, Neffen EL

Anais brasileiros de dermatologia 2018; (93(2)):271-273 doi:10.1590/abd1806-4841.20186836.

Adult-Onset Still's Disease: Molecular Pathophysiology and Therapeutic Advances.

Sfriso P, Bindoli S, Galozzi P

Drugs 2018; (78(12)):1187-1195 doi:10.1007/s40265-018-0956-9.

A comprehensive review on adult onset Still's disease.

Giacomelli R, Ruscitti P, Shoenfeld Y

Journal of autoimmunity 2018; (93()):24-36 doi:10.1016/j.jaut.2018.07.018.

Expand the differential…think beyond rheumatoid arthritis.

Schwartz C, Taylor A, Zaidi Z

BMJ case reports 2018; (2018()) doi:10.1136/bcr-2018-225618.

Treatment of refractory hemophagocytic lymphohistiocytosis with emapalumab despite severe concurrent infections.

Lounder DT, Bin Q, de Min C, Jordan MB

Blood advances 2019; (3(1)):47-50 doi:10.1182/bloodadvances.2018025858.

Recurring Lower Abdominal Pain and Fever as Initial Presentation of Adult Onset Still's Disease in the Absence of Arthralgia and Other System Involvement.

Sreh A, Rajaiah N, Saim M

European journal of case reports in internal medicine 2017; (4(6)):000619 doi:10.12890/2017_000619.

Tocilizumab for the treatment of adult-onset Still's disease.

Castañeda S, Martínez-Quintanilla D, Martín-Varillas JL, et al.

Expert opinion on biological therapy 2019; (19(4)):273-286 doi:10.1080/14712598.2019.1590334.

[Adult-onset Still's disease : Rare adult-onset autoinflammatory syndrome].

Oms E, Tronnier M

Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete 2019; (70(7)):530-534 doi:10.1007/s00105-019-4394-8.

Predictive factors and prognosis of macrophage activation syndrome associated with adult-onset Still's disease.

Yang XP, Wang M, Li TF, et al.

Clinical and experimental rheumatology 2019; (37 Suppl 121(6)):83-88.

Application of MS score in macrophage activation syndrome patients associated with adult onset Still's disease.

Wang R, Li T, Ye S, et al.

Annals of the rheumatic diseases 2021; (80(9)):e145 doi:10.1136/annrheumdis-2019-216286.

Pathogenesis, disease course, and prognosis of adult-onset Still's disease: an update and review.

Wang MY, Jia JC, Yang CD, Hu QY

Chinese medical journal 2019; (132(23)):2856-2864 doi:10.1097/CM9.0000000000000538.

Adult-onset Still's disease as a cutaneous marker of systemic disease.

Damevska K, França K, Nikolovska S, Gucev F

Clinics in dermatology 2019; (37(6)):668-674 doi:10.1016/j.clindermatol.2019.07.034.

Continuous Intravenous Anakinra Infusion to Calm the Cytokine Storm in Macrophage Activation Syndrome.

Monteagudo LA, Boothby A, Gertner E

ACR open rheumatology 2020; (2(5)):276-282 doi:10.1002/acr2.11135.

Tofacitinib-induced remission in refractory adult-onset Still's disease complicated by macrophage activation syndrome.

Honda M, Moriyama M, Kondo M, et al.

Scandinavian journal of rheumatology 2020; (49(4)):336-338 doi:10.1080/03009742.2020.1729405.

Silencing the cytokine storm: the use of intravenous anakinra in haemophagocytic lymphohistiocytosis or macrophage activation syndrome.

Mehta P, Cron RQ, Hartwell J, et al.

The Lancet. Rheumatology 2020; (2(6)):e358-e367 doi:10.1016/S2665-9913(20)30096-5.

Treatment of refractory adult onset Still's disease with tocilizumab-a single centre experience and literature review.

Reihl Crnogaj M, Čubelić D, Babić A, et al.

Rheumatology international 2020; (40(8)):1317-1325 doi:10.1007/s00296-020-04622-4.

Monogenic autoinflammatory diseases in children: single center experience with clinical, genetic, and imaging review.

Alsharief AN, Laxer RM, Wang Q, et al.

Insights into imaging 2020; (11(1)):87 doi:10.1186/s13244-020-00889-0.

Successful treatment of secondary macrophage activation syndrome with emapalumab in a patient with newly diagnosed adult-onset Still's disease: case report and review of the literature.

Gabr JB, Liu E, Mian S, et al.

Annals of translational medicine 2020; (8(14)):887 doi:10.21037/atm-20-3127.

A case report of Adult-onset Still's disease as a cause of severe mitral regurgitation.

Shah SH, Shah MA, Khan MS, Alghamdi FA

European heart journal. Case reports 2020; (4(4)):1-5 doi:10.1093/ehjcr/ytaa127.

Adult Onset Still's Disease: A Retrospective, Single-Center Study.

Hassan SA, Choudhry AS, Jamal S, et al.

Cureus 2020; (12(8)):e10008 doi:10.7759/cureus.10008.

A Case of Adult-Onset Still's Disease with Positive Antinuclear Antibodies.

Lakshman H, Athwal PSS, Gondi A, et al.

Cureus 2020; (12(10)):e10761 doi:10.7759/cureus.10761.

Adult-onset Still's disease accompanying noninfective endocarditis.

Oka K, Yuya Y, Yasuda M, Otsuka F

Journal of general and family medicine 2020; (21(6)):268-269 doi:10.1002/jgf2.339.

Anti-NLRP3 Inflammasome Natural Compounds: An Update.

Liu B, Yu J

Biomedicines 2021; (9(2)) doi:10.3390/biomedicines9020136.

Elevated serum gasdermin D N-terminal implicates monocyte and macrophage pyroptosis in adult-onset Still's disease.

Nagai H, Kirino Y, Nakano H, et al.

Rheumatology (Oxford, England) 2021; (60(8)):3888-3895 doi:10.1093/rheumatology/keaa814.

Risk of macrophage activation syndrome in patients with adult-onset Still's disease with skin involvement: A retrospective cohort study.

Ding Y, Tang S, Li S, et al.

Journal of the American Academy of Dermatology 2021; (85(6)):1503-1509 doi:10.1016/j.jaad.2021.02.005.

Adult-Onset Still's Disease: Clinical Aspects and Therapeutic Approach.

Tomaras S, Goetzke CC, Kallinich T, Feist E

Journal of clinical medicine 2021; (10(4)) doi:10.3390/jcm10040733.

The Role of NLRP3 Inflammasome in Cerebrovascular Diseases Pathology and Possible Therapeutic Targets.

Bai R, Lang Y, Shao J, et al.

ASN neuro 2021; (13()):17590914211018100 doi:10.1177/17590914211018100.

Current and emerging biological therapy in adult-onset Still's disease.

Ma Y, Meng J, Jia J, et al.

Rheumatology (Oxford, England) 2021; (60(9)):3986-4000 doi:10.1093/rheumatology/keab485.

Interleukin-6 inhibition: a therapeutic strategy for the management of adult-onset Still's disease.

Kaneko Y, Takeuchi T

Expert opinion on biological therapy 2022; (22(1)):79-85 doi:10.1080/14712598.2021.1942832.

Monogenic Autoinflammatory Diseases: State of the Art and Future Perspectives.

Di Donato G, d'Angelo DM, Breda L, Chiarelli F

International journal of molecular sciences 2021; (22(12)) doi:10.3390/ijms22126360.

The effectiveness of tocilizumab in treating refractory adult-onset Still's disease with dichotomous phenotypes: IL-18 is a potential predictor of therapeutic response.

Tang KT, Hsieh CW, Chen HH, et al.

Clinical rheumatology 2022; (41(2)):557-566 doi:10.1007/s10067-021-05921-2.

Adult-onset Still's disease with multiple lymphadenopathy: a case report and literature review.

Huang Z, Xu H, Min Q, et al.

Diagnostic pathology 2021; (16(1)):97 doi:10.1186/s13000-021-01159-3.

Interluekin-6 inhibitors for the treatment of adult-onset Still's disease.

Kaneko Y

Modern rheumatology 2022; (32(1)):12-15 doi:10.1093/mr/roab004.

Adult-Onset Still's Disease: A Case Report.

Shad I, Shafique M, Waris SA, et al.

Cureus 2022; (14(1)):e21033 doi:10.7759/cureus.21033.

Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: a study of 20 Moroccan adult patients.

Wafa A, Hicham H, Naoufal R, et al.

Clinical rheumatology 2022; (41(7)):2021-2033 doi:10.1007/s10067-022-06055-9.

NLRP3-Mediated Inflammation in Atherosclerosis and Associated Therapeutics.

Lu N, Cheng W, Liu D, et al.

Frontiers in cell and developmental biology 2022; (10()):823387 doi:10.3389/fcell.2022.823387.

Elevated White Blood Cell Count and Lactate Dehydrogenase Levels Are Important Markers for Diagnosing Relapse of Adult-onset Still's Disease under Tocilizumab Use.

Matsushita S, Sada K, Manabe A, et al.

Internal medicine (Tokyo, Japan) 2022; (61(24)):3743-3747 doi:10.2169/internalmedicine.9487-22.

[Assessment of MS-Score and HScore in timeliness of diagnosis of macrophage activation syndrome associated with adult-onset Still's disease].

Peng Z, Gao XM, Zhou S, et al.

Zhonghua yi xue za zhi 2022; (102(28)):2210-2214 doi:10.3760/cma.j.cn112137-20220321-00602.

JAK inhibitors in difficult-to-treat adult-onset Still's disease and systemic-onset juvenile idiopathic arthritis.

Gillard L, Pouchot J, Cohen-Aubart F, et al.

Rheumatology (Oxford, England) 2023; (62(4)):1594-1604 doi:10.1093/rheumatology/keac440.

Validation of the neutrophil-to-lymphocyte ratio as a new simple biomarker of adult onset Still's disease: A STROBE-Compliant prospective observational study.

Daghor Abbaci K, Ait Hamadouche N, Otmani F, et al.

Medicine 2022; (101(32)):e29970 doi:10.1097/MD.0000000000029970.

Adult-onset Still's disease: A disease at the crossroad of innate immunity and autoimmunity.

Rao S, Tsang LS, Zhao M, et al.

Frontiers in medicine 2022; (9()):881431 doi:10.3389/fmed.2022.881431.

Evaluation of Glycosylated Ferritin in Adult-Onset Still's Disease and Differential Diagnoses.

Guerber A, Garneret E, El Jammal T, et al.

Journal of clinical medicine 2022; (11(17)) doi:10.3390/jcm11175012.

Interleukin-1/6 Blockade for the Treatment of Severe Steroid-Refractory BNT162b2 Vaccine-Induced Adult-Onset Still's Disease.

Hugues B, Ben Amer H, Bril F, et al.

European journal of case reports in internal medicine 2022; (9(8)):003469 doi:10.12890/2022_003469.

[18F]FDG-PET/CT Demonstration of Young Adult Onset Stills Disease Mimicking Lymphoma.

Nawwar AA, Searle J, Hodby K, et al.

BJR case reports 2022; (8(3)):20210195 doi:10.1259/bjrcr.20210195.

Perspectives on the use of non-biological pharmacotherapy for adult-onset Still's disease.

Di Cola I, Cipriani P, Ruscitti P

Expert opinion on pharmacotherapy 2022; (23(14)):1577-1587 doi:10.1080/14656566.2022.2126764.

The Autoinflammatory Diseases Alliance Registry of monogenic autoinflammatory diseases.

Gaggiano C, Vitale A, Tufan A, et al.

Frontiers in medicine 2022; (9()):980679 doi:10.3389/fmed.2022.980679.

Flagellate dermatitis: An atypical skin finding in adult-onset Still's disease.

Toujani S, El Ouni A, Belhassen A, Bouslama K

Clinical case reports 2022; (10(10)):e6488 doi:10.1002/ccr3.6488.

Adult-Onset Still's Disease-A Complex Disease, a Challenging Treatment.

Macovei LA, Burlui A, Bratoiu I, et al.

International journal of molecular sciences 2022; (23(21)) doi:10.3390/ijms232112810.

[Adult-onset Still's disease : Diagnosis and treatment according to the new S2e guidelines of the German Society of Rheumatology (DGRh)].

Vordenbäumen S, Feist E

Zeitschrift fur Rheumatologie 2023; (82(2)):134-142 doi:10.1007/s00393-022-01293-3.

Proposal of a new diagnostic algorithm for adult-onset Still's disease.

Daghor-Abbaci K, Ait Hamadouche N, Makhloufi CD, et al.

Clinical rheumatology 2023; (42(4)):1125-1135 doi:10.1007/s10067-023-06509-8.

Reactivation of Parvovirus B19 Infection: An Uncommon Trigger of Macrophage Activation Syndrome in Adult-Onset Still's Disease.

Leelaviwat N, Armin S, Mekraksakit P, Nugent K

Cureus 2023; (15(4)):e37231 doi:10.7759/cureus.37231.

The NLRP3 inflammasome: contributions to inflammation-related diseases.

Chen Y, Ye X, Escames G, et al.

Cellular & molecular biology letters 2023; (28(1)):51 doi:10.1186/s11658-023-00462-9.

A case of adult-onset Still's disease in a patient after a car accident.

Yaman F, Kimiaei A

Clinical case reports 2023; (11(8)):e7510 doi:10.1002/ccr3.7510.

A Novel Therapy for a Rare Condition: Continuous Anakinra Infusion for a Patient With Macrophage Activation Syndrome.

Gullickson M, Nichols L, Scheibe M

Cureus 2023; (15(8)):e42968 doi:10.7759/cureus.42968.

Hemophagocytic lymphohistiocytosis accompanying Still's disease: A case report.

Ahmadzadeh A, Babadi N, Farsad F, et al.

Clinical case reports 2023; (11(10)):e7955 doi:10.1002/ccr3.7955.

Refractory Adult-onset Still's Disease Treated with a Combination of Methotrexate and Etanercept.

El Hasbani G, Ahmad Y, Cassetta M

Current rheumatology reviews 2024; (20(2)):219-222 doi:10.2174/0115733971244440230921100912.

Adult-onset Still's disease with concurrent thrombotic microangiopathy: Observations from pooled analysis for an uncommon finding.

Ananthaneni A, Shimkus G, Weis F, et al.

European journal of haematology 2024; (112(4)):484-492 doi:10.1111/ejh.14142.

Recent advances and evolving concepts in Still's disease.

Ruscitti P, Cantarini L, Nigrovic PA, et al.

Nature reviews. Rheumatology 2024; (20(2)):116-132 doi:10.1038/s41584-023-01065-6.

The latest advances in the use of biological DMARDs to treat Still's disease.

Di Cola I, Ruscitti P

Expert opinion on biological therapy 2024; (24(1-2)):63-75 doi:10.1080/14712598.2024.2307340.

Adult-onset Still's Disease: A Case Report.

Karki B, K C N, Mahato AK, et al.

JNMA; journal of the Nepal Medical Association 2023; (61(264)):662-664 doi:10.31729/jnma.8237.

Association between adult-onset still's disease and COVID-19: A report of two cases and brief review.

Fet-He S, Ibarra Lecompte G, Quiroz Alfaro AJ

SAGE open medical case reports 2024; (12()):2050313X241233197 doi:10.1177/2050313X241233197.

Cluster analysis defines four groups of Japanese patients with adult-onset Still's disease.

Shirahama Y, Kokuzawa A, Yamauchi Y, et al.

Modern rheumatology 2024; (34(6)):1213-1220 doi:10.1093/mr/roae023.

The efficacy and safety of JAK inhibitors in patients with adult-onset still's disease: A meta-analysis and systematic review.

Liao J, Tang Q, Xie X, et al.

International immunopharmacology 2024; (137()):112451 doi:10.1016/j.intimp.2024.112451.

An Extensive Analysis and Comparison of Bone Marrow Aspiration and Bone Marrow Trephine Biopsy at a Tertiary Care Hospital in Jharkhand for Various Hematological and Non-hematological Illnesses.

Jawed MA, Paswan MK, Mahto SK, et al.

Cureus 2024; (16(6)):e62661 doi:10.7759/cureus.62661.

Still's Disease Onset in Older Adults: Clinical Features, Diagnosis, and Management.

Tada Y, Maruyama A, Shirahama Y

Drugs & aging 2024; (41(9)):713-724 doi:10.1007/s40266-024-01137-6.

Anakinra in the management of adult-onset still's disease: a single-center experience.

Kilic B, Parlar K, Karup S, et al.

Internal and emergency medicine 2025; (20(1)):131-138 doi:10.1007/s11739-024-03766-6.

Efficacy and safety of therapies for Still's disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR/PReS guidelines for the management of Still's disease.

Bindoli S, De Matteis A, Mitrovic S, et al.

Annals of the rheumatic diseases 2024; (83(12)):1731-1747 doi:10.1136/ard-2024-225854.

EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease.

Fautrel B, Mitrovic S, De Matteis A, et al.

Annals of the rheumatic diseases 2024; (83(12)):1614-1627 doi:10.1136/ard-2024-225851.

Beyond the Norm: A Unique Case of Adult-Onset Still's Disease.

Attia B, Ismail MS, El-Ghobashy N, et al.

Cureus 2024; (16(8)):e68104 doi:10.7759/cureus.68104.

Hyperferritinemia: Important Differentials for the Rheumatologists.

Kaur M, Lo SWS, Liu Y, Yip K

Cureus 2024; (16(9)):e68588 doi:10.7759/cureus.68588.

Macrophage Activation Syndrome.

Nigrovic PA

Arthritis & rheumatology (Hoboken, N.J.) 2025; (77(4)):367-379 doi:10.1002/art.43052.

Delayed diagnosis of adult onset Still's disease in 2 cases: diagnostic dilemma in positive antinuclear antibody and tuberculosis endemic areas.

Rahman PA, Sakti PT

Postgraduate medicine 2025; (137(1)):100-107 doi:10.1080/00325481.2024.2433930.

The role of PET/CT scan in addressing autoinflammatory diseases; A case report of a young man presenting with fever, splenomegaly and exanthem.

Geladari EV, Pianou NK, Angelis NA, et al.

Hellenic journal of nuclear medicine 2024; (27(3)):243-246 doi:10.1967/s002449912759.

The role of C-reactive protein and ferritin in the diagnosis of HLH, adult-onset still's disease, and COVID-19 cytokine storm.

Goubran M, Spaner C, Stukas S, et al.

Scientific reports 2024; (14(1)):31306 doi:10.1038/s41598-024-82760-6.

Toward a personalized therapy of still's disease based on immunologic endotypes: a narrative review.

Chen PK, Wey SJ, Chen DY

Expert review of clinical immunology 2025; (21(4)):401-412 doi:10.1080/1744666X.2025.2465406.

Early identification of macrophage activation syndrome in adult-onset Still's disease: a case report and literature review.

Long T, Xu J, Lin BZ, Li SG

Frontiers in medicine 2025; (12()):1498928 doi:10.3389/fmed.2025.1498928.

Update in Hyperferritinemic Syndromes: Recognition and Management - A Scoping Review.

Vassilopoulos A, McCormick W, Lakhani A

The Brown journal of hospital medicine 2022; (1(3)):37667 doi:10.56305/001c.37667.

Characterization of Patients with Macrophage Activation Syndrome Secondary to Systemic Juvenile Idiopathic Arthritis.

Rogowska J, Kubicka J, Grabowska M, et al.

Clinical rheumatology 2025; (44(5)):2023-2029 doi:10.1007/s10067-025-07420-0.

The pathophysiology of hemophagocytic lympho-histiocytosis (HLH) syndrome and insights from animal models.

Mukherjee S, Kumar P

Clinical and experimental immunology 2025; (219(1)) doi:10.1093/cei/uxaf036.

Evaluation of clinical biomarkers for differential diagnosis of intravascular lymphoma and Still's disease.

Shima T, Yamauchi Y, Ohtsu M, et al.

Scientific reports 2025; (15(1)):23901 doi:10.1038/s41598-025-09401-4.

Current evidence on switching between biologic therapies for Still's disease: A systematic literature review.

Dagna L, Feist E, Horneff G, et al.

Seminars in arthritis and rheumatism 2025; (74()):152789 doi:10.1016/j.semarthrit.2025.152789.

Utilization of PET in diagnosing adult-onset Still's disease: a systematic review.

Cajamarca-Baron J, Castañeda-Gonzalez JP, Acelas-Gonzalez GE, et al.

Nuclear medicine communications 2025; (46(11)):1020-1028 doi:10.1097/MNM.0000000000002039.

Challenges on diagnosis and treatment of refractory adult-onset still disease in Sub-Saharan Africa: a case report.

Simeni Njonnou SR, Kemta Lekpa F, Essoka Essoka AR, et al.

Journal of medical case reports 2025; (19(1)):472 doi:10.1186/s13256-025-05477-8.

Early IL-1 Inhibition in Still's Disease: A Window of Opportunity for Improving Outcomes.

Bindoli S, Cadore C, Guidea I, et al.

ACR open rheumatology 2025; (7(10)):e70106 doi:10.1002/acr2.70106.

Febrile Rash, Shock, and Hyperferritinaemia in A Young Woman: Navigating the Diagnostic Overlap of Hemophagocytic Lymphohistiocytosis, Toxic Shock, and Viral Exanthema.

Srinivasaiah PH, Surabhi KV

European journal of case reports in internal medicine 2025; (12(12)):005887 doi:10.12890/2025_005887.

Advancing Precision Medicine in Adult-Onset Still's Disease: Insights into Biomarkers, Therapies, and COVID-19 Impacts.

Sahoo DP

Mediterranean journal of rheumatology 2025; (36(4)):509-523 doi:10.31138/mjr.020525.ahr.

Macrophage Activation Syndrome Associated with Adult-onset Still's Disease Successfully Treated with Plasma Exchange and Canakinumab: A Case Report.

Kiyohara W, Fujita Y, Hatta M, et al.

Internal medicine (Tokyo, Japan) 2026; doi:10.2169/internalmedicine.6642-25.

Tocilizumab combined with short-term high-dose glucocorticoids for rapid disease activity control and glucocorticoid reduction in adult-onset Still's disease: a single-center retrospective study.

Zhong SL, Lei YP, Zhou LX, et al.

Frontiers in immunology 2026; (17()):1732826 doi:10.3389/fimmu.2026.1732826.