Still syndrome

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The Still's disease (also Still's syndrome , Stillsche disease , Still's disease and Still'sche disease called) is an autoinflammatory disease that both children and adolescents, as can affect adults as well. Systemic juvenile idiopathic arthritis (SJIA) is used in children and adolescents . The exact incidence is unknown, but estimates are around 0.4-0.9 worldwide. The clinical picture of Still's disease, which only manifests itself in adulthood, is called AoSD ( Adult-onset Still's Disease ) . AoSD meets the definition of a rare disease as its prevalence is estimated at between one person per million in Europe to ten people per million in Japan.

Eric Bywaters first described AoSD as an inflammatory disease in young adulthood in the 1970s. Bywaters found that the disease was very similar to the systemic juvenile idiopathic arthritis previously described by Sir George Still in the 19th century. In recent years, more and more experts are of the opinion that the two forms of disease are the same disease picture, only with a different age onset. The clinical picture as a rheumatic disease form (or special form of chronic polyarthritis ) is associated with a high fever above 39 ° C, salmon-colored, fleeting rash (breastfeeding rash ), joint destruction, muscle pain , leukocytosis and an enlargement of the lymph nodes . Overactivity of the innate immune system is considered to be the cause of the disease .

diagnosis

Early diagnosis is important to avoid serious consequences, e.g. B. to avoid joint destruction and to be able to start an effective therapy . For the treatment of SJIA in particular, it is hypothesized that early effective treatment during a " window of opportunity " can fundamentally influence the long-term result and, in particular, reduce the risk of chronic joint wear.

Rare diseases such as SJIA and AoSD are usually identified through exclusion diagnostics . In the first step, it is mainly clarified whether obvious assumptions for the observed symptoms could possibly represent the cause of the disease. This is usually a lengthy process. Supportive diagnostic criteria are therefore helpful.

The classification criteria for the SJIA established by the ILAR ( International League of Associations for Rheumatology ) are:

  • Fever for at least two weeks (≥ 39 ° C for at least once a day with subsequent normalization to ≤ 37 ° C)
  • Arthritis in at least one joint (lasting more than six weeks)
  • At least one of the other symptoms:

The initiative PRO-KIND ("Projects for Classification, Monitoring and Therapy in Pediatric Rheumatology") of the Society for Child and Adolescent Rheumatology, which would like to develop consensus-oriented strategies to harmonize diagnostic and therapeutic approaches, shows, however, that up to 50% of those with SJIA diagnosed patients in Germany do not meet the requirements of the ILAR criteria. This is mainly due to the lack of chronic arthritis. The results suggest that chronic arthritis is not mandatory for the diagnosis and treatment of SJIA.

The Yamaguchi classification criteria are used to detect AoSD. If five of the listed criteria (at least two of them main criteria) are observed, an AoSD is suspected.

Main criteria Secondary criteria (minor criteria)
Fever over 39 ° C, intermittent, lasting for a week Sore throat
Arthralgia / arthritis for at least two weeks Lymphadenopathy and / or splenomegaly
Erythematous rash Transaminase increase
Increased white blood cell count> 10,000 / μl ANA or RF negativity

therapy

Most patients are initially still treated with glucocorticoids , but these are increasingly being replaced by biological agents such as interleukin- 1 and interleukin-6 antagonists as part of a treat-to-target strategy. Especially in the targeted treatment of the juvenile form, the human interleukin-1 receptor antagonist anakinra, produced using recombinant DNA technology , showed a high level of effectiveness when used as a first-line drug in monotherapy . This targeted therapy proved to be very effective, leading to early and persistent inactive disease in the majority of systemic JIA patients, greatly reducing glucocorticoid use, and preventing the development of long-term disease and therapy-related damage.

Individual evidence

  1. ↑ Ludwig Heilmeyer , Wolfgang Müller: The rheumatic diseases. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition ibid. 1961, pp. 309-351, here: pp. 331 f.
  2. ^ R. Gurion, TJA Lehman, LN Moorthy: Systemic Arthritis in Children: A Review of Clinical Presentation and Treatment . In: International Journal of Inflammation . tape 2012 , 2012, ISSN  2090-8040 , p. 1–16 , doi : 10.1155 / 2012/271569 , PMID 22235382 , PMC 3253447 (free full text) - ( hindawi.com [accessed January 10, 2020]).
  3. ↑ G Magadur-Joly, E Billaud, JH Barrier, YL Pennec, C Masson: Epidemiology of adult Still's disease: estimate of the incidence by a retrospective study in western France. In: Annals of the Rheumatic Diseases . tape 54 , no. 7 , July 1, 1995, ISSN  0003-4967 , p. 587-590 , doi : 10.1136 / ard.54.7.587 , PMID 7668903 , PMC 1009940 (free full text) - ( bmj.com [accessed January 10, 2020]).
  4. ↑ Kenji Wakai, Akihide Ohta, Akiko Tamakoshi, Yoshiyuki Ohno, Takashi Kawamura: Estimated Prevalence and Incidence of Adult Still's Disease: Findings by a Nationwide Epidemiological Survey in Japan . In: Journal of Epidemiology . tape 7 , no. 4 , 1997, ISSN  0917-5040 , p. 221–225 , doi : 10.2188 / jea.7.221 ( jst.go.jp [accessed January 10, 2020]).
  5. ↑ EG Bywaters: Still's disease in the adult. In: Annals of the Rheumatic Diseases . tape 30 , no. 2 , March 1, 1971, ISSN  0003-4967 , p. 121-133 , doi : 10.1136 / ard.30.2.121 , PMID 5315135 , PMC 1005739 (free full text) - ( bmj.com [accessed January 10, 2020]).
  6. ↑ George F. Still: On a Form of Chronic Joint Disease in Children . In: Medico-surgical transactions . tape 80 , 1897, ISSN  0959-5287 , p. 47–60.9 , PMID 20896907 , PMC 2036674 (free full text) - ( bmj.com [PDF; accessed January 10, 2020]).
  7. ↑ Mathieu Gerfaud-Valentin, Yvan Jamilloux, Jean IWAZ, Pascal Sève: Adult-onset Still's disease . In: Autoimmunity Reviews . tape 13 , no. 7 , July 2014, p. 708–722 , doi : 10.1016 / j.autrev.2014.01.058 ( elsevier.com [accessed on January 10, 2020]).
  8. ↑ Madiha Mahfoudhi, Imen Gorsane, Rafik Shimi, Sami Turki, Taieb Ben Abdallah: Adult Onset Still's Disease . In: International Journal of Clinical Medicine . tape 06 , no. 10 , 2015, ISSN  2158-284X , p. 716–724 , doi : 10.4236 / ijcm.2015.610095 ( scirp.org [accessed January 10, 2020]).
  9. ↑ Eugen Feist, Stéphane Mitrovic, Bruno Fautrel: Mechanisms, biomarkers and targets for adult-onset Still's disease . In: Nature Reviews Rheumatology . tape 14 , no. October 10 , 2018, ISSN  1759-4790 , p. 603–618 , doi : 10.1038 / s41584-018-0081-x ( nature.com [accessed January 10, 2020]).
  10. ↑ Ludwig Heilmeyer , Wolfgang Müller: The rheumatic diseases. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition ibid. 1961, pp. 309–351, here: pp. 331–333 ( special forms of chronic polyarthritis ).
  11. ↑ a b Nataša Toplak, Štefan Blazina, Tadej Avčin: The role of IL-1 inhibition in systemic juvenile idiopathic arthritis: current status and future perspectives. June 8, 2018, accessed January 10, 2020 .
  12. ↑ Clio P. Mavragani, Evangelos G. Spyridakis, Michael Koutsilieris: Adult-Onset Still's Disease: From Pathophysiology to Targeted Therapies . In: International Journal of Inflammation . tape 2012 , 2012, ISSN  2090-8040 , p. 1–10 , doi : 10.1155 / 2012/879020 , PMID 22792508 , PMC 3390042 (free full text) - ( hindawi.com [accessed January 10, 2020]).
  13. ↑ Sebastiaan J. Vastert, Wilco de Jager, Bo Jan Noordman, Dirk Holzinger, Wietse Kuis: Effectiveness of First-Line Treatment With Recombinant Interleukin-1 Receptor Antagonist in Steroid-Naive Patients With New-Onset Systemic Juvenile Idiopathic Arthritis: Results of a Prospective Cohort Study: First-Line Treatment With Recombinant IL-1Ra in New-Onset Systemic JIA . In: Arthritis & Rheumatology . tape 66 , no. 4 , April 2014, p. 1034-1043 , doi : 10.1002 / art.38296 ( wiley.com [accessed January 10, 2020]).
  14. ^ Ross E. Petty, Taunton R. Southwood, Prudence Manners, John Baum, David N. Glass: International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001 . In: The Journal of Rheumatology . tape 31 , no. 2 , February 2004, ISSN  0315-162X , p. 390-392 , PMID 14760812 ( jrheum.org [accessed January 10, 2020]).
  15. ↑ G. Horneff, I. Foeldvari, K. Minden, G. Ganser, J.-P. Haas: Ten years of experience in the German JIA etanercept registry: lessons learned from changing patient populations . In: Arthritis and Rheumatism . tape 31 , no. 05 , 2011, ISSN  0176-5167 , p. 334–342 , doi : 10.1055 / s-0037-1618085 ( thieme-connect.de [accessed on January 10, 2020]).
  16. ↑ a b PRO-KIND SJIA project collaborators, Claas H. Hinze, Dirk Holzinger, Elke Lainka, Johannes-Peter Haas: Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany . In: Pediatric Rheumatology . tape 16 , no. 1 , December 2018, ISSN  1546-0096 , p. 7 , doi : 10.1186 / s12969-018-0224-2 , PMID 29357887 , PMC 5778670 (free full text) - ( biomedcentral.com [accessed January 10, 2020]).
  17. ^ Nienke M. ter Haar, EH Pieter Dijkhuizen, Joost F. Swart, Annet Royen ‐ Kerkhof, Ayman el Idrissi: Treatment to Target Using Recombinant Interleukin ‐ 1 Receptor Antagonist as First ‐ Line Monotherapy in New ‐ Onset Systemic Juvenile Idiopathic Arthritis: Results From a five-year follow-up study . In: Arthritis & Rheumatology . tape 71 , no. 7 , July 2019, ISSN  2326-5191 , p. 1163–1173 , doi : 10.1002 / art.40865 , PMID 30848528 , PMC 6617757 (free full text) - ( wiley.com [accessed January 10, 2020]).