Systemic Juvenile Idiopathic Arthritis/Pediatric Still's Disease, a Syndrome but Several Clinical Forms: Recent Therapeutic Approaches

doi:10.3390/jcm11051357

. 2022 Mar 1;11(5):1357.

doi: 10.3390/jcm11051357.

Systemic Juvenile Idiopathic Arthritis/Pediatric Still's Disease, a Syndrome but Several Clinical Forms: Recent Therapeutic Approaches

Pierre Quartier^{1

2}

Affiliations

¹ Unité d'Immunologie-Hématologie et Rhumatologie Pédiatrique, RAISE Reference Centre, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, 149 Rue de Sèvres, 75015 Paris, France.
² INSERM 1231, Université de Paris, 45 Rue des Saints-Pères, 75006 Paris, France.

PMID: 35268449
PMCID: PMC8911482
DOI: 10.3390/jcm11051357

Systemic Juvenile Idiopathic Arthritis/Pediatric Still's Disease, a Syndrome but Several Clinical Forms: Recent Therapeutic Approaches

Pierre Quartier. J Clin Med. 2022.

. 2022 Mar 1;11(5):1357.

doi: 10.3390/jcm11051357.

Author

Pierre Quartier^{1

2}

Affiliations

¹ Unité d'Immunologie-Hématologie et Rhumatologie Pédiatrique, RAISE Reference Centre, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, 149 Rue de Sèvres, 75015 Paris, France.
² INSERM 1231, Université de Paris, 45 Rue des Saints-Pères, 75006 Paris, France.

PMID: 35268449
PMCID: PMC8911482
DOI: 10.3390/jcm11051357

Abstract

Background: Systemic Juvenile Idiopathic Arthritis (SJIA)/Pediatric Still's disease is associated with different phenotypes and outcomes from currently available treatments.

Methods: A review of opinion, based on personal experience in a reference pediatric rheumatology center and key publications, to explore the most important questions regarding disease heterogeneity and treatment approaches.

Results: A few situations deserve particular attention: 1/patients with recent-onset SJIA who may benefit from a treat-to-target approach with a key place for interleukin (IL)-1 inhibition; 2/SJIA patients refractory to Il-1 and IL-6 antagonists in whom several options may be discussed, including thalidomide or allogeneic hematopoietic stem cell transplantation; 3/SJIA patients with macrophage activation syndrome who may benefit from both well-used classical treatment and innovative approaches, such as anti-interferon gamma therapy or Janus Kinase (JAK) inhibitors; 4/SJIA with severe lung involvement, 5/SJIA patients who achieve complete remission on treatment, with some recent evidence that treatment may be reduced in intensity but not so easily withdrawn.

Conclusions: a case-by-case discussion with expert teams is recommended in this heterogeneous, often difficult-to-treat population of patients.

Keywords: Janus Kinase antagonists; Still’s disease; Systemic Juvenile Idiopathic Arthritis; allogeneic hematopoietic stem cell transplantation; biotherapy; interleukin-1; interleukin-18; interleukin-6; interstitial lung disease; macrophage activation syndrome.

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Conflict of interest statement

The author received consultancy or speaking fees from AbbVie, Bristol-Myers Squibb, Chugai-Roche, Lilly, Novartis, Novimmune, Pfizer and Swedish Orphan Biovitrum; and participated in a data safety monitoring board for Sanofi.

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References

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1. Erkens R., Esteban Y., Towe C., Schulert G., Vastert S. Pathogenesis and Treatment of Refractory Disease Courses in Systemic Juvenile Idiopathic Arthritis: Refractory Arthritis, Recurrent Macrophage Activation Syndrome and Chronic Lung Disease. Rheum. Dis. Clin. N. Am. 2021;47:585–606. doi: 10.1016/j.rdc.2021.06.003. - DOI - PubMed

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[1] Petty R.E., Southwood T.R., Manners P., Baum J., Glass D.N., Goldenberg J., He X., Maldonado-Cocco J., Orozco-Alcala J., Prieur A.-M., et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: Second revision, Edmonton, 2001. J. Rheumatol. 2004;31:390–392. - PubMed

[2] Petty R.E., Southwood T.R., Manners P., Baum J., Glass D.N., Goldenberg J., He X., Maldonado-Cocco J., Orozco-Alcala J., Prieur A.-M., et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: Second revision, Edmonton, 2001. J. Rheumatol. 2004;31:390–392. - PubMed

[3] Martini A., Ravelli A., Avcin T., Beresford M.W., Burgos-Vargas R., Cuttica R., Ilowite N.T., Khubchandani R., Laxer R.M., Lovell D.J., et al. Toward New Classification Criteria for Juvenile Idiopathic Arthritis: First Steps, Pediatric Rheumatology International Trials Organization International Consensus. J. Rheumatol. 2019;46:190–197. doi: 10.3899/jrheum.180168. - DOI - PubMed

[4] Martini A., Ravelli A., Avcin T., Beresford M.W., Burgos-Vargas R., Cuttica R., Ilowite N.T., Khubchandani R., Laxer R.M., Lovell D.J., et al. Toward New Classification Criteria for Juvenile Idiopathic Arthritis: First Steps, Pediatric Rheumatology International Trials Organization International Consensus. J. Rheumatol. 2019;46:190–197. doi: 10.3899/jrheum.180168. - DOI - PubMed

[5] Allantaz F., Chaussabel D., Stichweh D., Bennett L., Allman W., Mejias A., Ardura M., Chung W., Smith E., Wise C., et al. Blood leukocyte microarrays to diagnose systemic onset juvenile idiopathic arthritis and follow the response to IL-1 blockade. J. Exp. Med. 2007;204:2131–2144. doi: 10.1084/jem.20070070. - DOI - PMC - PubMed

[6] Allantaz F., Chaussabel D., Stichweh D., Bennett L., Allman W., Mejias A., Ardura M., Chung W., Smith E., Wise C., et al. Blood leukocyte microarrays to diagnose systemic onset juvenile idiopathic arthritis and follow the response to IL-1 blockade. J. Exp. Med. 2007;204:2131–2144. doi: 10.1084/jem.20070070. - DOI - PMC - PubMed

[7] Pardeo M., Bracaglia C., De Benedetti F. Systemic juvenile idiopathic arthritis: New insights into pathogenesis and cytokine directed therapies. Best Pract. Res. Clin. Rheumatol. 2017;31:505–516. doi: 10.1016/j.berh.2018.02.002. - DOI - PubMed

[8] Pardeo M., Bracaglia C., De Benedetti F. Systemic juvenile idiopathic arthritis: New insights into pathogenesis and cytokine directed therapies. Best Pract. Res. Clin. Rheumatol. 2017;31:505–516. doi: 10.1016/j.berh.2018.02.002. - DOI - PubMed

[9] Erkens R., Esteban Y., Towe C., Schulert G., Vastert S. Pathogenesis and Treatment of Refractory Disease Courses in Systemic Juvenile Idiopathic Arthritis: Refractory Arthritis, Recurrent Macrophage Activation Syndrome and Chronic Lung Disease. Rheum. Dis. Clin. N. Am. 2021;47:585–606. doi: 10.1016/j.rdc.2021.06.003. - DOI - PubMed

[10] Erkens R., Esteban Y., Towe C., Schulert G., Vastert S. Pathogenesis and Treatment of Refractory Disease Courses in Systemic Juvenile Idiopathic Arthritis: Refractory Arthritis, Recurrent Macrophage Activation Syndrome and Chronic Lung Disease. Rheum. Dis. Clin. N. Am. 2021;47:585–606. doi: 10.1016/j.rdc.2021.06.003. - DOI - PubMed

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Systemic Juvenile Idiopathic Arthritis/Pediatric Still's Disease, a Syndrome but Several Clinical Forms: Recent Therapeutic Approaches

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Systemic Juvenile Idiopathic Arthritis/Pediatric Still's Disease, a Syndrome but Several Clinical Forms: Recent Therapeutic Approaches

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