Systemic Juvenile Idiopathic Arthritis and Secondary Macrophage Activation Syndrome in Latvia from 2009 to 2020: A Nationwide Retrospective Study
Abstract
:1. Introduction
2. Materials and Methods
3. Results
4. Discussion
4.1. Limitations
4.2. Future Directions
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Year | No. of Patients | Children in Latvia | Incidence Per 100,000 Children |
---|---|---|---|
2009 | 3 | 388,449 | 0.8 |
2010 | 0 | 353,357 | 0 |
2011 | 4 | 360,216 | 1.1 |
2012 | 3 | 332,979 | 0.9 |
2013 | 4 | 347,018 | 1.2 |
2014 | 3 | 325,425 | 0.9 |
2015 | 8 | 348,660 | 2.3 |
2016 | 3 | 330,414 | 0.9 |
2017 | 0 | 356,527 | 0 |
2018 | 4 | 338,102 | 1.2 |
2019 | 2 | 358,813 | 0.6 |
2020 | 1 | 340,806 | 0.3 |
sJIA | MAS | |
---|---|---|
Fever | 100% (35/35) | 100% (10/10) |
Evanescent rash | 91.4% (32/35) | 90% (9/10) |
Arthritis/arthralgia | 88.6% (31/35) | 80% (8/10) |
Oligoarthritis | 45.7% (16/35) | 30% (3/10) |
Polyarthritis | 34.3% (12/35) | 30% (3/10) |
Arthralgia | 8.6% (3/35) | 20% (2/10) |
Lymphadenopathy | 68.6% (24/35) | 100% (10/10) |
Hepatomegaly | 62.9% (22/35) | 50% (5/10) |
Splenomegaly | 54.3% (19/35) | 50% (5/10) |
Myalgia | 31.4% (11/35) | 50% (5/10) |
Serositis | 14.3% (5/35) | 50% (5/10) |
Hemorrhagic rash | 17.1% (6/35) | 30% (3/10) |
Respiratory involvement | 11.4% (4/35) | - |
Gastrointestinal involvement | 8.6% (3/35) | - |
Cardiac involvement | 8.6% (3/35) | - |
Eye involvement | 8.6% (3/35) | 10% (1/10) |
Urogenital involvement | 2.9% (1/35) | - |
Nervous system involvement | 2.9% (1/35) | 20% (2/10) |
MAS Laboratory Criteria | Ferritin | Thrombocytes | ASAT | Triglycerides | Fibrinogen |
---|---|---|---|---|---|
>684 ng/mL | ≤181 × 103/L | >48 U/L | >1.76 mmol/L | ≤3.6 g/L | |
Patient 1 | 20,428 | 46 | 268 | 3.27 | 0.56 |
Patient 2 | 1479 | 1174 | 480 | N/A | 2.57 |
Patient 3 | 2722 | 231 | 42 | 2.52 | 3.08 |
Patient 4 | 14,611 | 98 | 231 | 4.92 | 0.96 |
Patient 5 | 5246 | 222 | 560 | 2.24 | 0.76 |
Patient 6 | 14,097 | 142 | 904 | 2.77 | 1.3 |
Patient 7 | 18,278 | 42 | 89 | 2.86 | 0.58 |
Patient 8 | 13,246 | 232 | 27 | 1.02 | 4.04 |
Patient 9 | 19,477 | 102 | 127 | 2.2 | 3.7 |
Patient 10 | 9856 | 175 | 80 | 2.18 | 2.59 |
sJIA | MAS | |
---|---|---|
NSAID 1 | 94.3% (33/35) | 90% (9/10) |
Corticosteroids | 100% (35/35) | 100% (10/10) |
Pulse form | 94.3% (33/35) | 100% (10/10) |
Peroral form | 100% (35/35) | 100% (10/10) |
Methotrexate | 74.3% (26/35) | 20% (2/10) |
Biological therapy | 48.6% (17/35) | 80% (8/10) |
Tocilizumab | 45.7% (16/35) | 80% (8/10) |
Anakinra | 2.9% (1/35) | - |
Cyclosporine | 28.6% (10/35) | 100% (10/10) |
Time Interval in Years | The Patient Number Who Stopped Medication in the Specified Time Interval | |||
---|---|---|---|---|
Methotrexate | Corticosteroid | Cyclosporine | Biological Therapy | |
<1y | 5 | 19 | 8 | 2 |
1-<2y | 4 | 7 | 2 | 9 |
2-<3y | 4 | 2 | 0 | 2 |
≥3y | 9 | 1 | 0 | 4 |
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Lukjanoviča, K.; Šlēziņa, I.; Dāvidsone, Z.; Šantere, R.; Budarina, K.; Staņēviča, V. Systemic Juvenile Idiopathic Arthritis and Secondary Macrophage Activation Syndrome in Latvia from 2009 to 2020: A Nationwide Retrospective Study. Medicina 2023, 59, 798. https://doi.org/10.3390/medicina59040798
Lukjanoviča K, Šlēziņa I, Dāvidsone Z, Šantere R, Budarina K, Staņēviča V. Systemic Juvenile Idiopathic Arthritis and Secondary Macrophage Activation Syndrome in Latvia from 2009 to 2020: A Nationwide Retrospective Study. Medicina. 2023; 59(4):798. https://doi.org/10.3390/medicina59040798
Chicago/Turabian StyleLukjanoviča, Kristīne, Ieva Šlēziņa, Zane Dāvidsone, Ruta Šantere, Kristīna Budarina, and Valda Staņēviča. 2023. "Systemic Juvenile Idiopathic Arthritis and Secondary Macrophage Activation Syndrome in Latvia from 2009 to 2020: A Nationwide Retrospective Study" Medicina 59, no. 4: 798. https://doi.org/10.3390/medicina59040798