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1. AB1701 IS THERE A DIFFERENCE IN THE NUMBER OF INVOLVED ORGAN SYSTEMS BETWEEN JUVENILE DIFFUSE AND LIMITED SUBTYPE SYSTEMIC SCLEROSIS PATIENTS?

Author

Foeldvari, I., primary, Klotsche, J., additional, Torok, K., additional, Kasapcopur, O., additional, Adrovic, A., additional, Feldman, B., additional, Antón, J., additional, Johnson, S. R., additional, Sztajnbok, F. R., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Khubchandani, R., additional, Stanevicha, V., additional, Schonenberg, D., additional, Al-Abadi, E., additional, Alexeeva, E., additional, Katsikas, M., additional, Sawhney, S., additional, Smith, V., additional, Appenzeller, S., additional, Avcin, T., additional, Kostik, M., additional, Lehman, T., additional, Malcova, H., additional, Marrani, E., additional, Pain, C., additional, Patwardhan, A., additional, Sifuentes-Giraldo, W. A., additional, Vasquez-Canizares, N., additional, Abu Al Saoud, S., additional, Costa Reis, P., additional, Janarthanan, M., additional, Moll, M., additional, Nemcova, D., additional, Santos, M. J., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Kaiser, D., additional, Kallinich, T., additional, Lazarevic, D., additional, Minden, K., additional, Nuruzzaman, F., additional, Opsahl Hetlevik, S., additional, Sparchez, M., additional, Uziel, Y., additional, and Helmus, N., additional

Published
2024
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2. POS0758 PRESENCE OF NAILFOLD CAPILLARY CHANGES CORRELATES WITH MORE SEVERE ORGAN INVOLVEMENT IN JUVENILE SYSTEMIC SCLERODERMA. RESULTS OF THE JUVENILE SCLERODERMA INCEPTION COHORT.

Author

Foeldvari, I., primary, Klotsche, J., additional, Torok, K., additional, Kasapcopur, O., additional, Adrovic, A., additional, Feldman, B., additional, Antón, J., additional, Johnson, S. R., additional, Sztajnbok, F. R., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Stanevicha, V., additional, Schonenberg, D., additional, Alexeeva, E., additional, Katsikas, M., additional, Khubchandani, R., additional, Sawhney, S., additional, Smith, V., additional, Al-Abadi, E., additional, Appenzeller, S., additional, Avcin, T., additional, Kostik, M., additional, Lehman, T., additional, Malcova, H., additional, Marrani, E., additional, Pain, C., additional, Patwardhan, A., additional, Sifuentes-Giraldo, W. A., additional, Vasquez-Canizares, N., additional, Abu Al Saoud, S., additional, Costa Reis, P., additional, Janarthanan, M., additional, Moll, M., additional, Nemcova, D., additional, Santos, M. J., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Kaiser, D., additional, Kallinich, T., additional, Lazarevic, D., additional, Minden, K., additional, Nielsen, S., additional, Nuruzzaman, F., additional, Opsahl Hetlevik, S., additional, Sparchez, M., additional, Uziel, Y., additional, and Helmus, N., additional

Published
2024
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3. POS0770 DIFFERENCES IN CLINICAL PRESENTATION BETWEEN JUVENILE SYSTEMIC SCLEROSIS PATIENTS WITH DIFFUSE AND LIMITED SUBTYPE. RESULTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT.

Author

Foeldvari, I., primary, Klotsche, J., additional, Torok, K., additional, Kasapcopur, O., additional, Adrovic, A., additional, Feldman, B., additional, Antón, J., additional, Johnson, S. R., additional, Sztajnbok, F. R., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Khubchandani, R., additional, Stanevicha, V., additional, Schonenberg, D., additional, Al-Abadi, E., additional, Alexeeva, E., additional, Katsikas, M., additional, Sawhney, S., additional, Smith, V., additional, Appenzeller, S., additional, Avcin, T., additional, Kostik, M., additional, Lehman, T., additional, Malcova, H., additional, Marrani, E., additional, Pain, C., additional, Patwardhan, A., additional, Sifuentes-Giraldo, W. A., additional, Vasquez-Canizares, N., additional, Abu Al Saoud, S., additional, Costa Reis, P., additional, Janarthanan, M., additional, Moll, M., additional, Nemcova, D., additional, Santos, M. J., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Kaiser, D., additional, Kallinich, T., additional, Lazarevic, D., additional, Minden, K., additional, Nuruzzaman, F., additional, Opsahl Hetlevik, S., additional, Sparchez, M., additional, Uziel, Y., additional, and Helmus, N., additional

Published
2024
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4. POS0773 STRATIFICATION OF PEDIATRIC SAPHO SYNDROME BASED ON SKIN MANIFESTATIONS: RESULTS FROM AN ITALIAN MULTICENTRIC STUDY (SAPHOPED)

Author

Matucci-Cerinic, C., primary, Attico, A., additional, Malattia, C., additional, Consolaro, A., additional, Rosina, S., additional, Breda, L., additional, La Bella, S., additional, Cattalini, M., additional, Ricci, F., additional, Conti, G., additional, Civino, A., additional, Licciardi, F., additional, Baldini, L., additional, Insalaco, A., additional, La Torre, F., additional, Pastore, S., additional, Filocamo, G., additional, Beretta, G. B., additional, Simonini, G., additional, Marrani, E., additional, Pistorio, A., additional, Volpi, S., additional, Caorsi, R., additional, Ruperto, N., additional, Viglizzo, G., additional, and Gattorno, M., additional

Published
2024
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7. POS0139 DIFFUSE JUVENILE SYSTEMIC SCLEROSIS PATIENTS SHOW DISTINCT ORGAN INVOLVEMENT, ANTIBODY PATTERN AND HAVE SIGNIFICANTLY MORE SEVERE DISEASE IN THE LARGEST JSSC COHORT OF THE WORLD. RESULTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT

Author

Foeldvari, I., primary, Klotsche, J., additional, Torok, K., additional, Kasapcopur, O., additional, Adrovic, A., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Feldman, B., additional, Sztajnbok, F. R., additional, Antón, J., additional, Stanevicha, V., additional, Johnson, S., additional, Khubchandani, R., additional, Schonenberg, D., additional, Al-Abadi, E., additional, Alexeeva, E., additional, Katsikas, M., additional, Sawhney, S., additional, Smith, V., additional, Appenzeller, S., additional, Avcin, T., additional, Kostik, M., additional, Lehman, T., additional, Malcova, H., additional, Marrani, E., additional, Pain, C., additional, Patwardhan, A., additional, Sifuentes-Giraldo, W. A., additional, Vasquez-Canizares, N., additional, Costa Reis, P., additional, Janarthanan, M., additional, Moll, M., additional, Nemcova, D., additional, Santos, M. J., additional, Abu Al Saoud, S., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Kaiser, D., additional, Kallinich, T., additional, Lazarevic, D., additional, Minden, K., additional, Nielsen, S., additional, Nuruzzaman, F., additional, Opsahl Hetlevik, S., additional, Uziel, Y., additional, and Helmus, N., additional

Published
2023
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8. POS0140 PATIENT AND PHYSICIAN REPORTED OUTCOMES OF JUVENILE SYSTEMIC SCLEROSIS PATIENTS SIGNIFICANTLY IMPROVE OVER 24 MONTHS OBSERVATION PERIOD IN THE JUVENILE SYSTEMIC SCLERODERMA INCEPTION COHORT

Author

Foeldvari, I., primary, Klotsche, J., additional, Kasapcopur, O., additional, Adrovic, A., additional, Torok, K., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Antón, J., additional, Feldman, B., additional, Khubchandani, R., additional, Kostik, M., additional, Lehman, T., additional, Marrani, E., additional, Katsikas, M., additional, Nemcova, D., additional, Santos, M. J., additional, Sztajnbok, F. R., additional, Appenzeller, S., additional, Battagliotti, C., additional, Berntson, L., additional, Brunner, J., additional, Harel, L., additional, Horneff, G., additional, Johnson, S., additional, Kallinich, T., additional, Minden, K., additional, Moll, M., additional, Nuruzzaman, F., additional, Patwardhan, A., additional, Schonenberg, D., additional, and Helmus, N., additional

Published
2023
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9. POS0141 APPLICATION OF CRISS SCORE, REVISED CRISS SCORE AND RCID SCORE IN PATIENTS WITH JUVENILE SYSTEMIC SCLEROSIS

Author

Klotsche, J., primary, Foeldvari, I., additional, Torok, K., additional, Del Galdo, F., additional, Furst, D., additional, Kasapcopur, O., additional, Adrovic, A., additional, Feldman, B., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Sztajnbok, F. R., additional, Antón, J., additional, Katsikas, M., additional, Stanevicha, V., additional, Appenzeller, S., additional, Avcin, T., additional, Johnson, S., additional, Kostik, M., additional, Malcova, H., additional, Marrani, E., additional, Sifuentes-Giraldo, W. A., additional, Khubchandani, R., additional, Nemcova, D., additional, Santos, M. J., additional, Schonenberg, D., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Kallinich, T., additional, Lehman, T., additional, Minden, K., additional, Moll, M., additional, Nielsen, S., additional, Patwardhan, A., additional, Smith, V., additional, and Helmus, N., additional

Published
2023
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12. Diffuse juvenile systemic sclerosis patients show distinct organ involvement, antibody pattern and have significantly more severe disease in the largest jSSc cohort of the world. Results from the juvenile scleroderma inception cohort

Author

Foeldvari, I, Klotsche, J, Torok, K, Kasapcopur, O, Adrovic, A, Terreri, MT, Sakamoto, AP, Feldman, B, Sztajnbok, F, Anton, J, Stanevicha, V, Johnson, S, Khubchandani, R, Schonenberg, D, Al-Abadi, E, Alexeeva, E, Katsicas, M, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Kostik, M, Lehman, T, Malcova, H, Marrani, E, Pain, C, Patwardhan, A, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Costa Reis, P, Janarthanan, M, Nemcova, D, Santos, MJ, Abu Al-Saoud, S, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Eleftheriou, D, Harel, L, Horneff, G, Kaiser, D, Kallinich, T, Lazarevic, D, Minden, K, Nielsen, S, Nuruzzaman, F, Opsahl Hetlevik, S, Uziel, Y, Helmus, N, Foeldvari, I, Klotsche, J, Torok, K, Kasapcopur, O, Adrovic, A, Terreri, MT, Sakamoto, AP, Feldman, B, Sztajnbok, F, Anton, J, Stanevicha, V, Johnson, S, Khubchandani, R, Schonenberg, D, Al-Abadi, E, Alexeeva, E, Katsicas, M, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Kostik, M, Lehman, T, Malcova, H, Marrani, E, Pain, C, Patwardhan, A, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Costa Reis, P, Janarthanan, M, Nemcova, D, Santos, MJ, Abu Al-Saoud, S, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Eleftheriou, D, Harel, L, Horneff, G, Kaiser, D, Kallinich, T, Lazarevic, D, Minden, K, Nielsen, S, Nuruzzaman, F, Opsahl Hetlevik, S, Uziel, Y, and Helmus, N

Published
2023

13. Is decreased body mass index-2 z score or less correlating with an organ involvement pattern? Results from the juvenile scleroderma inception cohort

Author

Foeldvari, I, Klotsche, J, Torok, K, Kasapcopur, O, Adrovic, A, Feldman, B, Sztajnbok, F, Anton, J, Terreri, MT, Sakamoto, AP, Khubchandani, R, Stanevicha, V, Al-Abadi, E, Alexeeva, E, Katsicas, M, Sawhney, S, Schonenberg, D, Smith, V, Appenzeller, S, Avcin, T, Lehman, T, Malcova, H, Marrani, E, Patwardhan, A, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Costa Reis, P, Janarthanan, M, Johnson, S, Kostik, M, Nemcova, D, Pain, C, Santos, MJ, Abu Al-Saoud, S, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Eleftheriou, D, Harel, L, Horneff, G, Kaiser, D, Kallinich, T, Lazarevic, D, Minden, K, Nuruzzaman, F, Opsahl Hetlevik, S, Uziel, Y, Helmus, N, Foeldvari, I, Klotsche, J, Torok, K, Kasapcopur, O, Adrovic, A, Feldman, B, Sztajnbok, F, Anton, J, Terreri, MT, Sakamoto, AP, Khubchandani, R, Stanevicha, V, Al-Abadi, E, Alexeeva, E, Katsicas, M, Sawhney, S, Schonenberg, D, Smith, V, Appenzeller, S, Avcin, T, Lehman, T, Malcova, H, Marrani, E, Patwardhan, A, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Costa Reis, P, Janarthanan, M, Johnson, S, Kostik, M, Nemcova, D, Pain, C, Santos, MJ, Abu Al-Saoud, S, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Eleftheriou, D, Harel, L, Horneff, G, Kaiser, D, Kallinich, T, Lazarevic, D, Minden, K, Nuruzzaman, F, Opsahl Hetlevik, S, Uziel, Y, and Helmus, N

Published
2023

14. Patient and physician reported outcomes of juvenile systemic sclerosis patients significantly improve over 24 months observation period in the juvenile systemic scleroderma inception cohort

Author

Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, MT, Sakamoto, AP, Anton, J, Feldman, B, Khubchandani, R, Kostik, M, Lehman, T, Marrani, E, Katsicas, M, Nemcova, D, Santos, MJ, Sztajnbok, F, Appenzeller, S, Battagliotti, C, Berntson, L, Brunner, J, Harel, L, Horneff, G, Johnson, S, Kallinich, T, Minden, K, Moll, M, Nuruzzaman, F, Patwardhan, A, Schonenberg, D, Helmus, N, Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, MT, Sakamoto, AP, Anton, J, Feldman, B, Khubchandani, R, Kostik, M, Lehman, T, Marrani, E, Katsicas, M, Nemcova, D, Santos, MJ, Sztajnbok, F, Appenzeller, S, Battagliotti, C, Berntson, L, Brunner, J, Harel, L, Horneff, G, Johnson, S, Kallinich, T, Minden, K, Moll, M, Nuruzzaman, F, Patwardhan, A, Schonenberg, D, and Helmus, N

Published
2023

15. Application of CRISS score, revised CRISS score and RCID score in patients with Juvenile systemic sclerosis

Author

Klotsche, J, Foeldvari, I, Torok, K, Del Gado, F, Furst, DE, Kasapcopur, O, Adrovic, A, Feldman, B, Terreri, MT, Sakamoto, AP, Sztajnbok, F, Anton, J, Katsicas, M, Stanevicha, V, Appenzeller, S, Avcin, T, Johnson, S, Kostik, M, Malcova, H, Marrani, E, Sifuentes-Giraldo, WA, Khubchandani, R, Nemcova, D, Santos, MJ, Schonenberg, D, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Eleftheriou, D, Harel, L, Horneff, G, Kallinich, T, Lehman, T, Minden, K, Moll, M, Nielsen, S, Patwardhan, A, Smith, V, Helmus, N, Klotsche, J, Foeldvari, I, Torok, K, Del Gado, F, Furst, DE, Kasapcopur, O, Adrovic, A, Feldman, B, Terreri, MT, Sakamoto, AP, Sztajnbok, F, Anton, J, Katsicas, M, Stanevicha, V, Appenzeller, S, Avcin, T, Johnson, S, Kostik, M, Malcova, H, Marrani, E, Sifuentes-Giraldo, WA, Khubchandani, R, Nemcova, D, Santos, MJ, Schonenberg, D, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Eleftheriou, D, Harel, L, Horneff, G, Kallinich, T, Lehman, T, Minden, K, Moll, M, Nielsen, S, Patwardhan, A, Smith, V, and Helmus, N

Published
2023

16. POS0172 DIFFUSE JUVENILE SYSTEMIC SCLEROSIS PATIENTS SHOW DISTINCT ORGAN INVOLVEMENT AND HAVE MORE SEVERE DISEASE IN THE LARGEST jSSc COHORT OF THE WORLD. RESULTS FROM THE THE JUVENILE SCLERODERMA INCEPTION COHORT. www.juvenile-scleroderma.com

Author

Foeldvari, I., primary, Klotsche, J., additional, Kasapcopur, O., additional, Adrovic, A., additional, Torok, K., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Feldman, B., additional, Sztajnbok, F. R., additional, Stanevicha, V., additional, Anton, J., additional, Johnson, S., additional, Khubchandani, R., additional, Alexeeva, E., additional, Katsikas, M., additional, Sawhney, S., additional, Smith, V., additional, Appenzeller, S., additional, Avcin, T., additional, Kostik, M., additional, Lehman, T., additional, Malcova, H., additional, Marrani, E., additional, Pain, C., additional, Schonenberg, D., additional, Sifuentes-Giraldo, W. A., additional, Vasquez-Canizares, N., additional, Costa Reis, P., additional, Janarthanan, M., additional, Moll, M., additional, Nemcova, D., additional, Patwardhan, A., additional, Santos, M. J., additional, Abu Al Saoud, S., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Cimaz, R., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Kaiser, D., additional, Kallinich, T., additional, Lazarevic, D., additional, Minden, K., additional, Nielsen, S., additional, Nuruzzaman, F., additional, Opsahl Hetlevik, S., additional, Uziel, Y., additional, and Helmus, N., additional

Published
2022
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17. AB1236 CLINICAL CHARACTERISTICS OF JUVENILE ONSET SYSTEMIC SCLEROSIS PATIENTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT COMPARED TO ADULT AGE JUVENILE-ONSET PATIENTS FROM EUSTAR. ARE THESE DIFFERENCES SUGGESTING RISK FOR MORTALITY?

Author

Foeldvari, I., primary, Klotsche, J., additional, Carreira, P., additional, Kasapcopur, O., additional, Torok, K., additional, Airò, P., additional, Iannone, F., additional, Allanore, Y., additional, Balbir-Gurman, A., additional, Schmeiser, T., additional, Sztajnbok, F. R., additional, Terreri, M. T., additional, Stanevicha, V., additional, Anton, J., additional, Feldman, B., additional, Khubchandani, R., additional, Alexeeva, E., additional, Johnson, S., additional, Katsikas, M., additional, Sawhney, S., additional, Smith, V., additional, Appenzeller, S., additional, Avcin, T., additional, Campochiaro, C., additional, De Vries-Bouwstra, J., additional, Kostik, M., additional, Lehman, T., additional, Marrani, E., additional, Schonenberg, D., additional, Sifuentes-Giraldo, W. A., additional, Vasquez-Canizares, N., additional, Janarthanan, M., additional, Malcova, H., additional, Moll, M., additional, Nemcova, D., additional, Patwardhan, A., additional, Santos, M. J., additional, Seskute, G., additional, Truchetet, M. E., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Cimaz, R., additional, Costa Reis, P., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Kaiser, D., additional, Kallinich, T., additional, Lazarevic, D., additional, Minden, K., additional, Nielsen, S., additional, Nuruzzaman, F., additional, Opsahl Hetlevik, S., additional, Uziel, Y., additional, Veale, D., additional, Hoffmann-Vold, A. M., additional, Gabrielli, A., additional, and Distler, O., additional

Published
2022
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18. POS1302 PATIENT AND PHYSICIAN REPORTED OUTCOMES OF JUVENILE SYSTEMIC SCLEROSIS PATIENTS SIGNIFICANTLY IMPROVE OVER 12 MONTHS OBSERVATION PERIOD IN THE JUVENILE SYSTEMIC SCLERODERMA INCEPTION COHORT. www.juvenile-scleroderma.com

Author

Foeldvari, I., primary, Klotsche, J., additional, Kasapcopur, O., additional, Adrovic, A., additional, Torok, K., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Feldman, B., additional, Anton, J., additional, Katsikas, M., additional, Stanevicha, V., additional, Sztajnbok, F. R., additional, Appenzeller, S., additional, Avcin, T., additional, Kostik, M., additional, Marrani, E., additional, Sifuentes-Giraldo, W. A., additional, Johnson, S., additional, Khubchandani, R., additional, Nemcova, D., additional, Santos, M. J., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Cimaz, R., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Janarthanan, M., additional, Kallinich, T., additional, Minden, K., additional, Moll, M., additional, Nielsen, S., additional, Patwardhan, A., additional, Schonenberg, D., additional, Smith, V., additional, and Helmus, N., additional

Published
2022
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19. POS1299 JUVENILE SYSTEMIC SCLEROSIS TREATMENT PRACTICES IN AN INTERNATIONAL COHORT AND COMPARISON TO RECENT SHARE CONSENSUS GUIDELINES.

Author

Foeldvari, I., primary, Torok, K., additional, Kasapcopur, O., additional, Adrovic, A., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Feldman, B., additional, Anton, J., additional, Sztajnbok, F. R., additional, Stanevicha, V., additional, Appenzeller, S., additional, Avcin, T., additional, Johnson, S., additional, Khubchandani, R., additional, Kostik, M., additional, Marrani, E., additional, Sifuentes-Giraldo, W. A., additional, Nemcova, D., additional, Santos, M. J., additional, Schonenberg, D., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Cimaz, R., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Janarthanan, M., additional, Kallinich, T., additional, Lehman, T., additional, Moll, M., additional, Nuruzzaman, F., additional, Patwardhan, A., additional, Smith, V., additional, and Helmus, N., additional

Published
2022
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20. Diffuse juvenile systemic sclerosis patients show distinct organ involvement and have more severe disease in the largest jSSc cohort of the world. Results from the the juvenile scleroderma inception cohort

Author

Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, MT, Sakamoto, AP, Feldman, B, Sztajnbok, F, Stanevicha, V, Anton, J, Johnson, S, Khubchandani, R, Alexeeva, E, Katsicas, M, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Kostik, M, Lehman, T, Malcova, H, Marrani, E, Pain, C, Schonenberg, D, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Costa Reis, P, Janarthanan, M, Moll, M, Nemcova, D, Patwardhan, A, Santos, MJ, Abu Al-Saoud, S, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Cimaz, R, Eleftheriou, D, Harel, L, Horneff, G, Kaiser, D, Kallinich, T, Lazarevic, D, Minden, K, Nuruzzaman, F, Opsahl Hetlevik, S, Uziel, Y, Helmus, N, Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, MT, Sakamoto, AP, Feldman, B, Sztajnbok, F, Stanevicha, V, Anton, J, Johnson, S, Khubchandani, R, Alexeeva, E, Katsicas, M, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Kostik, M, Lehman, T, Malcova, H, Marrani, E, Pain, C, Schonenberg, D, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Costa Reis, P, Janarthanan, M, Moll, M, Nemcova, D, Patwardhan, A, Santos, MJ, Abu Al-Saoud, S, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Cimaz, R, Eleftheriou, D, Harel, L, Horneff, G, Kaiser, D, Kallinich, T, Lazarevic, D, Minden, K, Nuruzzaman, F, Opsahl Hetlevik, S, Uziel, Y, and Helmus, N

Published
2022

21. Juvenile systemic sclerosis treatment practices in an international cohort and comparison to recent SHARE consensus guidelines

Author

Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, MT, Sakamoto, AP, Feldman, B, Anton, J, Sztajnbok, F, Stanevicha, V, Appenzeller, S, Avcin, T, Johnson, S, Khubchandani, R, Kostik, M, Marrani, E, Sifuentes-Giraldo, WA, Nemcova, D, Santos, MJ, Schonenberg, D, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Cimaz, R, Eleftheriou, D, Harel, L, Horneff, G, Janarthanan, M, Kallinich, T, Lehman, T, Moll, M, Nuruzzaman, F, Patwardhan, A, Smith, V, Helmus, N, Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, MT, Sakamoto, AP, Feldman, B, Anton, J, Sztajnbok, F, Stanevicha, V, Appenzeller, S, Avcin, T, Johnson, S, Khubchandani, R, Kostik, M, Marrani, E, Sifuentes-Giraldo, WA, Nemcova, D, Santos, MJ, Schonenberg, D, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Cimaz, R, Eleftheriou, D, Harel, L, Horneff, G, Janarthanan, M, Kallinich, T, Lehman, T, Moll, M, Nuruzzaman, F, Patwardhan, A, Smith, V, and Helmus, N

Published
2022

22. Patient and physician reported outcomes of juvenile systemic sclerosis patients significantly improve over 12 months observation period in the juvenile systemic scleroderma inception cohort

Author

Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, MT, Feldman, B, Anton, J, Katsicas, M, Stanevicha, V, Sztajnbok, F, Appenzeller, S, Avcin, T, Kostik, M, Marrani, E, Sifuentes-Giraldo, WA, Johnson, S, Khubchandani, R, Nemcova, D, Santos, MJ, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Cimaz, R, Eleftheriou, D, Harel, L, Horneff, G, Janarthanan, M, Kallinich, T, Minden, K, Moll, M, Nielsen, S, Patwardhan, A, Schonenberg, D, Smith, V, Helmus, N, Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, MT, Feldman, B, Anton, J, Katsicas, M, Stanevicha, V, Sztajnbok, F, Appenzeller, S, Avcin, T, Kostik, M, Marrani, E, Sifuentes-Giraldo, WA, Johnson, S, Khubchandani, R, Nemcova, D, Santos, MJ, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Cimaz, R, Eleftheriou, D, Harel, L, Horneff, G, Janarthanan, M, Kallinich, T, Minden, K, Moll, M, Nielsen, S, Patwardhan, A, Schonenberg, D, Smith, V, and Helmus, N

Published
2022

23. Clinical characteristics of juvenile onset systemic sclerosis patients from the juvenile scleroderma inception cohort compared to adult age juvenile-onset patients from EUSTAR. Are these differences suggesting risk for mortality?

Author

Foeldvari, I, Klotsche, J, Carreira, PE, Kasapcopur, O, Adrovic, A, Torok, K, Airò, P, Iannone, F, Allanore, Y, Balbir-Gurman, A, Schmeiser, T, Sztajnbok, F, Terreri, MT, Stanevicha, V, Anton, J, Feldman, B, Khubchandani, R, Alexeeva, E, Johnson, S, Katsicas, M, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Campochiaro, C, De Vries-Bouwstra, J, Kostik, M, Lehman, T, Marrani, E, Schonenberg, D, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Janarthanan, M, Malcova, H, Moll, M, Nemcova, D, Patwardhan, A, Santos, MJ, Seskute, G, Truchetet, ME, Veale, D, Hoffmann-Vold, AM, Gabrielli, A, Distler, O, Foeldvari, I, Klotsche, J, Carreira, PE, Kasapcopur, O, Adrovic, A, Torok, K, Airò, P, Iannone, F, Allanore, Y, Balbir-Gurman, A, Schmeiser, T, Sztajnbok, F, Terreri, MT, Stanevicha, V, Anton, J, Feldman, B, Khubchandani, R, Alexeeva, E, Johnson, S, Katsicas, M, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Campochiaro, C, De Vries-Bouwstra, J, Kostik, M, Lehman, T, Marrani, E, Schonenberg, D, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Janarthanan, M, Malcova, H, Moll, M, Nemcova, D, Patwardhan, A, Santos, MJ, Seskute, G, Truchetet, ME, Veale, D, Hoffmann-Vold, AM, Gabrielli, A, and Distler, O

Published
2022

25. Patients with juvenile systemic sclerosis have a distinct pattern of organ involvement. Results from thejuvenile systemic sclerosis inception cohort

Author

Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, MT, Sakamoto, AP, Feldman, B, Stanevicha, V, Anton, J, Sztajnbok, F, Khubchandani, R, Alexeeva, E, Katsicas, M, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Kostik, M, Lehman, T, Marrani, E, Schonenberg, D, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Janarthanan, M, Moll, M, Nemcova, D, Patwardhan, A, Santos, MJ, and Helmus, N

Subjects
ddc: 610, 610 Medical sciences, Medicine
Abstract

Introduction: Juvenile systemic sclerosis (jSSc) is a rare disease with a prevalence of around 3 in 1,000,000 children. To better capture the clinical manifestations of jSSc the juvenile systemic sclerosis inception cohort (jSScC, [link:https://www.juvenile-scleroderma.com/*https://www.juvenile-scleroderma.com/])[for full text, please go to the a.m. URL], Deutscher Rheumatologiekongress 2021, 49. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 35. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), Wissenschaftliche Herbsttagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)

Published
2021
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26. POS1304 JUVENILE SYSTEMIC SCLEROSIS (JSSC) PATIENTS WITH OVERLAP CHARACTERISTICS DO NOT HAVE MILD DISEASE. RESULTS FROM THE JSSC INCEPTION COHORT. WWW.JUVENILESCLERODERMA.COM

Author

Foeldvari, I., primary, Klotsche, J., additional, Kasapcopur, O., additional, Adrovic, A., additional, Torok, K., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Feldman, B., additional, Stanevicha, V., additional, Anton, J., additional, Sztajnbok, F. R., additional, Khubchandani, R., additional, Alexeeva, E., additional, Katsikas, M., additional, Sawhney, S., additional, Smith, V., additional, Appenzeller, S., additional, Avcin, T., additional, Kostik, M., additional, Lehman, T., additional, Marrani, E., additional, Schonenberg, D., additional, Sifuentes-Giraldo, W. A., additional, Vasquez-Canizares, N., additional, Janarthanan, M., additional, Moll, M., additional, Nemcova, D., additional, Patwardhan, A., additional, Santos, M. J., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Cimaz, R., additional, Costa Reis, P., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Johnson, S., additional, Kaiser, D., additional, Kallinich, T., additional, Lazarevic, D., additional, Minden, K., additional, Nielsen, S., additional, Nuruzzaman, F., additional, Opsahl Hetlevik, S., additional, Uziel, Y., additional, and Helmus, N., additional

Published
2021
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27. POS0751 COMORBIDITY AND LONG-TERM OUTCOME IN PATIENTS WITH CONGENITAL HEART BLOCK: PRELIMINARY DATA OF THE ITALIAN REGISTRY ON THE IMMUNE-MEDIATED CONGENITAL HEART BLOCK

Author

Fredi, M., primary, Rizzo, G., additional, Andreoli, L., additional, Bacco, B., additional, Bertero, T., additional, Bortoluzzi, A., additional, Ceccarelli, F., additional, Cimaz, R., additional, Conigliaro, P., additional, Corradi, F., additional, De Vita, S., additional, DI Poi, E., additional, Elefante, E., additional, Emmi, G., additional, Gerosa, M., additional, Govoni, M., additional, Hoxha, A., additional, Lojacono, A., additional, Marrani, E., additional, Marozio, L., additional, Mathieu, A., additional, Mosca, M., additional, Melissa, P., additional, Picchi, C., additional, Piga, M., additional, Priori, R., additional, Ramoni, V., additional, Ruffatti, A., additional, Simonini, G., additional, Tani, C., additional, Tonello, M., additional, Trespidi, L., additional, Urban, M. L., additional, Vezzoli, M., additional, Zatti, S., additional, Calza, S., additional, Brucato, A., additional, Franceschini, F., additional, and Tincani, A., additional

Published
2021
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28. POS0079 PATIENTS WITH JUVENILE SYSTEMIC SCLEROSIS HAVE A DISTINCT PATTERN OF ORGAN INVOLVEMENT.RESULTS FROM THE JUVENILE SYSTEMIC SCLEROSIS INCEPTION COHORT. WWW.JUVENILE-SCLERODERMA.COM

Author

Foeldvari, I., primary, Klotsche, J., additional, Kasapcopur, O., additional, Adrovic, A., additional, Torok, K., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Feldman, B., additional, Stanevicha, V., additional, Anton, J., additional, Sztajnbok, F. R., additional, Khubchandani, R., additional, Alexeeva, E., additional, Katsikas, M., additional, Sawhney, S., additional, Smith, V., additional, Appenzeller, S., additional, Avcin, T., additional, Kostik, M., additional, Lehman, T., additional, Marrani, E., additional, Schonenberg, D., additional, Sifuentes-Giraldo, W. A., additional, Vasquez-Canizares, N., additional, Janarthanan, M., additional, Moll, M., additional, Nemcova, D., additional, Patwardhan, A., additional, Santos, M. J., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Cimaz, R., additional, Costa Reis, P., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Johnson, S., additional, Kaiser, D., additional, Kallinich, T., additional, Lazarevic, D., additional, Minden, K., additional, Nielsen, S., additional, Nuruzzaman, F., additional, Opsahl Hetlevik, S., additional, Uziel, Y., additional, and Helmus, N., additional

Published
2021
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29. Differences sustained between diffuse and limited forms of juvenile systemic sclerosis in expanded international cohort. www.juvenile-scleroderma.com

Author

Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Terreri MT, Sakamoto AP, Stanevicha V, Sztajnbok F, Anton-Lopez J, Feldman B, Alexeeva E, Katsicas M, Smith V, Avcin T, Marrani E, Kostik M, Lehman T, Sifuentes-Giraldo WA, Vasquez-Canizares N, Appenzeller S, Janarthanan M, Moll M, Nemcova D, Patwardhan A, Santos MJ, Sawhney S, Schonenberg-Meinema D, Battagliotti C, Berntson L, Bica B, Brunner J, Costa-Reis P, Eleftheriou D, Harel L, Horneff G, Kaiser D, Kallinich T, Lazarevic D, Minden K, Nielsen S, Nuruzzaman F, Uziel Y, Helmus N, and Torok KS

Abstract

OBJECTIVES: To evaluate the baseline clinical characteristics of juvenile systemic sclerosis (jSSc) patients in the international Juvenile SSc Inception Cohort (jSScC), compare these characteristics between the classically defined diffuse (dcjSSc) and limited cutaneous (lcjSSc) subtypes, and among those with overlap features. METHODS: A cross-sectional study was performed using baseline visit data. Demographic, organ system evaluation, treatment, and patient and physician reported outcomes were extracted and summary statistics applied. Comparisons between dcjSSc and lcSSc subtypes and patients with and without overlap features were performed using Chi-square and Mann Whitney U-tests. RESULTS: At data extraction 150 jSSc patients were enrolled across 42 centers, 83% were Caucasian, 80% female, dcjSSc predominated (72%), and 17% of the cohort had overlap features. Significant differences were found between dcjSSc and lcjSSc regarding the modified Rodnan Skin Score, presence of Gottron's papules, digital tip ulceration, 6 Minute walk test, composite pulmonary and cardiac involvement. All more frequent in dcSSc except for cardiac involvement. DcjSSc patients had significantly worse scores for physician rated disease activity and damage. A significantly higher occurrence of Gottron's papules, musculoskeletal involvement and composite pulmonary involvement, and significantly lower frequency of Raynaud's phenomenon, were seen in those with overlap features. CONCLUSION: Results from a large international jSSc cohort demonstrate significant differences between dcjSSc and lcjSSc patients including more globally severe disease and increased frequency of ILD in dcjSSc patients, while those with lcSSc have more frequent cardiac involvement. Those with overlap features had an unexpected higher frequency of interstitial lung disease.

Published
2021

30. Patients with Juvenile Systemic Sclerosis Have a Distinct Pattern of Organ Involvement: Results from the Juvenile Systemic Sclerosis Inception Cohort

Author

Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, M, Sakamoto, AP, Sztajnbok, F, Feldman, B, Stanevicha, V, Anton-Lopez J, Khubchandani, R, Alexeeva, E, Johnson, S, Katsicas, MM, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Kostik, M, Lehman, T, Marrani, E, Schonenberg-Meinema, D, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Janarthanan, M, Malcova, H, Moll, M, Nemcova, D, Patwardhan, A, Santos, MJ, Battagliotti, C, Berntson, L, Bica, BERG, Brunner, J, Cimaz, R, Reis, PC, Eleftheriou, D, Harel, L, Horneff, G, Kaiser, D, Kallinich, T, Lazarevic, D, Minden, K, Nielsen, S, Nuruzzaman, F, Hetlevik, SO, Uziel, Y, and Helmus, N

Published
2021

31. Male Juvenile Systemic Sclerosis Patients Have More Severe Disease: Results from the International Juvenile Scleroderma Inception Cohort

Author

Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, M, Sakamoto, AP, Sztajnbok, F, Feldman, B, Stanevicha, V, Anton-Lopez J, Khubchandani, R, Alexeeva, E, Johnson, S, Katsicas, MM, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Kostik, M, Lehman, T, Marrani, E, Schonenberg-Meinema, D, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Janarthanan, M, Malcova, H, Moll, M, Nemcova, D, Patwardhan, A, Santos, MJ, Battagliotti, C, Berntson, L, Bica, BERG, Brunner, J, Cimaz, R, Reis, PC, Eleftheriou, D, Harel, L, Horneff, G, Kaiser, D, Kallinich, T, Lazarevic, D, Minden, K, Nielsen, S, Nuruzzaman, F, Hetlevik, SO, Uziel, Y, and Helmus, N

Published
2021

32. Juvenile systemic sclerosis (jSSc) patients with overlap characteristics do not have mild disease.Results from thejSSc inception cohort

Author

Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, MT, Sakamoto, AP, Feldman, B, Stanevicha, V, Anton, J, Sztajnbok, F, Khubchandani, R, Alexeeva, E, Katsicas, M, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Kostik, M, Lehman, T, Marrani, E, Schonenberg, D, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Janarthanan, M, Moll, M, Nemcova, D, Patwardhan, A, Santos, MJ, Helmus, N, Foeldvari, I, Klotsche, J, Kasapcopur, O, Adrovic, A, Torok, K, Terreri, MT, Sakamoto, AP, Feldman, B, Stanevicha, V, Anton, J, Sztajnbok, F, Khubchandani, R, Alexeeva, E, Katsicas, M, Sawhney, S, Smith, V, Appenzeller, S, Avcin, T, Kostik, M, Lehman, T, Marrani, E, Schonenberg, D, Sifuentes-Giraldo, WA, Vasquez-Canizares, N, Janarthanan, M, Moll, M, Nemcova, D, Patwardhan, A, Santos, MJ, and Helmus, N

Published
2021

33. Persistence of disease flares is associated to an inadequate colchicine dose in familial Mediterranean fever: a national multicentre longitudinal study

Author

Bustaffa, M, Mazza, F, Sutera, D, Carrabba, Md, Alessio, M, Cantarini, L, Obici, L, Rigante, D, Maggio, Mc, Insalaco A., Simonini G, Cattalini, M, Conti, G, Olivieri, An, Barone, P, Miniaci, A, Moressa, V, Magnolia, Mg, Breda, L, Montin, D, Spagnolo, A, Fabio, G, Orlando, F, Gaggiano, C, Mussinelli, R, Capozio, G, Celani, C, Marrani, E, Ricci, F, Calzatini, F, Lancieri, M, Ruperto, N, Gattorno, M, Gallizzi, R, Rigante D (ORCID:0000-0001-7032-7779), Conti G (ORCID:0000-0002-8566-9365), Bustaffa, M, Mazza, F, Sutera, D, Carrabba, Md, Alessio, M, Cantarini, L, Obici, L, Rigante, D, Maggio, Mc, Insalaco A., Simonini G, Cattalini, M, Conti, G, Olivieri, An, Barone, P, Miniaci, A, Moressa, V, Magnolia, Mg, Breda, L, Montin, D, Spagnolo, A, Fabio, G, Orlando, F, Gaggiano, C, Mussinelli, R, Capozio, G, Celani, C, Marrani, E, Ricci, F, Calzatini, F, Lancieri, M, Ruperto, N, Gattorno, M, Gallizzi, R, Rigante D (ORCID:0000-0001-7032-7779), and Conti G (ORCID:0000-0002-8566-9365)

Abstract

This study aimed at identifying the oersistence od disease in patients with FMF treated with colchicine. Almost 30% of FMF patients display a persistent disease activity not fulfilling the definition of colchicine resistance, but impacting on their quality of life. In most of these patients colchicine is under-dosed and maximum recommended dose is rarely used.

Published
2021

35. Under detection of interstitial lung disease in juvenile systemic sclerosis (jSSc)

Author

Foeldvari I, Klotsche J, Hinrichs B, Helmus N, Kasapcopur O, Adrovic A, Sztajnbok F, Terreri MT, Anton-Lopez J, Smith V, Katsicas M, Kostik M, Vasquez-Canizares N, Avcin T, Feldman B, Janarthanan M, Santos MJ, Sawhney S, Schonenberg-Meinema D, Sifuentes-Giraldo WA, Alexeeva E, Appenzeller S, Battagliotti C, Berntson L, Bica B, Costa Reis P, Eleftheriou D, Kallinich T, Lehman T, Marrani E, Minden K, Nielsen S, Nuruzzaman F, Patwardhan A, Khubchandani R, Stanevicha V, Uziel Y, and Torok KS

Subjects
respiratory system, behavioral disciplines and activities, respiratory tract diseases
Abstract

OBJECTIVES: Utilizing data obtained from a prospective international juvenile systemic sclerosis cohort (jSScC) to determine if pulmonary screening with forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (DLCO) is sufficient to assess the presence of interstitial lung disease (ILD) in comparison to high resolution computed tomography (HRCT) in jSSc. METHODS: The jSScC cohort database was queried for patients enrolled from January 2008 to January 2020 with recorded pulmonary function tests (PFT) parameters and HRCT to determine the discriminatory properties of PFTs parameters, FVC and DLCO, in detecting ILD. RESULTS: Eighty-six jSSc patients had both CT imaging and FVC values for direct comparison. Using findings on HRCT as the standard measure of ILD presence, the sensitivity of FVC in detecting ILD in jSSc was only 40%, the specificity was 77%, and AUC was 0.58. Fifty-eight jSSc patients had both CT imaging and DLCO values for comparison. The sensitivity of DLCO in detecting ILD was 76%, the specificity was 70%, and AUC was 0.73. CONCLUSION: The performance of PFTs in jSSc to detect underlying ILD was quite limited. Specifically, the FVC, which is one of the main clinical parameters in adult SSc to detect and monitor ILD, would miss approximately 60% of children that had ILD changes on their accompanying HRCT. The DLCO was more sensitive in detecting potential abnormalities in HRCT, but with less specificity than the FVC. These results support the use of HRCT in tandem with PFTs for the screening of ILD in jSSc.

Published
2020

36. SAT0500 HOW THE ADULT CRISS WORKS IN PEDIATRIC jSSc PATIENTS - RESULTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT

Author

Klotsche, J., primary, Foeldvari, I., additional, Kasapcopur, O., additional, Adrovic, A., additional, Torok, K., additional, Stanevicha, V., additional, Anton, J., additional, Marrani, E., additional, Terreri, M. T., additional, Sztajnbok, F. R., additional, Battagliotti, C., additional, Berntson, L., additional, Eleftheriou, D., additional, Horneff, G., additional, Nuruzzaman, F., additional, and Helmus, N., additional

Published
2020
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37. THU0499 IS THERE A DIFFERENT PRESENTATION OF JUVENILE SYSTEMIC DIFFUSE AND LIMITED SUBSET? DATA FROM THE JUVENILE SCLERODERMA INCEPTION COHORT. WWW.JUVENILE-SCLEORDERMA.COM

Author

Foeldvari, I., primary, Klotsche, J., additional, Kasapcopur, O., additional, Adrovic, A., additional, Torok, K., additional, Terreri, M. T., additional, Sakamoto, A. P., additional, Stanevicha, V., additional, Sztajnbok, F. R., additional, Anton, J., additional, Feldman, B., additional, Alexeeva, E., additional, Katsikas, M., additional, Smith, V., additional, Marrani, E., additional, Kostik, M., additional, Vasquez-Canizares, N., additional, Appenzeller, S., additional, Janarthanan, M., additional, Moll, M., additional, Nemcova, D., additional, Patwardhan, A., additional, Santos, M. J., additional, Sawhney, S., additional, Schonenberg, D., additional, Battagliotti, C., additional, Berntson, L., additional, Bica, B., additional, Brunner, J., additional, Costa Reis, P., additional, Eleftheriou, D., additional, Harel, L., additional, Horneff, G., additional, Kaiser, D., additional, Lazarevic, D., additional, Minden, K., additional, Nielsen, S., additional, Nuruzzaman, F., additional, Uziel, Y., additional, and Helmus, N., additional

Published
2020
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38. Comparing ultraviolet light A photo(chemo)therapy with Methotrexate protocol in childhood localized scleroderma: Evidence from systematic review and meta-analysis approach

Author

Marrani E, Foeldvari I, Anton-Lopez J, Cimaz R, and Simonini G

Subjects
Disease modifying therapy, Scleroderma, sense organs, Ultraviolet treatment, skin and connective tissue diseases, Pediatric rheumatology
Abstract

OBJECTIVE: Localized scleroderma is a skin fibrosing disorder that, if untreated, may result in severe disability. The purpose of this systematic review is to compare the present evidence concerning the effectiveness of Methotrexate versus phototherapy, alone or associated with Psoralen, in childhood localized scleroderma. METHOD: A systematic search between January 1996 and May 2017 was performed to identify studies investigating the efficacy of Methotrexate (MTX) or phototherapy (UVA) for treating localized scleroderma with onset =18 years. Due to a lack of validated clinical criteria, four clinical response criteria were used to assess the treatment efficacy as primary outcome. We determined a combined estimate of the proportion of children responding to MTX and UVA. RESULTS: A total of 19 studies was included (8 MTX; 11 UVA). In the methotrexate group, 193 children were included in the analysis; in the phototherapy group, a total of 48 treated children. For both groups age, disease subtype, glucocorticoids (GCs) use, and side effects of treatment were also analyzed. The meta-analysis suggested that UVA and MTX protocols have both a favorable effect in active lesions of childhood localized scleroderma. However, MTX resulted significantly superior to UVA, with or without Psoralen. CONCLUSION: Our study supports the combination of MTX and GCs in patients with a high risk of complication. Phototherapy with UVA1 could represent a therapeutic option in patients with limited scleroderma, where lesions do not cross joints and they do not lead to potential cosmetic changes.

Published
2018

42. PATIENT AND PHYSICIAN REPORTED OUTCOMES OF JUVENILE SYSTEMIC SCLEROSIS PATIENTS SIGNIFICANTLY IMPROVE OVER 24 MONTHS OBSERVATION PERIOD IN THE JUVENILE SYSTEMIC SCLERODERMA INCEPTION COHORT.

Author

Foeldvari, I., Klotsche, J., Kasapcopur, O., Adrovic, A., Torok, K., Terreri, M. T., Sakamoto, A. P., Antón, J., Feldman, B., Khubchandani, R., Kostik, M., Lehman, T., Marrani, E., Katsikas, M., Nemcova, D., Santos, M. J., Sztajnbok, F. R., Appenzeller, S., Battagliotti, C., and Berntson, L.

Published
2023
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44. Serum amyloid-A in Behçet's disease.

Author

Vitale, A, Rigante, Donato, Lopalco, F, Brizi, Mg, Caso, F, Franceschini, R, Denaro, R, Galeazzi, M, Punzi, L, Iannone, F, Lapadula, G, Simpatico, A, Marrani, E, Costa, L, Cimaz, R, Cantarini, L., Rigante, Donato (ORCID:0000-0001-7032-7779), Vitale, A, Rigante, Donato, Lopalco, F, Brizi, Mg, Caso, F, Franceschini, R, Denaro, R, Galeazzi, M, Punzi, L, Iannone, F, Lapadula, G, Simpatico, A, Marrani, E, Costa, L, Cimaz, R, Cantarini, L., and Rigante, Donato (ORCID:0000-0001-7032-7779)

Abstract

Serum amyloid-A (SAA) is an acute phase protein, synthesized by the liver and previously investigated as a marker of disease activity in many rheumatologic disorders. Its significance in Behçet’s disease (BD), a chronic inflammatory disorder at the crossroad between autoimmune and autoinflammatory syndromes, is still unraveled. Our aim was to assess the role of SAA levels as a potential marker of disease activity in patients with BD. According to our findings, the occurrence of oral aphthosis, neurological impairment, and ocular disease is significantly associated with SAA serum levels higher than 30, 50, and 150 mg/L, respectively. We also suggest that increased SAA levels might identify a thrombotic risk in BD with previous or concurrent vascular involvement.

Published
2014

50. Sodium thiosulfate for the treatment of calcinosis secondary to juvenile dermatomyositis

Author

Ilaria Pagnini, Simonini, G., Giani, T., Marrani, E., Moretti, D., Vannucci, G., Cantarini, L., and Cimaz, R.

Subjects
Male, Treatment Outcome, Antioxidants, Calcinosis, Child, Preschool, Dermatomyositis, Humans, Thiosulfates, Child, Preschool
Abstract

We report the successful use of sodium thiosulfate in a patient with juvenile dermatomyositis complicated by ulcerative skin disease and progressive calcinosis. This therapy may have a role in improving calcinosis, even if more studies are necessary to determine the safety and efficacy of this treatment in juvenile dermatomyositis-related calcinosis.

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