Ketut Dewi Kumara Wati
Department Of Pediatrics Faculty Of Medicine Udayana University

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Journal : Pediatric Sciences Journal

Challenging diagnostic of juvenile systemic sclerosis in limited sources: a case report Ronaldi, Melissa; Wati, Ketut Dewi Kumara; Gunawijaya, Eka; Yantie, Ni Putu Veny Kartika; Anandasari, Pande Putu Yuli
Pediatric Sciences Journal Vol. 5 No. 1 (2024): (Available online: 1 June 2024)
Publisher : Medical Faculty of Brawijaya University, Indonesia

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.51559/pedscij.v5i1.88

Abstract

Background: Juvenile Systemic Sclerosis (JSSc) is a rare chronic-autoimmune rheumatic disease leading to potential delay in diagnosis, especially in limited sources due to lack of knowledge and facility. This case report aimed to present a rare case of JSSc in limited sources of medical facilities. Case presentation: A 15-year-old girl was earlier suspected of having congenital heart disease due to the presentation of bluish fingers without any detectable clinical heart disease manifestation, including normal electrocardiography as well as echocardiography. The patient experienced pain and difficulty executing wrist endorotation, representing limited joint movement action. Further physical examinations showed pale, cold fingers and toes, bluish color in the peripheral represented sclerodactyly and arthritis. Skin showed multiple erythema-hypopigmentation papuls, in accordance with gottorn papule, gottorn sign. The antinuclear antibody titer was 1:320. Chest CT-Scan result was a fibrotic line at the inferior lobe of the left lung with the left diaphragm tenting due to a chronic inflammatory process. Skin biopsy shows an acanthotic epidermis, dermafibrocollagenous tissue with extensive fibrosis, and sclerosis with vascular proliferation. These findings meet JSSc PRES, ACR, and EULAR standard classification criteria, including 1 major and 4 minors. The treatment regimen was started with methylprednisolone 2 mg/kg/day, then tapering off once clinical manifestation alleviated, methotrexate 10 mg/BSA, and folic acid 1 mg a day. Long-term monitoring plans are also scheduled. Conclusion: Recognizing early symptoms of JSSc would provide a better outcome.
Co-Authors Adnyana, I Gusti Agung Ngurah Sugitha Ahmad Suryawan Anak Agung Ngurah Ketut Putra Widnyana Anak Agung Tri Yuliantini Anang Endaryanto Apsari, Putu Indah Budi Arini Setiawati Aryono Hendarto Ayu Setyorini Mestika Mayangsari Badriul Hegar Bagus Ngurah Putu Arhana BNP Arhana Budi Setiabudiawan Cempaka, Putu Mas Vina Paramitha Deborah Melati, Deborah Della Sabrina Marta Dewi Wulandari Dewi, Ni Luh Putu Mulia Laksmi Dina Muktiarti, Dina Dyah Kanya Wati Eka Gunawijaya Endy P. Prawirohartono Erfi Prafiantini Eva Jacomina Jemima Sapulete Gusti Ayu Putu Nilawati Hamid H A Haryadi Haryadi - Hendra Santoso Hendra Santoso Hendra Santoso Hindra Irawan Satari I Gusti Agung Ngurah Sugitha Adnyana I Gusti Ayu Putu Eka Pratiwi I gusti Lanang Sidhiarta I Gusti Lanang Sidiartha I Gusti Ngurah Sanjaya Putra I Ketut Suarta I Made Kardana I N Sartika I Nyoman Budi Hartawan I Nyoman Supadma I Nyoman Supadma I Putu Gede Karyana I Wayan Dharma Artana I Wayan Dharma Artana, I Wayan Dharma I Wayan Eka Satriawibawa I Wayan Gede Artawan Eka Putra I Wayan Gede Artawan Eka Putra, I Wayan Gede Artawan Eka I Wayan Gustawan Ida Bagus Krisna Jaya Sutawan Ida Bagus Subanada Irene Irene Johannes Hudyono Juergens, Stefanie Kadek Deddy Ariyanta Kadek Surya Jayanti, Kadek Surya Ketut Suarta Ketut Tangking Ketut Tuti Parwati Merati Komang Ayu Witarini Luh Putu Ratna Sundari Made Ardinata Made Bandem Kenny Wijaya Nugraha Made lndah Nastiti Utami Budha Maria Priskila Melisa Anggraeni Melva Louisa Merati, Tuti Parwati Metriani Nesa, Ni Nyoman Mohammad Juffrie Nesa, Ni Nyoman Metriani Ni Made Ayu Agustini Ni Nyoman Metriani Nesa Ni Putu Siadi Purniti Ni Putu Veny Kartika Yantie, Ni Putu Veny Kartika NP Veny Kartika Yantie Pande Putu Yuli Anandasari Purwoko, Agus Putu Ayu Widyanti Putu Ayunda Trisnia Putu Diah Vedaswari Putu Indah Budi Apsari Retno Asti Werdhani Ronaldi, Melissa Roni Naning Sawitri, Anak Agung Sagung Soetjiningsih Soetjiningsih Soetjiningsih Soetjiningsih Stefanie Juergens Suandi IKG Sumadiono Sumadiono supadma, I nyoman Susanto, Yohanes Widiantari, Ni Made Widiastari, A.A Ayu Widiastari, A.A Ayu Yvan Vandenplas Zakiudin Munasir