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Epistaksis berulang sebagai manifestasi dari lupus erythematosus sistemik pediatrik: sebuah laporan kasus

Abstract

Background: Although hematologic manifestation is among diagnostic criteria of Systemic Lupus Erythematosus (pSLE), severe recurrent epistaxis is unusual. We aim to present the case with epistaxis which is a rare manifestation of pediatric SLE.

Case presentation: A 12 years old girl referred from type B Hospital with recurrent epistaxis due to suspected aplastic anemia. Epistaxis started since 4 month prior admitted, otherwise the most current epistaxis had occurred 7 days prior admitted. The patient also showed fever, weakness, and bruising on the right shin with laboratory result showed bicytopenia. Further complete examination showed systemic autoimmune process based on Systemic Lupus International Collaborating Clinics Classification (SLICC) criteria. Score for SLICC criteria for the patient was 4 prior serologic testing result, then criteria working diagnosis of pSLE was fulfill. Massive and recurrent epistaxis occured that refractory to thrombocyte concentrate transfusion and standard nose tampon, hence, high dose methylprednisolone (HDMP) for three consecutive days was initiated followed by cyclophosphamide. The immunosuppresssive treatment stop the epistaxis, thrombocytopenia, bruising, and general condition improved. Serologic testing showed titer Antinuclear antibody with indirect immunofluorescence method (ANA IF) 1:1000, Anti-double stranded DNA (AntidsDNA) 557.1 IU/mL, and C3 complement 47.1 mg/dL. Mallar rash as typical manifestation of SLE appeared after immunosuppresssive treatment. Complete evaluation emphasized diagnosis pSLE with total score 11 for SLICC criteria.

Conclusion: pSLE can showed severe unusual manifestations, clinicians need to find other subtle signs and symptoms of systemic autoimmune process for possibility of diagnosis pSLE to get prompt treatment for the patient.

 

Latar belakang: Meskipun manifestasi hematologi merupakan salah satu kriteria diagnostik dari Systemic Lupus Erythematosus (pSLE ), epistaksis berulang yang parah tidak biasa. Kami bertujuan untuk menyajikan kasus dengan epistaksis yang merupakan manifestasi langka dari SLE pediatrik.

Presentasi kasus: Seorang anak perempuan berusia 12 tahun dirujuk dari Rumah Sakit tipe B dengan epistaksis berulang karena diduga anemia aplastik. Epistaksis dimulai sejak 4 bulan sebelum masuk, sebaliknya epistaksis terbaru terjadi 7 hari sebelum masuk. Pasien juga menunjukkan demam, lemas, dan memar di tulang kering kanan dengan hasil laboratorium menunjukkan bisitopenia. Pemeriksaan lengkap lebih lanjut menunjukkan proses autoimun sistemik berdasarkan kriteria Systemic Lupus International Collaborating Clinics Classification (SLICC). Skor kriteria SLICC untuk pasien adalah 4 hasil pemeriksaan serologis sebelumnya, maka kriteria diagnosis kerja pSLE terpenuhi. Terjadi epistaksis masif dan berulang yang refrakter terhadap transfusi konsentrat trombosit dan tampon hidung standar, karenanya, metilprednisolon dosis tinggi (HDMP) selama tiga hari berturut-turut dimulai diikuti oleh siklofosfamid. Pengobatan imunosupresif menghentikan epistaksis, trombositopenia, memar, dan kondisi umum membaik. Tes serologi menunjukkan titer Antinuclear antibody dengan metode indirect immunofluorescence (ANA IF) 1:1000, Anti double stranded DNA (AntidsDNA) 557,1 IU/mL, dan komplemen C3 47.1 mg/dL. Ruam malar sebagai manifestasi khas SLE muncul setelah pengobatan imunosupresif . Evaluasi lengkap menekankan diagnosis pSLE dengan skor total 11 untuk kriteria SLICC .

Simpulan: pSLE dapat menunjukkan manifestasi parah yang tidak biasa, dokter perlu menemukan tanda dan gejala halus lainnya dari proses autoimun sistemik untuk kemungkinan diagnosis pSLE untuk mendapatkan perawatan yang cepat bagi pasien.

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How to Cite

I G. N. Citra Jiwa Pramana, Wati, K. D. K. ., Sutawan, I. B. R. ., Ariawati, K. ., Widnyana, A. ., Lastariana, K. A. Y., Ratnawati, L. M., & Silakarma, D. . (2023). Epistaksis berulang sebagai manifestasi dari lupus erythematosus sistemik pediatrik: sebuah laporan kasus. Medicina, 54(1), 28–33. https://doi.org/10.15562/medicina.v54i1.1231

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I G. N. Citra Jiwa Pramana
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Ketut Dewi Kumara Wati
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Ida Bagus Ramajaya Sutawan
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Kadek Ayu Yani Lastariana
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