SYSTEMIC LUPUS ERYTHEMATOSUS, SISTITIS BERULANG, DAN ATRIAL SEPTAL DEFECT

KURNIAWATI ARIFAH, dr. Sumadiono, SpA(K).; Dr.dr. Pudjo Hagung W, PhD, SpA(K).

2017 | Tesis-Spesialis | SP ILMU KESEHATAN ANAK

Systemic lupus erythematosus (SLE) merupakan peyakit autoimun kronis, multisistem, dengan periode peningkatan aktivitas penyakit akibat peradangan di pembuluh darah dan jaringan ikat. SLE memiliki karakteristik klinis yang bersifat periodik, saat remisi tanpa gejala, saat periode flare (kambuh) pasien dapat mengalami berbagai morbiditas yang berat. SLE anak memiliki perjalanan penyakit yang lebih berat dibandingkan penderita dewasa karena keterlibatan organ lebih banyak dan perjalanan penyakit yang lebih berat. Mengingat manifestasi klinis perjalanan penyakit SLE sangat beragam dan risiko timbulnya sekuel pada SLE anak cukup tinggi, maka diperlukan pemantauan rutin untuk mengetahui aktivitas penyakit, respon pengobatan, efek samping pengobatan pada pasien SLE anak untuk meningkatkan kualitas hidup pasien. Pasien terdiagnosis SLE sejak Juni 2013, dari 11 kriteria SLE oleh ACR 1997, didapati adanya ulkus oral, serositis berupa efusi pleura bilateral minimal, nefritis, gangguan hematologi berupa pansitopenia, didukung hasil lab dsDNA dan ANA test positif. Diagnosis SLE ditegakkan dan mulai diberikan pengobatan sesuai protokol SLE berat karena adanya nefritis lupus. Selama terapi tersebut, anak mengalami ISK berulang. Evaluasi echokardiografi yang semula dilakukan untuk melihat ada tidaknya pericarditis menunjukkan adanya kelainan jantung berupa Atrial septal defect (ASD). Selama pengamatan kondisi anak baik, ASD menutup spontan, dan tidak terjadi infeksi berulang.

Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease with a highly variable clinical course. The disease course varies from a mild disease with long quiescence phases to a severe disease with frequent flares requiring long-term immunosuppressive therapy and leading to considerable organ damage. Pediatric patients with SLE have a more severe clinical course in comparison with their adult counterparts. Patients with SLE have an incurable, potentially devastating disease that occurs during a vulnerable period of psychosocial development, leading to specific and unique psychosocial stressors. A long term follow up studies in pediatric SLE patient are needed to better understand the disease prognosis and to promote patients optimal growth and development. The patient diagnosed as SLE since June 2013. She presented with oral ulcer, pleural effusion, nephritis, pancytopenia, positive dsDNA, and positive ANA test, fulfilling 6 of the 11 American College of Rheumatology (ACR) criteria for the classification of SLE. She got severe SLE treatment protocol. During those treatment, she had recurrent urinary tract infection (UTI) and major infection that required antibiotic treatment for more than 7 days, Echocardiography evaluation detected atrial septal defect (ASD) which previously asymptomatic. After a full course of SLE protocol, she achieved partial remission, the ASD was spontaneously closed, and recurrent infection episodes decreased.

Kata Kunci : Systemic lupus erythematosus, atrial septal defect, infeksi, Systemic lupus erythematosus, atrial septal defect, infection