Severe Systemic Lupus Erythematous with Lupus Nephritis and Anti Phospholipid Syndrome

Umar Muhammad Basalamah, dr. Cahya Dewi Satria, M.Kes, Sp.A(K)

2025 | Tesis-Spesialis | S2 Ilmu Kesehatan anak

Kami melaporkan kasus longitudinal seorang remaja perempuan usia 16 tahun dengan systemic lupus erythematosus (SLE) berat yang disertai lupus nefritis dan sindrom antifosfolipid (antiphospholipid syndrome/APS). Pasien datang dengan trombosis vena dalam ekstensif pada ekstremitas bawah kiri, dan diagnosis APS ditegakkan berdasarkan peningkatan persisten antibodi antikardiolipin IgM dan IgG. Keterlibatan ginjal ditetapkan melalui temuan hematuria dan proteinuria persisten, yang memenuhi kriteria lupus nefritis. Selama tindak lanjut 12 bulan, aktivitas penyakit berfluktuasi dengan skor SLEDAI 12–4, disertai efek samping kortikosteroid dan perkembangan fibrosis vena kronik. Pasien mendapatkan terapi kortikosteroid, asam mikofenolat, hidroksiklorokuin, inhibitor angiotensin-converting enzyme (ACE), serta siklofosfamid sebagai eskalasi terapi. Warfarin diberikan sebagai profilaksis sekunder tromboemboli. Selama pemantauan, status gizi dan kepatuhan terhadap terapi tetap baik. Kasus ini menegaskan tumpang tindih manifestasi klinis dan patofisiologi SLE dan APS pada remaja, serta pentingnya pendekatan terapeutik terindividualisasi dan pemantauan multidisiplin untuk mengoptimalkan luaran jangka panjang.

We report a longitudinal case of a 16-year-old girl diagnosed with severe systemic lupus erythematosus (SLE) complicated by lupus nephritis and antiphospholipid syndrome (APS). The patient initially presented with extensive deep vein thrombosis of the left lower extremity, later confirmed as APS with persistently elevated IgM and IgG anticardiolipin antibodies. Renal involvement was identified by persistent hematuria and proteinuria, fulfilling criteria for lupus nephritis. Over a 12?month follow-up, the patient exhibited fluctuating disease activity (SLEDAI 12–4), experienced steroid-induced side effects, and developed chronic venous fibrosis. She was managed with corticosteroids, mycophenolic acid, hydroxychloroquine, ACE inhibitors, and later cyclophosphamide, while warfarin provided secondary thrombosis prophylaxis. Despite recurrent disease activity, nutritional status and adherence remained good. This case highlights the overlapping pathophysiology of SLE and APS in adolescents, emphasizing the need for individualized immunosuppression, anticoagulation, and multidisciplinary follow-up to optimize long-term outcomes.

Kata Kunci : Systemic lupus erythematosus, Lupus nephritis, Antiphospholipid syndrome, Adolescents, Thrombosis