ILLUSTRASION CASE MEDICINA 2019, Volume 50, Number 3: 543-549 P-ISSN.2540-8313, E-ISSN.2540-8321 Management of systemic lupus erythemathous with Illustrasion case polymyositis overlap syndrome Doi: http://dx.doi.org/10.15562/medicina.v50i3.575 Suryo Gading,* Ketut Dewi Kumara Wati, Komang Ayu Witarini, Hendra Santoso, I Gusti Ngurah Suwarba CrossMark Volume No.: 50 ABSTRACT There has been an increase in SLE cases among children in Sanglah of Rheumatology. Neurologic examination and electromyography General Hospital. In the rare case, there is a possibility SLE occurs were significant for the decrease in motoric power on the right lower Issue: 3 not as a single entity but overlap with another connective tissue limb, gastrocnemius atrophy, steppage gait, and reduction of the disease. Polymyositis is a disease with a primary symptom of muscle sensory sensation of right L4-S1 dermatome. Hence, the diagnose weakness associated with muscle pain and swollen. Polymyositis very of SLE and polymyositis was concluded. This is a case of SLE overlap rarely becomes overlapping syndrome with SLE, occurring in 4-6% syndrome with polymyositis. The patient was treated with prednisone First page No.: 543 of SLE patients. The aim of this study is to describe clinical findings 2 mg/kg/day for 2 weeks, and also given ibuprofen 10 mg/kg/dose for and management of SLE and Polymyositis. This case is a 12-year-old pain relief, continued with azathioprine plan for one year. The patient girl presented with arthralgia and myalgia since one month before showed an excellent result with the disappearance of symptoms and P-ISSN.2540-8313 admission, accompanied by a 1-month episode of relapsing fever, normal laboratory examination. The conclusion of the study is SLE decrease in appetite, facial rash, photosensitivity, muscle weakness overlap syndrome with polymyositis treated with prednisone and numbness and tingling sensation on the right foot. Diagnosis of ibuprofen continue with azathioprine showed good outcome with the E-ISSN.2540-8321 SLE was based on the diagnostic criteria of the American College disappearance of symptoms and normal laboratory examination. Keywords: Systemic Lupus Erythematous, polymyositis, children, overlap syndrome Cite This Article: Gading, S., Wati, K.D.K., Witarini, K.A., Santoso, H., Suwarba, I.G.N. 2019. Management of systemic lupus erythemathous with polymyositis overlap syndrome. Medicina 50(3): 543-549. DOI:10.15562/Medicina.v50i3.575 ABSTRAK Peningkatan kasus SLE terjadi pada anak di Rumah Sakit Umum elektromiografi signifikan menunjukkan penurunan kekuatan Sanglah, pada kasus yang jarang SLE bukan sebagai satu kesatuan motorik pada tungkai kanan bawah, atrofi gastrocnemius, steppage namun tumpang tindih dengan penyakit jaringan ikat lainnya. gait, dan penurunan sensasi sensorik pada dermatom L4-S1 kanan. Polymyositis adalah penyakit dengan gejala utama kelemahan Oleh karena itu dapat disimpulkan kasus ini adalah kasus sindrom otot yang berhubungan dengan nyeri otot. Polymyositis sangat SLE overlap dengan polymyositis. Pasien diobati dengan prednison jarang menjadi sindrom yang tumpang tindih dengan SLE, namun 2 mg/kg/hari selama 2 minggu, dan juga diberikan ibuprofen terjadi pada 4-6% pasien SLE. Tujuan dari penelitian ini adalah 10 mg/kg/dosis untuk menghilangkan rasa sakit, dilanjutkan untuk menggambarkan manajemen dan temuan klinis pada SLE dengan pemberian azathioprin yang direncanakan selama satu dan Polymyositis. Kasus ini seorang perempuan berusia 12 tahun tahun. Pasien menunjukkan hasil yang baik dengan hilangnya gejala yang mengalami artralgia dan mialgia selama 1 bulan, disertai dan pemeriksaan laboratorium normal. Kesimpulan dari penelitian demam selama 1 bulan, penurunan nafsu makan, ruam pada wajah, ini adalah sindrom SLE overlap dengan polymyositis yang diobati fotosensitifitas, mati rasa, kelemahan otot dan kesemutan pada dengan prednison dan ibuprofen dilanjutkan dengan azathioprin kaki kanan. Diagnosis SLE dibuat berdasarkan kriteria diagnostik menunjukkan hasil yang baik dengan hilangnya gejala serta hasil Department of Child Health, American College of Rheumatology. Pemeriksaan neurologis dan pemeriksaan laboratorium yang normal. Faculty of Medicine,Udayana University Denpasar Kata kunci : Systemic Lupus Erythematous, polymyositis, children, overlap syndrome *Correspondence to: Cite Pasal Ini: Gading, S., Wati, K.D.K., Witarini, K.A., Santoso, H., Suwarba, I.G.N. 2019. Management of systemic lupus erythemathous with Suryo Gading, Department of Child polymyositis overlap syndrome. Medicina 50(3): 543-549. DOI:10.15562/Medicina.v50i3.575 Health, Faculty of Medicine,Udayana University Denpasar [email protected] INTRODUCTION Diterima: 2018-12-26 Systemic Lupus Erythematous (SLE) is a hetero- causing widespread clinical manifestation on one Disetujui: 2019-04-08 genic autoimmune disease caused by the formation or more organs and pathologically characterized by Publis: 2019-12-01 of antibodies against our own cellular nucleus, wide inflammation of blood vessel and connective 543 ILLUSTRASION CASE tissue.1 The incidence of SLE varies accordingly in to another, except in the right leg where the pain each country; in the United States, the prevalence of persisted. The pain was not alleviated by rest. The SLE averaged 51/100.000 per population. In other pain was severe enough to impede walking and felt countries data revealed an estimated 2.9/100.000 – persistently throughout the day. Arthralgia was also 400/100.000.2 Women tend to be 9 times more prone accompanied by myalgia. The joint involved was to have SLE compared to men. Sixty-five per cent palpated to be warm. The numbness was thought of SLE occurred in the age of 16-55 years old. The from the right foot to the dorsum foot. The numb- aetiology of SLE is yet to be defined. Multifactorial ness and tingling sensation were felt persistently pathogenesis including genetics, environment, and hormonal component, is estimated to be involved. Monozygotic twins have a higher risk compared to dizygotic. Ninety percent of SLE patients were women, this is expected to be related to the effect of hormone against X chromosome, yet the under- lying mechanism is still undefined.3,4 SLE is clinically manifested by a chronical and relapsing onset of a disease, and symptoms included continuous or intermittent fever, red rashes, buccal ulcer, and arthritis. Patients also experienced constitutional symptoms including fatigue, decrease in body weight, and anorexia.3,5 The American College of Rheumatology (ACR) in 1997 or Systemic Lupus International Collaborating Clinics (SLICC) in 2012 classification is the stan- dard guideline in classifying SLE with a sensitivity of 96% and specificity of 100%.1,2 Recommended laboratory examination included inflammation indicator, autoantibody test, organ-specific func- tional tests and examinations to monitor ther- apeutic effect. Initially, during the acute phase, laboratory examination would reveal an increase in LED but a normal C-RP.2,3 Hematologic examina- tion could manifest as anaemia, leucopenia, and/or thrombocytopenia. Urinary examination manifest as proteinuria, hematuria, and a cast of heme glan- Figure 1 Spine asymetrical deformity dular, or red blood cell on the urine. Antinuclear antibody (ANA) test could be used as a screening test for patients with SLE, with a positive result in 90-100% of SLE patients. Anti-dsDNA is a pathog- nomic criterion for SLE.6,7,8 Management of SLE aims to control inflamma- tion, achieve remission phase, increase the quality of life, decrease exacerbation, prevent serious organ damage, and decrease death.1,2 The mainstay ther- apy for SLE is glucocorticoid with close monitor- ing of side effects. Other options include Disease Modifying Anti Rheumatic Drugs (DMARDs) types of drug. Non-steroid anti-inflammation drugs could be used to manage musculoskeletal symptoms.1,2 CASE ILLUSTRATION This is a case of 12 years and 9-month girl from Buleleng Bali. The Patient presented with arthralgia for 1 month before admission, 10th October 2016. The pain was reported to move from one joint Figure 2 Legs and gait disorder 544 Medicina 2019; 50(3): 543-549 | doi: 10.15562/Medicina.v50i3.575 ILLUSTRASION CASE Table 1 pGALS screening problems pGALS screening Documentation Pain symptoms? Right Leg Problem on using shirt? No problem Problem when walking? Problem when walking Visible Movement Gait + + Arms - - Legs + + Spine + - pGALS screening found problems including: Gait and Legs Table 2 Classification criteria of systemic lupus erithemathous SLICC 2012 criteria ACR 1997 criteria Requires ≥ 4 criteria (at least 1 clinical and 1 - Butterfly rash laboratory criteria) Clinical Criteria: - Discoid lupus eash - Acute cutaneous lupus - Photosensitivity - Chronic cutaneous lupus - Oral or nasal mucocutaneus lesion - Oral or nasal mucocutaneus ulceration - Non erosive arthritis - Alopecia - Nephritis - Arthritis - Proteinuria > 0,5 g/24 hours, positive cylinder cell - Serocitis - Enchepalopathy (seizure, psychosis) - Renal Involvement - Peluritis or pericarditis (serocytis) - Neurologic (convulsion, phycosis, myielitis - Cytopenia (leukopenia <4000/mm3, atau limfopenia <1500/mm3, atau trombositopenia <100.000/mm3, not caused by drugs - Hemolytic Anemia - Positive immuoserology - Leukopenia (<4000/µL) or limfopenia (<1000/µL) Antibody against dsDNA - Trombositopenia (<100.000/µL) Antibody against nuclear antigen Sm Antibodiyantifosfolipid positive, based on : Immunologic criteria: • Anticardiolipin antibody IgG