tatalaksana lupus nefritis, dr. edi hidayat

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LUPUS NEFRITIS DIAGNOSIS DAN TERAPI

DIAGNOSIS DAN TERAPI LUPUS NEFRITIS

EDI HIDAYAT

Dr. Edi HidayatDIVISI GINJAL HIPERTENSI FK UNSRI PALEMBANG1PENDAHULUANLupus Eritematosus Sistemik: penyakit autoimun melibatkan banyak organ dengan gejala klinik beragamMerusak jaringan akibat deposit antibodi & komplek imun pada berbagai organ tubuhEtiologi genetik, infeksi, lingkungan dan hormonalWanita > Pria = 5 : 1, usia 15-40 tahunPrevalensi keterlibatan ginjal pada LES 30-65%RSMH Januari 2001-September 2005 terdapat 56 kasus Lupus Nefritis2Systemic Lupus Erythematosus (SLE)Chronic multisystem inflammatory disease Associated with abnormalities of immune systemResults from interactions among genetic, hormonal, environmental, and immunologic factors 3Pemicu4

5Patofisiologi

Patogenesis SLE. Interaksi antara gen dan lingkungan memicu respon imun abnormal berupa autoantibodi dan kompleks imun yang terdeposit di jaringan, mengaktivasi komplemen dan menimbulkan inflamasi yang dapat berlanjut menjadi kerusakan organ yang ireversibel. Ag, antigen; C1q, complement system; C3, complement component; CNS, central nervous system; DC, dendritic cell; EBV, Epstein-Barr virus; HLA, human leukocyte antigen; FcR, immunoglobulin Fc-binding receptor; IL, interleukin; MBL, mannose-binding ligand; MCP, monocyte chemotactic protein; PTPN, phosphotyrosine phosphatase; UV, ultraviolet.6

7Clinical Manifestations

8Kriteria ARA adalah sebagai berikut :Kriteria Definisi1. Malar RashFixed malarerythema, flat or raised2. Discoid RashErythematosis raised patches with kara totic scalling and folikular plugging, atripic scaring may occur in older lesions.PhotosensitivitySkin rash as an unusual reaction to sunlight by patient history or physician observation.4. Oral UlcersOral or nasopharinged ulcer, usually painless, observed by physician.5. ArthritisNonerosive arhritis involving two or more peripheral joints, characteristic by tenderness, swelling or effusion.6. Serositisa. Pleuritis (convincing history of pleuritic pain or rub heard by Physician or evidence of pleural effosion).b.Pericarditis (documented by ECG, rub or evidence of pericardial Effusion).7. Renal Disordera. Persistent proteinuria (> 0,5 g/24 hours or + 3)b. Celluler Cast of any type.8. Neurologic Disordera. Seizure (in the absence of other causes)b. Phsycosis (in the absence of other causes)9. Hematologic Disordera. Haemolytic anemia, orb. Leukopenia (4000/mm3 on oner or more occassions)c. Lymphopenia (1500/mm3 on 2 or more occassions) ord.Trombocytopenia (100.000/mm3 in the absence of offending drugs)10.Immunologic Disordera. Positive LE cell preparation, orb. Anti double stranded DNA, orc. Anti Sm, ord. BFP (false positive serologic test for syphilis positive for at least 6 months with negative TPI or FTA).11. Anti Nuclear AntibodyAn abnormal titer of ANA by immunofluoresence or an equivalent assay at any time and in the absence of drugs known to be associated with drug induce lupus syndrome.9The most widely used classification criteria for SLE are those developed by the American College of Rheumatology (ACR)Criteria of ACR were published in 1982 and were revised by a committee in 1997Multiple groups of investigators used new statistical methodology to refine the criteria for classification of SLE. The Systemic Lupus International Collaborating Clinics (SLICC) group is an international group of investigators dedicated to SLE clinical research.

10Clinical criteriaAcute cutaneous lupus, including: Lupus malar rash (do not count if malar discoid)Chronic cutaneous lupus, Oral ulcers Alopecia Synovitis involving 2 or more jointsSerositis Renal Urine proteinto-creatinine ratio (or 24-hour urine protein) representing 500 mg protein/24 hoursNeurologic Hemolytic anemiaLeukopenia (