MODERATE SLE WITH LUPUS NEFRITIS

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1 MODERATE SLE WITH LUPUS NEFRITIS Blondina Marpaung, Faisal Sinurat Rheumatology Division Departement of Internal Medicine University of North Sumatra ABSTRAK Medical Faculty of University of North Sumatra Lupus nephritis is one of the most serious manifestations of systemic lupus erythematosus (LES) and usually appear within 5 years after diagnosis. Lupus nephritis is histologically evident in most patients with SLE, even those who do not show clinical manifestations of kidney disease. Symptoms of lupus nephritis is generally associated with hypertension, proteinuria and renal failure. Reported a case of systemic lupus erythematosus (SLE) with severe lupus nephritis in a man 23-years old boy in the hospital Haji Adam Malik attending with a swollen face since 2 weeks ago accompanied by abdominal enlargement and swelling in the extremities. Patients also complained pallor and fatigue, joint pain, sores in the mouth and gums encountered, red rash on the arms and legs encountered. On physical examination found vital signs within normal limits, encountered anemia, weakened vesicular breath sounds in both lower courts of the lungs, additional sound not found. Ascites was found, discoid rash was found on both arms and legs, accompanied by edema in both arms and legs. On blood examination discovered hemoglobin level 9.7 mg / d, leukocytes 1,560 /ul; platelets 50,000 /ul. Urinalysis results obtained proteinuria 2 Positive, erythrocyte sediment 2-3 / LPB, leukocytes 2-5 / LPB, urine protein 545 (+++) mg%, 24-hour urine protein 5,450 mg, urea level 71 mg / dl, creatinine 1.31 mg / dl, uric acid 8.7 mg / dl, albumin 2.1 g /dl, CRP qualitative: negative, ANA test 161, anti ds-dna: 409 ng / ml. On chest x-ray found bilateral pleural effusion and bronkhopenumonia. In renal ultrasound examination was found in accordance with nephrotic pattern. Patients treated by injection of methylprednisolone puls therapy at a dose of 500 mg / day for 3 days, followed by administration of a dose of methyl prednisolone tablets from 0.5 to 0.6 mg / day with Cellcef (Mycophenolate Mofetil) 2 x 500 mg, furosemide injection, Captopril 2 x 6.25 mg. Diets high in protein (an extra egg white), and fluid balance ml. After administration of methylprednisolone pulse therapy for 3 days followed by administration of a cortico-steroid-sparing agent (MMF), proteinuria was reduced, edema is reduced and the patient's condition improves. Kata kunci: lupus nephritis, SLE, pulse therapy PENDAHULUAN 1

2 Lupus nephritis is one of the most serious manifestations of systemic lupus erythematosus (SLE) and usually appear within 5 years after diagnosis. Lupus nephritis is histologically evident in most patients with SLE, even those who do not show clinical manifestations of kidney disease. Symptoms of lupus nephritis is generally associated with hypertension, proteinuria and renal failure. In America, the prevalence of SLE is 1 case per 2000 inhabitants in the general population. Because of the difficulty of diagnosis and likely many cases go undetected, most researchers suggest that the prevalence may be closer to 1 case per 500-1,000 population. There is stil no prevalence data of SLE in Indonesia until now. Number of patients with SLE in Indonesia according to the Lupus Foundation of Indonesia (YLI) up to 2005 is estimated at 5,000 people. 1,2 CASE REPORT A man - 23-year old came to Haji Adam Malik Hospital Medan on April 16th 2014 with the main complaints is a swollen face (myxo-oedema ), This is experienced since 3 weeks before admission to the hospital. Swollen face, especially in the morning and decreases in the afternoon. accompanied by swelling in the legs. Since two weeks ago the patients also complained of enlargement of the abdomen. The patient complained of joint pain, especially in the shoulders, arms and knees. The pain is constantly moderate intensity, relieved by pain-killers, but reappeared when the patients stopped taking medication. Patients also complained reddish spots on both of his arms, hands and fingers, and became evident in the first week. Rash on the face was not found, hair loss was not found. Complaints of pain in the face and skin with redness if exposed to the sun was not found. History of pain in the mouth with sores on both corners of the lips was found. Pallor experienced since 3 months ago and more pronounced in the next 2 weeks. The history of Melena, haematochezia, History of Haematuria, bleeding gums, epistaxis denied by the patient. Bruises history - bruises on the skin was not be found. Pale in the face accompanied with fatigue and dizziness was found, especially during activity. History of disease like this before was denied by the patient, history of family members who suffer from the diseases was not found. On physical examination found sensorium compos mentis, blood pressure of 120/60 mmhg, pols 84 x / min, frequency of breathing 22 x / min. Anaemia was found, weakened vesicular breath sounds in both lower field of the lungs, additional sound not found. In abdominal examination, ascites was found. Tenderness in the shoulder joint and joints in 2

3 both hands and tenderness in both of knee joints was found. Discoid rash found on both arms and legs, accompanied by edema in both arms and legs. Routine blood examination found : hemoglobin level 9.7 mg / dl, hematocrit 28%, leukocytes 1,560 / ul, platelets 50,000 / mm3, MCV: 81 fl, MCH: 27.7 ρg, MCHC: 34.4 g%, counts (%): Neutrophils 51.3; Lymphocytes 35.3; Monocytes 12.8; Eosinophils 0; 0.6 basophils. Peripheral blood morphology suggest: Anemia normokrom + Leukopenia + Thrombocytopenia. Hemostatic function; Bleeding Time: 3'30 "minutes, Fibrinogen mg / dl, D-dimer was 352 ng / ml, Ferritin ng / ml, Iron 25μg / dl, TIBC 195 mg / dl. Hepatitis Immunoserology test, non-reactive for HBsAg, Anti-HCV Non-Reactive, Anti HIV (3 Methode) Non-Reactive, Anti HIV (Rapid I) Non-Reactive, Auto immune examination of ANA test 161, Anti-ds DNA 409, Qualitative CRP <0.7 mg / dl. Examination of Bone Marrow Puncture suggest: Myelodysplastic Syndrome (WHO Classification: RCMD). Urinalysis examination: protein obtained positive 2, the reduction (-), bilirubin (-), sediment 2-3 leukocytes / LPB, erythrocyte 2-3 / LPB, Epithelium: (-); Crystal (-); Casts: (-). The Urinary volume : ml / 24 hours, urine protein: 545 (+++) mg%, 24-hour urine protein: 5450 mg. Level of Ureum 71 (19-44) mg / dl, Creatinine: 1,31mg / dl, Uric Acid: 8.7 mg / dl. Liver function tests: Total Bilirubin 0.40 mg / dl, Direct Bilirubin 0.10 mg / dl, alkaline phosphatase (ALP) 73 U / L, AST / SGOT 95 U / L, ALT / SGPT 87 U / L, Gamma - GT 149 U / L, Total Protein 4,1g / dl, Albumin 2,1g / dl, Globulin 2.0 g / dl. Examination of electrolytes: sodium 137 meq / L, potassium 4.2 meq / L, chloride 106 meq / L, total cholesterol 121mg / dl, triglycerides 299 mg / dl, HDL cholesterol 21 mg / dl, LDL cholesterol 36 mg / dl, levels random blood glucose: 61.7 mg / dl. In the chest x ray examination found bilateral pleural effusions and Bronkhopneumonia. In renal ultrasound examination was found in accordance with nephrotic picture pattern. Patients diagnosed with moderate SLE with lupus nephritis + hipoalbumin. Patients treated by injection of methylprednisolone puls therapy at a dose of 500 mg / day for 3 days, followed by administration of a dose of methyl prednisolone tablets from 0.5 to 0.6 mg / day with Cellcef (Mycophenolate Mofetil) 2 x 500 mg, furosemide injection, Captopril 2 x 6.25 mg. Diets high in protein (an extra egg white), and fluid balance ml. the patient's condition improved, edema is reduced, discoid rash reduced, reduced of joints pain, and proteinuria was reduced and planned to discharge from the hospital, with medication of methylprednisolone 3 2-2, Cellcef (Mycophenolate Mofetil) 2 x 500 mg, Captopril 2 x 6.25 mg, furosemide tablet 2 x 4 mg. 3

4 DISCUSSION Renal involvement in LES is a manifestation of a common disease and is a strong predictor of a poor outcome. The prevalence of kidney disease in 8 large cohort study consisted of 2649 patients LES varies between 31-65%. A study analyzed the annual incidence of lupus nephritis in 384 patients at Johns Hopkins Medical Center between 1992 to 1994, and found the incidence of acute kidney disease by 10 percent. 3 Based on data from Asia, renal involvement ranges from 6-100% overall. 3 The diagnosis of SLE is made when we found more or equal to 4 criteria among the 11 criteria of the American Rheumatism Association (ARA). Hypertension in LES can occur as a complication of renal disease or as a side effect of steroid. 3-5 In these patients the diagnosis of LES is made based on the criteria of ARA are discoid rash, a history of joint pain on waist and knees, pleural effusion, abnormal Renal function, proteinuria settled 5,450 mg / 24 hour, hematologic abnormalities such as anemia, leukopenia and thrombocytopenia, immunological disorders such as anti ds-dna positive and the ANA test is increased. Diagnosis of Lupus nephritis clinically can be enforced if found renal abnormalities such as proteinuria with or without hematuria, hypertension, acute glomerulonephritis, nephrotic syndrome, decreased renal function and rapid decline of renal function. Proteinuria, or pathological urinary sediment on urinalysis examination, indicate the presence of lupus nephritis. Renal biopsy is needed to reveal the anatomical pathology of the kidneys to determine the classification of lupus nephritis to decide the treatment and the prognosis after treatment. If the patient is not willing to be biopsied or not allowed to be biopsied because of the physical situation, it would require an assessment of clinical symptoms are as follows: number Proteinuri, hematuria, hypertension, nephrotic syndrome, renal function impairment. 3,7 4

5 Table 1.Diagnostic Criteria of LES by American Rheumatism Association (ARA) 6 5

6 Table 2. Clnical Manifestations of Lupus Nefritis. 6 Nefritis Lupus Proteinuria Hematuria Hipertensi Sindrom Nefrotik Gangguan fungsi Ginjal Kelas I 1 gr/24 jam Tidak ada Tidak ada Tidak ada N Kelas II 1-3 gr/24 jam Tidak ada Tidak ada Tidak ada N Kelas III >3gr/24 jam Ada Ada Ada kreatinin pada 25-35% pada 25% pasien pasien Kelas IV >3 gr/24 jam Sering sering sering kreatinin pada 50% pasien Kelas V > 3 gr/24 jam Ya/tidak Ya/tidak Sering N atau Kelas VI 1 gr/24 jam Ya/tidak Ya/tidak Ya/tidak lambat In these patients found proteinuria positive 2 and 24 hours proteinuria 5,450 mg, and mild renal impairments, so that clinically diagnosed as lupus nephritis class III / IV. Although without a renal biopsy examination, the patient is clinically severe cases that require more aggressive treatment. This patients renal biopsy examination was done, but the results are inadequate biopsy sampling. Treatment of lupus nephritis is within a class like this: a. Class I Lupus Nefritis Does not require any specific treatment. Treatment is directed to extra renal symptoms. b. Class II Lupus Nefritis If it is not accompanied by significant proteinuria and urinary sediment is not active, it does not require specific treatment. If accompanied by Proteinuri, anti ds DNA titers were high with hematuria, given prednisone mg / kg / day for 6-12 weeks. Then slowly reduced (titrate) the doses (5-10 weeks) every 1-3 weeks, and dose adjustment to suppress the lupus activity. c. Class III and IV Lupus Nefritis Induction therapy The purpose of induction therapy is to achieve remission state of lupus activity characterized by a resolution of the symptoms of extra renal manifestations serologic being improved, as well as the resolution of the hematuria, crystal cell and serum 6

7 creatinine concentration is reduced or at least settled. The drugs used for induction therapy are: a. Pulse dose glukokortikoid For induction therapy can be administered glucocorticoid puls dose therapy, such as methylprednisolone at dose of mg iv / day to induce rapid inflammatory effects. After 3 days of administration, followed by oral prednisone at a dose of mg / day. Prednisone can be delivered with immunosuppressant drugs to another. b. Siklofosfamid Given at a dose of 750 mg / m2 every month for 6 months. Supplied with prednisone at a dose of 0.5mg / kg / day, which then lowered slowly until a dose of 0.25 milligrams / kg / day, especially for controlling the of extras renal symptoms. c. Mikofenolat mofetil Mycophenolate mofetil is used for induction therapy of lupus nephritis class III and IV. For induction therapy recommended dose of 1 g 2x a day given up to 6 months. d. Azatioprin Given at a dose of 2 mg / kg / day in combination with prednisone 0.5 mg / kg / day. Prednisone dose then lowered slowly - land up to 0.25 mg / kg / day. Given for 6 month. e. Rituximab Used to induce remission in patients with severe lupus nephritis, who did not respond with siklosfamid or MMF. The basic principle of treatment is to suppress the lupus inflammatory reaction, improve renal function, or at least maintain renal function from getting worse. With such treatment, lupus nephritis mortality decreases As well as hemodialysis for renal disorders and improve existing clinical circumstances. In these patients given induction therapy with pulse dose methylprednisolone 500 mg iv / day for 3 days. After 3 days of administration, followed by prednisone at a dose of mg / day. Furosemide injection, Captopril 2 x 6.25 mg. Diets high in protein (an extra egg ), and fluid balance ml. the patient's condition improved, edema is reduced, discoid rash is reduced, joints pain decreases, and decreases proteinuria and planned discharge from the hospital, and prescribe with methylprednisolone 3-2-2, Cellcef (Mycophenolate Mofetil) 2 x 500 mg, Captopril 2 x 6.25 mg, furosemide tablet 2 x 4 mg. 7

8 CONCLUSSION Reported a case of systemic lupus erythematosus (SLE) is with lupus nephritis + hipoalbumin. Diagnosis based on anamnesa / history, physical examination, laboratory and other investigations. On physical examination found vital signs within normal limits, encountered anemia, weakened vesicular breath sounds in both lower field of the lungs, additional sound was not found. In abdominal examination, ascites was found, discoid rash found on both arms and legs, accompanied by edema in both arms and legs. I Routine blood examination found : hemoglobin level 9.7 mg / dl, leukocytes 1,560 / ul; platelets 50,000 / ul. Urinalysis examination results obtained Positive 2 proteinuria, erythrocyte sediment 2-3 / LPB, leukocytes 2-5 / LPB, urine protein 545 (+++) mg%, 24-hour urine protein 5,450 mg, urea level 71 mg / dl, creatinine 1.31 mg / dl, uric acid 8.7 mg / dl, albumin 2.1 g / dl, CRP qualitative: negative, ANA test 161, anti ds-dna: 409 ng / ml. On chest x-ray examination found bilateal pleural effusion and bronkhopenumonia. In renal ultrasound examination was found in accordance with nephrotic picture pattern. Patients treated by injection of methylprednisolone puls therapy at a dose of 500 mg / day for 3 days, followed by administration of a dose of methyl prednisolone tablets from 0.5 to 0.6 mg / day with Cellcef (Mycophenolate Mofetil) 2 x 500 mg, furosemide injection, Captopril 2 x 6.25 mg. Diets high in protein (an extra egg white), and fluid balance ml. After administration of methylprednisolone pulse therapy for 3 days followed by administration of a steroid-sparing agent kortiko (MMF), proteinuria was reduced edema is reduced and the patient's condition improves. REFFERENCE 1. Setiadi S, Idris A, Buku ajar ilmu penyakit dalam, edisi VI, penerbit Interna Publishing, Jakarta, Maureen A, Hahn B, American college of rheumatology guide lines for screening, treatment and management of lupus nephritis, Arthritis care and research, American college of rheumatology, vol 64 p: Perhimpunan rheumatologi Indonesia, Diagnosis dan Penatalaksanaan Lupus Ertitematosus Sitemik 2011, Interna Publishing, Jakarta, Contrea G, Appel G, Mycophenolate Mofetil versus Cycloposphamide for induction lupus treatment of lupus nephritis, American Society of Nephrology, 2009 p

9 5. Tellingen A, Voskuyl A, Dutch guidelines for diagnosis and t heraphy of proliferative lupus nephritis, Departments of Nephrology VU university, Van Zuiden communications, 2012 p: Choi SJ, Woo JH, Induction and maintenance theraphy for lupus nephritis: a systemic review and meta analysis. National institute of Kidney disease, 2010 p: James PA, Oparil S, Evidence based guidelines for the management of lupus nephritis in adults. The journal of the American Medical Association, Desember Ortega LM, Schultz DR. Lupus Nephritis: pathology feautures, epidemiology and a guide to theapetic decisions, 2010;19 p: Saxena R, Mahajan T, Lupus Nephritis: current update, Biomed central, Austin HA, Boumpas DT, Improved clinical outcome of lupus nephritis during the past decade : importance of early diagnosis and treatment. Ann Rheum Dis 2014, 66: