Aziddin Gani Harahap Ilham Yasin Siregar Nurul Aini Hasibuan

ICD 10. M32.14 Kompetensi : II

Aziddin Gani Harahap Ilham Yasin Siregar Nurul Aini Hasibuan Pembimbing: dr Muhammad Nur, Sp.A

Definition • Lupus nephritis (LN) is a renal inflammatory process that is a common and serious caused by systemic lupus erimatosus , an autoimmune disease

Etiologi Lupus nephritis occurs when antibodies (antinuclear antibodies) and complement are formed in the kidney causing inflammation. This usually results in the occurrence of nephrotic syndrome (large protein excretion) and can be rapid

progression to renal failure

PATHOGENESIS

CLINICAL MANIFESTATIONS Renal Manifestations

CLINICAL MANIFESTATIONS Extrarenal Manifestations

Diagnosis • Clinical diagnosis of lupus nephritis is made when in a LES patient (at least 4 of 11 ARA criteria) have – 1 g / 24 h proteinuria – haematuria (> 8 erythrocytes / LBP) – decreased renal function by 30%

• a definite diagnosis of lupus nephritis is established by renal biopsy. • serologic examination

IMMUNOLOGIC TESTS IN LUPUS NEPHRITIS

ISN/RPS class I: minimal mesangial lupus nephritis. Light microscopy is normal

ISN/RPS class II: lupus nephritis (mesangial disease).

SN/RPS class III: focal proliferative lupus nephritis.

ISN/RPS class IV: lupus nephritis.

ISN/RPS class V: membranous lupus.

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The ISN/RPS biopsy classification should guide initial therapy.

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Patients assigned to ISN class I and class II need No therapy directed at the kidney.

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Class III and IV: Patients with focal or diffuse nephritis are at high risk of becoming ESRD and require aggressive therapy •

•

Give intravenous cyclophosphamide

Class V: Patients with membranous lupus nephritis

CASE REPORT

Case Ilustration • • • • • • •

Patient Identity Name/MR :K/941328 Age :14yo Parents :F/MZ Suku : Minang/Minang Address : Jl. Swakarya, Panam, Pekanbaru Admitted to hospital : june 26 2018

Chief Complaint • 4 days BATH, puffiness on the stomach is getting bigger

History of recent illness  4 day BATH, the patient complained of increasingly enlarged abdomen accompanied by swelling in the limbs, Patients also complain easily tired and weak, heartburn(+), nausea(+), gag(+), decreased appetite, oligouria with volume per day ±240 cc.  1 month BATH, patients complained of swollen limbs and stomach, the patient went to the M. Djamil Padang hospitals, then patients are diagnosed with lupus nephritis. The patient was treated for 10 days and get cyclophospamide treatment and PRC transfusion. patients are encouraged to continue treatment at Arifin Achmad hospitals

History of Pass illness • Same complaint before (-) • Patients diagnosed with LES since May 2017

History of family illness  Same complaint (-)  malignancy(-)

Parents  Dad Lecture  Mother housewife

Pregnancy history Meal history

Immunization history Growth history Development History House and environmental condition

Physical examination • • • • •

General condition consciousness Temperature Heart Rate Respiratory rate

: Tampak sakit sedang : Composmentis : 36,1oC : 86 t/m : 26 t/m Nutritional status  Height : 155 sentimeter  Weight : 47 kilogram, ascites BB-(20% x bb)  (47-9.4= 37.6 kg) 20/23 x 100 % = 86%  (GOOD nutritional status)

Physical examination Head : normochepali, facies cooley (-) Hair : black, hair easily revoked Eyes : • conjungtiva : pales(+/+) • Sklera : jaundice (-/-) • Pupil : isokor • Pupil Reflects : dirrected (+/+), undirrected(+/+) Nose : No abnormality

Ears : No abnormality Mouth :  Lips : dry(+)  Mucosa :Wet  Palatum : Intact  Tongue : Atrophy (-)  Teeth : karies (-), hipertrofi gingiva (-) Neck  Limf node : No enlargement  Stiff neck : not found

PF Thoraks Cor

• I • Pa • Pe :

: Ictus kordis not seen : Ictus kordis not palpated

– Right cardiac – Left cardia

: parasternal dextra line : midclavicula sinistra line

• A : regular S1-S2, gallop (-), murmur (-) Pulmo

• I : statis normochest, dinamis simetric between left and right, mass (-),retraction (-) • Pa :equally strong vocal fremitus • Pe : sonor • A : Vesikuler (+/+), ronkhi (-/-), wheezing (-/-)

Abdomen • I

: Large & bulging simetrically, venectation (-), scar(-), pupura(-), ptechie (-) • A : Bowel sounds (+) normal • Per : Tymphani • Pa : Tenderness (-), mass (-), hepatomegaly (-) splenomegaly (-)

Exstremity • warm, capilary refill time < 2 detik, udem ( • +),paled palm

Genital and neurological status  Normal

Laboratory finding Routine • Hemoglobin • Hematokrit • Leukosit • Trombosit • MCV • MCH • MCHC

: 6,5 g/dl : 16,6 % : 5560/Ul : 113.000 /mm3 :83,8 fl :27,8 pg :33,2 g/dl

Urinalisa (june 26 2018) • Protein : (+)3 • Bilirubin : (-) • Urobilinogen : (-) • Keton : (-) • Ph : 6,0 • eritrosit : 100/LPB • Berat jenis : 1010 • Warna : Kuning Muda • Kejernihan : keruh

Working Diagnosis Nefritis Lupus Hipertensi stage II LES

Initial treatment • • • • • • • • •

IVFD KAEN IB 10 tpm makro CPA 500-750 pulse dalam Nacl 0,9% 300 cc Metilprednison 2x12mg Captopril 3x12.5 mg Furosemid 2x 40mg Spironolacton 3x25mg Losartann 1x50 mg Tranfusi PRC 827 cc Diet 1500 Kkal

Follow Up

27/7/18

Nausea vomiting, full sense of being groomed (+), swollen on the legs and abdomen (+)

General condition: moderate consciousness: COMPOSMENTIS BP: 130/90 T : 36,9 HR : 86x/minutes RR : 19x/minutes Eyes: Anemic conjungtiva (+/+) Thoraks : Vesikuler +/+, Ronkhi (-). S1S2 reguler, murmur (-), gallop (-) Abdomen:Large & bulging simetrically, Ekstremitas: warm, capilary refill time < 2 detik, udem ( +),paled palm

27/7/18

Nausea vomiting, full sense of being groomed (+), swollen on the legs and abdomen (+)

General condition: moderate consciousness: COMPOSMENTIS BP: 140/100 T : 36,7 HR : 100x/minutes RR : 4x/minutes Eyes: Anemic conjungtiva (+/+) Thoraks : Vesikuler +/+, Ronkhi (-). S1S2 reguler, murmur (-), gallop (-) Abdomen:Large & bulging simetrically, Ekstremitas: warm, capilary refill time < 2 detik, udem ( +),paled palm

Nefritis lupus IVFD Nacl 0.9% 10 tpm makro Hipertensi CPA 500-750mg stage 2 dalam 300ml Nacl 0.9% Anemia Metilprednison 2x12 mg Captopril 3x12.5mg Losartan 1x50mg Tranfusi PRC labu ke 1 Nefritis lupus IVFD Nacl 0.9% 10 tpm makro Hipertensi stage CPA 500-750mg 2 dalam 300ml Nacl 0.9% Anemia Metilprednison 2x12 mg Captopril 3x12.5mg Amlodipin 1x10mg Losartan 1x50mg Tranfusi PRC Labu

Nausea vomiting, full sense of being groomed (+), swollen on the legs and abdomen (+)

General condition: moderate consciousness: COMPOSMENTIS BP: 140/100 T : 36,7 HR : 106x/minutes RR : 3x/minutes Eyes: Anemic conjungtiva (+/+) Thoraks : Vesikuler +/+, Ronkhi (-). S1S2 reguler, murmur (-), gallop (-) Abdomen:Large & bulging simetrically, Ekstremitas: warm, capilary refill time < 2 detik, udem ( +),paled palm

IVFD Nacl 0.9% 10 tpm makro Metilprednison 2x12 mg Captopril 3x12.5mg Amlodipin 1x10mg Losartan 1x50mg Pasang CDL Rencana Hemodialisa ke 1

29/06/1 swollen on the legs and 8

General condition: moderate consciousness: COMPOSMENTIS BP: 130/100 T : 36,5 HR : 110x/minutes RR : 23x/minutes Eyes: Anemic conjungtiva (-/-) Thoraks : Vesikuler +/+, Ronkhi (-). S1S2 reguler, murmur (-), gallop (-) Abdomen:Large & bulging simetrically, Ekstremitas: warm, capilary refill time < 2 detik, udem ( +),paled palm

Nefritis Lupus IVFD Nacl 0.9% 10 Hipertensi tpm makro stage 2 Metilprednison 2x12 mg Captopril 3x12.5mg Losartan 1x50mg Furosemid 2x40mg Spironolakton 3x25mg Diet 1500 Kkal

28/06/1 8

abdomen (+)

30/06/1 swollen on the legs and 8 abdomen (+)

Sunday 1/07/18

swollen on the legs and abdomen (+)

General condition: moderate consciousness: COMPOSMENTIS BP: 130/100 T : 36,5 HR : 108x/minutes RR : 21x/minutes Eyes: Anemic conjungtiva (-/-) Thoraks : Vesikuler +/+, Ronkhi (-). S1S2 reguler, murmur (-), gallop (-) Abdomen:Large & bulging simetrically, Ekstremitas: warm, capilary refill time < 2 detik, udem ( +),paled palm

General condition: moderate consciousness: COMPOSMENTIS BP: 140/100 T : 36,5 HR : 110x/minutes RR : 23x/minutes Eyes: Anemic conjungtiva (-/-) Thoraks : Vesikuler +/+, Ronkhi (-). S1S2 reguler, murmur (-), gallop (-) Abdomen:Large & bulging simetrically, Ekstremitas: warm, capilary refill time < 2 detik,

Nefritis Lupus Hipertensi stage 2 AKI

IVFD Nacl 0.9% 10 tpm makro CPA 500-750mg dalam 300ml Nacl 0.9% Furosemid 2x40 mg iv Spironolakton 3x25mg PO Metilprednison 2x12 mg Captopril 3x12.5mg Losartan 1x50mg Nefritis Lupus IVFD Nacl 0.9% 10 Hipertensi tpm makro stage 2 CPA 500-750mg dalam 300ml Nacl 0.9% Furosemid 2x40 mg iv Spironolakton

2/07/18

swollen on the legs and abdomen (+)

General condition: moderate consciousness: COMPOSMENTIS BP: 130/100 T : 36,5 HR : 108x/minutes RR : 21x/minutes Weight : 44 kg LP : 82 cm Eyes: Anemic conjungtiva (-/-) Thoraks : Vesikuler +/+, Ronkhi (-). S1S2 reguler, murmur (-), gallop (-) Abdomen:Large & bulging simetrically, Ekstremitas: warm, capilary refill time < 2 detik, udem ( +),paled palm

Nefritis Lupus Hipertensi stage 2 AKI

IVFD Nacl 0.9% 10 tpm makro CPA 500-750mg dalam 300ml Nacl 0.9% Furosemid 2x40 mg iv Spironolakton 3x25mg PO Metilprednison 2x12 mg Captopril 3x12.5mg

DISCUSSION

Patient came with edema as his chief complain within 10 days. In physical examination found that the abdoment’s diameter is 88 cm, undulation (+), and there is pitting edema in lower extremity

Edema in this patient because the abnormality of glomerulus caused by SLE, it make inflamation in interstitial cell and make increasing permeability of natrium channel.

Pons-Estel GJ.Understanding the Epidemiology and Progression of Systemic Lupus Erythematosus.Semin Arthritis Rheum 2010;39:257-68. Rekomendasi Perhimpunan Reumatologi Indonesia. Diagnosis dan Pengelolaan Lupus Eritematosus Sistemik. Jakarta: Perhimpunan Reumatologi Indonesia; 2011..

Grade II hypertension since the first treatment in hospital, the hypertension is caused the side effect of steroid.

Rauf S, Albar H, Aras J. Konsensus glomerulonefritis akut pasca streptokokus. Jakarta. 2012. Rachmadi D. Diagnosis dan penatalaksanaan glomerulonefritis akut. Ilmu Kesehatan Anak. 2010

This patient got some medicine such as cyclophospamide, metylprednisolon, captopril and losartan. The purpose of the therapy is to press the inflamation reaction of lupus, repair the kidney function

Captopril and losartan in this patient as anti hipertension as well as anti proteinuria. This effect related to the decrease of permeability glomerulus to macromolecul

Furosemid and spironolacton was given at the fourth day treatment as the therapy of edema and to decrease the kidney function because the patient had been acute kidney injury.

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