LUPUS NEPHRITIS REFRACTORY IN MALE PATIENT

LUPUS NEPHRITIS REFRACTORY IN MALE PATIENT

COIMBRA, LETÍCIA LOPES; RUIZ, ANDRESSA VIEIRA; BORGES, ANA CAROLINE MARTINS; SILVA, ALLETHÉA ROBERTHA SOUZA E; LEAL, MARIA EDUARDA GUIMARÃES; RUIZ, DANILO GARCIA; CUSTÓDIO, DANIELA MARIA EDILMA JAPIASSÚ; PEDREIRA, PAULO GEOVANNY; FREIRE, RAFAELA ALEN COSTA; MADRID, PAOLA BOTTIN; ROSSONI, HUGO DE CARLOS MACIEL

Pôster:

Kidney involvement in lupus occurs in about 60% of patients, and glomerular abnormalities demonstrated in renal biopsy determine most of the signs and symptoms of lupus nephritis, in addition to guiding treatment. We present the uncommon case of male with lupus with refractory renal involvement and difficult to control.

Case report

Patient T.S.L, 19 years old, male, hospitalized due to anasarca, arthritis of wrists, oliguria, beginning 60 days ago. Made use of benzathine penicillin to treat syphilis due to 1/32 VDRL reagent. At physical examination, the patient was in regular general condition, with diffuse petechiae in the body, periorbital edema, mild malar rash, lesion on hard palate, decreased pulmonary auscultation on bases, submacetic abdomen on percussion, ascites. Tests performed: Creatinine: 2,3, urea: 132, hemoglobin: 8, ultrasound of the abdomen with moderate ascites and visualized discrete bilateral pleural effusion. Echocardiogram with discrete pericardial effusion. Treponema IgM and non-reactive IgG. Diagnosis of systemic lupus erythematosus with the following criteria was performed: FAN homogeneous nuclear standard reagent titre 1: 640, native ANTI-DNA: reagent 1/80, ANTI-SM: reagent (480), polyarthritis, Anti-Cardiolipin: IgM: reagent 41.4) and IgG: reagent (45.5), serositis, malar rash, complement consumption, oral ulcers, anti-B2 glycoprotein 1 IgM: reagent and nephrite with 24-hour proteinuria: 5,565mg. Performed renal biopsy: diffuse proliferative Lupus Nephritis - grade IV. During hospitalization, the patient developed worsening renal function with a significant increase of nitrogenous slags, requiring serial hemodialysis. Pulse therapy with methylprednisolone 1g/day for 3 consecutive days and cyclophosphamide 1g/ month, in addition to hydroxychloroquine 400mg and prednisone 1mg/kg/day for 6 weeks, followed by gradual weaning. After the second dose of cyclophosphamide, the patient evolved with improved clinical status, increased diuresis, and improved renal function requiring no further hemodialysis. He was discharged from hospital and maintained pulse therapy with monthly cyclophosphamide. After 6 doses of the immunosuppressant, despite an improvement in renal function, patients present worsening of proteinuria 24hrs: 10,000mg, being classified as refractory lupus nephritis.

Conclusion

In the present report, the refractoriness of the disease was concluded even though adequate treatment, demonstrated through the persistence and increase of proteinuria, was carried out. About 10% to 29% of patients develop into chronic kidney disease, even with adequate treatment. The literature reports mainly from patients with the same profile described in this case, and relates the aggressive condition of the disease to glomerular inflammatory events. In these cases the therapy instituted by another immunosuppressive drug should be changed.

Kidney involvement in lupus occurs in about 60% of patients, and glomerular abnormalities demonstrated in renal biopsy determine most of the signs and symptoms of lupus nephritis, in addition to guiding treatment. We present the uncommon case of male with lupus with refractory renal involvement and difficult to control.

Case report

Patient T.S.L, 19 years old, male, hospitalized due to anasarca, arthritis of wrists, oliguria, beginning 60 days ago. Made use of benzathine penicillin to treat syphilis due to 1/32 VDRL reagent. At physical examination, the patient was in regular general condition, with diffuse petechiae in the body, periorbital edema, mild malar rash, lesion on hard palate, decreased pulmonary auscultation on bases, submacetic abdomen on percussion, ascites. Tests performed: Creatinine: 2,3, urea: 132, hemoglobin: 8, ultrasound of the abdomen with moderate ascites and visualized discrete bilateral pleural effusion. Echocardiogram with discrete pericardial effusion. Treponema IgM and non-reactive IgG. Diagnosis of systemic lupus erythematosus with the following criteria was performed: FAN homogeneous nuclear standard reagent titre 1: 640, native ANTI-DNA: reagent 1/80, ANTI-SM: reagent (480), polyarthritis, Anti-Cardiolipin: IgM: reagent 41.4) and IgG: reagent (45.5), serositis, malar rash, complement consumption, oral ulcers, anti-B2 glycoprotein 1 IgM: reagent and nephrite with 24-hour proteinuria: 5,565mg. Performed renal biopsy: diffuse proliferative Lupus Nephritis - grade IV. During hospitalization, the patient developed worsening renal function with a significant increase of nitrogenous slags, requiring serial hemodialysis. Pulse therapy with methylprednisolone 1g/day for 3 consecutive days and cyclophosphamide 1g/ month, in addition to hydroxychloroquine 400mg and prednisone 1mg/kg/day for 6 weeks, followed by gradual weaning. After the second dose of cyclophosphamide, the patient evolved with improved clinical status, increased diuresis, and improved renal function requiring no further hemodialysis. He was discharged from hospital and maintained pulse therapy with monthly cyclophosphamide. After 6 doses of the immunosuppressant, despite an improvement in renal function, patients present worsening of proteinuria 24hrs: 10,000mg, being classified as refractory lupus nephritis.

Conclusion

In the present report, the refractoriness of the disease was concluded even though adequate treatment, demonstrated through the persistence and increase of proteinuria, was carried out. About 10% to 29% of patients develop into chronic kidney disease, even with adequate treatment. The literature reports mainly from patients with the same profile described in this case, and relates the aggressive condition of the disease to glomerular inflammatory events. In these cases the therapy instituted by another immunosuppressive drug should be changed.

Palavras-chave:

DOI: 10.5151/sbr2019-160

Referências bibliográficas

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Como citar:

COIMBRA, LETÍCIA LOPES; RUIZ, ANDRESSA VIEIRA; BORGES, ANA CAROLINE MARTINS; SILVA, ALLETHÉA ROBERTHA SOUZA E; LEAL, MARIA EDUARDA GUIMARÃES; RUIZ, DANILO GARCIA; CUSTÓDIO, DANIELA MARIA EDILMA JAPIASSÚ; PEDREIRA, PAULO GEOVANNY; FREIRE, RAFAELA ALEN COSTA; MADRID, PAOLA BOTTIN; ROSSONI, HUGO DE CARLOS MACIEL; "LUPUS NEPHRITIS REFRACTORY IN MALE PATIENT", p-160-160. In: Anais do 36º Congresso Brasileiro de Reumatologia. [ISBN 978-85-212-1892-0]. São Paulo: Blucher, 2019.
ISSN 23577282, DOI 10.5151/sbr2019-160