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- W3153289936 abstract "Autosomal dominant polycystic kidney disease (ADPKD) is an inherited disease with a prevalence of 1:700 – 1:1000 people on the other lupus remains a rare pathology. The association between ADPKD and autoimmune disease is rarely reported in the literature. Several renal and extra renal manifestations are similar and make the diagnosis of one or the other disease difficult. We report the case of two patients who had such association: ADPKD and lupus. A 50 years old female patient with a family history of ADPKD ( her fadher, her paternal aunt who undergow hemodialysis and died, her brother 36 years old and her sister 40 years old have been hemodialysed since 20 years), she had high blood hypertension (HBP) and hypothyroidism. She was admitted in our Nephrology department for fever related to acute pyelonephritis. Her blood pressure (BP) was controlled 130/70 mmHg, weight: 99Kg, dip sticks showed hematuria but not protenuria , she has a malar erythrm of the face which appeared recently. She had a history of polyarthralgia. An abdominopelvic CT scan showing two 18.5cm multicystic kidneys with pleural and pericardial effusion. Laboratory studies showed kidney function impairment which was improved after rehydration. The hemogram showed lymphopenia. She had low proteinuria 0.68g/day with normal albuminemia and normal protidemia. Thinking of lupus, anti nuclear antibodies were requested and were positive to 1/200 with anti Sm +, there was no indication for renal biopsy. The diagnosis of lupus was retained ( we had more than 4 criteria). She recieved oral corticosteroid therapy with synthetic antimalarials. The second patient was a 56 years old female patient who was followed since many years for ADPKD with chronic renal failure. Recently she reports a photosensitivity and an erythema of the face. Her BP was correct without any other signs on physical examination. Her 24-hour proteinuria was 0.05g/day , the hemogram showed leukopenia ans lymphopenia. The electrophoresis of blood proteins showed hypergammaglobulinemia at 24.6g/l with positive anti nuclear antibodies to 1/320. We confirmed the diagnosis of lupus and the patient recieved oral corticosteroid therapy and synthetic antimalarials. The association between ADPKD and lupus has been described in the literature." @default.
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- W3153289936 date "2021-04-01" @default.
- W3153289936 modified "2023-10-18" @default.
- W3153289936 title "POS-471 WHEN ADPKD AND LUPUS MEET" @default.
- W3153289936 doi "https://doi.org/10.1016/j.ekir.2021.03.498" @default.
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