Hande Ece ÖZ, Mete KARA, Gonca KARABULUT, Yasemin KABASAKAL

Hipokalemik periyodik paralizi ile başlangıç gösteren primer Sjögren sendromu

Primer Sjögren sendromu (PSjS) heterojen klinik bulgularla seyreden kronik sistemik otoimmün bir hastalıktır. PSjS’de renal tutulum sıklığı %4.9 ve renal tutulum öncesi ortalama hastalık süresi iki yıl olarak bildirilmiştir. En sık renal tutulum tübülointerstisyel nefrit (TIN) olup daha az sıklıkla glomerülonefrit de görülebilmektedir. TIN bazı hastalarda izole elektrolit bozukluğu, distal renal tübüler asidoz (dRTA) yaparken bazı hastalarda ise son dönem böbrek yetmezliğine yol açabilmektedir (6). dRTA'un ise en sık klinik bulgusu hipokalemik paralizidir, bunun yanında nefrokalsinozis ve metabolik kemik hastalığına da yol açabilir. Bu yazıda PSjS’nin nadir bir başlangıç şekli olan renal tübüler asidoz ve hipokalemik periyodik paralizi olgusunu sunmaktayız.

Primary Sjögren’s syndrome presenting with hypokalemic periodic paralysis

Primary Sjögren’s syndrome (PSjS) is a chronic systemic autoimmune disease with heterogeneous clinical findings. The incidence of renal involvement in PSjS was reported as 4.9% and the mean duration of disease before renal involvement was two years. The most common renal involvement is tubulointerstitial nephritis (TIN) and less frequent glomerulonephritis. TIN may lead to isolated electrolyte disorder and distal renal tubular acidosis (dRTA), while in some patients it may lead to end-stage renal failure (6). The most common clinical manifestation of DRTA is hypokalemic paralysis, which may lead to nephrocalcinosis and metabolic bone disease. We report a rare initial form of PSjS presenting with renal tubular acidosis and hypokalemic periodic paralysis.

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