Abstract
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
References
- Qin B, Wang J, Yang Z, et al. Epidemiology of primary Sjögren's syndrome: a systematic review and meta-analysis. Ann Rheum Dis 2015;74:1983-9.
- Yang HX, Wang J, Wen YB, et al. Renal involvement in primary Sjögren's syndrome: A retrospective study of 103 biopsy‐proven cases from a single center in China. Int J Rheum Dis 2018(1):223-9.
- Jain A, Srinivas BH, Emmanuel D, Jain VK, Parameshwaran S, Negi VS. Renal involvement in primary Sjogren's syndrome: a prospective cohort study. Rheumatol Int 2018;38(12):2251-62.
- Evans RD, Laing CM, Ciurtin C, Walsh SB. Tubulointerstitial nephritis in primary Sjögren syndrome: clinical manifestations and response to treatment. BMC Musculoskeletal Disorders 2016 17:2. doi: 10.1186/s12891-015-0858-x.
- Nocturne G, Mariette X. Advances in understanding the pathogenesis of primary Sjögren's syndrome. Nat Rev Rheumatol 2013;9(9):544-56.
- Shen Y, Xie J, Lin L, Li X, et al. Combination cyclophosphamide/glucocorticoids provide better tolerability and outcomes versus glucocorticoids alone in patients with Sjogren's associated chronic ınterstitial nephritis. Am J Nephrol 2017;46(6):473-80.
Abstract
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
References
- Qin B, Wang J, Yang Z, et al. Epidemiology of primary Sjögren's syndrome: a systematic review and meta-analysis. Ann Rheum Dis 2015;74:1983-9.
- Yang HX, Wang J, Wen YB, et al. Renal involvement in primary Sjögren's syndrome: A retrospective study of 103 biopsy‐proven cases from a single center in China. Int J Rheum Dis 2018(1):223-9.
- Jain A, Srinivas BH, Emmanuel D, Jain VK, Parameshwaran S, Negi VS. Renal involvement in primary Sjogren's syndrome: a prospective cohort study. Rheumatol Int 2018;38(12):2251-62.
- Evans RD, Laing CM, Ciurtin C, Walsh SB. Tubulointerstitial nephritis in primary Sjögren syndrome: clinical manifestations and response to treatment. BMC Musculoskeletal Disorders 2016 17:2. doi: 10.1186/s12891-015-0858-x.
- Nocturne G, Mariette X. Advances in understanding the pathogenesis of primary Sjögren's syndrome. Nat Rev Rheumatol 2013;9(9):544-56.
- Shen Y, Xie J, Lin L, Li X, et al. Combination cyclophosphamide/glucocorticoids provide better tolerability and outcomes versus glucocorticoids alone in patients with Sjogren's associated chronic ınterstitial nephritis. Am J Nephrol 2017;46(6):473-80.
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | Supplement |
| Authors | |
| Publication Date | October 25, 2019 |
| Submission Date | October 19, 2018 |
| Published in Issue | Year 2019Volume: 58 Suppl: 1 (Rheumatology) |
