Session Title: Sjögren's Syndrome II - Clinical
Session Type: Abstract Submissions (ACR)
Background/Purpose: Primary Sjögren’s syndrome (pSS) may affect kidneys, causing either interstitial nephritis (IN) or glomerulonephritis (GMN). However, overt renal disease in pSS is rare and does not exceed 5% of patients. The aim of this study was to estimate the prevalence and investigate the clinical features and the outcome of clinically significant and biopsy documented renal involvement in a large cohort of pSS patients.
Methods: From a cohort of 715 patients who met the American-European criteria for pSS, those with clinically significant and biopsy documented renal involvement were identified and their clinical features were recorded. The primary outcomes included death, hemodialysis, lymphoma and chronic renal failure (CRF). Statistical analysis was performed to estimate the primary outcomes per 100 person-years.
Results: Thirty five (4.9 %) pSS patients had biopsy documented renal involvement, representing a total follow up time after renal diagnosis of 271.8 person-years. Twelve patients (34.3%) had IN, 18 (51.4%) had GMN and 5 (14.2%) developed both entities. Nine patients died (25.7%), 11 (31.4%) presented chronic renal failure (including 4 requiring chronic hemodialysis) and 9 (25.7%) developed malignant lymphoma. Seven out of 11 (63.6%) patients with CRF had IN while the remaining 4 patients (36.4%) had GMN. The corresponding rates for lymphoma, chronic renal failure and hemodialysis were estimated at 3.30 (95%CI, 1.72-6.35), 4.04 (95%CI, 2.24-7.29), and 1.47 (95%CI, 0.55-3.91) per 100 person-years, respectively.
Overall 5-year survival was 0.82 (0.62-0.92) with median not reached. Interestingly, eight out of nine (89%) reported deaths and eight out of nine malignant lymhomas (89%) were among the GMN group. After adjusting for age, type of renal involvement (IN vs GMN), lymphoma, CRF and hemodialysis, lymphoma remained the sole significant adverse predictor (adjusted Hazard Ratio, 5.96 , 95% CI 1.14-21.5) for survival. Causes of death included lymphoma (33.3%), cardiac arrest (22.2%) related to hemodialysis or CRF, infection (11.1%) related to CRF, stroke (11.1%) and cerebral haemorrhage (11.1%).
Conclusion: The long term prognosis of clinically significant and biopsy documented renal involvement in pSS varies. Patients with IN display a favorable prognosis while patients with GMN are in high risk to develop lymphoma and have poor survival.
A. V. Goules,
I. P. Tatouli,
A. A. Drosos,
F. N. Skopouli,
H. M. Moutsopoulos,
A. G. Tzioufas,
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ACR Meeting Abstracts - http://acrabstracts.org/abstract/clinically-significant-and-biopsy-documented-renal-involvement-in-primary-sjogrens-syndrome-clinical-presentation-and-outcome/