| Keywords [eng] |
Sjögren’s Syndrome, Primary Sjögren’s Syndrome, Kidney Injury, Autoimmune disease, Extra glandular Symptoms, Sicca, Renal Injury, Renal involvement, Glomerulonephritis, Tubular Interstitial Nephritis, Chronic Kidney Disease, Renal Injury, Lymphoma, Distal Renal Tubular Acidosis. |
| Abstract [eng] |
Sjögren’s syndrome (SS) is a systemic chronic autoimmune disease that primarily affects exocrine glands, leading to sicca symptoms such as dry mouth and dry eyes. However, SS can also present in extraglandular manifestations, including renal involvement, which is less common but can significantly impact patient outcomes. This thesis explores the pathophysiology, clinical manifestations, diagnosis, treatment, and prognosis of kidney injury in primary Sjögren’s syndrome (pSS). The most common renal complication in pSS is tubulointerstitial nephritis (TIN), a process that results from lymphocytic infiltration of the renal interstitium and could lead to distal renal tubular acidosis (dRTA). Less frequently, glomerulonephritis (GN) occurs due to immune complex deposits, leading to proteinuria, hematuria, and a severe decline in kidney function. The diagnosis of renal involvement in pSS is challenging due to limited data and the diversity on the clinical picture of the patient. The essential methods for detecting renal injury are urinalysis, serological tests, and biopsy. The histopathological findings in TIN include lymphocytic infiltration and interstitial fibrosis, whereas GN is characterized by immune complex deposits in the glomeruli. The management of renal injury in pSS depends on the disease severity. Starting from symptomatic management, such as potassium and bicarbonate supplementation, as first line treatment to glucocorticoids, immunosuppressants and in more severe cases plasma exchange. Prognosis varies; TIN generally follows a benign course, whereas GN is associated with worse prognosis and increased risk of lymphoma. Renal involvement presents significant diagnostic and therapeutic challenges. This thesis highlights the need for early detection to preserve kidney function, standardized diagnostic criteria and novel treatment strategies to improve patient outcomes. |
