Sjogren Disease and Nephrolithiasis: A Case Series and Review of the Literature

Background: Primary Sjogren Disease (pSD) is a chronic autoimmune disease characterized by a classic triad of keratoconjunctivitis sicca, xerostomia, and polyarthritis. The primary pathological feature of pSD is lymphoplasmacytic infiltration in glandular epithelial tissue, often affecting the salivary and lacrimal glands, leading to classic sicca symptoms (ocular and oral dryness). Sjogren Disease (SD) can be categorized as "primary" when occurring independently or "secondary" when accompanying another autoimmune connective tissue disorder such as rheumatoid arthritis, systemic lupus erythematosus, or systemic sclerosis. Additionally, systemic disease is common in pSD and can manifest with kidney dysfunction resulting in nephrolithiasis and distal renal tubular acidosis (dRTA). Methods: This report details a case series drawing patients from the literature as well as patients from our institution which serves to demonstrate key points in clinical hallmarks. We utilize a literature search with key words Sjogren Disease, nephrolithiasis, renal tubular acidosis, and nephrocalcinosis in addition to pSD patients with concomitant nephrolithiasis at our institution to characterize clinical and serologic findings as well as treatment modalities. Results: We find well demonstrated clinical hallmarks such as female predominance and presence of dRTA amongst the cohort of pSD patients. We also find that further research on pSD serologies could prove beneficial in risk stratifying those most likely to develop renal disease and nephrolithiasis. Furthermore, we review signs, symptoms, pathophysiology, and management of SD with added emphasis on associated renal disease including nephrolithiasis and dRTA. Conclusion: Overall, pSD associated renal disease remains an area of ongoing research and further study on patient serologies may aid clinicians in better serving and surveilling patients at risk of systemic involvement.