Spectrum of renal manifestations in scleroderma - a review of literature

SESSION TITLE: Critical Care Posters 6 SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/10/2023 12:00 pm - 12:45 pm PURPOSE: Systemic sclerosis (SSc) is a multisystem rheumatic disease characterized by vascular and fibrotic manifestations that can affect practically every organ including the kidneys. Scleroderma renal crisis (SRC) is the most common renal manifestation of diffuse systemic sclerosis. However, with the use of angiotensin-converting enzyme inhibitors (ACEi), the morbidity and mortality associated with SRC has significantly reduced. Renal manifestations in SSc other than SRC have been generally under-investigated and can be left untreated which can lead to grave consequences in this patient population. In this article, we will study the spectrum of renal involvement in systemic sclerosis (SSc). METHODS: The Preferred Reporting Items for Systematic Reviews (PRISMA) guidelines were followed. An extensive literature search was performed from inception to December 2022, on PubMed, Cochrane, and Google Scholar using MeSH terms and relevant keywords for ‘scleroderma’, ‘systemic sclerosis’ ‘renal injury’, ‘renal disease’ and ‘renal manifestations’. We included case reports, case series, and observational studies which reported the variety of renal manifestations other than scleroderma renal crisis (SRC) in patients with systemic sclerosis. The authors extracted data from the included studies using a pre-specified data collection form in Microsoft Excel. Data pertaining to the study year, patient characteristics, serological profile, renal biopsy findings, treatment, and the outcome was extracted. RESULTS: After the exclusion of duplicates and non-relevant articles, data was included from 35 articles and 52 patients. The median age was 54.4 years, 11 males (21.2%) and 41 females (78.8%). The most reported renal manifestations included: ANCA-associated vasculitis (n=22,42.3%), penicillamine-induced renal injury (n=8, 15.3%), scleroderma pulmonary-renal syndrome (n=6, 11.5%), oxalate nephropathy (n=5, 9.6%), Goodpasture syndrome (n=4, 7.7%), hemolytic uremic syndrome (n=2, 3.8%), nephrotic range proteinuria (n=2, 3.8%), renal artery stenosis (n=2, 3.8%), membranous glomerulonephritis (n=1, 1.9%). CONCLUSIONS: Renal involvement in patients with scleroderma can have a variable clinicopathological course. The spectrum of kidney involvement can range from asymptomatic reduction of the GFR to life-threatening scleroderma renal crisis. Therefore, it is essential that physicians closely monitor renal function (creatinine, eGFR) in these patients for any emerging renal dysfunction. In case of abnormal findings, the threshold for renal ultrasound and renal biopsy should be low, and specific treatment should be initiated promptly, to avoid permanent renal damage. CLINICAL IMPLICATIONS: The most studied form of renal involvement in SSc is Scleroderma Renal Crisis (SRC), which is associated with the most dramatic clinical course. Other forms of renal involvement including antineutrophil cytoplasmic antibody (ANCA) associated vasculitis, hemolytic uremic syndrome, an isolated reduction in glomerular filtration rate (GFR), and nephrotic range proteinuria should also be considered in differential diagnosis, as they can be associated with poor renal outcomes in scleroderma patients. DISCLOSURES: No relevant relationships by Larabe Farrukh No relevant relationships by Swati Mehta No relevant relationships by Mohammad Rehman No relevant relationships by Lisa Rosenberg No relevant relationships by Lee Shapiro