<p>Renal pathology and clinical associations in systemic sclerosis: a historical cohort study</p>

Abstract

Background The information guiding the treatment decision(s) for renal diseases in systemic sclerosis (SSc) is the renal pathological finding. This study aimed to evaluate the renal pathological diagnosis and its clinical feature among SSc. Method A historical cohort study was performed on adult Thai SSc patients who underwent renal biopsy during January 2005–December 2016. The renal pathologic findings and patient clinical characteristics were reviewed. Chi-square or Fisher’s exact test was applied to analyze the association between clinical manifestation and renal pathology. Results Of the 26 SSc patients identified (77% female), 46% had the diffuse cutaneous SSc subtype. The mean age at the time of biopsy was 53.2±14.4 years and median duration of disease was 2.4 years (IQR 0.5–7.0). Rapidly progressive glomerulonephritis (RPGN) was the most common renal manifestation (53.9%) followed by nephrotic syndrome (19.2%) and nephritis (11.5%). The pathological diagnosis included lupus nephritis (LN) class IV (26.9%), LN class V (19.2%), scleroderma renal crisis (SRC; 19.3%), progressive renal disease in scleroderma (7.7%), and IgA nephropathy (7.7%). The nephrotic syndrome was the most common renal feature among LN class V patients, whereas RPGN was the commonest renal presentation among LN class IV and SRC patients ( p =0.001). Dialysis treatment at the time of kidney biopsy was significantly higher in SRC patients than in the other groups ( p <0.001). The SRC tended to have more frequent cardiac involvement, pulmonary fibrosis, and shorter disease duration than the other groups. Conclusion This is the first report of renal pathologic findings in Thai SSc patients. RPGN is the commonest renal manifestation among SSc who underwent kidney biopsy; for whom LN was the most common pathological finding. Nephrotic syndrome is a clinical feature of glomerular diseases other than renal involvement in SSc.

Secondary Membranous Nephropathy. A Narrative Review

“…Some individuals are initially asymptomatic or show only mild proteinuria, microscopic haematuria, and occasional casts. These patients may follow an indolent course until hypertension and progressive deterioration of kidney function develop (144)(145)(146). Cases of MN have been reported.…”

Biomarkers in Systemic Sclerosis: An Overview

“…Scleroderma renal crisis (CRS) is the most frequent renal complication in SSc representing a medical emergency [19,[141][142][143][144][145][146][147][148][149][150]. The use of ACE inhibitors has reduced the occurrence of CRS.…”

“…CRS is characterized by malignant hypertension, microangiopathic haemolysis, microthrombosis, thrombocytopenia, vasospasm, and progressive renal failure which can be caused by a variety of causes, such as various drugs (e.g., corticosteroids, cyclosporine, and tacrolimus) [19,[141][142][143][144][145][146][147][148][149][150]. Pathologically, CRS is characterized by rather bland or subtle findings but may show the typical "onion bulb" findings, hyperplasia of the juxtaglomerular apparatus, membranous proliferation, renovascular endothelial damage, intimal proliferation, thrombotic angiopathy, microthrombi of fibrin, hemolysis, vasospasm, vascular occlusion, ischemia, necrosis, vascular remodeling and possibly fibrosis associated with hyperreninemia and accelerated hypertension [19,[141][142][143][144][145][146][147][148][149][150]. Anti-fibrillarin antibodies, anti-RNA polymerase III antibodies, and speckled pattern ANA have been closely associated with the development of SRD; however, anti-topoisomerase antibodies have also been associated with a high incidence of CRS in some populations [19,[141][142][143][144][145][146][147][148][149][150].…”

“…Pathologically, CRS is characterized by rather bland or subtle findings but may show the typical "onion bulb" findings, hyperplasia of the juxtaglomerular apparatus, membranous proliferation, renovascular endothelial damage, intimal proliferation, thrombotic angiopathy, microthrombi of fibrin, hemolysis, vasospasm, vascular occlusion, ischemia, necrosis, vascular remodeling and possibly fibrosis associated with hyperreninemia and accelerated hypertension [19,[141][142][143][144][145][146][147][148][149][150]. Anti-fibrillarin antibodies, anti-RNA polymerase III antibodies, and speckled pattern ANA have been closely associated with the development of SRD; however, anti-topoisomerase antibodies have also been associated with a high incidence of CRS in some populations [19,[141][142][143][144][145][146][147][148][149][150]. Antiphospholipid antibodies, especially IgG antiphospholipid antibodies, represent a significant risk factor for renal disease in SSc compared with antibody-negative patients [19,[141][142][143][144][145][146][147][148][149][150].…”

Kidney Involvement in Systemic Sclerosis

“…SSc may overlap with lupus nephritis [ 70 ], while the association with other glomerular diseases, as IgA nephropathy, IgM nephropathy, and membranoproliferative glomerulonephritis is infrequent [ 71 ]. Additionally, the possible association with ANCA positivity has been described, although uncommon (0–12%) and only in rare cases with clinical characteristics of ANCA-associated vasculitis [ 72 ].…”