Renal biopsy findings predicting outcome in scleroderma renal crisis

Scleroderma Renal Crisis

“…When expressed as binary variables (presence/absence), Penn et al found that the presence of mucoid edema or vascular thrombosis was associated with a suboptimal renal outcome (Penn, et al, 2007). We extended Penn et al observations by demonstrating that the severity and extent of acute vascular damage and its consequences, namely arterial thrombosis/fibrinoid changes and glomerular ischemic collapse, were indeed predictors of poor prognosis (Batal, et al, 2009). In contrast to the study by Penn et al, we could not associate mucoid changes with poor renal outcome.…”

“…However, this feature is not entirely specific for SRC (Okada, Lertprasertsuke, & Tsutsumi, 2000), and is not a consistent finding. We detected a prominent JGA hyperplasia only in 12% of our SRC cases (Batal, et al, 2009). Finally, it should be noted that vascular pathologic changes in scleroderma patients are not restricted to SRC.…”

“…When detected in the peritubular capillary of an allograft biopsy, it is very suggestive of the diagnosis of antibody-mediated rejection (Racusen et al, 2003). Using immunoperoxidase techniques, we could detect similar pattern of C4d staining in a subset of SRC patients who had poor renal outcome (Batal et al, 2009). Nevertheless, the presence and/or significance of C4d deposits in SRC should be confirmed in larger studies using the more specific immunofluorescence techniques.…”

“…In hemolytic uremic syndrome, Tostivint et al showed that thrombotic microangiopathic alterations were more frequently encountered in the glomeruli compared to small vessels [11/12 (92%) versus 4/12 (33%), p = .009] (Tostivint et al, 2002). In SRC, thrombi were more commonly detected in small vessels [11/17 (65%) small vessels thrombi versus 3/17 (18%) glomerular thrombi, P = .01] (Batal, et al, 2009). Small vessels in SRC may display thinning of the media and/or adventitial expansion.…”

Scleroderma renal crisis in a newly diagnosed mixed connective tissue disease resulting in dialysis-dependent chronic kidney disease despite angiotensin-converting enzyme inhibition

“…Fibrointimal sclerosis with adventitial fibrosis develops later in the course of SRC, causing onion skin-like lesions [10]. Glomerular endothelial cell swelling and thrombosis are the earliest findings, but are very rare [11]. Chronic glomerular changes varied from doublecontour and tram-tracking to ischaemic glomerular collapse [10,12].…”