Refractory scleroderma renal crisis precipitated after high-dose oral corticosteroids and concurrent intravitreal injection of bevacizumab

Abstract

Scleroderma renal crisis is a serious complication that can develop in certain patients with systemic sclerosis. Some risks have been identified as potential triggers of scleroderma renal crisis, including the high-dose oral corticosteroids. Here, we present a patient who developed clinically severe systemic sclerosis and scleroderma renal crisis after exposure to oral corticosteroids and intravitreal vascular endothelial growth factor blockade with bevacizumab for cotton wool spots. The patient’s scleroderma renal crisis was severe, progressive, and refractory to the standard of care therapy: oral captopril. Biopsy showed a diffuse thrombotic microangiopathy and findings consistent with scleroderma renal crisis. We hypothesize that depletion of systemic vascular endothelial growth factor with intravitreal anti–vascular endothelial growth factor injections likely contributed to the particularly severe presentation seen in this case. Though the finding of a monoclonal gammopathy of undetermined significance is another complicating factor, this case suggests that vascular endothelial growth factor inhibition may be a newly recognized trigger of scleroderma renal crisis.

Thrombotic Microangiopathy Syndromes—Common Ground and Distinct Frontiers

“…1 There is a distinct possibility that HTN may be provoked by complement abnormalities, which in turn worsen HTN and further dysregulate the complement system in a positive feedback loop. 97 This interplay that shows the bidirectionality of complement dysregulation in this situation is displayed in Supplementary Figure 2.…”

“…2 Scleroderma Renal Crisis-Related TMA Scleroderma renal crisis (SRC) is an enigma, with a terrible prognosis. 97 Despite this, the only agent in the armamentarium against this deadly disease is renal angiotensin aldosterone system inhibition (specifically with captopril). 97 Although complement blockade has been tried, it has not been uniformly successful; 96,97 plasmapheresis and antiendothelin agents are the other experimental therapies.…”

“…97 SRC remains morbid with a high mortality rate, 109 and often the treatment is supportive until the immune system recovers. 96,97 Treatment: The only firmly recommended therapy is renin angiotensin aldosterone system inhibition with captopril in particular. 97 Other experimental agents include antiendothelin inhibitors and TPE.…”

“…96,97 Treatment: The only firmly recommended therapy is renin angiotensin aldosterone system inhibition with captopril in particular. 97 Other experimental agents include antiendothelin inhibitors and TPE. 97 There have been some theoretical investigations and clinical reports of complement activation in SRC.…”

“…97 Other experimental agents include antiendothelin inhibitors and TPE. 97 There have been some theoretical investigations and clinical reports of complement activation in SRC. 109 Cancer-induced TMAs.…”