scholarly journals Calciphylaxis: Temporal Artery Calcification Preceding Widespread Skin Lesions and Penile Necrosis

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Manzoor A. Shah ◽  
Michael W. Roppolo

Temporal artery calciphylaxis has rarely been described in chronic kidney disease patients on dialysis. We report a case of 72-year-old Caucasian man with multiple comorbidities and end-stage renal disease on dialysis who presented with temporal artery calcification leading to bilateral loss of vision followed by extensive skin lesions including one on glans penis. While on peritoneal dialysis, he developed anterior ischemic optic neuropathy, had no improvement on high dose steroids, and temporal artery biopsy showed marked calcification without any evidence of vasculitis. Few weeks later on hemodialysis, he developed widespread cutaneous lesions on extremities and penile necrosis with skin biopsy revealing calciphylaxis. On literature review of calciphylaxis in chronic kidney disease, we found only four cases of temporal artery calciphylaxis leading to anterior ischemic optic neuropathy and blindness. We believe this is the first case in which the rare temporal artery calciphylaxis and the uncommon penile necrosis are being described together. The objective is to emphasize the need to recognize this condition early in the CKD patients on dialysis presenting with visual symptoms as the different treatment strategies may help prevent complete loss of vision and also modify or prevent a full blown calciphylaxis.

Temporal arteritis (Giant cell arteritis) is an autoimmune systemic disease affecting the elderly population characterized by granulomatous vasculitis involving the mid-large size vessels. Classical systemic symptoms include headache, skin sensitivity in the temporal region, and jaw fatigue. Inflammation involving the posterior ciliary arteries leads to arteritic anterior ischemic optic neuropathy which causes sudden vision loss. Temporal artery biopsy is the gold standard for diagnosis and should be applied to all patients suspected of the disease. As early diagnosis and treatment are of vital importance in terms of improvement of visual loss and prevention of other ocular involvement, Optic neuropathy is considered to be a neuro-ophthalmologic emergency. The gold standard in treatment is steroids. There is no consensus in the literature regarding the optimal therapeutic approach in cases with steroid resistance. Biological agents, especially tocilizumab, are promising in refractory cases. Up-to-date steroids are the first step in the treatment algorithm, tocilizumab is recommended in the second line; however, in cases with progression, abatacept, ustekinumab, and anakinra are recommended as last-line treatments.


Antioxidants ◽  
2021 ◽  
Vol 10 (9) ◽  
pp. 1466
Author(s):  
Jia-Ying Chien ◽  
Yu-Yau Chou ◽  
Jhih-Wei Ciou ◽  
Fang-Yun Liu ◽  
Shun-Ping Huang

Nonarteritic anterior ischemic optic neuropathy (NAION) is one of the most common acute optic neuropathies that affect the over 55-year-old population. NAION causes the loss of visual function, and it has no safe and effective therapy. Bardoxolone methyl (methyl 2-cyano-3,12-dioxooleana-1,9(11)-dien-28-oate; CDDO-Me; RTA 402) is a semisynthetic triterpenoid with effects against antioxidative stress and inflammation in neurodegeneration and kidney disease that activates the nuclear factor erythroid 2-related factor 2 (Nrf2) signaling pathway. Moreover, RTA 402 is an FDA-approved compound for the treatment of solid tumors, lymphoid malignancies, melanoma, and chronic kidney disease. Omaveloxolone (RTA 408) is an activator of Nrf2 and an inhibitor of NFκB, possessing antioxidative and anti-inflammatory activities in mitochondrial bioenergetics. RTA 408 is also under clinical investigation for Friedreich ataxia (FA). In this study, a rodent anterior ischemic optic neuropathy (rAION) model induced by photothrombosis was used to examine the therapeutic effects of RTA 402 and RTA 408. Treatment with RTA402 results in antiapoptotic, antioxidative stress, anti-inflammatory, and myelin-preserving effects on retinal ganglion cell (RGC) survival and visual function via regulation of NQO1 and HO-1, reduced IL-6 and Iba1 expression in macrophages, and promoted microglial expression of TGF-β and Ym1 + 2 in the retina and optic nerve. However, these effects were not observed after RTA 408 treatment. Our results provide explicit evidence that RTA 402 modulates the Nrf2 and NFκB signaling pathways to protect RGCs from apoptosis and maintain the visual function in an rAION model. These findings indicate that RTA 402 may a potential therapeutic agent for ischemic optic neuropathy.


2020 ◽  
Vol 223 (1) ◽  
pp. 109-112 ◽  
Author(s):  
Robert M Verdijk ◽  
Werner J D Ouwendijk ◽  
Robert W A M Kuijpers ◽  
Georges M G M Verjans

Abstract Background To test the hypothesis that varicella-zoster virus (VZV) infection contributes to temporal arteritis pathogenesis, comprehensive in situ analysis was performed on temporal artery biopsies of 38 anterior ischemic optic neuropathy (AION) patients, including 14 (37%) with giant cell arteritis. Methods Biopsies were completely sectioned, and, on average, 146 serial sections per patient were stained for VZV glycoprotein E. Results Four of 38 AION patients showed VZV glycoprotein E staining, but VZV infection was not confirmed by staining for VZV IE63 protein and VZV-specific polymerase chain reaction on adjacent sections. Conclusions This study refutes the premise that VZV is casually related to AION with and without giant cell arteritis.


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