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Diagnose This (August 27, 2018)

L. Nich Cossey, MD
By L. Nich Cossey, MD

Aug 27, 2018

Scleroderma in a renal biopsy at Arkana Laboratories
Camera information: Calibration= 1.000 Pixels per pixel; Capture format= 1392 x 1040, Full Frame HQ; Gamma= 0.64; Gain= 1.0 x; Exposure= 11.7 ms; Auto exposure= On; Image type= Colour; Shading= (None); Sharpening= Off; Black clip= 10; White clip= 230; Microscope information:

What is this finding and what is the likely etiology?

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The photomicrograph shows a portion of an artery with mucoid intimal edema, a change associated microangiopathic injury. This finding can be seen in the setting of thrombotic microangiopathy, especially cases of accelerated hypertension. In this patient accelerated hypertension was present as was a positive ANA, SCL70 and clinical features consistent with scleroderma making scleroderma renal crisis the most likely etiology of her accelerated hypertension.

Importantly, mucoid intimal edema can cause severe to complete luminal occlusion that does not recover after the initial insult as the mucoid intima often collagenizes and the luminal occlusion persists. This can lead to downstream renal ischemia, further renal dysfunction, and sometimes progression to ESRD despite clinical control of the severe hypertension. This sequence was events was seen in this case as the patient’s initial accelerated hypertension was controlled but her serum creatinine continued to climb despite supportive care and at last check she was dialysis dependent.

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Quick note: This post is to be used for informational purposes only and does not constitute medical or health advice. Each person should consult their own doctor with respect to matters referenced. Arkana Laboratories assumes no liability for actions taken in reliance upon the information contained herein.