Rheum for One More on the Differential Diagnosis: Fibromuscular Dysplasia in the Context of Renal Infarct

1Icahn School of Medicine at Mount Sinai, New York, NY
2Mount Sinai Hospital, New York, NY

Meeting: Hospital Medicine 2015, March 29-April 1, National Harbor, Md.

Abstract number: 529


Case Presentation: 45 year old quadriplegic female with history of hypertension, SLE (APLS negative) with vasculitis on chronic steroids, CAD status post bare metal stents on chronic anti-platelet therapy, and suprapubic catheter presented with two days of persistent nausea, vomiting, and right flank pain. Her pelvic discomfort was different from that associated with her previous urinary tract infections. At presentation, she reported cloudy urine without visible blood. Vitals were notable for tachycardia and fever to 39.3 C. Admission labs were notable for leukocytosis, acute kidney injury, and the urinalysis demonstrated cloudy urine with red blood cells and white blood cells. She was started on antibiotics and CT Abdomen was obtained to evaluate pelvic pain. Results revealed that the majority of the right kidney was non-enhancing, suggestive of right kidney infarct.

This presentation of renal infarct in a younger woman with early-onset hypertension raised concern for fibromuscular dysplasia (FMD).  Review of angiogram for myocardia infarction four years prior demonstrated multivessel spontaneous coronary artery dissection (SCAD), rather than what was thought to be stenosis from atherosclerosis. To pursue the diagnosis of FMD further, the patient underwent a renal ultrasound, which demonstrated flow characteristics consistent with FMD. The patient was continued on anti-platelet therapy and labile blood pressures were treated with uptitration of calcium channel blockers and the initiation of an ACE-inhibitor.

Discussion: Acute renal insufficiency in the context of presumed urosepsis is a common clinical scenario for a hospitalist. The differential broadened in this case after considering idiosyncrasies in her past medical history, honing in on the cause of her renal infarction, and close review of imaging with consultants. There is significant variability in the presentation of FMD. Patients most commonly present with hypertension from renal artery involvement. SCAD is a rare manifestation of FMD, though the majority of SCAD patients also have FMD in at least one non-coronary arterial territory. The presence of SCAD warrants investigation for underlying FMD.

The diagnosis of FMD is confirmed by various imaging modalities including CTA, MRA, or duplex ultrasonography, the gold standard remaining catheter-based digital subtraction angiography. About 80% of cases will have the classic “string of beads” appearance on imaging.

There is limited evidence-based management of FMD. Medical management is preferred for stable patients consisting of antiplatelet therapy, blood pressure control with an ACEi/ARB, and a beta-blocker for cardioprotective effects.

Conclusions: This case underscores the importance of reviewing imaging studies with the radiologist and reevaluating old diagnoses in the context of new findings. SCAD secondary to FMD can be medically managed with angiographic intervention such as angioplasty or stenting reserved for unstable patients.

To cite this abstract:

Babus L, Chawla H, Barley K, Tremblay D, Goetz C. Rheum for One More on the Differential Diagnosis: Fibromuscular Dysplasia in the Context of Renal Infarct. Abstract published at Hospital Medicine 2015, March 29-April 1, National Harbor, Md. Abstract 529. Journal of Hospital Medicine. 2015; 10 (suppl 2). https://www.shmabstracts.com/abstract/rheum-for-one-more-on-the-differential-diagnosis-fibromuscular-dysplasia-in-the-context-of-renal-infarct/. Accessed April 4, 2020.

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