Undetected Underlying Endocrine Pathology leading to Polyomavirus-associated Nephropathy (PVAN) Secondary to Transplantation Arya Dadhania MS1, Jeffery Rebhun MD ¹ Department of Internal Medicine, University of Illinois at Chicago, Chicago IL,

Chief Complaint: Uncontrolled DIAGNOSIS CURRENT RESEARCH Table 1: Relevant differential diagnoses for the resistant secondary HTN leading to the diagnosis of the underlying endocrine pathology. ❖ Currently, PA is not diagnosed until in patients until third to sixth decade of life. The degree of History of Present Illness: 61 year-old African American female with a complicated past medical history, which includes chronic kidney disease (CKD) s/p living related kidney transplant (LRKT) in (2012 c/b Differential Diagnoses Diseased state findings Diagnostic modality Patient findings Diagnosis hypertension is usually moderate to severe, resistant to antihypertensives and hypertensive hyperacute rejection), DDKT (2017), hypertension (HTN), hyperlipidemia (HLD), diabetes mellitus, obstructive Pheochromocytoma[1] Elevated metanephrines Blood Test Normal emergencies are not uncommon. Several studies have shown that patients with may be at higher risk than other patients with hypertension for target‐organ damage of the and sleep apnea (OSA), cerebrovascular accident (CVA), presents for follow up of her resistant hypertension. Initial Liddle's syndrome[2] Low plasma Blood Test Elevated aldosterone [5,6] diagnosis of HTN was made in 1996 following a pregnancy complicated by eclampsia. The patient was started Cushing's syndrome[3] Excess Blood Test Normal cortisol Primary kidney. on Norvasc 10 at the time. She was subsequently lost to follow up. The patient is presently without complaint, Renal stenosis[4] Renal Brui Ultrasound Normal ultrasound aldosteronism ❖ A simple screening algorithm can be put in place for early detection of PA (Figure 5). Potential and denies any , visual changes, nausea, or vomiting. Endorses mild LE edema. Conn's syndrome (PA) candidates for PA must be further investigated with confirmatory tests and then worked up for subtype Primary Elevated plasma Bilateral idiopathic Blood Test Elevated aldosterone classification.[6] aldosteronism [3] aldosterone Past Medical History: adrenal hyperplasia CKD s/p LRKT and DDKT, Deep in UE, CVA (2005), HTN, GERD, HLD, Anemia of Chronic ❖ CT scan findings of unilateral lipid rich is consistent with Conn’s syndrome, type of PA (Figure 3). Renal Failure, Eclampsia (1996), Secondary Hyperparathyroidism, Abdominal Cavity Hernia, Vit D Deficiency. DISCUSSION of DISEASE PROCESS Past Surgical History: LRKT, 2012, DDKT 03/2017. Figure 5: PA screening using 1996 2012 2017 March 2017 2018 February 2018 2019 May Medications: plasma aldosterone • Eclampsia • LRKT • DDKT November • Hospitalization November • Benign • Hyperacute • Required • Transplanted for AVF arm • Hospitalization adenoma of concentration‐to‐plasma Nifedipine 90mg, BID Isosorbide mononitrate 60mg, QD rejection but extensive kidney core swelling. BP due to right adrenal activity ratio. PAC, plasma no frank endarterectom biopsy positive 179/76. hypertensive gland. evidence of y and patch for polyoma • Suspicion of urgency. BP • Normal salivary aldosterone concentration; PRA, Clonidine patch, 0.3mg/week Hydralazine 100mg, TID thrombotic angioplasty of virus. See hyperaldosteron 230/100. and microangiopat left external Figure 4. ism. • Controlled metanephreines. ; PRC, Carvedilol 50mg, BID 25mg, QD hy iliac artery. • Started on • Referred to CT after • Resistant 2° plasma renin concentration. IVIG and scan (3/2016) additional HTN due to Doxazosin 4mg, HS Prednisone 5mg, QD leflunomide. that showed drugs. PA. Clinical , Volume: small adrenal • Outpatient nodule. work-up for 66, Issue: 5, Pages: 607-618, resistant First published: 13 February secondary Allergies: Diphenhydramine. HTN. 2007, DOI: (10.1111/j.1365- 2265.2007.02775.x) Figure 1: Chronological representation of the disease process and diagnosis of the underlying endocrine pathology. Social History: Patient lives with children and is on disability. She has left-sided deficits and walks using a cane/walker 1-2 times per week. Consumed alcohol 1-2 times per month for 38 years, tobacco (smoking) for PVAN 50 years. Patient indicates past use of marijuana. Eclampsia HTN ESRD LRKT DDKT (Figure 4) Review of Systems: Negative except as per HPI. ______Figure 2: Visual representation of contracting PVAN in a 61 year old female because of underlying endocrine pathology. ❖CONCLUSIONS Primary aldosteronism is a serious endocrine pathology that is often diagnosed much later after its OBJECTIVE: Figure 3: Extending manifestation causing target-organ damage, most often kidneys and the heart. Primary aldosteronism from the medial limb usually presents with resistant hypertension, (less sensitive), metabolic acidosis, muscle of the right adrenal Vital Signs: BP: 142/69 Pulse: 66 Temp: 97.2 weakness, hypomagnesemia. Recommended treatment for unilateral hyperplasia/adenoma is laparoscopic gland, there is redemonstration of a adrenalectomy or mineralocorticoid receptor antagonist (MRA) while, MRA only for patients with bilateral Physical Exam: No remarkable findings. [7] 0.7 x 0.8 cm nodule idiopathic hyperaldosteronism. with a Hounsfield Labs/Imaging: unit of -7, compatible ❖REFERENCES with a benign 1. Lenders, Jacques WM, et al. "Plasma metanephrines in the diagnosis of pheochromocytoma." Annals of internal adenoma. The medicine 123.2 (1995): 101-109. native kidneys 2. Botero-Velez, Mauricio, John J. Curtis, and David G. Warnock. "Liddle's Syndrome Revisited--A Disorder of Reabsorption in the Distal Tubule." New England Journal of Medicine 330.3 (1994): 178-181. appears severely Allograft kidney ultrasound: unrevealing for renal artery stenosis. 3. Sukor, Norlela, et al. "Role of unilateral adrenalectomy in bilateral primary aldosteronism: a 22-year single center atrophic. There is a Figure 4: Biopsy of the DDKT showing the experience." The Journal of Clinical Endocrinology & Metabolism 94.7 (2009): 2437-2445. 0.5 cm hypodense CT Abdomen-Pelvis: A small 1.2 x 0.7 cm nodule is seen arising from the medial limb of the right presence of polyoma virus in tubular cell 4. Drieghe, Benny, et al. "Assessment of renal artery stenosis: side-by-side comparison of angiography and duplex focus within the with internal Hounsfield unit of -1, compatible with a lipid rich adenoma (Figure 3). nuclei. Based on the presence of mild ultrasound with pressure gradient measurements." European heart journal 29.4 (2008): 517-524. lower pole of the inflammation, mild tubular atrophy, mild 5. Funder, John W., et al. "The management of primary aldosteronism: case detection, diagnosis, and treatment: an right kidney and a interstitial fibrosis and moderate polyoma endocrine society clinical practice guideline." 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