Case of Mixed Connective Tissue Disease Presenting with Scleroderma Renal Crisis

NKF 2017 Spring Clinical Meetings Abstracts Gustavo Borda, Ashfaq Balla. Metrowest Medical Center, Framingham, MA, USA CALCIPHYLAXIS…NOTHING LAX ABOUT IT! Ravina Patel, 59 Harold Sevelamer DiffuseMedical CrystalCenter, Colitis: A Case Szerlip, Induced Baylor University Dallas, TX, Report USA of a Rarely Reported Side Effect Calcific uremic arteriolopathy, commonly known as calciphylaxis is Samir Brahmbhatt, Michael Cruise, James Simon. Cleveland an acquired skin condition that affects patients on hemodialysis. Clinicdeposits Foundation, Cleveland, OH, USA Calcium in the dermal or fat arterioles leading to skin ischemia necrosis. The pathophysiology poorly understood,Attributed however 61 andAn Unusual Case of isHyponatremia to derangements in calcium- phosphate metabolism, warfarin use, and Trimethoprim-Sulfamethoxazole Therapy vitamin D supplementation may all play a role. It is imperative to recognize skin lesions as they increase both mortality Adam these Bsiso, Michael Sheffield, Robertoand Collazomorbidity. Maldonado. Methodist Dallas Medical Center, Dallas, TX, Our patient is a 70 year old female with Chronic Kidney Disease, USA Stage 4 who presented with confusion and sepsis. Patient complained 62 of Enteroccous CausingtheBacterial-Endocarditis severe leg pain.Species Prior to admission patient was ambulatory, Assohowever becoming more to “pain and deep ciatedprogressively Glomerulonephritis in anbedbound At Riskdue Population ulcers.” Wound care treatments at her rehab were ineffective. Patient Emma Bueno, Keyrillos Rizg, Mario Cisneros, Juan Chique denied any new medications, such as warfarin. Figueroa, Ryanheart, Kunjal, Andreea Raafat On physical exam: lungs, abdomen werePoenariu, benign. Her skin had F light purple to violet colored, tense, thickened,College plaque likeoflesions Makaray. University of Florida Medicinelocated on her R inner thigh, L inner thigh, and L posterior calf. Jacksonville, Jacksonville, FL, USA Lesions were exquisitely tender. 65 Pertinent Recurrent Microangiopathy Renal labsThrombotic included K of 6.8, HCO3 of 18, BUNinof a220, Cr of Allo5.23, calcium 7, phosphorus 2.5, PTH 1157, and a ca-phos product was graft: A Clinical Conundrum normal. For imaging, a three phase Technetium scan demonstrated Olivia Campa, Jose A. Morfin. University of California calcification within the subcutaneous tissues of the proximal thighs Davis, with Sacramento, CA, USA consistent calciphylaxis. was initiated on hemodialysis during this admission due to 68 Patient A Rare Case of Neonatal Bartter Syndrome uremic encephalopathy. Patient received 4 doses 1 2 of 1 sodium thiosulfate Tushar Chaturvedi , Rupesh Raina Akron Nephrology with hemodialysis and wound care. Initially she. had improvement of 2 Akron, OH, USA;afterCleveland herAssociates, lesions, however she died 2 months diagnosis. Clinic Akron Affecting 1-4% of End Stage Renal Disease patients, calciphylaxis is General, Akron, OH, USA rare. Even rarer is to find its occurrence in patients not yet on 71 hemodialysis An Unusual Case of Negative in a Patient like this patient. The exactAnion etiologyGap of calciphylaxis is with Hypercalcemia poorly understood so diagnosis and treatment options are limited. Early recognition order to delay majorThodima, complications such Raja. as Avantikais important Chenna,inPradeep Reddy Rasib infections, sepsis, and amputations. Ultimately though, this complex Albert Einstein, Philadelphia, PA, USA condition tends to take the lives of those affected. 74 Atypical Presentation of Metastatic Esthesioneuroblastoma with Ectopic ACTH Syndrome After 7 Years 234Vamsi Chilluru, Jay Hawkins. University of Nebraska Medical Center,CONNECTIVE Omaha, NE, USA CASE OF MIXED TISSUE DISEASE 86 PRESENTING Prosthetic WITH Valve SCLERODERMA Endocarditis RENAL from CRISIS Mycobacterium Patel, Komal Rosenstock, Chimaera InfectionJordan Causing Granulomatous Interstitial Northwell Health Lenox Hill Hospital, New York, NY USA Nephritis Mixed connective tissue disease (MCTD) is an overlap disorder comprised of a combination of multiple connective tissue Peter Jonathan Da Costa, Ahmed Abdalrhim, Ivan Porter, diseases including systemicCortese, lupus erythematosus, polymyositis, Fitzpatrick, Cherise Nabeel Aslam. Mayo Clinic, Sjogren’s syndrome, systemic sclerosis and arthritis. Renal involvement Jacksonville, FL, USA in MCTD occurs in 10% of patients, and most commonly presents as 87 membranous Health ornephritis Beauty:andAinCase of an Unusual Cause Severe very rare cases can present with of renal vasculopathy which is characteristic of scleroderma. This case involves Hypercalcemia a 47 year old Saudi Arabian female whom presented to the clinic with Gulshan Ariyaratne, Hilmer blood pressureDangol, of 160/100Sashi and creatinine of 2.2mg/dl fromNegrete. baseline of St. Youngstown OH, nausea, USA 0.5Elizabeth mg/dl 2 months prior. She Hospital, complainedYoungstown, of frontal headaches, oral intake, lower extremity edema. had aCases history with of 88 decreased Recurrent Denseand Disease: A Report ofShe Two MCTD with Raynaud’s syndrome and was being treated with Varying Presentations, Pathologic Findings and Patient methotrexate and methylprednisolone which had controlled her Outcomes symptoms. On examination, bilateral non-pitting lower extremity edema and swelling in herSharon fingers were noted. She did notEllis. have a Emory rash, Dilini Daswatta, Graves, Carla joint inflammation, nor evidence of skin tightening consistent with University, Atlanta, GA, USA scleroderma. Urinalysis showed 1+ protein, negative for blood, WBC, 90 andCrystalglobulin-Induced Nephropathy Keratopathy RBC. Urine protein creatinine ratio was 0.7 and and FeNa of 1.3%. Patient was started immediately on captopril every Matthew D’Costa, Sandhya Manohar, Joe 12.5mg Grande, Samih eight hours titrated to a blood pressure of less than 140/90. Patient’s Nasr, Marie C. Hogan. Mayo Clinic, Rochester, MN, USA autoimmune workup revealed positive ANA and negative for ds-DNA, 91 scleroderma Vitamin C Inducedrheumatoid Oxalate Nephropathy antibodies, factor, anti-ribonuclear protein, and antiphospholipid antibody. Patient’s were within normal Matthew D’Costa, Loren PT/INR/PTT Herrera Hernandez, Sandra limits. Blood pressure improved but her creatinine continued to rise to Herrmann. Mayo Clinic, Rochester, MN, USA 2.08mg/dL. Patient subsequently underwent renal biopsy which arterial onion skin lesions and vasculopathy consistent 92 showed A Case of Severe Hyponatremia and Acute Kidneywith Injury scleroderma renal crisis. There were no immune deposits or other Eddy DeJesus, Nasr, Rabih. Bronx Lebanon Hospital evidence of glomerulonephritis. Center, Bronx, NY, USA This case illustrates that MCTD can rarely present with hypertension, thrombotic microangiopathy, of 103 syndrome Acute of accelerated Glomerulonephritis Post Administration and acute kidney injury consistent with scleroderma renal crisis even Pegfilgrastim without a diagnosis of scleroderma. It is important to be cognizant of Hatem Elabd, Ansari, Belinda Jim. Jacobi Medical this possibility whenNaheed caring for such patients. Center, New York, NY, USA

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108 Refractory Hypomagnesemia During Pregnancy in a Patient with Gitelman Syndrome CROSSED HEMI-HYPERTROPHY WITH FLANK PAIN: A Thomas Frohwein, Susan Kim, SYNDROME Isaiarasi Gnanasekaran. CASE OF BECKWETH-WIEDEMANN AND Lincoln Medical and Mental Center, Bronx, NY, RECURRENT BILATERAL RENALHealth CALCULI Aditya S Pawar, Wisit Cheungpasitporn, Thomas R Schwab, Stephen B USA Erickson 110 Diagnosis and Treatment of Renal Vein Thrombosis (RVT) Mayo Clinic, Rochester, MN, USA. in the Setting of Lupus Nephritis and Antiphospholipid Beckwith-Wiedemann syndrome (BWS), a congenital condition Syndrome (APS) classified as an overgrowth syndrome, has a multitude of clinical manifestations including body size and organs. Its renal Pablo Garcia, Felixlarge Renneberg, Maanit Kohli, Steve Bibu, abnormalities include structural and nephromegaly. We Navya Kuchipudi, Anneanomalies Van Hoven, Shaunak Dwivedi. with bilateral nephrocalcinosis and present a rare presentation of BWS Saint Peter’s University Hospital, New Brunswick, NJ, USA recurrent kidney stones. 111A Scleroderma Renal in the Setting of Systemic 70-year-old male withCrisis known(SRC) BWS presented to the hospital with Sclerosis Sine Scleroderma (ssSSc) Pregnancy hadinfrequent stone passages recurrent bilateral flank pain. The patient in the past 45Garcia, years. Physical examination showed crossed Pablo Shaylika Chauhan, Maanit Kohli, Navya hemihypertrophy; arm andDwivedi. the contralateral were larger than Kuchipudi, one Shaunak Saint legPeter’s University their counterparts. Abdomen CT without contrast showed extensive Hospital, New Brunswick, NJ, USA bilateral medullary nephrocalcinosis and bilateral kidney stones, 121 Red with Eyesmedullary and Renal in Astone Middle Age spongeInsufficiency kidney. The largest measures consistent 1.4 xGentleman 1.0 cm in the lower pole of the right kidney [Figure1]. The analysis of the Gondal, passed stones wasMae 100% calcium Jeffery phosphate (apatite). Maryam Heidi Timbol, Turner. Yale His blood test revealed creatinine 1.1 mg/dL, Na 144 mmol/L, K 3.5 University, New Haven, CT, USA mmol/L, Cl 96 mmol/L, HCO3 28 mmol/L, Ca 9.6 mg/dL and Phos 3.6 125 Atypical of7.5. Behcet’s Leading to IGA mg/dL. His urineCase pH was 24-h urineDisease (volume of 3.4 L) was supersaturated with hydroxyapatite and brushite crystals. Otherwise, Nephropathy the patient alsoGupta, had hypocitraturia (120Papanagnou, mg/spec) with Yorg normal Al-Azzi. urine Sanjeev Anastasios calcium (173 mg/spec). Thus, the patient was diagnosed with BWS and Westchester Medical Center, Westchester, NY, USA medullary sponge kidney with bilateral nephrocalcinosis/kidney stones. 126 Cefepime and Toxicity Presenting as started. StatusAtEpilepticus in a Chlorthalidone potassium citrate were 1 year follow-up, his stone passages had considerable Patient with End Stage Renaldecreased. Disease Physicians should1,beJayaprakash aware of rareDasari manifestations medullary 1 1 , Aziz of Bakhous , Raed Mohit Gupta Such sponge kidney 1 and nephrocalcinosis1in patients with BWS. 1 Azzem , Donald Dumford , Patrick Gallegos , Rupesh complications can lead to recurrent bilateral kidney stones and CKD. Raina1. 1Cleveland Clinic Akron General, Akron, OH, USA 129 Tenofovir Causing Hospitalization Due to Severe Symptomatic Hypophosphatemia Sanjeev Gupta, Anastasios Papanagnau, Savneek Chugh. 236 Westchester Medical Center, Valhalla, NY, USA INCIDENCE AND TYPES OF KIDNEY STONES IN PATIENTS 130 The Significance of Anti-Phospholipase Antibodies in a WITH HORSESHOE KIDNEYS: A META-ANALYSIS Patient with Membranous Nephropathy Aditya S Pawar, WisitJayaprakash Cheungpasitporn, Michael A Mao, Stephen B Mohit Gupta, Dasari, Rupesh Raina, Pallavi Erickson Reddy. Cleveland Clinic Akron General, Akron, OH, USA 132 A Clinic, Mystery Case ofMN, Blood Mayo Rochester, USA.Leak Alarm Going On Seifeldin Hakim, Rhyan Maditz, Sadichhya Lohani, Sami The horseshoe kidney isUniversity the most common typeBeaumont of renal fusion Zarouk. Oakland William Hospital, anomaly. The incidence and characteristics of kidney stones in patients Oak, MI, USA withRoyal horseshoe kidneys are not well studied. The aim of this meta133 Previously Undiagnosed ANCA Vasculitis analysis was to evaluate the incidence and Associated types of kidney stones in Prepatients with One horseshoe kidneys. senting Month After Kidney Transplantation A systematic literature search was performed using MEDLINE, Ayman Hallab, Karthik Kannegolla, Mohammad S. Yaqub. EMBASE, and Cochrane Database of Systematic Reviews from the Indiana University School of Medicine, Indianapolis, IN, databases’ inception through November 15, 2016. Studies assessing the USA and types of kidney stones in patients with horseshoe kidneys incidence wereCase included. We applied model to estimate the 149 Report of aa random-effects Dialysis Dependent Immunotactoid incidence of kidney stones. study protocol was registered with Glomerulopathy withThe Favorable Response to Rituximab PROSPERO (International Prospective Register of Systematic 1 , Rohan Bhojwani2, Glen Markowitz3, Aaron Mehak Reviews; no.Idrees CRD42016052037). 4 1 2 Dommu . Griffin Hospital, USA; Results: Eleven observational studiesDerby, with 715CT, patients (335 Fairview adults and Hospital, 380 pediatric) with horseshoe kidneys3Columbia were enrolled. The estimated University, New Cleveland, OH, USA; 4 was 59% (95% CI, 18%–95%) in adults incidence kidney stones York,ofNY, USA; Nephrology Associates, Bridgeport, CT, with horseshoe kidneys. Kidney stones were less common in pediatric USA patients with horseshoe kidneys with an estimated incidence of 3% 150 DeCI,Novo Atypical Hemolytic Uremic Syndrome Fourteen (95% 1%-5%). The mean age of adult stone formers with horseshoe kidneys was 46.9 ± 5.9 years. Within reported studies, 91.5% of kidney Months After Renal Transplant stones were calcium-based stonesThompson, (62.1% CaOx, 22.6%Aurora CaP, 6.8% Olusola Isikalu, Jennifer Maria Posadas mixed CaOx/CaP), followed by uric acid stones (4.3%), struvite stones Salas. Medical University of South Carolina, Charleston, (3.8%) and others (0.4%). SC, USA Kidney stones are very common in adult patients with horseshoe kidneys with Lupus an estimated incidence of 59%. CalciumHistopathological based stones are 151 “Lone” Nephritis: A Seronegative, the most common kidney stones in adults with horseshoe kidneys. Diagnosis These findings may impact the prevention and clinical management of Jamal Janjua, Pratima Kamada,kidneys. Aiman Riaz. Gundersen kidney stones in patients with horseshoe Health System, La Crosse, WI, USA

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